scholarly journals Transformation of Low-Grade Mucinous Neoplasm of the Appendix With Pseudomyxoma Peritonei to High-Grade Sarcomatoid Carcinoma

2015 ◽  
Vol 7 (7) ◽  
pp. 571-574 ◽  
Author(s):  
Yu-Nung Chen ◽  
Jie-Jen Lee ◽  
Shih-Ping Cheng ◽  
Chung-Hsin Tsai
Keyword(s):  
Pseudomyxoma Peritonei ◽  
Sarcomatoid Carcinoma ◽  
Low Grade ◽  
High Grade ◽  
Mucinous Neoplasm

EP.FRI.999 Case report of a Low Grade appendiceal mucinous neoplasm (LAMN) and review of literature

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Kimberly Da Costa ◽  
Sivakumaran Sabanathan
Keyword(s):  
Intraperitoneal Chemotherapy ◽  
Pseudomyxoma Peritonei ◽  
Right Hemicolectomy ◽  
Low Grade ◽  
Mucinous Neoplasm ◽  
Mucinous Neoplasms ◽  
Mucocele Of The Appendix ◽  
Preoperative Ct ◽  
Inflammatory Processes ◽  
Progressive Accumulation

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.

scholarly journals Classification of mucinous appendiceal neoplasm and pseudomyoxma peritonei

2018 ◽  
Vol 8 (1) ◽  
pp. 1301-1307
Author(s):  
Arnab Ghosh
Keyword(s):  
Goblet Cell ◽  
Wide Spectrum ◽  
Low Grade ◽  
Controversial Issues ◽  
High Grade ◽  
Goblet Cell Carcinoid ◽  
Signet Ring Cells ◽  
Mucinous Neoplasm ◽  
Signet Ring

Mucinous appendiceal tumors are uncommon and include a wide spectrum of tumors whose classification remained controversial. Some of these mucin producing appendiceal tumors can disseminate to the peritoneal cavity leading to pseudomyxoma peritonei (PMP). Despite several attempts to classify mucinous tumors of appendix and PMP by different authors in the past, no universally accepted classification system was present. The controversial issues were discussed at the 2012 World Congress of the Peritoneal Surface Oncology Group International (PSOGI) in Berlin. A panel of 71 experts from 13 different countries was formed under the lead co-ordinator Norman J. Carr. A total of 4 rounds of questionnaires and one meeting were held. The opinion of the majority was taken into account. Importance of intactness of muscularis mucosae, pushing invasion and infiltrative invasion were emphasized. The entities Low grade appendiceal mucinous neoplasm (LAMN) and High grade appendiceal mucinous neoplasm (HAMN) were defined.. The terminologies suggested for Goblet cell carcinoid and adenoneuroendocrine carcinoma were goblet cell tumor and adenocarcinoma ex goblet cell carcinoid. Acellular mucin in peritoneum was not classified under PMP which was classified into 3 categories depending upon low grade , high grade cytologic features and presence of signet ring cells. It was suggested to report the extent of mucin and cells separately. A reporting format solely for mucinous appendiceal tumors was formulated by the panel. However, there are some grey areas which may have to be addressed in future.

A Patient With a Low-grade Mucinous Neoplasm Involving the Ovary and Pseudomyxoma Peritonei Originating in an Isolated Intestinal Duplication

2018 ◽  
Vol 37 (4) ◽  
pp. 338-343
Author(s):  
Michiel Simons ◽  
Inge Ebisch ◽  
Joanne de Hullu ◽  
Maaike van Ham ◽  
Marc Snijders ◽  
...  
Keyword(s):  
Pseudomyxoma Peritonei ◽  
Low Grade ◽  
Intestinal Duplication ◽  
Mucinous Neoplasm

scholarly journals Low-Grade Appendiceal Mucinous Neoplasm Involving the Endometrium and Presenting with Mucinous Vaginal Discharge

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Vera Vavinskaya ◽  
Joel M. Baumgartner ◽  
Albert Ko ◽  
Cheryl C. Saenz ◽  
Mark A. Valasek
Keyword(s):  
Pseudomyxoma Peritonei ◽  
Vaginal Discharge ◽  
Low Grade ◽  
Invasive Adenocarcinoma ◽  
Peritoneal Disease ◽  
Mucinous Neoplasm ◽  
Mucinous Neoplasms ◽  
Pathological Evaluation ◽  
Classification Management

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.

scholarly journals Altered linkage pattern of N-glycan sialic acids in pseudomyxoma peritonei

Glycobiology ◽  
2020 ◽  
Author(s):  
Pirjo Nummela ◽  
Annamari Heiskanen ◽  
Soili Kytölä ◽  
Caj Haglund ◽  
Anna Lepistö ◽  
...  
Keyword(s):  
Pseudomyxoma Peritonei ◽  
Peritoneal Dissemination ◽  
Sialic Acids ◽  
Low Grade ◽  
High Grade ◽  
Malignant Diseases ◽  
Linkage Pattern ◽  
Mucinous Neoplasms ◽  
Sialic Acid Binding ◽  
Tissue Specimens

Abstract Pseudomyxoma peritonei (PMP) is a highly mucinous adenocarcinoma growing in the peritoneal cavity and most commonly originating from the appendix. Glycans play an important role in carcinogenesis, and glycosylation is altered in malignant diseases, including PMP. We have previously demonstrated that fucosylation of N-glycans is increased in PMP, but we did not observe modulation of overall sialylation. As sialic acids can be attached to the rest of the glycan via α2,3- or α2,6-linkage, we have now analyzed the linkage patterns of sialic acids in tissue specimens of normal appendices, low-grade appendiceal mucinous neoplasms (LAMN), low-grade (LG) PMP and high-grade (HG) PMP. For the linkage analysis, the enzymatically released acidic N-glycans were first treated with ethyl esterification or α2,3-sialidase digestion followed by MALDI-TOF mass spectrometry. Significant increase in the relative abundance of α2,6-sialylated and decrease in α2,3-sialylated N-glycans was observed in PMP tumors as compared to the normal appendices (P < 0.025). More specifically, increased α2,6-sialylation (P < 0.05) and decreased α2,3-sialylation (P < 0.01) were detected in afucosylated and monofucosylated N-glycans of PMPs, whereas the less abundant multifucosylated glycans, containing terminal fucose, demonstrated increased α2,3-sialylation (P < 0.01). Importantly, the increase in α2,6-sialylation was also detected between PMP and the appendiceal precursor lesion LAMN (P < 0.01). The identified glycosylation alterations produce ligands for sialic acid-binding immunoglobulin-like lectins (Siglecs) and sialofucosylated glycans binding selectins, which play a role in the peritoneal dissemination and progression of the disease.

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