1,721,316 research outputs found

    Intraductal carcinoma of mammary-type apocrine epithelium arising within a papillary hydradenoma of the vulva. Report of a case and review of the literature

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    We report and immunohistochemically document the first (to the best of our knowledge) case of malignancy in which an intraductal carcinoma resembling apocrine breast cancer arose within a papillary hidradenoma of the vulva. Papillary hidradenoma is generally thought to originate from apocrine sweat glands, but a derivation from milk line remnants of the vulva should also be considered. Immunoreactivities for low- and high-molecular-weight cytokeratins, alpha-smooth-muscle-specific actin, carcinoembryonic antigen, S100 protein, and gross cytic disease fluid protein 15, an antigen of apocrine differentiation, show features that resemble those of an intraductal apocrine breast cancer. Positivity for gross cystic disease fluid protein 15 as well as the presence of estrogen and progesterone receptors suggest that tumor cells are controlled by ovarian steroid hormones. To our knowledge, no cases of malignancy arising from a papillary hidradenoma have been proved to date. Therefore, we also discuss previously reported cases of putative cancers that have developed in papillary hidradenomas. In the case presented herein, a local excision with a narrow rim of surrounding tissue was performed, and the patient was alive and well, without signs of recurrence, after 2 years of follow-up

    Renal cell carcinoma with clear cell and papillary features

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    CONTEXT: The diagnosis of primary renal cell carcinomas (RCCs) with both papillary architecture and cells with clear cytoplasm can be diagnostically challenging for practicing pathologists. The 4 main neoplasms in the differential diagnosis are clear cell RCC, papillary RCC, clear cell papillary RCC, and Xp11 translocation RCC. Accurate diagnosis has both prognostic and therapeutic implications. OBJECTIVE: To highlight the helpful cytomorphologic, immunohistochemical, and cytogenetic features of each of these entities to enable reproducible classification. DATA SOURCES: Published peer-reviewed literature was reviewed, accompanied by the authors' personal experiences. CONCLUSIONS: Key morphologic clues and a focused immunohistochemical panel, including CK7, α-methylacyl coenzyme A racemase (AMACR), TFE3, cathepsin K, and carbonic anhydrase IX (CAIX), now allow most resected RCCs with papillary architecture and clear cells to be accurately classified. In other cases, cytogenetic and molecular findings can establish the diagnosis. Despite these tools, some RCCs with papillary architecture and clear cells do not fit into any of the described entities and currently remain unclassified

    Angiomyolipoma/PEComa: the past, the present...and back to the future

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    Purpose of review: This review provides a comprehensive update on renal angiomyolipoma, a mesenchymal neoplasm within the PEComa family. It revisits its historical classification, evolving pathological understanding, and recent molecular insights, emphasizing its relevance in current diagnostic and pathogenetic contexts. Recent findings: Angiomyolipoma/PEComa has transitioned from being considered a hamartoma to a neoplasm with clonal origin and malignant potential in a few epithelioid angiomyolipoma/pure epithelioid PEComa. Advances in immunohistochemistry have identified new markers such as GPNMB, STING, and TRIM63, aiding differential diagnosis. Molecular studies highlight frequent TSC1/TSC2 mutations and mTOR pathway dysregulation. Emerging evidence suggests a noncanonical activation of TFEB via the cGAS-STING pathway in angiomyolipoma/PEComa. Summary: A thorough understanding of the histological subtypes and molecular drivers of angiomyolipoma/PEComa is essential for accurate diagnosis and risk stratification. However, the comprehension of its pathophysiological mechanisms remains not completely understood. The cGAS-STING-TFEB axis may explain the unique immunophenotype of the cellular element composing these neoplasms. Furthermore, this pathway could also be related to the unexpected presence of autophagy observed in angiomyolipoma/PEComa, with STING representing the missing piece in this intricate puzzle. Nevertheless, this proposed mechanism requires validation through further research

    WHO 2022 Classification of Kidney Tumors: what is relevant? An update and future novelties for the pathologist

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    : Classification systems reflect our technical abilities in the investigation of tumors and our current theories on tumor development. Herein, by providing a historical perspective on the evolution of classifying renal tumors, we assess the current WHO classification highlighting the novelties and the implications of these changes in daily clinical practice

    Cellular heterogeneity in lymphangiomyomatosis of the lung

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    The manuscript is a commentary and criticisms to the cellular heterogeneity in lymphangiomyomatosis of the lung reported in orginal papers
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