Benign soft tissue tumours

J.C. Fanburg-Smith L.D.R. Thompson

Myxoma

Myxoma is a benign soft tissue tumour characterized by bland spindle shaped cells enbedded in hypovascular, myxoid stroma. For details see Chapter 6 (Odontogenic tumours).

Leiomyoma

Definition

A benign tumour of smooth muscle phe-notype.

ICD-O code 8890/0

Synonyms

Angioleiomyoma; vascular leiomyoma; leiomyoblastoma

Epidemiology

Primary leiomyomas of the sinonasal tract are very rare {824,975,1144,1307, 1535,1796,2114,2635,2695}. There is a peak in the 6th decade, although men are younger than women by a decade at initial diagnosis. There is a female predilection, with a ratio of 3.5:1.

Etiology

Other than prior radiation, there are no known risk factors.

Localization

The turbinates are affected most frequently, {824,1144,1307,1535,1796, 2114,2635,2695} with isolated cases reported in the paranasal sinuses alone or in combination with the nasal cavity {1842}.

Clinical features

Nearly all patients have nasal obstruction, although nasal discharge, epistaxis, headaches and pain are also common {824,2114,2635,2695}.

Macroscopy

These tumours have an average size of 2 cm, but rare ones may be as large as 10 cm. They are sessile or polypoid, with a smooth, well circumscribed border.

Histopathology

Leiomyomas are located in the submu-cosa, separated from a typically intact mucosa. They are composed of spindled cells arranged in orderly fascicles, whorls and intersecting bundles. The cells have elongated, vesicular to stippled nuclei with blunt ends ("cigar-shaped"), surrounded by spindled, bipolar, fibrillar eosinophilic cytoplasm. They are highly differentiated, with little or no atypia, although rare cells may exhibit nuclear pleomorphism {2695}. Necrosis and invasion are absent, and mitotic activity is scarce. Mucinous degeneration, hyalinization or fibrosis, and adipocytes can be seen, but these features are usually focal and more likely seen in larger lesions {1144,1535,1796, 2695}. Vascular leiomyoma (angiomy-oma) contains capillary, cavernous or venous vascular spaces, with the smooth muscle cells being associated with the vessel walls and represents the most common type of benign smooth muscle tumour in this region.

Immunoprofile

The tumour cells are diffusely and strongly immunoreactive for actins, desmin, h-caldesmon and vimentin. The Ki-67 index is usually <5% {1144}.

Differential diagnosis

The differential diagnoses include sinonasal glomangiopericytoma, hae-mangioma, peripheral nerve sheath tumour and leiomyosarcoma.

Prognosis and predictive factors

Complete excision is curative.

Haemangioma

Definition

A benign neoplasm of vascular pheno-type.

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