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
411P - Comparative analysis of protein profiles of prognosis-associated proteins and KIT-related proteins in gastrointestinal stromal tumour
Presenter: Yoshiyuki Suehara
Session: Poster display session
Resources:
Abstract
412P - The evaluation of chromosomal changes in patients with osteosarcoma
Presenter: Nargiza Karimova
Session: Poster display session
Resources:
Abstract
413P - The state of molecular biological markers in osteosarcoma
Presenter: Djamilya Polatova
Session: Poster display session
Resources:
Abstract
414P - Clinical profile of gastrointestinal stromal tumour in Yangon General Hospital
Presenter: Khin Mu
Session: Poster display session
Resources:
Abstract
415P - Adult soft tissue myoepithelial carcinoma: Treatment outcomes and efficacy of chemotherapy
Presenter: Florence Chamberlain
Session: Poster display session
Resources:
Abstract
417P - Clinicopathological profile and survival outcomes of sarcomas from a regional cancer centre in South India
Presenter: Divya Bharathi
Session: Poster display session
Resources:
Abstract
418P - Lessons learnt from treatment of foot sarcomas: Analysis from dedicated sarcoma clinic in North India
Presenter: Satyajit Pawar
Session: Poster display session
Resources:
Abstract
423P - Effectiveness of first-generation 5HT3 receptor antagonist plus dexamethasone plus aprepitant in controlling delayed chemotherapy-induced nausea and vomiting in patients with colorectal cancer: A propensity score-matched analysis
Presenter: Toshinobu Hayashi
Session: Poster display session
Resources:
Abstract
424P - Cancer worry, genetic knowledge, and attitudes towards NGS multigene panel testing among Korean breast cancer patients
Presenter: Ji Soo Park
Session: Poster display session
Resources:
Abstract
425P - Economic and safety evaluation of 5-HT3 recepter antagonist conversion from palonosetron to granisetron in highly and moderately emetogenic chemotherapy: A prospective study
Presenter: Keisuke Yamakita
Session: Poster display session
Resources:
Abstract