Signet Ring Cell Colorectal and Appendiceal Cancer: A Small Signet Ring Cell Component Is Also Associated with Poor Outcome

Abstract

Background: Colorectal signet ring cell (SRC) carcinoma with ≥50% SRCs (SRC ≥ 50) has a poor prognosis, but the prognostic role of SRCs < 50% (SRC < 50) is unclear. The aim of this study was to provide a clinicopathological characterization of SRC colorectal and appendiceal tumours and analyse the importance of the SRC component size. Methods: All patients in the Swedish Colorectal Cancer Registry diagnosed with colorectal or appendiceal cancer in 2009–2020 at Uppsala University Hospital, Sweden, were included. The SRCs were verified, and the components estimated by a gastrointestinal pathologist. Results: Of the 2229 colorectal cancers, 51 (2.3%) had SRCs, with a median component size of 30% (interquartile range of 12.5–40) and 10 (0.45%) had SRC ≥ 50. The SRC tumours were primarily localized in the right colon (59%) and appendix (16%). No patients with SRCs had stage I disease, and 26 (51%) had stage IV, of whom, 18 (69%) had peritoneal metastases. The SRC tumours were often high grade with perineural and vascular invasion. The 5-year overall survival (OS) rate for patients with SRC ≥ 50 were 20% (95% confidence interval (CI) 6–70), for SRC < 50, 39% (95% CI 24–61); and for non-SRCs, 55% (95% CI 55–60). Among the patients with SRC < 50 and <50% extracellular mucin, the 5-year OS was 34% (95% CI 19–61), while those with ≥50% extracellular mucin had an OS of 50% (95% CI 25–99). The 5-year recurrence-free survival rates were 51% (95% CI 13–83) for patients with SRC tumours, as compared to 83% (95% CI 77–89) and 81% (95% CI 79–84) for mucinous and non-mucinous adenocarcinoma, respectively. Conclusions: The presence of SRCs was strongly associated with aggressive clinicopathological features, peritoneal metastases, and poor prognosis, also when they make up <50% of a tumour.

Incidental diagnosis of primary appendiceal signet-ring cell adenocarcinoma after appendectomy for acute appendicitis, a case report

“…Signet-ring cell adenocarcinoma, as a rare subtype of adenocarcinoma, is thought to be more aggressive in biological behavior. The 5-year survival was reported to be only 7% 5 . The nomenclature was described as signet-ring cell, simply for the morphological feature that the nucleus was pushed to polarization by the abundant intracellular mucin, and, microscopically, the tumor cell would appear to be like a signet-ring on H&E staining 6 .…”

Signet ring-cell colorectal adenocarcinoma: review and clinical case

“…Перстневидноклеточный компонент, как предполагают, повышает метастатический потенциал опухоли [22,23], в т. ч. с развитием метастазов редких локализаций, таких как кости, головной мозг, яичники и сердце. Смена морфотипа КРР с AC даже на частично перстневидноклеточный значительно ухудшает прогноз у больных [24,25].…”

Appendiceal Signet Ring Cell Carcinoma: An Atypical Cause of Acute Appendicitis—A Case Study and Review of Current Knowledge

“…If more than 50% of the tumor cells are of this type, it signifies the malignancy's potential for rapid progression and its aggressive nature [14]. Alarmingly, the 5-year survival rate for signet ring cell carcinoma is only 7%, making it one of the deadliest types of cancer [1]. The tumor frequently spreads to neighboring organs, which has led some experts to categorize primary signet ring cell carcinoma (ASRCC) as a separate entity from other appendiceal cancers [6].…”

“…Primary adenocarcinoma of the appendix is an uncommon malignancy, representing less than 0.5% of all gastrointestinal neoplasms [1]. Among primary appendiceal carcinomas, appendiceal signet ring cell carcinoma (ASRCC) is an even rarer subset, constituting only 4% of all cases [2].…”

“…This overlap makes diagnosing ASRCC challenging, as it is frequently unsuspected before surgery [6]. Furthermore, ASRCC is considered an aggressive cancer, with metastases to adjacent organs, lymph nodes, or the peritoneal cavity present at the time of diagnosis in 93% of cases [1]. Consequently, early diagnosis and appropriate management are crucial for improving patient outcomes in these rare and aggressive malignancies [7].…”