Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Painless unilateral proptosis is a frequent manifestation of numerous orbital neoplastic and non-neoplastic processes. Various mesenchymal tumors of both fibrohistiocytic and vascular origin are well-described causes. Solitary fibrous tumors (SFTs) are rare spindle-cell neoplasms usually found associated with serosal surfaces, especially the pleura, but they have recently been described in a number of extrapleural sites including the orbit. The authors describe the case of an 18-year-old man who presented with a 6-month history of painless proptosis in the right eye. A visible nontender mass in the right supermedial orbit producing ptosis of the upper lid was present. Magnetic resonance imaging (MRI) showed a well-circumscribed soft tissue mass located above the right globe with no obvious invasion of adjacent orbital structures. Uneventful surgical excision through a right frontal-orbitotomy approach was performed. Histological evaluation showed a solid, highly vascular tumor mass composed of spindle cells arranged in short ill-defined fascicles. Intense immunohistochemistry staining for CD34 and B-cell lymphoma 2 (BCL-2) differentiated the lesion from the more common hemangiopericytoma. Though considered benign, local recurrence and extraorbital extension of orbital SFTs have been described. Malignant behavior, including distant metastases, has been documented in as many as 20% of pleural cases with mortality rates as high as 50%. The natural history of this tumor in the orbit is unclear. The authors report the 35th case of orbital solitary fibrous tumor and discuss the differential diagnosis, histopathology, radiological features, and clinical course.
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PMID:Solitary fibrous tumor of the orbit. 1221 91

Intracavernous hemangiopericytoma/solitary fibrous tumor is an extremely rare tumor, with only seven cases reported. We present a case of intracavernous hemangiopericytoma/solitary fibrous tumor and review all cases reported in the literature. A 67-year-old man experienced numbness over the left half of the face. Magnetic resonance imaging revealed a left intracavernous tumor extending into Meckel's cave and the posterior fossa. We performed gamma knife surgery (GKS) which a prescribed dose to the tumor of 12 Gy, but tumor recurred 43 months after GKS. We performed partial tumor resection via a subtemporal interdural approach. The pathological diagnosis was hemangiopericytoma. Postoperatively, we performed second GKS with a prescribed dose of 15 Gy. Diplopia and ptosis improved markedly and the tumor initially reduced in size, but tumor regrowth was seen again 29 months after second GKS. Third GKS was performed with a prescribed dose of 15 Gy. Recurrence was not seen at 18 months after third GKS, but was identified about 2 years after third GKS. We performed fourth GKS with a prescribed dose to the residual tumor of 16 Gy. We report a rare case of intracavernous hemangiopericytoma originating in the cavernous sinus, but distinguishing between hemangiopericytoma and schwannoma is difficult for round, intracavernous tumors showing homogeneous enhancement without flow voids. GKS might be one of the options for residual and recurrent intracavernous hemangiopericytomas.
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PMID:Intracavernous Hemangiopericytoma: Case Report and Review of the Literature. 3159 58