Wang Ke, Wen Jia-Zhu, Zhou Shu-Xia, Ye Lin-Feng, Fang Chun, Chen Yan, Wang Hai-Xia, Luo Xiao
Department of Radiology, The Second People's Hospital of Quzhou, Quzhou 324000, Zhejiang Province, China.
Department of Radiology, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 310009, Zhejiang Province, China.
World J Clin Cases. 2023 Oct 6;11(28):6949-6954. doi: 10.12998/wjcc.v11.i28.6949.
Malignant proliferating trichilemmal tumor (MPTT) is an infrequent malignant neoplasm originating from cutaneous appendages, with only a handful of documented cases. This report delineates a unique instance of MPTT situated in the neck, accompanied by lymph node metastasis. A comprehensive exposition of its clinical trajectory and imaging manifestation is presented, aiming to enhance comprehension and management of this atypical ailment.
Patient concerns: A 79-year-old male presented with a longstanding right neck mass persisting for over six decades, exhibiting recent enlargement over the past year. Diagnoses: Enhanced magnetic resonance imaging of the neck unveiled an elliptical mass on the right neck side, characterized by an ill-defined border and a heterogeneous signal pattern. The mass exhibited subdued signal intensity on T1-weighted imaging (T1WI) and a heterogeneous high signal on T2-weighted imaging (T2WI), interspersed with a lengthy T1 and T2 cystic signal motif. Close anatomical association with the submandibular gland joint was noted, and intravenous gadolinium diethylene triamine pentaacetic acid administration facilitated conspicuous enhancement. Substantial enhancement of the solid segment prompted an initial preoperative diagnosis of malignant nerve sheath tumor. However, post-surgery histopathological and immunohistochemical analysis conclusively confirmed the diagnosis as malignant hyperplastic external hair root sheath tumor. Intervention: Complete excision of the tumor was successfully executed. Outcomes: The patient experienced a favorable postoperative recovery.
Malignant proliferative trichilemmal tumor external hair root sheath tumor is a cystic-solid lesion, appearing as low signal on T1WI images or high signal on T2WI with enhancement of the solid component. Suspicions of malignancy are heightened when the tumor border is indistinct, tissue planes are breached, or when linear or patchy high signals are observed in the subcutaneous tissue on T1 liver acquisition with volume acceleration enhanced images along with intermediate signal on T2WI and restricted diffusion on diffusion-weighted imaging images. Strong consideration for malignancy should arise if there are signs of compromised adjacent tissue relationships or direct invasion evident on imaging. We have incorporated the above-mentioned content into the entire manuscript.
恶性增殖性毛鞘瘤（MPTT）是一种罕见的起源于皮肤附属器的恶性肿瘤，仅有少数病例报道。本报告描述了一例位于颈部的MPTT的独特病例，并伴有淋巴结转移。本文全面阐述了其临床病程及影像学表现，旨在提高对这种非典型疾病的认识和管理水平。
患者情况：一名79岁男性，右侧颈部肿物存在六十余年，近一年来出现增大。诊断：颈部增强磁共振成像显示右侧颈部有一椭圆形肿物，边界不清，信号不均匀。该肿物在T1加权成像（T1WI）上呈低信号，在T2加权成像（T2WI）上呈不均匀高信号，其间夹杂着长T1和T2的囊性信号特征。注意到该肿物与下颌下腺关节关系密切，静脉注射钆喷酸葡胺后可见明显强化。实性部分的显著强化提示术前初步诊断为恶性神经鞘瘤。然而，术后组织病理学和免疫组化分析最终确诊为恶性增生性外毛根鞘瘤。干预措施：成功实施肿瘤完整切除。结果：患者术后恢复良好。
恶性增殖性毛鞘瘤（外毛根鞘瘤）是一种囊实性病变，在T1WI图像上表现为低信号，在T2WI上表现为高信号，实性成分有强化。当肿瘤边界不清、组织层面被破坏，或在肝脏容积加速采集增强图像的T1WI上皮下组织出现线性或斑片状高信号，同时T2WI呈中等信号且扩散加权成像图像上扩散受限，怀疑为恶性时应高度警惕。如果影像学上有相邻组织关系受损或直接侵犯的迹象，则应高度考虑为恶性。我们已将上述内容纳入整篇稿件。
