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PubMed2006

Immunohistochemical analysis for Mdm2 and p53 proteins in methylcholanthrene-induced mouse rhabdomyosarcomas.

3-methylcholanthrene (MC)-induced mouse 10 embryonal (ERSs) and 24 pleomorphic rhabdomyosarcomas (PRSs) of the dermis were examined immunohistochemically for nuclear reactivity of Mdm2, p53, and proliferating cell nuclear antigen (PCNA). ERSs were microscopically present in the rhabdium layer of the dermis from 10 to 13 weeks post injection (PI), and PRSs developed from 13 weeks PI. Moderate to marked Mdm2 reactivity was observed in each of the 10 ERSs, and 23 of the 24 PRSs. Moderate to marked p53 reactivity was observed in 5 of the 10 ERSs, and 19 of the 24 PRSs. p53 reactivity increased in PRSs compared with ERSs. The level of Mdm2 expression was significantly higher compared with p53 expression. Discordant Mdm2 overexpression was observed in 5 ERSs and 5 PRSs, and discordant p53 overexpression was observed in 1 PRSs, although co-overexpression of Mdm2 and p53 was observed in 5 ERSs and 18 PRSs. PCNA reactivity significantly increased in PRSs compared with ERSs. These results suggest that Mdm2 overexpression is an important pathogenic event in MC-induced mouse rhabdomyosarcomas, and its expression may be induced by p53-independent pathway.

Animals↗
PubMed2004

Prognostic significance of DNA ploidy and proliferative index (MIB-1 index) in childhood rhabdomyosarcoma.

The prognostic value of DNA ploidy and proliferative index (PI) are well established in many cancers, but their significance in childhood rhabdomyosarcoma (RMS) is unclear. We studied the DNA content and PI of 45 cases of childhood RMS obtained retrospectively. DNA histograms were hyperdiploid in 30 cases (67%), diploid in 6 (13%), tetraploid in 5 (11%), polyploid in 3 (7%), and nonclassifiable in 1 (2%). The 5-year overall survival rate by ploidy was 60% (3/5) in tetraploid, 57% (17/30) in hyperdiploid, and 0% in diploid and polyploid cases (P = .000002). The 5-year overall survival by a PI less than or greater than 19% was 62% (13/21) and 21% (5/24), respectively (P = .006). In multivariate analysis, DNA ploidy (P = .001) was an important independent prognostic factor. DNA content in childhood RMS is an important variable in predicting prognosis. DNA hyperdiploid and tetraploid rhabdomyosarcomas had a favorable prognosis, while DNA diploid and polyploid tumors had a poor prognosis.

Adolescent↗
PubMed2004

Expression of myogenic regulating factors, Myogenin and MyoD, in two canine botryoid rhabdomyosarcomas.

Myogenin and MyoD regulate the development of skeletal muscle, and their expressions are specific to the stages of myogenesis. Therefore, these myogenic regulatory proteins could be considered as sensitive and specific markers for rhabdomyosarcoma. In this report we investigated the immunohistochemical reactivities of myogenin and MyoD in two canine bladder botryoid rhabdomyosarcomas that were different in the degree of differentiation. MyoD was stained in the Ki-67 antigen-positive undifferentiated mesenchymal cells, which had proliferative activity similar to myoblasts differentiated from mesoblasts. In contrast, multinucleated neoplastic cells were positive for myogenin and alpha-sarcomeric actin but not for Ki-67 antigen, similar to the myotubes differentiated from myoblastic cells. The expressions of myogenin and MyoD were closely correlated to the histologic features of myogenic neoplastic cells.

Actins↗
PubMed2006

Vaginal rhabdomyosarcoma in a dog.

A 10-year-old, female, mongrel showed hemorrhage from vulva. By magnetic resonance image (MRI) and endoscopic examination, a multipapillary mass with a grape-like appearance was found around the urethral opening. Histologically, the mass consisted of variable-sized round-, spindle-to-polygonal-shaped tumor cells including many multinuclear cells. Mitotic figures were also frequently observed. In some areas, that tumor cells were loosely arranged, with intercellular myxoid components. Immunohistochemically, these tumor cells were strongly positive for vimentin and focally positive for desmin but negative for myoglobin. Thus, the case was diagnosed as a relatively poorly differentiated botryoid rhabdomyosarcoma by the macroscopic, histopathologic, and immunohistochemical identification. This is the first report of botryoid rhabdomyosarcoma developing in the vagina of a dog.

Animals↗
PubMed2005

Rhabdomyosarcoma: new windows of opportunity.

Rhabdomyosarcoma is a highly malignant, small blue cell tumor characterized by muscle differentiation. With modern treatment, more than 70% of children and adolescents with this disease are cured. Adequate biopsy to obtain sufficient tissue for accurate diagnosis and molecular characterization is critical. Patients must be assessed for tumor extent; the Intergroup Rhabdomyosarcoma Study (IRS) clinical group and Staging system is universally applied in North America. Multidisciplinary therapy is necessary to maximize cure rates. Local control relies on complete surgical excision when possible; those whose tumors are not completely excised and those with alveolar histology tumors require local irradiation to maximize local control. In North America, vincristine (Oncovin); Eli Lilly and Company, Indianapolis, http://www.lilly.com), dactinomycin (Cosmegen); Merck & Co., Inc., Whitehouse Station, NJ, http://www.merck.com), and cyclophosphamide are the standard chemotherapy agents. The IRS has used therapeutic window studies to confirm the predictive nature of preclinical xenograft models and to identify several new single agents and combinations of agents with activity in high-risk patient groups. Despite these efforts, the outcome for these high-risk patients remains poor. The next generation of Children's Oncology Group studies will evaluate the efficacy of topoisomerase-I inhibitors and dose-compression therapy approaches. New advances in molecular characterization of tumors, including gene-expression analysis, may identify new therapeutic targets that can be exploited by expanded preclinical drug discovery efforts, and hold the promise of revolutionizing risk-based therapies.

Combined Modality Therapy↗
PubMed2000

Results of rhabdomyosarcoma treatment in a developing country.

Fifty-one children (median age: 4.5 years; 4 months-16 years) diagnosed with rhabdomyosarcoma were treated in our center between 1980-1999. The primary sites were head and neck in 31.4%, the genito-urinary system in 21.6%, and extremities in 9.8% of the patients. The histopathologic subtypes were embryonal in 80.4%, alveolar in 9.8%, and undifferentiated in 9.8%. The majority of the patients were considered group III (47%) and group IV (25.5%) according the criteria of the Intergroup Rhabdomyosarcoma Study (IRS). Primary total tumour resection was performed in only 27.5% of the patients. The patients were treated with assigned regimens of IRS II and IRS III protocols. Radiotherapy was applied to 92.1% of the patients. Thirty-four patients (66.7%) were lost to follow up, and of the remaining 17 patients, 7 patients (41.2%) died, relapse occurred in 9 patients (52.9%) and 10 patients (58.8%) are alive. The percentage of cases lost to follow up during the first 10 years and the following 9 years of the study were 77.4% and 50%, respectively. In compliance with cancer treatment remains a major problem in developing countries.

Adolescent↗
PubMed1983

An adult case with rhabdomyosarcoma of the liver.

An autopsy case of primary rhabdomyosarcoma of the liver in a 70-year-old man is described. The case could not be clinically differentiated from hepatocellular carcinoma. The tumor showed typical features of rhabdomyosarcoma; phosphotungstic acid hematoxylin (PTAH)-positive myofibrils and cross-striations were observed in the cytoplasm of highly plemorphic tumor cells.

Aged↗
PubMed1999

Cervical epidural rhabdomyosarcoma with a leukemia-like presentation in an aged patient--case report.

A 77-year-old female presented with rhabdomyosarcoma manifesting as leukemia-like indications. Neuroimagings detected cervical and paravertebral masses. Immunohistochemical study of the surgically excised mass lesion from the cervical spine established the correct diagnosis. This leukemia-like presentation of rhabdomyosarcoma requires a multidisciplinary approach to establish the correct diagnosis and treatment.

Acute Disease↗
PubMed2002

Primary rhabdomyosarcoma associated with tumoral hemorrhage--case report.

A 42-year-old female presented with right hemiparesis. Computed tomography (CT) demonstrated a slightly heterogeneous high density mass over the left frontal convexity with peritumoral edema, with homogeneous enhancement by contrast material. Magnetic resonance imaging showed the left frontal convexity tumor as a heterogeneous low intensity area on the T1-weighted image and a heterogeneous high intensity area on the T2-weighted image, with homogeneous enhancement and dural tail sign after intravenous administration of gadolinium-diethylenetriaminepenta-acetic acid. After admission, the patient suddenly lost consciousness. CT demonstrated aggressive increase in the bulk of the tumor and large peritumoral hemorrhage. The tumor was totally removed. The histological diagnosis was rhabdomyosarcoma. Combined immunohistochemistry and electron microscopy techniques should be used to differentiate rhabdomyosarcoma from malignant meningioma.

Adult↗
PubMed1976

Rhabdomyosarcoma in the pediatric age group.

Forty-five consecutive cases of pediatric rhabdomyosarcoma were studied as to age at onset, histological type, primary site, stage of disease when first seen, method of treatment, and survival. It appears that rhabdomyosarcoma is a relatively radiosensitive tumor which can be controlled locally with radiation alone or in combination with surgery to excise the bulk of the tumor mass. On the other hand, chemotherapy must necessarily play a major role in treatment since most cases are first seen in the late stages of the disease. Close cooperation among a wide range of specialists, such as the pediatrician, radiation therapist, pediatric oncologist, pathologist, and surgeon is essential to accomplish a maximum therapeutic effect in the unfortunate children who develop this relatively rare but aggressive tumor.

Adolescent↗
PubMed1975

Rhabdomyosarcoma in childhood.

Fifty-four rhabdomyosarcomas in children affected mainly the pelvis and scrotum, 22 cases, head and neck, 19, and limbs and limb girdles, 11. Rhabdomyosarcomas of the female genital tract occurred only in children under 2 years, and those in the lower eyelid presented in the first year of life. A leiomyosarcoma-like appearance, and an undifferentiated small cell sarcoma sometimes mimicking Ewing's tumour, were patterns giving rise to diagnostic difficulty. Many fine gradations from undifferentiated embryonal to almost purely differentiated "pleomorphic" examples, made microscopic classification arbitrary. However, the histological pattern had no bearing on prognosis in children in this series. Of the 46 cases adequately followed, 43 are dead. Two of the 3 long-term survivors had paratesticular tumours.

Adolescent↗
PubMed1985

Large rhabdomyosarcoma of the urinary bladder in an adult. Case report.

A 56-year-old woman presented with frequency of micturition, haematuria and cardiac decompensation. Intravenous urography showed a large bladder tumour and an expansive lesion in the left kidney. The bladder tumour, removed by TUR, was found to be a rhabdomyosarcoma, and the resected tissue weighed about 310 g. Bilateral, selective renal angiography revealed a large arteriovenous aneurysm in the right kidney and a solid malignant tumour in the left. The aneurysmal vessels were ligated and cystourethrectomy and left nephrectomy were performed 2, 3 and 10 months, respectively, after the TUR. The excised urinary bladder contained no resicual tumour. A renal cell carcinoma was found in the excised left kidney. The patient recovered well, and three and a half years after TUR of the rhabdomyosarcoma she is fully active, with normal renal function and no evidence of disease.

Female↗
PubMedDate not supplied

Breast metastasis in adolescents with alveolar rhabdomyosarcoma of the extremities: report of two cases.

We describe two adolescent girls with alveolar rhabdomyosarcoma arising from extremities who developed bilateral breast metastasis in their clinical course. In both cases, there was widespread systemic disease at initial presentation. Although complete remission was achieved on the 25th week post-chemotherapy initiation, the first patient developed breast metastasis in addition to systemic recurrence on the 44th week and expired. For the second patient, breast metastasis was noted in addition to systemic disease at initial presentation. Aggressive chemotherapy with autologous transplant and radiotherapy were given in addition to bilateral subcutaneous total mastectomy. The patient remained in complete remission 3 months post-therapy. We postulate that adolescent females with alveolar rhabdomyosarcoma of the extremities have a high risk of developing breast metastasis in the pubertal period, and aggressive multidisciplinary treatment is indicated.

Adolescent↗
PubMedDate not supplied

Imaging of pediatric head and neck rhabdomyosarcomas with emphasis on magnetic resonance imaging and a review of the literature.

We present the magnetic resonance imaging (MRI), computed tomography (CT) and ultrasound appearances of three cases of pediatric head and neck rhabdomyosarcomas, involving the orbit, parameningeal region (nasopharynx and nasal sinus), and trapezius muscle anatomically. The histopathological subtypes included two embryonal and one alveolar. MRI is a superior imaging technique with its high contrast resolution of soft tissue tumors and multiplanar noninvasive capability. The literature on CT and MRI appearances of head and neck rhabdomyosarcomas is reviewed.

Adolescent↗
PubMed1996

Reduction of tumorigenicity by alpha 3 integrin in a rhabdomyosarcoma cell line.

The expression levels of integrin adhesion receptors have often been correlated with neoplastic transformation and invasiveness. To investigate more definitively the role of the integrin VLA-3 (alpha 3 beta 1) in tumor cell behavior, we transfected alpha 3 subunit cDNA into human rhabdomyosarcoma (RD) cells. Transfectants expressing high levels of alpha 3 beta 1 on their cell surface displayed an altered morphology and decreased anchorage-dependent growth in vitro. Cells expressing alpha 3 also displayed marked reduction in anchorage-independent growth in soft agar and in their ability to form tumors when injected subcutaneously into athymic nude mice. Thus, VLA-3 can repress the transformed phenotype of rhabdomyosarcoma tumor cells. Similar changes in morphology and growth characteristics were observed in cells expressing a chimeric molecule X3C4 in which the alpha 3 cytoplasmic domain had been exchanged with that of the alpha 4 integrin subunit. Therefore, alpha 3 inhibitory effects in RD cells appear not to require specific signalling through the alpha 3 cytoplasmic domain.

Amino Acid Sequence↗
PubMed2000

Colorectal adenocarcinoma as a second malignant neoplasm following rhabdomyosarcoma of the urinary bladder: a case report.

Following improvements in therapy for childhood malignancies, the striking increase in survival rate over the past 30 years has led to the increase risk of developing second malignant neoplasms (SMNs). We report a case of colorectal carcinoma as a SMN, following treatment for rhabdomyosarcoma. The patient was diagnosed with rhabdomyosarcoma of the urinary bladder at his age of three years, and developed adenocarcinoma in the colon 13 years later. Histologic examination of the surgical specimen revealed adenocarcinoma involving the rectosigmoid area with radiation colitis in its background. The tumor cells showed strong immunoreactivity for p53 protein, suggesting the role of irradiation and p53 mutation in carcinogenesis. This case emphasizes the need for dose observation in survivors of early childhood malignancies treated with radiation and multiagent chemotherapy.

Adenocarcinoma↗
PubMed1986

Primary rhabdomyosarcoma of long bone. A case report.

A case of embryonal rhabdomyosarcoma of the femur observed in an 11-year-old boy is reported, which simulated an osteosarcoma clinically and radiographically. To our knowledge, this is a second documented case of rhabdomyosarcoma of the long bone.

Child↗
PubMedDate not supplied

Primary cardiac rhabdomyosarcoma in a cat.

A seven-year-old domestic shorthair (DSH) cat was presented with anorexia and dyspnea. Pleural-pericardial effusion was detected with thoracic radiographs and echocardiography. Echocardiography demonstrated a large, soft-tissue mass in the right ventricular wall, protruding both into the pericardial space and into the right ventricle. Postmortem examination findings included a large mass in the right ventricular wall and multiple smaller masses on the external surface of the left ventricle and on the internal surface of the pericardium. Results of the histopathological and immunohistochemical examinations of the masses were consistent with rhabdomyosarcoma. This is the first reported case of primary cardiac rhabdomyosarcoma in the cat.

Animals↗