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At least 19 recordsLinked to original sources
PubMed1978

Primary tumors of the external and middle ear. III. A clinicopathologic study of embryonal rhabdomyosarcoma.

Embryonal rhabdomyosarcoma of the middle ear-mastoid is a highly lethal neoplasm of childhood in most cases, which clinically simulates a chronic infection. Extensive hemorrhage and necrosis in the biopsy specimen may obscure and often delay the diagnosis. Two of the six embryonal rhabdomyosarcomas in this study showed histologic evidence of differentiation. These same two patients were the only long-term survivors. Similar examples of "differentiated" embryonal rhabdomyosarcomas of the middle ear-mastoid with prolonged survival have been recorded individually in the literature. Therapeutic implications of this finding have been discussed.

Child↗
PubMed1979

Diagnostic value of histochemistry in embryonal rhabdomyosarcoma.

Embryonal rhabdomyosarcomas from the nasopharynx of two children were examined by histochemical methods commonly applied to muscle biopsies. These stains included nicotinamide adenine dinucleotide-tetrazolium reductase (NADH-TR), succinate dehydrogenase (SDH), PAS, PAS-diastase, myophosphorylase, calcium-mediated adenosine triphosphatase (ATPase) preincubated at high and low pH, and oil red O. Myofibrils were easily identified with ATPase and blood vessel walls were also stained. NADH-TR clearly showed longitudinal and cross-striations that were not seen with H&E or PTAH stains. The modified Gomori trichrome stain additionally contributed to the recognition of myofibrils. Some techniques of muscle histochemistry applied to fresh frozen sections of tumor tissue may provide evidence of muscular differentiation in otherwise poorly differentiated sarcomas for a more accurate diagnosis of rhabdomyosarcoma.

Child, Preschool↗
PubMed2002

[Embryonal rhabdomyosarcoma of the nasopharynx in a 3-year old girl].

A case of rhabdomyosarcoma embryonal of the nasopharynx in a 3-year-old girl has been described. Clinical manifestations and initial diagnostic difficulties resulting in delay of proper diagnosis were discussed. Division of RMS, etiology, differentiation of the most often applied clinical sign and modern therapeutic opportunities depending on the location, histopathological type, the disease advancement level and the patient's age were described. Late outcome and the initially positive results of the employed treatment were emphasized in the presented case. The authors would like to underline the necessity to apply all imaging methods available (CT scan and MRI), in order to determine the exact site and extensiveness of malignancy which is crucial for evaluation of appropriate treatment and therapy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Tumor-cell DNA content predicts outcome in children and adolescents with clinical group III embryonal rhabdomyosarcoma. The Intergroup Rhabdomyosarcoma Study Committee of the Children's Cancer Group and the Pediatric Oncology Group.

PURPOSE: The prognostic value of tumor-cell DNA content (ploidy) was evaluated in children with unresectable, nonmetastic rhabdomyosarcoma of embryonal histology. PATIENTS AND METHODS: Flow-cytometric techniques were used to estimate the ploidy of tumor specimens from 34 patients with embryonal rhabdomyosarcoma who were enrolled in the intergroup rhabdomyosarcoma study III (IRS III) from 1985 to 1991. Tumors were classified as diploid or hyperdiploid (DNA content, 1.1 to 1.8 times that of normal diploid cells). The influence of ploidy on clinical outcome was assessed by the Kaplan-Meier technique and Cox regression analysis with stepwise selection. RESULTS: Twelve of the tumor specimens were diploid and 22 were hyperdiploid. The patient groups defined by diploid or hyperdiploid tumors had similar presenting characteristics (eg, age, tumor size, and anatomic site). Significantly more children with hyperdiploid tumors achieved a complete response than did children with diploid tumors (85% v 42%; P = .01). The probability of progression-free survival at 5 years (+/- SE) was 91% +/- 6% for the hyperdiploid group, compared with 17% +/- 11% for the diploid group (P < .001). Hyperdiploidy was also associated with a significantly higher overall survival rate at 5 years: 96% +/- 4% versus 50% +/- 14% (P = .004). Ploidy retained its prognostic significance after adjustment for tumor site in the Cox regression model. CONCLUSION: Tumor-cell ploidy strongly correlates with outcome in children with nonmetastic, unresectable embryonal rhabdomyosarcoma. The two biologically distinct groups identified by this measure would benefit from further refinements in risk-directed therapy.

Adolescent↗
PubMed2006

Cutaneous embryonal rhabdomyosarcoma presenting as a nodule on cheek; a case report and review of literature.

We report a case of primary cutaneous rhabdomyosarcoma, solid embryonal type, presenting as a rapidly enlarging nodule on the right cheek of a 7-year-old boy. This lesion had begun as a pea-sized nodule 8 months previously, and, with suspected abscess, had been incised. It recurred 2 months later; at that time, incisional biopsy was consistent with malignant round cell tumor. Wide local excision of the tumor was then completed. Subsequent immunohistochemical staining with desmin and myoglobin confirmed embryonal rhabdomyosarcoma. The patient underwent radiation therapy followed by chemotherapy and continues to be disease free at 14 months after his wide local excision. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.

Cheek↗
PubMed2004

Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma.

Rhabdomyosarcomas are classified into three well-defined categories: embryonal, alveolar and pleomorphic rhabdomyosarcoma. Recently, seven cases of an unusual adult type of rhabdomyosarcoma with a prominent hyaline sclerosis have been described. We report the hitherto unreported cytogenetic changes of an adult sclerosing rhabdomyosarcoma. A 79-year-old woman underwent an amputation for a rapidly growing soft tissue mass in the anterior compartment of the right lower leg. The tumor infiltrated the tibia. On histology, a fascicular spindle to round cell proliferation, embedded in a prominent hyaline matrix, was seen. Immunohistochemistry showed focal desmin, myogenin and MyOD1 expression, and electron microscopy revealed Z-band material. Cytogenetic analysis disclosed a 44-49,XX,+del(1)(p22)[2],+11,+16[5],+18[12],+21[3],-22 [cp13] karyotype. Using fluorescent in situ hybridization (FISH) analysis, the tumor cells were negative for FOXO1A-disrupting translocations specific for alveolar rhabdomyosarcoma. The chromosomal composition of malignant cells resembled the pattern of numerical changes frequently observed in embryonal rhabdomyosarcoma, suggesting a close relationship of an adult sclerosing rhabdomyosarcoma with this entity.

Aged↗
PubMed1993

Spindle cell (leiomyomatous) rhabdomyosarcoma, a rare variant of embryonal rhabdomyosarcoma.

A rare case of the leiomyomatous variant of embryonal rhabdomyosarcoma is reported. A 13-year-old boy presented with a recurrent painless mass on the ventral side of the right forefoot. Microscopically, the tumor consisted of spindle-shaped and round tumor cells in a fascicular or storiform, focally hemangiopericytoma-like growth pattern. The cytoplasm of the spindle-shaped tumor cells was eosinophilic and fibrillary, in some areas resembling smooth muscle cells. Immunohistologically, all tumor cells were vimentin-positive, most of them also stained with antibodies to desmin and muscle specific actin (MSA). In addition, many tumor cells showed a co-expression of alpha-sarcomeric actin and myoglobin. All tumor cells were negative with alpha-smooth muscle actin. Two years after surgical treatment and chemotherapy the patient is well with no evidence of distant metastases. The clinicopathological features and differential diagnostic problems are discussed.

Adolescent↗
PubMed2006

Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma.

Both epidermal growth factor receptor (EGFR) and ErbB-2 play an important role in cancer biology and constitute promising molecular targets of therapy. EGFR and ErbB-2 expression has been observed in rhabdomyosarcoma cell lines but not analyzed systematically in rhabdomyosarcoma tumors. Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2. Immunostains were assessed for intensity (0: no immunostaining; 1: weak; 2: moderate; 3: strong) and percentage of at least 500 neoplastic cells exhibiting membranous or membranous and cytoplasmic immunostaining. EGFR and ErbB-2 expression was considered positive if the product of intensity and percentage was greater than 10. Patients had a median age of 5.7 years (range 8 months-19.1 years), and of 65/66 patients, 38 were males and 27 were females. Expression of ErbB-2 was identified in 22/66 (33%) cases and tended to be more frequent in the alveolar subtype (13/32, 41%, vs 9/34, 26%, P=0.30). Expression of EGFR was identified in 31/66 (47%) cases and correlated with the embryonal subtype (26/34, 76%, vs 5/32, 16%, P<0.0001) independent of stage, age, and gender. Coexpression of EGFR and ErbB-2 was identified in eight tumors, of which six were embryonal rhabdomyosarcoma. None of the cases exhibited EGFR or ErbB-2 gene amplification, as assessed using fluorescence in situ hybridization. Furthermore, analysis of 11 additional rhabdomyosarcoma tumors (six alveolar; five embryonal) revealed no evidence of mutations in EGFR exons 18, 19, 20, and 21. In summary, expression of EGFR and/or ErbB-2 is detected in a sizeable subset of rhabdomyosarcoma tumors without evidence of EGFR or ErbB-2 amplification or mutations in the EGFR tyrosine kinase domain. Notably, expression of EGFR correlates with the embryonal subtype, which is also more likely to coexpress EGFR and ErbB-2.

Adolescent↗
PubMed1994

[Bladder embryonal rhabdomyosarcoma in the adult].

Embryonal rhabdomyosarcoma of the bladder is a tumor thar presents sporadically in the adult patient and its treatment continues to be a controversy. One such case is presented herein. The epidemiological and histopathological features of this tumor type are described and the different therapeutic approaches advocated in the literature are discussed.

Aged↗
PubMed2006

Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications.

PURPOSE: Posttreatment genitourinary embryonal rhabdomyosarcoma often shows well differentiated rhabdomyoblasts, which are detectable on routine histological staining. Definite areas of residual undifferentiated rhabdomyosarcoma indicate residual/recurrent disease. However, the recent use of immunohistochemical staining with desmin and myogenin in resected specimens and surveillance biopsies following adjuvant therapy may demonstrate scant positive staining cells that appear undifferentiated on light microscopy. To our knowledge the clinical significance of this finding is currently unknown. Therefore, we reviewed our retrospective experience with genitourinary embryonal rhabdomyosarcoma to examine the relationship between immunostain positive undifferentiated cells and subsequent clinical outcome. MATERIALS AND METHODS: A total of 14 children with a median age of 2.75 years (range 8 months to 7 years) with genitourinary embryonal rhabdomyosarcoma were identified in the histopathology database. All had biopsy confirmation of the diagnosis, followed by multi-agent chemotherapy. Two children in whom there was obvious residual active tumor at the resection margins were excluded from further analysis. Histopathological findings in all patients on the resection/posttreatment biopsy were reviewed. All specimens were immunostained with desmin and myogenin to detect residual undifferentiated rhabdomyoblasts. The relation between histopathological findings and outcome was determined. RESULTS: There were 14 cases of genitourinary embryonal rhabdomyosarcoma. In 2 cases (14%) residual embryonal tumor was pathologically confirmed following initial treatment. In 12 cases no obvious residual tumor was present following initial therapy. Rhabdomyosarcoma affected the bladder in 10 cases and the vagina in 2. There were no distant metastases in any child. Ten patients underwent local resection following chemotherapy and 2 underwent followup biopsies only without resection. A total of 11 cases showed well differentiated, posttreatment rhabdomyoblasts that was identifiable on routine hematoxylin and eosin staining with margins apparently free of tumor and 1 showed no morphological evidence of residual rhabdomyosarcoma. However, all cases demonstrated at least scant abnormal desmin and myogenin positive cells in the specimens. Four patients had no further treatment and none had clinical recurrence. All were well 10 years (range 8 to 13) after treatment. Eight patients received further treatment (chemotherapy and/or radiotherapy) based on clinical and pathological findings, followed by further resection in 3. One patient died of disease but 7 were well a median of 7.2 years (range 8 months to 13 years) after treatment. CONCLUSIONS: The significance of undifferentiated myogenin/desmin positive cells in genitourinary embryonal rhabdomyosarcoma in the absence of morphological residual/recurrent embryonal rhabdomyosarcoma remains unclear since such cells can be detected in all cases of posttreatment embryonal rhabdomyosarcoma. In some cases findings are associated with clinical disease recurrence, while others with identical histopathological findings following initial treatment have no clinical sequelae even in the absence of further treatment. In genitourinary embryonal rhabdomyosarcoma close and regular clinical surveillance is essential. Desmin/myogenin immunohistochemistry to detect scattered undifferentiated cells does not appear to provide useful prognostic information.

Child↗
PubMed1986

Immunohistochemical study of embryonic rhabdomyosarcomas.

11 orbital embryonic rhabdomyosarcomas were studied immunohistochemically from patients under 4 years of age. One out of these cases, undifferentiated, showed to be myoglobin negative, and desmin positive. We point out the importance of this fact in immunohistochemical determination of the specificity of this entity.

Child, Preschool↗
PubMed1984

The ultrastructure of alveolar and embryonal rhabdomyosarcoma. A correlative light and electron microscopic study of 17 cases.

An ultrastructural study is presented of 8 alveolar rhabdomyosarcomas and 8 embryonal rhabdomyosarcomas, and one mesenchymoma with predominantly rhabdomyoblastic differentiation. Thin and thick myofilaments and Z-lines or Z-like densities were found in all. Intermediate filaments were found in 4 alveolar and 4 embryonal rhabdomyosarcomas. A correlation was noted between the light- and electron microscopic appearance of the tumours regarding the degree of differentiation. A wide differentiation spectrum of the tumour cells was observed which could be correlated partly with normal fetal myogenesis. Ultrastructural differences were demonstrated in the alveolar and embryonal types. Electron microscopy is considered to play an important role in the characterization and diagnosis of rhabdomyosarcoma and is a valuable tool in the differential diagnosis.

Adult↗
PubMed1980

[The cellular and tissue patterns of so-called embryonal rhabdomyosarcomas. Histological and histochemical investigations (author's transl)].

Embryonal rhabdomyosarcomas consist of two histological variants; it can be distinguished between solid embryonal rhabdomyosarcomas and botryoid sarcomas. We investigated 13 solid and 7 botryoid rhabdomyosarcomas and used 46 biopsies of 20 patients between 6 weeks and 14 years old. There were 12 male and 8 female patients. The histologic examination yielded variations of cytologic pattern and tissue structure. Besides undifferentiated sarcoma cells which constituted the majority of tumor cells and a changing amount of rhabdomyoblastic cells (but only 40% of all tumors showed cells with cytoplasmic cross striation) in 6 solid and 6 botryoid tumors some other cellular features could be observed. There were tumor cells with characteristics of lipoblasts and histiocytes, and occasionally the potency to form vascular spaces was detected, too. The histochemical examination proved the presence of glycosaminoglycans and mucoproteins. The glycosaminoglycans seemed to contain only a few sulphated groups. In agreement with a preceding electron microscopic study of botryoid sarcomas in the nasopharynx (Katenkamp et al. 1979) we stated that many so-called embryonal rhabdomyosarcomas show the basic pattern of an undifferentiated embryonal sarcoma and have some diverging cellular differentiations. Therefore, it is an open question whether the term "embryonal rhabdomyosarcoma" should be defined more precisely than usually done in the routine histological work today.

Adolescent↗
PubMed1999

Differentiated embryonal rhabdomyosarcoma in a cow.

An embryonal rhabdomyosarcoma was found in the pleura of a 2-year-old Holstein cow after first delivery. The most predominant cells in the tumor were relatively small in size, but considerable numbers of more differentiated cells of larger sizes mingled with the small cells. The most differentiated cells were characterized by multinucleation, abundant cytoplasm containing cross-striated fibrils, intense immunoreactivity for desmin, and weak or negative reactivity for vimentin. Such cells, lacking mitotic activity and displaying weak or no reactivity for proliferating cell nuclear antigen, were considered to be malignant counterparts of myotubes or muscle fibers. This neoplasm seems to follow normal skeletal muscle embryogenesis, and to be capable of differentiation into the final stage of muscle development.

Animals↗
PubMed1997

Pleural effusion cytology of embryonal rhabdomyosarcoma.

This case report concerns an embryonal rhabdomyosarcoma of the testis in a 31-yr-old white male patient who underwent radical left orchiectomy, followed by combined irradiation and chemotherapy, and who 2 yr later presented with dyspnea at rest, nonproductive cough, and lower back pain for 1 wk. Chest radiographs demonstrated a bilateral pleural effusion and diffuse infiltrating lesion of the pleurae, mimicking a mesothelioma. The pleural fluid displayed noncohesive, malignant, small, round cells about 2-5 times larger than mature lymphocytes. They had large, darkly stained, pleomorphic nuclei and bubbly cytoplasm with poorly defined borders. The diagnosis of embryonal rhabdomyosarcoma was supported by a positive myosin immunostaining and ultrastructural findings of intracytoplasmic actin and myosin-type microfilaments. Our case is also notable in that the tumor was a pure rhabdomyosarcoma involving a testicular origin, and the patient is the oldest reported in the literature.

Adult↗
PubMed1986

Embryonal rhabdomyosarcoma of the uterine corpus and cervix.

Embryonal rhabdomyosarcomas of the uterus and cervix are rare tumors with a very poor prognosis historically. Adjuvant multidrug chemotherapy combined with local radiation therapy and/or surgery has resulted in markedly improved survival rates. Six patients with embryonal rhabdomyosarcoma of the uterus/cervix are reported, with a compilation of 28 additional patients from the literature. Nineteen of 24 patients (79%) treated with combination therapy were alive and well 4-147+ months (median 61 months) after diagnosis. The six cases from USC Medical Center and affiliated hospitals are all well 17-144 months (mean 95 months) after diagnosis. Analysis of the collected series indicates that extent of disease is an important prognostic factor. Adjuvant chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) improves survival. Initial therapy for embryonal rhabdomyosarcoma confined to the uterus/cervix should include radical hysterectomy, pelvic lymphadenectomy, and adjuvant chemotherapy with VAC. Pelvic radiotherapy should be employed for involved surgical margins or positive nodes. Advanced disease managed initially with chemotherapy and radiotherapy may allow subsequent curative extirpative surgery.

Adolescent↗