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1/204. Epithelioid cell histiocytoma: a simulant of vascular and melanocytic neoplasms.

    Epithelioid cell histiocytoma (ECH) is an unusual and still poorly recognized variant of benign fibrous histiocytoma. Epithelioid cell histiocytoma differs from most benign fibrous histiocytomas in five important ways: the predominance of epithelioid cells, relative lack of secondary elements (such as giant cells, foamy, or hemosiderin-laden macrophages), relative sharp circumscription, prominent vascularity, and centering in the papillary dermis in most cases. A strong resemblance to melanocytic and vascular lesions has been noted, and a recent case was reported with features suggesting endothelial origin. Fifteen new cases of ECH, including one example of the rare deep cellular variant, are presented herein, with emphasis on features mimicking vascular and melanocytic neoplasms. Labeling with endothelial markers, including previously unreported CD-31 labeling, showed abundant vascular staining, which may be challenging to interpret, but which does not indicate an endothelial origin of ECH.
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keywords = neoplasm
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2/204. Malignant fibrous histiocytoma of the maxilla: a case report.

    Malignant fibrous histiocytoma is one of the most common sarcomas of soft tissues in late adult life. However, this neoplasm rarely arises in the oral and maxillofacial region with the maxilla presenting as an unusual site. A case report of a 40-year old woman with malignant fibrous histiocytoma involving the maxilla is discussed with relevant literature reviewed. The treatment chosen was partial maxillectomy. The patient was referred for cobalt therapy after surgery. Eight months after diagnosis, the patient died of the illness.
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3/204. Pleomorphic hyalinized angiectatic tumor of soft parts.

    Pleomorphic hyalinized angiectatic tumor of soft parts is a recently described neoplasm that most commonly affects the lower extremities. It is locally aggressive but has not been known to metastasize. This article presents a case of a softball-sized tumor on the dorsum of the foot that was identified as pleomorphic hyalinized angiectatic tumor of soft parts. Previously, it would have been misdiagnosed and treated as either a neurilemoma or a malignant fibrous histiocytoma.
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keywords = neoplasm
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4/204. Intrahepatic cholangiocarcinoma with extensive sarcomatous change: report of a case.

    A 77-year-old woman was admitted to our hospital with severe upper abdominal pain. ultrasonography showed a well-defined hypoechoic mass with heterogeneity in the left lobe of the liver, and computed tomography demonstrated a low-density mass with enhanced peripheral areas. magnetic resonance imaging revealed a mass with iso- to low signal intensity on T1-weighted images (WI) and heterogeneous high and low signal intensity on T2 WI. The tumor was found to be hypovascular by angiography. During 5 months of observation, the tumor increased in size, which strongly suggested malignancy. A laparotomy was performed under the provisional diagnosis of a neoplasm other than hepatocellular carcinoma, revealing that the hepatic mass had invaded the gastric wall. Therefore, a left hepatic lobectomy with dissection of the lymph nodes and hemigastrectomy was carried out. Histologically, the tumor was found to be composed of a large amount of sarcomatous elements and a small amount of adenocarcinomatous elements, both of which were partly intermingled. Immunohistochemically, the sarcomatous element demonstrated the features of malignant fibrous histiocytoma (MFH). Thus, a diagnosis of intrahepatic cholangiocarcinoma with MFH-like sarcomatous change was confirmed.
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keywords = neoplasm
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5/204. Cardiac autotransplant for surgical treatment of a malignant neoplasm.

    Because of their anatomic location, cardiac sarcomas often interfere with cardiac function. Excision is considered to palliate the cardiac defect, but complete excision is often difficult owing to access, particularly in left atrial tumors. Incomplete resection results in tumor recurrence. To achieve complete resection of a large left atrial sarcoma, we used the technique of cardiac explantation, extracorporeal resection of the tumor with cardiac reconstruction, and cardiac autotransplantation.
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ranking = 0.8
keywords = neoplasm
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6/204. Two unusual tumors in a patient with xeroderma pigmentosum: atypical fibroxanthoma and basosquamous carcinoma.

    xeroderma pigmentosum (XP) is a rare autosomal recessive disease, characterized by a genetic defect in dna repair. The consequence is a high incidence of skin cancers on sun-exposed cutaneous surfaces of affected children. First lesions appear in the first years of life: telangiectasia, actinic keratosis and keratoacanthomas. Squamous cell and basal cell carcinomas are the most frequent neoplasms. We report the case of a 6-year-old girl affected with XP, who developed two unusual tumors: an atypical fibroxanthoma and a basosquamous carcinoma. In both tumors, immunohistochemical study showed abnormal accumulation of the p53 protein, suggesting the presence of mutation of the p53 tumor suppressor gene. Such p53 mutations may be ultraviolet (UV)-induced, as they are frequently observed in tumors occurring in XP.
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7/204. Primary malignant fibrous histiocytoma of the duodenum.

    BACKGROUND/AIM: We report on a case of primary malignant fibrous histiocytoma of the duodenum. methods: Report of a case and review of the literature on the occurrence of this tumor in the alimentary tract. RESULTS: The neoplasm was detected incidentally at the time of diagnostic workup for upper gastrointestinal bleeding. Curative surgery using the Whipple procedure was carried out with uneventful postoperative course; no evidence of liver metastases was detected during intraoperative ultrasonography. The patient died of diffuse metastatic liver disease 2 months after surgical resection. Up-to-date review of the literature has added only 3 cases to those previously reported, including that reported by us. CONCLUSIONS: The biological behavior of malignat fibrous histiocytomas is extremely aggressive and mainly conditioned by size and histological grading. The treatment of choice, whenever possible, is based on early and complete surgical excision of the tumor. copyright copyright 1999 S. Karger AG, Basel
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keywords = neoplasm
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8/204. Combined intraepithelial squamous neoplasia and atypical fibroxanthoma of the cornea and limbus.

    PURPOSE: To investigate a case of an unusual neoplasm of the cornea and limbus. methods: A 59-year-old man presented with a highly vascularized, nodular mass involving the left cornea and limbus. An excisional biopsy and, subsequently, a superficial lamellar keratectomy and multiple conjunctival biopsies were performed. At the 6-month follow-up examination, repeat conjunctival biopsies were performed. RESULTS: Histopathologic examination of the corneal specimen showed a high-grade intraepithelial squamous neoplasia (in situ carcinoma) overlying an atypical fibroxanthoma. CONCLUSION: We report the clinical and histologic appearance of a corneal/limbal neoplasm consisting of an intraepithelial squamous neoplasia and an atypical fibroxanthoma.
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keywords = neoplasm
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9/204. Fibroxanthoma arising from the cranial dura mater.

    A primary xanthomatous tumor is very rare in the central nervous system (CNS). Here we report the case of a fibroxanthoma arising from the dura mater of the cerebrum that demonstrated no systemic disease or metabolic abnormalities. A 19-month-old, otherwise healthy boy was found to have an enlarged head. Magnetic resonance imaging demonstrated a left occipital dural mass lesion and an enlarged left cerebral hemisphere with ipsilateral ventricular enlargement. Subtotal removal of the tumor was performed through the left parieto-occipital craniotomy. The tumor was composed of a central fibrous portion, a peripheral xanthomatous area, and a boundary. The peripheral area of the tumor showed abundant uniform xanthomatous cells with a thin fibrous stroma and the mass was diagnosed as fibroxanthoma involving the dura. This may represent a distinct category of tumor, which is different from the previously reported cases of fibrous xanthoma and fibrous histiocytoma. Intracranial xanthomatous tumors may be heterogeneous in their origin and histological features. However, further studies are needed to elucidate their clinical features, biological behavior, and optimal treatment strategies.
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ranking = 0.0098052174155894
keywords = central nervous system, nervous system
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10/204. Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma.

    neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.
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ranking = 0.2
keywords = neoplasm
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