Sclerosing epithelioid fibrosarcoma as a rare cause of ascites in a young man: A case report

North Cheshire NHS Trust, Warrington Hospital, Cheshire, UK.
Journal of Medical Case Reports 07/2008; 2(1):248. DOI: 10.1186/1752-1947-2-248
Source: PubMed


Sclerosing epithelioid fibrosarcoma is a rare but distinct variant of fibrosarcoma that not only presents as a deep-seated mass on the limbs and neck but can also occur adjacent to the fascia or peritoneum, as well as the trunk and spine. We report the case of an intra-abdominal sclerosing epithelioid fibrosarcoma, which to best of the authors' knowledge has not been described previously. The patient discussed here developed lung metastases but is still alive 1-year post-diagnosis.
A 29-year-old man presented with a 2-week history of progressive abdominal distension and pain and was found to have marked ascites. A full liver screen was unremarkable with abdominal and chest computed tomography scans only confirming ascites. After a diagnostic laparotomy, biopsies were taken from the greater omentum and peritoneal nodules. Histopathology revealed a malignant tumour composed of sheets and cords of small round cells set in collagenized stroma. After further molecular investigation at the Mayo Clinic, USA, the diagnosis of a high-grade sclerosing epithelioid fibrosarcoma was confirmed.
Sclerosing epithelioid fibrosarcoma is an extremely rare tumour, which is often difficult to diagnose and which few pathologists have encountered. This case is particularly unusual because of the intra-abdominal origin of the tumour. Owing to the rarity of sclerosing epithelioid fibrosarcoma, there is no clear evidence regarding the prognosis of such a tumour, although sclerosing epithelioid fibrosarcoma is able to metastasize many years post-presentation. It is important that physicians and pathologists are aware of this unusual tumour.

Full-text preview

Available from: PubMed Central
  • Source
    • "Sclerosing epithelioid fibrosarcoma – histopathologically a benign tumor, clinically with high potential for infiltration and metastasis [47]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Cardiac tumors are assumed to be a rare entity. Metastases to the heart are more frequent than primary lesions. Sarcomas make up the majority of cardiac malignant neoplasms. Among them angiosarcoma is the most common and associated with the worst prognosis. Malignant fibrous histiocytoma comprises the minority of cardiac sarcomas and has uncertain etiology as well as pathogenesis. Transthoracic echocardiography remains the widely available screening examination for the initial diagnosis of a cardiac tumor. The clinical presentation is non-specific and the diagnosis is established usually at an advanced stage of the disease. Sarcomas spread preferentially through blood due to their immature vessels without endothelial lining. Surgery remains the method of choice for treatment. Radicalness of the excision is still the most valuable prognostic factor. Adjuvant therapy is unlikely to be effective. The management of cardiac sarcomas must be individualized due to their rarity and significant differences in the course of disease.
    Archives of Medical Science 02/2014; 10(1):135-48. DOI:10.5114/aoms.2014.40741 · 2.03 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma, described initially by Meis-Kindblom et al. in 1995 (Meis-Kindblom JM, Kindblom L-G, Enzinger FM. Sclerosing epithelioid fibrosarcoma: a variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19:979-993): more than 80 cases have been documented clinicopathologically since. Bone is a rare primary site for SEF, with only 2 cases so far reported. This paper documents the detailed clinical, histological, immunohistochemical, and ultrastructural features of a case occurring in the pubic bone of a 57-year-old diabetic woman presenting with a history of pain and compromised mobility involving her hip. Radiology revealed a destructive lesion in the right pubic bone. The lesion was resected, and 7 months postoperatively it recurred. The patient died following metastases to multiple bony sites and liver, some 4 years after the onset of symptoms. Histologically, the tumor was consistent with SEF, although it showed some anomalous immunostaining, which, however, is typical of the tumor (for example, for S-100 protein and epithelial membrane antigen). By electron microscopy, some rough endoplasmic reticulum was present, but also tonofibrils and desmosomes. The overall features were of an SEF with the ultrastructural but incomplete immunohistochemical evidence for divergent epithelial differentiation. The differential diagnosis of this tumor is discussed.
    Ultrastructural Pathology 04/2010; 34(2):99-104. DOI:10.3109/01913121003605576 · 1.08 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis), there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.
    Sarcoma 04/2010; 2010:431627. DOI:10.1155/2010/431627
Show more