Clinical Features Of Fptc And Implications For Patient Care

The clinical features of fPTC are beginning to emerge and are compared with the clinical features of sporadic PTC in Table 2. Loh first summarized the clinical features based upon a review of available published kindreds [16]. A more recent study from Japan has reported similar results [17]. Although these results are probably reliable, final confirmation of their accuracy must await the identification of the f PTC susceptibility genes, so that the genetically affected individuals can be unequivocally distinguished from those that are genetically unaffected.

The evaluation of large kindreds suggests that inheritance is autosomal dominant with partial penetrance, although it is possible that modifying genes play an important role. As with sporadic PTC, women are affected more frequently than men. Multifocal disease is more common in f PTC than in sporadic PTC, and the age of onset is somewhat younger in fPTC (mean = 38 y) than in sporadic PTC (mean = 48 y) [16, 28]. However, Uchino did not find an age difference between familial and sporadic PTC [17]. There seems to be a greater incidence of benign thyroid nodules associated with fPTC than with sporadic PTC [17]. There may be other malignancies associated with fPTC. Epidemiologic studies suggest an increased incidence of breast carcinoma in individuals with PTC [23], and one large kindred with fPTC is enriched in PRN and possibly other malignancies [9]. Although it has been, suggested that fPTC may be more aggressive than sporadic PTC [30], the differences on a whole seem to be modest. There are occasional kindreds in which fPTC seems to be more aggressive than sporadic PTC with some subjects dieing from this disorder. A more recent study does suggest a greater incidence of recurrent disease with fPTC than with sporadic PTC, but no increased incidence of death [17].

Two known familial tumor syndromes are associated with an increased incidence of PTC [29]. The frequency of PTC in familial adenomatous polyposis (FAP) is about

Table 2. Clinical characteristics of familial and sporadic papillary thyroid carcinoma (approximate ratios and percentages)

Characteristic

Familial PTC

Sporadic PTC

Age of Onset

mean = 38 [ 16]

mean 45-50 [28]

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