Rhabdomyosarcoma presents special diagnostic problems when it involves the uterine cervix in young children because tumor cells may lack marked atypia and may blend with the normal, immature, condensed, cellular stroma, rendering diagnosis difficult. Myogenic makers are a valuable ancillary technique for establishing a diagnosis of rhabdomyosarcoma. However, desmin positivity has been reported in cervical stromal cells, which can confound diagnosis. To determine whether immunohistochemical markers of skeletal muscle differentiation are helpful in the diagnosis of uterine botryoid rhabdomyosarcoma, we compared the immunohistochemical staining pattern of cervical rhabdomyosarcoma from 3 patients with that of normal uteri from age-matched autopsy controls by using antibodies for desmin, smooth muscle actin, muscle-specific actin, myoD1, myogenin, and WT-1. All tumors demonstrated at least focal immunopositivity for desmin, muscle-specific actin, smooth muscle actin, myoD1, and WT-1, and 1 tumor was also positive for myogenin. Autopsy controls showed only scattered subepithelial stromal immunoreactivity for desmin, muscle-specific actin, smooth muscle actin, and WT-1 and showed cytoplasmic, but not nuclear, immunopositivity for myoD1 and myogenin. Myometrium was diffusely positive for desmin and muscle-specific actin. We conclude that desmin, muscle-specific actin, smooth muscle actin, and WT1 are not specific for discriminating embryonal rhabdomyosarcoma from normal subepithelial cells in the female genital tract of children, although the number of immunopositive cells is consistently larger in rhabdomyosarcoma. Nuclear staining for myoD1 and myogenin appears not to occur in normal tissue, but it may be absent or sparse in embryonal rhabdomyosarcoma. Our findings indicate that, in this anatomic site, the diagnosis of rhabdomyosarcoma and in particular determination of tumor margins remain very reliant on histomorphology.
"Because of the glycogen and myofibrils contained, those tumor cells stain positively with the periodic acid-Schiff reagent and phosphotungstic acid . It expresses a DNA binding protein, MyoD1, which may turn out to be a lineage marker for rhabdomyosarcomas  . "
[Show abstract][Hide abstract] ABSTRACT: Rhabdomyosarcoma is a malignant tumor of the skeletal muscle cells. Those tumors mainly affect children and arise in primitive fetal mesenchyme even at sites that do not contain skeletal muscle. However, very few intracranial rhabdomyosarcomas have been reported. No previous case of primary intrasellar rhabdomyosarcoma except for 1 case has been reported in the literature.
A 44-year-old woman presented with fatigue and diplopia associated with amenorrhea and galactorrhea. Visual field evaluation revealed a superior temporal deficit in both eyes. A right afferent pupillary defect was present. Magnetic resonance imaging revealed an intrasellar tumor with suprasellar extension. The tumor involved the sella turcica and right cavernous sinus. The patient was preoperatively diagnosed as pituitary adenoma, which was confirmed pathologically as rhabdomyosarcoma after the operation.
Our findings suggest that rhabdomyosarcoma should be considered in the differential diagnosis of a primary intrasellar neoplasm.
[Show abstract][Hide abstract] ABSTRACT: In general, mesenchymal tumors are only rarely encountered in the uterine cervix, where they constitute less than 1% of all malignancies. In this report, the salient clinicopathologic features of the reported cases of primary mesenchymal lesions of the cervix are comprehensively reviewed. Included are lesions displaying skeletal muscle, smooth muscle, adipocytic, myofibroblastic, fibrohistiocytic, fibroblastic, neural, nerve sheath, neuroectodermal and vascular/pericytic differentiation, as well as those whose line(s) of differentiation is (are) currently uncertain. Where applicable, supplemental and unpublished data from the files of the Armed Forces Institute of Pathology (Washington, DC) are also detailed. For the published cases, the overall ratio of benign to malignant cases is approximately 1.9:1. The most commonly reported mesenchymal malignancies, listed in order of decreasing frequency, are embryonal rhabdomyosarcoma (approximately 150 cases), leiomyosarcoma (approximately 30 cases), undifferentiated endocervical sarcoma (17 cases), alveolar soft part sarcoma (11 cases), Ewing sarcoma (9 cases), malignant peripheral nerve sheath tumor (8 cases), and liposarcoma (4 cases). The most commonly reported benign mesenchymal tumors, reported with almost equal frequency, are hemangioma and leiomyoma. Each of the aforementioned lesions is associated with a relatively distinct clinicopathologic profile. Generalizations about the poor prognosis associated with “cervical sarcomas” are therefore no longer appropriate, and each case must be evaluated within the context of the reported cases of that specific histologic subtype.
Pathology Case Reviews 04/2006; 11(3):140-152. DOI:10.1097/01.pcr.0000217868.84282.77
[Show abstract][Hide abstract] ABSTRACT: An adult, captive Taiwanese monkey (Macaca cyclopis) presented clinically with a large, rapidly growing mass located in the crus of the left hind leg. The overlying skin was severely ulcerated and necrotic. Radiographs suggested an invasive neoplasm in soft tissue with no bone involvement. The animal's clinical condition progressively worsened over the next 3 months until it died. Necropsy revealed that the mass infiltrated the surrounding skeletal muscle with no evidence of distant metastasis. Microscopically, the mass was highly cellular and composed of round to spindle cells with frequent rhabdoid cells characterized by abundant eosinophilic glassy cytoplasmic inclusions and large, bizarre nuclei. Phosphotungstic acid hematoxylin staining failed to reveal distinct cross-striations within the neoplastic cells. Neoplastic cells were strongly positive for smooth muscle actin and vimentin but were negative for sarcomeric actin, myoglobin, desmin, cytokeratin, S100, and lysozyme. The gross, microscopic, and immunohistochemical findings supported the diagnosis of pleomorphic leiomyosarcoma.
Journal of veterinary diagnostic investigation: official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc 08/2009; 21(4):564-7. DOI:10.1177/104063870902100426 · 1.35 Impact Factor
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