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Query: UMLS:C0153690 (bone metastases)
 6,382 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary sarcomas of the aorta are extremely uncommon. Depending on histomorphology and immunohistochemical pattern, intimal sarcomas can show angiosarcomatous differentiation. Here, we describe the case of a 60-year-old woman with a primary intimal sarcoma of the aortic arch and signs of cerebral metastatic disease as the initial manifestation. After the patient experienced the onset of severe headaches, ataxia, and left-sided weakness, magnetic resonance imaging showed several brain lesions. Histologic assessment of a brain biopsy specimen revealed a malignant tumour composed of large pleomorphic cells that were positive for pancytokeratin and CD10. Radiation to the brain did not significantly improve the patient's symptoms, and cranial computed tomography (ct) imaging revealed several metastases, indicating lack of response. Because of the patient's smoking history, the presence of central nervous system and skeletal metastases on combined positron-emission tomography and ct imaging, and the focal pan-cytokeratin positivity of the tumour, carcinoma of the lung was favoured as the primary tumour. Despite chemotherapy with cisplatin and etoposide, the patient's neurologic symptoms and general condition deteriorated rapidly, and she died within a few days. At autopsy, an undifferentiated intimal sarcoma of the aortic arch was diagnosed. The primary tumour in the aorta consisted of large pleomorphic cells. Immunohistochemical analysis of the aortic tumour and brain metastases demonstrated diffuse positivity for vimentin and p53 and focal S-100 staining. In summary, we report a challenging case of advanced intimal sarcoma of the aortic arch with brain and bone metastases at initial presentation. Our report demonstrates the difficulties in diagnosing and treating this disease, and the need for multicentre studies to accrue more patients for investigations of optimal therapy.
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PMID:Uncommon case of brain metastasis in a patient with a history of heavy smoking. 2530 44

Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) is a novel entity of rare tumors with rather unique morphology and immunohistochemical profile. Until recently these tumors were characterized by indolent behavior. Herein, we describe a series of six primary and metastatic ESC-RCCs morphologically and immunophenotypically mimicking epithelioid angiomyolipoma (eAML). Retrospective review of unclassified RCCs with oncocytic phenotype yielded several candidate cases, 4 of which fulfilled diagnostic criteria after additional work-up. Three female patients and one male (median age 46) presented with unifocal tumors ranging from 1.5 cm to 20.5 cm (median 5 cm). On follow-up (median 32 months), 2 younger patients had no signs of tumor recurrence, but older patients presented with advanced disease. A 50 year-old female developed numerous bone metastases and tumor progression despite aggressive treatment. Two of these metastases were analyzed showing morphology and immunoprofile similar to the primary tumor. 50 year-old male had locally aggressive tumor invading adrenal gland and retroperitoneum. All cases exhibited ESC-like architecture of solid sheets, tight nests and variably sized cysts with hobnailed lining, as well as foci of diffuse growth with poorly cohesive brightly eosinophilic cells. Characteristic cytoplasmic stippling and coarse granularity was present in all cases including compact cytoplasmic "Leishmaniasis-like" globules. Due to high suspicion of eAML, immunostaining panels included melanocytic markers, cytokeratins and RCC-specific markers. All ESC-RCC were positive for CK20 and melanocytic markers Melan-A, Cathepsin-K or HMB45, as well as PAX8, whereas EMA, pan-cytokeratin, CK7, CKIT, CD10, CAIX were negative. Comparison with 5 eAML cases including 2 malignant tumors showed similar morphology and immune reactivity except for more frequent expression of HMB45 and lack of PAX8 positivity. In conclusion, we report 2 cases of aggressive ESC-RCC course including widespread bone metastases in addition to 2 typical indolent tumors. ESC-RCC and eAML could present with overlapping morphology and immunophenotype causing diagnostic difficulty and expanding our understanding of these rare tumors.
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PMID:Eosinophilic solid and cystic renal cell carcinoma mimicking epithelioid angiomyolipoma: series of 4 primary tumors and 2 metastases. 2988 6