作者：Roshan Ara Ghoorun1，刘智敏1，赵严冬2，熊斐3，饶雄辉4，任东林1

单位：1. 中山大学附属第六医院 结直肠外科，广东 广州 510275； 2. 中山大学附属第六医院 病理科，广东 广州 510275； 3. 中山大学附属第六医院 放射科，广东 广州 510275； 4. 中山大学附属第八医院 胃肠外科，广东 深圳 518033

Authors:  Roshan Ara Ghoorun1, LIU Zhimin1, ZHAO Yandong2, XIONG Fei3, RAO Xionghui4, REN Donglin1

Unit:  1.Department of Proctology, the Sixth Affiliated hospital of Sun Yat-Sen University, Guangzhou 510275, Guangdong, China； 2.Department of Pathology, the Sixth Affiliated hospital of Sun Yat-Sen University, Guangzhou 510275, Guangdong, China； 3.Department of Radiology, the Sixth Affiliated hospital of Sun Yat-Sen University, Guangzhou 510275, Guangdong, China； 4 Department of Gastrointestinal Surgery, the Eighth Affiliated hospital of Sun Yat-Sen University, Shenzhen 518033, Guangdong, China

摘要：

目的 盆腔增生性毛根鞘瘤(PTT)经常被误诊为其他疾病,导致多次手术,增加病人痛苦。在此,我们致力于探究PTT的临床病理表现和放射学特征以帮助诊断。方法 本研究仅纳入2011年9月至2018年12月间诊断为PTT的患者,回顾了其病变的临床病理特点和磁共振资料。结果  4位患者为良性PTT,4位患者为恶性PTT(MPTT)。病变通常潜伏数年,然后突然扩速发展。MPTT患者年龄偏小(P=0.12)。从MRI上看,良性PTT表现为实性囊肿,而MPTT表现为广泛的瘘道。纳入患者广泛局部切除术后,切缘阴性,且无局部复发或转移。从病理学来看,PTT表现为分叶状,无边缘浸润,可伴有毛鞘角化特点,而MPTT存在延伸到周围真皮的明显的非典型鳞状细胞,并伴有软骨基质反应。结论 临床医生可根据肿块明显且突然的进行性生长特点考虑为PTT,并应根据病变形态判断病变是否恶变。广泛的局部切除可得到一个良好的效果。

关键词：  增殖性毛根鞘瘤; 恶性增殖性毛根鞘瘤; 盆腔罕见实体瘤

Abstract：

Objective  Proliferating trichilemmal tumor (PTT) in the pelvis is often misdiagnosed for other diseases, resulting in multiple afflicting surgeries. Herein, we aim to investigate the clinicopathological and radiological findings of PTT to aid in diagnosis. Methods  Only patients diagnosed with PTT between September 2011 and December 2018 were included in this study. The clinicopathological characteristics and magnetic resonance imaging (MRI) of the lesions were reviewed. Results  4 patients had benign PTT whereas 4 other patients had malignant PTT (MPTT). The lesion was often dormant for years followed by a sudden rapid growth. MPTT patients were younger (P=0.12). On MRI, benign PTT appears as a solid cyst whereas MPTT as an extensive fistulous tract. There was no local recurrence or metastasis following a wide local resection with a negative margin. Pathologically, the tumors presented as lobulated nodules with a non-infiltrative border, with keratosis of the sheath. In MPTT, there was a distinct atypical squamous cord extending into the surrounding dermis with a cartilage matrix reaction. Conclusion  The clinician can suspect PTT based on a distinct sudden progressive growth pattern; and should suspect malignancy based on the lesion morphology. A wide local excision results in a promising outcome.

Key Words:  Proliferating trichilemmal tumor; Malignant proliferating trichilemmal tumor; Rare entity in the pelvic and perianal region