Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 6, 2023; 11(28): 6949-6954
Published online Oct 6, 2023. doi: 10.12998/wjcc.v11.i28.6949
Malignant proliferative ependymoma of the neck with lymph node metastasis: A case report
Ke Wang, Jia-Zhu Wen, Shu-Xia Zhou, Lin-Feng Ye, Chun Fang, Yan Chen, Hai-Xia Wang, Xiao Luo
Ke Wang, Jia-Zhu Wen, Shu-Xia Zhou, Lin-Feng Ye, Chun Fang, Yan Chen, Hai-Xia Wang, Department of Radiology, The Second People’s Hospital of Quzhou, Quzhou 324000, Zhejiang Province, China
Xiao Luo, Department of Radiology, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 310009, Zhejiang Province, China
Author contributions: Wang K and Luo X designed the study, prepared drafted the manuscript; Wen JZ proofed the manuscript in language; Ye LF and Zhou SX contributed to the acquisition of imaging data; Fang C and Chen Y interpreted the findings; Luo X and Wang HX provided critical manuscript revision for important intellectual content; All authors have read and approve the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xiao Luo, MD, Attending Doctor, Department of Radiology, The Second Affiliated Hospital of Zhejiang University School of Medicine, No. 88 Jiefang Road, Shangcheng District, Hangzhou 310009, Zhejiang Province, China. luoxiao1990@zju.edu.cn
Received: July 29, 2023
Peer-review started: July 29, 2023
First decision: August 10, 2023
Revised: August 21, 2023
Accepted: September 11, 2023
Article in press: September 11, 2023
Published online: October 6, 2023
Abstract
BACKGROUND

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.

CASE SUMMARY

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.

CONCLUSION

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.

Keywords: Magnetic resonance imaging, Proliferating trichilemmal tumour, Head and neck imaging, Lymph node metastasis, Computed tomography, Case report

Core Tip: Malignant proliferating trichilemmal tumor (PTT) is an infrequent malignant neoplasm originating from cutaneous appendages, with only a handful of documented cases. This report delineates a unique instance of malignant PTT 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.