Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the intergroup rhabdomyosarcoma studies (IRS)-II and -III

Background. This review of children and adolescents with nonorbital soft-ti ssue sarcoma of the head and neck was undertaken to describe late sequelae of treatment, as manifested primarily by problems with statural growth, fac ial and nuchal symmetry, dentition, vision and hearing, and school performa nce. Procedure. Four hundred sixty-nine patients entered the IRS-II and -II I protocols with localized, nonorbital soft-tissue sarcomas of the head and neck from 1978 through 1987. Their overall survival rate was 53% (250/469) at 5 years. Two hundred thirteen patients were surviving relapse-free 5 or more years after diagnosis, for whom there were serial height measurements at 2 or more years after initiation of therapy. Their median age at diagno sis was 5 years; the median length of follow-up was 7 years. All received m ultiple-agent chemotherapy, and all but 3 received irradiation to the prima ry tumor volume. Sixty-eight percent of the tumors arose in cranial paramen ingeal sites, 22% in nonparameningeal sites, and 10% in the neck. We review ed flow sheets submitted to the IRS Group Statistical Office to ascertain w hich late sequelae were recorded. Results. One hundred sixty-four patients (77%) had one or more problems recorded. One hundred ninety of the two hund red thirteen patients (89%) were under 15 years of age at study entry, and at follow-up 92 (48%) had failed to maintain their initial height velocity, which had decreased by more than 25 percentile points from the original va lue. Thirty-six of the one hundred ninety patients (19%) were receiving gro wth hormone injections. Hypoplasia or asymmetry of tissues in the primary t umor site was reported in 74 patients, and 13 underwent reconstructive surg ery. Poor dentition or malformed teeth were noted in 61 patients. Impaired vision developed in 37 patients, owing primarily to cataracts, corneal chan ges, and optic atrophy. Thirty-six patients had decreased hearing acuity, a nd 9 were fitted with hearing aids; 5 of these 9 had received cisplatin. Th irty-five patients were noted to have problems learning in school. Four pat ients developed a second malignancy (two sarcomas, one carcinoma, one leuke mia). Conclusions. Late sequelae affected the majority of these patients tr eated for soft-tissue sarcoma of the head and neck on IRS-II and -III. The potential impact of certain sequelae could be reduced by specific measures, such as surgical reconstruction and hormonal therapy. Late sequelae must b e taken into account in designing future curative treatments. Med. Pediatr. Oncol. 33:362-371, 1999. (C) 1999 Wiley-Liss. Inc.