Metastatic Malignant Perivascular Epithelioid Cell Tumor (PEComa) in Parotid Gland Diagnosed by Fine Needle Aspiration Biopsy- a Case Report and Literature Review

Shiguang Liu, Zaibo Li, Rulong Z. Shen
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引用次数: 3

Abstract

Perivascular Epithelioid Cell Tumors (PEComas) are exceedingly rare mesenchymal tumors in salivary glands; no case of metastatic malignant PEComas in salivary gland was described. Here we reported a case of a 74-year old female with a history of malignant PEComa of retro peritoneum, who underwent surgical resection 11 years ago. She had metastatic PEComas in her liver and right upper extremity 7 months prior to the current presentation, with a 0.6 cm soft mobile palpable nodule in the right parotid region without tenderness. Fine needle aspiration biopsy showed spindle-shaped cells with indistinct cell border, wispy acidophilic cytoplasm, and oval to cigar-shaped hyper chromatic nuclei with evenly distributed fine chromatin. No epithelioid form was identified. Immunohistochemistry study showed the neoplastic cells were positive for smooth muscle actin, h-caldesmon and negative for cytokeratin, S-100 and HMB-45. Diagnosis of metastatic malignant Perivascular epithelioid cell tumor was made and the patient was continued with chemotherapy. This case is the first report of metastatic malignant PEComa in parotid gland. The patient’s known history of malignant Perivascular epithelioid cell tumor is important when cytopathologists make the differential diagnosis for the mesenchymal neoplasm of salivary gland. Perivascular Epithelioid Cell Tumor (PEComa) is a family of mesenchymal neoplasms [1-2]. The most common PEComas include renal Angiomyolipoma (AML), pulmonary Lymphangioleiomyomatosis (LAM) and Clear Cell Sugar Tumor (CCST) of the lung. It is exceedingly rare in salivary glands, to our knowledge, only 3 cases of primary angiomyolipoma of parotid gland were reported [3]. No case of metastatic malignant PEComas in salivary gland was described. We report a case of metastatic malignant PEComa in parotid gland diagnosed by fine needle aspiration biopsy. The literatures on this rare mesenchymal neoplasm are reviewed.
细针穿刺活检诊断腮腺转移性恶性血管周围上皮样细胞瘤1例并文献复习
血管周围上皮样细胞瘤(PEComas)是一种极为罕见的涎腺间充质肿瘤;没有一例涎腺转移性恶性PEComas被描述。我们在此报告一位74岁女性,有腹膜后恶性PEComa病史,11年前接受手术切除。7个月前,她的肝脏和右上肢有转移性胸腺瘤,右侧腮腺区有0.6厘米软的可移动结节,无压痛。细针穿刺活检显示梭形细胞,细胞边界不清,细胞质嗜酸,细胞核呈椭圆形至雪茄状,细染色质分布均匀。未发现上皮样形态。免疫组化结果显示,肿瘤细胞平滑肌肌动蛋白、h-caldesmon阳性,细胞角蛋白、S-100、HMB-45阴性。诊断为恶性血管周围上皮样细胞瘤,继续化疗。本病例为首次报道的腮腺转移性恶性PEComa。当细胞病理学家对唾液腺间质肿瘤进行鉴别诊断时,患者已知的恶性血管周围上皮样细胞瘤病史是很重要的。血管周围上皮样细胞瘤(PEComa)是间质肿瘤的一个家族[1-2]。最常见的PEComas包括肾血管平滑肌脂肪瘤(AML),肺淋巴管平滑肌瘤病(LAM)和肺透明细胞糖瘤(CCST)。在唾液腺中极为罕见,据我们所知,原发性腮腺血管平滑肌脂肪瘤仅报道3例[3]。没有一例转移性恶性PEComas在唾液腺被描述。我们报告一例经细针穿刺活检诊断的腮腺转移性恶性PEComa。本文对有关这种罕见间质肿瘤的文献作一综述。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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