Fibrosarcoma in infants and children.A review of 110 cases

Abstract

This retrospective study summarizes the clinicopathologic findings in 110 children with fibrosarcoma. Seventy cases were selected from previous reports and 40 were from our files. The histologic criteria used for diagnosis are the same as those used for adults. Sixty-eight patients were in the first quinquennium of life, 13 in the second, and 29 in the third. Eleven patients died of their tumor, three in the first quinquennium and eight in ght third. Our findings indicate that children who are less than 5 years old have a 7.3% chance of developing metastatic spread even though the local recurrence rate is 43%. Children who are 10 years old or older had a metastatic rate of 50% at 5-year follow-up, a figure closely approximating that for adults. We believe that infants and children less than 5 years old may be treated initially by local excision of the lesion, without sacrificing significant function of the part. Recurrent tumor does not seem to predispose to metastatic disease.

Chest Wall Infantile Fibrosarcomas- A Rare Presentation

“…IF, arising in the head and neck, is commonly seen in older children. Other rare sites of origin include the mesenteries, retroperitoneum, mouth, presacral region, intestines, and lungs [3][4][5][6][7][8][9][10]. Its principal manifestation is a painless swelling with steady, sometimes rapid growth.…”

“…Metastasis is rare and occur mostly in the lungs, bones, and sometimes in lymph nodes; however, local recurrence is rather high and occurs in 20 % to 40 % of cases [4][5][6][7]. These patients should be followed up closely and monitored for local recurrence, which can occur as early as 6 weeks postoperatively and as late as 10 years after the initial excision.…”

“…Most of these tumors are discovered at birth (30 %) or in the first year of life, and presents a diagnostic and therapeutic challenge. It often arises in the arms or legs but may also arise in the head, neck, trunk, and rarely at other sites [3][4][5][6][7][8][9][10]. This report describes an unusual case of IF of the chest wall chest in a newborn with brief review of the relevant literature.…”

Orthopaedic Case of the Month: Rapidly Progressive Shoulder Soft Tissue Mass in an 8-week-old Girl

“…3 of cases present at birth [8,9,18,36]. Extremities are the most common location, whereas truncal lesions carry a poorer prognosis [4,32].…”

“…Although histologically identical to the adult-type fibrosarcoma, there are distinct cytogenetic and clinical characteristics [20]. Moreover, unlike its adult counterpart, congenital fibrosarcoma metastasizes in less than 10% of patients and carries a good prognosis, despite its ominous histologic appearance and locally aggressive behavior [3,4,14,36]. Five-and 10-year survival rates are approximately 80% to 90% [6,27,30,32], and amputation rates appear to be declining [3,8,30] MRI findings are generally nonspecific, and show a well-circumscribed, heterogeneous mass, with vascular elements, areas of necrosis, and hemorrhage.…”