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具有胸腺样成分的梭形上皮肿瘤(SETTLE):具有明确治疗意义的诊断挑战;病例报告。

Spindle epithelial tumor with thymus-like elements (SETTLE): a diagnostic challenge with distinct therapeutic implication; case report.

机构信息

Histopathology and Cytopathology, Department of Laboratory and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, 110085, India.

Head and Neck Oncosurgery, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, 110085, India.

出版信息

Diagn Pathol. 2024 Aug 13;19(1):108. doi: 10.1186/s13000-024-01527-9.

Abstract

Spindle epithelial tumor with thymus-like elements (SETTLE) is a rare malignant neoplasm of the thyroid gland which is believed to arise from intrathyroidal thymic tissue. It predominantly affects young adults and children presenting with a thyroid mass of variable duration and rarely occurs in adults. It has a high overall survival with a tendency for delayed metastasis. SETTLE is a biphasic lobulated tumor composed of spindle shaped cells along with glandular formations seen on histopathological examination. Despite its typical morphology it is commonly misdiagnosed on histopathology due to its rarity and overlapping morphology with other close mimics such as a carcinoma, synovial sarcoma and thymoma. Herein we report such a case occurring in a middle aged female presenting with a neck mass. She had an initial diagnosis of metastatic poorly differentiated squamous cell carcinoma possibly with an orophayngeal primary in view of co expression of CK, p40 and p16 on immunohistochemistry. The patient underwent surgical resection with modified neck dissection. On review at our hospital it was diagnosed as SETTLE and she remains disease free after a follow-up period of 1 year. Diligent histopathological examination espoused with a judicious panel of IHC markers in conjunction with clinicoradiological findings forms the mainstay of diagnosis. Diffuse and strong p16 immunoexpression has not been documented or evaluated in literature so far, and needs to be explored for its diagnostic utility in this rare entity.

摘要

具有胸腺样上皮成分的梭形细胞肿瘤(SETTLE)是一种罕见的甲状腺恶性肿瘤,被认为起源于甲状腺内胸腺组织。它主要影响年轻人和儿童,表现为甲状腺肿块,病程长短不一,在成年人中很少见。它的总体存活率较高,转移倾向延迟。SETTLE 是一种由梭形细胞和腺体型结构组成的双相分叶状肿瘤,组织病理学检查可见。尽管其形态典型,但由于其罕见性和与其他密切类似物(如癌、滑膜肉瘤和胸腺瘤)的形态重叠,在组织病理学上常被误诊。本文报告了一例发生在中年女性的病例,表现为颈部肿块。由于免疫组化 CK、p40 和 p16 的共表达,最初诊断为转移性低分化鳞状细胞癌,可能伴有口咽原发灶。患者接受了手术切除和改良颈清扫术。在我院复查时,诊断为 SETTLE,随访 1 年后无疾病。仔细的组织病理学检查,辅以明智的免疫组化标志物面板,并结合临床和影像学结果,是诊断的主要依据。目前尚未在文献中记录或评估弥漫性和强 p16 免疫表达,需要进一步研究其在这种罕见实体中的诊断效用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aff5/11321057/f60fb822c046/13000_2024_1527_Fig1_HTML.jpg

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