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PubMed2003

The development of VAC chemotherapy in rhabdomyosarcoma: what does one do for an encore?

The establishment of the Intergroup Rhabdomyosarcoma Study (IRS) Committee in 1972 by three extant American pediatric cooperative groups initiated five generations of studies in the multidisciplinary treatment of soft-tissue sarcomas. Taking the lead from the use of vincristine, actinomycin-D, and cyclophosphamide (VAC) by Dr. Ruth Heyn, Dr. Harold Mauer and colleagues developed a more robust VAC as well as three similar drug regimens with hematopoietic growth factor support, which, in the context of surgery with or without radiotherapy, achieved a 3-year failure-free survival rate of 83% (IRS-IV) in nonmetastatic embryonal rhabdomyosarcoma. This review documents the development of VAC and some of the new directions currently being pursued by the Children's Oncology Group Soft Tissue Committee.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1975

Rhabdomyosarcoma of the orbit in the newborn.

A full-term black boy had a 2- to 3-cm, round, bluish mass on his right lower eye-lid at birth, later diagnosed as rhabdomyosarcoma. It was cystic in nature and extended into the nasal cavity. The tumor was initially classified as neuroblastoma. The child died eitht months later and necropsy report confirmed an original ophthalmologic pathology diagnosis of embryonal rhabdomyosarcoma.

Autopsy↗
PubMed1986

Embryonic myosin heavy chain as a differentiation marker of developing human skeletal muscle and rhabdomyosarcoma. A monoclonal antibody study.

Hybridoma cell lines were obtained from the fusion of NS-O myeloma cells with spleen cells of mice immunized with bovine fetal skeletal myosin. A stable hybridoma clone, BF-G6, produced immunoglobulin G1 k antibodies reacting specifically with embryonic-type myosin heavy chains present in fetal but not in neonatal or adult human skeletal muscle, as determined by enzyme immunoassay and immunoblot analysis. Fetal but not adult skeletal muscle fibers were stained by this monoclonal antibody in indirect immunofluorescence assays; smooth muscle cells and cardiac muscle cells, as well as non-muscle cells were also unreactive. Solid tumors of infants and children were tested for reactivity with BF-G6 by immunofluorescence and immunoperoxidase staining. Embryonic myosin heavy chain was expressed in rhabdomyosarcomas but not in other types of tumor, except for Wilms' tumor. Rhabdomyosarcoma cells isolated from a bone marrow metastasis and grown in vitro for several months were also labelled by BF-G6. Embryonic myosin heavy chain can thus be used as a specific differentiation marker of normal and neoplastic skeletal muscle tissue.

Adolescent↗
PubMed1984

A metastasizing cardiac rhabdomyosarcoma in a CD 1 strain mouse.

A rhabdomyosarcoma arising in the ventricular myocardium, with a renal metastasis, is described in a 95-week-old male CD 1 strain mouse. Rhabdomyosarcoma has previously been recorded only in the BALB/c strain of mouse and never arising from heart muscle.

Animals↗
PubMed1990

Embryonal rhabdomyosarcoma of the ampulla of Vater with long-term survival following pancreaticoduodenectomy.

Rhabdomyosarcoma of the biliary tree is a rare cause of biliary tract obstruction in childhood. A 3-year-old child is reported here after presenting with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the ampulla of Vater. He underwent pancreaticoduodenectomy followed by adjuvant chemotherapy and irradiation. He is now well and free of disease 5 years following treatment. This child appears to be the first long-term survivor who has required pancreaticoduodenal resection for this lesion.

Ampulla of Vater↗
PubMed1992

Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

Cell Transformation, Neoplastic↗
PubMed1994

Retroperitoneal node biopsy in paratesticular rhabdomyosarcoma.

UNLABELLED: Retroperitoneal lymph node dissection (RPLND) was used in 121 Intergroup Rhabdomyosarcoma Study (IRS) III patients with nonmetastatic paratesticular (PT) rhabdomyosarcoma (RMS) to assess retroperitoneal lymph node (RPLN) involvement so as to determine the need for x-ray therapy (XRT). Clinical node evaluation (CNE) was accomplished by a computed tomography (CT) scan in 105 and a sonogram in six. Pathological node evaluation (PNE) was performed in 113: lymphadenectomy (9 bilateral, 85 unilateral) and biopsy in 19. Vincristine and actinomycin D were used for 1 year postoperatively in 89%; all patients who had positive PNE received RPLN XRT. This study compares CNE with PNE and evaluates predictors of relapse and survival. FINDINGS: There were clinically negative nodes (cN0) in 81% of the 121 patients. Among cN0 patients, 14% had positive nodes (pN1). Of the clinically positive (cN1) patients, 94% had pN1. RPLN relapse occurred in only two of the 121 patients. Initially both had cN0 and one had PNE that was negative. For all 121 patients, the 5-year survival was 91%. For cN0 patients, the 5-year survival was 96% compared with 69% for cN1 patients (P < .001). Among the children in whom treatment failed, nodes were cN1 in 5 of 11 (45%) compared with 15 of 107 (14%) in those whose treatment did not fail (P < .008). CONCLUSIONS: (1) Results of RPLN imaging studies were negative in 81% of patients with PT RMS (specificity 99%, sensitivity 57%). (2) RPLN recurrence is uncommon (even when RPLN are initially involved) if regional XRT and appropriate chemotherapy are used.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Alveolar rhabdomyosarcoma of the lung in a child.

The case of a 22-month-old boy with alveolar rhabdomyosarcoma of the lung is presented. Brain metastasis and recurrence of the right pulmonary hilum and parietal pleura developed 6, 11, and 24 months (respectively) after tumor resection. Chemotherapy and radiotherapy were effective. Neuron-specific enolase was very helpful in detecting metastasis and disease recurrence. Primary pulmonary rhabdomyosarcoma can be divided into two groups: tumor in the normal lung, and tumor in cystic lesions of the lung.

Biomarkers, Tumor↗
PubMed1985

Embryonal rhabdomyosarcoma: immunohistochemical characterization.

A 15-year-old boy was referred to the ear, nose, and throat clinic because of a swelling in the upper premolar region. The initial diagnosis of a poorly differentiated soft-tissue sarcoma was made. Further immunohistochemical studies established a definitive diagnosis of embryonal rhabdomyosarcoma. The tumor cells coexpressed both desmin, the component of muscle type intermediate filaments, and vimentin, which is typically found in mesenchymal tissues. Such coexpression is found in the early stages of myogenic differentiation. Another cytoskeletal protein, actin, was also found in relatively high concentrations. These results suggested the possible use of antibodies to these cytoskeletal proteins as histogenetic markers for the diagnosis of poorly differentiated rhabdomyosarcoma.

Actins↗
PubMed1988

Embryonal rhabdomyosarcoma: presentation as a parotid gland mass.

Solid tumors occur infrequently during childhood. Rhabdomyosarcoma is the most common soft tissue sarcoma occurring in children. Therapeutic advances during the last 15 years have lead to a vast improvement in cosmetic and functional results and in overall survival. A case report of rhabdomyosarcoma of the parotid gland and a review of the literature are presented to highlight these advances.

Adenoma, Pleomorphic↗
PubMed1991

Rhabdoid tumors of soft tissues: a clinicopathologic study of 26 cases enrolled on the Intergroup Rhabdomyosarcoma Study.

Twenty-six cases of malignant soft tissue tumors with features similar to renal rhabdoid tumors were identified among approximately 3,000 childhood sarcomas entered on Intergroup Rhabdomyosarcoma Studies I-III. The tumors consisted of polygonal cells with vesicular nuclei and prominent nucleoli and cytoplasmic intermediate filament inclusions as identified by electron microscopy and immunohistochemistry. The growth pattern was predominantly solid or solid-trabecular. Immunohistochemistry showed vimentin, wide spectrum keratin, and epithelial membrane antigen to be the most consistent antigenic phenotypes. Eleven patients were infants less than 1 year of age. The tumors affected predominantly soft tissues of proximal extremities, trunk, and retroperitoneum/pelvis/abdomen. Nineteen patients died within 1 to 82 months (median, 6 months) from the start of treatment. Five patients have survived the disease for 2 to 13 years. When compared with the survival analysis of 991 Intergroup Rhabdomyosarcoma Study II patients, it was obvious that this group of tumors fares very poorly (P less than .001). The tumor belongs to the group of soft tissue neoplasms showing mesenchymal and subtle epithelial differentiation, similar to epithelioid sarcoma. Because of its identifiable histology, site and age distribution, and poor outcome, it warrants a status as an independent entity.

Adolescent↗
PubMed1979

Intrascrotal rhabdomyosarcoma.

Rhabdomyosarcoma is the most frequent spermatic cord tumor of infants, children, and young adults, but also occurs as a primary tumor in the testis, epididumis, and testicular tunics. In the last fifteen years, 7 patients with intrascrotal rhabdomyosarcoma were treated at our institution, and an additional 155 cases were found on review of the English literature. On the basis of these 162 cases, incidence and survival statistics were calculated with particular attention to employed forms of therapy. An over-all survival of greater than 73 per cent should be obtainable with proper utilization of surgery, radiation therapy, and combination chemotherapy.

Adolescent↗
PubMed1980

Paratesticular rhabdomyosarcoma in an adult.

Embryonal rhabdomyosarcoma is a common tumor of children, rarely appearing in the scrotum from tunica vaginalis or paratesticular tissues. In adults it is rare and carries a particularly bad prognosis. A case of paratesticular rhabdomyosarcoma occurring in a seventy-three-year-old man who survived a total of five and one-half years from the time of his original operation, is presented.

Aged↗
PubMed1981

Combined modality therapy with conservation of organ function in childhood genitourinary rhabdomyosarcoma.

Recent refinements in the multimodal therapy of childhood genitourinary rhabdomyosarcoma have produced striking improvements in long-term survival rates while still preserving pelvic organ function in the majority of cases. Three illustrative cases of childhood pelvic rhabdomyosarcoma are presented. Chemotherapy and surgical staging are employed in all 3 cases, with 2 of the cases requiring additional local tumor excision and intraurethral radiation for control of residual microscopic tumor. While long-term follow-up has not been achieved, all 3 patients have had their pelvic organs preserved and remain tumor-free in follow-up periods ranging from twelve to twenty-four months after the initial diagnosis.

Child, Preschool↗
PubMed1989

Ultrasonography and diagnosis of pediatric genitourinary rhabdomyosarcoma.

Rhabdomyosarcoma is the most common tumor of the lower genitourinary tract in children during their first two decades of life. Four patients with genitourinary rhabdomyosarcoma are presented, with ultrasonographic and radiographic findings. The utility of ultrasound in the diagnosis of this pediatric tumor is emphasized.

Child↗
PubMed1985

A cytogenetic study of embryonal rhabdomyosarcoma.

In the cytogenetic analysis of an embryonal rhabdomyosarcoma after short-term culture, individual cells were found to contain multiple copies of chromosomes #2, #6, #8, #12, #13, #18, #20 and #21, and del(1)(:p21----qter). The tumor was hypotriploid (mode, 56 chromosomes). The relationship between these findings and published reports of karyotypes from rhabdomyosarcoma is discussed.

Aneuploidy↗
PubMed1991

Karyotypic characterization of a new human embryonal rhabdomyosarcoma cell line.

Chromosomal analysis of an advanced recurrent rhabdomyosarcoma of the embryonal type was performed on cell cultures in the 9th passage of in vitro cultivation. This tumor showed a modal karyotype of 54 and was characterized by multiple numerical and structural chromosome abnormalities, all present in high frequencies. Abnormalities observed in 100% of the cells included a der(1) chromosome with a short unidentified insertion between q31 and q32; a der(1) chromosome, arising from insertion at the same breakpoint of a longer segment with a duplicated 1q31 band and translocation of 13q23----qter to 1p36, a deleted tetrasomic 13q23----qter, and a der(4) chromosome showing 1p36----pter translocated to 4p13. Other common abnormalities included trisomy of chromosomes 8, 13, and 9p, deletions of chromosomes 6, 10, 11, and 12, and presence of marker chromosomes. Characterization of the established line at the 38th passage evidenced the persistence of both the modal karyotype and all the numerical and structural abnormalities previously found. The results of this study provide further evidence of the major involvement of alterations in chromosome 1 in the progression of rhabdomyosarcoma.

Child↗