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Large Unilateral Neck Mass in Submandibular Region
J Oral Maxillofac Surg 70:842-850, 2012
Large Unilateral Neck Mass in Submandibular Region Adam Weiss, DDS,* Garry Shnayder, DDS,† Jonathan Tagliareni, DDS,‡ Edmund Wun, DDS,§ Earl Clarkson, DDS,储 and Harry Dym, DDS¶ ical examinations, including thyroid hormonal evaluation, were within normal limits. The patient’s chest x-ray showed no abnormalities. A panorex radiograph of the patient was taken and showed no obvious odontogenic sources of infection. The patient was sent for a magnetic resonance imaging (MRI) study. A T1-weighted MRI of the facial bones, orbits, and paranasal sinuses was performed without contrast using 5-mm contiguous helically obtained axial images. As shown in Figures 3 and 4, the MRI showed a bright mass that was mostly lateral and inferior to the mandible; however, a projection of the mass extended medially and superiorly compressing the pterygoid muscle and displacing the right submandibular gland inferiorly. A mass effect was noted particularly on the lateral submandibular gland, which was deviated medially and was somewhat compressed. After gadolinium administration, no abnormal enhancement was seen.
A 44-year-old male presented to the oral and maxillofacial surgery clinic at The Brooklyn Hospital Center. The patient’s chief complaint was an enlarging asymptomatic mass to the right side of his neck in the submandibular region, as shown in Figures 1 and 2. The patient noticed the mass 10 years ago but noticed a slow increase in size in the past 5 years. The patient’s medical history was significant for hypertension, asthma, and obesity. The patient denied any surgeries in the past and any drug and alcohol use. However, the patient did admit to being a 25-year pack-a-day cigarette smoker. The patient was afebrile and not in acute distress. A comprehensive head and neck examination showed a 6.5 ⫻ 4.5 ⫻ 2 cm doughy and well-circumscribed mass in the right submandibular region extending down into the neck. Extraorally, there was no discharge, no breakdown, and no erythema. The mass was not warm to the touch and the mandibular division of the fifth trigeminal nerve was intact bilaterally. There was no palpable lymphadenopathy omolateral or contralateral to the lesion. The patient had no complaints of restricted neck movement. Intraorally, there was no trismus and no gross caries seen. The patient was missing teeth 30 and 32, which were extracted many years ago. The patient’s uvula was at the midline; there was no floor of the mouth or vestibular swelling and no purulent drainage noted intraorally. The patient showed normal salivary flow. Routine hematologic and biochem-
Differential Diagnosis A good rule of thumb when evaluating neck masses is to use the “rule of 80,”1 which pertains to adults over the age of 40. This rule states that 80% of nonthyroid neck masses are neoplastic. Of the neoplastic masses, 80% are malignant. Of the malignant masses, 80% are secondary. Of the secondary masses, 80% occur above the clavicle. A secondary way of predicting the diagnosis based on duration would be to use the “rule of 7’s.”1 If a mass has been present for 7 days, it is likely inflammatory in nature; if present for 7 months, it is likely neoplastic in nature, and if present for 7 years, it would most likely be developmental. Despite these generalizations, it is important to obtain an accurate history and perform a thorough examination when diagnosing neck masses. A thorough examination of the oral cavity, nasopharynx, and the larynx is critical. The clinician should evaluate both sides of the neck to estimate the dimension of the mass. Determining the exact region of the neck in which the mass is located is important and asking the patient to turn the head from left to right helps assess painful versus nonpainful masses. The color and temperature of the skin overlying the mass may also
Received from The Brooklyn Hospital Center, Department of Oral and Maxillofacial Surgery, Brooklyn, NY. *Senior Resident in Oral and Maxillofacial Surgery. †Fellow in Oral and Maxillofacial Surgery. ‡Fellow in Oral and Maxillofacial Surgery. §Chief Resident in Oral and Maxillofacial Surgery. 储Director. ¶Chairman. Address correspondence and reprint requests to Dr Weiss: The Brooklyn Hospital Center, Department of Oral and Maxillofacial Surgery, 121 Dekalb Ave, Brooklyn, NY 11201; e-mail: [email protected] © 2012 American Association of Oral and Maxillofacial Surgeons
0278-2391/12/7004-0$36.00/0 doi:10.1016/j.joms.2011.02.126
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FIGURE 1. Preoperative presentation showing large mass in the right submandibular region. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
provide an important diagnostic clue. The age and gender of the patient and duration and rate of development, as well as palpation and auscultation, are primary considerations in arriving at a differential diagnosis. A cranial nerve examination is also important because abnormal findings can indicate nerve involvement by tumor and a poorer prognosis. Fineneedle aspiration (FNA) biopsy is an inexpensive, rapid, and relatively accurate diagnostic tool for evaluating neoplastic and nonneoplastic lesions, especially in superficial or easily palpable masses. FNA is not without error and should not replace sound clinical judgment.2 Neck masses can be categorized into the 3 following categories: inflammatory, neoplastic (benign, malignant), and congenital. Inflammatory masses are the most common cause for a neck swelling in children and young adults. Patients presenting with inflammatory neck masses often present with a fever and complain of discomfort. The neck mass with accompanying pain may have an odontogenic or nonodontogenic origin. The neck masses can occur from acute or chronic lymphadenitis, may have a viral origin (cytomegalovirus and mononucleosis), bacterial origin (parotid sialadenitis, cat scratch disease), parasitic origin (toxoplasmosis), or can be a manifestation of a granulomatous disease (sarcoidosis, scrofula). In individuals over the age of 40, neck masses are most commonly neoplastic in nature. Both benign
843 and malignant neck masses can be encountered. Lipomas are a type of benign neoplasm frequently encountered in the neck region.3 Neurofibromas in the neck may occur as solitary tumors or as part of neurofibromatosis (von Recklinhausen’s disease). Schwannomas can appear very similar clinically to solitary neurofibromas. The main difference between these 2 tumors is that the nerve usually enters a neurofibroma centrally, whereas the schwannoma tumor cells always stay on the outside of the nerve.4 Benign salivary gland tumors, such as pleomorphic adenoma, usually appear as a unilateral, asymptomatic mass that generally is solitary, freely mobile, and slow growing. Desmoplastic fibromas usually occur in the posterior cervical triangle. They originate from the roots of the first or second cervical nerves and are hard, fixed lesions. The clinician may also encounter a carotid body tumor, which is a painless lateral neck mass that is more movable laterally than vertically. This compressible mass may be pulsatile or present with a bruit.5 Glomus jugulare are solid, fixed tumors and are usually found in the bifurcation of the carotid artery.6 This can actually displace the internal and external carotid arteries.
FIGURE 2. Preoperative presentation showing large mass in the right submandibular region. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
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FIGURE 3. T1-weighted MRI taken in the axial view. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
Malignant tumors of the neck can originate from tissues in the surrounding region or may present as a metastasis from a tumor from a distant site. Some of the common primary malignant tumors causing cervical nodal distant metastases are those originating from the thyroid, breast, lungs, and colon. It is critical to examine the cervical lymph nodes during the head and neck examination when malignancy is suspected. Metastatic lesions may present in the neck region with an unknown primary lesion. These unknown head and neck primaries are generally squamous cell carcinomas. Also, squamous cell carcinomas of the tonsillar region are frequently manifested as solitary neck masses with cystic lesions that affect the lymph node. Patients with malignant cervical adenopathy with no immediately apparent primary tumor represent 3% to 10% of all head and neck cancers.7 Hodgkin’s lymphoma and non-Hodgkin’s lymphoma are frequently found as neck masses. Clinically it is very hard to make a distinction between the 2. Neck lymphomas usually present with fever, fatigue, and malaise and are often found in the posterior triangle.8 Lymphomas can occur on both sides of the neck and can be either small and hard or large and soft on palpation. Lymphomas may present in any age
group, but they are the most common malignant tumors of the head and neck in the pediatric population, representing 10% of all malignancies.9 Salivary gland tumors are another category of malignant tumors that can present as neck masses. The most common that present as a primary or metastatic lesion in the neck are mucoepidermoid carcinoma and adenoid cystic carcinoma. Mucoepidermoid carcinoma is the most common salivary gland malignancy and the second most common malignancy of the submandibular gland. Adenoid cystic carcinoma is the second most common malignant salivary gland tumor and the most common in the submandibular gland. Skin cancers can also spread and present as neck masses. These are the most common malignancies in the United States. Like other head and neck malignancies, lymphatic spread is associated with decreased survival.10 Cutaneous head and neck and rarely intraoral melanomas also frequently give regional metastases to the neck.11 Finally, the third category that can be divided into congenital and developmental neck masses are the rarest. Examples of congenital cysts that can present as neck masses include dermoid cysts, epidermoid
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FIGURE 4. T1-weighted MRI taken in the sagittal view. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
cysts, and teratomas. Dermoid and epidermoid cysts are mostly found at the midline of the floor of the mouth.12 Teratomas are rare, usually found at birth, can cause respiratory difficulty, and can occur anywhere on the neck. Lymphangiomas or cystic hygromas present as soft, diffuse, multicystic compressible masses. These masses are usually found during infancy and are rarely seen during adulthood. Branchial cleft cysts of the neck are usually developed from the second branchial arch. They are often found anterolaterally to the jugular vein and carotid artery and are typically located anterior to the sternocleidomastoid muscle. Usually present as asymptomatic unless infected, they therefore can achieve a considerable size before they are diagnosed.13 The most common developmental cyst found in the neck is the thyroglossal duct cyst. The typical presentation of the thyroglossal duct cyst is a large mass in the midline of the neck below the hyoid bone that moves with swallowing or protrusion of the tongue. Cysts of the thyroglossal duct can be found from the foramen cecum at the base of the tongue to the pyramidal lobe of the thyroid gland.14 Hemangiomas are often present at birth and rapidly proliferate in the first years of life, usually
followed by a slow involution. These lesions are more common in females (3:1) and 60% are located in the head and neck region.15
Surgery Performed and Subsequent Course The risks, benefits, and alternative treatments were discussed with the patient. Treatment options included doing nothing or excision of the lesion, and benefits included removal of source of discomfort. Risks were discussed with the patient, including paresthesia, motor nerve paralysis, bleeding, swelling, pain, and infection. The patient elected to have the lesion excised under general anesthesia. The patient was brought to the operating room and placed under general endotracheal anesthesia. The patient was prepped and draped in a sterile fashion. A no. 15 blade was used to make the initial submandibular skin incision extending to the most inferior portion of the extended lesion. Blunt dissection was performed down to the level of the lesion as shown in Figure 5. Dissection was then performed down to the level of the lesion capsule at which point intracapsu-
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FIGURE 5. Intraoperative photograph showing blunt dissection of the mass. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
lar dissection was performed until the mass was freed in all aspects, medially, laterally, inferiorly, and superiorly. The mass was dissected from the right submandibular gland, which it displaced inferiorly and medially, as shown in Figure 6. No attempt was made to evacuate the contents of the lesion. The mass was removed in total and measured approximately 9 cm in length and 5 to 6 cm in width, as shown in Figure 7. As seen in Figure 8, the mass was submerged in formalin and floated, confirming the diagnosis of a lipoma. The specimen was sent to pathology for further study. The surgical site was irrigated with copious amounts of sterile saline. A no. 7 Jackson Pratt drain was inserted and secured with silk sutures. Closure was completed and a telfa dressing and bacitracin was applied to the wound. The patient was extubated and transported to the recovery room in stable condition. The patient was admitted and stayed
FIGURE 7. Surgical specimen. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
overnight for observation. A noneventful postoperative course was observed and the patient was discharged the day after the surgery was performed.
Pathology Diagnosis The histological diagnosis of this mass was a lipoma. Lipomas have a characteristic histological appearance. These benign tumors are histologically similar to adipose tissue; however, they are differentiated by the presence of a fibrous capsule.16 As shown in Figure 9 representing the histology of the sample submitted from our case, lipomas are composed microscopically of mature white adipose tissue arranged in lobules, many of which are surrounded by a deli-
FIGURE 6. Intraoperative photograph showing the relationship of mass to right submandibular gland.
FIGURE 8. Specimen shown floating in formalin solution.
Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
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FIGURE 9. Microphotograph of specimen shown at high power view showing mature adipose tissue arranged in lobules. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
cate fibrous capsule. These tumors frequently exhibit varying numbers of collagen strands coursing through the lesion and one will occasionally observe small blood vessels as well. When this fibrous connective tissue forms a more significant portion of the tumor, the lesion is then described as a fibrolipoma.
Discussion The lipoma has rarely been reported in the oral cavity, larynx, and pharynx. It occurs more frequently in other areas, in particular, the subcutaneous tissues of the neck.17 Lipomas are slow growing, nearly always benign tumors that are well-circumscribed tumors of mature adipose tissue. On gross examination, they are smooth, well circumscribed, lobulated, greasy to the touch, and yellow to orange in color. The epithelium is usually very thin and the superficial blood vessels are readily visible over the surface. Lipomas are the most common neoplasms of mesenchymal origin, arising in any location where fat is normally present. Solitary lipomas are generally more common in women, whereas multiple tumors (referred to as lipomatosis) are more common in men.18 Benign lipomatous tumors have been subclassified according to their histological features and growth pattern into classic lipomas (solitary or multiple), fibrolipoma, angiolipoma, infiltrating lipoma, intramuscular lipoma, hibernoma, pleomorphic lipoma, lipoblastomatosis, and diffuse lipoblastomatosis.19,20 Lipomas can also be further classified based on either size or weight. A tumor is classified as a giant lipoma if the size is greater than 10 cm in 1 dimension or the weight is greater than 1,000 g.21
847 Lipomas are rare in the first 2 decades of life, usually developing in the fifth and sixth decades when fat begins to accumulate in inactive, underexercised individuals. In general, this tumor is more commonly found in obese people and can increase in size during a period of rapid weight gain.22 According to Das Gupta,23 obesity and local growth of adipose tissue may both be responsible for the formation of a lipoma. This is known as the hypertrophy theory. In contrast, the metaplasia theory holds that lipomatous development represents the abnormal differentiation in situ of mesenchymal cells into lipoblasts.24 However, others have suggested that trauma and chronic irritation may cause the proliferation of soft tissue and play a part in the formation of a lipoma.25 Lipomas usually occur sporadically, but rarely they can be associated with several inherited disorders, including hereditary multiple lipomatosis, Gardner’s syndrome, and Madelung’s disease.26 Hereditary multiple lipomatosis is characterized by widespread, symmetric lipomas appearing most often over the extremities and trunk.27 Gardner’s syndrome is an autosomal-dominant disorder that can also be associated with lipomatosis and is characterized by intestinal polyposis, cysts, and osteomas.26 Madelung’s disease or benign symmetric lipomatosis involves an unusual distribution of fatty tissue surrounding the cervical region, shoulders, and proximal upper extremities. Patients with this disease are often longterm alcoholics and present with the characteristic “horse-collar” cervical appearance.28 There are different imaging modalities that can be used in confirming the preoperative diagnosis of a lipoma. The computed tomography (CT) can distinguish cystic from solid lesions. A CT scan provides a definitive diagnosis of lipoma in almost all cases by calculating the actual density of the suspected mass (via the CT attenuation number). Fat, being the only soft tissue with a density less than water, has a negative CT attenuation number. A lipoma will characteristically appear as a homogeneous mass with few septations, low CT attenuation number, and no contrast enhancement. MRI and CT not only show the lipomatous nature of the tumor preoperatively but also accurately determine its exact size, location, and extensions. This is critical in determining a preoperative surgical plan. The MRI as used on the patient described in our case has an advantage over a CT in its soft tissue capabilities and it is useful when the distinction between the mass and surrounding soft tissue is poor. It is important to understand that neither CT nor MRI can differentiate a lipoma from a liposarcoma.29 This distinction can only be made with certainty by a careful histopathological examination. Ultrasound is another study that is readily available,
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inexpensive, noninvasive, and accurate that can be used in determining the nature of unknown neck mass.30 Lipomas may present in the neck as large isolated masses present for long periods without much change over the years. The treatment of these benign tumors is a simple excision. The recurrence of these tumors is rare.31 True lipomas recur in only 1% to 2% of cases after adequate excision, so a recurrence should raise the suspicion of a liposarcoma, especially if the tumor is not superficially located.32 Lipomas tend to form in the subcutaneous tissue, which is in contrast to the liposarcoma that forms from the deeper tissues, an important clinical distinction. Upon performing a PubMed search for head and neck lipomas, it is evident that literature on head and neck lipomas is limited and has mostly been in the form of separate case reports. We have highlighted a few of the cases published in the literature to show the variability in size, location, and type that these tumors can present with in the head and neck region. There is a report of a 55-year-old obese female that presented with soft, nontender chin swelling.33 Intraoral examination showed a large fixed mass filling the lower sulcus between the right lateral incisor and the left second molar covered by slightly inflamed mucosa. The tumor was resected and the diagnosis was submucosal and intramuscular benign lipoma. Rarely, a lipoma has been known to infiltrate surrounding tissues, most notably skeletal muscle, in which case it is referred to as an infiltrating lipoma. Do et al34 reported a case of a 22-month-old boy with a history of painless swelling in the right submandibular area for 2 months that rapidly enlarged. CT showed a 5 ⫻ 5-cm expansive mass with a clearly demarcated low density at the right submandibular area. The histopathologic diagnosis was a lipoblastoma. Lipoblastoma is a rare, benign tumor arising from embryonic white fat cells and usually occurs in infants younger than 3 years or in childhood.35 This type of tumor typically presents as a rapidly enlarging, painless mass and the clinical symptoms are mostly associated with tumor size and anatomical location.36 There are also several reports of lipomas of the parotid gland. One such report involves a 44-year-old female with a 3-year history of a slowly enlarging left preauricular mass that suddenly had become painful and tender.37 The lipoma was not attached to the overlying skin and appeared to be in the superficial lobe of the left parotid gland. Another case report describes a 47-year-old male with a deep lobe parotid gland lipoma.38 A physical examination showed a 4 ⫻ 3-cm soft and painless mass in the left parotid region. Lipomas account for 2% to 3% of the benign parotid tumors.39 Interestingly, Rosado et al report a case of a 73-year-old woman with a 2-year history of a gradually
enlarging, painful mass located in the left parotid region.40 Surgical excision was performed, obtaining a red-wine-colored mass located in the upper peripheral part of the superficial parotid lobe. Immunohistochemical analysis led to the diagnosis of an angiomyolipoma of the parotid gland. Angiomyolipomas are benign mesenchymal tumors, commonly found in the kidneys, but rare in the head and neck region.41 No recurrences have been reported after head and neck angiomyolipoma excision. Lipomas constitute 4.4% of all intraoral tumors. The cheek is the most common location of intraoral lipomas, accounting for 32% to 50% of cases.42 Our literature search came up with a rare case of an intraoral lipoma of the tongue and submandibular space.42 A 45-year-old Nigerian tribesman presented with swelling of the lower jaw and tongue for approximately 10 years. The patient had a grossly enlarged tongue and the dorsum of the tongue consisted of areas of normal papillae interrupted by exophytic, smooth, and fluctuant outgrowths. The facial examination showed bilateral symmetrical swelling of the submandibular region that would enlarge and regress depending on the movements of the tongue. A patient can on occasion present with a lipoma that can be very troublesome and even life-threatening if left unattended. One case report describes a retropharyngeal lipoma causing sleep apnea syndrome.43 A 56-year-old man presented with a 2-year history of upper airway obstruction, snoring, and frequent episodes of sleep apnea. An examination of the oropharynx showed a soft, nonpulsating, submucosal mass, covered by normal mucosa, bulging from the posterior pharyngeal wall, mainly on the left side. The tumor extended from the inferior part of the nasopharynx as far as the margin of the epiglottis. Unfortunately, lipomas of the retropharyngeal area will usually grow to a large size before they are found, and the initial symptom is often related to the airway obstruction. Additionally, we found a case study describing a 62-year-old female that presented with a sialolipoma in the right submandibular region.44 This patient had a swelling in the right submandibular region for 2 to 3 years. The mass was palpable and movable in the submandibular triangle. Microscopically, the tumor mass was mainly composed of mature adipocytes and was separated from the normal salivary gland by a fibrous capsule. The normal acinar cells of the salivary gland were infiltrated with mature adipocytes. Another interesting case in the literature involves a 40-year-old man who presented with a painless tumor in the left submandibular region.45 The 7 ⫻ 4-cm tumor was removed by surgical excision. There was a hard formation extracted from the fatty mass measuring 3.5 ⫻ 2 cm. This is an extremely rare case in
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which a hard mass of osseous metaplasia was found within the lipoma. Fanburg-Smith et al presented a clinicopathologic study in 2002 of 18 cases of liposarcoma of the oral and salivary gland region.46 In adults it is the most common soft tissue sarcoma but it is rare in and around the mouth. Only 3% of all liposarcomas occur in the head and neck region.46 It normally appears in a middle-aged person as a slowly enlarging, painless, and nonulcerated submucosal mass but some lesions grow rapidly and become ulcerated early in their growth. Development from a pre-existing benign lipoma is very rare and most cases arise de novo.46 The authors found that these rare tumors occur most commonly in the buccal mucosa, tongue, and parotid gland. Most of the tumors in their study were well differentiated. These tumors were composed of lobules of mature fat, with thickened or widened fibrous septa and scattered atypia. The atypical nuclei were large, hyperchromatic, and prominent even at lower magnifications. No patients in this review had metastases or died because of the liposarcoma. The only tumors that locally recurred in this study, regardless of subtype, were greater than 5.0 cm in maximal dimension. Complete local excision and careful patient follow-up, without adjuvant therapy, appears to be the best treatment for liposarcomas found in the oral and salivary gland regions.46 The case report of a large lipoma in the right submandibular region of the neck presented in this article showed the importance of approaching masses of the neck in a systematic fashion. A mass of the neck has a very extensive differential diagnosis so an accurate and complete examination is essential. An accurate history and complete head and neck examination often narrows the diagnostic possibilities, thus obviating the need for excessive testing and invasive procedures. There is always the possibility for malignancy in any age group; therefore, close follow-up and an aggressive approach are best for long-term favorable outcomes. As shown in this case of a large lipoma, information regarding the slowly growing mass, absence of fixity and cranial nerve involvement, lack of intracranial extension, and no lymphadenopathy, along with normal peripheral blood examination, reduced the likelihood of a malignant lesion. Additionally, FNA, CT, and MRI imaging can each be used alone or all the tests can be used in conjunction in determining a preoperative diagnosis of a neck mass.
References 1. Skandalakis J, Colborn G, Weidaran T, et al: Surgical Anatomy: The Embrylogic and Anatomic Basis of Modern Surgery. Paschalidis Medical Publications. McGraw-Hill Publishing, Greece, 2004
849 2. Spearman M, Curtin H, Dusenbury D, et al: Computed tomography directed fine needle aspiration of the skull base parapharyngeal and infratemporal fossa masses. Skull Base Surg 5:199, 1995 3. Rapidis AD: Lipoma of the oral cavity. Int J Oral Surg 11:30, 1982 4. Apostolidis C, Anterriotis D, Rapidis AD, Angelopoulos AP: Solitary intraosseous neurofibroma of the inferior alveolar nerve: Report of a case. J Oral Maxillofac Surg 59:232, 2001 5. Perskey MS, Setton A, Niimi Y, et al: Combined endovascular and surgical treatment of head and neck paragangliomas—a team approach. Head Neck 24:423, 2002 6. Rood JP, Langdon JD, Rapidis AD, et al: Carotid sheath tumor—a diagnostic challenge. J Oral Surg 53:554, 1982 7. Adams JR, O’Brien CJ: Unknown primary squamous cell carcinoma of the head and neck: A review of diagnosis, treatment and outcomes. Asian J Surg 25:188, 2002 8. Daskalopoulou D, Papanastasiou C, Markidou S, et al: The diagnostic value of fine needle aspiration cytology in Waldeyer’s ring lymphomas. Eur J Cancer [Oral Oncol] 37:36, 2001 9. Bonilla JA, Healy GB: Management of malignant head and neck tumors in children. Pediatr Clin North Am 36:1443, 1989 10. Rowe DE, Carroll RJ, Day CL Jr: Prognostic factors for local recurrence, metastasis, and survival rates in squamous cell carcinoma of the skin, ear, and lip. Implications for treatment modality selection. J Am Acad Dermatol 26:976, 1992 11. Rapidis AD, Apostolidis C, Vilos G, et al: Primary malignant melanoma of the oral mucosa. J Oral Maxillofac Surg 61:1132, 2003 12. Rapidis AD, Angelopoulos AP, Skouteris CA: Dermoid cyst of the floor of the mouth. Report of a case. Br J Oral Surg 19:43, 1981 13. Rapidis AD, Faratzis G, Lagogiannis G, et al: Large swelling of the lateral neck. J Oral Maxillofac Surg 63:820, 2005 14. Al-Khateeb TH, Al Zoubi F: Congenital neck masses: A descriptive retrospective study of 252 cases. J Oral Maxillofac Surg 65:2242, 2007 15. Enjolras O, Riche MC, Merland JJ, Escande JP: Management of alarming hemangiomas in infancy: A review of 25 cases. Pediatrics 85:491, 1990 16. Kim YH, Reiner L: Ultrastructure of lipoma. Cancer 50:102, 1982 17. Messeguer H, Ortega G, Galvez M: Pharyngeal iipoma: An ORL Ibero. America 3:247, 1994 18. Koh HK, Bhawan J: Tumours of the skin, in Moschella SL, Hurley HJ (eds): Dermatology (ed 3). Philadelphia, PA, WB Saunders, 1992, pp 1721-1808 19. Salvatore C, Antonio B, Del Vecchio W, et al: Giant infiltrating lipoma of the face: CT and MR imaging findings. AJNR Am J Neuroradiol 24:283, 2003 20. Copcu E, Sivrioglu N: Posterior cervical giant lipomas. Plast Reconstr Surg 115:2156, 2005 21. Sanchez MR, Golomb FM, Moy JA, Potozkin JR: Giant lipoma: Case report and review of the literature. J Am Acad Dermatol 28:266, 1993 22. Abd El-Monem M, Gaafar A, Magdy E: Lipomas of the head and neck: Presentation variability and diagnostic work up. J Laryngol Otol 120:27, 2006 23. Das Gupta TK: Tumors and tumor-like conditions of the adipose tissue. Curr Probl Surg 1:1, 1978 24. Ashley DJB: Evans Histological Appearances of Tumours (ed 3). Edinburgh, Livingstone, 1978, p 54 25. MacGregor AB, Dyson DP: Oral lipoma: A review of the literature and report of twelve new cases. J Oral Surg 21:770, 1966 26. Salam GA: Lipoma excision. Am Fam Physician 65:901, 2002 27. Koh HK, Bhawan J: Tumour of the skin, in Moschella SL, Hurley HJ (eds): Dermatology (ed 3). Philadelphia, PA, WB Saunders, 1992, pp 1721-1808 28. Schuler FA, Graham JK, Horton CE: Benign symmetrical lipomatosis (Madelung’s disease): Case report. Plast Reconstr Surg 57:662, 1976 29. Rosell A, Garcia-Arranz G, Llavero MT, et al: Imaging case study of the month. Lipoma of the retropharyngeal space. Ann Otol Rhinol Laryngol 107:726, 1998
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30. Knappe M, Louw M, Gregor RT: Ultrasonography-guided fineneedle aspiration for the assessment of cervical metastases. Arch Otolaryngol Head Neck Surg 126:1091, 2000 31. Lee J, Fernandes R: Neck masses: Evaluation and diagnostic approach. Oral Maxillofac Surg Clin North Am 20:321, 2008 32. Das Gupta TK: Tumors and tumor-like conditions of the adipose tissue, in Ravitch MM, Ellison EH, Julian OC, et al (eds): Current Problems in Surgery. Chicago, IL, YearBook Medical, 1970 33. Pilissier A, Sawaf M, Shabana AM: Infiltrating (intramuscular) benign lipoma of the head and neck. J Oral Maxillofac Surg 49:1231, 1991 34. Do NY, Cho SI, Park JH, Choi JY: Lipoblastoma arising from the submandibular region. J Pediatr Surg 43:E13, 2008 35. Sakaida M, Shimizu T, Kishioka C, Majima Y: Lipoblastoma of the neck: A case report and literature review. Am J Otolaryngol 25:266, 2004 36. Rasmussen IS, Kirkegaard J, Kaasbol M: Intermittent airway obstruction in a child caused by cervical lipoblastoma. Aeta Anaesthesiol Scand 41:945, 1997 37. Malave DA, Ziccardi VB, Greco R, et al: Lipoma of the parotid gland: Report of a case. J Oral Maxillofac Surg 52:408, 1994 38. Ozcan C, Unal M, Talas D, Görür K: Deep lobe parotid gland lipoma. J Oral Maxillofac Surg 60:449, 2002
39. Waltz AE, Perzik SL: Lipomatous lesions of the parotid area. Arch Otolaryngol 102:230, 1976 40. Rosado P, Villalain L, De Vicente JC, et al: Angiomyolipoma of the parotid gland: Report of a case and review of the literature. J Oral Maxillofac Surg 68:2609, 2010 41. Farrow GM, Harrison EG Jr, Utz DC, et al: Renal angiomyolipoma. A clinicopathologic study of 32 cases. Cancer 22:564, 1968 42. Daltilo D, Taiige J, Nwanas EJ: Intraoral lipoma of the tongue and submandibular space: Report of a case. J Oral Maxillofac Surg 54:915, 1996 43. Girolamo S, Marine L, Galli A, et al: Retropharyngeal lipoma causing sleep apnea syndrome. J Oral Maxlllofac Surg 56:1003, 1998 44. Jang YW, Kim SG, Hyunkyung P, et al: Sialolipoma: Case report and review of 27 cases. Oral Maxillofac Surg 13:109, 2009 45. Dutescu N, Georgescu L, Hary M, et al: Lipoma of submandibular space with osseous metaplasia. J Oral Surg 35:611, 1973 46. Fanburg-Smith J, Furlong M, Childers E: Liposarcoma of the oral and salivary gland region: A clinicopathologic study of 18 cases with emphasis on specific sites, morphologic subtypes, and clinical outcome. Mod Pathology 15:1020, 2002
Large Unilateral Neck Mass in Submandibular Region Adam Weiss, DDS,* Garry Shnayder, DDS,† Jonathan Tagliareni, DDS,‡ Edmund Wun, DDS,§ Earl Clarkson, DDS,储 and Harry Dym, DDS¶ ical examinations, including thyroid hormonal evaluation, were within normal limits. The patient’s chest x-ray showed no abnormalities. A panorex radiograph of the patient was taken and showed no obvious odontogenic sources of infection. The patient was sent for a magnetic resonance imaging (MRI) study. A T1-weighted MRI of the facial bones, orbits, and paranasal sinuses was performed without contrast using 5-mm contiguous helically obtained axial images. As shown in Figures 3 and 4, the MRI showed a bright mass that was mostly lateral and inferior to the mandible; however, a projection of the mass extended medially and superiorly compressing the pterygoid muscle and displacing the right submandibular gland inferiorly. A mass effect was noted particularly on the lateral submandibular gland, which was deviated medially and was somewhat compressed. After gadolinium administration, no abnormal enhancement was seen.
A 44-year-old male presented to the oral and maxillofacial surgery clinic at The Brooklyn Hospital Center. The patient’s chief complaint was an enlarging asymptomatic mass to the right side of his neck in the submandibular region, as shown in Figures 1 and 2. The patient noticed the mass 10 years ago but noticed a slow increase in size in the past 5 years. The patient’s medical history was significant for hypertension, asthma, and obesity. The patient denied any surgeries in the past and any drug and alcohol use. However, the patient did admit to being a 25-year pack-a-day cigarette smoker. The patient was afebrile and not in acute distress. A comprehensive head and neck examination showed a 6.5 ⫻ 4.5 ⫻ 2 cm doughy and well-circumscribed mass in the right submandibular region extending down into the neck. Extraorally, there was no discharge, no breakdown, and no erythema. The mass was not warm to the touch and the mandibular division of the fifth trigeminal nerve was intact bilaterally. There was no palpable lymphadenopathy omolateral or contralateral to the lesion. The patient had no complaints of restricted neck movement. Intraorally, there was no trismus and no gross caries seen. The patient was missing teeth 30 and 32, which were extracted many years ago. The patient’s uvula was at the midline; there was no floor of the mouth or vestibular swelling and no purulent drainage noted intraorally. The patient showed normal salivary flow. Routine hematologic and biochem-
Differential Diagnosis A good rule of thumb when evaluating neck masses is to use the “rule of 80,”1 which pertains to adults over the age of 40. This rule states that 80% of nonthyroid neck masses are neoplastic. Of the neoplastic masses, 80% are malignant. Of the malignant masses, 80% are secondary. Of the secondary masses, 80% occur above the clavicle. A secondary way of predicting the diagnosis based on duration would be to use the “rule of 7’s.”1 If a mass has been present for 7 days, it is likely inflammatory in nature; if present for 7 months, it is likely neoplastic in nature, and if present for 7 years, it would most likely be developmental. Despite these generalizations, it is important to obtain an accurate history and perform a thorough examination when diagnosing neck masses. A thorough examination of the oral cavity, nasopharynx, and the larynx is critical. The clinician should evaluate both sides of the neck to estimate the dimension of the mass. Determining the exact region of the neck in which the mass is located is important and asking the patient to turn the head from left to right helps assess painful versus nonpainful masses. The color and temperature of the skin overlying the mass may also
Received from The Brooklyn Hospital Center, Department of Oral and Maxillofacial Surgery, Brooklyn, NY. *Senior Resident in Oral and Maxillofacial Surgery. †Fellow in Oral and Maxillofacial Surgery. ‡Fellow in Oral and Maxillofacial Surgery. §Chief Resident in Oral and Maxillofacial Surgery. 储Director. ¶Chairman. Address correspondence and reprint requests to Dr Weiss: The Brooklyn Hospital Center, Department of Oral and Maxillofacial Surgery, 121 Dekalb Ave, Brooklyn, NY 11201; e-mail: [email protected] © 2012 American Association of Oral and Maxillofacial Surgeons
0278-2391/12/7004-0$36.00/0 doi:10.1016/j.joms.2011.02.126
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FIGURE 1. Preoperative presentation showing large mass in the right submandibular region. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
provide an important diagnostic clue. The age and gender of the patient and duration and rate of development, as well as palpation and auscultation, are primary considerations in arriving at a differential diagnosis. A cranial nerve examination is also important because abnormal findings can indicate nerve involvement by tumor and a poorer prognosis. Fineneedle aspiration (FNA) biopsy is an inexpensive, rapid, and relatively accurate diagnostic tool for evaluating neoplastic and nonneoplastic lesions, especially in superficial or easily palpable masses. FNA is not without error and should not replace sound clinical judgment.2 Neck masses can be categorized into the 3 following categories: inflammatory, neoplastic (benign, malignant), and congenital. Inflammatory masses are the most common cause for a neck swelling in children and young adults. Patients presenting with inflammatory neck masses often present with a fever and complain of discomfort. The neck mass with accompanying pain may have an odontogenic or nonodontogenic origin. The neck masses can occur from acute or chronic lymphadenitis, may have a viral origin (cytomegalovirus and mononucleosis), bacterial origin (parotid sialadenitis, cat scratch disease), parasitic origin (toxoplasmosis), or can be a manifestation of a granulomatous disease (sarcoidosis, scrofula). In individuals over the age of 40, neck masses are most commonly neoplastic in nature. Both benign
843 and malignant neck masses can be encountered. Lipomas are a type of benign neoplasm frequently encountered in the neck region.3 Neurofibromas in the neck may occur as solitary tumors or as part of neurofibromatosis (von Recklinhausen’s disease). Schwannomas can appear very similar clinically to solitary neurofibromas. The main difference between these 2 tumors is that the nerve usually enters a neurofibroma centrally, whereas the schwannoma tumor cells always stay on the outside of the nerve.4 Benign salivary gland tumors, such as pleomorphic adenoma, usually appear as a unilateral, asymptomatic mass that generally is solitary, freely mobile, and slow growing. Desmoplastic fibromas usually occur in the posterior cervical triangle. They originate from the roots of the first or second cervical nerves and are hard, fixed lesions. The clinician may also encounter a carotid body tumor, which is a painless lateral neck mass that is more movable laterally than vertically. This compressible mass may be pulsatile or present with a bruit.5 Glomus jugulare are solid, fixed tumors and are usually found in the bifurcation of the carotid artery.6 This can actually displace the internal and external carotid arteries.
FIGURE 2. Preoperative presentation showing large mass in the right submandibular region. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
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FIGURE 3. T1-weighted MRI taken in the axial view. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
Malignant tumors of the neck can originate from tissues in the surrounding region or may present as a metastasis from a tumor from a distant site. Some of the common primary malignant tumors causing cervical nodal distant metastases are those originating from the thyroid, breast, lungs, and colon. It is critical to examine the cervical lymph nodes during the head and neck examination when malignancy is suspected. Metastatic lesions may present in the neck region with an unknown primary lesion. These unknown head and neck primaries are generally squamous cell carcinomas. Also, squamous cell carcinomas of the tonsillar region are frequently manifested as solitary neck masses with cystic lesions that affect the lymph node. Patients with malignant cervical adenopathy with no immediately apparent primary tumor represent 3% to 10% of all head and neck cancers.7 Hodgkin’s lymphoma and non-Hodgkin’s lymphoma are frequently found as neck masses. Clinically it is very hard to make a distinction between the 2. Neck lymphomas usually present with fever, fatigue, and malaise and are often found in the posterior triangle.8 Lymphomas can occur on both sides of the neck and can be either small and hard or large and soft on palpation. Lymphomas may present in any age
group, but they are the most common malignant tumors of the head and neck in the pediatric population, representing 10% of all malignancies.9 Salivary gland tumors are another category of malignant tumors that can present as neck masses. The most common that present as a primary or metastatic lesion in the neck are mucoepidermoid carcinoma and adenoid cystic carcinoma. Mucoepidermoid carcinoma is the most common salivary gland malignancy and the second most common malignancy of the submandibular gland. Adenoid cystic carcinoma is the second most common malignant salivary gland tumor and the most common in the submandibular gland. Skin cancers can also spread and present as neck masses. These are the most common malignancies in the United States. Like other head and neck malignancies, lymphatic spread is associated with decreased survival.10 Cutaneous head and neck and rarely intraoral melanomas also frequently give regional metastases to the neck.11 Finally, the third category that can be divided into congenital and developmental neck masses are the rarest. Examples of congenital cysts that can present as neck masses include dermoid cysts, epidermoid
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FIGURE 4. T1-weighted MRI taken in the sagittal view. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
cysts, and teratomas. Dermoid and epidermoid cysts are mostly found at the midline of the floor of the mouth.12 Teratomas are rare, usually found at birth, can cause respiratory difficulty, and can occur anywhere on the neck. Lymphangiomas or cystic hygromas present as soft, diffuse, multicystic compressible masses. These masses are usually found during infancy and are rarely seen during adulthood. Branchial cleft cysts of the neck are usually developed from the second branchial arch. They are often found anterolaterally to the jugular vein and carotid artery and are typically located anterior to the sternocleidomastoid muscle. Usually present as asymptomatic unless infected, they therefore can achieve a considerable size before they are diagnosed.13 The most common developmental cyst found in the neck is the thyroglossal duct cyst. The typical presentation of the thyroglossal duct cyst is a large mass in the midline of the neck below the hyoid bone that moves with swallowing or protrusion of the tongue. Cysts of the thyroglossal duct can be found from the foramen cecum at the base of the tongue to the pyramidal lobe of the thyroid gland.14 Hemangiomas are often present at birth and rapidly proliferate in the first years of life, usually
followed by a slow involution. These lesions are more common in females (3:1) and 60% are located in the head and neck region.15
Surgery Performed and Subsequent Course The risks, benefits, and alternative treatments were discussed with the patient. Treatment options included doing nothing or excision of the lesion, and benefits included removal of source of discomfort. Risks were discussed with the patient, including paresthesia, motor nerve paralysis, bleeding, swelling, pain, and infection. The patient elected to have the lesion excised under general anesthesia. The patient was brought to the operating room and placed under general endotracheal anesthesia. The patient was prepped and draped in a sterile fashion. A no. 15 blade was used to make the initial submandibular skin incision extending to the most inferior portion of the extended lesion. Blunt dissection was performed down to the level of the lesion as shown in Figure 5. Dissection was then performed down to the level of the lesion capsule at which point intracapsu-
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FIGURE 5. Intraoperative photograph showing blunt dissection of the mass. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
lar dissection was performed until the mass was freed in all aspects, medially, laterally, inferiorly, and superiorly. The mass was dissected from the right submandibular gland, which it displaced inferiorly and medially, as shown in Figure 6. No attempt was made to evacuate the contents of the lesion. The mass was removed in total and measured approximately 9 cm in length and 5 to 6 cm in width, as shown in Figure 7. As seen in Figure 8, the mass was submerged in formalin and floated, confirming the diagnosis of a lipoma. The specimen was sent to pathology for further study. The surgical site was irrigated with copious amounts of sterile saline. A no. 7 Jackson Pratt drain was inserted and secured with silk sutures. Closure was completed and a telfa dressing and bacitracin was applied to the wound. The patient was extubated and transported to the recovery room in stable condition. The patient was admitted and stayed
FIGURE 7. Surgical specimen. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
overnight for observation. A noneventful postoperative course was observed and the patient was discharged the day after the surgery was performed.
Pathology Diagnosis The histological diagnosis of this mass was a lipoma. Lipomas have a characteristic histological appearance. These benign tumors are histologically similar to adipose tissue; however, they are differentiated by the presence of a fibrous capsule.16 As shown in Figure 9 representing the histology of the sample submitted from our case, lipomas are composed microscopically of mature white adipose tissue arranged in lobules, many of which are surrounded by a deli-
FIGURE 6. Intraoperative photograph showing the relationship of mass to right submandibular gland.
FIGURE 8. Specimen shown floating in formalin solution.
Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
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FIGURE 9. Microphotograph of specimen shown at high power view showing mature adipose tissue arranged in lobules. Weiss et al. Large Unilateral Neck Mass in Submandibular Region. J Oral Maxillofac Surg 2012.
cate fibrous capsule. These tumors frequently exhibit varying numbers of collagen strands coursing through the lesion and one will occasionally observe small blood vessels as well. When this fibrous connective tissue forms a more significant portion of the tumor, the lesion is then described as a fibrolipoma.
Discussion The lipoma has rarely been reported in the oral cavity, larynx, and pharynx. It occurs more frequently in other areas, in particular, the subcutaneous tissues of the neck.17 Lipomas are slow growing, nearly always benign tumors that are well-circumscribed tumors of mature adipose tissue. On gross examination, they are smooth, well circumscribed, lobulated, greasy to the touch, and yellow to orange in color. The epithelium is usually very thin and the superficial blood vessels are readily visible over the surface. Lipomas are the most common neoplasms of mesenchymal origin, arising in any location where fat is normally present. Solitary lipomas are generally more common in women, whereas multiple tumors (referred to as lipomatosis) are more common in men.18 Benign lipomatous tumors have been subclassified according to their histological features and growth pattern into classic lipomas (solitary or multiple), fibrolipoma, angiolipoma, infiltrating lipoma, intramuscular lipoma, hibernoma, pleomorphic lipoma, lipoblastomatosis, and diffuse lipoblastomatosis.19,20 Lipomas can also be further classified based on either size or weight. A tumor is classified as a giant lipoma if the size is greater than 10 cm in 1 dimension or the weight is greater than 1,000 g.21
847 Lipomas are rare in the first 2 decades of life, usually developing in the fifth and sixth decades when fat begins to accumulate in inactive, underexercised individuals. In general, this tumor is more commonly found in obese people and can increase in size during a period of rapid weight gain.22 According to Das Gupta,23 obesity and local growth of adipose tissue may both be responsible for the formation of a lipoma. This is known as the hypertrophy theory. In contrast, the metaplasia theory holds that lipomatous development represents the abnormal differentiation in situ of mesenchymal cells into lipoblasts.24 However, others have suggested that trauma and chronic irritation may cause the proliferation of soft tissue and play a part in the formation of a lipoma.25 Lipomas usually occur sporadically, but rarely they can be associated with several inherited disorders, including hereditary multiple lipomatosis, Gardner’s syndrome, and Madelung’s disease.26 Hereditary multiple lipomatosis is characterized by widespread, symmetric lipomas appearing most often over the extremities and trunk.27 Gardner’s syndrome is an autosomal-dominant disorder that can also be associated with lipomatosis and is characterized by intestinal polyposis, cysts, and osteomas.26 Madelung’s disease or benign symmetric lipomatosis involves an unusual distribution of fatty tissue surrounding the cervical region, shoulders, and proximal upper extremities. Patients with this disease are often longterm alcoholics and present with the characteristic “horse-collar” cervical appearance.28 There are different imaging modalities that can be used in confirming the preoperative diagnosis of a lipoma. The computed tomography (CT) can distinguish cystic from solid lesions. A CT scan provides a definitive diagnosis of lipoma in almost all cases by calculating the actual density of the suspected mass (via the CT attenuation number). Fat, being the only soft tissue with a density less than water, has a negative CT attenuation number. A lipoma will characteristically appear as a homogeneous mass with few septations, low CT attenuation number, and no contrast enhancement. MRI and CT not only show the lipomatous nature of the tumor preoperatively but also accurately determine its exact size, location, and extensions. This is critical in determining a preoperative surgical plan. The MRI as used on the patient described in our case has an advantage over a CT in its soft tissue capabilities and it is useful when the distinction between the mass and surrounding soft tissue is poor. It is important to understand that neither CT nor MRI can differentiate a lipoma from a liposarcoma.29 This distinction can only be made with certainty by a careful histopathological examination. Ultrasound is another study that is readily available,
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inexpensive, noninvasive, and accurate that can be used in determining the nature of unknown neck mass.30 Lipomas may present in the neck as large isolated masses present for long periods without much change over the years. The treatment of these benign tumors is a simple excision. The recurrence of these tumors is rare.31 True lipomas recur in only 1% to 2% of cases after adequate excision, so a recurrence should raise the suspicion of a liposarcoma, especially if the tumor is not superficially located.32 Lipomas tend to form in the subcutaneous tissue, which is in contrast to the liposarcoma that forms from the deeper tissues, an important clinical distinction. Upon performing a PubMed search for head and neck lipomas, it is evident that literature on head and neck lipomas is limited and has mostly been in the form of separate case reports. We have highlighted a few of the cases published in the literature to show the variability in size, location, and type that these tumors can present with in the head and neck region. There is a report of a 55-year-old obese female that presented with soft, nontender chin swelling.33 Intraoral examination showed a large fixed mass filling the lower sulcus between the right lateral incisor and the left second molar covered by slightly inflamed mucosa. The tumor was resected and the diagnosis was submucosal and intramuscular benign lipoma. Rarely, a lipoma has been known to infiltrate surrounding tissues, most notably skeletal muscle, in which case it is referred to as an infiltrating lipoma. Do et al34 reported a case of a 22-month-old boy with a history of painless swelling in the right submandibular area for 2 months that rapidly enlarged. CT showed a 5 ⫻ 5-cm expansive mass with a clearly demarcated low density at the right submandibular area. The histopathologic diagnosis was a lipoblastoma. Lipoblastoma is a rare, benign tumor arising from embryonic white fat cells and usually occurs in infants younger than 3 years or in childhood.35 This type of tumor typically presents as a rapidly enlarging, painless mass and the clinical symptoms are mostly associated with tumor size and anatomical location.36 There are also several reports of lipomas of the parotid gland. One such report involves a 44-year-old female with a 3-year history of a slowly enlarging left preauricular mass that suddenly had become painful and tender.37 The lipoma was not attached to the overlying skin and appeared to be in the superficial lobe of the left parotid gland. Another case report describes a 47-year-old male with a deep lobe parotid gland lipoma.38 A physical examination showed a 4 ⫻ 3-cm soft and painless mass in the left parotid region. Lipomas account for 2% to 3% of the benign parotid tumors.39 Interestingly, Rosado et al report a case of a 73-year-old woman with a 2-year history of a gradually
enlarging, painful mass located in the left parotid region.40 Surgical excision was performed, obtaining a red-wine-colored mass located in the upper peripheral part of the superficial parotid lobe. Immunohistochemical analysis led to the diagnosis of an angiomyolipoma of the parotid gland. Angiomyolipomas are benign mesenchymal tumors, commonly found in the kidneys, but rare in the head and neck region.41 No recurrences have been reported after head and neck angiomyolipoma excision. Lipomas constitute 4.4% of all intraoral tumors. The cheek is the most common location of intraoral lipomas, accounting for 32% to 50% of cases.42 Our literature search came up with a rare case of an intraoral lipoma of the tongue and submandibular space.42 A 45-year-old Nigerian tribesman presented with swelling of the lower jaw and tongue for approximately 10 years. The patient had a grossly enlarged tongue and the dorsum of the tongue consisted of areas of normal papillae interrupted by exophytic, smooth, and fluctuant outgrowths. The facial examination showed bilateral symmetrical swelling of the submandibular region that would enlarge and regress depending on the movements of the tongue. A patient can on occasion present with a lipoma that can be very troublesome and even life-threatening if left unattended. One case report describes a retropharyngeal lipoma causing sleep apnea syndrome.43 A 56-year-old man presented with a 2-year history of upper airway obstruction, snoring, and frequent episodes of sleep apnea. An examination of the oropharynx showed a soft, nonpulsating, submucosal mass, covered by normal mucosa, bulging from the posterior pharyngeal wall, mainly on the left side. The tumor extended from the inferior part of the nasopharynx as far as the margin of the epiglottis. Unfortunately, lipomas of the retropharyngeal area will usually grow to a large size before they are found, and the initial symptom is often related to the airway obstruction. Additionally, we found a case study describing a 62-year-old female that presented with a sialolipoma in the right submandibular region.44 This patient had a swelling in the right submandibular region for 2 to 3 years. The mass was palpable and movable in the submandibular triangle. Microscopically, the tumor mass was mainly composed of mature adipocytes and was separated from the normal salivary gland by a fibrous capsule. The normal acinar cells of the salivary gland were infiltrated with mature adipocytes. Another interesting case in the literature involves a 40-year-old man who presented with a painless tumor in the left submandibular region.45 The 7 ⫻ 4-cm tumor was removed by surgical excision. There was a hard formation extracted from the fatty mass measuring 3.5 ⫻ 2 cm. This is an extremely rare case in
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which a hard mass of osseous metaplasia was found within the lipoma. Fanburg-Smith et al presented a clinicopathologic study in 2002 of 18 cases of liposarcoma of the oral and salivary gland region.46 In adults it is the most common soft tissue sarcoma but it is rare in and around the mouth. Only 3% of all liposarcomas occur in the head and neck region.46 It normally appears in a middle-aged person as a slowly enlarging, painless, and nonulcerated submucosal mass but some lesions grow rapidly and become ulcerated early in their growth. Development from a pre-existing benign lipoma is very rare and most cases arise de novo.46 The authors found that these rare tumors occur most commonly in the buccal mucosa, tongue, and parotid gland. Most of the tumors in their study were well differentiated. These tumors were composed of lobules of mature fat, with thickened or widened fibrous septa and scattered atypia. The atypical nuclei were large, hyperchromatic, and prominent even at lower magnifications. No patients in this review had metastases or died because of the liposarcoma. The only tumors that locally recurred in this study, regardless of subtype, were greater than 5.0 cm in maximal dimension. Complete local excision and careful patient follow-up, without adjuvant therapy, appears to be the best treatment for liposarcomas found in the oral and salivary gland regions.46 The case report of a large lipoma in the right submandibular region of the neck presented in this article showed the importance of approaching masses of the neck in a systematic fashion. A mass of the neck has a very extensive differential diagnosis so an accurate and complete examination is essential. An accurate history and complete head and neck examination often narrows the diagnostic possibilities, thus obviating the need for excessive testing and invasive procedures. There is always the possibility for malignancy in any age group; therefore, close follow-up and an aggressive approach are best for long-term favorable outcomes. As shown in this case of a large lipoma, information regarding the slowly growing mass, absence of fixity and cranial nerve involvement, lack of intracranial extension, and no lymphadenopathy, along with normal peripheral blood examination, reduced the likelihood of a malignant lesion. Additionally, FNA, CT, and MRI imaging can each be used alone or all the tests can be used in conjunction in determining a preoperative diagnosis of a neck mass.
References 1. Skandalakis J, Colborn G, Weidaran T, et al: Surgical Anatomy: The Embrylogic and Anatomic Basis of Modern Surgery. Paschalidis Medical Publications. McGraw-Hill Publishing, Greece, 2004
849 2. Spearman M, Curtin H, Dusenbury D, et al: Computed tomography directed fine needle aspiration of the skull base parapharyngeal and infratemporal fossa masses. Skull Base Surg 5:199, 1995 3. Rapidis AD: Lipoma of the oral cavity. Int J Oral Surg 11:30, 1982 4. Apostolidis C, Anterriotis D, Rapidis AD, Angelopoulos AP: Solitary intraosseous neurofibroma of the inferior alveolar nerve: Report of a case. J Oral Maxillofac Surg 59:232, 2001 5. Perskey MS, Setton A, Niimi Y, et al: Combined endovascular and surgical treatment of head and neck paragangliomas—a team approach. Head Neck 24:423, 2002 6. Rood JP, Langdon JD, Rapidis AD, et al: Carotid sheath tumor—a diagnostic challenge. J Oral Surg 53:554, 1982 7. Adams JR, O’Brien CJ: Unknown primary squamous cell carcinoma of the head and neck: A review of diagnosis, treatment and outcomes. Asian J Surg 25:188, 2002 8. Daskalopoulou D, Papanastasiou C, Markidou S, et al: The diagnostic value of fine needle aspiration cytology in Waldeyer’s ring lymphomas. Eur J Cancer [Oral Oncol] 37:36, 2001 9. Bonilla JA, Healy GB: Management of malignant head and neck tumors in children. Pediatr Clin North Am 36:1443, 1989 10. Rowe DE, Carroll RJ, Day CL Jr: Prognostic factors for local recurrence, metastasis, and survival rates in squamous cell carcinoma of the skin, ear, and lip. Implications for treatment modality selection. J Am Acad Dermatol 26:976, 1992 11. Rapidis AD, Apostolidis C, Vilos G, et al: Primary malignant melanoma of the oral mucosa. J Oral Maxillofac Surg 61:1132, 2003 12. Rapidis AD, Angelopoulos AP, Skouteris CA: Dermoid cyst of the floor of the mouth. Report of a case. Br J Oral Surg 19:43, 1981 13. Rapidis AD, Faratzis G, Lagogiannis G, et al: Large swelling of the lateral neck. J Oral Maxillofac Surg 63:820, 2005 14. Al-Khateeb TH, Al Zoubi F: Congenital neck masses: A descriptive retrospective study of 252 cases. J Oral Maxillofac Surg 65:2242, 2007 15. Enjolras O, Riche MC, Merland JJ, Escande JP: Management of alarming hemangiomas in infancy: A review of 25 cases. Pediatrics 85:491, 1990 16. Kim YH, Reiner L: Ultrastructure of lipoma. Cancer 50:102, 1982 17. Messeguer H, Ortega G, Galvez M: Pharyngeal iipoma: An ORL Ibero. America 3:247, 1994 18. Koh HK, Bhawan J: Tumours of the skin, in Moschella SL, Hurley HJ (eds): Dermatology (ed 3). Philadelphia, PA, WB Saunders, 1992, pp 1721-1808 19. Salvatore C, Antonio B, Del Vecchio W, et al: Giant infiltrating lipoma of the face: CT and MR imaging findings. AJNR Am J Neuroradiol 24:283, 2003 20. Copcu E, Sivrioglu N: Posterior cervical giant lipomas. Plast Reconstr Surg 115:2156, 2005 21. Sanchez MR, Golomb FM, Moy JA, Potozkin JR: Giant lipoma: Case report and review of the literature. J Am Acad Dermatol 28:266, 1993 22. Abd El-Monem M, Gaafar A, Magdy E: Lipomas of the head and neck: Presentation variability and diagnostic work up. J Laryngol Otol 120:27, 2006 23. Das Gupta TK: Tumors and tumor-like conditions of the adipose tissue. Curr Probl Surg 1:1, 1978 24. Ashley DJB: Evans Histological Appearances of Tumours (ed 3). Edinburgh, Livingstone, 1978, p 54 25. MacGregor AB, Dyson DP: Oral lipoma: A review of the literature and report of twelve new cases. J Oral Surg 21:770, 1966 26. Salam GA: Lipoma excision. Am Fam Physician 65:901, 2002 27. Koh HK, Bhawan J: Tumour of the skin, in Moschella SL, Hurley HJ (eds): Dermatology (ed 3). Philadelphia, PA, WB Saunders, 1992, pp 1721-1808 28. Schuler FA, Graham JK, Horton CE: Benign symmetrical lipomatosis (Madelung’s disease): Case report. Plast Reconstr Surg 57:662, 1976 29. Rosell A, Garcia-Arranz G, Llavero MT, et al: Imaging case study of the month. Lipoma of the retropharyngeal space. Ann Otol Rhinol Laryngol 107:726, 1998
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30. Knappe M, Louw M, Gregor RT: Ultrasonography-guided fineneedle aspiration for the assessment of cervical metastases. Arch Otolaryngol Head Neck Surg 126:1091, 2000 31. Lee J, Fernandes R: Neck masses: Evaluation and diagnostic approach. Oral Maxillofac Surg Clin North Am 20:321, 2008 32. Das Gupta TK: Tumors and tumor-like conditions of the adipose tissue, in Ravitch MM, Ellison EH, Julian OC, et al (eds): Current Problems in Surgery. Chicago, IL, YearBook Medical, 1970 33. Pilissier A, Sawaf M, Shabana AM: Infiltrating (intramuscular) benign lipoma of the head and neck. J Oral Maxillofac Surg 49:1231, 1991 34. Do NY, Cho SI, Park JH, Choi JY: Lipoblastoma arising from the submandibular region. J Pediatr Surg 43:E13, 2008 35. Sakaida M, Shimizu T, Kishioka C, Majima Y: Lipoblastoma of the neck: A case report and literature review. Am J Otolaryngol 25:266, 2004 36. Rasmussen IS, Kirkegaard J, Kaasbol M: Intermittent airway obstruction in a child caused by cervical lipoblastoma. Aeta Anaesthesiol Scand 41:945, 1997 37. Malave DA, Ziccardi VB, Greco R, et al: Lipoma of the parotid gland: Report of a case. J Oral Maxillofac Surg 52:408, 1994 38. Ozcan C, Unal M, Talas D, Görür K: Deep lobe parotid gland lipoma. J Oral Maxillofac Surg 60:449, 2002
39. Waltz AE, Perzik SL: Lipomatous lesions of the parotid area. Arch Otolaryngol 102:230, 1976 40. Rosado P, Villalain L, De Vicente JC, et al: Angiomyolipoma of the parotid gland: Report of a case and review of the literature. J Oral Maxillofac Surg 68:2609, 2010 41. Farrow GM, Harrison EG Jr, Utz DC, et al: Renal angiomyolipoma. A clinicopathologic study of 32 cases. Cancer 22:564, 1968 42. Daltilo D, Taiige J, Nwanas EJ: Intraoral lipoma of the tongue and submandibular space: Report of a case. J Oral Maxillofac Surg 54:915, 1996 43. Girolamo S, Marine L, Galli A, et al: Retropharyngeal lipoma causing sleep apnea syndrome. J Oral Maxlllofac Surg 56:1003, 1998 44. Jang YW, Kim SG, Hyunkyung P, et al: Sialolipoma: Case report and review of 27 cases. Oral Maxillofac Surg 13:109, 2009 45. Dutescu N, Georgescu L, Hary M, et al: Lipoma of submandibular space with osseous metaplasia. J Oral Surg 35:611, 1973 46. Fanburg-Smith J, Furlong M, Childers E: Liposarcoma of the oral and salivary gland region: A clinicopathologic study of 18 cases with emphasis on specific sites, morphologic subtypes, and clinical outcome. Mod Pathology 15:1020, 2002