Niwa Takayoshi, Konishi Takaaki, Sasahara Asako, Sato Ayaka, Morizono Arisa, Harada Mayumi, Nishioka Kotoe, Fukuoka Osamu, Makise Naohiro, Saito Yuki, Ando Mizuo, Yoshimoto Takako, Shikama Takeshi, Yamashita Satoshi, Tanabe Masahiko, Seto Yasuyuki
Department of Breast and Endocrine Surgery, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
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.
Surg Case Rep. 2022 Sep 9;8(1):166. doi: 10.1186/s40792-022-01521-7.
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.
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.
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.
上皮样血管内皮瘤（EHE）是一种罕见的、生长缓慢的恶性血管肿瘤，由独特的黏液样透明基质中的上皮样内皮细胞组成。它最常累及躯体软组织、肺、肝和骨。在此，我们描述一例发生于腋窝区域的EHE病例。
一名61岁男性因多发肝血管瘤接受观察。氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描显示右腋窝肿瘤有特异性摄取。患者被转诊至我科进一步检查腋窝肿瘤。在右腋窝可触及一个质地柔软、活动度差的肿瘤。增强计算机断层扫描显示右腋窝肿瘤及肝血管瘤增大。此外，还发现双肺多发结节、左肾血管平滑肌脂肪瘤和左肾上腺腺瘤。超声检查显示甲状腺两叶均有肿块，腋窝有一个30毫米的分叶状低回声肿块，边界清晰但粗糙，内部回声不均匀。对甲状腺和腋窝肿瘤进行了细针穿刺细胞学检查：甲状腺肿瘤为V类，怀疑为甲状腺乳头状癌（PTC）；左颈内静脉上组淋巴结为V类，怀疑为PTC转移；腋窝肿瘤为III类，怀疑为含少量上皮样细胞的间叶组织肿瘤。双肺多发结节诊断为源自甲状腺癌的转移瘤。我们将这些疾病诊断为IVB期T1b（m）N1bM1（肺）的PTC和起源不明的右腋窝肿瘤。然而，推测其为原发性间叶组织肿瘤或肺癌或隐匿性乳腺癌的淋巴结转移。我们进行了全甲状腺切除术、左颈淋巴结清扫术和右腋窝肿瘤切除术。组织病理学检查显示甲状腺肿瘤为PTC，腋窝肿瘤为EHE。该EHE显示核异型性、坏死和高有丝分裂象。因此，被认为是高危EHE。
我们遇到了一例罕见的原发性皮下腋窝EHE伴肺转移甲状腺癌。由于我们的病例被归类为高危EHE，密切随访是合适的。
