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Treatment of genitourinary rhabdomyosarcoma in children.

We report on 27 children with rhabdomyosarcoma of the bladder, prostate, uterus, cervix and vagina treated with combined therapy of surgery, radiotherapy and multiple drug chemotherapy. Of the 27 patients 18 are alive for 13 to 147 months (median 86 months) and 9 have retained a functional bladder. Extirpative surgery followed by adjuvant chemotherapy and occasionally radiation therapy was most effective for local tumor control and 7 of 10 patients survived, including 5 with an intact functional bladder. Primary use of chemotherapy in 13 patients with extensive disease resulted in complete tumor control in only 3 but 7 survived, including 4 with an intact bladder. Radiation therapy used concomitantly with chemotherapy failed to control the local tumor but with subsequent extirpative surgery all 4 patients survived.

Adolescent↗

Paratesticular rhabdomyosarcoma in childhood.

The clinical and morphologic features of 18 patients with paratesticular rhabdomyosarcoma are reviewed. Although medical attention was sought frequently within 2 months the lesion was mistaken initially for other more common scrotal conditions in more than a third of the patients, delaying a correct diagnosis for 3 weeks to 4 months. Half of the patients had retroperitoneal lymph node metastases. However, with multimodal therapy 61 per cent remain free of disease at a median of 6 years from diagnosis.

Adolescent↗

Rhabdomyosarcoma of the paratesticular tissues.

We report 2 cases of rhabdomyosarcoma of the paratesticular tissues. Differentiation from chronic epididymal infection, such as tuberculosis, may be difficult but it must not delay operation. Lymphography is of limited value in delineating lymph node metastases. In both cases secondary spread was suggested but not confirmed histologically. Lymphography, because of a definite mortality rate of 0.1 per cent and morbidity rate of 1 per cent, would appear not to be indicated.

Adolescent↗

Rhabdomyosarcoma of the bladder and prostate in children.

We compare 8 patients wth rhabdomyosarcoma of the bladder and prostate who underwent either a radical extirpative operation, chemotherapy and radiotherapy or biopsy, chemotherapy and radiotherapy. The excellent survival in the former and the uniformly fatal outcome in the latter suggest that chemotherapy and radiotherapy are not substitutes for a radical extirpative operation. Indeed, proper care of these patients includes all 3 modalities.

Child↗

Paratesticular rhabdomyosarcoma in children.

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.

Child↗

Paratesticular rhabdomyosarcoma in childhood.

Ten boys with paratesticular embryonal rhabdomyosarcoma were treated in a 44-year period. Two prognostically distinct stages of the disease and appropriate treatments are described. Children with non-infiltrating tumors (stage IA) are curable (3 patients are alive and free of tumor after 8 to 44 years) and radical orchiectomy with adjunctive radiation or, preferably, chemotherapy is adequate for these patients. Conversely, in those children with infiltrative tumors retroperitoneal nodal metastases invariably develop. Early retroperitoneal lymphadenectomy with adjuvant radiation and multidrug cyclic chemotherapy is life-saving (3 are alive and free of tumor after 2 1/2 to 4 years). However, delayed recognition and treatment of retroperitoneal disease, despite agressive therapy, is frequently disappointing--2 of 4 patients died after 2 and 6 years; another, who had advanced disease, is lost to followup and also is presumed dead and 1 is alive and free of tumor after 6 1/2 years.

Adolescent↗

Embryonal rhabdomyosarcoma of the prostate.

Embryonal rhabdomyosarcoma of the prostate is typically a rare mesenchymal tumor of young boys, presenting with obstructive urinary symptoms or an abdominal mass. The origin is probably in undifferentiated mesenchymal cells that persist into adult life. Histologically, the tumor cells resemble normal muscle development in a 7 to 10-week fetus. Treatment is combined radical surgery and chemotherapy, and usually radiotherapy as well. The prognosis is extremely poor in all patients. Our case is added to the few in the literature in which this disease occurred in a man more than 50 years old.

Humans↗

Urogenital rhabdomyosarcoma in childhood.

Recent advances in chemotherapy and radiotherapy have improved the prognosis of urogenital rhabdomyosarcoma. Preliminary reports suggest that a mutilative operation may no longer be essential to cure this disease.

Antineoplastic Agents↗

Rhabdomyosarcoma of the spermatic cord: report of 3 cases with review of the literature.

Rhabdomyosarcoma of the spermatic cord is a rare, highly malignant lesion, for which the optimum treatment has yet to be ascertained. Results of treatment of 3 patients are presented, making the total reported experiences 53 cases. Two of our 3 patients are alive. One survivor underwent retroperitoneal lymphadenectomy and the other received radical orchiectomy only. The third patient had metastasis at the time of diagnosis and subsequently died. Because of a paucity of knowledge concerning the disease, its propensity for youthful patients and its highly malignant characteristics, all treatment modalities must be considered and tailored to the needs of the individual patient.

Adolescent↗

Case report of embryonal rhabdomyosarcoma of the testis with an 8-year survival.

The low incidence of embryonal rhabdomyosarcoma prevents any one person or institution from presenting any meaningful data in the form of effective treatment based on clinical experience. We realize that this is an antidotal case but the stakes are high when treating any malignancy of the young. Therefore, we advocate early and aggressive therapy using all modalities of treatments, and cite this case which initially seemed hopeless but to date has all the hallmarks of a cure.

Adolescent↗

Rhabdomyosarcoma. From the laboratory to the clinic.

The prospect of identifying and developing new agents for treatment of rhabdomyosarcomas is discussed in the light of current prognosis for children with advanced stage disease. Preliminary attempts to identify tumor-specific agents using in vitro cell culture show potential promise, but as yet remain unproven. The more complex system of identifying therapeutically active agents using human tumor xenografts has demonstrated usefulness. The potential problems associated with this system are discussed.

Animals↗

Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT.

Rhabdomyosarcoma is the most common primary orbital malignancy of childhood. It can present insidiously, mimicking other (benign) processes clinically and radiographically. CT and MR imaging are crucial in the diagnostic evaluation, treatment planning, and follow-up monitoring of the disease. Such imaging, especially when contrast is used, can accurately detect and state the extent of tumor involvement.

Adolescent↗

Malignant soft tissue tumors (malignant fibrous histiocytoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma): an electron microscopic study.

A comparative ultrastructural analysis of malignant soft tissue tumors (malignant fibrous histiocytoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma) revealed similar ultrastructural features in this group of tumors. However, by electron microscopy these tumors can be differentiated on the basis of cytoplasmic and extracytoplasmic features (myosin filaments, lipid droplets, and perinuclear intermediate filaments, for example). This is even true of less well differentiated tumors and tumor cells. These findings support and amplify the concept of a common histogenesis for tumors of mesenchymal origin. Paradoxical features observed by light microscopy warrant further study by electron microscopy if the correct diagnosis is to be made in atypical cases, such as apparent malignant fibrous histiocytoma with cross striations.

Adult↗

Cytologic "differentiation" in childhood rhabdomyosarcomas following polychemotherapy.

The effectiveness of polychemotherapy in young patients with rhabdomyosarcomas has been well established. The morphologic alterations in the tumor tissue, however, have not been widely reported. Therefore, in a group of 15 patients from 1 to 24 years of age, specimens of tumor tissue obtained before and after polychemotherapeutic treatment were compared. No morphologic changes, other than fibrosis and necrosis, occurred in patients who initially had virtually undifferentiated tumors. When moderately or well-differentiated areas were present in the initial specimens, these areas showed proportionate increases in the follow-up specimens. Moreover, the cellular characteristics of round rhabdomyoblasts and strap cells with or without cross-striations became more distinct after treatment. However, cell types that were not present in the initial specimens were never found in follow-up specimens. It was concluded that the major role of polychemotherapy is the selective destruction of undifferentiated tumor cells; further differentiated cells are stimulated either directly or indirectly to reach their maximal inherent differentiation levels, but it does not appear that transitions from one cell type to the other occur.

Adolescent↗

DNA-aneuploidy in rhabdomyosarcomas as compared with other sarcomas of childhood and adolescence.

In the current study DNA-ploidy was determined in a group of 11 desmin-positive rhabdomyosarcomas and found to be aneuploid in all of them. In three cases, synchronous metastases could be studied as well. In two of them a hypotetraploid peak was observed not present in the primary tumor. In two other cases residual tumors obtained after chemotherapy appeared to have DNA-profiles similar to those observed in their primary tumors. These findings suggest either a selection of metastasizing clones or tumor progression. No apparent selection of clones responding to therapy could be established. For comparison, a group of other childhood sarcomas (four Ewing's sarcomas, two primitive neuroectodermal tumors, and two epithelioid sarcomas) was also studied. All except one of these cases appeared to have diploid or near-diploid stemlines. These results suggest that DNA-ploidy may be used as an additional criterion in the differential diagnosis of childhood sarcomas.

Adolescent↗

Nasal pleomorphic adenoma with skeletal muscle differentiation: potential misdiagnosis as rhabdomyosarcoma.

Pleomorphic adenoma can show diverse lines of differentiation in the epithelial and myoepithelial elements, such as cartilage, bone, and fat. Myoid differentiation, however, has not been documented. We report an unusual case of nasal pleomorphic adenoma which shows focal skeletal muscle differentiation. The tumor was discovered only after successful radiation therapy for an undifferentiated carcinoma of the nasopharynx. Apart from being of morphological interest, the presence of cells with skeletal muscle differentiation also raises the practical issue of possible confusion with rhabdomyosarcoma in a biopsy. The distinguishing features are lack of cytological atypia and presence of a neoplastic glandular element.

Adenoma, Pleomorphic↗

Long survival of a patient with paratesticular rhabdomyosarcoma with inferior vena cava involvement.

A 13-year-old boy with a paratesticular embryonal rhabdomyosarcoma and a large thrombus into the inferior vena cava reaching the suprahepatic vein is presented. We used cardiopulmonary bypass with deep hypothermic circulatory arrest to realize a complete exeresis of the tumor and thrombus, followed by systemic chemotherapy and radiotherapy. Ten years later the patient is alive and doing well without any sequelae.

Adolescent↗

Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature.

OBJECTIVES: Sarcoma botryoides rarely arises in the uterine cervix. It usually presents in the adolescent age group. The peak incidence is in the second decade. A combined modality approach to treating rhabdomyosarcoma (RMS) has markedly improved survival. We present two cases of cervical RMS. One of them had a very aggressive clinical course. CASE: Two cases of RMS in young women who presented with rapidly progressive cervical tumor are reported. They were treated with two-stage surgical procedure and chemotherapy. CONCLUSIONS: A combined modality approach to treating RMS using multidrug chemotherapy, radiotherapy, and surgery has markedly improved survival. One of our cases has been free of disease for more than 5 years.

Adolescent↗