Biphasic solitary fibrous tumour: a report of two cases with epithelioid features

Awasthi, Ramesh Chandra; O’Neill, Jennifer; Keen, C. E.; Sarsfield, P.; Devaraj, V.S.; Stone, C. A.; Smith, Mark E.

doi:10.1007/s00428-005-0099-8

Cited by 8 publications

(16 citation statements)

References 10 publications

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“…Since then, five cases of epithelioid SFT affecting orbit [6] ischioanal fossa [8,7] and neck [7] have been reported.…”

Section: Resultsmentioning

confidence: 99%

“…Microscopically, three of the cases reported showed a biphasic morphology coexisting areas of typical fusocellular SFT and areas of epithelioid appearance [6,7], one of the cases was predominantly epithelioid [5], and the other one showed only epithelioid areas[8]. In three of the cases, epithelioid areas were described having benign non-malignant appearance.…”

Section: Resultsmentioning

confidence: 99%

“…Few cases of soft tissue epithelioid solitary fibrous tumor affecting mediastinum [5], orbit [6], neck [7] and ischioanal fossa [7,8] have been described. We present a case of solitary fibrous tumor of the thigh showing distinct biphasic morphology and demonstrating epithelioid differentiation.…”

Section: Introductionmentioning

confidence: 99%

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Solitary fibrous tumor of the thigh with epithelioid features: a case report

et al. 2007

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Background: Extrapleural Solitary Fibrous tumors (SFTs) have been increasingly reported. The retroperitoneum, deep soft tissues of proximal extremities, abdominal cavity, trunk, head and neck are the most common extraserosal locations reported. Microscopically they show a wide range of morphological features, and so the differential diagnosis is extensive. Immunohistochemically, they commonly express CD34, vimentin, bcl-2 and CD99. Epithelial membrane antigen (EMA) and smooth muscle actin (SMA) may occasionally be expressed. Epithelioid morphology in extrapleural SFT has only very occasionally been described (five cases reported), some of them with biphasic pattern and others with malignant characteristics.

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“…Since then, five cases of epithelioid SFT affecting orbit [6] ischioanal fossa [8,7] and neck [7] have been reported.…”

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Solitary fibrous tumor of the thigh with epithelioid features: a case report

et al. 2007

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“…Immunohistochemical examination has to rule out not only sarcomatoid carcinomas but also other mesenchymal tumors such as clear cell sarcoma, which rarely presents in the gastrointestinal tract [15,45,54] and can be identified by its strong expression of S-100 protein and by fluorescence in situ hybridization detection of the characteristic EWS-ATF1 translocation. Furthermore, CD34 is expressed in more than 70% of GISTs, but may also be found in solitary fibrous tumors that may exhibit an epithelioid or spindle cell phenotype [2]. The latter are constantly completely negative for the KIT receptor.…”

Section: Diagnosis Of Gistmentioning

confidence: 98%

Mutation analysis of gastrointestinal stromal tumors: increasing significance for risk assessment and effective targeted therapy

et al. 2007

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Molecular characterization of gastrointestinal stromal tumors (GISTs) plays an increasing role not only for the patient's prognosis but also for treatment options and in the context of resistance to therapy. Several mutational subtypes in KIT or platelet-derived growth factor receptor-alpha (PDGFRalpha) have been identified to be correlated with a different clinical behavior of GISTs. In KIT exon 11, deletions in the proximal part are associated with a high metastatic risk, whereas duplications in the distal part lead to a less aggressive phenotype. GISTs of the small bowel with a duplication in KIT exon 9 are often high risk tumors. In contrast, PDGFRalpha exon 18 mutated GISTs tend to have a low malignant potential. The authors suggest to include these molecular data together with classical parameters such as mitotic count and tumor size into the risk assessment of GISTs. The first choice for treatment of GISTs is still the surgical resection. In advanced tumors, which cannot be R0 resected, the neoadjuvant treatment with the tyrosine kinase inhibitor imatinib is now well established. Furthermore, an adjuvant treatment of locally R0-resected intermediate and high risk tumors is evaluated in several international clinical trials. For metastatic disease, treatment with imatinib is still the first option, but with new upcoming substances, the molecular characterization of GISTs may become mandatory. Very recently, it has been shown that sunitinib may be especially effective in GISTs with KIT exon 9 mutation, whereas these tumors show only an intermediate response to imatinib. A European Organisation for Research and Treatment of Cancer clinical trial randomizing patients according to their mutational status is under preparation. Secondary resistance to imatinib treatment is increasing, at least partly due to secondary mutations in the tyrosine kinase domain of the KIT receptor. Once a lesion has been shown to carry such a mutation, the local excision may be useful, mean while still responding metastases are further controlled by continuing imatinib. Taken together, the molecular characterization of GISTs turns out to play a central role before and during the treatment with tyrosine kinase inhibitors, which have improved the treatment of GIST patients dramatically.

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“…Histological features (lack of whorls and psammoma bodies) and the immunophenotype (EMA and E-cadherin negative, cytokeratin, desmin and CD34 positive) exclude meningioma. Although CD34 positivity does raise the possibility of SFT, a neoplasm that has been reported at a meningeal location,2 the epithelioid component would be very unusual,5 as would the cytokeratin-positive and desmin-positive immunophenotype. In addition, substantial variability in the cellularity of the spindle cell component and a haemangiopericytic vasculature, both features typical of SFT, were lacking.…”

Section: Discussionmentioning

confidence: 99%