Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 6, 2020; 8(19): 4466-4474
Published online Oct 6, 2020. doi: 10.12998/wjcc.v8.i19.4466
Primary rhabdomyosarcoma: An extremely rare and aggressive variant of male breast cancer
Cătălin Bogdan Satală, Ioan Jung, Tivadar Jr Bara, Patricia Simu, Iunius Simu, Madalina Vlad, Rita Szodorai, Simona Gurzu
Cătălin Bogdan Satală, Department of Pathology, Clinical County Emergency Hospital, Tirgu Mures 540139, Romania
Ioan Jung, Rita Szodorai, Department of Pathology, George Emil Palade University of Medicine, Pharmacy, Sciences and Technology, Targu Mures 530149, Romania
Tivadar Jr Bara, Department of Surgery, Clinical County Emergency Hospital, Targu Mures 540136, Romania
Patricia Simu, Iunius Simu, Department of Radiology, George Emil Palade University of Medicine, Pharmacy, Sciences and Technology, Targu Mures 530149, Romania
Madalina Vlad, Simona Gurzu, Department of Pathology, Clinical County Emergency Hospital, Targu Mures 540136, Romania
Simona Gurzu, Department of Pathology, George Emil Palade University of Medicine, Pharmacy, Science and Technology, Targu Mures 530149, Romania
Simona Gurzu, Research Center (CCAMF), University of Medicine, Pharmacy, Sciences and Technology, Targu Mures 540139, Romania
Author contributions: Satală CB drafted the article, searched for the literature and contributed to the diagnosis; Jung I contributed to the diagnosis and immunohistochemical assessment; Bara TJ performed the surgical intervention and clinical follow-up; Simu P and Simu I contributed to the diagnosis with the imaging assessment and literature data review; Vlad M performed the macroscopic assessment and contributed to the histological diagnosis; Szodorai R was involved in literature review; Gurzu S supervised the histological and immunohistochemical diagnosis and conferred the final agreement for publication; Satala CB and Jung I have equal contribution to the paper.
Informed consent statement: Signed consent of patient was obtained, before surgery.
Conflict-of-interest statement: None declared.
CARE Checklist (2016) statement: The authors have read the CARE Checklist statement and the manuscript was prepared and revised according to the CARE statement checklist of items.
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: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Simona Gurzu, MD, PhD, Chief Doctor, Dean, Director, Full Professor, Research Scientist, Department of Pathology, George Emil Palade University of Medicine, Pharmacy, Science and Technology, 38 Gheorghe Marinescu Street, Targu Mures 530149, Romania. simonagurzu@yahoo.com
Received: May 24, 2020
Peer-review started: May 24, 2020
First decision: July 25, 2020
Revised: July 28, 2020
Accepted: September 4, 2020
Article in press: September 4, 2020
Published online: October 6, 2020
Processing time: 126 Days and 5.4 Hours
Abstract
BACKGROUND

Rhabdomyosarcoma (RMS) of the breast, a mesenchymal neoplasm with skeletal muscle differentiation, is an extremely rare tumour in males, with less than 30 cases published in English-language literature. We report on the first case of a male breast RMS, with an unusual ectomesenchymal/neuroectodermal component.

CASE SUMMARY

A 55-year-old, previously healthy male, underwent a radical left mastectomy for an ulcerated tumour mass, occupying the breast and left anterior thoracic wall. The biopsy specimen indicated the presence of a tumour with neural origins, namely a peripheral neuroectodermal tumour (PNET). The surgical specimens identified two components. The rhabdomyosarcomatous component (over 70%) was represented by large pleomorphic cells with positivity for desmin, sarcomeric actin and myogenin. The PNET-like ectomesenchymal component, which was admixed with the RMS cells, and was also revealed during the preoperative biopsy, consisted of small cells which expressed neurofilament, neuron specific enolase and CD99. The microscopic examination, along with the immunohistochemical profile, allowed the diagnosis of an RMS, with unusual ectomesenchymal differentiation. The patient refused the postoperative oncologic therapy and died three months after surgery.

CONCLUSION

In patients with RMS of the breast, the PNET-like ectomesenchymal component increases the diagnosis difficulty, especially in biopsy specimens. This differentiation can be immunohistochemically proven and might highlight the possible development of high-grade sarcoma of the breast from remnants of the embryological ectodermal layer.

Keywords: Mammary gland; Rhabdomyosarcoma; Ectomesenchymoma; Male; Case report; CD99

Core Tip: In this paper we presented an exceedingly rare and aggressive case of mammary rhabdomyosarcoma (RMS) in a male patient. We found no cases previously published in international journals, reporting an RMS with a peripheral neuroectodermal tumour-like ectomesenchymal component, namely neuroectodermal differentiation. The specific nature of the case was indicated by the presence of the ectomesenchymal component in the biopsy specimen, the case being firstly diagnosed as a tumour with neural differentiation. These findings emphasize the need for an attentive microscopic evaluation of mesenchymal tumours in the mammary region, to further confirm or infirm the presence of a second highly-malignant tumour population.