Epithelioid Hemangioendothelioma in the Area of the Neurovascular Bundle of the Upper Arm Mimicking a Schwannoma of the Ulnar Nerve

Abstract

Epithelioid hemangioendothelioma (EH) is a rare vascular malignant sarcoma. To date, there are only two published case reports of EH mimicking nerve sheath tumors of a peripheral nerve. A 41-year-old woman presented with a painful node on the inside of the upper arm and motor deficits of the ulnar nerve. Magnetic resonance imaging (MRI) follow-up and neurosonography revealed a slowly progressive, contrast-enhancing tumor in the area of the neurovascular bundle. A schwannoma of the ulnar nerve was suspected. At surgery, the tumor was adherent to the brachial artery, but not to nerves. Pathology revealed an EH. EH may mimic a nerve sheath tumor and should be taken into consideration, especially because of its potential for metastasis and fatal progression.

Keywords

epithelioid hemangioendothelioma - peripheral nerve - vascular neoplasm

Patient Consent

The patient has consented to the submission of the case report for submission to the journal.

Publikationsverlauf

Eingereicht: 23. Juni 2020

Angenommen: 22. September 2020

Artikel online veröffentlicht:
12. April 2021

• References

• 1 Süß P, Volz F, Lang C. et al. A case of large meningeal epitheloid hemangioendothelioma with WWTR1-CAMTA1 gene rearrangement and slow growth over 15 years. J Neuropathol Exp Neurol 2018; 77 (10) 871-876
• 2 Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 1982; 50 (05) 970-981
• 3 Kelahan LC, Sandhu FA, Sayah A. Multifocal hemangioendothelioma of the lumbar spine and response to surgical resection and radiation. Spine J 2015; 15 (11) e49-e56
• 4 Deyrup AT, Tighiouart M, Montag AG, Weiss SW. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol 2008; 32 (06) 924-927
• 5 Kerry G, Marx O, Kraus D. et al. Multifocal epithelioid hemangioendothelioma derived from the spine region: case report and literature review. Case Rep Oncol 2012; 5 (01) 91-98
• 6 Kopniczky Z, Tsimpas A, Lawson DD. et al. Epithelioid hemangioendothelioma of the spine: report of two cases and review of the literature. Br J Neurosurg 2008; 22 (06) 793-797
• 7 McGinity M, Bartanusz V, Dengler B, Birnbaum L, Henry J. Pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, fibroma-like variant of epithelioid sarcoma) of the thoracic spine. Eur Spine J 2013; 22 (Suppl. 03) S506-S511
• 8 Nuthakki S, Fessell D, Lal N, Shirkhoda A, Irwin T, Irwin R. Epithelioid hemangioendothelioma mimicking a nerve sheath tumor clinically and on MR imaging. Skeletal Radiol 2007; 36 (Suppl. 01) S58-S62
• 9 Zingale A, Bruno G, Giuffrè F, Carpinteri M, Albanese V. Hemangioendothelioma mimicking a median nerve neoplasm. Case report. J Neurosurg Sci 1993; 37 (02) 119-122
• 10 Kransdorf MJ. Benign soft-tissue tumors in a large referral population: distribution of specific diagnoses by age, sex, and location. AJR Am J Roentgenol 1995; 164 (02) 395-402
• 11 Tanas MR, Sboner A, Oliveira AM. et al. Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma. Sci Transl Med 2011; 3 (98) 98ra82
• 12 Doyle LA, Fletcher CD, Hornick JL. Nuclear expression of CAMTA1 distinguishes epitheloid hemangioendothelioma from histologic mimics. Am J Surg Pathol 2016; 40 (01) 94-102
• 13 Reynolds Jr DL, Jacobson JA, Inampudi P, Jamadar DA, Ebrahim FS, Hayes CW. Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol 2004; 182 (03) 741-744
• 14 Gherman CD, Fodor D. Epithelioid hemangioendothelioma of the forearm with radius involvement. Case report. Diagn Pathol 2011; 6: 120
• 15 Uribeetxebarria AL, Álvarez AC, García SC, Albizu ME, Eraso JV, Mancisidor KB. Epithelioid hemangioendothelioma: a diagnostic challenge. CT and MRI appearance: from typical to bizarre. ECR. 2016 ; Poster C-1000