Primary signet ring cell carcinoma of the pancreas: Cytopathology review of a rare entity

Hussein, Siba El; Khader, Samer

doi:10.1002/dc.24324

Cited by 20 publications

(12 citation statements)

References 55 publications

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“…SRCC of the pancreas resembles the more common gastric counterpart and is composed of at least 80% of histologically-proven poorly-cohesive cells with well-defined cytomorphological characteristics. Indeed, scattered non-cohesive cells with vacuolated cytoplasm and irregular nuclei are detected in this histotype; the signet ring appearance of these cells is due to the cytoplasmic mucin that moves the nuclei toward cell periphery (Figure 1) [141]. Of all cases of pancreatic cancer, SRCC occurs with the lowest frequency (<1%): consistently only few cases are reported in the literature [40,[142][143][144][145][146][147][148].…”

Section: Signet-ring Cell Carcinomamentioning

confidence: 98%

Morphologic and Molecular Landscape of Pancreatic Cancer Variants as the Basis of New Therapeutic Strategies for Precision Oncology

Bazzichetto

Luchini

Cognetti

et al. 2020

IJMS

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To date, pancreatic cancer is still one of the most lethal cancers in the world, mainly due to the lack of early diagnosis and personalized treatment strategies. In this context, the possibility and the opportunity of identifying genetic and molecular biomarkers are crucial to improve the feasibility of precision medicine. In 2019, the World Health Organization classified pancreatic ductal adenocarcinoma cancer (the most common pancreatic tumor type) into eight variants, according to specific histomorphological features. They are: colloid carcinoma, medullary carcinoma, adenosquamous carcinoma, undifferentiated carcinoma, including also rhabdoid carcinoma, undifferentiated carcinoma with osteoclast-like giant cells, hepatoid carcinoma, and signet-ring/poorly cohesive cells carcinoma. Interestingly, despite the very low incidence of these variants, innovative high throughput genomic/transcriptomic techniques allowed the investigation of both somatic and germline mutations in each specific variant, paving the way for their possible classification according also to specific alterations, along with the canonical mutations of pancreatic cancer (KRAS, TP53, CDKN2A, SMAD4). In this review, we aim to report the current evidence about genetic/molecular profiles of pancreatic cancer variants, highlighting their role in therapeutic and clinical impact.

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Section: Signet-ring Cell Carcinomamentioning

confidence: 98%

Morphologic and Molecular Landscape of Pancreatic Cancer Variants as the Basis of New Therapeutic Strategies for Precision Oncology

Bazzichetto

Luchini

Cognetti

et al. 2020

IJMS

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“…The accumulation of cytoplasmic mucin causes the nuclei to migrate to the periphery, giving them their signet cell form. 6 Based on observations of SRCC biological activity in gastric cancer, theories on how advanced disease pathology in SRCCs is inherited at the time of presentation have been established. 7 The diffuse-type tumors do not produce macroscopic or fungating lesions and are characterized by poor cell adhesion and lack of gland development; these tumors originated from signet ring cell, according to the Lauren classification developed in 1965.…”

Section: Introductionmentioning

confidence: 99%

Surgery improves survival in bladder signet-ring cell carcinoma-a population-based study

Alradhi

Safi

Tao

et al. 2022

Therapeutic Advances in Urology

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Objectives: The purpose of this study is to determine the therapeutic value of surgery in individuals with urinary bladder signet ring cell carcinoma (SRCC). Surgery has not been examined as a prognostic factor for urinary bladder cancer (SRCC). Materials and Methods: Using the Surveillance, Epidemiology, and End Results program (SEER), patients with urinary bladder SRCC who presented from 1975 to 2018 were included in a retrospective study. The effect of surgical therapy on cause-specific survival (CSS) and overall survival (OS) was examined using univariate and multivariate Cox regression models. We subdivided 595 patients with SRCC into 2 groups, as follows: 496 who underwent surgery; and 99 who did not undergo surgery. Results: Males had high predominance in all cases in both groups ( p = 0.04). Moderate and poor differentiation (III–IV) were observed in the majority of patients who underwent surgery (77.2 vs 58.6, p ⩽ 0.001) and had no insurance ( p ⩽ 0.001). By using KM, the OS and CSS of the surgery group were found to be significantly better than those of the non-surgery group ( p = 0.001,%) after adjusting for the variables of age, race, sex, primary site, grade, stage, lymph node removal, chemotherapy record, radiotherapy record, insurance, and marital status in the multivariate Cox proportional hazard model (hazard ratio [HR]= 0. 592; 95% confidence interval [CI] = 0.449–0.782; p = 0.0001). In comparison with chemotherapy and radiation, which resulted in poorer survival rates, surgery considerably improved survival outcomes in urinary bladder SRCC. The nomogram prediction model was built with C-index values of 0.70 and 73 for OS and CSS prediction, respectively. AUC in OS values were 0.77, 0.76, and 0.74, whereas AUC in CSS were 0.83, 0.80, and 0.79 for the 1-, 3-, and 5-year survival nomograms, respectively. Conclusion: Surgery was a significant independent predictor of bladder SRCC survival. Patients who underwent surgery had higher CSS and OS than people who did not undergo surgery. Surgery also led to better survival than the combination of the different treatment modalities.

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“…A signet ring has a flattened surface at the top of the ring with an engraved or raised symbol, historically used to stamp a seal into correspondence. Comparatively, these cells acquire a signet cell morphology due to accumulation of abundant mucin in cytoplasm, leading to nuclei dislocation to the periphery [ 3 ] ( Figure 1 ). Formally, a cancer is labelled a SRCC if greater than 50% of tumor cells show prominent intracytoplasmic mucin and an eccentrically placed crescent-shaped nucleus [ 4 ], whereas adenocarcinomas with less than 50% signet ring cells are classified as “adenocarcinomas with a signet ring cell component” [ 5 ].…”

Section: Introduction—overview Of Signet Ring Cell Adenocarcinomasmentioning

confidence: 99%

Epidemiology of Signet Ring Cell Adenocarcinomas

Benesch

Mathieson

2020

Cancers

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Signet ring cell adenocarcinomas (SRCCs) are a rare histological subtype of adenocarcinomas with a poor prognosis, typically due to advanced disease at diagnosis. A signet ring cell, mimicking its moniker, contains abundant intracytoplasmic mucin that pushes the nucleus to the periphery. In these cancers, this cell feature comprises more than 50% of the tumor. Despite predilection for the gastrointestinal tract, and in particular the stomach, primary SRCCs are also described in other sites, typically in case reports. This literature, however, lacks a standardized overview of the SRCC disease entity. Using a retrospective cohort approach, we summarize the clinicodemographic and mortality outcomes of SRCCs in thirteen primary sites, comprising 95% of all SRCCs in the Surveillance, Epidemiology, and End Results Program (SEER), a population-level cancer database covering nearly one-third of the United States population. SRCCs general trends compared to matching nonvariant adenocarcinomas are earlier age of onset, with initial presentation favoring higher rates of regional or distant disease presentation and poor tumor differentiation. After multivariable analysis, SRCCs typically have worse overall survivals, but substantial variances exist depending on tumor location. Identifying SRCCs at earlier disease stages is likely the single most important intervention to improving outcomes for these patients.

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Primary signet ring cell carcinoma of the pancreas: Cytopathology review of a rare entity

Cited by 20 publications

References 55 publications

Morphologic and Molecular Landscape of Pancreatic Cancer Variants as the Basis of New Therapeutic Strategies for Precision Oncology

Morphologic and Molecular Landscape of Pancreatic Cancer Variants as the Basis of New Therapeutic Strategies for Precision Oncology

Surgery improves survival in bladder signet-ring cell carcinoma-a population-based study

Epidemiology of Signet Ring Cell Adenocarcinomas

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