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PubMed1979

Recent experience in the treatment of rhabdomyosarcoma.

Twenty-two patients with rhabdomyosarcoma have presented in the years 1970--1977. Of 8 patients who had inadequate postoperative chemotherapy and radiotherapy, or none at all, only 2 patients with small completely resectable tumors are alive and well at 6 and 4 yr after surgery. Fourteen patients had intensive combination chemotherapy in addition to appropriate surgery and radiotherapy. Five have died and the remaining nine are well and free of disease at follow up ranging from 9 mo to over 5 yr. A continued place in the treatment regime is advocated for early surgery aimed at total excision of the primary tumor where this is feasible.

Adolescent↗
PubMed1982

Rhabdomyosarcoma of the biliary tree: the case for aggressive surgery.

Embryonal rhabdomyosarcoma of the bile ducts is associated with a poor prognosis because complete resection rarely is considered possible. We reviewed the literature and report our experience with the management of 3 consecutive patients. The patients were 1, 4, and 9 yr old at presentation, and had jaundice, cachexia, and an abdominal mass. The tumor arose in the common hepatic duct in 2 patients, and in the left hepatic duct in the other. All had tumor extension into both lobes of the liver and residual disease was noted after resection. Two patients were given tumor bed irradiation and all received multiple-drug chemotherapy. Two patients are alive, jaundice free, and with no evidence of disease at 9 mo and 14 yr follow-up. The other patient was a long-term survivor, but developed local recurrent tumor after cessation of chemotherapy and expired 33 mo after diagnosis. The traditional anatomic criteria of hepatic tumor resectability may not apply to botryoid sarcoma of the bile ducts. Aggressive surgery combined with the new adjuvant therapies appears to provide the best chance for a longer survival.

Antineoplastic Agents↗
PubMed1982

Primary chemotherapy in the treatment of children with bladder--prostate tumors in the Intergroup Rhabdomyosarcoma Study (IRS-II).

Twenty-nine children (24, male; 5, female) with non-disseminated rhabdomyosarcomas of the bladder or prostate were treated (1978-1980) by a primary chemotherapy regimen consisting of vincristine, actinomycin D, and cyclophosphamide ("Pulse" VAC), with or without local radiotherapy. During the initial 20 wk of chemotherapy, nine children achieved a Clinical Complete Response (CCR). Three of these are without evidence of disease (NED) and have functional bladders, two following partial cystectomy. Four who achieved a CCR subsequently relapsed or remained biopsy positive, but are at present NED following radiotherapy and anterior exenteration. Two patients who achieved CCR status relapsed and have died of disease. Twelve patients had a Clinical Partial Response (CPR) in less than 20 wk and two others in less than 40 wk. Seven of these are NED with intact bladders following chemotherapy-radiotherapy; and an additional patient is NED following partial cystectomy. Four patients in the CPR group have been treated by exenteration following failure to achieve complete response, and are NED. One patient has died, and one has progressive disease. Six patients had an inadequate response to chemotherapy (NR). Anterior exenteration was carried out in three, and two of these have survived. The overall results in these 29 patients are: (A) alive and disease-free with functional bladders, 11; (B) alive and disease-free following anterior exenteration, 10; and (C) dead or death from tumor anticipated, 8. The function of retained bladders (11) has been satisfactory.

Adolescent↗
PubMed1983

Rhabdomyosarcoma in childhood: analysis of survival in 98 cases.

Ninety-eight infants and children with rhabdomyosarcoma were analyzed for age, stage, site, and therapy as they relate to survival. Age and sex were not factors. Survival was 91% (10/11) for Stage I, 86% (13/15) for Stage II, 35% (12/34) for Stage III, and 5.2% (2/38) for Stage IV. Overall survival was 37% (37/98); however, 75% had advanced disease at diagnosis. Primary tumor site was genitourinary (GU) (31), extremity (17), head-neck (14), trunk (14), orbit (8), paratesticular (4), retroperitoneal (3), paraspinal (3), buttocks (3), and perianal (1). Survival was favorable in orbital, paratesticular, and (GU) sites. Survival was 20% (9/45) before and 52% (28/53) after chemotherapy and irradiation. The only survivors had embryonal cell histology. Tumor stage and site are important prognostic indicators. Chemotherapy improves survival in Stage I (91%) and Stage II (86%) and shrinks bulky Stage III tumors allowing less radical procedures in selected sites (e.g., GU). Survival is poor in Stage III (35%) and dismal in Stage IV (5.2%) despite combined therapy. Relapse was fatal despite attempts at second-look resection, and altered chemotherapy and irradiation.

Adolescent↗
PubMed1985

Sarcomas of the vagina and uterus: the Intergroup Rhabdomyosarcoma Study.

During a 12-year period (1972-1984), 43 patients with sarcomas of the female genital tract were admitted to the Intergroup Rhabdomyosarcoma Study (IRS), including 31 with primary tumors of the vagina; and 12 with tumors of the uterus, including the cervix. Thirty-four of these can be evaluated on the basis of periods of observation from 18 months to 12 years. Primary tumors of the uterus were a distinct group, distinguished from those arising in the vagina by patient age-range and probably by prognosis, as well as site. Patients with vaginal tumors, with a mean age of 1.8 years, responded to a multimodality approach employing combinations of chemotherapy (vincristine sulfate and actinomycin D, or the aforementioned two drugs with cyclophosphamide +/- doxorubicin hydrochloride), irradiation, and/or surgery, with only one tumor-related death, among 24 evaluable patients. In contrast, among the patients with primary uterine tumors, in which the mean age was greater than 14 years, four of ten evaluable patients died secondary to tumor relapse or progression.

Adolescent↗
PubMed1987

Rhabdomyosarcoma arising in a congenital giant nevus associated with neurocutaneous melanosis in a neonate.

The case of a newborn with a pedunculated embryonal rhabdomyosarcoma arising from a pigmented giant congenital nevus located on the posterior part of the neck and upper trunk is presented. The nevus was associated with melanosis of the leptomeninges and central nervous system. The pedunculated tumor was removed and the infant was given subsequent chemotherapy. In spite of well-tolerated chemotherapy, he died on the 30th day of life due to a staphylococcal pneumonia.

Central Nervous System Diseases↗
PubMed1989

Rhabdomyosarcoma of the prostate in childhood: current challenges.

Since 1978, six boys with prostatic rhabdomyosarcoma have been treated at our institution. Three had localized disease and were managed by initial biopsy, vincristine, actinomycin-D, and cyclophosphamide (VAC) chemotherapy, and bladder-sparing surgery with or without irradiation. Further combination chemotherapy ("pulse" VAC, Adriamycin, VP-16, cisplatin, and ifosfamide) was continued for 20 to 22 months following the induction course. Two boys had microscopic residual disease undetected by frozen section and unresponsive to radiotherapy. Subsequent total cystectomy 4 and 7 months later resulted in eradication of disease. In one patient, preservation of the bladder was achieved at the age of 3 months for 8 years. Artificial sphincter inserted to cure his urinary incontinence failed because of ischemia secondary to cuff compression and scar tissue. He is alive today with a modified Koch pouch urinary diversion. Of the 50% who had metastatic disease at presentation, two were dead within 12 months despite aggressive chemotherapy and irradiation. The third is currently on treatment. Although chemotherapy has markedly improved the prognosis, surgery is still necessary in most cases for cure. Bladder salvage is a desirable goal; however, residual microscopic disease, difficulty with frozen-section disease detection, and poor tissue vascularization for subsequent sphincter replacement remain significant obstacles.

Adolescent↗
PubMed1996

The argument for conservative, delayed surgery in the management of prostatic rhabdomyosarcoma.

Exenteration is no longer required for most patients who have rhabdomyosarcoma (RMS) of the prostate. This site comprised only about 5% of newly diagnosed cases in the IRS-III (1984-1991). The mean age at the time of diagnosis was 5.3 yrs (range, 0 to 19 years). Most tumors were relatively large, had embryonal histology, and were clinically localized but unresectable without major loss of organ function. The 44 patients with group III tumors (gross residual disease) were treated according to the IRS-III protocol. Forty-three of them underwent biopsy only, and one patient had subtotal resection as the initial procedure. The average number of surgical procedures per patient was two (range, one to five). Six of the 44 patients had no additional surgery. The second-look procedures performed in the other 38 patients included exenteration (14), prostatectomy (7), cystoscopic/perineal needle biopsy (8), laparotomy with biopsy (6), and subtotal excision with bladder salvage (3). Additional surgery was required for four patients, for evaluation of a residual mass, postoperative fistula, ureteral stricture, or small bowel obstruction. Six patients with relapse or residual disease underwent additional chemotherapy and late exenteration (3), prostatectomy (1), or biopsy (2). Four of the six have been cured, one is in treatment for a second malignancy, and the other has residual disease after exenteration. Thirty-six of the 44 patients with group III tumors have been cured (minimum follow-up period, 6 years; range, 6 to 11 years), compared with 23 of the 47 in IRS-II (1978-1984) (P = .001). Two of the six deaths in this group were caused by infection. The bladder salvage rate for those cured of their disease also was better (64% v 57% for IRS-II). The two patients with group IIA tumors were cured by gross primary excision, local radiotherapy, and vincristine and actinomycin therapy. By contrast, all patients with metastatic disease (group IV) died of the tumor. Conservative, delayed surgery, performed after intensive chemotherapy with or without radiotherapy, yields a better cure rate while maintaining a high rate of bladder salvage in children with group III prostatic RMS.

Adolescent↗
PubMed1997

Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature.

Rhabdomyosarcoma (RMS) of the lung is a very rare lesion, but the association with cystic adenomatoid malformation (CCAM) is unlikely to be a coincidence. Although the etiologic factors predisposing infants and children to pulmonary neoplasms are unknown, pulmonary developmental abnormalities may play a pathogenetic role. A case of embryonal pulmonary RMS is described, which was discovered within a congenital CCAM in a 22-month-old child. The hypothesis regarding histogenesis of this neoplasm are also briefly discussed. Because of the risk of malignant change, early removal of the congenital cystic lesions of the lung is advisable.

Biopsy↗
PubMed1998

Combined resection of abdominal aorta and inferior vena cava for retroperitoneal rhabdomyosarcoma invading the aortoiliac bifurcation.

The authors report on an 11-year-old boy with retroperitoneal alveolar rhabdomyosarcoma at the aortoiliac bifurcation. The tumor was resected en bloc together with the infrarenal abdominal aorta and the inferior vena cava. The aortic defect was repaired successfully with a Y-shaped Dacron graft. The authors conclude that tumor resection with aortic reconstruction can be a choice for unresectable retroperitoneal tumors in children.

Aorta, Abdominal↗
PubMed1992

The use of continent diversion in the management of rhabdomyosarcoma of the prostate in childhood.

Three children with rhabdomyosarcoma of the prostate treated with combination chemotherapy and radiation therapy underwent radical extirpation with continent diversion to the urethra. This urological procedure is technically feasible even in the small child (2 years old) and offers chance for cure. Radical cystectomy with continent diversion need not be considered as mutilating surgery.

Child, Preschool↗
PubMed1989

Testicular carcinoma in situ associated with rhabdomyosarcoma of the spermatic cord.

A 12-year-old boy had an embryonal rhabdomyosarcoma in the distal portion of the spermatic cord. The tumor partially surrounded the testis, infiltrated the testicular tunics and formed an intratesticular nodule near the rete testis. The unaffected testicular parenchyma exhibited the characteristic germ cells of carcinoma in situ. We describe an association between these 2 types of tumors.

Carcinoma in Situ↗
PubMed1989

Mortality in pediatric paratesticular rhabdomyosarcoma: a multivariate analysis.

We performed a retrospective analysis of 28 children presenting with confirmed paratesticular embryonal rhabdomyosarcoma to examine factors predictive of fatal outcome. Complete surgery, defined as radical inguinal orchiectomy with clear microscopic margins plus radical, retroperitoneal lymph node dissection with extirpation of all gross disease, was performed in 21 patients (75 per cent). All patients received chemotherapy and 20 received radiation therapy according to protocol. Of the patients 16 have survived for more than 5 years with no evidence of disease. Univariate analysis revealed that only the presence of parenchymal metastases at diagnosis (p less than or equal to 0.040) and unresectability (p less than or equal to 0.003) were significant predictors of fatal outcome. Multivariate analysis showed that unresectability was the most important predictor of mortality with an estimated relative risk of 5.8.

Adolescent↗
PubMed1988

Fibrous hamartoma of infancy masquerading as a rhabdomyosarcoma of the spermatic cord.

Fibrous hamartoma of infancy is an uncommon nonmalignant fibroproliferative tumor found predominantly in the upper torso within the first 2 years of life. We report a case of fibrous hamartoma of infancy, which presented as a rapidly growing scrotal mass clinically indistinguishable from a rhabdomyosarcoma of the spermatic cord. The clinical characteristics of this neoplasm as well as management are discussed.

Diagnosis, Differential↗
PubMed1986

Rhabdomyosarcoma of the penis.

We report a case of rhabdomyosarcoma of the penis, which was treated with local excision and chemotherapy.

Combined Modality Therapy↗