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MYC Analysis by Fluorescent In Situ Hybridization and Immunohistochemistry in Primary Adrenal Angiosarcoma (PAA): a Series of Four Cases

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Abstract

Primary adrenal angiosarcomas (PAA) are rare with 36 cases reported in the English literature. MYC protein expression and gene amplification have been detected in secondary angiosarcoma (AS), and a subset of primary AS. The aim of this study was to report the clinicopathologic features of PAA and examine these tumors for MYC amplification and protein expression in a small series of four cases (resection, n = 4). Three had available material for ancillary studies and were investigated for MYC gene abnormalities and protein expression using fluorescent in situ hybridization (FISH) and immunohistochemistry (IHC), respectively. Tumors occurred in three females and one male with a mean age of 69 (53–75) years. The sizes ranged from 8.5 to 15 (mean 11.5) cm and were epithelioid in morphology. All tumors had prominent necrosis, and the mitotic count ranged from 4 to 41/10 high-power fields (HPFs) (mean 20/10 HPFs, ×400). Immunohistochemically, the tumor cells were positive for CD31 in 4/4 cases, CD34 in 1/4 cases, and cytokeratin in 4/4 cases. The mean follow-up period was 10.8 (3–19) months, of which three patients died of disease with distant metastases, and one patient was alive with disease. MYC nuclear staining was identified in the three cases tested. Two cases showed polysomy of chromosome 8 without MYC amplification or rearrangement. Two MYC-positive cases by IHC demonstrated copy number gain in chromosome 8, and one MYC-positive case was not associated with a chromosome 8/MYC gene abnormality. In the context of new targeted therapies, MYC positivity in PAA may be clinically valuable in treating patients with these aggressive neoplasms.

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Acknowledgments

The authors would like to thank Sharon Campbell from the Department of Pathology at the Massachusetts General Hospital and Karen Dresser from the Department of Pathology at the University of Massachusetts Medical School for their technical expertise in performing the immunohistochemical stains.

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The authors declare that they have no conflicts of interest.

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Authors and Affiliations

  1. Department of Pathology, UMass Memorial Healthcare—University of Massachusetts Medical School, One Innovation Drive, Biotech 3, Worcester, MA, 01605, USA

    Kristine M. Cornejo, Lloyd Hutchinson & Maryann St. Cyr

  2. Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Kristine M. Cornejo, Vania Nose, Patrick J. McLaughlin, A. John Iafrate & Peter M. Sadow

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  1. Kristine M. Cornejo
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  2. Lloyd Hutchinson
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Correspondence to Kristine M. Cornejo.

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Kristine M. Cornejo holds a MD; Lloyd Hutchinson holds a PhD; Maryann St. Cyr holds a MT (ASCP); Vania Nose holds a MD and PhD; Patrick J. McLaughlin holds a MT and MLS (ASCP); A. John Iafrate holds a MD and PhD; Peter M. Sadow holds a MD and PhD.

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Cornejo, K.M., Hutchinson, L., Cyr, M.S. et al. MYC Analysis by Fluorescent In Situ Hybridization and Immunohistochemistry in Primary Adrenal Angiosarcoma (PAA): a Series of Four Cases. Endocr Pathol 26, 334–341 (2015). https://doi.org/10.1007/s12022-015-9385-4

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