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Epidemiology, histopathology, clinical outcomes and survival of 50 cases of appendiceal mucinous neoplasms: Retrospective cross-sectional single academic tertiary care hospital experience.

ABSTRACT:

Background

Appendicular neoplasms are rare, most commonly as carcinoids followed by appendicular mucinous neoplasms (AMN). To date, there remains controversy regarding the best treatment of AMN and factors affecting its prognosis.

Method

Retrospective chart review of patients operated for appendicular pathology (January 2011-December 2018, follow up to December 2020) at our institution. For all AMN patients, data included pre-operative clinical presentation, and operative/post-operative findings.

Results

12454 patients underwent appendectomy, of whom 50 (0.4%) had AMN histopathologically (mean age = 47.2). Most patients had laparoscopic appendectomy as primary surgery. Low grade AMN was the most common subtype (n = 41, 82%), and pseudomyxoma peritonei (PMP) was found in 8 (16%) patients. Based on histopathology and margin involvement, the 50 patients were categorized into 3 prognostic categories of recurrence risk (no risk, 24 patients; low risk, 8; high recurrence risk, 18 patients). Disease-free survival (DFS) was lowest for high recurrence risk group (P < 0.001). Eleven (22%) patients had AMN involving resection margin, of whom 3 had no completion surgery and had no recurrence. Higher tumor markers were associated with lower DFS, however it was not statistically significant.

Conclusion

AMNs are rare but serious due to the risk of PMP. Laparoscopic approach for AMN may be feasible. Prognostic categories were significantly inversely correlated with recurrence risk; hence useful in predicting prognosis. Contrary to previous proposals, AMNs with acellular mucin at margin or local acellular mucin spillage may not require secondary surgery, especially if the patient is in low recurrence risk group. Tumor markers may predict risk of recurrence.

SUBMITTER: Aleter A

PROVIDER: S-EPMC8010208 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Introduction The prevalence of appendiceal mucinous neoplasms (AMN) is about 0.2%–0.3% in the specimens of the appendix. The LAMN may appear unremarkable or can present as mucin-filled, crystally dilated tissues. The diagnosis of early-stage AMN is mostly incidental. It is of vital importance to know the features of LAMN for a timely diagnosis Case presentation A 46-year-old male came with the complaint of right iliac fossa swelling along with severe intensity pain and a single episode of vomiting. A 4 × 4cm tender, soft, cystic, mobile swelling was found upon the examination. Contrast-enhanced Computerized tomography (CT) scan of the abdomen revealed the appendix diameter of 10mm well-circumscribed cystic measuring 2.1 × 2 cm. Therefore, an open resection surgery was performed. The histopathological report revealed an intraepithelial lesion composed of flat mucinous epithelial cells having eosinophilic cytoplasm and low-grade nuclear atypia. Case discussion The AMNs are very rare neoplasms. The imaging modalities that can be diagnostic methods are abdominal and transvaginal ultrasonography (US), and abdominal computed tomography (CT). The low-grade features of the appendiceal mucinous neoplasms have the possibility of recurrence. The CT-scan findings of appendiceal lump>1.3cm along with cystic dilation and the calcification of the wall are the suggestive features of AMN. There is a high chance of dissemination and port site seeding in case of pneumoperitoneum. Conclusion This rare case suggests the significance of adding AMNs as a differential diagnosis in patients with abdominal pain and choosing the right approach to treat such patients to avoid complications. Highlights • The incidence of low-grade appendiceal mucinous neoplasms (LAMN) is quite low.• The diagnosis of the LAMN is incidental and should be included in the differentials for abdominal pain.• There should be proper examination along with appropriate investigations for patients presenting with abdominal pain.• The timely diagnosis and the appropriate management allow better outcomes.• Laparoscopy is preferred as compared to open resection.