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
436P - Assessing the quality of life of Filipino cancer patients: A survey of Filipino oncologists
Presenter: Frederic Ivan Ting
Session: Poster display session
Resources:
Abstract
437P - Correlation between bio-impedance analysis and abdominal CT scan to diagnose decreased muscle mass in adult cancer patients
Presenter: Andree Kurniawan
Session: Poster display session
Resources:
Abstract
438P - Evaluating mitochondrial biomarkers between fatigue subclasses identified using latent class analysis in early-stage breast cancer patients
Presenter: Yi Long Toh
Session: Poster display session
Resources:
Abstract
440P - Accuracy of risk scoring system to determine delayed chemotherapy induced nausea and vomiting (CINV) in cancer patients
Presenter: Jada Harika
Session: Poster display session
Resources:
Abstract
441P - A pilot cross-sectional study on incidence of liver toxicity in cancer patients on western anti-cancer drug therapy with or without concurrent Chinese herbal medicine
Presenter: Tsz Him So
Session: Poster display session
Resources:
Abstract
442P - Relationship between QOL and support elderly patients with permanent colostomies
Presenter: Yukiko Orii
Session: Poster display session
Resources:
Abstract
443P - The effectiveness of individual nutritional counselling for patients with advanced cancer undergoing chemotherapy: A preliminary study
Presenter: Saori Koshimoto
Session: Poster display session
Resources:
Abstract
444P - The prophylactic effect of 0.1% fluorometholone eye drops on eye disorders caused by high-dose cytarabine
Presenter: Takayuki Tsuchiya
Session: Poster display session
Resources:
Abstract
445P - Safety and feasibility of extending flushing interval every 3 months for maintenance of TICVPS in CRC patients after completion of curative intended treatments
Presenter: Sang Bo Oh
Session: Poster display session
Resources:
Abstract
446P - Accuracy of risk scoring system to determine chemotherapy induced nausea and vomiting (CINV) in cancer patients receiving first cycle chemotherapy
Presenter: Jada Harika
Session: Poster display session
Resources:
Abstract