Metastatic Sclerosing Epithelioid Fibrosarcoma in Bone Marrow

Sclerosing epithelioid fibrosarcoma (SEF) is an aggressive neoplasm thought to be related to low-grade fibromyxoid sarcoma, which typically occurs in middle-aged adults in the deep soft tissues of the lower extremities and trunk. It comprises nests and cords of relatively uniform epithelioid polygonal cells with clear or eosinophilic cytoplasm in densely sclerotic stroma, and it is typically associated with EWSR1 gene rearrangements, and most commonly EWSR1-CREB3L1 fusions. As primary SEF can arise in bone, and bone is also a common metastatic site for SEF, its recognition at this site is important. We illustrate bone marrow showing diffuse infiltration by SEF and highlight the potential for confusion with a range of neoplasms such as carcinoma, hematolymphoid neoplasms, and other sarcomas.

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Hybrid Low-Grade Fibromyxoid Sarcoma and Sclerosing Epithelioid Fibrosarcoma of the Pancreas

Journal of Gastrointestinal Cancer ◽

10.1007/s12029-020-00369-0 ◽

2020 ◽

Vol 51 (3) ◽

pp. 1025-1029

Author(s):

Sage P Kramer ◽

Christopher J Bowman ◽

Zhen J Wang ◽

Kathleen M Sheahon ◽

Eric K Nakakura ◽

...

Keyword(s):

Low Grade ◽

Sclerosing Epithelioid Fibrosarcoma ◽

Fibromyxoid Sarcoma

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SCLEROSING EPITHELIOID FIBROSARCOMA

The Professional Medical Journal ◽

10.29309/tpmj/2015.22.03.1359 ◽

2015 ◽

Vol 22 (03) ◽

pp. 370-373

Author(s):

Xiang Longquan ◽

Henry Mwakyoma

Keyword(s):

Light Microscopy ◽

Subcutaneous Tissue ◽

Clinical Analysis ◽

Low Grade ◽

Review Of The Literature ◽

Epithelioid Cells ◽

Sclerosing Epithelioid Fibrosarcoma ◽

Eosinophilic Cytoplasm

Sclerosing epithelioid fibrosarcoma (SEF) is a rare subtype of Fibrosarcomaand was first reported in 1995. This tumour is rarely encountered and can easily be mistaken byclinicians for other diagnoses. This paper reports one case of SEF and review related literature.Objectives: To determine the clinical pathologic characteristics and discuss differentialdiagnosis of sclerosing epithelioid fibrosarcoma (SEF). Methods: One case of SEF was studiedby clinical analysis, light microscopy and the review of the literature. Results: The patient wasan adult and had a tumor located in the subcutaneous tissue of the left leg, which waspainless and grew slowly. Macroscopically, it was described as nodular and non-encapsulated.The cut section was gray-white, firm to elastic in consistency. The microscopic examinationshowed that the round to ovoid epithelioid cells with clear or eosinophilic cytoplasm arrangedin cords, nests, sheets or alveolar pattern. The stroma showed a dense hyalinized collagenousbackground. Conclusions: Sclerosing epithelioid fibrosarcoma is a low-grade variant offibrosarcoma, histologically, it should be differentiated from a variety of tumors which have theepithelioid appearance and sclerosing stroma.

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Hybrid sclerosing epithelioid fibrosarcoma/low grade fibromyxoid sarcoma arising in the small intestine with distinct HEY1-NCOA2 gene fusion

Pathology ◽

10.1016/j.pathol.2020.04.010 ◽

2020 ◽

Vol 52 (5) ◽

pp. 607-610

Author(s):

Khaled A. Murshed ◽

Adham Ammar

Keyword(s):

Small Intestine ◽

Gene Fusion ◽

Low Grade ◽

Sclerosing Epithelioid Fibrosarcoma ◽

Fibromyxoid Sarcoma

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Sclerosing Epithelioid Fibrosarcoma of the Cecum: A Radiation-Associated Tumor in a Previously Unreported Site

Archives of Pathology & Laboratory Medicine ◽

10.5858/2007-131-1825-sefotc ◽

2007 ◽

Vol 131 (12) ◽

pp. 1825-1828 ◽

Cited By ~ 5

Author(s):

Jared C. Frattini ◽

Julie Ann Sosa ◽

Susanne Carmack ◽

Marie E. Robert

Keyword(s):

Ionizing Radiation ◽

Nuclear Reactor ◽

Soft Tissues ◽

Atomic Bomb ◽

External Beam Radiation ◽

Low Grade ◽

Beam Radiation ◽

Sclerosing Epithelioid Fibrosarcoma ◽

First Case ◽

Radiation Induced

Abstract Data from the nuclear reactor explosion in Chernobyl and the atomic bomb detonations in Hiroshima and Nagasaki demonstrated an association between ionizing radiation and tumoriogenesis. There is a significant association between external beam radiation and radiation-induced sarcoma. Sclerosing epithelioid fibrosarcoma is a rare form of malignant fibrosarcoma that is low grade and indolent with distinct immunohistopathologic characteristics that usually occurs in the soft tissues of the extremities. A 62-year-old man from Kiev who aided in the cleanup at Chernobyl presented with crampy abdominal pain, nausea, and vomiting. His workup revealed a cecal mass, and the final pathology from his laparotomy confirmed sclerosing epithelioid fibrosarcoma with metastasis to the liver. In addition to a review of the literature, we report the first case of sclerosing epithelioid fibrosarcoma arising from the large bowel. Exposure to ionizing radiation from Chernobyl could have played a role in the development of his tumor.

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The cytological features of low-grade fibromyxoid sarcoma as a hybrid tumor with sclerosing epithelioid fibrosarcoma

The Journal of the Japanese Society of Clinical Cytology ◽

10.5795/jjscc.60.272 ◽

2021 ◽

Vol 60 (5) ◽

pp. 272-278

Author(s):

Akiko YOSHIDA ◽

Shinji UMANO ◽

Shizuki TAKEMURA ◽

Suzuko MORITANI ◽

Ryoji KUSHIMA

Keyword(s):

Low Grade ◽

Sclerosing Epithelioid Fibrosarcoma ◽

Fibromyxoid Sarcoma ◽

Cytological Features ◽

Hybrid Tumor

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Hairy Cell Lymphoma: A Potentially Under-Recognized Entity

Rare Tumors ◽

10.4081/rt.2017.6518 ◽

2016 ◽

Vol 9 (1) ◽

pp. 1-3 ◽

Cited By ~ 3

Author(s):

William A. Hammond ◽

Abhisek Swaika ◽

David Menke ◽

Han W. Tun

Keyword(s):

Bone Marrow ◽

Iliac Crest ◽

Soft Tissues ◽

Cell Lymphoma ◽

Bone Marrow Involvement ◽

Leukemic Cells ◽

Low Grade ◽

Hairy Cell ◽

Cell Leukemia ◽

Diagnostic Pitfalls

Hairy cell leukemia (HCL) is a low grade B-cell lymphoproliferative disorder that typically presents with splenomegaly, cytopenias, and diffuse bone marrow infiltration. There have been few cases in the literature of HCL presenting as lymphomas in extra-nodal locations, such as soft tissues and bones without circulating leukemic cells, splenomegaly, or iliac crest bone marrow involvement. We present an additional case presenting as a thoracic mass, and discuss potential diagnostic pitfalls and management of these rare cases.

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Sclerosing Epithelioid Fibrosarcoma of the Kidney: First Reported Case in a Young Child

Pediatric and Developmental Pathology ◽

10.1177/1093526620977738 ◽

2021 ◽

pp. 109352662097773

Author(s):

Justin L Kurtz ◽

Serena Y Tan ◽

Florette K Hazard

Keyword(s):

Clinical Course ◽

Distant Metastases ◽

Renal Tumors ◽

Low Grade ◽

Visceral Organ ◽

Deep Soft Tissue ◽

Sclerosing Epithelioid Fibrosarcoma ◽

Patient Reported ◽

Aggressive Clinical Course ◽

Middle Aged Adults

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma primarily arising in the deep soft tissue of the extremities and trunk. Despite having the morphologic appearance of a low-grade sarcoma, it generally has an aggressive clinical course with frequent local recurrences and distant metastases. It typically occurs in middle aged adults and is characterized by immunoexpression of MUC4 and recurrent gene fusions, most commonly EWSR1-CREB3L1. We report a primary renal SEF in a 4-year-old male. To our knowledge, this is the youngest patient reported with SEF and the second case of SEF in a pre-adolescent child. It is the eleventh reported case of primary renal SEF in the literature. While SEF arising in visceral organs is rare, the kidney is the most common primary site of any visceral organ. This case demonstrates SEF can occur in pre-adolescents, is an important consideration when evaluating sarcomas in young children, and should be considered in the differential diagnosis for primary renal tumors.

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Giant Low-Grade Fibromyxoid Sarcoma in the Neck

Korean Journal of Otorhinolaryngology - Head and Neck Surgery ◽

10.3342/kjorl-hns.2019.00857 ◽

2020 ◽

Vol 63 (9) ◽

pp. 432-435

Author(s):

Jong Min Park ◽

Hye Rin Lim ◽

Jo Heon Kim ◽

Dong Hoon Lee

Keyword(s):

Neck Region ◽

Surgical Excision ◽

Late Recurrence ◽

Low Grade ◽

Head And Neck Region ◽

Long Term Follow Up ◽

Fibromyxoid Sarcoma ◽

Middle Aged Adults

Low-grade fibromyxoid sarcoma (LGFS) is a soft tissue tumor that rarely occurs in the head and neck region. It occurs mainly in the proximal extremities and the trunk and is prevalent in the young and middle-aged adults. In the present case, LGFS was present at an atypical location and at an unusual age. The treatment of choice for LGFS is radical wide surgical excision with a clear margin. Long-term follow-up is essential for all patients with LGFS, as it has the potential for late recurrence or metastasis.

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