Idiopathic cervical fibrosis-a case report with review of literature

Abstract

Idiopathic cervical fibrosis is a rare fibrosclerosing lesion of the head and neck region simulating a malignant process. It is designated as a tumefactive fibroinflammatory lesion constituting a heterogeneous clinical condition with an unknown cause and pathogenesis. Infection, Hodgkin’s lymphoma, sarcoidosis, Autoimmune disease, trauma was considered as etiological factors in the past but recently considered to be idiopathic. We report a case of idiopathic cervical fibrosis involving the jaw destroying the ramus of the mandible and extending into the adjacent soft tissue planes upto skin. Biopsy revealed dense fibrous tissue, bands of hyalinised collagen admixed with inflammatory infiltrate composing of lymphocytes, plasma cells encasing vascular and neural structures. Lymphocyte markers (CD 3 & CD20) and vimentinwas done to confirm the lymphocytes and fibrous tissue. A diagnosis of Idiopathic cervical fibrosis was made after excluding Fibromatosis, Nodular Fascitis, Malignant Fibrous Histiocytoma and Fibrosarcoma. It is difficult to diagnose these conditions clinically and the X-ray and CT findings may be misleading. Biopsy is the only way to make the diagnosis along with the added marker study and IHC profile. Treatment includes surgical excision, steroid therapy and radiation used alone or in combination.