Immunohistochemical Biomarkers of Mesenchymal Neoplasms in Endocrine Organs: Diagnostic Pitfalls and Recent Discoveries
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Immunohistochemical Biomarkers of Mesenchymal Neoplasms in Endocrine Organs: Diagnostic Pitfalls and Recent Discoveries Yin P. Hung 1 & Jason L. Hornick 1
# Springer Science+Business Media, LLC, part of Springer Nature 2018
Abstract Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. In this review, we address potential pitfalls in diagnosing selected mesenchymal neoplasms arising within or near endocrine organs, including dedifferentiated liposarcoma, synovial sarcoma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma. For each of these tumor types, we review clinical and pathologic features, histologic clues to distinguish them from endocrine neoplasms, and recently developed immunohistochemical markers that can be particularly useful for establishing the correct diagnosis. Keywords Sarcoma . Immunohistochemistry . Dedifferentiated liposarcoma . Synovial sarcoma . Angiosarcoma . PEComa . Epithelioid sarcoma . Ewing sarcoma . Neuroblastoma
Introduction Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. Given the remarkable clinicopathologic and molecular diversity of mesenchymal tumors [1], an exhaustive overview of mesenchymal tumors that can involve endocrine organs either as a primary tumor or metastasis is beyond the scope of this review. Here, we will instead focus on relatively common pitfalls in diagnosing mesenchymal tumors in or near endocrine organs. We will focus on selected examples with spindle cell, epithelioid, and round cell morphology, including ded ifferentiated liposarc oma, synov ia l sarc oma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma. For each of these seven
* Jason L. Hornick [email protected] 1
Department of Pathology, Brigham and Women’s Hospital and Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA
tumor types, we will briefly review the clinicopathologic and genetic features, histologic clues to distinguish them from endocrine neoplasms, and recently developed immunohistochemical markers that can be particularly useful for establishing the correct diagnosis.
Dedifferentiated Liposarcoma Dedifferentiated liposarcoma, first introduced by Evans in 1979 [2], represents the non-lipogenic form of well-differentiated liposarcoma/atypical lipomatous tumor. Dedifferentiated liposarcoma typically presents in adulthood, with the most common
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