Primary fibrosarcoma of maxilla in an 8‑year‑old child: A rare entity

Abstract

Fibrosarcoma (FS) is a malignant mesenchymal neoplasm of the fibroblasts that is uncommon in the head and neck and constitutes less than 1% of malignancies and approximately 6% of the soft tissue sarcomas. FSs rarely occur before the third decade except infantile type. This condition primarily affects long bones, and its occurrence in the cranium is rare (15%), with the mandible being the most commonly involved cranial site. Here a case of primary FS in anterior maxilla of an 8-year-old male child is reported. This article is presented to document the rarity of FSs in the jaws of children with review of literature.

Central Primary Fibrosarcoma Maxilla - A Rare Presentation

“…In some cases it may present as cystic lesion or with well circumscribed margins as documented by a few authors and then the differential of infected dental cyst and central desmoplastic fibroma arise. Ameloblastic fibrosarcoma, Ewing's sarcoma, and radiolucent osteosarcoma can also show similar features [6,9,10]. On Computerized Tomography Scan (CT) it may present as destructive bony lesion, commonly associated with extra osseous soft tissue mass, as seen in this case [11].…”

“…It is more common in 5-6th decade, with equal sex predilection. However, an occasional case has been reported in pediatric age group too [6]. Patients are mostly asymptomatic.…”

“…Patients mostly present with complain of swelling in jaw with or without pain. Other complaints are loosening of teeth, trismus, fracture and paresthesia [3,5,6].…”

“…Secondary FS may arise in pre-existing fibrous dysplasia, paget's disease, bone infarct, cyst andosteomyelitis or may present as malignant transformation of pre-existing giant cell tumors of bone or transformation of ameloblastic fibroma [6,7]. It has also been documented to be induced by prior history of irradiation [4].…”

Intraoral Soft Tissue Fibrosarcoma: A Case Report and Review

“…Different authors have mentioned a cutoff age of 2 years and upto 5 years. 25 The histological appearance of fibrosarcoma does not allow a distinction between a tumor of the bone from one arising in soft tissue. 26 Histologically, the degree of differen tiation is variable, from being comparable to a benign fibroma to a highly anaplastic tumor, thus presenting a diagnostic dilemma to histopathologists.…”