Thyroid Papillary Adenocarcinoma in a Woman with Peutz-Jeghers Syndrome.

Abstract

We present a rare case of Peutz-Jeghers(P-J) syndrome in a 29-year-old woman who developed adenocarcinoma of the thyroid and polyps in the gastrointestinal tract. Polyps removed from the stomach, colon and ileum showed features typical of hamartomas. Histological examination indicated that the tumor resected from the right lobe of the thyroid was a papillary adenocarcinoma. This appears to be the first precise report of a P-J syndrome associated with thyroid carcinoma. This case adds an example of predisposition of P-J syndrome to extra intestinal malignancy and indicates the importance of long-term observation of malignancy.

The association of adrenocortical carcinoma and thyroid cancer in a child with Peutz-Jeghers syndrome

“…There are only 5 reported cases of thyroid cancer in association with PJS in the literature [12][13][14][15][16]. All 5 were young adults ranging in age from 21 to 33 years: 3 had papillary, one had unspecified thyroid cancer, and the pathologic type in the fifth case was not mentioned.…”

Differentiated Thyroid Carcinoma (DTC) in a Young Woman with Peutz-Jeghers Syndrome: Are these Two Conditions Associated?

“…We present the case of a young woman aff ected by PJS with a recently diagnosed papillary thyroid cancer. The case provides an opportunity to discuss the association between PJS and thyroid cancer, which has not been frequently reported in literature ( Yamamoto et al, 1992 ) and is yet to be thoroughly demonstrated.…”

“…A real esteem of the most common endocrine cancers in PJS is not available in literature ( Giardiello et al, 1987 ;Boardman et al, 1998 ;Hearle et al, 2006a ). However, endocrine tissues can be a site of cancer development ( Yamamoto et al, 1992 ), and especially so the testis ( Giardiello et al, 1987 ;Cant ú et al, 1980 ;Dubois et al, 1982 ) ( Table 1 ). Diff erentiated thyroid cancer (DTC) accounts for at least 94 % of all thyroid cancers, the remaining 6 % being due to anaplastic thyroid carcinoma (1 % ) and to medullary thyroid carcinoma (MTC) (5 % ) (Schlumberger and Pacini, 2003;Sherman, 2003 ).…”

Thyroid Abnormalities in Peutz-Jeghers Syndrome: Report of a New Observation and Review of the Literature

“…To date, there are two reports of thyroid papillary adenocarcinoma in women with Peutz-Jeghers syndrome 11,12 The significance of the thyroid findings in this patient is not known. The majority of cancers do not occur in the gastrointestinal lumen, but in organs in which hamartomatous polyps are not found.…”