Abstract
Background
Peutz-Jeghers Syndrome (PJS) is a rare autosomal dominant disorder characterized by gastrointestinal hamartomatous polyps and mucocutaneous pigmentation. Patients with PJS have increased risk for gastrointestinal, breast, and female genital tract cancers.
Case
Multiple genital tract cancers in a 34-year-old woman with PJS are described. The patient, who was admitted to our department with severe vaginal bleeding, was performed right salpingo-oophorectomy because of pure gonadoblastoma in 1996. In 2003, concomitant to cervical carcinoma, breast cancer was diagnosed. Patient underwent left modified radical mastectomy due to the invasive papillary carcinoma. The patient received six cycles combination chemotherapy and radiation therapy because of stage IIIB cervical cancer.
Conclusion(s)
This is the first case report presenting PJS associated with multiple genital tract tumors including ovarian gonadoblastoma in literature. The clinical significance of these tumors in PJS patients has been reviewed.
Similar content being viewed by others
References
Thull DL, Vogel VG (2004) Recognition and management of hereditary breast cancer syndromes. Oncologist 9:13–24
Giardiello FM, Welsh SB, Hamilton SR, Offerhaus GJ, Gittelsohn AM, Booker SV et al (1987) Increased risk of cancer in the Peutz-Jeghers Syndrome. N Engl J Med 316:1511–1514
Papageorgiou T, Stratakis CA (2002) Ovarian tumors associated with multiple endocrine neoplasias and related syndromes (Carney complex, Peutz-Jeghers Syndrome, von Hippel Lindau disease, Cowden’s disease). Int J Gynecol Cancer 12:337–347
Tokatli F, Tuncbilek N, Puyan FO et al (2004) Peutz-Jeghers syndrome combined with breast cancer, cervical carcinoma and ovarian gonadoblastoma: a case report. J BUON 9:469–472
Scully RE (1970) Gonadoblastoma. A review of 74 cases. Cancer 25:1340–1356
Nakashima N, Nagasaka T, Fukata S et al (1989) Ovarian gonadoblastoma with dysgerminoma in a woman with two normal children. Hum Pathol 20:814–816
Talerman A, Verp MS, Senekjian E, Gilewski T, Vogelzang N (1990) True hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, 46,XX/46,XY karyotype, and a successful pregnancy. Cancer 66:2668–2672
Tavossoli FA (1983) A combined germ cell-gonadal stromal-epithelial tumor of the ovary. Am J Surg Pathol 7:73–84
Talerman A (1994) Germ cell tumors of the ovary. In: Kurman RJ (ed) Blaustein’s pathology of the female genital tract. 4th edn. Springer, New York, pp 849–914
Nomura K, Furusato M, Nikaido T, Aizawa S (1991) Ovarian sex cord tumor with annular tubules. Report of a case. Acta Pathol Jpn 41:701–706
Ahn GH, Chi JG, Lee SK (1986) Ovarian sex cord tumor with annular tubules. Cancer 57:1066–1073
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kilic-Okman, T., Yardim, T., Gücer, F. et al. Breast cancer, ovarian gonadoblastoma and cervical cancer in a patient with Peutz-Jeghers Syndrome. Arch Gynecol Obstet 278, 75–77 (2008). https://doi.org/10.1007/s00404-007-0535-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00404-007-0535-x