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The intermediate filament desmin was present in three canine botryoid rhabdomyosarcomas. The use of desmin as a diagnostic tool may, as in these tumours in man, be of value in the classification of canine sarcomas where the origin of the tumour is not always apparent from routine histological sections.
In an adult male rhesus monkey, a large pelvic mass causing lysis of the ilium and destruction of pelvic musculature was diagnosed as a spontaneous rhabdomyosarcoma by the use of histological, ultrastructural and immunohistochemical techniques.
Rhabdomyosarcoma is a rare malignant neoplasm of the biliary tract. Including all the historical recent probable, and established cases that are carefully classified by Hayes and Synder, only 24 cases have been reported to date. Because of the extreme rarity of this tumor in this location, correct diagnosis was not made until second laparotomy or autopsy in several reported cases. The present report describes a child who recently underwent excision of such a tumor at our institution.
In order to examine surgical factors predictive of fatal outcome in patients presenting with histologically verified rhabdomyosarcoma of the urinary bladder, we performed a retrospective analysis of cases presenting between the years 1970 and 1985 and treated by protocol. Twenty-five patients were identified and data were complete for univariate and multivariate analysis on all. Staging was done according to the criteria of the International Union Against Cancer (TNM). Median age at presentation was 14.7 years and 10 patients were younger than 10 years. Median follow-up was 4.8 years overall and 8.4 years in survivors. Four patients presented with involvement of regional lymph nodes and three with distant metastases. Complete surgical resection, defined as negative microscopic margins, was accomplished by total cystectomy in 14 patients, and partial cystectomy in two. In this group cystectomy was performed prior to chemotherapy and radiation in five and after in 10 (persistent disease). Three salvage cystectomies were performed in patients who recurred after initial complete responses to chemotherapy and radiation therapy. Thirteen patients received a median of 3,000 cGy (range, 1,800 to 5,000 cGy) of external beam pelvic irradiation, and two received brachytherapy. All patients received multiple agent chemotherapy according to either the T2 or T6 protocol. There are 11 disease-free survivors (44%) and 10 of these have been followed for more than 6 years. One patient is alive with disease 6.5 years after diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)
In order to examine factors predictive of fatal outcome in children presenting with histologically confirmed extremity rhabdomyosarcoma, we performed a retrospective analysis of our institutional experience from 1970 to 1985. Thirty-five patients were identified and staged according to international criteria (TNM). Variables evaluated for their predictive effect on fatal outcome included (1) tumor invasiveness, (2) tumor size, (3) anatomic location of the primary, (4) regional lymph node involvement, (5) distant metastases at presentation, (6) complete surgical resection, (7) use of amputation, and (8) alveolar histologic subtype. Significant predictors of mortality included (1) tumor invasiveness (P less than or equal to .0001), (2) regional node involvement (P less than or equal to .0002), (3) distant metastases at the time of presentation (P less than or equal to .001), (4) alveolar histology (P less than or equal to .001), (5) size of primary (P less than or equal to .007), and (6) completeness of surgical resection (P less than or equal to .05). In multivariate analysis, local tumor invasiveness was the most important predictor of fatal outcome with an associated relative risk of 18. We conclude that local tumor invasiveness is the most important determinant of clinical stage.
Between 1968 and 1982, 10 children (7 boys and 3 girls) with a mean age of 3.2 years affected by bladder (8 cases) and prostate (2 cases) rhabdomyosarcoma (RMS)--all histologically embryonal--were seen. Three (1 with bone metastases at diagnosis and 2 with stage III tumor) underwent radical surgery; all died because of progression of the disease. Seven children were treated with conservative surgery: 3 solid type bladder tumors were removed together with a narrow strip of apparently healthy bladder wall, sparing 30% to 70% of the bladder; 3 botryoid type bladder tumors were removed bluntly and implantation sites fulgurated; in 1 prostate tumor osmotic cytolysis of the neoplastic mass was achieved with multiple injections of hypertonic saline solution (NaCl 30%). Surgery was followed by chemotherapy with a vincristine actinomycin D and cyclophosphamide protocol and radiotherapy (20 to 39.5 Gy). Six of the seven are alive and disease free with a normal functional bladder after 10 to 21 years. The validity of a primary conservative surgical approach with preservation of the bladder, followed by adjuvant therapy, is emphasized.
A case of embryonal rhabdomyosarcoma of the vagina associated with pregnancy is described. This patient delivered a healthy baby by elective classical Caesarean section; following this she was treated by radiotherapy and chemotherapy. The patient is well after 3 years of treatment.
There have been only a few published reports on the dental-facial effects of radiation therapy and the subsequent oral management of these patients. A case involving a 13-year-old black male patient with a history of rhabdomyosarcoma is presented. The patient received 4,050 rads of radiation to the right middle ear when he was 2 years of age. His residual medical and dental difficulties are apparently complications from the initial therapy. Examination of the oral cavity revealed bimaxillary micrognathia and marked loss of vertical dimension. A Class II facial profile with Class I molar relationship was observed. The mandible was thin and hypoplastic, with a small knife-edge alveolar ridge. The remaining eighteen erupted permanent teeth were very mobile, and root development had ceased after only initial formation. All of the teeth except the first permanent molars were extracted, and immediate partial dentures were inserted at the time of surgery. A discussion of therapeutic considerations follows the case report.
Between 1944 and 1984 16 patients with rhabdomyosarcoma of the oral soft tissues were treated at the M.D. Anderson Hospital and Tumor Institute. Before 1968 treatment consisted only of surgery. Chemotherapy and radiotherapy were used when surgery was impossible or in an effort to treat recurrent disease. After 1968 treatment design was multidisciplinary and included (excision) biopsy, followed by a combination of high-dose radiotherapy and multidrug chemotherapy. Of the six patients treated before 1968, five (83%) died of disease. In eight of the ten patients treated after 1968, follow-up was 4 years or more. Of these eight patients, seven (87.5%) are free of disease. The clinicopathologic aspects, treatment, complications of treatment, and survival figures are discussed.
To define a useful and prognostically relevant classification system for rhabdomyosarcomas (RMSs), tissue sections of 113 well-documented, protocol-treated cases were retrieved from the files of the Emma Kinderziekenhuis Amsterdam, the Netherlands, and reclassified by a panel of pediatric pathologists. The following subtypes were recognized: embryonal RMS (n = 66), alveolar RMS (including the solid variant) (n = 16), botryoid RMS (n = 11), embryonal sarcoma (n = 6), and spindle cell RMS (n = 5). Nine cases were classified as RMS not otherwise specified (NOS). The above-mentioned histopathological subtypes correlated significantly with survival (P = .005) in patients with nonparameningeal tumors. Indeed, the best prognosis was observed in patients with spindle cell RMS, embryonal sarcoma, and botryoid RMS (10-year survival rates of 80% to 86%). Patients with embryonal RMS had an intermediate prognosis (10-year survival rate of 55%) and patients with alveolar RMS fared poorly (10-year survival rate of 9%). Survival rate was poor in patients with a localized parameningeal tumor, irrespective of histopathological subtype (10-year survival rate of 33%). Furthermore, this study confirmed the known impact on prognosis of localization (P = .008) and tumor node metastasis (TNM) stage (P = .0005). Classification of RMS subtypes proved to be fairly well reproducible (kappa ranging from 0.47 to 0.85 and percentage of concordance ranging from 50% to 85%). The best agreement was noted in botryoid RMS and the worst in embryonal sarcoma. However, improvement of agreement was noted for the latter subtype during the consecutive classification sessions. In summary, this study shows the strong prognostic value of histopathological subtypes and parameningeal tumor localization.
In the absence of neurofibromatosis, vestibular schwannomas (acoustic neuromas) are rarely found in children. We report a case of a 13-year-old boy who presented with unilateral vestibular schwannoma 11 years after resection of an orbital rhabdomyosarcoma. The coincidence of these tumors has not been previously reported.
Treatment of childhood embryonal rhabdomyosarcoma of the lower genitourinary tract is in the process of undergoing drastic changes. Extensive surgery with excision of the lesion and its contiguous structures leaving the patient with significant physical disability is being replaced by biopsy and aggressive combined chemotherapy along with radiation therapy. Although it would appear that survival may be improved by this approach, caution is recommended and extirpation advised should the tumor fail to respond rapidly to chemotherapy.
A case is reported of a twenty-two-month-old child who has survived for seven years without recurrence after multimodal therapy of a rhabdomyosarcoma of the prostate. The patient was treated with preoperative irradiation, radical surgery, and intraoperative and postoperative actinomycin D.
A case of embryonal rhabdomyosarcoma of the spermatic cord is presented and literature reviewed. The spread of this disease to the retroperitoneal lymph nodes and the improved results with retroperitoneal lymphadenectomy and radical orchiectomy are stressed.
This article discusses 2 patients with embryonal rhabdomyosarcoma of the spermatic cord and presents a survey of the literature and therapeutic methods. In recent years the prognosis of this malignant tumor has improved significantly by virtue of a combination of radical inguinal orchiectomy and retroperitoneal lymph node dissection with supplementary chemotherapy and/or radiotherapy.
A paratesticular rhabdomyosarcoma occurred in a child with factor IX deficiency and neurofibromatosis, illustrating the need to consider carefully the various etiologic possibilities of a soft-tissue mass in a child with neurofibromatosis and/or a bleeding disorder.
Between 1959 and 1983, 16 patients with pelvic rhabdomyosarcoma were treated at Roswell Park Memorial Institute (RPMI). A multidisciplinary approach was utilized consisting of surgery, radiotherapy (XRT), and combined chemotherapy which resulted in prolonged survivals of thirteen to one hundred forty-four months in 10 patients. This improved survival was observed despite the fact that 63 per cent of the patients (10/16) presented with advanced stages of the disease. Herein we present a retrospective study of these 16 patients.
Pure rhabdomyosarcomas are extremely rare and lethal primary neoplasms of the ovary. The available literature revealed 14 such cases. This report describes the light and electron microscopic findings of another case. At the electron microscopic level, rhabdomyogenesis recapitulates skeletal muscle differentiation during early fetal life. Rhabdomyoblasts in the ovary may arise from either the uncommitted stromal fibroblasts or from fibroblasts of endometriotic stroma. The age of patients at diagnosis ranged between 13 months and 86 years with 60% of women aged 40 years and older. About 50% of the cases were diagnosed with disease beyond the ovary, and survival ranged between 18 days and 15 months after diagnosis. Aggressive combination therapy including surgery, radiation, and chemotherapy appears to be the only hope for improved survival.