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1/144. Malignant melanoma of the choroid in neurofibromatosis.

    A 60-year-old white woman with generalized neurofibromatosis and multiple melanocytic hamartomas of the iris developed an unusual choroidal mass, with secondary sensory retinal separation in the left eye. Ophthalmoscopically the tumor had a peculiar donut configuration that was caused by a large focus of central necrosis within a spindle B melanoma.
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2/144. A case of synchronous double primary lung cancer with neuroendocrine features.

    We report a case of unique double primary lung cancers with neuroendocrine features in a 63-year-old male smoker. The mass in the left lower lobe (LLL) was a small cell/large cell carcinoma with spindle cell sarcomatous areas and organoid structure. The mass in the left upper lobe (LUL) was a tubular adenocarcinoma with neuroendocrine features including organoid nests showing occasional rosette formation, nuclear palisading in the periphery of the nests and positive immunoreaction for CD56, chromogranin a and synaptophysin. The difference in histological structures between the two masses led us to diagnose double primary lung cancer. The combination of small cell lung carcinoma and spindle cell carcinoma is very uncommon. The relationship between LLL and LUL tumors remains unclear. Multiple lung cancers with neuroendocrine features have only rarely been reported in the literature. The patient in our case died of widespread cancer 2 years and 4 months after the surgery without adjuvant chemotherapy, a longer postoperative survival time than in cases of ordinary extensive small cell lung cancer. Multiple lung cancers with neuroendocrine features are extremely rare and similar cases have not been reported in the literature. Neuroendocrine differentiation has attracted widespread attention and, therefore, examining neuroendocrine features in lung cancers is important.
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ranking = 7.4054233790372
keywords = spindle cell, spindle
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3/144. Primary smooth muscle tumor of the liver encasing hepatobiliary cystadenoma without mesenchymal stroma.

    We describe a 59-year-old Japanese woman with a large mass of her liver encasing cystic components. Radiologic imaging showed the mass to be hypervascular, and surgical resection disclosed a white tumor. The solid portion was immunohistochemically characterized as a smooth muscle tumor. The cystic components were multilocular and lined with columnar epithelium, consistent with a hepatobiliary cystadenoma. The epithelium strongly stained for CA19-9. The subepithelial space was occupied by collagenous connective tissue interspersed with a small number of spindle-shaped cells. The cystic lesions lacked the mesenchymal stroma between the epithelium and connective tissue layer. There have been no previous reports of a hepatic smooth muscle tumor encasing a hepatobiliary cystadenoma. Because of the pathogenesis of the cystadenoma, it is possible to assume that the smooth muscle tumor also arose from the cells composing the biliary duct in association with the development of the cystadenoma.
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4/144. Multifocal intraocular malignant melanoma: report of two cases and review of the literature.

    PURPOSE: To describe two eyes from two patients with multifocal primary intraocular melanoma. DESIGN: Two case reports. methods: The history and histologic findings in the enucleated eyes of two patients with multifocal intraocular melanoma are described in comparison to previously reported cases. MAIN OUTCOME MEASURES: Pathologic examination of enucleated eyes. RESULTS: One of the two eyes contained mixed cell type melanomas, and one eye contained spindle cell type melanomas. Examination of serial sections showed no continuity between the intraocular melanomas. There were no associated ocular or systemic conditions with the multifocal intraocular melanomas. CONCLUSIONS: Multifocal primary intraocular melanoma is rare. There is no known predisposing factor to this condition.
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ranking = 3.7027116895186
keywords = spindle cell, spindle
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5/144. Combined nevi of the conjunctiva.

    OBJECTIVE: To report the clinical and histologic features of combined nevi of the conjunctiva, a type of nevus that is not uncommon in the skin but rarely has been reported in the conjunctiva. methods: Conjunctival nevi and melanomas in the files of the University of california at san francisco eye pathology Laboratory from 1984 to 1999 were reviewed for the presence of features of both standard nevocytic nevi and blue nevi. Clinical histories and, when available, clinical photographs were obtained. RESULTS: Thirty combined nevi were discovered between 1984 and 1999. One case before 1984 had been incorrectly diagnosed as a junctional nevus. The dendritic and spindle-shaped blue nevus cells had been overlooked because they were not recognized as distinct from the standard nevocytic nevus cells. The recognition of blue as well as a brown color, a deep as well as a superficial component in the lesion, or a history of pigmentation since birth may help to establish the correct clinical diagnosis and prevent an unnecessarily deep surgical resection. Although growth of the lesion or "satellites" in some may favor a clinical diagnosis of melanoma, no lesions in this series were malignant. CONCLUSION: Despite a paucity of reports of combined nevi of the conjunctiva in the medical literature, this type of nevus, a combination of a nevocytic and a blue nevus, is common and has been overlooked in the past.
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6/144. Double cancer - hepatocellular carcinoma and intrahepatic cholangiocarcinoma with a spindle-cell variant.

    Intrahepatic cholangiocarcinoma (ICC) with a spindle-cell variant is very rare. We report here a surgical patient who had double cancer - hepatocellular carcinoma (HCC) and ICC with a spindle-cell variant. In this 70-year-old man, who had a history of hepatic resection for HCC about 2 years previously, two large discrete masses were identified in the right lobe of the liver. A right lobectomy of the liver was performed. Pathological findings revealed that one tumor was a typical HCC, and the other was ICC with sarcomatous lesions. Immunohistochemical examinations of the sarcomatous lesions in ICC demonstrated that some of the spindle cells were positive for keratin, epithelial membrane antigen, and vimentin, but negative for S-100 protein, desmin, and actin. From these findings, we concluded that the sarcomatous lesions of ICC were not a true sarcoma, but sarcomatous transformation of cholangiocarcinoma cells, that is, a spindle-cell variant of ICC.
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ranking = 10.702711689519
keywords = spindle cell, spindle
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7/144. Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin.

    The authors report a case of sinonasal teratocarcinosarcoma in a 37-year-old man, which was located in the anterior skull base and extended to the right nasal cavity and paranasal sinuses. The tumor was surgically resected twice, but it could not be removed completely. Microscopically, it was mainly composed of primitive cell nests within a moderately cellular stroma. The components of squamous cell epithelia with focal teratoid appearance and adenocarcinomatous differentiation were observed. There were many rhabdomyoblasts scattered in the nests and stroma. Ultrastructurally, the primitive cells had many neural processes with parallel microtubules, resembling olfactory neuroblastoma. Rhabdomyoblasts showed various degrees of skeletal muscle differentiation. Some of the stromal spindle cells had actin filaments with dense patches and dense core granules. Immunohistochemically, the primitive cells were positive for epithelial markers, neuron-specific enolase, synaptophysin, and myogenic regulatory proteins. The rhabdomyoblasts showed immunoreactivity for myoid markers, cytokeratin, epithelial membrane antigen, and synaptophysin. Most of the stromal spindle cells were positive for smooth muscle actin, neuron-specific enolase and synaptophysin. The immunohistochemical and ultrastructural findings suggest that primitive cells had the most primitive phenotype of placodes, and support the possibility that sinonasal teratocarcinosarcoma is essentially a neuroectodermal tumor with divergent differentiation.
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ranking = 7.4054233790372
keywords = spindle cell, spindle
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8/144. Oncocytoma in melanocytoma of the spinal cord: case report.

    OBJECTIVE AND IMPORTANCE: Oncocytoma in the central nervous system is extremely unusual. The first reported example of oncocytoma in a melanocytoma of the spinal cord was successfully excised, and its pathological appearance is described. CLINICAL PRESENTATION: A 71-year-old woman presented with a 25-year history of back pain and myelographic evidence of a lumbar spinal cord mass. After declining surgical treatment for two decades, she elected eventually to have the mass excised. Preoperative magnetic resonance imaging revealed a large intraspinal mass that spanned spinal levels L3 through S1. TECHNIQUE: The mass was excised en bloc through posterior laminectomies, and histopathological analysis revealed a benign neoplasm composed predominantly of monotonous sheets of plump oncocytes. Electron microscopy confirmed that the cytoplasm of the oncocytes was packed full of mitochondria. Focal areas of the tumor contained spindle cells, with abundant intracytoplasmic granular deposits of brown melanin pigment that contained melanosomes. Positive Fontana-Masson, HMB-45, and S-100 staining confirmed the final diagnosis of melanocytoma, oncocytic variant. CONCLUSION: The first reported case of oncocytoma arising in spinal melanocytoma is described. After surgical excision, the patient recovered completely and has remained free of symptoms for 4 years.
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ranking = 3.7027116895186
keywords = spindle cell, spindle
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9/144. Selected case from the Arkadi M. Rywlin International pathology Slide Seminar: desmoplastic fibroblastoma (collagenous fibroma).

    Desmoplastic fibroblastoma (collagenous fibroma) is a rare and recently described benign soft tissue tumor. It is more common in men, median age is 50 years, the history is usually long, and the usual size is around 3 cm. Most lesions are subcutaneous but approximately 25% involve skeletal muscle. Histologically, they are sparsely cellular with stellate and spindle-shaped fibroblasts-myofibroblasts that are separated by a collagenous or myxocollagenous matrix. Mitoses are minimal or absent. Tumor cells are focally positive for muscle-specific and alpha smooth muscle actins, and rarely, for keratins. The S-100 is usually negative but a very few cases have been positive. It can be confused with one of the fibromatoses, myxomas of various kinds and neural tumors. It neither recurs nor metastasizes.
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10/144. Thymic carcinosarcoma consisting of squamous cell carcinomatous and embryonal rhabdomyosarcomatous components. Report of a case and review of the literature.

    A case of thymic carcinosarcoma in an 83-year-old Japanese man is presented. He died of superior vena cava syndrome caused by a rapidly enlarged anterior mediastinum tumor eight months after initial symptoms. autopsy revealed a 16 x 12 x 25 cm-sized, tan yellow, whitish tumor with a multinodular and microcystic appearance located in the left anterior mediastinum, which involved the residual thymus. The tumor had directly invaded the left pleura, and had metastasized to the right lung and spleen. Histologic examinations of the primary tumor showed a sarcomatous component consisting of racquet- or spindle-shaped cells with cross striations, and small nests of atypical squamous cells scattered throughout the tumor; neither transition between the two components nor intermediate cells with both epithelial and mesenchymal features was seen. Electron microscopic and immunohistochemical examinations confirmed the rhabdomyomatous differentiation of the sarcomatoid component. To our knowledge, there have been only two reported cases showing histologic features similar to the present tumor. For the histogenesis of thymic carcinosarcoma, we propose two hypotheses. The first is that sarcomatous cells are derived from carcinomatous cells by tumoral metaplasia. Secondly, that this type of tumor originates from thymic primitive cells with multidirectional differentiation potential. In accordance with the latter, we consider that the present tumor originated from thymic primitive cells. Thymic carcinosarcoma is a highly malignant tumor, and most patients die within a year. Appropriate therapies must be developed.
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