Chemotherapy alone for the treatment of congenitalfibrosarcoma: Is surgery always needed?

Primary colonic congenital infantile fibrosarcoma presenting as meconium peritonitis

“…In our case, we did not provide chemotherapy, however, when complete resection is not possible due to involvement of vital structures, post-operative chemotherapy, and occasionally radiation treatment is recommended [7]. There are a few reports indicating that large or unresectable lesions, respond well to up front, primary chemotherapy, followed by surgical resection [7,8]. The chemotherapy regimens that have been used consist of vincristine, adriamycin (or actinomycin-D), cyclophosphamide, and etoposide or ifosfamide.…”

“…If the pathologic margins are negative, no further therapy in the form of chemo or radiation is required. In our case, we did not provide chemotherapy, however, when complete resection is not possible due to involvement of vital structures, post-operative chemotherapy, and occasionally radiation treatment is recommended [7]. There are a few reports indicating that large or unresectable lesions, respond well to up front, primary chemotherapy, followed by surgical resection [7,8].…”

“…Pathologically, there may also be difficulty in distinguishing the lesion from infantile fibromatosis, which is benign [6]. There are reports of surgery or chemotherapy as sole treatment for CIF [7][8][9]. There are very few cases of CIF arising from the bowel [10].…”

Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations

“…Complete excision is the treatment of choice [175]. Cases treated with pre-operative chemotherapy [171] or with chemotherapy alone [176] have done well. Axial tumours seem to behave more aggressively [177], but there are no histological or genetic predictors of behaviour.…”

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

“…Chemoresponsiveness of congenital fibrosarcoma is unclear [6,30], however neoadjuvant chemotherapy may be used, in an attempt to decrease tumor size and reduce the surgical morbidity. Chemotherapy also has been used for unresectable lesions [3,16,17,22,27,37]. The most common regimen used combines vincristine, actinomycin D, and cyclophosphamide (VAC) [6,30]; its side effect is well established and is tolerable in infants [10,22,23].…”