Background: The Peritoneal Surface Oncology Group International (PSOGI) recommends pathologic reporting of tumor cellularity in patients with pseudomyxoma peritonei (PMP) undergoing cytoreductive surgery and hyperthermic intraperitoneal chemoperfusion (CRS-HIPEC). We investigated the prognostic significance of PMP cellularity, or lack thereof (acellular mucin), following CRS-HIPEC.
Methods: We reviewed clinical data for 310 CRS-HIPEC procedures in low-grade (American Joint Committee on Cancer grade G1) PMP with acellular mucin (n = 19), scant cellularity (n = 30), or moderate cellularity (n = 242). Kaplan-Meier survival curves and multivariate Cox regression models identified prognostic factors affecting oncologic outcomes.
Results: Compared with patients with acellular mucin, those with scant and moderate cellularity had higher PCI and less-frequent complete macroscopic resection. After an estimated median follow-up of 49 months, 4 patients (14%) with scant cellularity and 127 patients (56%) with moderate cellularity progressed, while none of the patients with acellular mucin progressed. While the median progression-free survival (PFS) was not reached for patients with acellular mucin or scant cellularity (estimated 5-year PFS probability of 100 and 83%, respectively), patients with moderate cellularity demonstrated a median PFS of 32 months (estimated 5-year PFS probability of 27%). In a multivariate model, degree of disease cellularity, or lack thereof (acellular mucin), was an independent predictor of PFS but not overall survival.
Conclusions: Early disease progression is unlikely in patients with acellular mucin undergoing CRS-HIPEC, as opposed to a 14% recurrence rate with scant cellularity. Thorough pathologic assessment for cellularity, or lack thereof (acellular mucin), is vital for accurate prognostication of disease progression for patients with low-grade PMP undergoing CRS-HIPEC.
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http://dx.doi.org/10.1245/s10434-017-6214-7 | DOI Listing |
Case Rep Pathol
December 2024
Department of Obstetrics and Gynecology, Democritus University of Thrace, Alexandroupolis, Greece.
Appendiceal neoplasms are usually asymptomatic or associated with mild, nonspecific symptoms. Due to the rarity of the disease and the lack of specific symptoms, this clinical entity escapes the diagnostic consideration of the gynecologist, when women come in with right iliac fossa pain. A case is presented of a 56-year-old woman with a mass in the right small pelvis, which was preoperatively diagnosed as originating from the ovary.
View Article and Find Full Text PDFCancer Med
October 2024
Human Development and Health, Faculty of Medicine, University of Southampton, Southampton, UK.
Background And Aim: Pseudomyxoma peritonei (PMP) is an unusual condition with unique behaviour caused by a mucinous neoplasm, usually arising from the appendix. The aim of this study was to evaluate the prevalence of genomic alterations in clinical specimens of PMP using a targeted assay and correlate the findings with clinical, pathological and outcome data. Sequencing data from 223 patients were analysed.
View Article and Find Full Text PDFAnn Surg Oncol
December 2024
Department of Surgery, St. Antonius Hospital, Nieuwegein, The Netherlands.
Background: International consensus on classifications of appendiceal mucinous neoplasms (AMNs) and associated pseudomyxoma peritonei (PMP) have been carefully made but clinicopathological associations supporting decision making remain scarce.
Objective: This study aimed to assess interdependence between AMNs and PMP and provide directions for clinical management.
Methods: This two-center retrospective cohort study reviewed patients with PMP treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy between 2005 and 2021.
Cureus
August 2024
Department of Pathology and Laboratory Medicine, School of Medicine, Kanazawa Medical University, Uchinada, JPN.
Myoepitheliomas are rare salivary gland-type tumors. The tumors are divided into four histological subtypes (spindle cell, plasmacytoid, epithelioid, and clear cell) and two variants (reticular and mucinous). A myoepithelioma of the mucinous variant, also referred to as mucinous or secretory myoepithelioma, is a novel variant of myoepithelioma characterized by the presence of extracellular mucin.
View Article and Find Full Text PDFJ Gastrointest Surg
November 2024
Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, United States. Electronic address:
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