Case report | Volume 36, Article 131, 25 Jun 2020 | 10.11604/pamj.2020.36.131.18668

Sclerosing epithelioid fibrosarcoma: rare and serious

Mounir Yahyaoui, Mohammed Benhammou, Soufiane Aharram, Jawad Amghar, Mohammed Sadougui, Omar Agoumi, Abdelkrim Daoudi

Corresponding author: Mounir Yahyaoui, A Trauma-Orthopedics Service, University Hospital Mohammed 6th Oujda, Morocco

Received: 13 Mar 2019 - Accepted: 19 Jun 2019 - Published: 25 Jun 2020

Domain: Surgical oncology

Keywords: Sclerosing epithelioid fibrosarcoma, surgery, adjuvant treatment, recurrences, metastases

©Mounir Yahyaoui et al. Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cite this article: Mounir Yahyaoui et al. Sclerosing epithelioid fibrosarcoma: rare and serious. Pan African Medical Journal. 2020;36:131. [doi: 10.11604/pamj.2020.36.131.18668]

Available online at: https://www.panafrican-med-journal.com/content/article/36/131/full

Home | Volume 36 | Article number 131

Case report

Sclerosing epithelioid fibrosarcoma: rare and serious

Sclerosing epithelioid fibrosarcoma: rare and serious

Mounir Yahyaoui1,&, Mohammed Benhammou1, Soufiane Aharram1, Jawad Amghar1, Mohammed Sadougui1, Omar Agoumi1, Abdelkrim Daoudi1

 

1A Trauma-Orthopedics Service, University Hospital Mohammed 6th Oujda, Morocco

 

 

&Corresponding author
Mounir Yahyaoui, A Trauma-Orthopedics Service, University Hospital Mohammed 6th Oujda, Morocco

 

 

Abstract

Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare form of soft tissue sarcoma. It is characterized by a slow evolution, with local recurrences and late metastases that are mainly pulmonary and pleural in about 50% of cases. The treatment is based on the surgery which must be as wide as possible. The efficacy of adjuvant therapy in the control of SEF is not yet demonstrated. Chemotherapy is used in recurrences, some have also proposed radiotherapy. Long-term follow-up of patients with SEF is therefore essential.

 

 

Introduction    Down

Enzinger and Weiss were the first in 1988 to describe a variant of fibrosarcoma with a predominance of epithelioid cells [1]. In 1995, Meis-Kindblom et al. reported a series of 25 cases and introduced the term "sclerosing epithelioid fibrosarcoma" (SEF) [2]. This new histological entity is distinguished from other fibrosarcomas by slow growth. Histological diagnosis can be difficult, and the differential diagnosis should be discussed with benign soft tissue tumors, metastatic carcinomas, and high grade sarcomas. Of the 50 cases reported in the literature, local recurrences and distant metastases have been described [2, 3]. Metastases are rare and usually occur several years after the initial surgical treatment.

 

 

Patient and observation Up    Down

M.M female, had for 20 years a mass of the inner side of the right thigh painless, gradually increasing in volume, and evolving in a context of apyrexia and conservation of the general state. The clinical examination showed a mass at the antero-internal surface of the right thigh (Figure 1) of 18/12cm, well limited, firm consistency, non-flapping, painless on palpation, mobile in relation to the superficial plane and adherent to the deep one, with no inflammatory signs or satellite lymphadenopathies with a normal neurovascular examination. The patient underwent standard radiography of the thigh (Figure 2) revealing opacity of the soft tissues, and MRI (Figure 3) which showed a sub fascial mass of the inner side of the right thigh, well-defined, contours irregular in places, in heterogeneous hypersignal T2/isosignal T1, closing areas in hyposignal T1 and T2 in relation to calcifications, and raising intensively and heterogeneously after injection of contrast product. This mass drove the superficial veins and respected the neighboring muscular structures. She first benefited from a surgical biopsy with regard to the mass (Figure 4) whose pathology showed epithelioid cells sometimes fusiform arranged in bundles or single file and marked by fibrosis, the nuclei were chromatic with a Abundant cytoplasm, the figures of the mitoses were few. Immunohistochemically, cells expressed EMA, but were negative for AE1/AE3, PS100, Desmin, AML. It is therefore a FLNCC grade II of sclerosing epithelioid fibrosarcoma (Figure 5). The extension assessment was negative (thoraco-abdominopelvic CT and bone scintigraphy), and after a multidisciplinary consultation meetings, the decision was a first surgery. A large excision was made taking the path of the biopsy including without seeing the tumor with a margin of safety of 4cm reaching the superficial plane of sartorius (Figure 6). The anatomopathological result confirmed the same diagnosis with resection margins at R0. The patient is a candidate for adjuvant radiotherapy.

 

 

Discussion Up    Down

Sarcomas are malignant tumors with mesenchymal differentiation. Fibrosarcoma accounts for 1 to 2% of all sarcomas [4]. There are several variants, including sclerosing epithelioid fibrosarcoma, which is a rare variant of poor prognosis [5]. It is composed of epithelioid cells arranged in cell clusters or cords in an abundant hyalinized collagenic stroma [6]. Among the 50 cases reported in the literature [2, 3], there were 26 women for 24 men, with an average age of 45 years (range: 14-87 years); only 3 patients were under 20 years old. Delayed diagnosis is common in non-specific symptoms such as trailing pain. SEF can reach all parts of the body, but with preferential localization for the proximal limb, trunk, head and neck [2, 3]. SEF is considered a low grade sarcoma. Local recurrences and metastases are not uncommon. The local recurrence rate is about 50%, with an average delay of 3.5 years (range: 2 months-11 years). Metastases occur in 43 to 86% of cases, with a delay of 3 to 14 years [2, 3]. They are localized preferably in the lungs, the pleura and the bone. The diagnostic and therapeutic strategy of soft tissue tumors is currently well codified. It depends on the size and superficial or deep nature of the tumor. Biopsy excision of a small soft tissue tumor as soon as it is superficial may be considered. If the tumor is large as in our case, a locoregional extension assessment must be performed before any surgical procedure. It is essentially based on an MRI that can guide the biopsy and plan surgical resection. The preliminary surgical biopsy, which must be performed without risk of dissemination, establishes the benign or malignant nature of the tumor. In addition, it makes possible, as far as possible, typing and histological grading for sarcomas [7, 8]. The treatment is based on surgery which must be as wide as possible [9]. The efficacy of adjuvant therapy in the control of SEF is not yet demonstrated [10]. Chemotherapy is used in cases of recurrence based on a combination of several molecules including adriamycin and ifosfamide [11-13]. Some also proposed radiotherapy [13]. Regular and long-term follow-up is recommended since recurrences or distant metastases can be seen late [2].

 

 

Conclusion Up    Down

SEF is a rare form of soft tissue sarcoma. It is characterized by a slow evolution, with local recurrences and late metastases that are mainly pulmonary and pleural in about 50% of cases. Long-term follow-up of patients with SEF is therefore essential.

 

 

Competing interests Up    Down

The authors declare no competing interests.

 

 

Authors´ contributions Up    Down

All authors contributed to the conduct of this research and read and approved the final version of the manuscript.

 

 

Figures Up    Down

Figure 1: clinical aspect of the mass

Figure 2: X-ray of the thigh

Figure 3: MRI appearance of the mass

Figure 4: biopsy first

Figure 5: anatomopathological result

Figure 6: wide excision of the tumor

 

 

References Up    Down

  1. Enzinger FM. Fibrosarcoma. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. 2nd ed. St Louis, MO: Mosby, 1988:201-22.

  2. Meis-Kindblom JM, Kindblom LG, Enzinger FM. Sclerosing epithelioid fibrosarcoma: a variant of fibrosarcoma simulating carcinoma. Am J Surg Pathol. 1995;19(9):973-93. PubMed | Google Scholar

  3. Battiata P, Casler J. Sclerosing epithelioid fibrosarcoma: a case report. Ann Otol Rhinol Laryngol. 2005;114(2):87-9. PubMed | Google Scholar

  4. Fisher C. Soft tissue sarcomas: diagnosis, classification and prognostic factors. Br J Plast Surg. 1996;49(1):27-33. PubMed | Google Scholar

  5. Genevay M, Coindre J-M, Guillou L. Entités récentes en pathologie tumorale des tissues mous. 2e partie. Ann Pathol. 2003;23:135-48.

  6. Meis-Kindblom JM, van den Berg E, Kindblom LG, Molenaar WM. Sclerosing epitheliod fibrosarcoma:106-107. In: Word Health Organization classification of tumors-pathology and genetics tumors of soft tissue and bone. IARC Press: Lyon, 2002.

  7. Fletcher CDM, Rydholm A, Singer S, Sundaram M, Coindre JM. Soft tissue tumours: epidemiology, clinical features, histopathological typing and grading:12-18. In: Word Health Organization classification of tumors-pathology and genetics tumors of soft tissue and bone. IARC Press: Lyon, 2005.

  8. Dujardin F, Debled M, Guillemet C, Simonet J, Hamidou H, Cambon-Michot C et al. Prise en charge des tumeurs des parties molles de l'appareil locomoteur de l'adulte. Rev Chir Orthop. 2006;92(7):637-50. Google Scholar

  9. Enzinger FM, Weiss SW, Goldblum JR. Soft tissue tumors. 4th ed. Mosby: St. Louis; 2001.

  10. Bilsky MH, Schefler AC, Sandberg DI, Dunkel IJ, Rosenblum MK. Sclerosing epitheliod fibrosarcomas involving the neuraxis: report of three cases. Neurosurgery. 2000 Oct;47(4):956-9; discussion 959-60. PubMed | Google Scholar

  11. Reid R, Barrett A, Hamblen DL. Sclerosing epithelioid fibrosarcoma. Histopathology. 1996 May;28(5):451-5. PubMed | Google Scholar

  12. Arnould L, Jouannelle C, Mege F, Maillefert F, Fargeot P, Devillebichot C, Collin F. Le fibrosarcome épithelioïde sclérosant: un fibrosarcome d´évolution très lente. Ann Pathol. 2000;20:154-7. Google Scholar

  13. Antonescu CR, Rosenblum MK, Pereira P, Nascimento A, Woodruff JM. Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of clinicopathologically distinct tumor. Am J Surg Pathol. 2001;25(6):699-709. PubMed | Google Scholar

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Case report

Sclerosing epithelioid fibrosarcoma: rare and serious

Case report

Sclerosing epithelioid fibrosarcoma: rare and serious

Case report

Sclerosing epithelioid fibrosarcoma: rare and serious