Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Spindle cell (leiomyomatous) rhabdomyosarcoma, a rare variant of embryonal rhabdomyosarcoma. 851 10

A general practitioner was consulted by a 15-year-old girl, virgo, suffering from foetid vaginal discharge. The girl was seen by a gynaecologist after antimicrobial treatment had failed. Further investigations revealed that a embryonal rhabdomyosarcoma was present, a sarcoma botryoides. The tumour originating from the cervix uteri was resected completely after which chemotherapy was started. One year later there were no sequelae or indications of metastases. Sarcoma botryoides has a better prognosis than other types of rhabdomyosarcoma. The prognosis is also influenced by the site of origin, which is favourable for the cervix.
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PMID:[An rare cause of vaginal discharge: sarcoma botryoides]. 976 70

Rhabdomyosarcoma is the most common soft tissue sarcoma in children. Primary breast location has been reported rarely in the literature. Most rhabdomyosarcomas encountered in the breast more commonly are metastatic disease from some primary foci in another part of the body. This report addresses the case of an adolescent girl who had primary embryonal rhabdomyosarcoma of the breast with no evidence of local invasion or metastatic disease within the spectrum of Li-Fraumeni syndrome.
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PMID:Primary embryonal rhabdomyosarcoma of the breast in an adolescent female: a case report. 980 22

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.
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PMID:Spindle cell rhabdomyosarcoma in adults. A case report and literature review. 1067 75

Advances in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas continue in the Intergroup Rhabdomyosarcoma Study Group (IRSG) and European cooperative groups. The use of molecular biology techniques in soft tissue sarcomas are redefining the classic pathology of these small blue cell tumors. Improvements in imaging, radiotherapy, and surgery, in part, deserve credit for the better survival seen in all cooperative trials. These advances confound the interpretation of consecutively run chemotherapy trials using historical comparisons. The IRSG has reported improvement in the prognosis of both nonmetastatic and metastatic embryonal rhabdomyosarcoma as attributable to three, three-drug regimens that use cyclophosphamide at 2.2 g/m2 in either maintenance or induction and maintenance therapy. Patients of any age with metastatic, nonembryonal, and those over 10 years of age with metastatic embryonal rhabdomyosarcoma continue to have a poor prognosis, which even megatherapy has failed to change. The doublet of ifosfamide and etoposide in combination with vincristine, actinomycin D, and cyclophosphamide at 2.2 g/m2 achieved a remarkable 3-year survival of 58% in patients with metastatic rhabdomyosarcoma and undifferentiated soft tissue sarcoma. The topoisomerase I inhibitor, topotecan, has recently been found by the IRSG to have a 57% overall response rate in patients with metastatic alveolar rhabdomyosarcoma. Topotecan has completed testing with cyclophosphamide in a phase II window study in newly diagnosed patients with metastatic disease and has been incorporated into a randomized trial in intermediate risk patients in IRSG-V. Molecular studies in IRSG-V will be applied in the detection of occult bone marrow metastases and the evaluation of resection margins at initial and second-look surgery. Long-term follow-up will be required in patients with gross residual sarcoma randomized to conventional and hyperfractionated radiotherapy in IRSG-IV to assess late effects. Although older patients with unfavorable histology and metastatic disease continue to have a poor prognosis, the overall 5-year survival of children and adolescents with nonmetastatic and metastatic rhabdomyosarcoma is approaching 80%. As molecular discoveries advance the diagnosis and detection of rhabdomyosarcoma, it is hoped that the futuristic molecular based treatment strategies in development and early testing will further improve survival in high-risk patients with metastatic soft tissue sarcoma.
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PMID:Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas. 1075 91

In a 5-year-old Holstein cow, a neoplasm composed of a large intramuscular mass and multiple metastases in the lungs and lymph nodes was diagnosed as a pleomorphic rhabdomyosarcoma. This neoplasm was characterized by marked variation in tumor cell size and giant cells with single bizarre nuclei. Although the presence of cross striations and myoglobin could be confirmed, expression of alpha-smooth muscle actin (SMA) was also recognized in a few cells. Neoplastic cells showing intense staining for desmin, vimentin and proliferating cell nuclear antigen irrespective of their size differed from those in an embryonal rhabdomyosarcoma that exhibited a wide spectrum of differentiation, reminiscent of normal skeletal myogenesis. The cellular pleomorphism and SMA expression seemed to be characteristic of deviation from normal muscle cells or satellite cells in adult muscle.
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PMID:Pleomorphic rhabdomyosarcoma in a cow. 1121 54

We report a case of rhabdomyosarcoma which occurred in a mediastinal teratoma in a 44-year-old man. Presentation symptoms were chest pain, hoarseness and a cough. Diagnosis was fortuitous, performed by the histological and immunohistochemical study of a mediastinal tumour biopsy specimen that showed embryonal carcinoma and yolk sac tumour components associated with the rhabdomyosarcoma. After cisplatin-based chemotherapy (bleomycin-etoposide-cisplatin), surgical resection of the residual mediastinal tumour was performed. Histological and immunohistochemical study of this tumour confirmed the presence of mature teratoma and embryonal rhabdomyosarcoma. Evolution was marked by a local extension of the mediastinal tumour, occurrence of multiple metastases and bone marrow involvement. The patient died 8 months after diagnosis despite chemotherapy and radiotherapy. A review of the literature reveals that the development of rhabdomyosarcoma in primary mediastinal teratomas is unusual in adults. The diagnostic, therapeutic and prognostic implications of such an association are reviewed.
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PMID:Rhabdomyosarcoma arising in mediastinal teratoma in an adult man: a case report. 1188 12

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.
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PMID:Embryonal rhabdomyosarcoma presenting in an adult: a case report and discussion of immunohistochemical staining. 1254

We report the case of a 6-week-old girl who presented with a pedunculated embryonal rhabdomyosarcoma arising in a giant congenital melanocytic nevus (GCMN) on her lower back. There was no associated leptomeningeal involvement. The patient underwent surgical resection of the rhabdomyosarcoma at age 2 months, with subsequent chemotherapy consisting of actinomycin D and vincristine. No recurrences or metastases of tumor have been noted at 5 months of age.
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PMID:Rhabdomyosarcoma arising in a giant congenital melanocytic nevus. 1496 30

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood, the majority of which are of the embryonal rhabdomyosarcoma (ER) variety. Present day treatment protocols involve a combination of aggressive surgery, chemotherapy, and radiation therapy. Embryonal rhabdomyosarcoma of the larynx is rare and unlike ER of other regions exhibits excellent response to multimodality treatment without the need for extensive surgery. We report a case of cervical metastases in a 29-year-old man 13 years after treatment of his laryngeal ER. To our knowledge, this is the first reported case of late neck metastases in ER of the larynx and the second reported case of delayed presentation of recurrent disease. A 25-year review of all published reports of ER of the larynx was conducted that highlights the move toward organ preservation with the multimodality treatment protocols. Embryonal rhabdomyosarcoma of the larynx is highly responsive to combination chemoradiotherapy, allowing for excellent cure rates without the need for extensive surgery. Late relapses warrant long-term follow-up.
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PMID:Laryngeal embryonal rhabdomyosarcoma: a case of cervical metastases 13 years after treatment and a 25-year review of existing literature. 1549 73


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