Soft‐tissue sarcomas other than rhabdomyosarcoma in children

Abstract

Forty-seven children with nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) were treated by the Hematology-Oncology Service at Texas Children's Hospital, Houston, Texas, between 1958 and 1990. The male:female ratio was 1:1, and the median age was 11 years (3 weeks-16 years). A preexisting condition was found in 9/47 (19%) patients including neurofibromatosis (3), Down's syndrome (1), spina bifida (1), congenital facial asymmetry (1), giant pigmented nevus (1), juvenile onset diabetes mellitus (1), and acquired immune deficiency syndrome (1). The site of primary tumor was head and neck (3), trunk (33), and extremities (11). Twenty-four patients (51%) have survived free of disease with a median follow-up of 5 years (4 months-22 years). No patient whose disease recurred achieved a second remission. Of the 19 patients with group I disease, 16 (84%) survived free of disease. Wide excision of the primary tumor, with no microscopic residual disease, was associated with the greatest chance of disease-free survival.

Adjuvant Radiotherapy for Pediatric and Young Adult Nonrhabdomyosarcoma Soft-Tissue Sarcoma

“…Our study had a slightly greater 5-year LC rate of 93% and 77% for patients treated with RT with negative and positive margins, respectively. Our series has confirmed the results of others that margin status affects LC (3,8,(11)(12)(13)(14). Patients with wide margins have had notably superior LC compared with those with close margins (95% vs. 83%).…”

Pediatric soft tissue sarcomas

“…70 Consistent with other published reports, slightly more than half of the tumors (82 of 154) were invasive, and most of these were greater than 5 cm in maximum diameter [71][72][73][74] In the only prospective series of pediatric NRSTS, 75 patients were seen at Pediatric Oncology Group institutions. 75 In this report, 63 percent of patients had either Stage III or Stage IV disease at diagnosis.…”

“…[71][72][73][74] High-grade tumors are frequently associated with other poor prognostic features-such as large size (>5 cm) and local invasiveness-that make complete surgical resection more difficult. Although some data suggest that high-grade tumors have a worse prognosis, it is unclear to what degree grade is prognostically significant independent of surgical resectability.…”

A Case of Congenital Infantile Fibrosarcoma of Sigmoid Colon Manifesting as Pneumoperitoneum in a Newborn

“…Congenital infantile fibrosarcoma (CIF) is a very rare type of nonrhabdomyosarcoma soft-tissue sarcomas, usually occurring in the 1st year of life, with approximately 40% of lesions present at birth and more than 80% diagnosed within 1 yr (6). It accounts for 5%-10% of all soft-tissue sarcomas in infants younger than 1 yr and is slightly more common in boys (7, 8). …”