Rare Pathology Case Report: Low-Grade Endometrial Stromal Sarcoma Forming Sex Cord- and Endometrioid Gland-Like Differentiation in Metastatic Foci.

IF 0.7 Q4 PATHOLOGY Case Reports in Pathology Pub Date : 2024-09-04 eCollection Date: 2024-01-01 DOI:10.1155/2024/4073869
Haneen Al-Maghrabi, Ghadeer Mokhtar, Jaudah Al-Maghrabi
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Abstract

Low-grade endometrial stromal sarcomas (LGESSs) are indolent tumors with a slow progression rate that tend to recur locally. They represent up to 10% of all primary sarcomas of the uterus and endometrium and only 0.2% of all genital tract tumors. They are commonly present in a younger demographic compared to other uterine tumors, with patients' ages typically between 42 and 58 years old. Although the overall 5-year survival rate is excellent, it has a natural history of delayed metastases which may manifest even decades after the disease was first diagnosed. They typically present as poorly defined lesions infiltrating the myometrium, along with extensive engagement of surrounding vascular structures. LGESS may display variants of different morphologies such as smooth muscle, fibromyxoid, sex cord-like, and endometrioid-type gland differentiation. These variations can pose a diagnostic challenge. The occurrence of this differentiation in a metastatic focus rather than in the primary tumor is seldom recorded in the literature. We present a case of a 51-year-old lady with a history of LGESS who was treated with surgery and radiotherapy and then presented after 12 years with an inferior vena cava (IVC) mass, which was confirmed histologically to be metastatic LGESS. Immunohistochemistry studies reveal strong positivity for CD10, WT1, and PR. These markers were negative in the sex cord and endometrioid gland-like differentiation counterparts. The patient had her initial follow-up appointment after the IVC mass resection, and she was in good health with no complications. To the best of our knowledge, this case represents a unique instance of metastatic LGESS exhibiting both sex cord and endometrioid gland-like differentiation that has not been observed in the primary tumor.

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罕见病理病例报告:转移灶中形成性索和子宫内膜样腺体样分化的低级别子宫内膜间质肉瘤
低分化子宫内膜间质肉瘤(LGESSs)是一种进展缓慢、倾向于局部复发的懒癌。它们占子宫和子宫内膜原发性肉瘤的 10%,仅占生殖道肿瘤的 0.2%。与其他子宫肿瘤相比,这种肿瘤的发病人群通常较为年轻,患者年龄一般在 42 岁至 58 岁之间。虽然总的 5 年生存率很高,但它有延迟转移的自然史,甚至可能在首次确诊后几十年才出现。它们通常表现为浸润子宫肌层的界限不清的病变,并广泛累及周围血管结构。LGESS 可表现为不同形态的变异,如平滑肌型、纤维瘤型、性索状型和子宫内膜样腺体分化型。这些变异会给诊断带来挑战。文献中很少记录这种分化出现在转移灶而非原发肿瘤中。我们报告了一例 51 岁女士的病例,她曾有 LGESS 病史,接受过手术和放疗,12 年后出现下腔静脉(IVC)肿块,经组织学证实为转移性 LGESS。免疫组化研究显示,CD10、WT1 和 PR 呈强阳性。这些标记物在性索和子宫内膜样腺体分化对应物中呈阴性。患者在 IVC 肿块切除术后进行了首次复诊,她的健康状况良好,没有出现任何并发症。据我们所知,该病例是转移性 LGESS 的一个独特病例,它同时表现出性索和子宫内膜样腺样分化,而这在原发肿瘤中尚未观察到。
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审稿时长
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