Spinal Sclerosing Epithelioid Fibrosarcoma at the Cervicothoracic Junction

Zhang, Jing; Chou, Dean

doi:10.1016/j.wneu.2018.03.105

Cited by 5 publications

(12 citation statements)

References 26 publications

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“…SEF most commonly originates in the deep soft tissues of the extremities and limb girdles. Primary intraosseous SEFs are rare 6,11,14–18 . We found in our literature search that, including the cases in our series, 40 primary SEFs of bone have been reported in the English‐language literature, as single case reports and two series 5–18 .…”

Section: Discussionmentioning

confidence: 66%

“…We found in our literature search that, including the cases in our series, 40 primary SEFs of bone have been reported in the English‐language literature, as single case reports and two series 5–18 . Fourteen of these 40 (35%) originated in the spine, 6,11,14–18 13 in the long bones of the extremities (33%), 9,11,16,18 nine in the skull and craniofacial bones (22%), 7,10,12,13,16,18 two in the pelvic bones (5%), 5,8 and two in the ribs (5%) 11,18 …”

Section: Discussionmentioning

confidence: 86%

“…Importantly, when considering all of the intraosseous SEFs reported in the literature, we observed a higher rate of disease‐specific death for spinal SEF than for SEF arising in other bone sites (50% versus 33%). We speculated that this is probably due to site‐specific prognostic implications related to surgical treatment 5–18 …”

Section: Discussionmentioning

confidence: 99%

“…SEF presents most often as a deep‐seated intramuscular mass located in the lower extremities or pelvic girdle, followed by the trunk, upper extremities, shoulder girdle, and head and neck region 1 . SEFs can also rarely occur as primary bone neoplasms, 5–16 including some arising in the spine 6,11,14–18 . Primary SEF of the spine is diagnostically challenging, owing to its extreme rarity and morphological overlap with other mesenchymal and non‐mesenchymal tumours.…”

Section: Introductionmentioning

confidence: 99%

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Primary sclerosing epithelioid fibrosarcoma of the spine: a single‐institution experience

et al. 2021

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Aims To present our experience on spinal sclerosing epithelioid fibrosarcoma (SEF) and review the existing literature pertaining to SEF of the spine. Methods and results Six cases of spinal SEF were reviewed, and a literature search of all primary SEFs of the spine was performed. All tumours occurred in adults (median age, 41 years) and were located all along the spine, the lumbar vertebrae being the most commonly involved. All patients presented with pain that they had experienced for months. The mean tumour size at diagnosis was 52 mm. Five tumours showed a spectrum of microscopic features consistent with pure SEF, and one showed a hybrid morphology with areas of low‐grade fibromyxoid sarcoma. All were diffusely and strongly positive for mucin 4. Two cases were initially misdiagnosed as epithelioid haemangioendothelioma and aggressive chondroblastoma. Fluorescence in‐situ hybridisation showed rearrangements of either FUS or EWSR1 in four cases. Reverse transcription polymerase chain reaction showed the presence of FUS–CREB3L1 and EWSR1–CREB3L1 fusion transcripts in two cases and one case, respectively. Of five patients with follow‐up data available, two developed one or more local recurrences and three patients had metastatic disease. Distant metastases were mainly to other osseous locations, followed by lungs and lymph nodes. At last follow‐up, three patients had died of disease and one was alive with multiple metastases. Conclusions SEF is an aggressive sarcoma that can involve the spine. It is important to recognise the spine as the primary location of SEF, in order to avoid misdiagnosis as more common primary spinal neoplasms, which can impact on therapeutic approaches.

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Section: Discussionmentioning

confidence: 66%

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Primary sclerosing epithelioid fibrosarcoma of the spine: a single‐institution experience

et al. 2021

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“…It has been reported to have relatively high incidence of local recurrence (>50% of cases), metastasis (40-80% of cases) and tumor-related mortality (2,3). SEF typically involves the extremities or the torso (2), followed by the abdominal viscera (4,5) and head and neck areas (6)(7)(8). Primary SEF in the bone was first reported in 2001 (9).…”

Section: Introductionmentioning

confidence: 99%