A Rare Case of Juvenile Fibromatosis Infiltrating Neck Subcutis in a 3-year Old Girl

Background: Fibromatosis is a rare benign tumor of fibro-myofibroblastic origin. This non-metastasizing tumor has high potential to locally invade and recur after surgical excision. Since 1950, only 99 cases of fibromatosis of head and neck in pediatric age-group are reported in the literature. We present the 100th case in this category. Case-report: A three year old girl presented with a right sided neck mass progressively increasing in size since one year. On imaging the tumor was found extending from submandibular to supraclavicular region, which on incisional biopsy showed features consistent with the diagnosis of Juvenile Fibromatosis. Histologically, it may be confused with other entities under broad diagnostic category ‘fibromatoses’, but immuno-histochemical analysis confirmed the diagnosis. Patient underwent wide local excision and was followed-up for two years with no features of recurrence. Conclusion: A rare and interesting tumor of pediatric age group with high tendency to recur after surgical excision. But surgical resection with tumor free margins prognostically decreases the chance of recurrence. Case Report *Corresponding author: Dr. Pallavi Agrawal, (M.D., DNB, PDCC) Consultant Pathologist, Room no. 314, Department of Pathology, Mahavir Cancer Institute And Research Centre, Patna (Bihar) 801505, India Phone: +91 7739165410 E-mail: [email protected]