Prognostic significance of localized extra-appendiceal mucin deposition in appendiceal mucinous neoplasms Journal Article


Authors: Yantiss, R. K.; Shia, J.; Klimstra, D. S.; Hahn, H. P.; Odze, R. D.; Misdraji, J.
Article Title: Prognostic significance of localized extra-appendiceal mucin deposition in appendiceal mucinous neoplasms
Abstract: Appendiceal mucinous neoplasms confined to the mucosa are benign, whereas those with disseminated peritoneal mucin deposits often follow an indolent, but malignant, course. Not infrequently, appendiceal mucinous neoplasms are associated with localized periappendiceal mucin deposits, but lack diffuse peritoneal involvement. Mucin deposits in these cases may be acellular or contain neoplastic epithelium (cellular mucin). Although some investigators consider both acellular and cellular periappendiceal mucin to pose no, or minimal, risk for recurrent disease, the biologic importance of localized extra-appendiceal mucin has never been evaluated. We identified 65 patients with appendiceal mucinous neoplasms, all of whom had localized periappendiceal mucin deposits without diffuse peritoneal involvement, and assessed them for the presence of extra-appendiceal epithelium and clinical outcome. Forty-nine (75%) appendices were submitted in total for histologic evaluation. Most (77%) cases showed acellular periappendiceal mucin, but 15 (23%) had scant extra-appendiceal epithelium (range: 1 to 12 cell clusters). Upon follow-up (mean: 48mo), 2 (4%) patients with acellular periappendiceal mucin developed diffuse peritoneal disease, but neither of these appendices was submitted in total for histologic evaluation. In contrast, 5 of 15 (33%) patients with cellular periappendiceal mucin developed mucinous ascites, including 1 who eventually died of disease (P=0.03). Thus, patients with appendiceal mucinous neoplasms and acellular periappendiceal mucin are unlikely to develop recurrent disease. However, microscopic examination of the entire appendix is necessary, as lesions with extra-appendiceal tumor cells are more likely to progress to disseminated disease and result in death of the patient, even if the mucin is paucicellular and confined to the periappendiceal region.©2009 by Lippincott Williams & Wilkins.
Keywords: adult; child; controlled study; school child; aged; aged, 80 and over; middle aged; major clinical study; histopathology; follow up; metabolism; neoplasm recurrence, local; adenocarcinoma, mucinous; pathology; tumor recurrence; colloid carcinoma; ascites fluid; mucinous adenocarcinoma; mucin; appendix tumor; disseminated peritoneal adenomucinosis; pseudomyxoma peritonei; low-grade appendiceal mucinous neoplasm; appendiceal mucinous neoplasm; appendix cancer; peritoneal disease; appendiceal neoplasms; mucins
Journal Title: American Journal of Surgical Pathology
Volume: 33
Issue: 2
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 2009-02-01
Start Page: 248
End Page: 255
Language: English
DOI: 10.1097/PAS.0b013e31817ec31e
PUBMED: 18852679
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 8" - "Export Date: 30 November 2010" - "CODEN: AJSPD" - "Source: Scopus"
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  1. David S Klimstra
    978 Klimstra
  2. Jinru Shia
    720 Shia