Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas
Background Primary breast sarcoma (PBS) is rare, comprising approximately 1% of breast malignancies. Rhabdomyosarcoma (RMS) accounts for an extremely small proportion of PBSs, often leading to delayed histologic confirmation. Methods Upon reviewing Asan Medical Center’s pathology database between 20...
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doaj-37f26221dcdb406fa996de34a9e4a5f52020-11-24T20:53:19ZengKorean Society of Pathologists & the Korean Society for CytopathologyJournal of Pathology and Translational Medicine2383-78372383-78452019-09-0153530831610.4132/jptm.2019.07.2216855Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast SarcomasJunyoung Shin0Hee Jeong Kim1Dae-Yeon Kim2Gyungyub Gong3Kyung-Ja Cho4 Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pediatric Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, KoreaBackground Primary breast sarcoma (PBS) is rare, comprising approximately 1% of breast malignancies. Rhabdomyosarcoma (RMS) accounts for an extremely small proportion of PBSs, often leading to delayed histologic confirmation. Methods Upon reviewing Asan Medical Center’s pathology database between 2000 and 2018, 41 PBS cases were retrieved, including three cases of primary RMS of the breast. Their clinicopathological features were analyzed, and the literature related to PBS and primary RMS of the breast was reviewed. Results We identified three primary breast RMS cases from our institution database, comprising 7.3% of PBS: one case each of spindle cell/sclerosing RMS (ssRMS), alveolar RMS (aRMS), and embryonal RMS (eRMS). All cases involved adolescents or young adults (14, 16, and 25 years, respectively) who underwent mastectomy or radiotherapy and were confirmed using immunohistochemical testing for myogenin, desmin, and myogenic differentiation. The ssRMS patient experienced recurrence at the operation site 4 months post-surgery despite undergoing concurrent chemoradiotherapy. The aRMS patient had multiple metastases at diagnosis and showed FAX3-FOXO1 fusion transcripts; she died 22 months after the diagnosis. The eRMS patient had enlarged axillary lymph nodes; post-radiotherapy, the lesion recurred as multiple metastases to the bone and lung. She died 18 months post-diagnosis. Conclusions Our experience on RMS cases suggests that spindle cell or small round cell malignancy in breasts of young female should raise suspicion for the possibility of primary or secondary RMS. To our knowledge, this is the second report of primary breast ssRMS and it may help clinicians who encounter this rare disease in the future.http://www.jpatholtm.org/upload/pdf/jptm-2019-07-22.pdfSpindle cell rhabdomyosarcomaSclerosing rhabdomyosarcomaPrimary sarcoma of the breast |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Junyoung Shin Hee Jeong Kim Dae-Yeon Kim Gyungyub Gong Kyung-Ja Cho |
spellingShingle |
Junyoung Shin Hee Jeong Kim Dae-Yeon Kim Gyungyub Gong Kyung-Ja Cho Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas Journal of Pathology and Translational Medicine Spindle cell rhabdomyosarcoma Sclerosing rhabdomyosarcoma Primary sarcoma of the breast |
author_facet |
Junyoung Shin Hee Jeong Kim Dae-Yeon Kim Gyungyub Gong Kyung-Ja Cho |
author_sort |
Junyoung Shin |
title |
Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas |
title_short |
Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas |
title_full |
Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas |
title_fullStr |
Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas |
title_full_unstemmed |
Primary Rhabdomyosarcoma of the Breast: Study of Three Cases at One Institution with a Review of Primary Breast Sarcomas |
title_sort |
primary rhabdomyosarcoma of the breast: study of three cases at one institution with a review of primary breast sarcomas |
publisher |
Korean Society of Pathologists & the Korean Society for Cytopathology |
series |
Journal of Pathology and Translational Medicine |
issn |
2383-7837 2383-7845 |
publishDate |
2019-09-01 |
description |
Background Primary breast sarcoma (PBS) is rare, comprising approximately 1% of breast malignancies. Rhabdomyosarcoma (RMS) accounts for an extremely small proportion of PBSs, often leading to delayed histologic confirmation. Methods Upon reviewing Asan Medical Center’s pathology database between 2000 and 2018, 41 PBS cases were retrieved, including three cases of primary RMS of the breast. Their clinicopathological features were analyzed, and the literature related to PBS and primary RMS of the breast was reviewed. Results We identified three primary breast RMS cases from our institution database, comprising 7.3% of PBS: one case each of spindle cell/sclerosing RMS (ssRMS), alveolar RMS (aRMS), and embryonal RMS (eRMS). All cases involved adolescents or young adults (14, 16, and 25 years, respectively) who underwent mastectomy or radiotherapy and were confirmed using immunohistochemical testing for myogenin, desmin, and myogenic differentiation. The ssRMS patient experienced recurrence at the operation site 4 months post-surgery despite undergoing concurrent chemoradiotherapy. The aRMS patient had multiple metastases at diagnosis and showed FAX3-FOXO1 fusion transcripts; she died 22 months after the diagnosis. The eRMS patient had enlarged axillary lymph nodes; post-radiotherapy, the lesion recurred as multiple metastases to the bone and lung. She died 18 months post-diagnosis. Conclusions Our experience on RMS cases suggests that spindle cell or small round cell malignancy in breasts of young female should raise suspicion for the possibility of primary or secondary RMS. To our knowledge, this is the second report of primary breast ssRMS and it may help clinicians who encounter this rare disease in the future. |
topic |
Spindle cell rhabdomyosarcoma Sclerosing rhabdomyosarcoma Primary sarcoma of the breast |
url |
http://www.jpatholtm.org/upload/pdf/jptm-2019-07-22.pdf |
work_keys_str_mv |
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