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以口咽肿物为表现的母细胞样套细胞淋巴瘤

Blastoid Mantle Cell Lymphoma Presenting as an Oropharyngeal Mass.

作者信息

Macapagal Sharina, Wannaphut Chalothorn, Takahashi Toshiaki, Yinadsawaphan Thanaboon, Nishimura Yoshito, Acoba Jared

机构信息

Internal Medicine, University of Hawaii John A. Burns School of Medicine, Honolulu, USA.

Hematology and Oncology, The Queen's Medical Center, Honolulu, USA.

出版信息

Cureus. 2024 Mar 18;16(3):e56378. doi: 10.7759/cureus.56378. eCollection 2024 Mar.

Abstract

Blastoid mantle cell lymphoma (MCL) is an extremely rare neoplasm with a dismal prognosis. MCL with an initial presentation in the oral cavity has been rarely reported. This report describes a 75-year-old male who presented with an oropharyngeal mass causing dysphonia and intermittent hypoxia. A biopsy and immunophenotyping confirmed MCL, favoring the blastoid variant. Imaging showed a 4.2 cm left oropharyngeal polypoid mass with extensive lymphadenopathy. His prognosis was considered unfavorable with elevated Ki-67 index, blastoid morphology, and p53 positivity of malignant cells. There was no central nervous system involvement. He received palliative radiation, resulting in profound tumor reduction and resolution of symptoms. An intensive chemoimmunotherapy was not deemed beneficial due to age, comorbidities, absence of TP53 mutation, and a personal preference for a less aggressive treatment. This case highlights the importance of risk-adapted and personalized management approaches in a very unique presentation of blastoid MCL.

摘要

母细胞样套细胞淋巴瘤(MCL)是一种极其罕见的肿瘤,预后很差。最初表现为口腔病变的MCL鲜有报道。本报告描述了一名75岁男性,他因口咽肿物导致声音嘶哑和间歇性缺氧就诊。活检及免疫表型分析确诊为MCL,倾向母细胞样变异型。影像学检查显示左侧口咽有一个4.2 cm的息肉样肿物,并伴有广泛的淋巴结病。鉴于其Ki-67指数升高、母细胞样形态以及恶性细胞p53阳性,其预后被认为不佳。未发现中枢神经系统受累。他接受了姑息性放疗,肿瘤明显缩小,症状得以缓解。由于患者年龄、合并症、无TP53突变以及个人倾向于采取侵袭性较小的治疗方法,强化化疗免疫治疗被认为并无益处。该病例突出了在母细胞样MCL这种非常独特的表现中,采用风险适应型和个性化管理方法的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78fd/11022918/8e5df374bd2d/cureus-0016-00000056378-i01.jpg

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