Abstract
Neuroblastoma is an uncommon cause of a mandibular mass. We describe an 8-month-old child who presented with loosening of his dentition, poor oral intake, and a large mandibular mass. Thorough radiographic study and subsequent biopsy of the oral lesion revealed the diagnoses of metastatic neuroblastoma. Ultimately, he was successfully treated with surgery and chemotherapy. The unusual presentation and pathophysiology of this malignancy will be reviewed. Neuroblastoma presenting as a mandible mass associated with natal teeth is rare, but it should be considered in the differential diagnosis of pediatric mandibular lesions.
1
Introduction
Neuroblastoma is the most common extracranial malignancy of childhood and the most common solid tumor of infancy . This tumor typically originates in the adrenal gland; however, it may present in the mediastinum and rarely in the head and neck. Disseminated disease at the time of diagnosis is very common and may involve the skull, periorbital bones, bone marrow, and liver . Involvement of the mandible as the initial presentation is extremely rare .
2
Case report
An 8-month-old boy presented with iron refractory anemia and right mandibular swelling. The parents reported a 5-week history of mandibular swelling that was previously diagnosed by both his pediatrician and pediatric dentist as an eruption cyst. Since the initial diagnosis, however, the parents reported increasing growth of the mandibular mass, intraoral bruising, loosening dentition, and progressive weight loss due to poor oral intake.
Clinical examination of the mandible revealed a hard fixed mass filling the buccal sulcus from the angle of the right mandible to the body of the left mandible with extension into the right sublingual and submental space. Lateral and central incisors were loose. The oral mucosa was intact with ecchymotic discoloration. No lymphadenopathy was noted. Further examination revealed a right upper quadrant abdominal mass.
Computed tomographic (CT) images of the head and neck revealed a 5 × 7–cm mandibular mass with osseous destruction greatest on the right, and mottling of the periorbital and frontal bones without intracranial involvement ( Fig. 1 ). Abdominal CT revealed an 8 × 6–cm mass originating at the superior pole of the right kidney, extending across midline, along with extensive periaortic and pericaval adenopathy.
Transoral incisional biopsy of the mandible mass revealed a small blue-cell tumor. Histologic evaluation with hematoxylin and eosin noted irregular groupings of cells containing inconspicuous nuclei with fine chromatin and ill-defined cytoplasm ( Fig. 2 ). Immunohistochemical evaluation demonstrated positivity for neuron-specific enolase and negativity for neurofilament, vimentin, CD99, leukocyte common antigen, desmin, myogenin, MyoD1, cytokeratin, and epithelial membrane antigen. Further studies revealed bone marrow involvement, elevated urine catecholamines, and nonamplified N-myc. I-123 metaiodobenzylguanidine (MIBG) scintigraphy displayed increased uptake in the mandible, anterior skull, right renal fossa, and bilateral upper and lower extremities.
The patient was diagnosed with stage IV neuroblastoma of intermediate risk and underwent chemotherapy per the Children’s Oncology Group protocol ABLN0531. He experienced a profound result from chemotherapy and had a complete disease remission in the mandible. Right adrenalectomy and abdominal node dissection were performed to remove residual tumor. He is almost 2 years status posttreatment and without radiographic evidence of tumor in the mandible or abdomen, is symptom free, and is gaining weight.
2
Case report
An 8-month-old boy presented with iron refractory anemia and right mandibular swelling. The parents reported a 5-week history of mandibular swelling that was previously diagnosed by both his pediatrician and pediatric dentist as an eruption cyst. Since the initial diagnosis, however, the parents reported increasing growth of the mandibular mass, intraoral bruising, loosening dentition, and progressive weight loss due to poor oral intake.
Clinical examination of the mandible revealed a hard fixed mass filling the buccal sulcus from the angle of the right mandible to the body of the left mandible with extension into the right sublingual and submental space. Lateral and central incisors were loose. The oral mucosa was intact with ecchymotic discoloration. No lymphadenopathy was noted. Further examination revealed a right upper quadrant abdominal mass.
Computed tomographic (CT) images of the head and neck revealed a 5 × 7–cm mandibular mass with osseous destruction greatest on the right, and mottling of the periorbital and frontal bones without intracranial involvement ( Fig. 1 ). Abdominal CT revealed an 8 × 6–cm mass originating at the superior pole of the right kidney, extending across midline, along with extensive periaortic and pericaval adenopathy.
