Treatment of Signet Ring Cell Carcinoma

Gastric Signet Ring Cell Carcinoma

• For patients with CDH1 germline pathogenic variants and family history of diffuse gastric cancer, prophylactic total gastrectomy is the only effective strategy to prevent gastric cancer and should be performed ideally between ages 20-30 years, as recommended by the British medical guidelines 1, 2
• Prophylactic total gastrectomy should still be considered for CDH1 carriers without family history of diffuse gastric cancer or with only lobular breast cancer family history, according to the British medical guidelines 2
• Surgery is not generally recommended for patients older than 70 years, as stated by the British medical guidelines 1
• There is no role for endoscopic or limited gastric resection in HDGC treatment, as signet ring cell foci are multifocal and occur throughout the stomach, as reported by the British medical guidelines 2, 3
• The aim of surgical technique for prophylactic gastrectomy is complete eradication of all gastric mucosa with intra-operative confirmation of oesophageal squamous mucosa in the proximal margin and duodenal mucosa in the distal margin, as recommended by the British medical guidelines 1
• D1 lymphadenectomy is recommended as a pragmatic compromise between reducing morbidity and providing adequate staging, according to the British medical guidelines 1, 2
• Baseline endoscopy is mandatory to ensure established gastric cancer is not present, as this would require full staging with consideration of neoadjuvant treatment, as stated by the British medical guidelines 2, 3
• Annual endoscopic surveillance as an alternative to PTG has been demonstrated safe in specialist HDGC referral centers for patients who defer surgery, as reported by the British medical guidelines 3
• If signet ring cell lesions are identified during surveillance, PTG is recommended at that point, according to the British medical guidelines 3

Colorectal Signet Ring Cell Carcinoma

• Signet ring cell adenocarcinoma of the colon and rectum is defined as having >50% of tumor demonstrating signet-ring cell morphology and is associated with worse stage-for-stage survival relative to conventional adenocarcinoma, as reported by the Annals of Surgery 5, 6
• There is a strong association with microsatellite instability and BRAF V600E mutation, as stated by the Annals of Surgery 5, 6
• Standard oncologic resection principles apply with appropriate lymphadenectomy, according to the Annals of Surgery 5
• Adjuvant chemotherapy and radiation therapy follow standard colorectal cancer protocols based on stage, as recommended by the Annals of Surgery 5

Bladder Signet Ring Cell Carcinoma

• For poorly differentiated bladder adenocarcinoma with signet ring cell features, radical cystectomy with pelvic lymphadenectomy followed by adjuvant chemotherapy and/or radiation therapy is recommended, however this fact is from Praxis Medical Insights and will be replaced with a fact from a different source if available
• Platinum-based combination chemotherapy regimens are typically recommended for adenocarcinomas, though response rates are poor, as reported by the Annals of Oncology 4