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.

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.

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

2014 ◽  
Vol 45 (8) ◽  
pp. 1737-1743 ◽  
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

Prognostic value of the volume time index (VTI) ratio for patients with appendiceal cancer with peritoneal carcinomatosis treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC).

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e15704-e15704
Author(s):  
Josh Karpes ◽  
Daniel Dotta ◽  
Oliver Fisher ◽  
Raphael Shamavonian ◽  
Nayef Alzahrani ◽  
...  
Keyword(s):  
Overall Survival ◽  
Prognostic Factor ◽  
Peritoneal Dissemination ◽  
Tumour Volume ◽  
Peritoneal Metastases ◽  
Low Grade ◽  
High Grade ◽  
Appendiceal Cancer ◽  
Appendix Cancer ◽  
Significant Difference

e15704 Background: Completeness of cytoreduction score (CC-score) and tumour volume (as expressed by the peritoneal cancer index (PCI)) have been demonstrated as important post-operative prognostic factors in those patients with appendix cancer with peritoneal metastases undergoing CRS/HIPEC. A previous analysis included a pre-operative factor and demonstrated the tumour marker to volume index (CA19-9/PCI) in patients who had low grade appendiceal cancer with peritoneal metastases was an independent prognostic factor for overall survival (OS). This analysis aims to evaluate VTI as a prognostic factor in low- and high-grade appendix neoplasms with peritoneal dissemination managed with CRS/HIPEC. Methods: A retrospective cohort study of all patients diagnosed with appendiceal cancer with peritoneal dissemination managed with CRS/IPC from 1996 to 2017 was performed by analysing the survival effect of the ratio of tumour volume to the time between initial tumour resection and CRS/HIPEC. VTI was stratified into low versus high groups, and tumour grade was divided into low grade: diffuse peritoneal adenomucinosis (DPAM); and high grade: peritoneal mucinous carcinomatosis (PMCA). Results: Two hundred and sixty-four patients were included. For both DPAM and PMCA, there was no statistically significant difference in overall survival between patients with a low versus high VTI. For both low and high VTI in DPAM, the median survival was not reached, p= 0.586. For PMCA, those with a low VTI had an overall survival of 63 months (95%CI 48-NR), versus those with a high VTI 69 months (95%CI 45-NR), p= 0.97. There was no statistically significant difference in the median recurrence free survival (RFS) between low and high VTI for both DPAM and PMCA. Conclusions: The volume to time ratio for appendix cancer with peritoneal dissemination was not an independent prognostic indicator for overall survival in patients undergoing CRS/HIPEC, suggesting that this index is not a valuable pre-operative planning tool.

Splenic Metastases from Mucinous Neoplasms of the Appendix and Colon

2006 ◽  
Vol 92 (2) ◽  
pp. 104-112 ◽  
Author(s):  
Jacobo Cabanas ◽  
Rodrigo Gomes da Silva ◽  
Luis Zappa ◽  
Jesus Esquivel ◽  
Carlos Cerruto ◽  
...  
Keyword(s):  
Peritoneal Dissemination ◽  
Clinical Course ◽  
Clinical Information ◽  
Sigmoid Colon Cancer ◽  
High Grade ◽  
Mucinous Tumor ◽  
Splenic Parenchyma ◽  
Mucinous Neoplasms ◽  
Splenic Tumor ◽  
Splenic Metastases

Aims and Background Splenic metastases associated with mucinous intraabdominal tumors have been an enigma in the radiologic and oncology literature. These focal parenchymal defects from a non-metastasizing malignancy within an organ that rarely develops metastatic foci, even with high-grade cancer, were studied. Methods Information on 9 patients who underwent splenectomy with intraparenchymal splenic masses associated with appendiceal or colorectal mucinous tumors with peritoneal dissemination was collected. The CT scan, the histopathology and the clinical parameters of these patients were studied. A literature review searching for prior reports of this subject was performed. Results Eight of these patients had mucinous appendiceal tumors and 1 a mucinous sigmoid colon cancer. All patients had mucinous carcinomatosis at some time in their clinical course. These splenic tumor masses had a CT image compatible with metastases and not compatible with mucinous tumor layered out of the splenic capsule. None of the patients had evidence of metastases to other sites such as liver or lymph nodes. All patients had a mucinous histopathology. Splenectomy may be associated with prolonged survival. Conclusions From our review of the clinical information available on these 9 patients, these splenic lesions were thought to be an entrapment of mucinous tumor within splenic surface trabeculae, which expand into the splenic parenchyma resembling metastatic disease. These CT findings may be more accurately referred to as splenic pseudometastases.

Relapse Risk after Nonmyeloablative Hematopoietic Cell Transplantation for Malignant Diseases.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 703-703
Author(s):  
Christoph Kahl ◽  
Barry Storer ◽  
Marco Mielcarek ◽  
Mike Maris ◽  
Brenda Sandmaier ◽  
...  
Keyword(s):  
High Risk ◽  
Myeloid Leukemia ◽  
Hematopoietic Cell Transplantation ◽  
Lymphocytic Leukemia ◽  
Low Grade ◽  
High Grade ◽  
Malignant Diseases ◽  
Relapse Risk ◽  
Standard Risk ◽  
Relapse Rates

Abstract Nonmyeloablative hematopoietic cell transplantation (NMHCT) has become an immunotherapeutic option for patients with a variety of malignant diseases. The susceptibility to malignancy-eradicating graft-versus-tumor effects, however, appears to depend on type and stage of the underlying diseases. Thus, the aim of this study was to estimate relapse risk after NMHCT according to pretransplant disease characteristics. Between 1997 and 2004, 595 consecutive patients (median age, 55; range, 5–74 years) received related (n=383) or unrelated (n=212) grafts after preparation with total body irradiation (TBI, 200 cGy, n=168) alone, or in combination with fludarabine (90 mg/m2, n=423). The relapse rate per person year (PY) at risk, which corrected for variation in follow-up time and the competing risk of non-relapse mortality, was calculated for 27 different disease and stage categories. No corrections were made for graft rejections, all of which resulted in relapse. The overall relapse rate per PY was 0.37. As shown in Table 1, patients with chronic lymphocytic leukemia, low-grade Non-Hodgkin lymphoma (NHL) and multiple myeloma had the lowest relapse rates per PY (0.00, 0.09, 0.09; “low-risk”). In contrast, patients with advanced myeloid diseases had a relapse risk per PY of >0.50 (“high-risk”). The increased incidence of graft rejection among patients with chronic myeloid leukemia and myelodysplastic syndrome, however, may have contributed to the higher relapse risk observed in this group. Patients with lymphoproliferative diseases not in remission (Hodgkin disease and high-grade NHL excluded), and myeloid malignancies in remission at transplant had relapse rates per PY of 0.20 – 0.50 (“standard-risk”). For patients deemed at “high risk” for relapse after NMHCT, intensification of the preparative regimens, and/or cytoreductive strategies before or after transplant need to be considered in the design of future transplant protocols. Relapse Risk Categories Low Risk (n) Relapse Rates per PY Standard Risk (n) Relapse Rates per PY High Risk (n) Relapse Rates per PY CLL, chronic lymphocytic leukemia; MM, multiple myeloma; MDS, myelodysplastic syndrome; AML, acute myeloid leukemia; CML, chronic myeloid leukemia; ALL acute lymphocytic leukemia; CMML, chronic myelomonocytic leukemia; CR, complete remission; CP/AP/BP, chronic-/accelerated-/blast-phase. CLL, CR (4) 0.00 Waldenstrom′s (7) 0.23 high-grade NHL, no CR (18) 0.61 MM, CR (23) 0.09 MM, no CR (109) 0.26 Hodgkin, CR (7) 0.67 low-grade NHL, CR (22) 0.09 CLL, no CR (52) 0.28 Hodgkin, no CR (26) 0.83 low-grade NHL, no CR (49) 0.19 Myelofibrosis (5) 0.28 MDS (RAEB/RAEB-t, 16) 0.51 MDS (RA/RARS, 18) 0.29 MDS, previous chemo (17) 0.65 AML > CR1 (31) 0.30 AML, no CR (10) 0.69 High-grade NHL, CR (16) 0.31 AML, antecedent MDS (31) 0.94 CML, CP1 (27) 0.34 CMML (7) 0.74 AML, CR1 (43) 0.42 CML, CP2 (7) 1.05 ALL, CR1 (9) 0.45 CML, AP/BP (11) 1.05 ALL, > CR1 (7) 0.88 ALL, no CR (4) 1.08

Use of FDG-PET Imaging for Patients with Disseminated Cancer of the Appendix

2010 ◽  
Vol 76 (12) ◽  
pp. 1338-1344 ◽  
Author(s):  
Payam Rohani ◽  
Stephen D. Scotti ◽  
Perry Shen ◽  
John H. Stewart ◽  
Gregory B. Russell ◽  
...  
Keyword(s):  
Pet Imaging ◽  
Fdg Pet ◽  
Peritoneal Dissemination ◽  
Low Grade ◽  
High Grade ◽  
Positron Emission ◽  
Pet Ct ◽  
The Mean ◽  
Ct Data ◽  
Abdominal Quadrant

The goal of this study is to evaluate the use of positron emission tomography (PET) in evaluation of patients with peritoneal dissemination of carcinoma of appendiceal origin (PDA). Thirty-three patients with PDA, who had preoperative PET or PET/CT imaging, were analyzed. Using operative, pathology, and PET ± CT data, presence or absence of disease in each abdominal quadrant was noted and the use of 18fluoro-deoxy-glucose (FDG) PET for each quadrant was evaluated. The mean age was 52, and there were 17 males; 58 per cent had low-grade lesions. PET was positive in only 35 per cent of cases overall (30 and 41% sensitivity for low-grade and high-grade, respectively). PET without CT sensitivity for low-grade and high-grade lesions was 21 and 8 per cent, respectively. PET imaging has limited use for patients with PDA. We do not recommend the use of FDG-PET for patients with PDA from cancer of the appendix.

scholarly journals A Functional Spatial Analysis Platform for Discovery of Immunological Interactions Predictive of Low-Grade to High-Grade Transition of Pancreatic Intraductal Papillary Mucinous Neoplasms

2018 ◽  
Vol 17 ◽  
pp. 117693511878288 ◽  
Author(s):  
Souptik Barua ◽  
Luisa Solis ◽  
Edwin Roger Parra ◽  
Naohiro Uraoka ◽  
Mei Jiang ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Subsequent Development ◽  
Ductal Adenocarcinoma ◽  
Low Grade ◽  
High Grade ◽  
Cellular Interactions ◽  
Spatial Interactions ◽  
Intraductal Papillary Mucinous Neoplasms ◽  
Remnant Pancreas ◽  
Mucinous Neoplasms

Intraductal papillary mucinous neoplasms (IPMNs), critical precursors of the devastating tumor pancreatic ductal adenocarcinoma (PDAC), are poorly understood in the pancreatic cancer community. Researchers have shown that IPMN patients with high-grade dysplasia have a greater risk of subsequent development of PDAC in the remnant pancreas than do patients with low-grade dysplasia. In this study, we built a computational prediction model that encapsulates the spatial cellular interactions in IPMNs that play key roles in the transformation of low-grade IPMN cysts to high-grade cysts en route to PDAC. Using multiplex immunofluorescent images of IPMN cysts, we adopted algorithms from spatial statistics and functional data analysis to create metrics that summarize the spatial interactions in IPMNs. We showed that an ensemble of models learned using these spatial metrics can robustly predict, with high accuracy, (1) the dysplasia grade (low vs high grade) and (2) the risk of a low-grade cyst progressing to a high-grade cyst. We obtained high classification accuracies on both tasks, with areas under the curve of 0.81 (95% confidence interval: 0.71-0.9) for task 1 and 0.81 (95% confidence interval: 0.7-0.94) for task 2. To the best of our knowledge, this is the first application of an ensemble machine learning approach for discovering critical cellular spatial interactions in IPMNs using imaging data. We envision that our work can be used as a risk assessment tool for patients diagnosed with IPMNs and facilitate greater understanding and investigation of the cellular interactions that cause transition of IPMNs to PDAC.

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.

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