Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors analyzed 1422 orbital tumors examined 1955-1986 in the eye pathology laboratory. The 5 leading malignant orbital tumors were adenocarcinoma (207 cases), squamous cell carcinoma (135 cases), rhabdomyosarcoma (52 cases), lymphosarcoma (39 cases), and malignant mixed tumor (29 cases). The 5 leading benign orbital tumors were cavernous hemangioma (304 cases), benign mixed tumor (109 cases), inflammatory pseudo-tumor of the orbit (101 cases), dermoid cyst (100 cases), and optic meningioma (65 cases). Rare tumors of the orbit included 1 case each of alveolar soft tissue sarcoma, chondrosarcoma, mesenchymal chondrosarcoma, synovial sarcoma, giant cell tumor, granular myoblastoma, metastatic leiomyosarcoma of the uterus and metastatic lymphoepithelioma. The criteria for pathological classification and differential diagnosis are discussed.
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PMID:[Histopathologic classification of 1422 orbital tumors]. 186 Apr 6

The immunohistochemical distribution of alpha and beta subunits of S-100 protein (S-100 alpha, S-100 beta, respectively) in 138 cases of human brain tumors was investigated by the avidin-biotin immunoperoxidase method. Brain tumors can be divided into four groups: group 1 [S-100 alpha (+) and/or S-100 beta (+)]; astrocytoma, glioblastoma, ependymoma, subependymoma, oligodendroglioma, choroid plexus papilloma, gangliocytoma, meningioma, chordoma, malignant melanoma. Group 2 [S-100 alpha (+) and S-100 beta (-)]; pineoblastoma, pituitary adenoma, craniopharyngioma, rhabdomyosarcoma. Group 3 [S-100 alpha (-) and S-100 beta (+)]; acoustic Schwannoma. Group 4 [S-100 alpha (-) and S-100 beta (-)]; medulloblastoma malignant lymphoma, germinoma. The S-100 beta immunoreactivity pattern in brain tumors was similar to those obtained using conventional anti-S-100 protein sera. In the first group of brain tumors both the number of positively stained tumor cells and the staining intensity were generally greater for S-100 beta than for S-100 alpha with a few exceptions including one gemistocytic astrocytoma, one subependymoma, one malignant melanoma, and some cases of glioblastomas. As to the relationship between malignancy and S-100 protein in glioma, S-100 beta immunoreactivity decreased according to degree of malignancy, while that of S-100 alpha varied, suggesting a heterogeneity of tumor cells in glioblastomas. Immunostaining for S-100 alpha and S-100 beta might become a useful diagnostic procedure in brain tumors and may give us more detailed and precise data of S-100 protein in brain tumors.
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PMID:Immunohistochemical study on the distribution of alpha and beta subunits of S-100 protein in brain tumors. 188 40

We reviewed patient records of 99 consecutive orbital exenterations performed between 1969 and 1988. Patients ranged in age from 2 to 86 years (mean, 55.9 years). Classification of cases on histopathologic criteria showed 32 exenterations were performed for squamous cell carcinoma originating in the paranasal sinus (13), skin (12), conjunctiva (six), and lacrimal sac (one). Orbital exenteration was performed for treatment of other epithelial malignancy in basal cell carcinoma (eight), sebaceous carcinoma (six), adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor (one), and transitional cell carcinoma (one). Exenterations were performed for melanoma of the conjunctiva (ten), nasosinus (three), skin (two), orbit (two), and choroid (one). Exenterations were also performed as treatment for mucormycosis (five), meningioma (three), fibrosarcoma (two), rhabdomyosarcoma (two), hemangiopericytoma (two), orbital cellulitis (one), fibrous histiocytoma (one), schwannoma (one), lymphangioma (one), benign lymphoepithelial lesion (one), and undifferentiated malignancy (one).
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PMID:A 20-year series of orbital exenteration. 195 84

The radiologist must have a thorough knowledge of the normal anatomy and the pathologic spectrum of the skull base to determine the extent of abnormality and to help plan the surgical approach. The authors describe and present examples of congenital, benign, and malignant lesions that affect this region, including cephalocele, fracture, fistula, juvenile angiofibroma, meningioma, chordoma, pituitary adenoma, chondrosarcoma, nasopharyngeal carcinoma, and rhabdomyosarcoma. Metastatic, infectious, and other miscellaneous processes are also discussed. Imaging strategies with computed tomography and magnetic resonance imaging to aid in the diagnosis are suggested.
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PMID:CT and MR imaging of the central skull base. Part 2. Pathologic spectrum. 221 72

The correction of marked blepharoptosis in patients with severe or potential keratopathy will worsen the keratopathy and possibly lead to the complications of corneal ulceration and endophthalmitis. The conjunctival flap--cosmetic shell--ptosis procedure is well suited to this difficult management problem. Patients are initially treated with a conjunctival flap to protect their cornea. Subsequently they are fit with a cosmetic shell, and finally they undergo surgery to correct their ptosis. This three-stage procedure has produced excellent cosmetic and functional results in two patients, one of whom had ptosis and severe radiation-induced keratopathy following the treatment of a rhabdomyosarcoma; the other patient had severe ptosis associated with lack of corneal sensation and orbicularis function following removal of a cerebral meningioma.
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PMID:Conjunctival flap-cosmetic shell-ptosis procedure. Treatment of blepharoptosis in severe keratopathy. 259 74

A 20-year-old man had a left parapharyngeal neoplasm that displayed features of sarcoma of the nerve sheath, meningioma with an osseous component, rhabdomyosarcoma, and ganglioneuroma. The diverse cellular population suggests a tumor origin from the ectomesenchymal remnants of the neural crest. The mesenchymal part of the crest would differentiate into a variety of mesenchymal neoplastic elements, and the neuroectodermal (neuroepithelial) portion into the ganglioneuroma. These various neoplastic constituents then would form a neoplasm of mixed mesenchymal and neuroepithelial origin, or an ectomesenchymoma.
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PMID:Parapharyngeal neoplasm of mixed mesenchymal and neuroepithelial origin. 624 67

The 14 tumors reported in Rubinstein-Taybi syndrome since 1989, when added to the 22 previously reported, are beginning to show a pattern of neural and developmental tumors, especially of the head, which is malformed in the syndrome. Among the neoplasms were 12 of the nervous system: 2 each of oligodendroglioma, medulloblastoma, neuroblastoma, and benign meningioma, a pheochromocytoma, and 3 other benign tumors; 2 of nasopharyngeal rhabdomyosarcoma; and 1 each of leiomyosarcoma, seminoma, and embryonal carcinoma. Among the other benign tumors were an odontoma, a choristoma, a dermoid cyst, and 2 pilomatrixomas.
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PMID:Tumors in Rubinstein-Taybi syndrome. 955 2

Primary rhabdomyosarcoma of the meninges, a very rare brain tumor, is reported. Cytologic findings by squash preparation were useful as an adjunct to frozen section diagnosis during surgery. The cytologic features of rhabdomyosarcoma without cross-striation have some similarities to those of gemistocytic astrocytomas and anaplastic meningiomas, but the cytoplasmic filaments of rhabdomyosarcoma are different from those of gemistocytic astrocytoma and anaplastic meningioma. Histologically the tumor was embryonal rhabdomyosarcoma with a partially botryoid pattern. The tumor cells have no cross-striations but react positively to antimyoglobin serum on immunoperoxidase staining. In this case, intracytoplasmic filaments resembling poorly formed myofibrils were found on electron microscopic study. The histologic and immunohistologic findings suggest that this rare mesenchymal malignancy might arise from primitive totipotential cells.
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PMID:Meningeal rhabdomyosarcoma. Report of a case with cytologic, immunohistologic and ultrastructural studies. 776 28

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.
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PMID:Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass. 812 54

Fibrous orbital tumors present clinically and radiographically in a broad spectrum ranging from a benign mass, to locally aggressive tumor, to invasive malignancy. Pathologic analysis and diagnosis are often challenging, usually based on a combination of light microscopy, immunohistochemistry, and electron microscopic findings. Some light microscopic and immunohistochemical findings, however, are relatively characteristic. A storiform or cartwheel pattern and vimentin staining are characteristic of fibrous histiocytoma. A herringbone pattern is usually found in fibrosarcoma. A "patternless pattern" and CD34 staining is found most commonly in solitary fibrous tumor. CT and MR imaging findings, as well as clinical presentation, in fibrous orbital lesions are often difficult to distinguish from those of other orbital masses, although there may be useful clues. Benign fibrous lesions are usually well-circumscribed and may chronically remodel bone, whereas more aggressive malignant fibrous tumors tend to have infiltrating margins and may destroy bone on CT or MR imaging. With malignant fibrous masses, enhancement patterns on CT or MR imaging may be more inhomogeneous, with avascular or necrotic nonenhancing regions. At MR imaging, benign lesions tend to be homogeneous on T1, T2, and postgadolinium T1-weighted images, whereas malignant soft tissue lesions may change their pattern from homogeneous on T1-weighted images to heterogeneous with low signal septations on T2-weighted images. Low T2 signal comprising part or all of a fibrous lesion correlates with dense collagen fibers, with a less cellular matrix. Areas of hyperintensity on T2-weighted images correspond with a more cellular matrix of fibroblasts and other cells. Calcification within a tumor, however, may give a similar appearance. Thus, if a lesion has predominantly low signal on T2-weighted images, or less specifically has low signal septations, then a fibrous orbital lesion with high collagen content may be ranked higher in the differential diagnosis (see Figs. 2E and 3B). When T2 signal is intermediate-to-high, then one has a difficult time narrowing the differential diagnosis. Radiographically, distinguishing these lesions from other fibrous orbital lesions, as well as from other varieties of orbital masses, is difficult. Differential diagnosis of fibrous orbital masses includes all the fibrous lesions described in this article, in addition to schwannoma (Fig. 7), neurofibroma (Figs. 4 and 8), hemangiopericytoma (Figs. 9 and 10), rhabdomyosarcoma, meningioma, lymphoma, and metastasis (Figs. 11 and 12). A history of prior orbital irradiation for retinoblastoma or other tumors may raise the possibility of radiation-induced secondary tumors, such as MFH, fibrosarcoma, and osteosarcoma. Determining the extent of orbital involvement remains the primary goal of the radiologist. The final diagnosis still rests with the pathologist.
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PMID:Fibrous histiocytoma and fibrous tissue tumors of the orbit. 1002 37


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