A rare case of Juvenile Fibromatosis infiltrating neck subcutis in a 3-year old girl

  • Amit Kumar Senior Resident Department of Pathology Mahavir Cancer Institute And Research Centre, Patna (Bihar)
  • Rakesh Mehra Consultant Radiologist Department of Radiology Mahavir Cancer Institute And Research Centre, Patna (Bihar)
  • Tanushree Narain DNB Resident Department of Pathology Mahavir Cancer Institute And Research Centre, Patna (Bihar)
  • Neha Garg DNB Resident Department of Pathology Mahavir Cancer Institute And Research Centre, Patna (Bihar)
  • Pallavi Agrawal Consultant Pathologist M.D., DNB, PDCC Department of Pathology Mahavir Cancer Institute And Research Centre, Patna (Bihar)
Keywords: Juvenile, Fibromatosis, neck, submandibular

Abstract

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.

References

1. Basha SM, Dutt SC, Murthy NS, Syed G. Juvenile fibromatosis of the temporomandibular joint: A rare case report. Dent Res J, 2014; 11(2): 284-87.
2. Wilkins SA, Waldron CA, Matthews WH et al. Aggressive fibromatosis of the head and neck. Am J Surg. 1975; 130: 412-16.
3. Seper L, Burger H, Vormoor J, Joos U, Kleinheinz J. Aggressive fibromatosis involving the mandible: Case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005:99; 30-8.
4. Lee JC, Thomas JM, Phillips S et al. Aggressive fibromatosis: MRI features with pathologic correlation. Am J Roentgenol, 2006; 186(1): 247-54.
5. Carr RJ, Zaki GA, Leader MB et al. Infantile fibromatosis with involvement of the mandible. Brit J Oral Maxillofacial Surg. 1992: 30; 257-262.
6. Perez Cruet MJ, Burke JM. Weber R et al. Aggressive fibromatosis involving the cranial base in children. Neurosurgery, 1998;43: 1069-102.
7. Debner Louis P. Soft Tissue, Peritoneum and Retroperitoneum: In pediatric Surgical Pathology. 2nd Ed. Baltimore, USA. Williams and Wilkins 1987: 885-90.
8. Halperin E, Brady L, Perez C, Wazer D. Principles and practice of radiation oncology, 6th Ed. Lippincott, Williams and Wilkins, Philadelphia 2013;(78):1535.
Published
2016-05-08
Section
Case Report