Abstract YO18
Case summary
Peutz-Jegher syndrome(PJS) , an autosomal dominant disorder with mutation in STK11 gene,is characterized by multiple polyps throughout gastrointestinal tract, and mucocutaneous pigmented lesion. Patient with PJS increase risk of multiple cancers, including breast, GI, and gynecologic cancer.Here, we reported a known case of 18 year-old female patient with PJS who had incidentally diagnosed with metastatic sex cord stromal tumor of ovary. At the age of 6 year-old, she initially presented with mucocutaneous pigmented lesion of oral mucosa, which was biopsied confirmed PJS in 2012, she presented with intussusception and underwent small bowel resection. After recovery, she had been under surveillance endoscopy since then. In 2018,had recurrent abdominal pain. EGD and colonoscopy showed multiple hamartomatous polyps at gastric, small bowel, rectosigmoid colon. Due to large small bowel polyps, she underwent small bowel resection. A 2-cm peritoneal mass at jejunum was found and removed. Post-operative CT-scan demonstrated large bilateral ovarian masses. She subsequently underwent TAH with BSO. The pathological report of both peritoneal and bilateral ovarian masses was consistent with sex cord stromal tumor arising from ovaries (Granulosa cell: Juvenile type).The immunohistochemistry staining was positive for CK, CD 56, Alpha-inhibin, calrethinin, CD99 and WT1. She received systemic chemotherapy with (BEP regimen ;bleomycin, etoposide, cisplatin) for 6 cycles with no evidence of disease. A previous report showed that patients with PJS had 21% lifetime risk of ovarian cancer, which started after the age of 30 yaer-old. Sex cord stromal tumor is considered uncommon ovarian cancer that is associated with PJS. Patients usually present with sign of hormone productions and/or symptom of ovarian mass. Similar to this case ,most patients might present with advanced stage. Though ovarian cancer screening with CA-125 and transvaginal ultrasound had been proposed for PJS patient, none of which is considered standard of care. To date, there is no evidence to support routine screening of ovarian cancer in patients with PJS.
Clinical trial identification
Editorial acknowledgement
Resources from the same session
154P - Genetic characteristics of participants in the Australian Pancreatic Screening Study
Presenter: Krithika Murali
Session: Poster display session
Resources:
Abstract
155P - Mean Platelet Volume (MPV) is it a new prognostic marker in resectable carcinoma stomach?
Presenter: Girish M. S
Session: Poster display session
Resources:
Abstract
156P - A positive feedback between IDO1 metabolite and COL12A1 via MAPK pathway to promote gastric cancer metastasis
Presenter: Zhen Xiang
Session: Poster display session
Resources:
Abstract
157P - Lymph node ratio (LNR) a better prognostic factor after D2 gastrectomy
Presenter: Jitin Yadav
Session: Poster display session
Resources:
Abstract
158P - A clinical significance of preoperative C-reactive protein/albumin ratio in patients with extrahepatic bile duct cancer
Presenter: Kim Jinkook
Session: Poster display session
Resources:
Abstract
159P - The relation between obesity and cancer of gastrointestinal tract in Korea: The data from Statistic Korea between 2001 and 2016
Presenter: Hee Man Kim
Session: Poster display session
Resources:
Abstract
160P - Clinical outcomes of second-line chemotherapy after progression on nab-paclitaxel plus gemcitabine in patients with metastatic pancreatic adenocarcinoma
Presenter: Jooyoung Ha
Session: Poster display session
Resources:
Abstract
161P - Chitinase 3-Like 1 gene (T/C) polymorphism and serum YKL-40 in hepatocellular carcinoma
Presenter: Alshimaa Alhanafy
Session: Poster display session
Resources:
Abstract
162P - Hypofractionated radiotherapy for pulmonary metastases from hepatocellular carcinoma: Treatment response and prognostic factors affecting survival
Presenter: In Young Jo
Session: Poster display session
Resources:
Abstract
163P - Excision repair cross-complementation group 1 and 2 (ERCC1/2) Single nucleotide polymorphisms and chemotherapy treatment outcome in Cholangiocarcinoma
Presenter: Thanachai Sanlung
Session: Poster display session
Resources:
Abstract