An Unusual Case of Sex Cord Tumor With Annular Tubules With Malignant Transformation in a Patient With Peutz-Jeghers Syndrome

被引：29

作者：

Barker, Douglas ^{[1
]}

Sharma, Rohini ^{[1
]}

McIndoe, Angus ^{[2
]}

Blair, Ed ^{[5
]}

Hall, Marcia

Gabra, Hani ^{[2
,4
]}

El-Bahrawy, Mona ^{[3
]}

机构：

[1] Univ London Imperial Coll Sci Technol & Med, Dept Expt Med, London, England

[2] Univ London Imperial Coll Sci Technol & Med, Div Gynaecol Oncol, London, England

[3] Univ London Imperial Coll Sci Technol & Med, Dept Histopathol, London, England

[4] Univ London Imperial Coll Sci Technol & Med, Ovarian Canc Act Res Ctr, London, England

[5] John Radcliffe Hosp, Dept Genet, Oxford OX3 9DU, England

来源：

INTERNATIONAL JOURNAL OF GYNECOLOGICAL PATHOLOGY | 2010年 / 29卷 / 01期

关键词：

Peutz-Jeghers Syndrome; Sex cord tumor with annular tubules; LKB1; STK11; Missense mutation; STROMAL TUMORS; CELL TUMOR; OVARY; LKB1;

D O I：

10.1097/PGP.0b013e3181b6a7c2

中图分类号：

R71 [妇产科学];

学科分类号：

100211 ;

摘要：

We report a case of a 54-year-old Caucasian woman with an earlier diagnosis Of Peutz-Jeghers Syndrome (PJS) and sex cord tumor with annular tubules (SCTAT). The sex cord stromal tumors showed aggressive malignant behavior with repeated recurrence and metastasis. This is an unusual behavior of SCTAT in patients with PJS, with only 2 such cases reported earlier. Genetic analysis revealed that the patient has a new (unreported earlier) missense mutation of the LKB1 gene. A review of the literature reporting the clinicopathologic features and biologic behavior of SCTAT in patients with and without PJS is presented. We discuss the presentation and management of this case and highlight the importance of considering the possibility of aggressive behavior of these tumors in the management of patients with PJS.

引用

页码：27 / 32

页数：6

共 31 条

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