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Review
. 2006 Aug;21(4):752-7.
doi: 10.3346/jkms.2006.21.4.752.

Peutz-Jeghers Syndrome with multiple genital tract tumors and breast cancer: a case report with a review of literatures

Seung-Hun Song  1 , Jae-Kwan Lee, Ho-Suk Saw, Sang-Yong Choi, Bum-Hwan Koo, Aeree Kim, Bum-Woo Yeom, Insun Kim
Affiliations
Review

Peutz-Jeghers Syndrome with multiple genital tract tumors and breast cancer: a case report with a review of literatures

Seung-Hun Song et al. J Korean Med Sci. 2006 Aug.

Abstract

We report here on the multiple genital tract neoplasms in a 41-yr-old Korean woman with Peutz-Jeghers Syndrome (PJS). The patient presented with lower abdominal pain. Her previous medical history was PJS and breast cancer. Pelvic ultrasound showed a multilocular cyst at the right adnexal region, diagnosed as bilateral ovarian mucinous borderline tumors. An ovarian sex cord tumor with annular tubules was incidentally diagnosed together with a minimal deviation adenocarcinoma of the uterine cervix and mucinous metaplasia of both the Fallopian tubal mucosa and the endometrium. Although the cases of multiple genital tract tumors with PJS has rarely been reported, the present case appears to be the first in Korea in which the PJS syndrome was complicated by multiple genital tract tumors and infiltrating carcinoma of the breast. The clinical significance of the multiple genital tract tumors and breast cancer associated with PJS is reviewed.

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Figures

Fig. 1
Fig. 1
The Peutz-Jegher polyp composed of hyperplastic, dilated mucous glands and central radiating smooth muscle bundles. Surface epithelium undergoes hemorrhagic necrosis due to intussception (H-E, ×40).
Fig. 2
Fig. 2
The invasive ductal carcinoma of the breast (H-E, ×100).
Fig. 3
Fig. 3
(A) The uterine endocervix shows a nodular pale pink tumor, infiltrating into deep cervical wall. (B) Bilateral ovarian multilocular cysts contain mucus.
Fig. 4
Fig. 4
The endocervical adenocarcinoma composed of well-differentiated, infiltrative glands (H-E, ×100).
Fig. 5
Fig. 5
(A) Mucinous tumor of the ovary shows papillary growth and nuclear stratification (H-E, ×200). (B) The sex cord element composed of multiple hyaline materials surrounded by clear cells (H-E, ×200).
Fig. 6
Fig. 6
(A) The salpingeal mucosa shows mucinous metaplasia (H-E, ×100). (B) Mucinous metaplasia of endometrial glands (H-E, ×100).
See this image and copyright information in PMC

References

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