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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↗
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↗
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↗
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↗
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↗
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↗