- Email: [email protected]
Metastatic Neuroblastoma in the Mandibular Condyle: Report of a Case
1941
BABER, ABUBAKER, AND LASKIN
the inflammatory effect of any viral infection, inhaled carbogen to improve the blood supply in the perilymph, dextran 70 to improve blood circulation, anticoagulants, vasodilators,2 antibiotics,5 and middle-ear exploration.1,4,8,10 Surgery is reserved for perilymph fistulae and for hearing deterioration with significant vertigo and acoustic neuroma.4 Important prognostic indicators include the severity of initial hearing loss and vertigo, time to initial audiogram, an elevated erythrocyte sedimentation rate, age less than 15 years and greater than 60 years, a midfrequency audiogram configuration, and hearing status of the opposite ear.1 Any of the potential causes suggested in the literature could be implicated in our case of SNHL. It is extremely important that a patient’s complaints are taken seriously. Early referral to an appropriate specialist for thorough assessment and treatment is mandatory. Acknowledgment Medical photography was performed at the Poole Hospital National Health Service Trust, Poole, UK.
References 1. Byl FM Jr: Sudden hearing loss: Eight years’ experience and suggested prognostic table. Laryngoscope 94:647, 1984 2. Plasse HM, Mittleman M, Frost J: Unilateral sudden hearing loss after open heart surgery. A detailed study of seven cases. Laryngoscope 91:101, 1981 3. Goodhill V, Harris I, Brockman SJ: Sudden deafness and labyrinthine window rupture. Ann Otol Rhinol Laryngol 82:2, 1973 4. Pau H, Selvadurai D, Murty GE: Reversible sensorineural hearing loss after non-otological surgery under general anaesthetic. Postgrad Med J 76:304, 2000 5. Park P, Toung JS, Smythe P, et al: Unilateral sensorineural hearing loss after spine surgery: Case report and review of literature. Surg Neurol 66:415, 2006 6. Farrel RW, Pemberton MN, Parker AJ, et al: Sudden deafness after dental surgery. Br Med J [Clin Res] 303:1270, 1991 7. Evan KE, Tavill MA, Goldberg AN, et al: Sudden sensorineural hearing loss after general anaesthesia for nonotological surgery. Laryngoscope 107:747, 1997 8. Goodhill V: Labyrinthine membrane ruptures in sudden sensorineural hearing loss. Proc R Soc Med 69:565, 1976 9. Segal S, Man A, Winerman I: Labyrinthine membrane rupture caused by elevated intratympanic pressure during anaesthesia. Am J Otol 5:308, 1984 10. Fisch U: Management of sudden deafness. Otolaryngol Head Neck Surg 91:3, 1983 J Oral Maxillofac Surg 66:1941-1945, 2008
Metastatic Neuroblastoma in the Mandibular Condyle: Report of a Case M. Andrew Baber, DDS,* Omar Abubaker, DDS, PhD,† and Daniel M. Laskin, DDS, MS‡ Neuroblastoma is a relatively common pediatric malignancy. It has a broad clinical spectrum ranging from spontaneously regressing disease with high survival to fulminating systemic progression.1 Presentation of neuroblastoma in the maxillofacial region is an infrequent occurrence and is often insidious. AlReceived from the Department of Oral and Maxillofacial Surgery, Virginia Commonwealth University Medical Center, School of Dentistry, Richmond, VA. *Formerly, Chief Resident; and Currently, Private Practice, Rogers, AR. †Professor and Chairman. ‡Professor and Chairman Emeritus. Address correspondence and reprint requests to Dr Abubaker: VCU School of Dentistry, Department of Oral and Maxillofacial Surgery, 520 North 12th Street, Room 239, Richmond, VA 232980566; e-mail: [email protected] © 2008 American Association of Oral and Maxillofacial Surgeons
0278-2391/08/6609-0024$34.00/0 doi:10.1016/j.joms.2007.06.689
though there are numerous case reports describing metastatic neuroblastoma to the mandible,2-13 there are no published case reports describing metastasis specifically to the mandibular condyle. We report a case of metastatic neuroblastoma of the mandibular condyle and review features of the disease pertinent to the oral and maxillofacial surgeon.
Report of a Case In September 2006, a 15-year-old Caucasian female presented to the Oral and Maxillofacial Surgery Clinic at the Virginia Commonwealth University Medical Center on referral from her general dentist. Her chief complaint was, “The left side of my face is swollen and it hurts.” The patient reported a 2- to 3-week history of moderate to severe leftsided facial pain that was aching in nature. Her mother had taken her to a general dentist who placed her on antibiotics for a questionable abscess. This treatment did not improve her symptoms. About 3 days before her visit to our clinic, the patient’s grandmother noticed the left side of the patient’s face was slightly swollen. When initially questioned, she felt as if her symptoms had been of limited duration, but
1942
NEUROBLASTOMA OF THE MANDIBULAR CONDYLE
when questioned further she admitted to having some mild intermittent pain in her face for the last 7 months and also having noticed that her teeth were not fitting together as they had previously. She denied any significant medical, surgical, family, or social history. A review of systems showed the patient had had a recent 20-pound weight loss, difficulty eating, and pain in the right shoulder, mid-thoracic back, and left lower quadrant of the abdomen. Focused physical examination showed a firm, slightly tender, 3 ⫻ 4 cm swelling of the left preauricular area (Fig 1). The chin was deviated slightly to the right and there was notable further deviation to the right on mouth opening. Maximum interincisal opening was 23 mm. Intraoral examination showed that the mandibular dental midline was deviated to the right about 3 mm and there was an edge-to-edge relationship of the right posterior teeth. The neck examination was negative for lymphadenopathy; however, there was notable swelling over the medial aspect of the right clavicle and slight enlargement of the thyroid gland. The cranial nerve examination was normal. A panoramic radiograph showed significant radiolucency of the left condyle, with indistinct trabeculation, loss of cortical borders, and a mottled appearance (Fig 2). The differential diagnosis included benign or malignant primary tumor and metastatic disease from a distant primary tumor. A maxillofacial and neck computed tomography (CT) scan was obtained and it showed a large, soft-tissue mass in the left masseteric space, bony destruction of the mandibular condyle, and a lytic lesion of the left mandibular ramus (Figs 3, 4). In addition, there also were multiple lytic lesions
FIGURE 2. Panoramic radiograph showing destruction of left mandibular condyle. Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
involving the medial aspect of the right clavicle and multiple areas of decreased attenuation in both lobes of the thyroid. A fine needle aspiration biopsy of the mandibular condyle was carried out, and the preliminary diagnosis was small, round, blue-cell tumor. At this point, the pediatric oncology service was consulted and asked to assume care of the patient. The patient was admitted to the pediatric oncology service and underwent a complete workup for malignant disease. The CBC and differential white cell count, INR, PT, PTT, urinalysis, metabolic panel, and liver and thyroid function tests were all normal. Lateral and PA chest x-rays; chest,
FIGURE 1. Notable left preauricular swelling.
FIGURE 3. Maxillofacial CT scan, coronal view showing bony destruction of the left mandibular condyle.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
BABER, ABUBAKER, AND LASKIN
1943
FIGURE 4. Maxillofacial CT scan, axial view showing large softtissue mass of the left masseteric space. Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008. FIGURE 6. Chest radiograph showing metastatic deposits.
abdomen and pelvis CT scans with contrast; head, cervical, thoracic, and lumbar magnetic resonance imaging; and whole body Tc-99 bone and I-131 tumor scans were obtained and these showed widespread metastatic disease, including masses in the chest, abdomen, and pelvis, as well as multiple sites of bony metastasis (Figs 5, 6). The patient underwent biopsy of her right clavicular mass and bilateral posterior iliac crest bone marrow biopsies to establish a definitive histologic diagnosis. Microscopic ex-
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
amination of the specimens showed a stroma-poor tumor with immunostains consistent with a poorly differentiated neuroblastoma (Figs 7, 8). The disease was deemed stage IV and, due to the widespread nature of the metastases, nonsurgical treatment was considered the best option. A portacath was placed and chemotherapy was initiated with vincristine, cyclophosphamide, and daunomycin, as well as
FIGURE 5. Abdominal CT scan showing large masses.
FIGURE 7. Histologic slide (H&E, 400⫻). The tumor consists of small, round, blue cells with a high nucleus to cytoplasmic ratio dissecting between skeletal muscle fibers. No evidence of maturation can be identified in this poorly differentiated neuroblastoma.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
1944
FIGURE 8. Histologic slide (H&E, 600⫻). At higher magnification, apoptotic bodies are easily identifiable (arrows). Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
pegfilgrastim for myelosuppression. The tumors were responsive to this chemotherapeutic regimen and drastically decreased in size. The eventual plan was for an autologous stem-cell transplant.
Discussion Neuroblastic tumors include neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. They are classified into 1 of these 3 types based on the balance between neural-type cells and Schwann-type cells.1 Neuroblastoma typically represents one end of the spectrum as a poorly differentiated tumor, whereas ganglioneuroma is the well-differentiated benign counterpart on the other end of the spectrum.1,14 Neuroblastomas may be further classified as undifferentiated, poorly differentiated, or differentiating.15 Neuroblastoma is almost exclusively a tumor of children. More than 600 cases are diagnosed each year in the United States.16 It is the third most common childhood cancer overall and the most common solid extracranial tumor in children.15,16 It accounts for 6% to 10% of all childhood cancers and 15% of all pediatric cancer deaths in the United States.17 Incidence rates are age-dependent. The median age at diagnosis is 17.3 months, with 40% diagnosed before age 1 year.15 There is some ethnic predilection, with the incidence in infants being greater in Caucasians than African-Americans; however no ethnic differences are present among older children.15 Neuroblastomas arise from primordial neural crest cells that migrate during embryogenesis to form the adrenal medulla and sympathetic ganglia.17 They can arise anywhere throughout the sympathetic nervous system. However, the adrenal gland is the most common location.16,18 Adrenal neuroblasto-
NEUROBLASTOMA OF THE MANDIBULAR CONDYLE
mas are thought to arise from residual microscopic neuroblastic nodules that should normally regress by birth or shortly thereafter.19 Clinical presentation of neuroblastoma is varied and symptoms usually relate to the location of the primary or metastatic lesions. These symptoms often include abdominal masses and pain or constipation, back pain, bone pain, fever, weight loss, anemia, periorbital ecchymosis, and proptosis.20 Neuroblastomas metastasize by both hematogenous and lymphatic routes and may spread to a wide variety of locations including lymph nodes, bone marrow, cortical bone, dura, the orbits, liver, and skin.21 Tumor infiltration of the orbits is usually unilateral and causes a characteristic periorbital ecchymosis, ptosis, and proptosis.22 Because neuroblastomas arise from the sympathetic nervous system, they are characterized by defective catecholamine synthesis, which can result in the accumulation of the intermediates homovanillic acid, vanillylmandelic acid, and dopamine.23 These catecholamine intermediates may give rise to adrenergic symptoms such as tachycardia or hypertension.23 Treatment of neuroblastoma can consist of surgery, chemotherapy, radiation, or a combination of these modalities. Chemotherapy is the primary treatment for advanced-stage neuroblastomas.17 The tumor is generally considered radiosensitive, although there is little benefit for disease that is not in an advanced stage.17 However, in higher risk neuroblastomas, radiation has been shown to decrease local relapse rates.17 Of the 13 cases of metastatic neuroblastoma to the mandible referenced previously,2-13 there are no reported occurrences in the condyle. This illustrates the rare nature of this particular case. Although uncommon, neuroblastoma should still be included in the differential diagnosis when a radiolucency suspected of being a metastatic lesion is discovered in the mandible of a child. Such patients may have deviation of the mandible on mouth opening, changes in the occlusion, mobile teeth, and unexplained pain. Key clinical findings related to metastatic neuroblastoma in other parts of the maxillofacial region include proptosis, periorbital ecchymosis, Horner’s syndrome, heterochromidia iridis (different colors of the iris or portions of the iris),23 and masses.
References 1. Russell H, Shohet J, Nuchtern J: Epidemiology, pathogenesis, and pathology of neuroblastoma. Up To Date Online 14.2:1, 2007 2. Bhattacharyya I, Williamson A, Cohen DM, et al: Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 88:586, 1999
1945
KEMP ET AL 3. Chan AR, Johnston DH, Stoneman D: Disseminated neuroblastoma with initial presentation as an intraoral mass: Case report. Pediatr Dent 16:310, 1994 4. Haddad A, Azouz EM, al-Jedher S, et al: Metastatic neuroblastoma presenting as a mandibular mass. Can Assoc Radiol J 43:436, 1992 5. Borle RM, Hazare VK, Bhowate RR, et al: Neuroblastoma metastatic to the mandible. J Oral Maxillofac Surg 49:1124, 1991 6. Boraz RA: Neuroblastoma: Case involving metastases to the mandible. Pediatr Dent 7:315, 1985 7. Newland JR, McClendon JL, Lynch DP: Metastatic neuroblastoma of the jaws. J Oral Maxillofac Surg 43:549, 1985 8. Hardt NP: Metastatic neuroblastoma in the mandible. Report of a case. Oral Surg Oral Med Oral Pathol 41:314, 1976 9. Snyder MB, Cawson RA: Jaw and pulpal metastasis of an adrenal neuroblastoma. Oral Surg Oral Med Oral Pathol 40:775, 1975 10. Chakrabarty PB, Das S, Dash BC: Neuroblastoma with metastasis to mandible. Report of a case and review of the literature. Indian J Cancer 12:219, 1975 11. Angelopoulos AP, Tilsn HB, Stewart FW: Metastatic neuroblastoma of the mandible: Review of literature and report of case. J Oral Surg 30:93, 1972 12. DeLeon EL, Finney RA, Ruth A, et al: Neuroblastoma with metastasis to maxilla and mandible: Review of literature and report of case. J Oral Surg 28:773, 1970 13. Hoffman S, Green GH: Neuroblastoma with metastasis to the mandible: Report of case. J Oral Surg 24:75, 1966 14. Peuchmaur M, d’Amore ES, Joshi VV, et al: Revision of the International Neuroblastoma Pathology Classification: Confir-
15.
16.
17. 18.
19. 20. 21. 22. 23.
mation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular. Cancer 98:2274, 2003 Schwab M, Shimada H, Joshi V, et al: Neuroblastic tumours of adrenal gland and sympathetic nervous system, in Pathology and Genetics of Tumours of the Nervous System. Lyon, France, World Health Organization, 2000 Goodman MT, Gurney JG, Smith MA, et al: Sympathetic nervous system tumors. Cancer Incidence and Survival among Children and Adolescents: United States SEER Program, 19751995. Bethesda, MD, National Cancer Institute, 1999 Kim S, Chung D: Pediatric solid malignancies: Neuroblastoma and Wilms tumor. Surg Clin N Am 86:469, 2006 Castleberry RP, Shuster JJ, Smith EI: The Pediatric Oncology Group experience with the international staging system criteria for neuroblastoma. Member Institutions of the Pediatric Oncology Group. J Clin Oncol 12:2378, 1994 Brodeur GM, Maris JM: Principles and Practice of Pediatric Oncology. Philadelphia, PA, Lippincott Williams & Wilkins, 2002 Golden CB, Feusner JH: Malignant abdominal masses in children: Quick guide to evaluation and diagnosis. Pediatr Clin North Am 49:1369, 2002 DuBois SG, Kalika Y, Lukens JN, et al: Metastatic sites in stage IV and IVS neuroblastoma correlate with age, tumor biology, and survival. J Pediatr Hematol Oncol 21:181, 1999 Musarella MA, Chan HS, DeBoer G, et al: Ocular involvement in neuroblastoma: Prognostic implications. Ophthalmology 91: 936, 1984 Russell H, Shohet J, Nuchtern J: Clinical presentation, diagnosis, and staging evaluation of neuroblastoma. Up To Date Online 14.2:1, 2007 J Oral Maxillofac Surg 66:1945-1948, 2008
Persistent Melanoma In Situ: Case Report and Review Spencer Kemp, DDS,* George Gallagher, DMD, DMSc,† Sadru Kabani, DMD, MS,‡ and Robert Moskal, DMD§ Oral melanoma is a rare disease, representing fewer than 1% of all melanomas.1 At the time of diagnosis cases are typically more advanced than the much more common cutaneous form.1 Whether this discrepancy is due to differences in clinical detectability or to a true difference in biological behavior is not clear. The prognosis for oral melanoma is quite poor, *Assistant Professor, Department of Oral Pathology, Boston University School of Dental Medicine, Boston, MA. †Professor, Department of Oral Pathology, Boston University School of Dental Medicine, Boston, MA. ‡Professor, Department of Oral Pathology, Boston University School of Dental Medicine, Boston, MA. §Private Practice, Franklin, MA. Address correspondence and reprint requests to Dr Kemp: 100 East Newton Street, Boston, MA 02118; e-mail: [email protected] © 2008 American Association of Oral and Maxillofacial Surgeons
0278-2391/08/6609-0025$34.00/0 doi:10.1016/j.joms.2007.11.010
with a 5-year survival rate of less than 50% according to most studies.1-3 We report a case of persistent melanoma in situ that presented clinically as a patchy brown macular lesion with irregular borders affecting the maxillary ridge and, later, the palate and buccal mucosa area of a 74-year-old male. There were 2 recurrences after excision over a 30-month period. The recurrences demonstrated atypical melanocytic hyperplasia, and no invasive component was detected in any of the biopsy specimens. Based on the poor prognosis of oral melanoma, close long-term follow-up of such a case is imperative. The clinical and microscopic features of the case are presented, along with a brief review of the relevant literature.
Report of a Case A 74-year-old Caucasian male presented in April 2003 with a brown macular lesion surrounding the buccal, pala-
BABER, ABUBAKER, AND LASKIN
the inflammatory effect of any viral infection, inhaled carbogen to improve the blood supply in the perilymph, dextran 70 to improve blood circulation, anticoagulants, vasodilators,2 antibiotics,5 and middle-ear exploration.1,4,8,10 Surgery is reserved for perilymph fistulae and for hearing deterioration with significant vertigo and acoustic neuroma.4 Important prognostic indicators include the severity of initial hearing loss and vertigo, time to initial audiogram, an elevated erythrocyte sedimentation rate, age less than 15 years and greater than 60 years, a midfrequency audiogram configuration, and hearing status of the opposite ear.1 Any of the potential causes suggested in the literature could be implicated in our case of SNHL. It is extremely important that a patient’s complaints are taken seriously. Early referral to an appropriate specialist for thorough assessment and treatment is mandatory. Acknowledgment Medical photography was performed at the Poole Hospital National Health Service Trust, Poole, UK.
References 1. Byl FM Jr: Sudden hearing loss: Eight years’ experience and suggested prognostic table. Laryngoscope 94:647, 1984 2. Plasse HM, Mittleman M, Frost J: Unilateral sudden hearing loss after open heart surgery. A detailed study of seven cases. Laryngoscope 91:101, 1981 3. Goodhill V, Harris I, Brockman SJ: Sudden deafness and labyrinthine window rupture. Ann Otol Rhinol Laryngol 82:2, 1973 4. Pau H, Selvadurai D, Murty GE: Reversible sensorineural hearing loss after non-otological surgery under general anaesthetic. Postgrad Med J 76:304, 2000 5. Park P, Toung JS, Smythe P, et al: Unilateral sensorineural hearing loss after spine surgery: Case report and review of literature. Surg Neurol 66:415, 2006 6. Farrel RW, Pemberton MN, Parker AJ, et al: Sudden deafness after dental surgery. Br Med J [Clin Res] 303:1270, 1991 7. Evan KE, Tavill MA, Goldberg AN, et al: Sudden sensorineural hearing loss after general anaesthesia for nonotological surgery. Laryngoscope 107:747, 1997 8. Goodhill V: Labyrinthine membrane ruptures in sudden sensorineural hearing loss. Proc R Soc Med 69:565, 1976 9. Segal S, Man A, Winerman I: Labyrinthine membrane rupture caused by elevated intratympanic pressure during anaesthesia. Am J Otol 5:308, 1984 10. Fisch U: Management of sudden deafness. Otolaryngol Head Neck Surg 91:3, 1983 J Oral Maxillofac Surg 66:1941-1945, 2008
Metastatic Neuroblastoma in the Mandibular Condyle: Report of a Case M. Andrew Baber, DDS,* Omar Abubaker, DDS, PhD,† and Daniel M. Laskin, DDS, MS‡ Neuroblastoma is a relatively common pediatric malignancy. It has a broad clinical spectrum ranging from spontaneously regressing disease with high survival to fulminating systemic progression.1 Presentation of neuroblastoma in the maxillofacial region is an infrequent occurrence and is often insidious. AlReceived from the Department of Oral and Maxillofacial Surgery, Virginia Commonwealth University Medical Center, School of Dentistry, Richmond, VA. *Formerly, Chief Resident; and Currently, Private Practice, Rogers, AR. †Professor and Chairman. ‡Professor and Chairman Emeritus. Address correspondence and reprint requests to Dr Abubaker: VCU School of Dentistry, Department of Oral and Maxillofacial Surgery, 520 North 12th Street, Room 239, Richmond, VA 232980566; e-mail: [email protected] © 2008 American Association of Oral and Maxillofacial Surgeons
0278-2391/08/6609-0024$34.00/0 doi:10.1016/j.joms.2007.06.689
though there are numerous case reports describing metastatic neuroblastoma to the mandible,2-13 there are no published case reports describing metastasis specifically to the mandibular condyle. We report a case of metastatic neuroblastoma of the mandibular condyle and review features of the disease pertinent to the oral and maxillofacial surgeon.
Report of a Case In September 2006, a 15-year-old Caucasian female presented to the Oral and Maxillofacial Surgery Clinic at the Virginia Commonwealth University Medical Center on referral from her general dentist. Her chief complaint was, “The left side of my face is swollen and it hurts.” The patient reported a 2- to 3-week history of moderate to severe leftsided facial pain that was aching in nature. Her mother had taken her to a general dentist who placed her on antibiotics for a questionable abscess. This treatment did not improve her symptoms. About 3 days before her visit to our clinic, the patient’s grandmother noticed the left side of the patient’s face was slightly swollen. When initially questioned, she felt as if her symptoms had been of limited duration, but
1942
NEUROBLASTOMA OF THE MANDIBULAR CONDYLE
when questioned further she admitted to having some mild intermittent pain in her face for the last 7 months and also having noticed that her teeth were not fitting together as they had previously. She denied any significant medical, surgical, family, or social history. A review of systems showed the patient had had a recent 20-pound weight loss, difficulty eating, and pain in the right shoulder, mid-thoracic back, and left lower quadrant of the abdomen. Focused physical examination showed a firm, slightly tender, 3 ⫻ 4 cm swelling of the left preauricular area (Fig 1). The chin was deviated slightly to the right and there was notable further deviation to the right on mouth opening. Maximum interincisal opening was 23 mm. Intraoral examination showed that the mandibular dental midline was deviated to the right about 3 mm and there was an edge-to-edge relationship of the right posterior teeth. The neck examination was negative for lymphadenopathy; however, there was notable swelling over the medial aspect of the right clavicle and slight enlargement of the thyroid gland. The cranial nerve examination was normal. A panoramic radiograph showed significant radiolucency of the left condyle, with indistinct trabeculation, loss of cortical borders, and a mottled appearance (Fig 2). The differential diagnosis included benign or malignant primary tumor and metastatic disease from a distant primary tumor. A maxillofacial and neck computed tomography (CT) scan was obtained and it showed a large, soft-tissue mass in the left masseteric space, bony destruction of the mandibular condyle, and a lytic lesion of the left mandibular ramus (Figs 3, 4). In addition, there also were multiple lytic lesions
FIGURE 2. Panoramic radiograph showing destruction of left mandibular condyle. Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
involving the medial aspect of the right clavicle and multiple areas of decreased attenuation in both lobes of the thyroid. A fine needle aspiration biopsy of the mandibular condyle was carried out, and the preliminary diagnosis was small, round, blue-cell tumor. At this point, the pediatric oncology service was consulted and asked to assume care of the patient. The patient was admitted to the pediatric oncology service and underwent a complete workup for malignant disease. The CBC and differential white cell count, INR, PT, PTT, urinalysis, metabolic panel, and liver and thyroid function tests were all normal. Lateral and PA chest x-rays; chest,
FIGURE 1. Notable left preauricular swelling.
FIGURE 3. Maxillofacial CT scan, coronal view showing bony destruction of the left mandibular condyle.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
BABER, ABUBAKER, AND LASKIN
1943
FIGURE 4. Maxillofacial CT scan, axial view showing large softtissue mass of the left masseteric space. Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008. FIGURE 6. Chest radiograph showing metastatic deposits.
abdomen and pelvis CT scans with contrast; head, cervical, thoracic, and lumbar magnetic resonance imaging; and whole body Tc-99 bone and I-131 tumor scans were obtained and these showed widespread metastatic disease, including masses in the chest, abdomen, and pelvis, as well as multiple sites of bony metastasis (Figs 5, 6). The patient underwent biopsy of her right clavicular mass and bilateral posterior iliac crest bone marrow biopsies to establish a definitive histologic diagnosis. Microscopic ex-
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
amination of the specimens showed a stroma-poor tumor with immunostains consistent with a poorly differentiated neuroblastoma (Figs 7, 8). The disease was deemed stage IV and, due to the widespread nature of the metastases, nonsurgical treatment was considered the best option. A portacath was placed and chemotherapy was initiated with vincristine, cyclophosphamide, and daunomycin, as well as
FIGURE 5. Abdominal CT scan showing large masses.
FIGURE 7. Histologic slide (H&E, 400⫻). The tumor consists of small, round, blue cells with a high nucleus to cytoplasmic ratio dissecting between skeletal muscle fibers. No evidence of maturation can be identified in this poorly differentiated neuroblastoma.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
1944
FIGURE 8. Histologic slide (H&E, 600⫻). At higher magnification, apoptotic bodies are easily identifiable (arrows). Baber, Abubaker, and Laskin. Neuroblastoma of the Mandibular Condyle. J Oral Maxillofac Surg 2008.
pegfilgrastim for myelosuppression. The tumors were responsive to this chemotherapeutic regimen and drastically decreased in size. The eventual plan was for an autologous stem-cell transplant.
Discussion Neuroblastic tumors include neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. They are classified into 1 of these 3 types based on the balance between neural-type cells and Schwann-type cells.1 Neuroblastoma typically represents one end of the spectrum as a poorly differentiated tumor, whereas ganglioneuroma is the well-differentiated benign counterpart on the other end of the spectrum.1,14 Neuroblastomas may be further classified as undifferentiated, poorly differentiated, or differentiating.15 Neuroblastoma is almost exclusively a tumor of children. More than 600 cases are diagnosed each year in the United States.16 It is the third most common childhood cancer overall and the most common solid extracranial tumor in children.15,16 It accounts for 6% to 10% of all childhood cancers and 15% of all pediatric cancer deaths in the United States.17 Incidence rates are age-dependent. The median age at diagnosis is 17.3 months, with 40% diagnosed before age 1 year.15 There is some ethnic predilection, with the incidence in infants being greater in Caucasians than African-Americans; however no ethnic differences are present among older children.15 Neuroblastomas arise from primordial neural crest cells that migrate during embryogenesis to form the adrenal medulla and sympathetic ganglia.17 They can arise anywhere throughout the sympathetic nervous system. However, the adrenal gland is the most common location.16,18 Adrenal neuroblasto-
NEUROBLASTOMA OF THE MANDIBULAR CONDYLE
mas are thought to arise from residual microscopic neuroblastic nodules that should normally regress by birth or shortly thereafter.19 Clinical presentation of neuroblastoma is varied and symptoms usually relate to the location of the primary or metastatic lesions. These symptoms often include abdominal masses and pain or constipation, back pain, bone pain, fever, weight loss, anemia, periorbital ecchymosis, and proptosis.20 Neuroblastomas metastasize by both hematogenous and lymphatic routes and may spread to a wide variety of locations including lymph nodes, bone marrow, cortical bone, dura, the orbits, liver, and skin.21 Tumor infiltration of the orbits is usually unilateral and causes a characteristic periorbital ecchymosis, ptosis, and proptosis.22 Because neuroblastomas arise from the sympathetic nervous system, they are characterized by defective catecholamine synthesis, which can result in the accumulation of the intermediates homovanillic acid, vanillylmandelic acid, and dopamine.23 These catecholamine intermediates may give rise to adrenergic symptoms such as tachycardia or hypertension.23 Treatment of neuroblastoma can consist of surgery, chemotherapy, radiation, or a combination of these modalities. Chemotherapy is the primary treatment for advanced-stage neuroblastomas.17 The tumor is generally considered radiosensitive, although there is little benefit for disease that is not in an advanced stage.17 However, in higher risk neuroblastomas, radiation has been shown to decrease local relapse rates.17 Of the 13 cases of metastatic neuroblastoma to the mandible referenced previously,2-13 there are no reported occurrences in the condyle. This illustrates the rare nature of this particular case. Although uncommon, neuroblastoma should still be included in the differential diagnosis when a radiolucency suspected of being a metastatic lesion is discovered in the mandible of a child. Such patients may have deviation of the mandible on mouth opening, changes in the occlusion, mobile teeth, and unexplained pain. Key clinical findings related to metastatic neuroblastoma in other parts of the maxillofacial region include proptosis, periorbital ecchymosis, Horner’s syndrome, heterochromidia iridis (different colors of the iris or portions of the iris),23 and masses.
References 1. Russell H, Shohet J, Nuchtern J: Epidemiology, pathogenesis, and pathology of neuroblastoma. Up To Date Online 14.2:1, 2007 2. Bhattacharyya I, Williamson A, Cohen DM, et al: Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 88:586, 1999
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KEMP ET AL 3. Chan AR, Johnston DH, Stoneman D: Disseminated neuroblastoma with initial presentation as an intraoral mass: Case report. Pediatr Dent 16:310, 1994 4. Haddad A, Azouz EM, al-Jedher S, et al: Metastatic neuroblastoma presenting as a mandibular mass. Can Assoc Radiol J 43:436, 1992 5. Borle RM, Hazare VK, Bhowate RR, et al: Neuroblastoma metastatic to the mandible. J Oral Maxillofac Surg 49:1124, 1991 6. Boraz RA: Neuroblastoma: Case involving metastases to the mandible. Pediatr Dent 7:315, 1985 7. Newland JR, McClendon JL, Lynch DP: Metastatic neuroblastoma of the jaws. J Oral Maxillofac Surg 43:549, 1985 8. Hardt NP: Metastatic neuroblastoma in the mandible. Report of a case. Oral Surg Oral Med Oral Pathol 41:314, 1976 9. Snyder MB, Cawson RA: Jaw and pulpal metastasis of an adrenal neuroblastoma. Oral Surg Oral Med Oral Pathol 40:775, 1975 10. Chakrabarty PB, Das S, Dash BC: Neuroblastoma with metastasis to mandible. Report of a case and review of the literature. Indian J Cancer 12:219, 1975 11. Angelopoulos AP, Tilsn HB, Stewart FW: Metastatic neuroblastoma of the mandible: Review of literature and report of case. J Oral Surg 30:93, 1972 12. DeLeon EL, Finney RA, Ruth A, et al: Neuroblastoma with metastasis to maxilla and mandible: Review of literature and report of case. J Oral Surg 28:773, 1970 13. Hoffman S, Green GH: Neuroblastoma with metastasis to the mandible: Report of case. J Oral Surg 24:75, 1966 14. Peuchmaur M, d’Amore ES, Joshi VV, et al: Revision of the International Neuroblastoma Pathology Classification: Confir-
15.
16.
17. 18.
19. 20. 21. 22. 23.
mation of favorable and unfavorable prognostic subsets in ganglioneuroblastoma, nodular. Cancer 98:2274, 2003 Schwab M, Shimada H, Joshi V, et al: Neuroblastic tumours of adrenal gland and sympathetic nervous system, in Pathology and Genetics of Tumours of the Nervous System. Lyon, France, World Health Organization, 2000 Goodman MT, Gurney JG, Smith MA, et al: Sympathetic nervous system tumors. Cancer Incidence and Survival among Children and Adolescents: United States SEER Program, 19751995. Bethesda, MD, National Cancer Institute, 1999 Kim S, Chung D: Pediatric solid malignancies: Neuroblastoma and Wilms tumor. Surg Clin N Am 86:469, 2006 Castleberry RP, Shuster JJ, Smith EI: The Pediatric Oncology Group experience with the international staging system criteria for neuroblastoma. Member Institutions of the Pediatric Oncology Group. J Clin Oncol 12:2378, 1994 Brodeur GM, Maris JM: Principles and Practice of Pediatric Oncology. Philadelphia, PA, Lippincott Williams & Wilkins, 2002 Golden CB, Feusner JH: Malignant abdominal masses in children: Quick guide to evaluation and diagnosis. Pediatr Clin North Am 49:1369, 2002 DuBois SG, Kalika Y, Lukens JN, et al: Metastatic sites in stage IV and IVS neuroblastoma correlate with age, tumor biology, and survival. J Pediatr Hematol Oncol 21:181, 1999 Musarella MA, Chan HS, DeBoer G, et al: Ocular involvement in neuroblastoma: Prognostic implications. Ophthalmology 91: 936, 1984 Russell H, Shohet J, Nuchtern J: Clinical presentation, diagnosis, and staging evaluation of neuroblastoma. Up To Date Online 14.2:1, 2007 J Oral Maxillofac Surg 66:1945-1948, 2008
Persistent Melanoma In Situ: Case Report and Review Spencer Kemp, DDS,* George Gallagher, DMD, DMSc,† Sadru Kabani, DMD, MS,‡ and Robert Moskal, DMD§ Oral melanoma is a rare disease, representing fewer than 1% of all melanomas.1 At the time of diagnosis cases are typically more advanced than the much more common cutaneous form.1 Whether this discrepancy is due to differences in clinical detectability or to a true difference in biological behavior is not clear. The prognosis for oral melanoma is quite poor, *Assistant Professor, Department of Oral Pathology, Boston University School of Dental Medicine, Boston, MA. †Professor, Department of Oral Pathology, Boston University School of Dental Medicine, Boston, MA. ‡Professor, Department of Oral Pathology, Boston University School of Dental Medicine, Boston, MA. §Private Practice, Franklin, MA. Address correspondence and reprint requests to Dr Kemp: 100 East Newton Street, Boston, MA 02118; e-mail: [email protected] © 2008 American Association of Oral and Maxillofacial Surgeons
0278-2391/08/6609-0025$34.00/0 doi:10.1016/j.joms.2007.11.010
with a 5-year survival rate of less than 50% according to most studies.1-3 We report a case of persistent melanoma in situ that presented clinically as a patchy brown macular lesion with irregular borders affecting the maxillary ridge and, later, the palate and buccal mucosa area of a 74-year-old male. There were 2 recurrences after excision over a 30-month period. The recurrences demonstrated atypical melanocytic hyperplasia, and no invasive component was detected in any of the biopsy specimens. Based on the poor prognosis of oral melanoma, close long-term follow-up of such a case is imperative. The clinical and microscopic features of the case are presented, along with a brief review of the relevant literature.
Report of a Case A 74-year-old Caucasian male presented in April 2003 with a brown macular lesion surrounding the buccal, pala-