1. Primary Intranodal Epithelioid Hemangioendothelioma with Molecular Confirmation
- Author
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Jennifer L. Vazzano, Ashley Patton, Gabriel Tinoco, and O. Hans Iwenofu
- Subjects
Adult ,Male ,Calcium-Binding Proteins ,Sarcoma ,Middle Aged ,Pathology and Forensic Medicine ,Positron Emission Tomography Computed Tomography ,Trans-Activators ,Hemangioendothelioma, Epithelioid ,Humans ,Surgery ,Anatomy ,Child ,In Situ Hybridization, Fluorescence ,Transcription Factors - Abstract
Epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular tumor with indolent biology, characterized by reciprocal t(1;3)(p36.6;q25) with resultant WWTR1::CAMTA1 gene fusion in the vast majority of cases, regardless of anatomic location. Only a small subset, exhibiting well formed vasoformative features will contain YAP1::TFE3 gene fusion. Primary intranodal EHE is exquisitely rare. We report a case in a 54-year-old male with persistent left groin mass with discomfort for nine months. A CT of the abdomen and pelvis showed a minimally enlarged left inguinal lymph node measuring 2.8 cm with no other masses or lymphadenopathy. PET/CT and MRI imaging of the abdomen showed no evidence of disease elsewhere. Sections showed an epithelioid vasoformative neoplasm, centrally necrotic and involving a lymph node. The cells were arranged in anastomosing cords with intracytoplasmic lumens, resembling “signet ring cells". By immunohistochemistry, the tumor cells were positive for vimentin, CD31, CD34, ERG and CAMTA1; and negative for AE1/3, CAM 5.2, KRT7, KRT20, desmin, actin, HMB-45 and S-100. Ki-67 proliferation index was estimated at
- Published
- 2022