scholarly journals Mucinous neoplasm of the appendix: management according to reviewed histologic spectral aspects

Author(s):  
Carlos Augusto Gomes ◽  
Cleber Soares Junior ◽  
Federico Coccolini ◽  
Giulia Montori ◽  
Rafaela Clara Resende da Silva ◽  
...  

Of the diseases affecting the cecal appendix, mucinous neoplasms, although rare, are the most common epithelial tumor. They are often asymptomatic and diagnosed by anatomopathological exams of sections, collected during appendectomies due to acute appendicitis. These tumors have the potential to become malignant, and their early surgical handling are the most important prognostic factor. So, clinical, image, operatory and histologic spectral aspects should always take into account. The present study reports a case of mucinous neoplasm of the appendix that manifested with intestinal partial obstruction and suggests the need for a comprehensive and uniform classification, which could address the best disease first approach. The information was obtained through a patient records review, photographic documentation of the diagnostic tests and histologic study of operative specimen. In addition, the manuscript opens a discussion about the approach of choice of this complex neoplasia, since great variability persists in clinical and pathological records, due to variations in classifications and definitions. This variability in disease nomenclature may significantly impact the treatment and prognosis.

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Kimberly Da Costa ◽  
Sivakumaran Sabanathan

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.


2020 ◽  
Vol 21 (16) ◽  
pp. 5794 ◽  
Author(s):  
Paola Mattiolo ◽  
Seung-Mo Hong ◽  
Gaetano Paolino ◽  
Borislav C. Rusev ◽  
Giovanni Marchegiani ◽  
...  

The intraductal oncocytic papillary neoplasm (IOPN) of the pancreas has been recognized by WHO classification as a unique intraductal papillary mucinous neoplasm (IPMN) category. IOPN is composed of oxyphil cells, usually expressing MUC5AC, MUC6, and Hep Par-1, and harboring PRKACA/B fusion genes as their genetic hallmark. Although IOPNs are associated with an infiltrative adenocarcinoma in up to 30% of cases, the survival rate after surgical resection approaches 100%. This highlights the importance of the correct IOPN diagnosis, above all in cases with an associated invasive component. In this study, the immunohistochemical expression of CD117 was investigated in 111 IPMNs, including 17 oncocytic, 45 gastric, 20 pancreatico-biliary, and 29 intestinal IPMNs. We also tested the expression of MUC5AC, MUC6, and Hep Par-1 in the IOPN cohort. CD117 positivity was significantly more frequent in IOPNs compared to the other IPMN subtypes (p < 0.0001). Furthermore, within IOPN, a lower or absent CD117, MUC5AC, MUC6, and Hep Par-1 expression tended to be associated with the presence of an infiltrative component. Our findings shed light into the biology of these complex lesions, which are confirmed to be a distinctive IPMN subtype; notably, CD117 emerged as a potential, additional tool in the differential diagnosis of IPMNs.


2003 ◽  
Vol 124 (4) ◽  
pp. A441
Author(s):  
Mauricio Zapiach ◽  
Dhiraj Yadav ◽  
Thomas C. Smyrk ◽  
Joel G. Fletcher ◽  
Randall K. Pearson ◽  
...  

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Sawsan Ismail ◽  
Rama Darwisho ◽  
Mohammed Ali ◽  
Maen Haidar ◽  
Mohammad Adib Houreih ◽  
...  

Abstract Background Intraductal papillary mucinous neoplasms are rare papillary pancreatic neoplasms arising from major pancreatic ducts, characterized by duct dilation and mucin secretion. They comprise approximately 1% of all exocrine neoplasms and are classified according to their anatomical sites into main duct-type, branch duct-type, and mixed-type intraductal papillary mucinous neoplasms. Histological examination plays a crucial role in distinguishing and classifying intraductal papillary mucinous neoplasms into gastric, intestinal, pancreatobiliary, and oncocytic subtypes. Case presentation We present the case of a 70-year-old Syrian woman who was admitted to our hospital due to an intermittent epigastric pain accompanied by diarrhea and weight loss with a recent diagnosis of diabetes mellitus. Following clinical, laboratory, and radiological examination, distal pancreatectomy involving the body and the tail of the pancreas was performed. Interestingly, histological examination of the resected specimens revealed the diagnosis of a mixed-type intraductal papillary mucinous neoplasm with a unique combination of gastric and pancreatobiliary subtypes. Conclusion To the best of our knowledge, the combination of multiple histological subtypes of intraductal papillary mucinous neoplasms has been recorded in a few studies with reference to the challenging histological detection. Herein, we report a rare case with a significant histological combination, highlighting the difficulties in differential diagnosis due to the absence of ancillary techniques, with a brief review on diagnostic methods, histological characteristics and surgical recommendations.


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Vera Vavinskaya ◽  
Joel M. Baumgartner ◽  
Albert Ko ◽  
Cheryl C. Saenz ◽  
Mark A. Valasek

Primary appendiceal mucinous lesions are uncommon and represent a spectrum from nonneoplastic mucous retention cysts to invasive adenocarcinoma. Low-grade appendiceal mucinous neoplasms (LAMNs) represent an intermediate category on this spectrum and can be classified according to whether or not they are confined to the appendix. Although LAMNs are frequently confined to the appendix, they can also spread to the peritoneum and clinically progress as pseudomyxoma peritonei (i.e., mucinous ascites). Thus, the appropriate classification of appendiceal primary neoplasia is essential for prognosis and influences clinical management. In addition, the precise classification, management, and clinical outcome of patients with disseminated peritoneal disease remain controversial. Here, we report an unusual case of LAMN with pseudomyxoma peritonei that initially presented with mucinous and bloody vaginal discharge. Pathological evaluation revealed low-grade appendiceal mucinous neoplasm with secondary involvement of the peritoneum, ovaries, and endometrial surface. Therefore, LAMN should be considered in the differential diagnosis of mucinous vaginal discharge.


2021 ◽  
pp. 1-8
Author(s):  
Tatsuro Yamaguchi ◽  
Kohei Murata ◽  
Tetsuya Shiota ◽  
Hiroshi Takeyama ◽  
Shingo Noura ◽  
...  

Introduction: Recently, “low-grade appendiceal mucinous neoplasms” (LAMNs) have been proposed as one subtype of appendiceal mucinous neoplasms, characterized by a villous or flat proliferation of mucinous epithelium with low-grade cytologic atypia. The aim of this study was to clarify the clinicopathological characteristics of LAMN. Methods: In this multi-institutional cohort study, we retrospectively analyzed the clinicopathological characteristics in appendiceal neoplasms patients who underwent treatment from 2000 to 2017. Results: In total, 922 patients were enrolled, with 279 (30.3%) cases of LAMN, and 93 (10.1%) cases of non-LAMN disease. In comparison with patients with non-LAMN disease, those with LAMN had significantly lower levels of CA19-9 (p = 0.045), a lower frequency of T4 tumors (p < 0.0001), a lower frequency of lymph node metastasis (p < 0.0001), and a lower frequency of distant metastasis (p < 0.0001). Survival analysis revealed that patients with LAMN had a significantly better prognosis than did those with non-LAMN disease (p < 0.001). Among the patients with distant metastasis, those with LAMN had a significantly better prognosis than did those with non-LAMN disease (p = 0.0020), but among the patients without distant metastasis, the difference between the 2 groups was not significant (p = 0.26). However, among patients who underwent complete resection, the difference in prognosis between the 2 groups was not significant (p = 0.10). Conclusions: A multicenter retrospective study revealed that the clinicopathological characteristics of LAMN was different from those of non-LAMN.


Cancers ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 2031
Author(s):  
Y.H. Andrew Wu ◽  
Atsushi Oba ◽  
Laurel Beaty ◽  
Kathryn L. Colborn ◽  
Salvador Rodriguez Franco ◽  
...  

Intraductal papillary mucinous neoplasms (IPMN) are common but difficult to manage since accurate tools for diagnosing malignancy are unavailable. This study tests the diagnostic value of the main pancreatic duct (MPD) diameter for detecting IPMN malignancy using a meta-analysis of published data of resected IPMNs. Collected from a comprehensive literature search, the articles included in this analysis must report malignancy cases (high-grade dysplasia (HGD) and invasive carcinoma (IC)) and MPD diameter so that two MPD cut-offs could be created. The sensitivity, specificity, and odds ratios of the two cutoffs for predicting malignancy were calculated. A review of 1493 articles yielded 20 retrospective studies with 3982 resected cases. A cutoff of ≥5 mm is more sensitive than the ≥10 mm cutoff and has pooled sensitivity of 72.20% and 75.60% for classification of HGD and IC, respectively. Both MPD cutoffs of ≥5 mm and ≥10 mm were associated with malignancy (OR = 4.36 (95% CI: 2.82, 6.75) vs. OR = 3.18 (95% CI: 2.25, 4.49), respectively). The odds of HGD and IC for patients with MPD ≥5 mm were 5.66 (95% CI: 3.02, 10.62) and 7.40 (95% CI: 4.95, 11.06), respectively. OR of HGD and IC for MPD ≥10 mm cutoff were 4.36 (95% CI: 3.20, 5.93) and 4.75 (95% CI: 2.39, 9.45), respectively. IPMN with MPD of >5 mm could very likely be malignant. In selected IPMN patients, pancreatectomy should be considered when MPD is >5 mm.


Author(s):  
Anne Puchar ◽  
Raphael Pellevoizin ◽  
Christophe Bontoux ◽  
Thomas Depoilly ◽  
Sofiane BENDIFALLAH ◽  
...  

Appendiceal mucinous neoplasm (AMN) is defined as a distended, mucous-filled appendix. It is a rare condition seen in only 8% of cases of appendiceal tumors. Appendiceal mucocele associated with endometriosis is extremely rare with only 16 cases described in the literature to date. In view of the scarcity of the finding, few data are available on preoperative assessment of AMN in women with endometriosis. The goal of the present study was to describe the imaging features that could be suggestive of AMN in the context of endometriosis, as well as the specific management and outcomes.


2016 ◽  
Vol 46 (2) ◽  
pp. 71-74
Author(s):  
David S. Williams

Intraductal papillary mucinous neoplasms are cystic growths often noted incidentally in the pancreatic ducts. Left untreated, some may progress to invasive cancer.


2017 ◽  
Vol 5 ◽  
pp. 2050313X1774101 ◽  
Author(s):  
Hiroyuki Tokue ◽  
Hideo Morita ◽  
Azusa Tokue ◽  
Yoshito Tsushima

Complications associated with intraductal papillary mucinous neoplasms, such as acute pancreatitis, perforation, and fistula formation, have been documented. Intraductal papillary mucinous neoplasm with intratumoral hemorrhage is rare. To the best of our knowledge, there have been no previous reports of intraductal papillary mucinous neoplasm rupture and bleeding with intra-abdominal hemorrhage. A 74-year-old woman complained of acute upper right abdominal pain. She was under follow-up for an intraductal papillary mucinous neoplasm in the pancreatic head. Contrast-enhanced computed tomography revealed intraductal papillary mucinous neoplasm rupture and bleeding with intra-abdominal hemorrhage. The bleeding was treated with selective endovascular embolization of a branch of the gastroduodenal artery. Follow-up examinations are recommended even for intraductal papillary mucinous neoplasm patients without malignant findings because of the potential risk of rupture and bleeding with intra-abdominal hemorrhage. Clinicians should be aware of this possibility to ensure that patients are appropriately treated.


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