Fig. 1.53 Solitary fibrous tumour of nasal cavity. A Circumscribed tumour beneath epithelium. B Bland-looking spindly cells are tightly intertwined with collagen fibers.

Extrapleural solitary fibrous tumour

ICD-O code 8815/1

Solitary fibrous tumours are tumours of CD34-positive fibroblasts which often show a prominent haemangiopericytoma-like vascular pattern. They are exceedingly uncommon in the upper respiratory tract, where they comprise <0.1% of all neoplasms. All ages can be affected without a gender predilection. Tumours can affect the nasal cavity, nasopharynx or paranasal sinuses. Patients present with nasal obstruction, epistaxis or other nonspecific symptoms. The tumour is usually polypoid and firm. It is composed of a variably cellular proliferation of bland spindle-shaped cells with nondescript growth pattern associated with "ropy" keloidal collagen bundles and interlaced thin-walled vascular spaces. The latter may be prominent and exhibit a haeman-giopericytoma-like pattern. Solitary fibrous tumours are immunoreactive for CD34 and bcl-2, and generally lack actin immunoreactivity. The diagnosis rests on a combination of architectural, cytomor-phologic, and immunophenotypic features. The differential diagnoses include sinonasal glomangiopericytoma, fibrous histiocytoma, leiomyoma, schwannoma, synovial sarcoma, and fibrosarcoma. Complete surgical removal yields the best patient outcome. Occasional cases may potentially show a malignant behaviour {158,834,997,1706,2600,2800, 2914}. See WHO Classification of Tumours of Soft Tissue and Bone {775}.

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