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Case Report
Metastatic proximal-type epithelioid sarcoma presenting as a chest lesion with a pathological fracture of the humeral neck
1 Registrar in Radiology, Department of Radiology, Teaching Hospital Peradeniya, Peradeniya, Sri Lanka
2 Head, Consultant Radiologist, Department of Radiology, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka
3 Head, Professor, Consultant Pathologist, Department of Pathology, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka
4 Professor, Consultant Radiologist, Department of Radiology, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka
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Rajashilpa Mulacharige Madhuwan Sasira Kasun Kumara
Karandagolla, Post Code 20738,
Sri Lanka
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Article ID: 101345Z01RK2022
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Kumara RMMSK, Jeevani U, Prabodini MAM, Sulochana W, Badra HP. Metastatic proximal-type epithelioid sarcoma presenting as a chest lesion with a pathological fracture of the humeral neck. Int J Case Rep Images 2022;13(2):137–141.ABSTRACT
Introduction: Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which occurs mostly in young males and is much rarer than classic distal sub type. A young male, with a known diagnosis of neurofibromatosis type 1 (NF1) presenting with metastatic proximal-type epithelioid sarcoma is reported.
Case Report: A 30-year-old male with known NF1, hypothyroidism, epilepsy, presented with a right shoulder pain of two weeks duration. Plain radiographs revealed an expansile lytic lesion, an aggressive periosteal reaction in right humeral head with an underlying pathological fracture. A well-defined extrapulmonary mass with a lobulated margin in upper zone of right hemithorax with inferior rib erosion of 3rd rib and the 3rd intercostal space widening were also observed. Contrast-enhanced computed tomography (CECT) chest and abdomen confirmed peripherally located poorly enhancing soft tissue mass with lobulated margins arising from posterior chest wall along with a subdiaphragmatic deposit. Multiple lytic lesions in right head of humerus with an underlying pathological fracture of surgical neck, T2, T6, and L3 vertebral bodies, and pelvic bones were observed too. Diagnosis of malignant peripheral nerve sheath tumor with metastases was made and ultrasound-guided biopsy of chest wall lesion was performed. Histology and immunohistochemistry (IHC) revealed a proximal type epithelioid sarcoma. The patient was managed with palliative surgery and oncological follow-up.
Conclusion: Presentation of proximal-type epithelioid sarcoma with disseminated metastases in a patient with NF1 masquerading malignant peripheral nerve sheath tumor (MPNST) and the challenges of diagnosis on clinical, radiological, and histopathology as well as IHC perspectives of this rare disease entity is highlighted.
Keywords: Epithelioid sarcoma, Metastatic, Pathological fracture, Proximal type
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Acknowledgments
The authors thank staff of the Department of Radiology, Teaching Hospital Peradeniya and Department of Pathology, Faculty of Medicine, University of Peradeniya, those who supported in the establishment of the diagnosis of this case report.
Author ContributionsRajashilpa Mulacharige Madhuwan Sasira Kasun Kumara - Substantial contributions to conception and design, Acquisition of data, Interpretation of data, Drafting the article, Final approval of the version to be published
Udupihille Jeevani - Substantial contributions to conception and design, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Mathara Arachchige Madusha Prabodini - Substantial contributions to conception and design, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Wijetunge Sulochana - Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Hewavithana Padma Badra - Interpretation of data, Drafting the article, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2022 Rajashilpa Mulacharige Madhuwan Sasira Kasun Kumar. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
