Low Grade Appendiceal Mucinous Neoplasms- A Short Case Series with Review of Literature

  • S Salapathi Department of Histopathology, Apollo Specialty Hospitals, Vanagaram, Chennai -600095 India
  • B Rajeshwari Department of Histopathology, Apollo Specialty Hospitals, Vanagaram, Chennai -600095 India
  • Sadiya Niamath Department of Histopathology, Apollo Specialty Hospitals, Vanagaram, Chennai -600095 India
  • Mitra Ghosh Department of Histopathology, Apollo Specialty Hospitals, Vanagaram, Chennai -600095 India
Keywords: Mucinous neoplasms, Appendix, Pseudomyxoma peritonei

Abstract

Low-grade appendiceal mucinous neoplasm (LAMN) is a rare malignancy and is found in less than 0.3% of appendectomy specimens. Patients with LAMN can present with abdominal pain and mimic clinically as appendicitis, intussusception or obstruction, hence high clinical suspicion is needed for correct diagnosis and management. The exact nature and nomenclature of these tumors when they disseminate into the peritoneum is controversial. The prognosis of LAMN is highly dependent on the presence or absence of neoplastic epithelium outside the appendix. Tumors with extra appendiceal acellular mucin in the right lower quadrant only rarely recur but tumors with extra appendiceal mucin and neoplastic epithelium recur more often. LAMNs with diffuse peritoneal seeding of neoplastic epithelium and mucin have a progressive clinical course that frequently results in death of the patient.  Hence whenever a case of mucinous neoplasm of the appendix is encountered, it is essential that the entire appendix be submitted for histopathologic examination and should be thoroughly evaluated for presence of extra appendiceal mucin and neoplastic epithelium, and also for the type of invasion in order to obtain the correct diagnosis and to predict the risk of recurrence. We present here three cases of low grade appendiceal tumors with their varied clinical presentation and histopathological findings, for their rarity and clinical importance.

References

Misdraji J. Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei. Mod Pathol. 2015 ;28:67-79.

Misdraji J, Yantiss RK, Graeme-Cook FM, Balis UJ, Young RH. Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases. Am J Surg Pathol 2003; 27:1089–103.

Pai RK, Beck AH, Norton JA, Longacre TA. Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol 2009;33:1425–39.

Gonzalez HH, Herard K, Mijares MC. A Rare Case of Low-grade Appendiceal Mucinous Neoplasm: A Case Report . Cureus 2019;11: e3980. DOI 10.7759/cureus.3980

Ramaswamy V. Pathology of mucinous appendiceal tumors and pseudomyxoma peritonei . Indian J Surg Oncol. 2016, 7:258-67.

Padmanaban V, Morano WF, Gleeson E, Aggarwal A, Mapow BL, Stein DE et al. Incidentally discovered low-grade appendiceal mucinous neoplasm: a precursor to pseudomyxoma peritonei. Clin Case Rep. 2016; 4:1112- 6.

Misdraji J. Epithelial neoplasms of Appendix. In: Odze and Goldblum’s Surgical Pathology of the GI Tract, Liver, Biliary Tract, and Pancreas. 3rd edition. Philadelphia: Elsevier Saunders; 2015. p 779-91.

Carr NJ, Sobin LH. Adenocarcinoma of the appendix, In: Bosman FT, Carneiro F, Hruban RH, et al. editors. World Health Organization Classification of Tumours of the Digestive System. IARC Press: Lyon, France; 2010, pp 122–5.

McGregor JK, McGregor DD. Adenocarcinoma of the appendix. Surgery 1960;48:925–35.

Steinberg M, Cohn I Jr. Primary adenocarcinoma of the appendix. Surgery 1967;61:644–60.

Uihlein A, McDonald JR. Primary carcinoma of the appendix resembling carcinoma of the colon. Surg Gynecol Obstet 1943;76: 711–4.

Wilson R. Primary carcinoma of the appendix. Am J Surg 1962; 104:238–49.

Woodruff R, McDonald JR. Benign and malignant cystic tumors of the appendix. Surg Gynecol Obstet 1940;71:750–5.

Gibbs NM. Mucinous cystadenoma and cystadenocarcinoma of the vermiform appendix with particular reference to mucocele and pseudomyxoma peritonei. J Clin Pathol 1973;26:413–21.

Higa E, Rosai J, Pizzimbono CA, Wise L. Mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma of the appendix: a re-evaluation of appendiceal ‘mucocele.’ Cancer 1973; 32:1525–41

Carr NJ, McCarthy WF, Sobin LH. Epithelial noncarcinoid tumors and tumor-like lesions of the appendix: a clinicopathologic study of 184 patients with a multivariate analysis of prognostic factors. Cancer 1995; 75:757-768.

Caspi B, Cassif E, Auslender R, Herman A, Hagay Z, Appelman Z. The onion skin sign. a specific sonographic marker of appendiceal mucocele. J Ultrasound Med 2004; 23: 117-21.

Persaud T, Swan N, Torreggiani WC. Giant mucinous cystadenoma of the appendix. Radiographics 2007; 27: 553-57.

Tirumani SH, Fraser-Hill M, Auer R, Shabana W, Walsh C, Lee F et al. Mucinous neoplasms of the appendix: a current comprehensive clinicopathologic and imaging review. Cancer Imaging 2013; 13: 14–25.

Li X, Zhou J, Dong M, Yang L. Management and prognosis of low-grade appendiceal mucinous neoplasms: A clinicopathologic analysis of 50 cases. Eur J Surg Oncol. 2018;44:1640-5.

McDonald JR, O'Dwyer ST, Rout S, Chakrabarty B, Sikand K, Fulford PE et al. Classification of and cytoreductive surgery for low-grade appendiceal mucinous neoplasms. Br J Surg 2012;99:987-92.

Yu XR, Mao J, Tang W, Meng XY, Tian Y,Du ZL. Low-grade appendiceal mucinous neoplasms confined to the appendix: clinical manifestations and CT findings. J Investig Med 2020;68:75–81.

Nishikawa G, Sekine S, Ogawa R, Matsubara A, Mori T, Taniguchi H et al. Frequent GNAS mutations in low-grade appendiceal mucinous neoplasms. Br J Cancer 2013;108:951–8.

Werth R. Klinische und anatomische untersuchungen zur lehre von den bauchgeschwuelsten und der lapartomie. Arch f Gynaek 1884;24:100–18.

Misdraji J, Young RH: Primary epithelial neoplasms and other epithelial lesions of the appendix (excluding carcinoid tumors). Semin Diagn Pathol. 2004, 21:120-33.

Yantiss RK, Shia J, Klimstra DS, Hahn HP, Odze RD, Misdraji J. Prognostic significance of localized extra-appendiceal mucin deposition in appendiceal mucinous neoplasms. Am J Surg Pathol. 2009;33:248-55.

Gonzalez-Moreno S, Sugarbaker PH. Right hemicolectomy does not confer a survival advantage in patients with mucinous carcinoma of the appendix and peritoneal seeding. Br J Surg 2004; 91:304-11.

Foster JM, Gupta PK, Carreau JH, Grotz TE, Blas JV, Gatalica Z et al. Right hemicolectomy is not routinely indicated in pseudomyxoma peritonei. Am Surg 2012;78:171-7.

Published
2021-03-31
Section
Case Report