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

Abstract

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,

A Primary Sclerosing Epithelioid Fibrosarcoma of the Pubic Bone, with Evidence of Divergent Epithelial Differentiation

“…The most convincingly demonstrated cases of SEF are those with the typical histology, a fibroblastic ultrastructure (rER and collagen secretion granules [3]) and an immunophenotype also of fibroblastic markers only (vimentin). However, any group of tumors can show phenotypic heterogeneity and a variety of essentially nonfibroblastic markers have been reported in SEF: EMA, S-100 protein, NSE, and cytokeratin [1,2,4,5,7,8,11,12,14,15,18,21,24,25]. Therefore, the immunostaining in our case for not only vimentin but also EMA, S-100, CD34, CD99, and NSE is consistent with the literature [1,8] and indicative of a complex and divergent phenotype.…”

Sclerosing Epithelioid Fibrosarcoma – A Rare Sacral Lesion and Literature Review

“…However, concerning this controversial definition, recently, WHO "Classification of Tumours of Soft Tissue and Bone" review (2013), includes sclerosing epithelioid fibrosarcoma, on malignant Fibroblastic/Myofibroblastic tumours, along with adult fibrosarcoma, myxofibrosarcoma and low-grade fibromyxoid sarcoma [3]. A propensity for local recurrence and late metastasis, findings of necrosis and large anaplastic figures, and an accumulation of p53 by immunohistochemistry that is paradoxically always found, corroborate this classification [9,13,14].…”

“…It lacks immunoreactivity for epithelial membrane antigen; cytokeratins (CAM5.2 or AE1/3), HMB-45, neuronspecific enolase, desmin, smooth muscle actin, or muscle common actin (HHF-35) [8] Focal and weak immunostaining for EMA, S-100 protein and more rarely for cytokeratins may be seen in a minority of cases [19]. Despite this uncommon report, other entities, like clear cell sarcoma and malignant peripheral nerve sheath tumour may need to be distinguished from SEF given that all these tumours may express S-100 protein [4,14].…”

“…This is particularly difficult to the diagnosis on visceral organs because of its rarity and its epithelioid appearance, closely mimicking carcinomas [18]. A strong and diffuse cytoplasmic reactivity for vimentin is a characteristic feature of sclerosing epithelioid fibrosarcoma, and 70% of the cases show MUC4 positivity [7,14,16,17]. It lacks immunoreactivity for epithelial membrane antigen; cytokeratins (CAM5.2 or AE1/3), HMB-45, neuronspecific enolase, desmin, smooth muscle actin, or muscle common actin (HHF-35) [8] Focal and weak immunostaining for EMA, S-100 protein and more rarely for cytokeratins may be seen in a minority of cases [19].…”

Radiofrequency ablation and percutaneous permanent iodine-125 implantation as salvage therapy for giant recurrent sclerosing epithelioid fibrosarcoma of the chest wall: A case report

“…The most commonly affected sites are the lower extremities, limb girdles and trunk, followed by the upper extremities, the head and neck, and the abdominal inguinal region (2,3). The tumor may also arise in the bones, neural system, cecum, ovaries and kidneys (4)(5)(6)(7)(8). SEF is an unusual variant of fibrosarcoma that is formed from epithelioid cells arranged in strands and nests in a background of a highly sclerotic collagenous stroma.…”