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PubMed1985

[A case of embryonal rhabdomyosarcoma of the kidney].

A case of embryonal rhabdomyosarcoma of the left kidney is reported. A 16-year-old boy was admitted with the complaint of left abdominal pain and fever on January 6, 1983. Radiological examination showed a tumor of the left kidney; and, nephrectomy was performed. Histopathologically the entire tumor was composed of undifferentiated round cells. Diagnosis of embryonal rhabdomyosarcoma was made on the basis of special stains including immunohistochemical study with nervous tissue specific enolase. Although radiation and chemotherapy were performed postoperatively, the tumor recurred and the patient died on October 22, 1983. The problems of differential diagnosis of embryonal rhabdomyosarcoma from sarcomatous types of nephroblastoma, particularly rhabdoid tumor and other undifferentiated renal tumors were discussed. Fifteen rhabdomyosarcoma of the kidney including our case have been reported in the Japanese literature.

Adolescent↗
PubMed2007

Embryonal rhabdomyosarcoma of the cervix with focal pleomorphic areas.

An embryonal rhabdomyosarcoma (sarcoma botryoides) of the cervix occurring in a 30-year-old woman is described. In addition to typical areas of the embryonal rhabdomyosarcoma, including cartilaginous elements, the neoplasm was characterised by the presence of foci composed of highly pleomorphic cells. The significance of this finding is uncertain. These foci may represent areas of dedifferentiation in an embryonal rhabdomyosarcoma.

Adult↗
PubMed1977

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↗
PubMed1965

Embryonal sarcoma and embryonal rhabdomyosarcoma of the orbit.

A series of 34 cases of embryonal sarcoma and embryonal rhabdomyosarcoma of the orbit has been placed histologically into three groups according to their maximum degree of differentiation at any stage: (a) embryonal sarcoma, (b) non-striated embryonal rhabdomyosarcoma, and (c) striated embryonal rhabdomyosarcoma. The patients were then followed up. This paper presents a summary of the clinical course of the whole series and an account of eight typical cases; the age and sex incidence and survival rates are shown and the histology, treatment, and prognosis are discussed.

Adolescent↗
PubMed1983

Cell necrosis and endocytosis (apoptosis) in an embryonal rhabdomyosarcoma of the orbit.

An orbital embryonal rhabdomyosarcoma, which was excised from the orbit of an 8-year-old girl, was studied by light microscopy and transmission electron microscopy. Cells within the tumour demonstrated by light microscopy diffuse areas of necrosis and isolated single-cell necrosis. There were many viable tumour cells with intracytoplasmic vacuolar structures which contained basophilic granules. Ultrastructural studies showed close apposition between well-preserved tumour rhabdomyoblasts and degenerate or necrotic tumour cells: degenerate cells and condensed cell fragments were observed within the cytoplasm of the well-preserved tumour cells. Some cells which showed degenerative changes had features which suggested that they had ingested more than one degenerate cell on separate occasions. This phenomenon may be regarded as a variant of selective individual cell death, currently referred to as apoptosis, which has not been previously reported in a case of embryonal rhabdomyosarcoma. The patient remains free of tumour recurrence four years following treatment with combined radiotherapy and chemotherapy.

Cell Survival↗
PubMed2003

Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix.

INTRODUCTION: Embryonal rhabdomyosarcoma (RMS) of the female genital tract usually occurs in the vagina during childhood. In rare cases, RMS can originate in the uterine cervix, with an incidence peak in the second decade. Recent studies have suggested that it is possible to limit surgery to local excision in stage I cases. CASE REPORT: We present the case of a 13-year-old girl diagnosed with an anaplastic (pleomorphic) subtype embryonal RMS of the endocervix, who was treated successfully with polypectomy followed by ifosfamide-vincristine-actinomycin combination chemotherapy and brachytherapy. The patient exhibits no evidence of recurrence and has normal menstrual function 36 months following initial diagnosis.

Adolescent↗
PubMed1998

Integrated multimodality therapy for embryonal rhabdomyosarcoma of the lower genital tract in postpubertal females.

Embryonal rhabdomyosarcoma of the female lower genital tract is generally regarded as a neoplasm occurring in childhood, but has also been reported in adults. The philosophy of therapy, largely based on data obtained from pediatric patients, has evolved slowly from ultraradical surgery, without adjuvant therapy, to neoadjuvant chemotherapy followed by less radical surgery and postoperative radiation. We report here three cases of lower genital tract rhabdomyosarcoma in postpubertal females. A failure to observe complete responses from any single treatment modality suggests that for embryonal rhabdomyosarcoma in adult and adolescent women a multimodality approach to therapy is essential.

Adolescent↗
PubMed2000

Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Intergroup Rhabdomyosarcoma Study IV.

PURPOSE: To compare failure-free survival (FFS) and survival for patients with local or regional embryonal rhabdomyosarcoma treated on the Intergroup Rhabdomyosarcoma Study (IRS)-IV with that of comparable patients treated on IRS-III. PATIENTS AND METHODS: Patients were retrospectively classified as low- or intermediate-risk. Low-risk patients were defined as those with primary tumors at favorable sites, completely resected or microscopic residual, or orbit/eyelid primaries with gross residual disease and tumors less than 5 cm at unfavorable sites but completely resected. Intermediate-risk patients were all other patients with local or regional tumors. RESULTS: Three-year FFS improved from 72% on IRS-III to 78% on IRS-IV for patients with intermediate-risk embryonal rhabdomyosarcoma (P =.02). Subset analysis revealed two groups that benefited most from IRS-IV therapy. FFS at 3 years for patients with resectable node-positive or unresectable (group III) embryonal rhabdomyosarcoma arising at certain favorable sites (head and neck [not orbit/eyelid or parameningeal] and genitourinary [not bladder or prostate]) improved from 72% on IRS-III to 92% on IRS-IV (P =.01). Similarly, 3-year FFS for patients with completely resected tumor or with only microscopic disease remaining (group I or II) at unfavorable sites improved from 71% on IRS-III to 86% on IRS-IV (P =.04). Only patients with unresectable embryonal rhabdomyosarcoma (group III) at unfavorable sites had no improvement in outcome on IRS-IV (3-year FFS for IRS-III and IRS-IV, 72% and 75%, respectively; P =.31). CONCLUSION: IRS-IV therapy benefited certain subgroups of patients with intermediate-risk embryonal rhabdomyosarcoma. A doubling of the intensity of cyclophosphamide (or ifosfamide equivalent) dosing per cycle between IRS-III and IRS-IV is thought to be a key contributing factor for this improvement.

Adolescent↗
PubMed2003

Embryonal rhabdomyosarcoma presenting in an adult: a case report and discussion of immunohistochemical staining.

Embryonal rhabdomyosarcoma is the most common soft tissue sarcoma of childhood but is rarely seen in adults. We report an embryonal rhabdomyosarcoma of the cheek in a 21-year-old Filipino man presenting as a rapidly enlarging mass. An incisional biopsy was consistent with embryonal rhabdomyosarcoma. The patient failed to respond to one cycle of chemotherapy after initial diagnosis and workup for metastatic disease. Wide local excision of the tumor was then completed with the use of the Mohs technique to clear the glabrous cutaneous margins during surgery. A Mohs surgeon and dermatopathologist were involved in interpretation of the Mohs sections. Subsequent immunohistochemical staining revealed a poorly differentiated spindled and epithelioid cell tumor, which stained diffusely positive for vimentin, S-100, and MyoD1. Rare rhabdomyoblasts were present and stained positively for desmin, muscle-specific actin, and phosphotungstic acid hematoxylin. The patient subsequently underwent radiation therapy for a total of 6,000 cGy, followed by two cycles of chemotherapy. The patient continues to be disease-free at 22 months after his wide local excision.

Adult↗
PubMed2005

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst.

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

Embryonal rhabdomyosarcoma of prostate in an adult--a diagnostic dilemma.

Embryonal rhabdomyosarcoma of the prostate is a rare. Highly malignant tumour. The median age of occurrence is five years, but sporadic cases have been reported in adults' To the best of our knowledge, till date, fewer than ten cases have been reported of which only two are above the age of sixty years. We report a case of embryonal rhabdomyosarcoma of prostate in a patient more than sixty years of age. If one is not aware of this entity, one can make a mistake in the diagnosis as well as treatment.

Aged↗
PubMed1999

Trisomy 8 in embryonal rhabdomyosarcoma detected by fluorescence in situ hybridization.

Embryonal rhabdomyosarcoma is the most common malignant soft-tissue tumor in childhood. Cytogenetic studies of this tumor are rare. In one study trisomy 8 was found to be a primary cytogenetic abnormality. In view of the findings of trisomy 8 in a multitude of cancers, we conducted a pilot study to test the hypothesis that a subset of rhabdomyosarcoma also exists with trisomy 8. Accordingly, archival tissues of 12 cases of rhabdomyosarcoma were retrieved and fluorescence in situ hybridization (FISH) using a chromosome 8-specific, alpha-satellite probe was undertaken on formalin-fixed paraffin-embedded tissue sections using the protocol optimized in the Cytogenetics Laboratory at Rhode Island Hospital. The results obtained demonstrated that 6 of 12 tumors showed chromosome 8 trisomy, when a 15% threshold is adopted. In addition, one case was borderline, with 11% of the cells found positive for three fluorescent signals. Future experiments utilizing additional specimens from our centers as well as from other laboratories are needed to confirm and extend the findings of the present study.

Cell Differentiation↗
PubMed1994

Findings of magnetic resonance and colour-flow Doppler imaging of orbital embryonal rhabdomyosarcoma.

The authors describe a case of embryonal rhabdomyosarcoma of the orbit evaluated with magnetic resonance imaging (MRI) and colour-flow Doppler imaging. MRI demonstrated a markedly enhancing tumour with no discrete vessels. Colour-flow Doppler ultrasonography showed evidence of perfusion; however, discrete vascular channels were difficult to visualize, and no evidence of large vessels within the tumour was found at surgery. The differential diagnosis of this condition is discussed, as well as the potential role of colour-flow Doppler imaging in the assessment of orbital tumours, a role in which this method has not yet been widely applied.

Child, Preschool↗
PubMed1995

[Establishment and characterization of a childhood embryonal rhabdomyosarcoma cell line with nonrandom chromosome alterations].

A childhood embryonal rhabdomyosarcoma cell line, designated as TS-RM-1, was established from transplanted tumor in nude mouse. TS-RM-1 cells were small, spindle to polygonal shaped and cytoplasm was rich in glycogen. Estimated population doubling time was 31 hours and the distribution of chromosome number was in the range of 88 to 98. The karyotype of TS-RM-1 cells revealed nonrandom structural chromosome alterations, including der(3)t(1;3)(q12;p12-14),16q-,17q+ and 21q+. In immuno-cytohistochemical study, both TS-RM-1 cells and the primary tumor were positive for desmin and vimentin. TS-RM-1 cell line may be useful for studying the association between embryonal rhabdomyosarcoma and a specific alteration in chromosome 3.

Animals↗
PubMed1983

[Labial embryonal rhabdomyosarcoma. Value of cell culture and electron microscopy, histogenesis].

The authors report the case of a labial embryonal rhabdomyosarcoma of a baby. This appears to be an exceptional localization. The study of the first biopsy which was too superficial leads to the thought of a capillary angioma. At the age of 6 months the lesion is 35 mm in diameter, a lobulated formation, typically botryoid. The excision after a frozen section control was in healthy tissue. At the age of 10 months, there is a local relapse with a nodule of 18 mm with left submandibular lymph nodes. A second surgical operation allows the culture of the tumoral tissue. The conventional histopathological examinations shows the aspect of an embryonal rhabdomyosarcoma. On the ultrastructural examination one can visualize in some cells an intracytoplasmic filamentous material. The cellular proliferation in culture, after May Grunwald Giemsa coloration is monomorphous, spindle shaped and of fibroblastic aspect. The ultrastructural study of this cellular proliferation after trypsinization on the 7th day shows some cells including an intracytoplasmic filamentous material. On the data of the cellular culture, the ultrastructural studies and the review of the literature, the authors discuss the possibility of the relationship between the cells of embryonal rhabdomyosarcomas and the fibroblasts and myofibroblasts. Embryonal sarcoma seems to be a better denomination than embryonal rhabdomyosarcoma, as regards the histogenesis.

Age Factors↗
PubMed2006

Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature.

BACKGROUND: Embryonal rhabdomyosarcoma is a rare sarcoma which characteristically occurs in non genitourinary sites in children. CASE: We present a case of uterine embryonal rhabdomyosarcoma in a postmenopausal patient who presented with increasing abdominal girth, early satiety, weight loss, and pelvic pain. CONCLUSION: Embryonal rhabdomyosarcoma does not commonly originate from the uterine corpus, and it is rarely seen in postmenopausal patients. A review of the literature confirms the unique nature of this case.

Aged↗
PubMed1993

Clonal karyotypic evolution in an embryonal rhabdomyosarcoma with trisomy 8 as the primary chromosomal abnormality.

An embryonal rhabdomyosarcoma was analyzed cytogenetically. In primary cultures fed a serum-containing medium, 11 clones with karyotypic abnormalities were found. One had trisomy 8 only. The other 10 clones had trisomy 8 as well as additional evolutionary changes that included trisomy for part or all of chromosome 2, isochromosomes for the short and long arms of chromosome 11, isochromosomes for the long arm of chromosome 8, and extra copies of chromosome 8, some of which had an interstitial deletion in 8q. In those primary cultures that had grown in a chemically defined, serum-free medium and in all passaged cultures, trisomy 8 was the only aberration. Our findings and a survey of published information point to gain of one chromosome 8 as a frequent primary karyotypic abnormality in embryonal rhabdomyosarcomas. Trisomy for part or all of chromosomes 2 and 11 and additional gains of chromosome 8 material seem to be common secondary changes.

Abdominal Neoplasms↗