Pleomorphic Adenoma

ICD-O:8940/0

Pleomorphic adenoma (PA) has rarely been reported in the larynx [88, 219, 323]. To date, only about 20 cases have been described. Men predominate slightly over women, and the patients' ages range from 15 to 82 years [88]. The supraglottis is by far the most common site of origin. PA, which may grow up to several centimetres in diameter, occurs as a submucosal mass without ulceration. Histologically, PA shows all the characteristics of a tumour arising in the major salivary glands (see Chap. 5). The diagnosis of laryngeal PA should be considered very carefully due to its rarity in this location and other tumours, such as chondrosarcoma, adenoid

ICD-O:9120/0

Laryngeal haemangiomas (LH) are uncommon lesions of vascular origin, defined as benign proliferation of the blood vessels. They are divided into two distinct clinicopathologic entities: the neonatal and adult forms.

Neonatal LH is a rare congenital malformation characteristically involving the subglottic area. Although the lesion is present at birth, symptoms become clinically evident during the first 6 months. A progressive crouplike disease with inspiratory stridor turns into biphasic stridor as the obstruction progresses. Characteristically, the symptoms are intermittent, accentuated during crying, when the vessels are filled up under increased pressure [195, 196, 290, 312, 328].

Subglottic LH appears more frequently in girls, with a ratio of 2:1 [334]. A co-existence of haemangiomas of the skin and mucosa of the oral cavity and pharynx, as well as in other organs [195, 337], may also be an important indicator of disease.

The gross appearance of the lesion ranges from a flat to polypoid, soft, compressible submucosal mass, pink-reddish to blue in colour. The lesion is usually one-sided, and is located in the posterolateral subglottic area. However, some haemangiomas are horseshoe-shaped and are present as a bilateral subglottic reddish swelling [195]. The diagnosis is based on characteristic clinical features and endoscopic appearance. Biopsy should be avoided because of an increased risk of excessive bleeding.

Laryngeal haemangiomas of adults, which are usually localised in the glottic and supraglottic region, are seen as inconspicuous, submucosal, reddish blue lesions. Common symptoms are hoarseness, dyspnoea and/or foreign body sensation [336].

Histologically, subglottic LHs are divided into capillary and cavernous forms. The majority of lesions are of the capillary type [334], consisting of the proliferating capillaries that infiltrate the surrounding submuco-sal structures (Fig. 7.10).

Vascular channels may be of various sizes, lined with plump endothelial cells in which some regular mitoses may be present. Vascular tissue is intertwined with fibrous tissue and infiltrated with a variable amount of inflammatory cells. Focally, depositions of haemosider-in may be found in the fibrous stroma. It is important to note that haemangiomas in infants are considerably more cellular than adult ones [48]. The cavernous form of LH is less frequent, composed of proliferation of large angiectatic vascular spaces, lined with thin, spindle-shaped endothelial cells, and filled with erythrocytes. This type of lesion is more common in adults. Immuno-histochemical analysis helps to confirm endothelial cell proliferations in both forms, with positivity for CD31, CD34 and factor XIIIa antigens.

In differential diagnosis, various lesions of vascular origin, such as the vascular type of the vocal cord polyp (VCP), pyogenic granuloma, intravascular papillary endothelial hyperplasia and even angiosarcoma, must be considered. The vascular variant of VCP is usually characterised by highly angiectatic vascular spaces surrounded by massive leakage of fibrin as amorphous hyaline pink material, a feature not characteristic of capillary haemangioma. Pyogenic granuloma is diffusely infiltrated with neutrophils, which is not the case in haemangiomas, if the covering epithelium is intact. Papillary endothelial hyperplasia represents an organisation of thrombosis with papillary proliferation of the endo-thelial cells, which is not a striking histological feature of haemangioma. The distinction between the cellular variant of haemangioma and angiosarcoma may sometimes be problematic. However, anastomoses of the vas cular channels, lined with considerably pleomorphic en-dothelial cells with evident pathologic mitoses, certainly favour a diagnosis of angiosarcoma [48].

Subglottic LH of infants is generally a self-limited but potentially fatal lesion, causing progressive airway obstruction. Various treatment modalities have been proposed, including expectant policy, systemic steroids and interferon alfa-2a applications, CO2 laser excision and tracheostomy, but no very promising treatments are yet available [139, 196, 328].

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