A rare tumour in the pelvis presenting with lower urinary symptoms: «sclerosing epithelioid fibrosarcoma»

Arya, Manit; García-Montes, F.; Patel, Hitesh; Emberton, Mark; Mundy, Anthony R.

doi:10.1053/ejso.2000.1023

Cited by 15 publications

(14 citation statements)

References 2 publications

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“…[2][3][4][5][6][7][8][9][10][11][12] In particular, in the recent series of 16 patients by Antonescu et al, 9 the authors concluded that sclerosing epithelioid fibrosarcoma is a clinicopathologically distinct low grade fibrosarcoma that is closely related to low grade fibromyxoid sarcoma [13][14][15] and hyalinising spindle cell tumour with giant rosettes. [16][17][18][19][20] These three tumours belong to the same family of so called ''fibrosing fibrosarcomas'', which are sarcomas that exhibit fibroblastic differentiation and show prominent widespread fibrosis readily evident both grossly and microscopically.…”

Section: Discussionmentioning

confidence: 99%

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Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

Chow

2004

Journal of Clinical Pathology

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Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the tumour were followed by metastatic recurrence of the tumour in the lung and scalp six years after initial presentation. The tumour showed typical histology of sclerosing epithelioid fibrosarcoma. The radiological features confirmed its primary location in the sacrum. The patient declined chemotherapy and died of disseminated disease eight years after initial presentation. Review of the literature confirms the fact that sclerosing epithelioid fibrosarcoma, despite its low grade, is a clinicopathologically distinct tumour with full malignant potential, the recurrence, metastasis, and mortality rate being 48%, 60%, and 35%, respectively. Sclerosing epithelioid fibrosarcoma can occur as a primary bone tumour, the clinical behaviour of which is probably similar to its soft tissue counterpart.

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Section: Discussionmentioning

confidence: 99%

“…Following their description, there were a few series and case reports validating their findings. [2][3][4][5][6][7][8][9][10][11][12] In this article, we report a patient with sclerosing epithelioid fibrosarcoma arising from the sacrum, and its clinical course is described and compared with that in the soft tissue.…”

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confidence: 99%

Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

Chow

2004

Journal of Clinical Pathology

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“…Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare soft‐tissue neoplasm. Since the first description of this lesion in 1995 by Meis‐Kindblom et al, 1 a few additional reports have been published in the English literature 2–9 . This tumor has been described as a variant of fibrosarcoma with a histology characterized by small‐ to medium‐sized round or ovoid cells with pale cytoplasm, arranged in cords or nests separated by a prominent sclerotic hyalinized matrix.…”

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confidence: 99%

“…SEF usually arise in the deep soft tissue and are frequently associated with the adjacent fascia or periosteum. Most lesions are located in the lower extremities, limb girdles and trunk 1–9 . Recently, three cases of SEF involving the neuraxis have been reported 7 .…”

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confidence: 99%

Overexpression of MDM2 in a sclerosing epithelioid fibrosarcoma: Genetic, immunohistochemical and ultrastructural study of a case

Jiao

Nakamura

Sugai

et al. 2002

Pathology International

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Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare soft-tissue neoplasm. Here, we describe the molecular genetic alterations and histological, immunohistochemical and ultrastructural features of a primary SEF arising in the retroperitoneum. The tumor consisted of uniform small round to ovoid epithelioid cells, arranged in nests and cords and surrounded by a prominent hyalinized collagenous matrix. The tumor cells expressed only vimentin. Ultrastructurally, the tumor cells showed features of fibroblasts, with an abundant rough endoplasmic reticulum in the cytoplasm. Neither p53 gene mutations nor p53 protein overexpression were detected, but more than 70% of all tumor cells showed strong immunoreactivity with murine double minute 2 (MDM2). Our results suggest that MDM2 overexpression is likely to play a role in tumorigenesis in this lesion in p53-dependent or p53-independent pathways. To our knowledge, the present study is the first molecular genetic study of this rare lesion. Further studies will be necessary to clarify the molecular basis of tumorigenesis of this rare lesion.

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“…1 Other primary sites have been described in the literature, including the pituitary gland, oral cavity, lung, bone, intra-abdominal, liver, pancreas, cecum, ovary, penis, and neuraxis. [2][3][4][5][6][7][8][9][10][11][12][13] Despite being histologically low-grade, SEF has a high local recurrence rate. Originally reported as a rate of 53% at a median followup of 4.8 years, variable rates (30-50%) have been described due to inconsistent reporting of followup.…”

Section: Discussionmentioning

confidence: 99%

Sclerosing epithelioid fibrosarcoma metastasizing to the penile shaft

Bell

Brimo

Jung

et al. 2016

CUAJ

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We present the case of a 50-year-old man with a periurethral mass. He was previously known for sclerosing epithelioid fibrosarcoma (SEF) of the left foot, having an amputation for local recurrence with >2 cm negative margins. A solid periurethral mass was surgically excised seven months later, yielding the diagnosis of metastatic SEF. This is the first documented metastasis of SEF to the penis. These sarcomas have proven difficult to treat, with high recurrence rates despite a multimodal approach.

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A rare tumour in the pelvis presenting with lower urinary symptoms: «sclerosing epithelioid fibrosarcoma»

Cited by 15 publications

References 2 publications

Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

Overexpression of MDM2 in a sclerosing epithelioid fibrosarcoma: Genetic, immunohistochemical and ultrastructural study of a case

Sclerosing epithelioid fibrosarcoma metastasizing to the penile shaft

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