Prognosis and predictive factors

The overall survival rate of patients with PLGA is excellent {164,342,696, 697,808}. A review of series with large numbers of cases and with long-term follow-up revealed a local recurrence rate between 9% and 17% and a regional metastases rate from 9-15% {342,697}. Distant metastases have seldom been reported {342,697}. Deaths attributed to tumour are unusual, and they occurred after prolonged periods {342,697}. In studies which accepted tumours with a predominant papillary configuration a higher incidence of cervical lymph node metastasis was reported {697}. The status of such tumours within the spectrum of PLGA is controversial. Dedifferentiation of PLGA has been reported and carries a less favourable prognosis. Such tumours should not be included under the rubric of typical PLGA {2368}.

Treatment consists of complete surgical excision. Neck dissection should be added for those patients with cervical adenopathy.

Cribriform adenocarcinoma of the tongue

A possible variant is cribriform adenocarcinoma of the tongue, but it is not yet clear whether this represents a genuine entity or just an unusual growth pattern in PLGA, with which there appears to be some overlap {1718}. So far described only in one series, all cases presented with a mass in the tongue, usually the posterior part, and synchronous metastases in lateral neck lymph nodes, but no distant spread. There was an equal sex incidence and the mean age at presentation was 50.4 years (range 25-70).

The tumour grows beneath the surface epithelium and infiltrates soft tissue. It is divided by fibrous septa into lobules, which are solid or cribriform. A characteristic feature is that some nearly solid islands have a glomeruloid arrangement of broad microfollicular papillae separated from a layer of peripheral columnar cells by a narrow cleft. Small numbers of tubules are seen, and occasional spindling of tumour cells may occur. The nuclei are uniform, pale and often overlap, closely mimicking those of papillary carcinoma of the thyroid. Mitotic figures are sparse. No necrosis or significant

Fig. 5.9 Polymorphous low-grade adenocarcinoma (PLGA). A Destructive invasion of adjacent tissues. B PLGA with papillary cystic formations. C Cribriform growth pattern.

haemorrhage is seen, and the stroma only focally positive. Thyroglobulin stain-includes hyalinized areas, and rarely ing is consistently negative. psammoma bodies. The tumours are positive for cytokeratin, and more patchi-ly for S-100 protein. Myoepithelial markers, such as actin are either negative or

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