Myogenic Markers in the Evaluation of Embryonal Botryoid Rhabdomyosarcoma of the Female Genital Tract
ABSTRACT 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.
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- "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  . "
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.Surgical Neurology 02/2007; 68 Suppl 2:S29-33; discussion S33. DOI:10.1016/j.surneu.2007.01.079 · 1.67 Impact Factor
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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 · 1.23 Impact Factor
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ABSTRACT: Benign and malignant soft tissue tumors and pseudotumors can rarely arise anywhere in the female genital tract. Their pathologic features as well as behavior typically overlap with those described in tumors involving typical locations. However, due to their rarity, not infrequently these tumors represent a diagnostic challenge. Their diagnosis should be based on careful gross examination, thorough sampling, and morphologic evaluation, applying a selected immunohistochemical panel and molecular studies. Accurate classification of these tumors is important because their clinical behavior, prognosis, and therapy differ markedly. This article outlines several mesenchymal lesions reported in the female genital tract, encompassing recent histologic, immunohistochemical, and molecular findings, with special emphasis on problems in the differential diagnosis.Surgical Pathology Clinics 12/2009; 2(4):755-783. DOI:10.1016/j.path.2009.08.018