Recurrent hard palate lesion in a child

J Oral Max\ilofac Surg 57:l 127-l 131, 1999

Recurrent

Hard

Palate

Lesion

in a Child

Roberl D. Mu&am, DMD, * Dean K. White, DDS, MSD, f and Jack E. Gotcher, Jr, DMD, PbD$ Case Presentation

Differential

A 6-year-old boy was referred on January 25, 1997 for further treatment of a recurrent midline hard palate lesion. The mass was present for approximately 8 weeks before it was treated with an incisional biopsy and local extirpation. It recurred 3 weeks after the local incision. The patient had no specific head and neck, oral or nasal complaints previous to the original biopsy and treatment. His medical history was significant for seasonal allergic rhinitis and recurrent upper respiratory infections, At the time of his presurgery workup, he was taking amoxicillin 250 mg 3 times daily and augmentin 250 mg 3 times daily for a “touch of pneumonia.” Physical examination showed a 2 X 2.5 cm pedunculated midline mass on the posterior hard palate extending to the vibrating line of the soft palate (Fig 1). The lesion was erythematous and superficially ulcerated. No other significant head and neck findings were noted. Adventitious breath sounds were heard bilaterally, and a slight productive cough was present. The patient weighed 95 lb and was 4 feet 3 inches tall. No other general body physical examination abnormalities were recorded. Coronal and axial computed tomography (CT) without contrast of the midface showed a sharply punched out, well-demarcated body defect of the hard palate located posterior and to the left of the midline (Fig 2). The oral soft tissue component of the bony lesion was obscured by the proximity of the tongue and could not be distinguished radiographically. The bony involvement appeared to extend superiorly to the posterior aspect of the bony nasal septum. A soft tissue component was noted extending into the left nasopharynx. Routine red blood cell count, hematocrit, hemoglobin measurement, and platelet count were within normal limits. The white blood cell count was slightly elevated (13.9 K&L) and the mean corpuscular volume (73 pm3) and mean corpuscular hemoglobin concentration (34 g/dL) were slightly depressed.

Jack E. Gotcher, Jr, DMD, PhD

*Staff Affairs;

Oral

and Maxillofacial

Professor

Surgery,

and

University

Surgeon,

Division

of

Kentucky

Chief

Department of

Oral

College

of

In the initial assessment of this patient, the typical screening categories of neoplastic, inflammatory, infectious, and developmental abnormalities should be considered. Given the short time of onset in this 6-year-old child, the category of a developmental abnormality may be excluded initially. Inflammatory lesions also may be largely excluded, except as causative factors in masses, such as described later. When considering the category of infectious lesions, the findings of a slight productive cough and adventitial breath sounds, along with a slightly elevated white blood cell count, indicate that some form of infectious pulmonary process may be present. No mention is made of positive findings on a chest radiograph, which might be useful in this differential diagnosis. Among the infectious pulmonary diseases that have oral manifestations is tuberculosis. This chronic infectious disease is becoming more common in the United States. The primary infection may be asymptomatic, or accompanied by a fever and productive cough. Oral lesions are certainly more uncommon than pulmonary lesions and are frequently secondary infections arising from sputum or blood. Such lesions are found on the palate, tongue, and lips, and are usually associated with regional lymphadenopathy. The oral lesions may appear as an ulcer or a mass, such as the described case. Evidence of exposure is based on a positive tuberculin or purified protein derivative test, and the presence of active disease is related to the finding of lesions in the lung apex (or other areas) on a chest radiograph. Confirmation is made by mycobacterial strains of tissue obtained at biopsy, along with culture of infected sputum. Histoplasmosis is a common fungal infection that may have oral lesions in addition to the more common pulmonary findings. Acute histoplasmosis is usually asymptomatic or only mildly symptomatic, with flulike symptoms. The disease may progress to a chronic form with cough, weight loss, fatigue, and infiltrative lesions on chest radiographs. Oral lesions usually present as ulcerative areas, although some lesions appear as a white or red irregular surface. Oral lesions are generally limited to the severe, disseminated form of histoplasmosis, often found in debilitated or immu-

of Veterans

and

Maxillofacial

Dentistry,

Lexing-

ton, KY tprofessor

and Head,

of Kentucky

College

*Associate

Professor

and Maxillofacial Knoxville, Address Division College

Oral and Maxillofacial

of Dentistry, and Acting

Surgery,

TN. correspondence

University and

of Oral and Maxillofacial of Dentistry, 800 Rose

Pathology,

Lexington,

Department

of Tennessee reprint

University

KY.

Chairman,

Medical

requests

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to Dr Marciani:

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of Kentucky 40536; e-mail:

[email protected] o 1999 American

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FIGURE extending nontender.

RECURRENT

1. View of the pedunculated, ulcerated, midline palatal mass to the vibrating line of the soft palate. The mass was soft and

nocompromised patients. There is no evidence of such a compromised physical status in the patient in this case. More uncommon than histoplasmosis is the fungal disease blastomycosis. Acute and chronic blastomycosis present similarily to histoplasmosis, with the initial infection being asymptomatic or only mildly symptomatic. As in histoplasmosis, the oral lesions usually appear as ulcerative areas, but sometimes as a reddish or white intact surface. Oral lesions usually result from disseminated disease, although local primary disease can occasionally occur. Blastomycosis is usually diagnosed by KOH cytologic preparations or histologic sections. Blastomycosis may also be cultured from fresh biopsy or cytologic material. Again, there is no evidence of any compromise in the immune system or physical status of the child in this case. In the category of neoplastic lesions, a number of tissues may have the potential to form a lesion such as described. In this area, connective tissue, neural, muscle, salivary gland, and hematologic sources need to be considered. Benign connective tissue neoplasia includes fi-

FIGURE 2. An axial demarcated, punched-out

CT scan of the palate showing lesion of the posterior hard palate.

a well-

HARD

PALATE

LESION

broma, fibrous histiocytoma, fibromatosis, myofibromatosis, and peripheral giant cell granuloma. The fibroma, while very common, usually is a smooth pink nodule, and it is rarely found in the palate, Also, the fibroma does not recur on removal, unlike the mass described here. The giant cell fibroma deserves mention because it occurs in the first 3 decades of life and is common on the palate. However, it has a different appearance, with a nodular or papillary surface, and it rarely recurs. The peripheral giant cell granuloma is fairly common and has a 10% recurrence rate. However, it is almost always found on the gingiva, and usually in older adults. Fibromatosis of the craniofacial area presents as a firm mass of either rapid or slow onset. It most commonly occurs in children and young adults. Fibromatosis can erode or destroy adjacent bone, as described in this case. It has a behavior between a benign lesion and a fibrosarcoma, and has been reported to have a 23% recurrence rate. Myofibromatosis has a similar clinical appearance to fibromatosis, although myofibromatosis acts in a more benign manner than fibromatosis. This neoplasm is a proliferation of myofibroblasts, with characteristics of both fibroblasts and smooth muscle. Malignant lesions of fibrous tissue origin to be considered include fibrosarcoma and malignant fibrous histiocytoma. The fibrosarcoma usually presents as a slow-growing mass, more commonly in children and young adults. The biologic behavior of some fibrosarcomas may resemble the more aggressive variation of the fibromatoses, with recurrence rates from 20% to 60%. Finally, the malignant fibrous histiocytoma presents as an expanding mass, with or without ulceration, similar to the fibrosarcoma. This tumor occurs mainly in older persons and has a recurrence rate of about 40%. Benign neural tissue neoplasms to be considered in this differential diagnosis include the palisaded, encapsulated neuroma, the neurilemoma or schwannoma, the neurofibroma, and neurofibromatosis. Although the palisaded encapsulated neuroma is common, and may occur on the hard palate, it rarely recurs, and is usually found in an older group than the case described (usually 5th to 7th decade). Also, the lesions are smaller than the lesion described here. The Schwannoma is an uncommon neoplasm arising from the Schwann cell at the nerve periphery. It is slow growing, encapsulated, and is associated with a nerve trunk. The midline palatal position of the mass described is unlikely to be a Schwannoma, because there are no major nerve trunks in the area. Also, the Schwannoma does not recur, in contrast to the lesion in question. The neurofibroma, which contains elements of both Schwann cells and fibroblasts, is a common nerve neoplasm, occurring in young persons as a slow-growing, painless mass. However, this lesion

MARCMNI,

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does not have a significant recurrence rate. Neurofibromatosis is a fairly common condition that is hereditary in nature, although many patients with neurofibromatosis will have no family history of the disease. Patients with neurofibromatosis typically present with multiple cutaneous nodules representing neurofibromas, as well as the presence of multiple cafe au Zait spots on the skin. However, the finding of an isolated mass, and the absence of associated findings in this case, are inconsistent with a diagnosis of neurofibromatosis. Neurofibromatosis may recur or transform into neurofibrosarcoma in about 5% of cases. Neurofibrosarcoma is an uncommon malignancy of peripheral nerves, with about 50% of cases arising from neurofibromatosis. This tumor most commonly occurs in young adults, sometimes presenting as a rapidly growing mass. Tumors typically occur in areas of the oral cavity other than the hard palate. Recurrence is common and the overall prognosis is poor. Benign lesions of muscle origin include the leiomyoma and rhabdomyoma. The former is a neoplasm of smooth muscle that presents as a slow-growing, firm nodule, which may occur in the hard palate. It usually does not recur after excision. The rhabdomyoma of the fetal type may occur in children. It is a benign neoplasia of skeletal muscle origin and presents clinically as a single or nodular mass and may occur on the soft palate. Recurrence of this lesion is not common, but has been reported. Malignant lesions of muscle tissue to be considered in the differential diagnosis include the rhabdomyosarcoma and the alveolar soft part sarcoma. The rhabdomyosarcoma is the most common soft tissue sarcoma in children. This tumor, especially the embryonal type, occurs mainly in the first decade of life. It is rapidly growing mass, with the palate being a common intraoral site. The alveolar soft part sarcoma is a rare neoplasm of uncertain origin. It commonly occurs in children and, curiously, mostly tiects the right side of the body. Tumors have been reported in the pharyngeal area. It has a high rate of recurrence and a poor prognosis. Uncommonly, minor salivary gland pathology may be responsible for a mass in the hard and soft palate region in children. Benign tumors, such as pleomorphic or monomorphic adenoma, can be found in the palate, but are typically seen in older adults, not children. Malignancies, such as mucoepidermoid carcinoma, are also possible, but are also unlikely in the pediatric population. Both benign and malignant lymphoproliferative disorders may be responsible for masses in the palate of children. Lymphoid hyperplasia can develop in the posterior hard palate due to enlargement of extranodal lymphoid tissue. The lesion appears as a slowgrowing, boggy swelling underneath the intact oral

mucosa. Malignant lymphoproliferative disorders, such as acute lymphocytic leukemia or extranodal nonHodgkin’s lymphoma, also may produce similarappearing masses in the hard and soft palate of children. A tumor-like growth of leukemia cells in an extranodal site is referred to as a chloroma. The lack of significant changes in the peripheral blood of this patient would tend to weigh against a lymphoproliferative disorder, although some changes might be present only in the marrow in the early stages. To summarize, several infectious and neoplastic conditions, both benign and malignant, have to be considered as possibilities in this case of a recurring hard palate mass in a b-year-old child. From this discussion, the most likely diagnostic possibilities would be (in order of most likely to least likely): neurofibroma or neurofibromatosis, fibromatosis, myofibromatosis, leukemic chloroma, extranodal nonHodgkin’s lymphoma, benign lymphoid hyperplasia, tuberculosis, rhabdomyoma, fibrosarcoma, and giant cell fibroma. The diagnosis of neurofibroma/neurofibromatosis fits several of the characteristics given in the patient’s history and physical examination. SUBSEQUENTCOURSE After the patient’s respiratory infection had improved, he was taken to the operating room and placed under general anesthesia. Specimens secured at the original incisional biopsy were previously reviewed and considered. Afibrohistiocytic reaction and chronic inflammation were reported, with the caveat that the original specimen might represent a fibrous histiocytoma or a fibrous reaction to an adjacent focus of infection. The circumference of the palatal lesion was identified, and an incision site was transcribed with a marking pen approximately 0.5 cm from the oral margins of the lesion. Electrocautery and a scalpel were used to create a full-thickness soft tissue incision that extended through periosteum to bone over the hard palate and into the depth of the muscular soft palate posteriorly (Fig 3). Periosteum,

FIGURE 3. The intraoperative appearance 1 after the initial surgical outlining incision.

of the lesion seen in Figure

1130

FIGURE 4. A

pedicled posterior palatal flap transposed from the right side of the palate to cover the soft tissue defect created by the extirpation of the lesion seen in Figure 1

nasal rnucosa, and soft palate tissue were reflected or incised in mass, delivered as a single piece, and sent for frozen section microscopic examination. The frozen section diagnosis was “spindle cell neoplasm, most probably benign; definitive diagnosis deferred to permanent sections.” The bony defect was reduced with a round bur on a rotary handpiece, and a full-thickness mucoperiosteal palatal flap pedicled off the right anterior palatine artery was developed and transposed to cover the surgical defect @ig 4). A previously constructed palatal splint was relined and secured in position on the palate with interdental wires. Microscopic examination of permanent sections stained with hematoxylin and eosin and immunohistochemical stains, and electron microscopy resulted in “findings consistent with myofibromotosis” @ig 5). The patient experienced an uneventful postoperative course. Clinical examinations (Fig 6) and postsurgery axial

RECURRENT HARD PALATE LESION

FIGURE 6. palate

The healed palatal flap 5 months is entirely covered with soft tissue.

postoperatively.

The

and coronal CT scans secured at 5 months were unremarkable for new or recurrent tumor growth. One year posttreatment, the patient denied oral-nasal reflux or nasal obstruction. Continued follow-up examinations and periodic CT scans are planned.

Discussion Fibromatosis and myofibromatosis are considered part of a broad group of fibrous proliferations. The fibromatoses have a biologic behavior and histopathologic pattern intermediate between benign fibrous lesions and fibrosarcoma. Myofibromatosis is a benign proliferation composed of fibroblasts and myofibroblasts, which are cells that exhibit features of smooth muscle. The

FIGURE

5. Photomicrograph showing plump, spindle-shaped cells, some with prominent eosins ahilic cvtoalasm. The cells form / I small bundles and swirls and are embedded in a matrix of collagen and ground substance. little variation in cell size, shape, and staining characteristics exists [hematoxylineosin stain, original magnification x201

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lesion is not encapsulated, exhibits an infiltrative growth pattern, and does not metastasize. Most cases develop in neonates and infants, and it is only rarely reported in adults. Solitary and multicentric nodular forms, with and without visceral involvement, have been described. Conversely, fibromatosis of the head and neck, usually occurs in young children or young adults.‘J Myofibromatosis most frequently originates as a solitary lesion in the dermis or subcutaneous tissues of the head and neck, but multiple lesions may occur. In the case presented, bony involvement probably represented erosion of the lesion into bone rather than a true intraosseous lesion that expanded into the oral and nasal cavities. Infantile and adult myofibromatosis subtypes have been reported based on their clinicopathologic features. Two forms of the process are recognized: multicentric and solitary. The multicentric form occurs in infants and may tiect the dermis, muscle, internal organs, and skeleton. The solitary form is more common than the multicentric variety and is found in the head and neck region, including the oral cavity. It is twice as common in males than in females and occurs in infants, children, and adults. Solitary infantile myofibromatosis, or myofibroma of the oral cavity, is an uncommon condition, with only 32 reported cases in the English language literature.3 Four additional cases of these solitary myofibroblastic lesions have been presented.3 Similar lesions have been reported in the nasal cavity,* periorbita,5 skull6 and orbital bone.’ Solitary cutaneous myofibroma in adults is described as being similar in appearance to that of infantile myofibromatosis. The differential diagnosis in the cutaneous adult form should include neurothekeoma, plexiform fibrous histiocytoma, nodular fasciitis, cutaneous inflammatory pseudotumors, dermatomyofibroma, leiomyoma, and other forms of fibromatosis tiecting the skin and superficial soft tissues.8 Microscopically, myofibromatosis comprises a proliferation of both fibroblasts and myofibroblasts that show ultrastructural features similar to fibroblasts and smooth muscle cells, respectively. The infantile form often shows a biphasic pattern consisting of plump, spindle-shaped cells that exhibit a prominent eosinophilic cytoplasm and are arranged in short bundles (fascicles) or nodules, or in areas that appear hemangiopericytoma-like. The combination is less noticeable in lesions found in older patients, and the lesion may consist of sheets of fibroblast-like cells arranged in fascicles and supported by collagen and ground sub-

stance. Mitotic figures may be present, and some cellular variation may exist. Immunohistochemical staining of lesional tissue shows an admixture of cells. Some are positive only for vimentin, an intermediate cytoskeletal filament typically associated with mesenchymal cells, and represent fibroblasts. Other cells are positive for vimentin and smooth muscle actin and are representative of myofibroblasts. Ultrastructurally, cells typical of fibroblasts are present, and cells that also exhibit cytoplasmic microfilaments with areas of focal condensation beneath the cell membrane are evident. These are consistent with myofibroblasts. Generally, solitary and multicentric lesions confined to the soft tissue and bone have an excellent prognosis. They tend to regress spontaneously in infants; in older patients there is little tendency for regression.9 The prognosis is much less favorable (death may occur) in newborns and infants with multiple visceral lesions because of respiratory and intestinal involvement.1° The clinical differential diagnosis of the solitary form in the oral cavity includes many of the connective tissue, lymphoid, and salivary gland neoplasms. The microscopic differential diagnosis includes fibrous histiocytoma, nodular fasciitis, neurofibroma, leiomyoma, and other forms of fibromatosis. Combinations of light microscopic, immunohistochemical, and electron microscopic examinations help in differentiating these neoplasms.

References 1. Neville BW, Damm DD, Allen CM, et al: Oral and Maxillofacial Pathology. Philadelphia, PA, Saunders, 1995, p 369 2. Beham A, Badve S, Suster S, et al: Solitary myofibroma in adults: Clinicopathological analysis of a series. Histopathology 22:335, 1993 3. Lingen MW, Mostofi RS, Solt DB: Myofibromas of the oral cavity. Oral Surg Oral Med Oral Path Oral Radio1 Endod 80:297, 1995 4. Walsh RM, Leen EJ, Gleeson MJ: Myofibromatosis of the nasal cavity: Report of two cases. J Laryngol Otol 110:574, 1996 5. Linder JS, Harris GJ, Sequra AD: Periorbital infantile myofibromatosis. Arch Ophthalmol 114:219, 1996 6. Queralt JA, Poirier VC: Solitary infantile myofibromatosis of the skull. AJNRAm J Neuroradiol16:476, 1995 7. Dully MT, Harris M, Hornblass A: Infantile myofibromatosis of orbital bone: A case report with computed tomography, magnetic resonance imaging, and histologic findings. Ophthalmology 104:1471,1997 8. Guitart J, Ritter JH, Wick MR: Solitary cutaneous myofibromas in adults: Report of six cases and discussion of differential diagnosis. J Cutan Path01 23:437, 1996 9. Requena L, Kutzner H, Huge1 H, et al: Cutaneous adult myofibroma: A vascular neoplasm. J Cutan Path01 23:445, 1996 10. Enzinger FM, Weiss SW: Soft Tissue Tumors (ed 3). St Louis, MO, Mosby Year Book, 1995, pp 238-245