Breast cancer, ovarian gonadoblastoma and cervical cancer in a patient with Peutz-Jeghers Syndrome

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 cance...

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Bibliographic Details
Published in:Archives of gynecology and obstetrics 2008-07, Vol.278 (1), p.75-77
Main Authors: Kilic-Okman, Tulay, Yardim, Turgut, Gücer, Fatih, Altaner, Semsi, Yuce, M. Ali
Format: Article
Language:English
Subjects:
Adenocarcinoma - diagnosis
Adenocarcinoma - therapy
Adult
Antiretroviral drugs
Biomarkers, Tumor - blood
Breast cancer
Breast Neoplasms - diagnosis
Breast Neoplasms - surgery
Cancer therapies
Carcinoma, Papillary - diagnosis
Carcinoma, Papillary - surgery
Case Report
Cervical cancer
Endocrinology
Fatal Outcome
Female
Gonadoblastoma - diagnosis
Gonadoblastoma - surgery
Gynecology
Human Genetics
Humans
Mastectomy
Medicine
Medicine & Public Health
Neoplasms, Multiple Primary
Obstetrics/Perinatology/Midwifery
Ovarian Neoplasms - diagnosis
Ovarian Neoplasms - surgery
Peutz-Jeghers Syndrome - complications
Tumors
Uterine Cervical Neoplasms - diagnosis
Uterine Cervical Neoplasms - therapy
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Summary: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.
ISSN:0932-0067
1432-0711
DOI:10.1007/s00404-007-0535-x