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PubMed2002

Perianal rhabdomyosarcoma presenting as a perirectal abscess: A report of 11 cases.

BACKGROUND PURPOSE: The organs and soft tissues of the pelvis are some of the most common primary sites for rhabdomyosarcoma (RMS) in children and adolescents. In most cases a mass is detectable on clinical examination, and the initial concern is focused on the possibility of a neoplasm. The current report concerns 11 patients, each presented with a painful perineal-perianal mass suggesting an abscess to the extent that each one of these patients was treated initially with antibiotics or incision and drainage for several weeks to months before the pathologic diagnosis of RMS was established. METHODS: The authors reviewed the clinical histories of all patients with perirectal/perianal RMS from their respective institutions to identify cases in which the initial clinical diagnosis or impression was that of a perirectal abscess. Pathologic material was reviewed in all cases. RESULTS: Eleven patients, 7 of whom were girls, ranged in age from 1 to 16 years at diagnosis (median age, 14 years). Fever accompanied the clinical presentation in 2 patients. None of the patients had a past medical history of illness that may have predisposed them to a perirectal abscess, although one patient had a family history of inflammatory bowel disease. Duration of symptoms ranged from 1 month to 1 year (mean, 4.6 months). Each patient presented with a tender perianal/perineal nodule or mass. Inguinal adenopathy was present in 6 patients at diagnosis. White blood cell counts ranged from 6,600/mm(3) to 24,500/mm(3). LDH levels ranged from 414 to 3,432 U/L. The average time from presentation to pathologic diagnosis of RMS was 2.1 months. Nine of the 11 cases showed an alveolar pattern. All were high-stage disease. Of 7 patients with follow-up longer than 1 year, 2 (29%) are alive without disease. CONCLUSION: This report presents the need to consider the possibility of a malignant neoplasm, in this case RMS, in a child or adolescent with a putative perirectal abscess that fails to respond in the expected manner to incision and drainage and antibiotic therapy.

Abscess↗

PubMed1999

Clinical aspects of alveolar rhabdomyosarcoma with translocation t(1;13)(p36;q14) and hypotetraploidy.

Although most cases of alveolar rhabdomyosarcoma (RMS) are characterized by the chromosomal translocation t(2;13)(q35;q14), several cases have been reported with a variant t(1;13)(p36;q14). We present the clinical, morphological and cytogenetic features of an alveolar RMS in a 4-year-old boy. Chromosomal analysis revealed a hypertriploid to hypotetraploid karyotype with a t(1;13)(p36;q14) in all tumor cells. It appears that alveolar RMS with t(1;13) occurs in younger children and displays a higher incidence to upper and lower extremity than tumors with t(2;13).

Aneuploidy↗

PubMed1998

Development of ganglioneuroma following successful treatment for orbital rhabdomyosarcoma.

A female infant presented with a left orbital embryonal rhabdomyosarcoma at the age of 3 months. She was successfully treated for this tumour with chemo- and radiotherapy. Eight years later she developed a ganglioneuroma in the same area which was treated surgically, but recurred at the age of 19. Re-examination of all of the specimens using immunohistochemistry confirmed that the initial and successive diagnoses had been correct. Two further explanations for this rare sequence of events are considered: whether the initial biopsy had been unrepresentative of the whole tumour, or whether there had indeed been two separate tumours arising in the same area. The theories of this previously undocumented occurrence are discussed further.

Adult↗

PubMed1976

[Pulmonary rhabdomyosarcoma (author's transl)].

A 55-years old patient is reported who became ill with recurrent attacks of coughing and haemoptysis some 3 weeks before death. An extensive mediastinal tumor was demonstrated radiologically. Pathoanatomical examination showed a pleomorphic rhabdomyosarcoma of the lower left pulmonary lobe with metastases in the right lung and regional lymph nodes.

Autopsy↗

PubMed2006

[Embryonal rhabdomyosarcoma of the orbita].

A 2(1/2) year old girl was presented with an acute, painless orbital swelling without signs of local or systemic inflammation. CT and MRT scanning revealed a tumor which by open biopsy disclosed embryonal rhabdomyosarcoma. By means of this case we present the current diagnostic and therapeutic approaches for childhood soft tissue sarcomas. At present our patient receives a poly-chemotherapy which has already shown a good response. For local tumor control, a complete surgical resection as well as radiotherapy may be indicated and have to be considered carefully with respect to the possible late effects. This case demonstrates that in a child with a unilateral, progressive orbital tumor, the differential diagnosis of a soft tissue sarcoma has to be ruled out.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2006

A rare case of primary rectal rhabdomyosarcoma in an adult.

We report an extremely rare case (1:200 000) of primary rectal rhabdomyosarcoma (RMS) in an adult. In the literature, this case report is the first one dealing with an adult patient. The diagnosis was assessed by computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET), endoscopy and histological examination. Neoadjuvant systemic chemotherapy with adriamycin and isofosfamide followed by anterior rectum resection was performed. This article should implicate RMS as a rare differential diagnosis of atypical rectal tumours in adults.

Adult↗

PubMed1992

Primary rhabdomyosarcoma of the diaphragm. Report of a case presenting with hemothorax.

A rare case of embryonal rhabdomyosarcoma of the diaphragm occurring in an adult male and presenting with hemothorax is reported. The unusual clinical features of this patient underline the need for an accurate preoperative evaluation. The surgical procedure consisted of left thoracotomy with resection of the neoplasm, including a portion of the diaphragm muscle, and then reconstruction. One month after discharge chemo- and radiotherapy were carried out. The immunohistochemical study proved to be very helpful for the pathological diagnosis. A three years follow-up with patient alive and disease-free confirms that a multimodal approach may prove effective in the long term.

Adult↗

PubMedDate not supplied

[Analysis of prognostic factors in rhabdomyosarcoma. Preliminary univariate and multivariate results of the Cooperative Soft Tissue Sarcoma Study (CWS-81)].

The characteristics of 129 children with rhabdomyosarcoma entered into the CWS-81 study between 1981 and 1984 were examined for their relationship to prognosis. Prognosis was defined as relapse-free survival time. The trial was stratified to clinical groups depending on the surgical procedure at onset. So, each group had to be analysed separately. The patient characteristics of group I/IIA mostly related to prognosis were primary site (disease in extremities, unfavorable; paratesticular primary, favorable) and tumor infiltration into adjacent bone (bone infiltration, unfavorable). In group IIB/III the degree of tumor response within 7-9 weeks, chemotherapy exclusively given, was the only characteristic significantly related to prognosis (greater than 2/3 tumor reduction, favorable; less than 2/3 tumor reduction, unfavorable). Other prognostic factors as histological subtype, primary site, tumor diameter, lymph node involvement, bone infiltration, sex and age had no significant influence. The tumor diameter was the only characteristic with significant influence in group IV. The larger the tumor diameter, the poorer was prognosis. Furthermore, the alveolar subtype was seen predominantly in non-responding tumors of group IV. In group III, we fitted the patient characteristics in a multivariate regression model (Cox's model). The degree of tumor response within 7-9 weeks was analysed to be the main hazard function related to prognosis. In future, the degree of tumor response within a certain time can be used as the measurement of effectiveness of chemotherapy. So, in patients of group III and IV a risk adapted therapeutic procedure can be undertaken.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1991

Embryonal rhabdomyosarcoma of the common bile duct.

A 2 4/12-year-old boy with obstructive jaundice caused by a tumor of the portal area that proved to be an embryonal rhabdomyosarcoma of the common bile duct is reported. The diagnosis was made clinically, radiographically, ultrasonographically, and histologically. Surgery was elected as the primary therapy; there were no intra- or postoperative complications. The prognosis and its improvement by means of aggressive chemotherapy and local irradiation are discussed.

Child, Preschool↗

PubMed1982

[Embryonal rhabdomyosarcoma with infiltration of the orbit and the paranasal sinuses (author's transl)].

The authors report on a case of an orbital embryonal rhabdomyosarcoma in a 12-year-old boy. Treatment consisted of radiotherapy, chemotherapy, and local excision of the tumor, the excision, however, without any mutilating surgery. Altogether, seven children have been treated in the same way at the pediatric clinic of the University of Munich. All seven are quite well and none of them show any signs of recidivism. The longest follow-up observation period is now four years.

Child↗

PubMed1982

[Rhabdomyosarcoma of the pulmonary artery (author's transl)].

A case of a 55-year-old man with the histological diagnosis rhabdomyosarcoma of the left pulmonary artery has been seen. Lung scanning and pulmonary arteriography are the clues for the diagnostical procedure. 55 cases from the literature are reviewed and clinical findings of the early and late stages of the diseases are discussed. Surgical treatment is the therapy of choice if ever possible; aggressive chemotherapy might be an acceptable alternative.

Cyclophosphamide↗

PubMed1981

Rhabdomyosarcoma of the bile ducts.

A 4 and one half-year-old girl with rhabdomyosarcoma of the bile ducts who was managed by excision of the entire tumour mass followed by chemotherapy has survived four years without evidence of recurrence.

Bile Duct Neoplasms↗

PubMed1992

The changing role of surgery in the management of rhabdomyosarcoma.

A retrospective analysis was performed of 56 patients presenting to Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland, over a 25 year period with a diagnosis of rhabdomyosarcoma, in an effort to assess the changing role of surgery in the management of this disease. There were 38 males and 18 females; the age range at presentation was from birth to 13 years with a median of three years. Head and neck tumors occurred most frequently (23) followed by pelvic (14), paratesticular (6), extremities (6), gastrointestinal (4) and thoracic (3). Prior to 1971 there were no survivors while a doubling of survival has been observed within the past decade (31% to 63%). The minimum period of follow-up was four years. Surgical intervention ranged from total primary excision (20) to biopsy only (21) with total or subtotal resection performed electively in 15. Surgical evaluations, including examinations under anesthesia, staging laparotomy and second look laparotomy were also invaluable in assessing disease status. The incorporation of multiagent chemotherapy together with more selective use of radiotherapy has enabled a more conservative role to be adopted by the surgical oncologist thereby minimizing longterm sequelae.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1994

Paratesticular rhabdomyosarcoma (RMS) and paraaortic lymphadenectomy.

This study is based on the experience of the Italian Cooperative Studies "AIEOP-RMS 79 and RMS 88". Between October 1979 and December 1989 299 children under 15 years of age with a histologically proven rhabdomyosarcoma (RMS) were examined. We analyzed the problem of the paratesticular localization paying particular attention to the role of surgery in dealing with paraaortic nodes. In the first study (RMS 79) the surgical exploration of retroperitoneal nodes was required: this procedure performed in 11 out of 14 patients affected by paratesticular RMS confirmed the negative data observed with diagnostic investigations. In RMS 88 study the sampling of retroperitoneal nodes was not required in cases with negative radiological findings but suggested in those cases with uncertain signs; two out of 8 patients underwent a laparotomy. In these two cases the results were negative. These 22 children were treated with an adjuvant chemotherapy after surgery and at present they show no evidence of disease.

Adolescent↗

PubMed1973

A type-C virus in human rhabdomyosarcoma cells after inoculation into NIH Swiss mice treated with antithymocyte serum.

A type-C RNA virus has been isolated that replicates readily in human and other primate cells. It was obtained from a human rhabdomyosarcoma cell (RD) that had been serially transplanted in immunosuppressed NIH Swiss mice, a strain of mouse from which infectious type-C virus has not been isolated. Various other human tumor cells, similarly transplanted, remained free of overt type-C virus expression. The virus growing in the RD cells, AT-124, has a group-specific antigen and an RNA-dependent DNA polymerase immunologically related to murine type-C viruses, but a host range similar to that of the RD-114 virus. The new isolate is either a previously undescribed, endogenous type-C virus from NIH Swiss mice or a recombinant with both mouse and human type-C genetic information.

Animals↗

PubMed1985

O6-Alkylguanine-DNA alkyltransferase activity correlates with the therapeutic response of human rhabdomyosarcoma xenografts to 1-(2-chloroethyl)-3-(trans-4-methylcyclohexyl)-1-nitrosourea.

Immune-deprived female CBA/CaJ mice bearing xenografts of six different human rhabdomyosarcoma lines were treated with 1-(2-chloroethyl)-3-(trans-4-methylcyclohexyl)-1-nitrosourea (MeCCNU). Tumor responses were compared with levels of O6-methylguanine-DNA methyltransferase activity because of evidence indicating that repair of DNA interstrand cross-link precursors, mediated by the transferase, may be an important determinant of MeCCNU cytotoxicity. Levels of methyltransferase in tumor extracts were measured by determining the loss of O6-methylguanine from 3H-labeled methylated DNA. Five of the six tumor lines examined showed either no response to MeCCNU or regrowth after an incomplete response. In each instance, the extent of tumor regression correlated with the level of O6-methylguanine-DNA methyltransferase activity in tumor extracts. The single highly drug sensitive line was totally devoid of the activity. These results suggest that O6-methylguanine-DNA methyltransferase levels in human tumor cells may be a clinically useful predictor of sensitivity to the chloroethylnitrosoureas.

Animals↗

PubMed1989

A model for embryonal rhabdomyosarcoma tumorigenesis that involves genome imprinting.

Embryonal rhabdomyosarcomas (malignant pediatric tumors of striated muscle origin) have been shown to arise from cells that are clonally isodisomic for loci on chromosome 11p. We determined the parental origin of alleles in this genomic region in familial and sporadic cases of this disease and found that isodisomic chromosome 11p alleles in each tumor were of paternal origin. We have developed a modification of Knudson's two-hit model from these data that is capable of explaining the preferential allele retention and of resolving the apparent contradiction between such specific and early events in several embryonal tumors and discrepancies in the inheritance of predisposition in some of these diseases.

Alleles↗

PubMed1993

Rhabdomyosarcoma arising in transgenic mice harboring the beta-globin locus control region fused with simian virus 40 large T antigen gene.

The beta-globin locus control region (LCR) confers a high level of erythroid-specific and copy-number-dependent expression to human globin genes in transgenic mice. Simian virus 40 T (tumor) antigen (Tag) with its own natural enhancer causes choroid plexus tumors in mice. We investigated the effect of the LCR on Tag gene expression, reasoning that mice harboring a LCR-Tag fusion gene might develop hematopoietic malignancies. To test this hypothesis we introduced an enhancerless Tag gene downstream of a LCR cassette into the germ lines of mice. The phenotypes of the transgenic mice depended on the copy number of the transgene. While mice with 1-2 copies matured normally, those with 3-7 copies developed rhabdomyosarcomas in different anatomic sites at high frequency and showed hyperplasia of the pancreatic islet cells which progressed to pancreatic islet tumors. In addition, the mice bearing 7 copies of the transgene had hypoglycemia and were stunted in growth. Mice with more than 10 copies were markedly stunted in growth and died within 2-4 weeks. Tag expression was detected at high levels in the mouse tumors but not in any other tissues, including the hematopoietic cells.

Animals↗