1/34. Primary orbital leiomyoma and leiomyosarcoma.A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/34. myoepithelioma of the lacrimal gland: report of a case with potentially malignant transformation.myoepithelioma of the lacrimal gland is extremely rare and only four cases, one of which was malignant, have been reported in detail. The present report describes a case of lacrimal gland myoepithelioma in a Japanese male with histological features suggestive of potentially malignant transformation. The excised tumor consisted of two components, a central nodular component and a peripheral component surrounding the former. These components were separated by a fibrous tissue. Microscopically, both components were comprised almost entirely of spindle-shaped cells, but with some epithelioid cells containing glycogen granules. Extracellular spaces in the peripheral component were filled with eosinophilic materials with the occasional crystalloid structures, which were immunoreactive for collagen type i. Neoplastic cells were immunoreactive focally for vimentin and S-100, but negative for cytokeratins, epithelial membrane antigen, muscle actin, smooth muscle actin, desmin, myosin, and glial fibrillary acidic protein. The neoplastic cells in the central component showed nuclear pleomorphism and atypia with a higher frequency of mitotic figures, and higher labelings of proliferation markers than those in the peripheral component. Neither invasion, necrosis, nor hemorrhage was observed in the tumor. From these findings we proposed a diagnosis of potentially malignant myoepithelioma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/34. Extramedullary plasmacytoma of the orbit.PURPOSE: To report a case of extramedullary plasmacytoma of the orbit. methods: A 71-year-old patient presented with diplopia eyelid fullness and limitation of ocular motility in the left eye. visual acuity was counting fingers, intraocular pressure 34 mmHg and fundus eye examination showed choroidal folds in the involved eye. RESULTS: CT scan showed a mass filling the superior and external left orbit without bone destruction. A biopsy was performed revealing that the tumour was composed of plasmacytoid cells positive with immunohistochemical stains for Kappa light chains and epithelial membrane antigen. Systemic work up was negative. The diagnosis of extramedullary orbital plasmacytoma was made. The patient was treated with external beam radiotherapy (40 Gy) and has remained disease free for four years (49 months). CONCLUSION: Extramedullary plasmacytomas of the orbit are extremely rare tumours. Accurate and early diagnosis is essential for the therapeutic approach. Extensive medical work up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving the orbit is needed when the diagnosis of orbital extramedullary plasmacytoma is suspected because treatment and prognosis are very different.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/34. Ongoing hypermutation in the Ig V(D)J gene segments and c-myc proto-oncogene of an AIDS lymphoma segregates with neoplastic B cells at different sites: implications for clonal evolution.To investigate the role of somatic Ig hypermutation in the evolution of AIDS-associated B cell lymphomas, we analyzed the Ig V(D)J and c-myc genes expressed by neoplastic B cells in two extranodal sites, testis and orbit, and clonally related cells in the bone marrow. testis and orbit B cells expressed differentially mutated but collinear V(H)DJ(H), V kappa J kappa and c-myc gene sequences. Shared mutations accounted for 10.2%, 8.4%, and 4.3% of the overall V(H)DJ(H), V kappa J kappa, and c-myc gene sequences. Tumor-site specific V(H)DJ(H), V kappa J kappa, and c-myc mutations were comparable in frequency, and a single point-mutation gave rise to an EcoRI site in the testis c-myc dna. Both shared and tumor site-specific V(H)DJ(H), V kappa J kappa, and c-myc mutations displayed predominance of transitions over transversions. The "neoplastic" V(H)DJ(H) sequence was expressed by about 10(-5) cells in the bone marrow, and contained two of the three orbital, but none of the testicular V(H)DJ(H) mutations. The nature and distribution of the Ig V(D)J mutations found in the kappa chain suggested a selection by antigen in testis and orbit. Our data suggest that, in AIDS-associated B cell lymphomas, the Ig hypermutation machinery targets V(H)DJ(H), V kappa J kappa, and c-myc genes with comparable efficiency and modalities.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/34. Hepatocellular carcinoma metastatic to the orbit.Orbital metastases of hepatocellular carcinoma are rare. The authors report a case of hepatocellular carcinoma metastatic to the orbit. A 56-year-old woman with hepatitis b infection as confirmed serologic test for hepatitis b surface antigen (HBsAg) demonstrated superior displacement of the left eyeball. The clinical appearance of the patient demonstrated an inferior displacement of the right eye. MRI disclosed an orbital mass that extended toward the lower eyelid and into the temporal fossa with destruction of the lateral orbital wall. Upon subsequent abdominal computed tomographic scans, the primary tumor was found in the liver. A biopsy specimen of the orbit showed trabecular structures with eosinophilic cytoplasm. bile canaliculi and sinusoids were also seen. The tumor cells showed vesicular nuclei with mitoses. Immunohistochemical markers such as high-molecular-weight keratin and alpha-fetoprotein showed a positive response. However, immunohistochemical markers such as low-molecular-weight keratin and polyclonal carcinoembryonic antigen showed as negative. We diagnosed the tumor as a hepatocellular carcinoma metastatic to the orbit. A review of the pertinent literature disclosed relatively few occurrences of hepatocellular carcinoma metastasizing to the orbit.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/34. Ectopic chordoma with orbital invasion.PURPOSE: To report a rare ectopic chordoma within the orbital wall. methods: Case report. RESULTS: A 63-year-old woman developed swelling of the eyelid, tearing, blurred vision, and progressive proptosis RE of 1 month's duration. neuroimaging studies revealed an osteolytic mass with epicenter at the sphenozygomatic suture that eroded intracranially, invaded into the orbit, and compressed orbital soft tissues. Surgical debulking was done followed by radiation treatment. The pathologic findings of physaliphorous epithelial cells with multiple vacuoles containing mucin, prominent nuclei, and positive immunohistochemical staining for S-100, vimentin, epithelial membrane antigen, and pancytokeratin were diagnostic for chordoma. CONCLUSION: Orbital wall ectopic localization of a chordoma distant from the clivus is a rare occurrence.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/34. Epithelioid haemangioendothelioma of the sphenoid bone.The authors report a case of cranial EH occurred in the left sphenoid bone that was totally excised without transfusion. A 26-year-old woman presented with a 1-year history of progressing exophthalmos in the left eye. A cranial X-ray showed a mixed osteolytic and sclerotic expansile mass lesion in the left sphenoid bone. Neuroradiologic imaging study revealed a left temporosphenoidal extra-axial expansile mass lesion with heterogeneous enhancement after contrast enhancement associated with destruction and erosion of the temporal and sphenoid bones. The cerebral parenchyma and orbital contents were compressed without any sign of infiltration. Bone scan showed hot uptake in the left orbital region. Angiogram demonstrated marked neoplastic angiogenesis from the middle meningeal artery and other branches of left external carotid artery, for which preoperative embolisation was tried in vain. Cranio-orbito-zygomatic approach was undergone in two stages because of the patient's religious belief (patient was Jehovah's witness) and profuse bleeding during the first surgery. Two weeks after initial operation, second surgery was carried out with total excision of the residual mass in the left orbital ridge. Histopathological examination revealed typical epithelioid cell cords or nests in myxoid stroma with a positive immunoreactivity to factor viii-related antigen. The authors report an unusual case of EH involving sphenoid and temporal bone in a young woman. Preoperative embolisation is thought to be absolutely essential before removal. Because there is no convincing data to advise radiation/chemotherapy, total resection and close follow-up may be reasonable.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/34. Sinonasal undifferentiated carcinoma with orbital invasion: report of three cases.PURPOSE: To report three patients with sinonasal undifferentiated carcinoma (SNUC) that invaded the orbit. methods: Retrospective small case series. The clinical, radiographic, and pathologic features of three patients with SNUC were reviewed. RESULTS: Three patients with SNUC that invaded the orbit were evaluated. A biopsy was performed on the tumors, which were composed of small, hyperchromatic cells with numerous mitoses and areas of necrosis. Immunohistochemical staining was positive for cytokeratins AE1.3, epithelial membrane antigen, and neuron-specific enolase in all three tumors. Electron microscopic examination showed absence of neurosecretory granules and presence of basement membrane production. Two patients were treated with surgical resection and postoperative chemotherapy and/or radiation. One patient was treated with preoperative radiation and chemotherapy. CONCLUSIONS: Sinonasal undifferentiated carcinoma is a high-grade tumor that arises in the nasal and paranasal sinuses and may invade the orbit. SNUC should be distinguished from other small, round, blue cell tumors, in particular, esthesioneuroblastoma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/34. Solitary fibrous tumor of the orbit. Case report and review of the literature.BACKGROUND: Solitary fibrous tumor (SFT), which usually presents in the pleura and is thought to be mesothelial in nature, has been recently discovered in extrapleural sites, including the orbit. Presently ultrastructural studies show absence of epithelial-mesothelial features, and reactivity of the tumor cells to CD34 antigen on immunohistochemical analysis suggests the mesenchymal origin of such tumors. CASE DESCRIPTION: A 40-year-old woman had a 4-year history of progressive swelling of her right upper lid and a slow-growing palpable mass of the orbit. CT and MR imaging showed a well circumscribed, nonenhanced extraconal mass with mild erosion of the right orbital roof. The tumor was totally excised. Histological examination disclosed a spindle-cell tumor in a dense fibrous tissue. immunohistochemistry showed positive staining for vimentin and CD34. We review the clinical, diagnostic, and surgical features of 22 orbital SFTs including the present case. CONCLUSIONS: Orbital SFT generally pursues a slow, indolent, and nonaggressive course, reaches a size up to 4.5 cm, and can be cured by a single excision. It must be immunohistochemically differentiated from other spindle-cell tumors of the orbit.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/34. Histiocytoid variant of eccrine sweat gland carcinoma of the eyelid and orbit: report of five cases.OBJECTIVE: To study the clinicopathologic features of the histiocytoid variant of adenocarcinoma of the eccrine sweat gland of the eye and orbit. DESIGN: Retrospective case series. PARTICIPANTS: Five patients undergoing orbital and eyelid biopsy as a diagnostic procedure. methods: The authors examined the clinical histories and pathologic findings of five patients with eccrine adenocarcinoma of the eyelid with orbital invasion. MAIN OUTCOME MEASURES: Clinical and histopathologic examinations, including routine histopathology, immunohistochemistry, and electron microscopy studies. RESULTS: The tumors presented as insidious, diffusely infiltrative, firm cutaneous masses in the periocular area that later infiltrated the orbit. Histopathologic examination revealed that the tumors infiltrated the dermis and were composed of cells with a histiocytic to signet ring appearance. Tumor cells exhibited intracellular mucin production. Immunohistochemical stains were positive in tumor cells for low and high molecular weight cytokeratins, carcinoembryonic antigen, and epithelial membrane antigen. Electron microscopic examination showed lumen formation and intracytoplasmic mucin in tumor cells. CONCLUSIONS: The histiocytoid variant of adenocarcinoma of the eccrine sweat gland of the eyelid may present as an insidious tumor and diffusely invade the orbit. These cases may be confused with metastatic adenocarcinoma.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
| | Next -> |