Subcutaneous axillary primary epithelioid hemangioendothelioma: report of a rare case

Takayoshi Niwa; Takaaki Konishi; Asako Sasahara; Ayaka Sato; Arisa Morizono; Mayumi Harada; Kotoe Nishioka; Osamu Fukuoka; Naohiro Makise; Yuki Saito; Mizuo Ando; Takako Yoshimoto; Takeshi Shikama; Satoshi Yamashita; Masahiko Tanabe; Yasuyuki Seto

doi:10.1186/s40792-022-01521-7

Subcutaneous axillary primary epithelioid hemangioendothelioma: report of a rare case

Surg Case Rep. 2022 Sep 9;8(1):166. doi: 10.1186/s40792-022-01521-7.

Authors

Takayoshi Niwa¹, Takaaki Konishi¹, Asako Sasahara¹, Ayaka Sato¹, Arisa Morizono¹, Mayumi Harada¹, Kotoe Nishioka¹, Osamu Fukuoka², Naohiro Makise^{3

4}, Yuki Saito², Mizuo Ando^{2

5}, Takako Yoshimoto¹, Takeshi Shikama¹, Satoshi Yamashita¹, Masahiko Tanabe⁶, Yasuyuki Seto^{1

7}

Affiliations

¹ Department of Breast and Endocrine Surgery, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
² Department of Otorhinolaryngology and Head and Neck Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
³ Department of Pathology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
⁴ Division of Surgical Pathology, Chiba Cancer Center, 666-2 Nitona-Cho, Chuo-Ku, Chiba-Shi, Chiba, 260-8717, Japan.
⁵ Department of Otolaryngology-Head and Neck Surgery, Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Japan, 2-5-1 Shikata-Cho, Kita-Ku, Okayama, 700-8558, Japan.
⁶ Department of Breast and Endocrine Surgery, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan. masahikotanabe@g.ecc.u-tokyo.ac.jp.
⁷ Department of Gastrointestinal Surgery, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.

Abstract

Background: Epithelioid hemangioendothelioma (EHE) is a rare and slow-growing malignant vascular neoplasm composed of epithelioid endothelial cells within a distinctive myxohyaline stroma. It most commonly involves somatic soft tissue, lungs, liver and bone. Herein, we describe a case of EHE arising in the axillary region.

Case presentation: A 61-year-old man was under observation for multiple hepatic hemangiomas. Fluorodeoxyglucose-positron emission tomography/computed tomography showed specific uptake in a right axillary tumor. The patient was referred to our department for further investigation of the axillary tumor. An elastic-soft and poorly mobile tumor was palpable in the right axilla. Contrast-enhanced computed tomography showed a right axillary tumor and enlarged hepatic hemangiomas. In addition, multiple nodules in both lungs, a left renal angiomyolipoma, and left adrenal adenoma were revealed. Ultrasonography showed masses in both lobes of the thyroid gland, and a 30-mm lobulated hypoechoic mass in the axilla with well-defined and rough borders, showing internal heterogeneity. Fine-needle aspiration cytology was performed on the thyroid and axillary tumors: the thyroid tumor was class V, raising suspicion of papillary thyroid cancer (PTC); the left superior internal jugular node was class V, raising suspicion of metastasis of PTC; and the axillary tumor was class III, raising suspicion of a mesenchymal tumor with few epithelioid cells. The multiple lung nodules were diagnosed as metastatic tumors derived from thyroid cancer. We diagnosed these diseases as PTC of T1b(m)N1bM1(lung) Stage IVB and a right axillary tumor of unclear origin. However, it was assumed to be a primary mesenchymal tumor or a lymph node metastasis from lung cancer or occult breast cancer. We performed total thyroidectomy, left cervical lymph node dissection, and right axillary tumor excision. Histopathologic examination revealed the thyroid tumor as a PTC and the axillary tumor as an EHE. The EHE showed nuclear atypia, necrosis and high mitotic figures. Hence, it was considered to be a high-risk EHE.

Conclusions: We experienced a rare primary subcutaneous axillary EHE with metastatic thyroid cancer in the lung. Since our case was classified as a high-risk EHE, a close follow-up would be appropriate.

Keywords: Axillary tumor; Epithelioid hemangioendothelioma; Papillary thyroid cancer.