Primary sclerosing epithelioid fibrosarcoma presenting as lombosciatic syndrome: Case report and literature review

Khezami, Karim; Gharbi, Ahmed; Chabaane, Mohamed; Bennour, Mohamed Amine; Nouri, H.

doi:10.1016/j.ijscr.2021.105657

Cited by 3 publications

(3 citation statements)

References 22 publications

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“…In contrast to reported literature where soft tissue primary site was common [3], our patient had primary in the bone, with multiple lytic lesions in the D10 vertebra. The initial bone marrow and liver/spleen involvement led us to work along the lines of haemato-lymphoid malignancy.…”

Section: Discussioncontrasting

confidence: 87%

“…These tumours mostly occur in the lower extremities and limb girdles, trunk and to a lesser extent, in the upper limb girdles and neck [2]. Primary SEF of the visceral organs is exceedingly rare, with only a very few case reports [3]. It has been noted that local recurrences occur in more than 50% of cases, with spread to distant sites in more than 40% of cases.…”

Section: Introductionmentioning

confidence: 99%

“…It has been noted that local recurrences occur in more than 50% of cases, with spread to distant sites in more than 40% of cases. The most common sites of distant metastasis include the lungs (70%), osseous dissemination to multiple bones (41%) and pleura/chest wall (11%) [3]. Poor prognostic factors basically include a proximal site of origin, large tumour size, presence of tumour recurrence and metastases.…”

Section: Introductionmentioning

confidence: 99%

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Metastatic sclerosing epithelioid fibrosarcoma in an adult male from a tertiary care centre in India: a case report

Santhosh¹,

Mehta²,

Barwad³

et al. 2022

ecancer

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Background: Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare subtype of soft tissue sarcoma and the data from India is sparse. It is an unusual variant of fibrosarcoma that commonly arises in the soft tissues of the limb, head and neck, trunk and occasionally in the visceral organs and bones. This entity is commonly reported in the middle age group, men and women alike. Pathological clinchers include MUC 4 (Mucin 4, cell surface associated) positivity by immunohistochemistry, FUS-CREB3L1 fusion and EWSR1 rearrangement. This disease is notoriously known for its local recurrence and metastatic spread. Response to systemic therapy is poor and relapses are frequent. The role of targeted and immunotherapy is not well defined. Case presentation:Here we report a 46-year-old gentleman who presented to the Sarcoma Medical Oncology Clinic in our centre. He had primary involvement of right pubic bone with metastasis to liver, lung and diffuse lytic bony lesions. His diagnosis was reviewed multiple times before coming to final diagnosis of SEF. His molecular test for EWSR1 rearrangement was positive by fluorescence in-situ hybridisation. He did not respond to palliative doxorubicin, pazopanib and gemcitabine and docetaxel. Conclusion:Through this case report, we would like to highlight the rarity of this sarcoma, its classical pathological features, its close relationship to low-grade fibromyxoid sarcoma and the limited therapeutic options available. Hence, there is a need for further research in this entity.

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Section: Discussioncontrasting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Metastatic sclerosing epithelioid fibrosarcoma in an adult male from a tertiary care centre in India: a case report

Santhosh¹,

Mehta²,

Barwad³

et al. 2022

ecancer

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Sclerosing epithelioid fibrosarcoma of the jaw: a case report and literature review

Amer,

Lazim,

Berstecher

et al. 2024

Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology

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Sclerosing Epithelioid Fibrosarcoma Harboring the EWSR1–CREB3L1 Gene Fusion: The Importance of Molecular Classification in Pediatric Sarcomas

Offenbacher,

Kaswan,

Fabish

et al. 2024

Journal of Pediatric Hematology/Oncology

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Background: Sclerosing epithelioid fibrosarcoma (SEF) is a very rare soft tissue sarcoma that most commonly presents in middle-aged and elderly adults but has been rarely seen in children. SEF is a very aggressive tumor with over 50% of patients experiencing local recurrence and 40% to 80% of patients experiencing distant metastatic spread. This disease has been shown to be resistant to chemotherapy and is classically treated with surgical excision. Case: We describe the case of a 10-year-old girl with Graves’ disease who presented with protruding eyes (to a greater extent on the left side) and was found to have a large mass in her left inferior rectus muscle that was diagnosed as SEF. After treatment with incomplete resection, due to the benign-appearing nature of the tumor on imaging, and proton radiation therapy, she remains disease-free at 18 months post-therapy. Discussion: SEF is typically identified via genetic testing and recognition of the EWSR1–CREB3L1 gene fusion as well as MUC4 expression via immunohistochemistry. DNA methylation profiling, which has traditionally been used in brain tumors, can also efficiently identify this tumor, and we recommend expanding the use of this technology for difficult to classify pediatric sarcomas.

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Primary sclerosing epithelioid fibrosarcoma presenting as lombosciatic syndrome: Case report and literature review

Cited by 3 publications

References 22 publications

Metastatic sclerosing epithelioid fibrosarcoma in an adult male from a tertiary care centre in India: a case report

Metastatic sclerosing epithelioid fibrosarcoma in an adult male from a tertiary care centre in India: a case report

Sclerosing epithelioid fibrosarcoma of the jaw: a case report and literature review

Sclerosing Epithelioid Fibrosarcoma Harboring the EWSR1–CREB3L1 Gene Fusion: The Importance of Molecular Classification in Pediatric Sarcomas

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