Sclerosing epithelioid fibrosarcoma

Reid, Richard; Barrett, A.; Hamblen, David L.

doi:10.1046/j.1365-2559.1996.342383.x

Cited by 51 publications

(54 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Positive results were found in 81% of putative LGFMS and only 7 of 52 (13%) controls; of these seven positive non-LGFMS, four had been previously labeled 'SEF'. Along similar lines, one of the 4 genetically confirmed LGFMS reported by Reid et al 69 showed at least in part classical features of SEF. Rehki et al 70 also reported a hybrid SEF-LGMFS showing FUS rearrangement.…”

Section: Genetic Features and Relationship To Lgfmssupporting

confidence: 66%

Selected topics in the pathology of epithelioid soft tissue tumors

Folpe

2014

Modern Pathology

View full text Add to dashboard Cite

Epithelioid morphology, mimicking carcinoma, is a key or defining feature of several soft tissue tumors and may be seen in a wide variety of other tumors. This review will focus on those tumors defined at least in part by their epithelioid morphology, in particular epithelioid sarcoma, epithelioid malignant peripheral nerve sheath tumor, and sclerosing epithelioid fibrosarcoma. The role of loss of the SMARCB1 tumor-suppressor gene in the pathogenesis of these epithelioid soft tissue tumors will be discussed, as will their differential diagnosis with non-mesenchymal tumors, in particular carcinoma and melanoma.

show abstract

Section: Genetic Features and Relationship To Lgfmssupporting

confidence: 66%

Selected topics in the pathology of epithelioid soft tissue tumors

Folpe

2014

Modern Pathology

View full text Add to dashboard Cite

show abstract

“…[1][2][3] This case is unusual, however, in three respects. It has areas of high grade morphology with large zones of tumour necrosis, surrounded by highly cellular and mitotically active epithelioid areas.…”

Section: Discussionmentioning

confidence: 99%

“…1 3 5 In our case, not only were these areas more extensive, but they were surrounded by more obviously malignant epithelioid areas, indicative of a higher grade tumour, a feature not described previously. Indeed previous publications [1][2][3] have stressed relatively bland cytological features and the risk of erroneous benign diagnoses.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5] The importance of ultrastructural examination in supporting a diagnosis made on morphological and immunohistochemical criteria has recently been highlighted. 3 We describe a further case of sclerosing epithelioid fibrosarcoma in which focal, histologically atypical areas were accompanied by immunohistochemical and ultrastructural features suggestive of nerve sheath, in particular perineurial, diVerentiation, and discuss the relevance of these findings with respect to diagnosis and possible histogenesis.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma

Hanson

Pearson²,

Eyden³

et al. 2001

Journal of Clinical Pathology

View full text Add to dashboard Cite

Sclerosing epithelioid fibrosarcoma is a recently described sarcoma in which ultrastructural evidence of fibroblastic differentiation forms part of the diagnostic criteria. This report describes a further case of this tumour, which showed evidence of both fibroblastic and perineurial diVerentiation by immunohistochemistry and electron microscopy, and which had areas of high grade morphology. The tumour metastasised and the patient died of disease 12 months after presentation. The relevance of these findings to diagnosis and diVerentiation in these tumours is discussed. (J Clin Pathol 2001;54:721-723) Keywords: sclerosing epithelioid fibrosarcoma; nerve sheath diVerentiation; high grade morphology Since publication of the first series of 25 cases of this distinctive rare variant of fibrosarcoma in 1995, 1 a further eight cases, including a single cytogenetic study, have been reported. 2-5The importance of ultrastructural examination in supporting a diagnosis made on morphological and immunohistochemical criteria has recently been highlighted. 3 We describe a further case of sclerosing epithelioid fibrosarcoma in which focal, histologically atypical areas were accompanied by immunohistochemical and ultrastructural features suggestive of nerve sheath, in particular perineurial, diVerentiation, and discuss the relevance of these findings with respect to diagnosis and possible histogenesis. Case reportA 54 year old man presented with a three month history of lower abdominal pain and distension. There was no other medical history of note. Clinical examination and radiology revealed a palpable abdominal mass, which appeared to arise from the pelvis. At laparotomy a large retroperitoneal mass was excised. The tumour was noted to displace but not to infiltrate the bladder, right external iliac vessels, and right ureter.Six weeks after surgery, the patient complained of lower backache. Imaging studies were considered highly suggestive of a lumbar metastasis. An isolated, probably metastatic, costal lesion was also identified. Local radiotherapy to the vertebra was followed by six courses of chemotherapy with adriamycin and ifosfamide.Ten months after primary surgery, he re-presented with severe instability pain of the lumbar spine secondary to his metastatic disease. Palliative vertebral excision was undertaken and subsequent orthopaedic reexploration revealed a recurrent pelvic tumour, together with peritoneal and hepatic metastases. The patient died 12 months after initial presentation. A necropsy was not performed. Macroscopic appearancesThe retroperitoneal tumour was a bosselated, firm, pale grey mass, which measured 140 × 95 × 80 mm and weighed 504 g. It had a variegated grey-white/yellow cut surface, with areas of yellow necrosis, and was partly covered by peritoneum. Representative tissue blocks were fixed in 10% neutral buVered formalin,

show abstract

“…All other purported positive markers have typically been either weakly, equivocally, or inconsistently expressed. Scattered, weak and inconsistent staining has been reported with CD34, S-100, CAM 5.2, bcl-2, and EMA [1,2,8,[12][13][14]. A positive CD99 stain led Folk et al [5] to perform RT-PCR for the t(11; 22)(q24; q12) translocation in two cases, which were both negative.…”

Section: Discussionmentioning

confidence: 99%

Sclerosing Epithelioid Fibrosarcoma of the Oral Cavity

Elkins

Wakely

2011

Head and Neck Pathol

View full text Add to dashboard Cite

Sclerosing epithelioid fibrosarcoma (SEF) rarely occurs outside the somatic soft tissue. Until recently no consistently specific genetic alteration had been associated with SEF. Molecular testing of the FUS gene rearrangement involving chromosome 16 [at one time considered specific for low-grade fibromyxoid sarcoma (LGFMS) and its variant, LGFMS with giant collagen rosettes), may be a nonrandom abnormality in some cases of SEF.We present an example of a rare FUS-positive SEF that arose in the floor of mouth of a 56 year old male. Light microscopy, exhaustive immunohistology, and FISH examination showing chromosome rearrangement using the FUS break-apart probe led to an erroneous diagnosis of LGFMS with giant collagen rosettes. An outside expert agreed with that diagnosis citing the FISH results as confirmatory. Upon review almost 2 years later after local recurrence, the classic histopathologic features of SEF were noted instead. This example suggests that at least a subset if not most examples of SEF are part of the LGFMS ''family'' of neoplasms, and reiterates the value of careful histologic examination in an age of increasingly sophisticated and presumably specific molecular results.

show abstract

Sclerosing epithelioid fibrosarcoma

Cited by 51 publications

References 0 publications

Selected topics in the pathology of epithelioid soft tissue tumors

Selected topics in the pathology of epithelioid soft tissue tumors

Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma

Sclerosing Epithelioid Fibrosarcoma of the Oral Cavity

Contact Info

Product

Resources

About