Juvenile Hyaline Fibromatosis- A rare cause of cervical swelling
Keywords:
Juvenile Hyaline Fibromatosis, Submandibular Swelling, Mandibular Osteolysis, Histopathology, Case Report.Abstract
Background: Juvenile Hyaline Fibromatosis (JHF) is a rare autosomal recessive connective tissue disorder characterized by abnormal hyaline deposition. Mandibular involvement is uncommon and may mimic malignancy.
Case Presentation: A 6-year-old female child presented with a gradually progressive left submandibular swelling for four months. Examination revealed a firm, non-tender mass measuring 6 × 4 cm. Ultrasonography demonstrated a heterogeneous lesion, while fine-needle aspiration cytology suggested a spindle-cell lesion. Computed tomography revealed extensive osteolytic changes involving the left mandibular body and ramus. Surgical exploration revealed a dumbbell-shaped mass extending into the floor of the oral cavity with associated mandibular destruction. Complete gross excision was performed. Histopathological examination demonstrated spindle-shaped cells arranged in fascicles with intervening hyaline bands, suggestive of Juvenile Hyaline Fibromatosis.
Conclusion: JHF should be considered in the differential diagnosis of paediatric submandibular swellings with mandibular osteolysis. Histopathological evaluation is essential for diagnosis, while long-term follow-up is recommended because of the risk of recurrence.
