Patients and methods dental abnormalities in survivors of acute lymphoblastic leukemia (ALL). We reviewed the clinical records and panoramicWe reviewed the medical records and panoramic dental radiographs of 423 survivors of ALL who were treated on one of radiographs of all children treated for ALL at our institution children who are nearing dental maturity are less likely to (n ‫؍‬ 80), hypodontia in 8.5% (n ‫؍‬ 36), taurodontia in 5.9% (n ‫؍‬ 25), and over-retention of primary dentition in 4.0% develop dental sequelae, we included only those children (n ‫؍‬ 17). Patients who were р8 years old at diagnosis or who who were diagnosed on or before their 13th birthday and received cranial irradiation therapy developed more dental excluded those whose radiographs documented complete perabnormalities than did those Ͼ8 years and those who did not manent dentition or whose therapy did not conform to stan-

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We have identified a distinctive malignant soft tissue neoplasm that occurred in the head and neck region of six children. Histologically, these neoplasms presented an array of features ranging from low-grade spindle cell to high-grade fibrohistiocytic histologies and often had myoid characteristics. Ultrastructural and immunohistochemical studies indicated that they contained neoplastic myofibroblasts that were variably positive for vimentin (4 positive/4 tested), alpha-smooth muscle actin (4/5), muscle-specific actin (5/5), desmin (2/5), and v-src protein substrate p80/85 (4/5). Three patients died of rapidly progressive unresectable local disease, one died of metastatic and local disease, and two are alive 13 months and 8 years after wide resection. We conclude that these neoplasms form a distinctive subset of pediatric soft tissue sarcomas that display an aggressive clinical behavior, typically with local recurrence, and exhibit features of myofibroblastic differentiation.

To define the long-term dental sequelae of therapy for childhood rhabdomyosarcoma of the head and neck, and to identify factors in their development, we retrospectively reviewed the serial panoramic radiographs and clinical records of 22 survivors of head or neck rhabdomyosarcoma who had been followed for at least 5 years. Patients were divided into four groups based upon age at the time of therapy and three groups based upon radiation doses. All patients had received similar multiagent chemotherapy. Dental sequelae of oncotherapy occurred in over half of the long-term survivors of head and neck rhabdomyosarcoma. The abnormalities comprised root stunting in 54%, microdontia in 23%, and hypodontia in 50% of patients; 36% had multiple abnormalities. Microdontia and multiple abnormalities were more prevalent in patients treated at the earliest age, and abnormalities tended to be more prevalent with increasing doses of radiation. Five patients (23%) developed severe cosmetic and/or functional sequelae necessitating surgical and/or orthodontic intervention. The high frequency of dental sequelae we observed suggests that meticulous long-term dental and radiographic follow-up are needed. Early detection and treatment of the complications of therapy will expedite their correction and minimize morbidity.

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