Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases

Low-grade Appendiceal Mucinous Neoplasm (LAMN) is a rare malignant neoplasm seen in less than 0.3% of appendectomy specimens. Patients with LAMN can present with abdominal pain, mimicking appendicitis clinically. In later stages, it can rupture, leading to Pseudomyxoma peritonei (PMP). Therefore,...

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Main Authors: Chandan Chowdhuri, Ashwinkumar K Barsagade, Sivaganesh Alias Porko, Avnika jasuja, Suparna Dubey
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2023-07-01
Series:Journal of Clinical and Diagnostic Research
Subjects:
Online Access:https://jcdr.net/articles/PDF/18232/63453_CE[Ra1]_F(SHU)_PF1(AN_IS)_PF2(AG_KM)_PFA(AG_KM)_PN(KM).pdf
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author Chandan Chowdhuri
Ashwinkumar K Barsagade
Sivaganesh Alias Porko
Avnika jasuja
Suparna Dubey
author_facet Chandan Chowdhuri
Ashwinkumar K Barsagade
Sivaganesh Alias Porko
Avnika jasuja
Suparna Dubey
author_sort Chandan Chowdhuri
collection DOAJ
description Low-grade Appendiceal Mucinous Neoplasm (LAMN) is a rare malignant neoplasm seen in less than 0.3% of appendectomy specimens. Patients with LAMN can present with abdominal pain, mimicking appendicitis clinically. In later stages, it can rupture, leading to Pseudomyxoma peritonei (PMP). Therefore, a high clinical suspicion is needed for correct diagnosis and management. The prognosis of LAMN depends on the presence or absence of mucin/neoplastic epithelium outside the appendix. For LAMN confined to the appendix, appendectomy alone is sufficient for management, with conservative follow-up. For LAMN with extruded acellular mucin localised to the appendiceal serosa, management involves appendectomy and PMP surveillance. For all mucinous lesions of the appendix, it is essential to submit the entire appendix for histopathologic examination, thoroughly evaluating the presence of mucin/neoplastic epithelium involving the serosa/extra appendiceal tissue for accurate staging and to predict the risk of recurrence and determine further management. Here four cases of LAMN due to their rarity and clinical importance is presented. This study of four cases emphasises that the clinical diagnosis of this rare malignancy is difficult, and radiological investigations were not available as these patients presented with clinical features mimicking appendicitis. Emergency appendectomy was performed in all cases. LAMN should be considered as a differential diagnosis because if not diagnosed and treated in the early stages, it can lead to dreaded complications like PMP. The histopathological examination of all four cases of LAMN showed dissecting acellular mucin in various stages of progression in the appendicular wall, but none involving the serosa. This highlights the importance of timely surgical intervention and histopathological examination for an exact diagnosis and staging of LAMN for further follow-up or treatment.
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spelling doaj.art-4ee1db48bb5c44e9a2aa149a7f5029b42023-08-07T11:59:18ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2023-07-01177061010.7860/JCDR/2023/63453.18232Low-grade Appendiceal Mucinous Neoplasm: A Series of Four CasesChandan Chowdhuri0Ashwinkumar K Barsagade1Sivaganesh Alias Porko2Avnika jasuja3Suparna Dubey4Assistant Professor, Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar, India.Associate Professor, Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar, India.Associate Professor, Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar, India.Assistant Professor, Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar, India.Professor and Head, Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar, India.Low-grade Appendiceal Mucinous Neoplasm (LAMN) is a rare malignant neoplasm seen in less than 0.3% of appendectomy specimens. Patients with LAMN can present with abdominal pain, mimicking appendicitis clinically. In later stages, it can rupture, leading to Pseudomyxoma peritonei (PMP). Therefore, a high clinical suspicion is needed for correct diagnosis and management. The prognosis of LAMN depends on the presence or absence of mucin/neoplastic epithelium outside the appendix. For LAMN confined to the appendix, appendectomy alone is sufficient for management, with conservative follow-up. For LAMN with extruded acellular mucin localised to the appendiceal serosa, management involves appendectomy and PMP surveillance. For all mucinous lesions of the appendix, it is essential to submit the entire appendix for histopathologic examination, thoroughly evaluating the presence of mucin/neoplastic epithelium involving the serosa/extra appendiceal tissue for accurate staging and to predict the risk of recurrence and determine further management. Here four cases of LAMN due to their rarity and clinical importance is presented. This study of four cases emphasises that the clinical diagnosis of this rare malignancy is difficult, and radiological investigations were not available as these patients presented with clinical features mimicking appendicitis. Emergency appendectomy was performed in all cases. LAMN should be considered as a differential diagnosis because if not diagnosed and treated in the early stages, it can lead to dreaded complications like PMP. The histopathological examination of all four cases of LAMN showed dissecting acellular mucin in various stages of progression in the appendicular wall, but none involving the serosa. This highlights the importance of timely surgical intervention and histopathological examination for an exact diagnosis and staging of LAMN for further follow-up or treatment.https://jcdr.net/articles/PDF/18232/63453_CE[Ra1]_F(SHU)_PF1(AN_IS)_PF2(AG_KM)_PFA(AG_KM)_PN(KM).pdfappendicectomymucinpseudomyxoma peritonei
spellingShingle Chandan Chowdhuri
Ashwinkumar K Barsagade
Sivaganesh Alias Porko
Avnika jasuja
Suparna Dubey
Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases
Journal of Clinical and Diagnostic Research
appendicectomy
mucin
pseudomyxoma peritonei
title Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases
title_full Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases
title_fullStr Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases
title_full_unstemmed Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases
title_short Low-grade Appendiceal Mucinous Neoplasm: A Series of Four Cases
title_sort low grade appendiceal mucinous neoplasm a series of four cases
topic appendicectomy
mucin
pseudomyxoma peritonei
url https://jcdr.net/articles/PDF/18232/63453_CE[Ra1]_F(SHU)_PF1(AN_IS)_PF2(AG_KM)_PFA(AG_KM)_PN(KM).pdf
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AT sivaganeshaliasporko lowgradeappendicealmucinousneoplasmaseriesoffourcases
AT avnikajasuja lowgradeappendicealmucinousneoplasmaseriesoffourcases
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