研究动态
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具有双侧睾丸受累和唾液腺肿块的侵袭性蕈样肉芽肿,表现出表型转换。

Aggressive Form of Mycosis Fungoides With Bilateral Testicular Involvement and Salivary Gland Mass, Exhibiting Phenotypic Switch.

发表日期:2024 Jul 12
作者: Ricardo Rolim, Tiago Maia, Alina Ionita, Mariana Cravo, José Cabeçadas
来源: Cell Death & Disease

摘要:

我们报告一例 72 岁男性因蕈样肉芽肿 (MF) 累及双侧睾丸的病例。患者 6 个月前被诊断为 MF,分期时发现睾丸受累。 MF 的罕见行为是早期内脏受累且没有造血淋巴器官受到影响,尽管进行了手术和化疗,患者仍会在最初诊断后 14 个月死亡。 T 细胞受体基因重排研究证实,肿瘤细胞表现出从皮肤中的 CD4/CD8- 谱到睾丸中的 CD4-/CD8- 谱的表型转变,并且是同一克隆,这使得这是第三例报告的 MF 病例影响睾丸,并且首先通过克隆性研究来证实它。临床演变可能与其独特的生物学有关,表现为肿瘤细胞转变为更具侵袭性的 CD4-/CD8-谱,以及疾病早期扩展到不常见的内脏部位。版权所有 © 2024 Wolters Kluwer Health, Inc. 所有权利预订的。
We report the case of a 72-year-old man with bilateral testicular involvement by mycosis fungoides (MF). The patient was diagnosed with MF 6 months prior, and testicular involvement was found upon staging. The rare behavior of MF, with early visceral involvement and no hematolymphoid organs affected, had a poor prognosis with patient death 14 months after the original diagnosis despite surgery and chemotherapy. The neoplastic cells showed a phenotypic switch from a CD4+/CD8- profile in the skin to a CD4-/CD8- in the testis and the same clone, confirmed with T-cell receptor gene rearrangement studies, making this the third reported case of MF affecting the testis and the first with clonality studies to confirm it. The clinical evolution may be related to its distinctive biology showcased in the neoplastic cell's switch to a more aggressive CD4-/CD8- profile and the early extension of the disease to an uncommon visceral site.Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.