Management of Low-Grade Appendiceal Mucinous Neoplasms (LAMN): An International Survey of Surgeons Performing CRS and HIPEC

Abstract A mucocele refers to an appendix that has dilated due to progressive accumulation of mucus within its lumen. Appendiceal mucocele is a rare cause of an acute abdomen. They represent 0.2-0.7% of all appendix specimens. LAMN account for less than 0.3% of appendicectomy specimens. We present a 38 year old man with an acute RIF’s pain who went on to have CT scan which revealed a mucocele of appendix but did not show any features of perforation or pseudomyxoma peritonei. He had a laparoscopic converted to open appendicectomy. The proximal 2 cm of appendix was oedematous but normal calibre. Histology revealed a low grade appendiceal mucinous neoplasm (LAMN) that was completely excised. The mucocele of the appendix was first described by Rokitansky in 1842. Appendix mucocele may come as a consequence of obstructive or inflammatory processes, cystadenomas or cystadenocarcinomas. Appendiceal mucinous neoplasms commonly presents in the sixth decade of life and our patient was much younger in comparison. Several literatures suggest the value of preoperative CT imaging in obtaining diagnosis and also in planning further treatment. Appendicectomy or a right hemicolectomy is treatment of choice based on presence or absence of following factors 1. Perforated mucocele 2. Involvement of the base of the appendix. 3. Positive lymph nodes of mesoappendix and ileocolic. Patients with malignancy or pseudomyxoma peritonei are likely to require cytoreductive surgery, heated intraoperative intraperitoneal chemotherapy, early postoperative intraperitoneal chemotherapy.

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Outcomes of Low-Grade Appendiceal Mucinous Neoplasms with Remote Acellular Mucinous Peritoneal Deposits

Annals of Surgical Oncology ◽

10.1245/s10434-018-7003-7 ◽

2018 ◽

Vol 26 (1) ◽

pp. 118-124 ◽

Cited By ~ 9

Author(s):

Campbell S. Roxburgh ◽

Yaniv M. Fenig ◽

Andrea Cercek ◽

Jinru Shia ◽

Rachel M. Rassam ◽

...

Keyword(s):

Low Grade ◽

Mucinous Neoplasms

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GNAS is frequently mutated in both low-grade and high-grade disseminated appendiceal mucinous neoplasms but does not affect survival

Human Pathology ◽

10.1016/j.humpath.2014.04.018 ◽

2014 ◽

Vol 45 (8) ◽

pp. 1737-1743 ◽

Cited By ~ 41

Author(s):

Aatur D. Singhi ◽

Jon M. Davison ◽

Haroon A. Choudry ◽

James F. Pingpank ◽

Steven A. Ahrendt ◽

...

Keyword(s):

Low Grade ◽

High Grade ◽

Mucinous Neoplasms

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Low-Grade Appendiceal Mucinous Neoplasms: A Single Institution Experience of 64 Cases With Clinical Follow-up and Correlation With the Current (Eighth Edition) AJCC Staging

International Journal of Surgical Pathology ◽

10.1177/1066896919883679 ◽

2019 ◽

Vol 28 (3) ◽

pp. 252-258 ◽

Cited By ~ 2

Author(s):

Mary Wong ◽

Brad Barrows ◽

Alexandra Gangi ◽

Stacey Kim ◽

Richard B. Mertens ◽

...

Keyword(s):

Progressive Disease ◽

Low Grade ◽

Single Institution ◽

Mucinous Neoplasms ◽

Pathologic Staging ◽

Eighth Edition ◽

Ajcc Staging ◽

Disease Free ◽

Histopathologic Features

Background. In this single-institution study, we applied the current (eighth edition) American Joint Committee on Cancer pathologic staging criteria to 64 low-grade mucinous neoplasms of the appendix (LAMNs), examined their histopathologic features, and studied the patients’ clinical outcomes. Design. Sixty-four LAMNs, with a median follow-up of 52 months, were reviewed. Results. The distribution of pathologic stages was pTis (n = 39), pT3 (n = 1), pT4a (n = 5), pT4aM1a (n = 8), and pT4aM1b (n = 11). Recurrence was observed in only 2 patients (both with pT4aM1b disease), one of whom died of disease. All remaining patients were disease-free after a median clinical follow-up of 60 months. Conclusions. Our study confirms that pTis LAMNs have an excellent prognosis and suggests that pT4a and pT4aM1a LAMNs may also have a low risk of developing progressive disease.

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Prognostic Significance of Tumor Markers in Low Grade Appendiceal Mucinous Neoplasms: A Multi-Institutional Collaborative

Journal of the American College of Surgeons ◽

10.1016/j.jamcollsurg.2020.08.562 ◽

2020 ◽

Vol 231 (4) ◽

pp. e210-e211

Author(s):

Wasay Nizam ◽

Jordan Cloyd ◽

Sean Dineen ◽

Sameer H. Patel ◽

Harveshp Darabshah Mogal ◽

...

Keyword(s):

Tumor Markers ◽

Prognostic Significance ◽

Low Grade ◽

Mucinous Neoplasms

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Is there a role for neoadjuvant chemotherapy in the management of non-carcinoid epithelial neoplasms of the appendix?

Journal of Clinical Oncology ◽

10.1200/jco.2007.25.18_suppl.15115 ◽

2007 ◽

Vol 25 (18_suppl) ◽

pp. 15115-15115

Author(s):

M. H. Katz ◽

P. F. Mansfield ◽

C. Eng ◽

R. A. Wolff ◽

P. Diaz ◽

...

Keyword(s):

Neoadjuvant Chemotherapy ◽

Standard Of Care ◽

Low Grade ◽

High Grade ◽

Appendiceal Neoplasms ◽

Tertiary Center ◽

Standardized Treatment ◽

Crs And Hipec ◽

Epithelial Neoplasms

15115 Background: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC) are considered the standard of care for patients with pseudomyxoma peritonei (PMP) and carcinomatosis of appendiceal origin. The role of neoadjuvant chemotherapy (CTX) in the management of these patients is unknown. Methods: Retrospective analysis of all patients evaluated for the treatment of appendiceal epithelial neoplasms at a tertiary center between 1992 and August 2006. All diagnoses were confirmed pathologically and classified by a single group of pathologists. Patients with carcinoid tumors or metastases to the appendix were excluded. Tumor histology, stage, peritoneal-based disease, and the potential for complete cytoreduction dictated treatment. Results: 250 consecutive patients were evaluated, 140 of whom had low grade disease. 114 underwent CRS and HIPEC. Median follow-up was 24 mos from referral. Average time to referral was 13 mos after diagnosis (range 0–243); 85% had undergone prior surgical therapy (median 1.3 operations, range 1–4); 22% had previous CTX consisting of many different regimens. 5- and 10-year survival for patients with low grade tumors treated with CRS and HIPEC alone (n=80) were 84% and 68%, respectively. 21 patients with low grade tumors had CTX prior to CRS and HIPEC. There was no effect on overall survival (p = 0.61). 5-and 10-year survival of 39 patients with low grade histology who did not receive CRS and HIPEC was 55% and 30%, respectively (p = 0.009). 83 patients with intermediate and high-grade disease who received CTX but not CRS and HIPEC had a 5- year survival of 27%. 5-year survival for patients with intermediate or high grade disease who underwent CRS and HIPEC (n=13) was 67%. Conclusions: Patients with peritoneal-based disease from non-carcinoid epithelial neoplasms of the appendix who undergo CRS and HIPEC have a more favorable survival. Currently there is no survival advantage to the use of CTX before CRS and HIPEC for low grade appendiceal neoplasms. The role of neoadjuvant CTX and biologic agents for patients with high grade neoplasms needs to be determined. Early referral to a peritoneal malignancy center will help standardized treatment for these patients. No significant financial relationships to disclose.

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