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Appendiceal mucinous neoplasm — Clinical Guidelines

Overview

Appendiceal mucinous neoplasms encompass a spectrum of tumors ranging from benign to malignant, including pseudomyxoma peritonei (PMP), with variable biological behaviors and treatment approaches 1.

Diagnosis

Colonoscopy: Can identify appendiceal polyps and early malignancies, including carcinoma in situ 3.

Pathological Classification: Utilizes various systems to categorize tumors based on grade and spread 1.

Imaging: Not explicitly detailed; typically includes CT and MRI for staging and assessing extent 1.

Histopathology: Essential for definitive diagnosis, distinguishing between benign and malignant lesions 1 4.

Management

Simple Surveillance/Laparoscopic Appendectomy: For low-grade, localized lesions 1.

Complete Cytoreductive Surgery (CRS) with Hyperthermic Intraperitoneal Chemotherapy (HIPEC): Recommended for high-grade tumors and PMP 1.

Systemic Chemotherapy: Adjunctive therapy, specific drug classes not detailed in abstracts 1.

Right Hemicolectomy: Treatment of choice for proven appendiceal adenocarcinoma 5.

Special Populations

Ovarian Metastasis: Routine appendectomy recommended in cases presenting with bilateral Krukenberg tumors, suspecting appendiceal origin 4.

No Specific Guidance: Limited evidence for management in pregnancy, pediatrics, or elderly patients 1 3 5.

Key Recommendations

Tailor treatment based on pathological grade and extent of disease, ranging from surveillance to CRS with HIPEC (Evidence: Strong 1).

Routine appendectomy is advised in patients presenting with ovarian tumors suspicious for appendiceal origin (Evidence: Moderate 4).

Right hemicolectomy is indicated for confirmed cases of appendiceal adenocarcinoma (Evidence: Strong 5).

References

1 Delhorme JB, Villeneuve L, Bouché O, Averous G, Dohan A, Gornet JM et al.. Appendiceal tumors and pseudomyxoma peritonei: French Intergroup Clinical Practice Guidelines for diagnosis, treatments and follow-up (RENAPE, RENAPATH, SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO, ACHBT, SFR). Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver 2022. link 2 Shaib W. In Reply. The oncologist 2020. link 3 Hardin RD. Colonoscopic diagnosis of an appendiceal polyp with carcinoma in situ. Journal of clinical gastroenterology 1986. link 4 Hirschfield LS, Kahn LB, Winkler B, Bochner RZ, Gibstein AA. Adenocarcinoid of the appendix presenting as bilateral Krukenberg's tumor of the ovaries. Immunohistochemical and ultrastructural studies and literature review. Archives of pathology & laboratory medicine 1985. link 5 Reenalda PE, Bruggink ED. Tumors of the appendix. The Netherlands journal of surgery 1984. link 6 Mehzad M, Aflaki B, Afghari H. Adenocarcinoma of the appendix: report of an unusual case. Diseases of the colon and rectum 1978. link

Appendiceal mucinous neoplasm