Recurring digital fibrous tumor of childhood [proceedings].
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Biomedical subjects
Publications and source records attributed to R B Raney.
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The clinical characteristics, surgical treatment, clinical grouping, chemotherapy, radiotherapy, complications and survival of paratesticular rhabdomyosarcoma are discussed. When the combined approach of radical inguinal orchiectomy, retroperitoneal node dissection, chemotherapy and radiotherapy when indicated is used a survivorship in excess of 80 per cent can be anticipated for all stages of disease. A combined multidisciplinary approach to this lesion is stressed to achieve improved survivorship in this previously lethal lesion.
Twenty-seven previously untreated children with gross residual (20) or metastatic (seven) rhabdomyosarcoma were treated with pulse-VAC (vincristine weekly for 12 doses plus dactinomycin and cyclophosphamide simultaneously given daily for 5 days) and radiotherapy. Toxicity during the 12-week induction period included 23 of 27 (85%) with an absolute neutrophil count (ANC) under 500/mm3; 16/27 (59%) were given intravenous (I.V.) antibodies. Three patients developed Gram-negative sepsis and two of them died. In the first 12 weeks, eight children had a complete response (CR) and another 10 a good partial response (PR), a total of 18 of 27 favorable responses (67%). At 12 weeks, 20 patients received either intermittent pulse-VAC (Regimen H) or a pulse of adriamycin plus vincristine and cyclophosphamide alternating with pulse-VAC (Regimen I) every 4--6 weeks. After this first "maintenance," only seven patients (35%) developed an ANC under 500/mm3 and only three (15%) were given I.V. antibiotics. Severe toxicity disappeared with drug dose reduction in subsequent courses. The overall CR rate was 59% with a PR rate of 15%, a total of 74% favorable responses. This rate is not significantly better than that obtained by previous IRS chemotherapy and radiotherapy schedules for patients with gross residual and metastatic rhabdomyosarcoma. Future studies in these patients will concentrate on diminishing myelosuppression while shortening the rest period between pulses, in order to deliver more drug per unit time.
The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.
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Review of the clinical and roentgenographic findings in 16 children with craniofacial sarcomas has revealed that CT is the most accurate method for delineation of the sarcomas and is indispensable for proper therapy planning and post therapy evaluation. The patients were examined and treated according to a protocol which required a CT examination before any type of therapy and after each mode of therapy, chemotherapy and radiotherapy. Chemotherapy alone was noted to produce marked reduction in tumor volume. The orbital sarcomas, which are most frequent, are small tumors without bone destruction. On the other hand, the nasopharyngeal, paranasal sinus and middle ear sarcomas are large tumors frequently associated with extensive bone destruction.
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A congenital malignant peripheral nerve sheath tumor contained small rhabdomyosarcomatous portions in both the retroperitoneal pelvic primary and in the subpleural lung metastasis. There was no family history of Von Recklinghausen's disease. Of the 10 patients with this tumor previously reported, the youngest is 14 yr old, and eight had familial neurofibromatosis.
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Lymphoblasts from 23 children with acute lymphocytic leukemia (ALL) and 10 with lymphoblastic lymphoma (LBL) were studied by complement-dependent microcytoxicity tests with two nonhuman primate antisera defining leukemia-associated and lymphoma-associated antigens. Cells form 15 patients with ALL and 1 with LBL reacted only with antiserum to chronic lymphatic leukemia (CLL). These group-I patients were predominantly female. Most were pancytopenic and lacked mediastinal widening and T-cell markers; lymphoblasts from 15 were periodic acid-Schiff-positive. Cells from 8 male patients reacted only with antiserum to converted lymphosarcoma (LS). All these group-II patients expressed T-cell markers; 5 had mediastinal enlargement and 2, an abdominal mass. Six of the 8 were PAS-negative. Cells from 9 patients reacted with both antisera. The group-III patients demonstrated some characteristics of each of the above groups. Patients whose lymphoblasts reacted with CLL antiserum presented with clinical and laboratory features indicative of a good prognosis, i.e., ALL with PAS positivity and no T-cell markers or localized mass. Patients whose cells reacted with LS antiserum often had bad prognostic features: mediastinal or abdominal mass, expression of T-cell markers, and PAS negativity. These antisera appear able to differentiate childhood ALL from LBL. The distinction is important prognostically and perhaps therapeutically.
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