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1/101. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease.

    We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease.
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2/101. Double cancers of the lung and esophagus associated with a sarcoid-like reaction in their regional lymph nodes: report of a case.

    A case of double cancers of the lung and esophagus associated with a sarcoid-like reaction in their regional lymph nodes is reported. A 73-year-old man with hemosputum was found to have a mass in his right lower lung field on a chest X-ray. Based on a diagnosis of lung cancer, a right middle and lower lobectomy with a dissection of the lymph nodes was performed. Microscopically, a well developed granulomatous reaction was seen in the dissected mediastinal and hilar lymph nodes. Three years after the pulmonary resection, he was admitted to our hospital because of dysphagia. A diagnosis of lower esophageal cancer was made. A lower esophagectomy with a total gastrectomy was performed. A sarcoid-like reaction comprising epithelioid cells and giant cells was seen in the regional lymph nodes. No clinical findings indicative of systemic sarcoidosis were observed. This rare condition may therefore help to improve our overall understanding of the relationship between malignant neoplasms and sarcoid-like reactions in the regional lymph nodes.
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3/101. Carcinoma in villous adenoma of ascending colon associated with sarcoid reaction in the regional lymph nodes.

    A 79-year-old woman was admitted to our hospital due to continuous anal bleeding. colonoscopy showed a huge villous tumor on the middle area of the ascending colon. A typical right colectomy and lymph node dissection were performed. The resected specimen showed a villous type tumor located on the ascending colon. The histopathologic investigation demonstrated a moderately differentiated adenocarcinoma arising in a tubulovillous adenoma and extending to the submucosa. Although there was no evidence of metastatic carcinoma in the dissected lymph nodes, epithelioid cell granulomas with multinucleated giant cells lacking in the central caseous necrosis suggested sarcoid reaction.
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4/101. Synchronous multicentric giant cell tumour: a case report with review of literature.

    Synchronous multicentric giant cell tumour (MGCT) is a rare occurrence. We report a young woman who presented with a synchronous skull and lower shaft femur giant cell tumour, who had previously received radiotherapy to both the sites, it being deemed inoperable at initial assessment.
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5/101. Malignant melanoma in a burn scar.

    Cancers of various sorts are occasionally encountered in burn scars. These lesions are usually squamous cell carcinomas, and the burn scars are usually old. Very rarely, malignant melanoma is encountered. An 87-year-old nursing home patient who had been burned by a lightening strike at age 16 was evaluated. She had sustained a wound covering 2% or 3% of her body surface involving her neck and the upper portion of her anterior trunk that had required several grafts. A lesion was noted over the suprasternal notch approximately 3 months before admission. The biopsy was reported as malignant melanoma. She was subsequently treated by wide reexcision with an associated Z-plasty for neck release. Because of the patient's age and the presence of four areas of regional lymph nodes nearby into which metastasis might spread, no lymph node dissections were carried out. The specimen from the reexcision was reported as squamous cell carcinoma in situ, melanoma in situ, and multinucleated giant cell reaction, acute and chronic infiltrates. The wound margins were clear.
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6/101. Giant-cell fibroblastoma and dermato fibro sarcoma protuberans: the same tumoral spectrum? Report of two cases of association in children.

    We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.
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keywords = giant
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7/101. Giant angiomyolipoma associated with marked pulmonary lesions suggesting lymphangioleiomyomatosis in a patient with tuberous sclerosis.

    The association between tuberous sclerosis (TS) and angiomyolipoma (AML) is well known. A patient with TS and giant AML mimicking Renal Cell Carcinoma (RCC), measuring 29 x 18 x 11 cm, weighing 4700 gr is presented. Imaging studies revealed coexistent pulmonary lymphangioleiomyomatosis and concurrent renal and pulmonary involvement is extremely rare in patients in TS. We believe that the growth potential of this hamartomatous lesion may reach to a life threatening size.
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8/101. Composite tumor consisting of dermatofibrosarcoma protuberans and giant cell fibroblastoma associated with intratumoral endometriosis. Report of a case.

    We present a unique case of composite skin tumor of the vulva consisting of dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) with an intratumoral focus of endometriosis. A 31-year-old female with a 10-year-history of a recurring subcutaneous tumor in the vulvar area underwent excision of the seventh recurrence of the tumor. Microscopic examination revealed a composite fibrohistiocytic tumor consisting of DFSP and GCF. Additionally, a focus of endometriosis within the tumor tissue was found. Malignant transformation of extragonadal endometriosis has already been described; we present, however, the occurrence of a focus of endometriosis within the tissue of a hormonally independent soft tissue tumor. There is a possible link to the occurrence of cutaneous endometriosis at previous surgery sites and in the scars. The presence of endometriosis within the soft tissue tumor represents, to the best of our knowledge, a previously undescribed collision phenomenon.
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keywords = giant
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9/101. Ovarian giant cell tumor with cystadenocarcinoma.

    A 25-year-old woman had a malignant ovarian tumor with two distinct tumor patterns: pseudomucinous cystadenocarcinoma and malignant giant cell tumor of bone. One area of the tumor had a lining of tall columnar epithelium with abundant mucin production; however, another area of the same tumor had a number of large multinucleated giant cells that resembled osteoclasts, intermingled with mononuclear stromal cells. There was no blending of the two tumor patterns. Mitoses were frequent in both areas. A probable histogenesis of the tumor from a malignant teratoma is suggested.
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keywords = giant
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10/101. Giant cell carcinoma of the liver: occurrence in a patient with ileal carcinoid, medullary breast carcinoma and pulmonary aspergillosis.

    Pure giant cell carcinoma of the liver is a rare tumor that is usually associated with cirrhosis. Its occurrence in a young woman without evidence of cirrhosis, and in association with two other uncommon tumors, is described in this report.
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