Pediatric nonrhabdomyosarcomas of the extremities. Influence of size, invasiveness, and grade on outcome
B. N. Rao, V. M. Santana, D. Parham, C. B. Pratt, I. D. Fleming, M. Hudson, J. Fontanesi, P. Philippe and M. J. Schell
Department of Surgery, St Jude Children's Research Hospital, Memphis, Tenn. 38101.
Surgery alone or supplemental radiation is effective in local control in a
majority of patients with nonrhabdomyosarcoma of the soft tissue. To
determine the factors influencing subsequent survival, a retrospective
review of 64 children with nonrhabdomyosarcoma of the soft tissue of the
extremities was performed. Using the American Joint Committee on Cancer
staging system, 32 patients had noninvasive T1 lesions, and 31 of these
patients survived disease free. Twenty-two patients had lesions that were
smaller than 5 cm; 19 of these lesions were grade G1-2. All 22 patients
survived. Ten patients had tumors that were 5 cm or larger, and all were
G1-2; nine of these patients survived. Thirty-two patients had invasive
(T2) lesions; seven of these lesions were smaller than 5 cm, but four were
G3. Only one patient survived. Eighteen (72%) of 25 lesions that were 5 cm
or larger were G3. Fifteen (83%) of these 18 patients underwent relapse,
with 12 patients dying of the disease. Important prognostic factors
included G3 lesions, with a 72% (18 of 25 patients) failure rate, and T2
lesions, which were generally larger than 5 cm, of higher grade, and
underwent relapse more frequently than T1 lesions. These patients with
histologic G3 and T2 lesions may benefit from chemotherapy.