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Case Reports
. 2024 Dec 11;16(12):e75544.
doi: 10.7759/cureus.75544. eCollection 2024 Dec.

Metastatic Sclerosing Epithelioid Fibrosarcoma at Diagnosis: A Case Report

Isabel R Miguel  1 , Mavilde Arantes  2 , Rafael Matias  1 , Ana M Ferreira  3 , Mariana Afonso  4
Affiliations
Case Reports

Metastatic Sclerosing Epithelioid Fibrosarcoma at Diagnosis: A Case Report

Isabel R Miguel et al. Cureus. .

Abstract

Sclerosing epithelioid fibrosarcoma (SEF) is a rare and aggressive neoplasm composed of epithelioid cells arranged in strands and nests embedded in a highly sclerotic collagenous stroma. We report a case of a 36-year-old man who started with lumbar pain, with extension to both legs, night sweats, and weight loss. He underwent magnetic resonance imaging (MRI) of the lumbar spine; computed tomography (CT) scan of the chest, abdomen, and pelvis; and [18F]-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) scan. The CT scan revealed a 13 cm thoracic mass, the MRI presented with diffuse neoplastic invasion of the vertebrae, and the PET showed hepatic, bone, and possibly pulmonary metastases. The histological diagnosis was compatible with SEF. The disease progressed very quickly, namely, with an episode of spinal cord compression, which made the patient paraplegic. He underwent surgery and, subsequently, radiotherapy (RT). Due to the clinical and analytical evolution, it was not possible to initiate systemic treatment and the patient ultimately passed away. In conclusion, SEF is an aggressive type of sarcoma that affects middle-aged patients, with high rates of distant metastases and mortality. The usual treatment is surgery followed by either radiotherapy or chemotherapy. However, further clinical trials are needed to find more systemic target therapies.

Keywords: metastatic sarcoma; oncology; palliative care; radiation oncology; sclerosing epithelioid fibrosarcoma.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Health Ethics Committee of the Portuguese Institute of Oncology (CES-IPOP) issued approval 048/024. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Magnetic resonance imaging T1-weighted imaging (MRI T1W1) sequence: sagittal plane
Neoplastic invasion of the vertebrae of D12, L1, L3, and L4 (arrows)
Figure 2
Figure 2. Magnetic resonance imaging T1-weighted imaging (MRI T1W1) sequence: axial plane
Neoplastic invasion of the sacrum and of the iliac bone, with an extension to the iliac muscle (arrow)
Figure 3
Figure 3. Chest, abdomen, and pelvic computed tomography (CT): coronal plane
Hypodense mass, adjacent to the right part of the diaphragm and with extension into the thoracic cavity
Figure 4
Figure 4. Chest, abdomen, and pelvic computed tomography (CT): axial plane
Hypodense mass extending into the thoracic cavity, with areas of calcification, measuring 12 x 13 cm
Figure 5
Figure 5. [18F]-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT)
a) Disseminated neoplasic involvement; b) thoracic mass (arrow); c) spinal canal lesion at the level of D9 (arrow)
Figure 6
Figure 6. Histological evaluation: hematoxylin and eosin (H&E) staining
Malignant neoplasm composed of epithelioid cells, disposed in sheaths and trabecula in a dense and sclerohialinized stroma
Figure 7
Figure 7. Immunohistochemical study
Strong and diffuse positivity for MUC4
Figure 8
Figure 8. Radiotherapy treatment plan – spine lesions of D2-D3
Colorwash distribution of dose: sagittal (a) and axial (b) planes. The red line corresponds to the planning target volume (PTV).
Figure 9
Figure 9. Radiotherapy treatment plan – spine lesions of D8-D11
Colorwash distribution of dose: sagittal (a) and axial (b) planes. The red line corresponds to the planning target volume (PTV).
See this image and copyright information in PMC

References

    1. Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma. Meis-Kindblom JM, Kindblom LG, Enzinger FM. Am J Surg Pathol. 1995;19:979–993. - PubMed
    1. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Sbaraglia M, Bellan E, Dei Tos AP. Pathologica. 2021;113:70–84. - PMC - PubMed
    1. Fibrosarcoma: a review and update. Folpe AL. Histopathology. 2014;64:12–25. - PubMed
    1. Sclerosing epithelioid fibrosarcoma: in-depth review of a genetically heterogeneous tumor. Murshed KA, Al-Bozom I, Ammar A. APMIS. 2021;129:455–460. - PubMed
    1. The clinicopathological spectrum of sclerosing epithelioid fibrosarcoma: report of an additional series with review of the literature. Peng Y, Zhang D, Lei T, et al. Pathology. 2023;55:355–361. - PubMed

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