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PubMed1984

Paraaortic lymphadenectomy is not necessary in the treatment of localized paratesticular rhabdomyosarcoma.

Paraaortic lymphadenectomy is routinely recommended to treat paratesticular rhabdomyosarcoma (RMS) because of the high incidence of lymph node involvement. Taking into account the effectiveness of chemotherapy to sterilize micrometastases and aiming to reduce short-term and long-term side effects due to lymph node dissection, the RMS Group of the SIOP decided to avoid lymphadenectomy in Stage I paratesticular RMS defined by (1) complete tumor removal with negative cord section and (2) negative pedal lymphangiography. A series of 19 children (mean age 5 years, 6 months), treated between 1971 and 1981, were analyzed. Eighteen of 19 patients received adjuvant chemotherapy, using vincristine, actinomycin, cyclophosphamide, singly or alternately with vincristine, Adriamycin (doxorubicin) every 3 weeks. Duration was either 18 months (7 cases) or 8 months (11 cases). Fourteen patients are in first remission with a follow-up of more than 3 years (median 5 years); three are disease-free 35, 23, and 20 months since diagnosis; two patients are alive without evolutive disease, 32 and 56 months after an abdominal relapse. Results obtained in this series demonstrate the ineffectiveness of systematic paraaortic lymphadenectomy in Stage I paratesticular RMS and the effectiveness of chemotherapy to eradicate occult micrometastases, which obviously may exist in paraaortic lymph nodes in spite of apparently normal lymphographic findings.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1984

Hypercalcemia preferentially occurs in unusual forms of childhood non-Hodgkin's lymphoma, rhabdomyosarcoma, and Wilms' tumor. A study of 11 cases.

Unusual clinical, radiologic, or histologic findings were found in 11 of 17 cases of hypercalcemia associated with childhood tumors. Four children had undifferentiated lymphoblastic lymphoma with extensive bone involvement, but no visceral or neurologic involvement. At diagnosis, four adolescents with rhabdomyosarcoma had numerous metastases, particularly in the breasts and bone marrow. Three infants had renal tumor without bone metastases. Histologically, their tumors differed from classical nephroblastoma and resembled the malignant rhabdoid tumors of the kidney. These findings allow individualization of three distinct groups of tumors with unusual features which may suggest the presence of hypercalcemia. These tumors appear to have a poor prognosis since all patients but one died of their malignancy.

Adolescent↗
PubMed1985

Alveolar rhabdomyosarcoma of the female genitalia.

Eight cases of alveolar rhabdomyosarcoma of the female genitalia were diagnosed from 1963 to 1983 at The University of Texas M. D. Anderson Hospital. The primary sites were vulva in two, perineum in five, and broad ligament in one patient. When possible, therapy was initiated with local tumor excision (five patients). Surgery was followed by local or regional radiation (six patients) and chemotherapy (seven patients). Of the eight patients, five died within 9 months, one died 27 months after diagnosis, and only two are 5-year survivors. The aggressive behavior of this tumor is evidenced by autopsy findings of widespread metastases. Metastatic disease to the bone was present in four patients and to the breast in three patients. Local disease was controlled in two patients who died of distant metastases. Current therapy recommendations include excisional surgery, local radiation, and combination chemotherapy. A need for more effective chemotherapeutic programs is evident.

Adolescent↗
PubMed1986

Rhabdomyosarcoma of pterygoid fossa. Resection for cure utilizing an innervated facial flap and craniofacial reconstruction.

Tumors of the pterygoid fossa are often regarded as unresectable because of their anatomic inaccessibility. The rapidly developing techniques of craniofacial surgery have advanced sufficiently to now allow safe ablative surgery in this area and yet preserve the functional status and cosmetic appearance of the patient. A technique utilizing a bicoronal incision that is extended to the angle of the mandible on the involved side is described. This allows wide exposure of the bony structures at the lateral base of the skull while maintaining the integrity of the facial nerve within the cutaneous flap. Temporary removal of the zygomatic arch achieves direct access to and visualization of the contents of the temporal and pterygoid fossae. Skull, mandibular, and maxillary bone adjacent to tumor can easily and safely be resected to obtain complete tumor-free margins. Craniectomy bone is harvested and split into inner and outer tables to reconstruct the bony defects. This approach was successfully utilized in a 5-year-old boy with a Group III rhabdomyosarcoma with residual tumor following combined chemo- and radiotherapy. He remains tumor-free at 15 months, postoperation. The technique can be adapted for a variety of mass lesions located at the anterior base of the skull, both intra- and extracranially. Morbidity and mortality should be minimal with an experienced craniofacial team.

Child, Preschool↗
PubMed1987

Primary pulmonary rhabdomyosarcoma of the lung in children. Report of two cases presenting with spontaneous pneumothorax.

This report describes two cases of pulmonary rhabdomyosarcoma occurring in children, ages 21 and 30 months at diagnosis. Both patients presented with spontaneous pneumothorax and had cystic changes in the affected lung by chest x-ray. Ultrastructural and immunohistochemical studies supported the myogenic phenotype of these neoplasms. The authors were unable to confirm that either of these tumors had unequivocally originated in a congenital cyst although previous authors have reported this association. An apparent morphologic spectrum of embryonic appearing neoplasms including pulmonary blastoma and a malignant mesenchymoma have been observed in the lungs of children.

Child, Preschool↗
PubMed1987

Laryngeal rhabdomyosarcoma. Case report and literature review.

A case report is presented, believed to be the first documented example of pleomorphic rhabdomyosarcoma developing in the larynx of a child. A review of the literature then follows, with evaluation of the epidemiology, treatment options, and survival of patients with this disease. Cure of this malignancy is more likely with early diagnosis and aggressive treatment.

Child, Preschool↗
PubMed1987

Rhabdomyosarcoma of the uterine cervix. Sarcoma botryoides.

Twenty-one cases of sarcoma botryoides of the uterine cervix, including four previously unreported cases, are reviewed. The age of the patients ranged from 5 months to 48 years, with a peak incidence in the group aged 14 to 18 years. Eighty percent of the patients are alive, with a mean follow-up period of 68 months. Seventy-five percent of the patients had Group I disease, of whom 88% are alive. Eleven of 14 patients (79%) receiving vincristine and dactinomycin based chemotherapy are alive. There were five patients with recurrent disease (24%) of whom two (40%) are alive. The prognosis for cervical sarcoma botryoides is similar to that of other female genital tract embryonal rhabdomyosarcomas. Primary therapy should consist of vincristine and dactinomycin based chemotherapy. Surgery should be guided by the response to initial chemotherapy and should attempt to conserve the function of the bladder, rectum, vagina, and ovaries.

Adolescent↗
PubMed1988

Primary rhabdomyosarcoma of the kidney. A light microscopic, immunohistochemical, and electron microscopic study.

Primary rhabdomyosarcoma of the kidney is a rare and highly aggressive tumor in the adult population. A case is reported in a 70-year-old woman with the diagnosis confirmed by immunohistochemistry and electron microscopy. This is the first case studied using the immunoperoxidase technique and the second with electron microscopic examination. To make a diagnosis of primary sarcoma, of the kidney, three criteria must be met: (1) a metastatic sarcoma must be ruled out; (2) the tumor must arise from renal parenchyma; and (3) a sarcomatoid variant of renal cell carcinoma needs to be excluded. The literature is reviewed and available clinical and pathologic details are summarized.

Aged↗
PubMed1988

Rhabdomyosarcoma in patients older than 40 years of age.

The original diagnosis of rhabdomyosarcoma (RM) was critically evaluated by histology, immunohistochemistry, and electron microscopy in a retrospective series of 25 patients older than 40 years of age. Only two of the 25 patients (8%) were verified to have RM by strict criteria. By light microscopy, the true RM had a spindle or round cell appearance and were subtyped as embryonal RM, although some pleomorphism was present. Sarcomeric differentiation was revealed by electron microscopy, and desmin and muscle actin by immunohistochemistry. Both of these tumors arose in the urogenital organs, one in the urinary bladder and the other in the prostatic region. Both patients died within 3 months of the diagnosis. The other tumors not verified as RM were pleomorphic or spindle cell sarcomas (n = 17), ten of which were considered to represent malignant fibrous histiocytoma, or had desmin and/or muscle actin, and were verified as leiomyosarcomas by electron microscopy (n = 2). There were single cases of undifferentiated carcinoma, probable neuroendocrine carcinoma, extraskeletal osteosarcoma, and pleomorphic liposarcoma. The average survival for the non-RM tumor patients was 32 months. The results show that true RM do occur in the elderly, but they are very rare. Such tumors may have clinicopathologic properties similar to embryonal RM in children both in regard to the site of origin and to the histologic picture. True RM seems to carry a worse prognosis than other pleomorphic sarcomas, and this emphasizes the need for accurate diagnosis.

Adult↗
PubMed1989

Characterization of pyruvate kinase from human rhabdomyosarcoma in relation to immunohistochemical and morphological criteria.

We have compared the pyruvate kinase (PK) isoenzyme pattern of three rhabdomyosarcomas with foetal skeletal muscle tissue of 19 and 23 weeks of gestation, together with adult muscle in relation to immunohistochemical and morphological criteria. In foetal tissue of 19 weeks of gestation a focal immunopositivity for desmin and myoglobin was observed, whereas in tissue of 23 weeks an overall positivity for these proteins was present. Two of the three neoplasms were poorly differentiated and of the alveolar subtype. They were desmin immunoreactive. Some large spindle-shaped cells expressed myoglobin. The third one was more differentiated in microscopic characteristics and all cells showed immunoreactivity for desmin and myoglobin. In the foetal tissues five forms of pyruvate kinase isoenzymes were present with K2M2 as the predominant form. In adult muscle tissue only M4 was present. The tumors were characterized by a profound shift to the K-type, whereas the M4-type was not expressed at all. A difference in isoenzyme composition of pyruvate kinase was found between the morphologically less differentiated tumors and the more differentiated tumor; in the latter more M-subunits were expressed.

Adolescent↗
PubMed1989

Prognosis in childhood rhabdomyosarcoma of the extremity.

From 1970 to 1987, 34 patients younger than 22 years of age with extremity rhabdomyosarcoma were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC). All patients were treated according to protocols consisting of surgery, radiotherapy, and multiple drug chemotherapy. Sixteen of 34 patients survived, and 14 continue to be disease-free; the 5-year survival rate was 44%. A retrospective univariate analysis of data according to the TNM staging system was undertaken. Tumor invasion, regional nodal involvement, distant metastases, and alveolar histologic condition each had a significant impact on survival. However, in multivariate analysis, the stage of disease at diagnosis was the most important predictor of survival outcome.

Adolescent↗
PubMed1990

A solitary testicular relapse of a rhabdomyosarcoma in an adult.

A case of recurrent rhabdomyosarcoma in an adult is reported. The primary disease at presentation was in the maxillary sinus with metastatic spread to regional lymph nodes. A complete response (CR) and a 14-month disease-free interval (DFI) were obtained with combination therapy. The sole site of relapse was an intratesticular mass. Management implications are discussed.

Adult↗
PubMed1990

Primary chemotherapy with or without radiation therapy and/or surgery for children with localized sarcoma of the bladder, prostate, vagina, uterus, and cervix. A comparison of the results in Intergroup Rhabdomyosarcoma Studies I and II.

A major objective of the second Intergroup Rhabdomyosarcoma Study (IRS-II) (1978 to 1984) was to preserve the bladder without compromising the survival of children with localized genitourinary sarcomas arising in or near the bladder. After incisional biopsy, 109 patients with localized, gross residual sarcoma of the prostate (43 patients), bladder (43 patients), vagina (20 patients), or cervix/uterine corpus (3 patients) were treated with vincristine, dactinomycin, and cyclophosphamide (VAC). After two to four drug courses, radiation therapy and/or surgery were used to treat patients with residual or recurrent tumor. The 3-year survival rate of patients treated on this primary chemotherapy regimen (70%) was similar to that of the primary surgery regimens of IRS-I (78%; P = 0.46), but the 3-year disease-free survival rate was significantly inferior (52% versus 70%; P = 0.02). Since the IRS-II encouraged bladder preservation at the onset of therapy, the percentage of patients with bladder and prostate tumors who retained the bladder was initially substantially higher in IRS-II (97%) than in IRS-I (58%). However, the percentages of 95 patients with bladder-prostate (BP) tumors in IRS-II who retained the bladder and were alive at 2 and 3 years after starting treatment were only 33% and 22%, respectively, compared with 26% and 23%, respectively, in the 66 patients with BP tumors in IRS-I. Thus, sequential treatment with primary chemotherapy, followed by radiation therapy and/or surgery as given in IRS-II, failed to improve the eventual bladder salvage rate.

Adolescent↗
PubMed1991

Magnetic resonance imaging of cardiac rhabdomyosarcoma. Quantifying the response to chemotherapy.

This report illustrates the use of cardiac magnetic resonance imaging (MRI) to quantify the initial extent of a cardiac rhabdomyosarcoma and, more importantly, its response to chemotherapy. Image slices spanning the heart and adjacent structures were analyzed using Simpson's rule applied to the image slices to estimate the tumor volume initially, then after 5 weeks, and again after 4 months of chemotherapy. A substantial, progressive reduction in tumor volume during chemotherapy was shown. After chemotherapy was discontinued, an increase in tumor volume was shown. It is suggested that, in addition to being useful in patient care, the technique may be useful in clinical investigations by providing an objective, quantitative measure of tumor response to therapy.

Adult↗
PubMed1991

Prognostic factors of alveolar rhabdomyosarcoma in childhood. An International Society of Pediatric Oncology study.

Pretreatment characteristics of 57 patients with alveolar rhabdomyosarcoma (RMS) registered by the International Society of Pediatric Oncology (SIOP) between January 1975 and December 1983 were identified to perform a prognostic factor study. Living status and disease-free survival (DFS) time were assessed for all patients on January 1, 1988. By multi-variate analyses, lymph node involvement (P = 0.0003) and tumor invasiveness (P = 0.007) were identified as the most significant covariates correlated with survival. A model including N stage (P = 0.001) and age (P = 0.03) was selected for predicting DFS rates. Children between 5 and 10 years of age had better DFS rates than those younger than 5 years of age or those older than 10 years of age. The authors suggest a prognostic classification that could allow treatment to be adjusted according to clinical staging.

Adolescent↗
PubMed1992

Prostatic embryonal rhabdomyosarcoma in adults. A clinicopathologic review.

Embryonal rhabdomyosarcoma of the prostate is a rare, highly malignant tumor that occurs predominantly in male infants and children, in whom it is the most common prostatic sarcoma. Six cases occurring in adults have been published, and the authors report three additional cases. The natural history is characterized by rapid growth, with the typical formation of large pelvic or abdominal masses, often leading renal failure due to bilateral ureteric obstruction. The tumor eventually disseminates widely, mainly to the lungs, bone, liver, and serosal surfaces, and unlike most other sarcomas, regional lymph node metastases are common. Combined modality therapy has resulted in marked improvement in survival rates and reduced surgical morbidity for children with these tumors. However, in adults the prognosis remains poor, with all patients dying of disseminated disease within 16 months of histologic diagnosis (mean survival, 8 months).

Adult↗
PubMed1993

Parvovirus B19 as a cause of chronic anemia in rhabdomyosarcoma.

BACKGROUND: Several reports have been published of anemia due to infection by parvovirus in patients receiving chemotherapy for leukemia. However, parvovirus B19 has not been previously identified as a cause of chronic anemia in children with solid tumors. METHODS: A child with rhabdomyosarcoma developed chronic anemia, with need for regular blood transfusions during chemotherapy. An infectious etiology was sought. RESULTS: B19 viremia and an absent antibody response was present during the period of chronic anemia during chemotherapy. After completion of chemotherapy, the viremia cleared, IgM and IgG antibody to B19 virus were detected, and the anemia resolved. CONCLUSIONS: Parvovirus must be considered as a cause of anemia in patients undergoing chemotherapy for solid tumors as well as leukemia.

Anemia, Aplastic↗
PubMed1993

Aggressive chemotherapy, organ-preserving surgery, and high-dose-rate remote brachytherapy in the treatment of rhabdomyosarcoma in infants and young children.

BACKGROUND: External beam radiation therapy often is avoided in the treatment of rhabdomyosarcoma (RMS) in young children because of the long-term sequelae. Conventional brachytherapy can reduce these problems, but its use is limited in young children because of radiation exposure to parents and care-givers. This is the first reported use of high-dose-rate remote brachytherapy (HDR) to treat RMS in young children. METHODS: Seven young children with RMS were treated from January 1990 through September 1991 with multiagent chemotherapy, organ preserving surgery, and HDR. The primary tumor sites included the tongue, buccal mucosa, chest wall, vagina, and clitoris. A minimum peripheral dose of 36 Gy HDR was administered in 12 fractions (twice a day) at 3 Gy per fraction for a period of 3 days. The treatment was given on an outpatient basis without requiring prolonged patient sedation or immobilization. Each treatment lasted 2-5 minutes. RESULTS: All seven children are alive and without evidence of tumor with a median follow-up of 30 months (range, 18-35 months) from diagnosis. The treatments have been reasonably well tolerated with some acute skin toxicity. There has been relatively good organ growth and function during this short follow-up period. CONCLUSION: The use of HDR radiation in these patients eliminated radiation exposure to care-givers and permitted constant nursing care and interaction among the parents, nursing personnel, and child. Treatments can be given on an outpatient basis, without requiring prolonged patient sedation or immobilization. Local control of tumor with preservation of organ function was achieved. HDR in young children should be restricted to controlled clinical trials until long-term morbidity and efficacy results are obtained from pilot studies.

Antineoplastic Combined Chemotherapy Protocols↗