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
518P - Real world prospective clinical impact of finding actionable genomic alterations by plasma cell-free DNA next generation sequencing in advanced non-small cell lung cancer
Presenter: Beung chul Ahn
Session: Poster display session
Resources:
Abstract
508P - Efficacy and safety of anti-PD-1 antibody SHR-1210 combined with apatinib in first-line treatment for advanced lung squamous carcinoma: A phase II study
Presenter: Jinliang Wang
Session: Poster display session
Resources:
Abstract
525P - Retrospective analysis of outcomes of cisplatin and irinotecan combination chemotherapy for unresectable thymic carcinoma
Presenter: Akito Fukuda
Session: Poster display session
Resources:
Abstract
524P - A study in recurrent small cell lung cancer patients, comparing weekly paclitaxel, irinotecan and temozolomide in second-line: A prospective study from a south Indian tertiary cancer hospital
Presenter: LALATENDU MOHARANA
Session: Poster display session
Resources:
Abstract
505P - PD-L1 expression in ALK rearranged NSCLC: All questions answered?
Presenter: Amrith B P
Session: Poster display session
Resources:
Abstract
487P - Afatinib versus gefitinib or erlotinib in first-line setting for Malaysia patients with EGFR mutant advanced lung adenocarcinoma
Presenter: Chee Shee Chai
Session: Poster display session
Resources:
Abstract
492P - Feasibility of rebiopsy and sequential treatment of EGFR tyrosine kinase inhibitors in real world patients with EGFR mutant non-small cell lung cancer
Presenter: Heekyung Ahn
Session: Poster display session
Resources:
Abstract
513P - Phase II study of vitamin B12 and folate supplementation for patients undergoing chemotherapy with pemetrexed
Presenter: Shingo Kitagawa
Session: Poster display session
Resources:
Abstract
493P - Is exon 19 deletion different from exon 21 mutation in advanced non-small cell lung cancer: A single centre experience
Presenter: Sarita Shrivastva
Session: Poster display session
Resources:
Abstract
494P - Comparison of pattern of disease progression and prevalence of acquired T790M mutation in Malaysia patients with EGFR mutant lung adenocarcinoma upon failure of first-line afatinib, gefitinib and erlotinib
Presenter: Chee Shee Chai
Session: Poster display session
Resources:
Abstract