In this article, we describe a case of conventional epithelioid hemangioendothelioma (EHE) arising within an extra-adrenal myelolipoma. This composite neoplasm arose in the mediastinum of a 51-year-old female. The tumor was composed of a large myelolipoma that contained nodules of EHE consisting of CD31-positive epithelioid endothelial cells that grew in solid cords and were enmeshed in a basophilic hyalinized stroma. Both EHE and myelolipoma are characterized genetically by alterations of WWTR1. We demonstrated the expression of CAMTA-1 chimeric protein by immunohistochemistry both in the neoplastic endothelial cells of EHE and some of the endothelial cells lining the blood vessels in the myelolipoma. To the best of our knowledge, this is the first report of a malignant vascular neoplasm arising in association with myelolipoma.
SungCTShettyAMeniasCOet al. Collision and composite tumors; radiologic and pathologic correlation. Abdom Radiol (NY). 2017;42:2909-2926.
2.
ShihALauwersGYBalabaudCBioulac-SagePMisdrajiJ.Simultaneous occurrence of focal nodular hyperplasia and HNF1A-inactivated hepatocellular adenoma: a collision tumor simulating a composite FNH-HCA. Am J Surg Pathol. 2015;39:1296-1300.
3.
LamAKChanSSLeungM.Synchronous colorectal cancer: clinical, pathological and molecular implications. World J Gastroenterol. 2014;20:6815-6820.
4.
TanasMRMaSJadaanFOet al. Mechanism of action of a WWTR1(TAZ)-CAMTA1 fusion oncoprotein. Oncogene. 2016;35:929-938.
5.
FluckeUVogelsRJde Saint Aubain SomerhausenNet al. Epithelioid hemangioendothelioma: clinicopathologic, immunohistochemical, and molecular genetic analysis of 39 cases. Diagn Pathol. 2014;9:131.
6.
NollABooneJCunninghamMMammenJTawfikO.Low grade B cell lymphoma arising in a background of multifocal extra-adrenal myelolipoma. Ann Clin Lab Sci. 2013;43:441-446.
7.
LiuDKumarSA.An exceedingly rare adrenal collision tumor: adrenal adenoma-metastatic breast cancer-myelolipoma. J Community Hosp Intern Med Perspect. 2017;7:241-244.
8.
TimoneraERPaivaMELopesJMEloyCvan der KwastTAsaSL.Composite adenomatoid tumor and myelolipoma of adrenal gland: report of 2 cases. Arch Pathol Lab Med. 2008;132:265-267.
9.
DuregonEVolanteMGuzzettiSRapaIVatranoSPapottiM.Images in endocrine pathology: unique composite adrenal adenomatoid tumor, ganglioneuroma, myelolipoma, and cortical nodular hyperplasia. Endocr Pathol. 2017;28:276-279.
10.
SeniutaPCazenave-MaheJPLe TreutATrojaniM.Adrenal myelolipoma and Castleman’s pseudotumor. A case of association in a retroperitoneal tumor [in French]. J Urol (Paris). 1989;95:511-514.
11.
ChangKCChenPIHuangZHLinYMKuoPL.Adrenal myelolipoma with translocation (3;21)(q25;p11). Cancer Genet Cytogenet. 2002;134:77-80.
12.
ErraniCZhangLSungYSet al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosomes Cancer. 2011;50:644-653.
13.
CappelloFFarinaFDi FeliceVet al. Defective apoptosis as potential mechanism in the tumorogenesis of myelolipoma. Eur J Histochem. 1999;43:15-18.
14.
AntonescuCRLe LoarerFMosqueraJMet al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosomes Cancer. 2013;52:775-784.
15.
ShiQPanSBaoYFanHDiaoY.Primary mediastinal myelolipoma: a case report and literature review. J Thorac Dis. 2017;9:E219-E225.
16.
MehtaCRapariaKBharatA.Anterior mediastinal myelolipoma. Ann Thorac Surg. 2017;103:e81.
17.
KaplanSRadiceJC.Myelolipoma clavicle. Prensa Med Argent. 1950;37:139-141.
18.
YildizBD.Giant extra-adrenal retroperitoneal myelolipoma with incidental gastric mesenchymal neoplasias. Int Surg. 2015;100:1018-1020.
19.
HisamatsuHSakaiHTsudaSShigematsuKKanetakeH.Combined adrenal adenoma and myelolipoma in a patient with Cushing’s syndrome: case report and review of the literature. Int J Urol. 2004;11:416-418.
20.
FondaPde SantiagoEGuijarroMGamalloC.Mediastinal myelolipoma with leukocytosis. BMJ Case Rep. 2013;2013:bcr2013010349.
21.
BishopEEbleJNChengLet al. Adrenal myelolipomas show nonrandom X-chromosome inactivation in hematopoietic elements and fat: support for a clonal origin of myelolipomas. Am J Surg Pathol. 2006;30:838-843.
22.
FowlerMRWilliamsRBAlbaJMByrdCR.Extra-adrenal myelolipomas compared with extramedullary hematopoietic tumors: a case of presacral myelolipoma. Am J Surg Pathol. 1982;6:363-374.
23.
ZattoniDBalzarottiRRossoR.The management of bilateral myelolipoma: case report and review of the literature. Int J Surg Case Rep. 2015;12:31-36.
24.
BurrowsSDrakeWMJrSingleyTL.Large retroperitoneal myelolipoma associated with acute myelogenous leukemia. Am J Clin Pathol. 1969;52:733-736.
25.
HagspielKD.Manifestation of Hodgkin’s lymphoma in an adrenal myelolipoma. Eur Radiol. 2005;15:1757-1759.
26.
GheithSBoulayRCornfieldD.Small lymphocytic lymphoma/chronic lymphocytic leukemia in a pelvic myelolipoma. Int J Clin Exp Pathol. 2009;2:95-98.
27.
WeissSWEnzingerFM.Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50:970-981.
28.
WeissferdtAMoranCA.Epithelioid hemangioendothelioma of the bone: a review and update. Adv Anat Pathol. 2014;21:254-259.
29.
DeyrupATTighiouartMMontagAGWeissSW.Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol. 2008;32:924-927.
30.
AroraKSidhuJ.Extra-adrenal myelolipoma containing small lymphocytic lymphoma/chronic lymphocytic leukemia: a case report and review of the literature. Case Rep Hematol. 2016;2016:7364951.
31.
BraletMPTerrisBVilgrainVet al. Epithelioid hemangioendothelioma, multiple focal nodular hyperplasias, and cavernous hemangiomas of the liver. Arch Pathol Lab Med. 1999;123:846-849.
32.
TakahashiKNishimuraM.A unique collision of epithelioid hemangioendothelioma and bronchioloalveolar carcinoma in a 54-year-old Japanese woman. Jpn J Thorac Cardiovasc Surg. 2003;51:311-313.
