Pooled analysis of rhabdoid pancreatic carcinomas: Should they be resected?

Abstract

Background

Pancreatic undifferentiated carcinomas with rhabdoid features (PUCR) are infrequent, yet incredibly aggressive neoplasms. Recent advances in the histopathological understanding of PUCR have been made, however the optimal treatment of PUCR remains unclear and the decision to operate on the neoplasm is left to the physicians own judgement. Most of the literature published on this neoplasm constitutes case reports and case series, therefore our aim is to present a pooled analysis including the up-to-date literature and elucidate whether surgical treatment is the finest choice for PUCR.

Methods

This pooled analysis compared the data from 9 articles and a case that presented to our unit, yielding 28 cases. Treatment modalities reported in the literature were noted, and the surgical and post-operative adjuvant chemotherapy and >3month survival were screened for dependence using a Chi-square test.

Results

The patient's median survival following a surgical resection of the neoplasm was three months. 77.7% who were treated surgically died within one year following the surgery. The relationship between surgical resection and survival calculated using the chi-square is not significant (p-value: 0.261). The median survival of patients who received post-operative chemotherapy was 7 months. The relationship between the post-operative adjuvant chemotherapy and survival 3 months is also not significant (p-value: 0.065)

Conclusions

The aggressive nature of PUCR results in a rapid deterioration regardless of the treatment modality chosen. Surgery even in resectable patients doesn't carry a significant survival benefit. Chemotherapy should remain the mainstay of therapy for this patient cohort.