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BJS Open Jul 2021Mucinous appendiceal neoplasms with peritoneal dissemination (PD) show a wide spectrum of clinical behaviour. Histological grade has been correlated with prognosis, but...
BACKGROUND
Mucinous appendiceal neoplasms with peritoneal dissemination (PD) show a wide spectrum of clinical behaviour. Histological grade has been correlated with prognosis, but no universally accepted histological grading has been established. The aim of this systematic review was to provide historical insight to understand current grading classifications, basic histopathological features of each category, and to define which classification correlates best with prognosis.
METHODS
MEDLINE and the Cochrane Library were searched for studies that reported survival across different pathological grades in patients with mucinous neoplasm of the appendix with PD treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. PRISMA guidelines were followed.
RESULTS
Thirty-eight studies were included. Ronnett's classification was the most common (9 studies). Classifications proposed by the Peritoneal Surface Oncology Group International (PSOGI) (6 studies) and the seventh or eighth edition of the AJCC (7 studies) are gaining in popularity. Nine studies supported a two-tier, 12 a three-tier, and two a four-tier classification system. Three studies demonstrated that acellular mucin had a better prognosis than low-grade pseudomyxoma peritonei in the PSOGI classification or M1bG1 in the eighth edition of the AJCC classification. Four studies demonstrated that the presence of signet ring cells was associated with a worse outcome than high-grade pseudomyxoma peritonei in the PSOGI classification and M1bG2 in the eighth edition of the AJCC.
CONCLUSION
There is a great need for a common language in describing mucinous neoplasms of the appendix with PD. Evolution in terminology as a result of pathological insight turns the four-tiered PSOGI classification system into a coherent classification option.
Topics: Appendiceal Neoplasms; Appendix; Cytoreduction Surgical Procedures; Humans; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 34355239
DOI: 10.1093/bjsopen/zrab059 -
Journal of Cancer Research and Clinical... Jun 2022Abscess or fistula of the anal region is an uncommon presentation of malignancy. Under the assumption of a benign condition, diagnostics is often delayed, resulting in...
Non-mucinous adenocarcinomas and squamous cell carcinomas of the anal region masquerading as abscess or fistula: a retrospective analysis and systematic review of literature.
PURPOSE
Abscess or fistula of the anal region is an uncommon presentation of malignancy. Under the assumption of a benign condition, diagnostics is often delayed, resulting in advanced tumour stages at first diagnosis. Due to the case rarity, treatment guidelines for cancers of anorectal region masquerading as abscess or fistula are missing.
METHODS
We analysed all patients presenting with an abscess or fistula of the anal region in our department between January 2004 and August 2020. The malignancies were included to our study to acquire data on clinical presentation, treatment and outcome. Furthermore, a systematic review to present adenocarcinomas and squamous cell carcinomas associated to an abscess or fistula was performed.
RESULTS
0.5% of the patients treated for an abscess or fistula of the anal region met the selection criteria. Mean time from the onset of symptoms to diagnosis of malignancy was 100 days. Histology revealed adenocarcinoma and squamous cell carcinoma each in two patients. All patients had locally advanced tumours without distant metastases, in two cases with regional lymph-node metastases. Neoadjuvant chemoradiation was applied in two patients. All patients underwent abdomino-perineal resection of the rectum. The overall outcome reveals a recurrence-free survival of 4.5 and 3 years for two patients. Further two patients died within 5 months after the primary resection.
CONCLUSION
Advanced carcinomas of the anorectal region may masquerade as abscess or fistula, cause diagnostic problems and delay oncologic treatment. However, even in these very advanced situations, surgical therapy with curative intent should be attempted.
Topics: Abscess; Adenocarcinoma; Carcinoma, Squamous Cell; Fistula; Humans; Rectum; Retrospective Studies
PubMed: 34338860
DOI: 10.1007/s00432-021-03747-8 -
Cancers Jun 2021Tumor mutational burden (TMB) is a numeric index that expresses the number of mutations per megabase (muts/Mb) harbored by tumor cells in a neoplasm. TMB can be... (Review)
Review
Tumor mutational burden (TMB) is a numeric index that expresses the number of mutations per megabase (muts/Mb) harbored by tumor cells in a neoplasm. TMB can be determined using different approaches based on next-generation sequencing. In the case of high values, it indicates a potential response to immunotherapy. In this systematic review, we assessed the potential predictive role of high-TMB in pancreatic ductal adenocarcinoma (PDAC), as well as the histo-molecular features of high-TMB PDAC. High-TMB appeared as a rare but not-negligible molecular feature in PDAC, being present in about 1.1% of cases. This genetic condition was closely associated with mucinous/colloid and medullary histology ( < 0.01). PDAC with high-TMB frequently harbored other actionable alterations, with microsatellite instability/defective mismatch repair as the most common. Immunotherapy has shown promising results in high-TMB PDAC, but the sample size of high-TMB PDAC treated so far is quite small. This study highlights interesting peculiarities of PDAC harboring high-TMB and may represent a reliable starting point for the assessment of TMB in the clinical management of patients affected by pancreatic cancer.
PubMed: 34206554
DOI: 10.3390/cancers13133119 -
United European Gastroenterology Journal Oct 2020Individuals with a very high lifetime risk of developing pancreatic ductal adenocarcinoma; for example, hereditary pancreatitis and main-duct or mixed-type intraductal...
Prophylactic total pancreatectomy in individuals at high risk of pancreatic ductal adenocarcinoma (PROPAN): systematic review and shared decision-making programme using decision tables.
BACKGROUND
Individuals with a very high lifetime risk of developing pancreatic ductal adenocarcinoma; for example, hereditary pancreatitis and main-duct or mixed-type intraductal papillary mucinous neoplasm, may wish to discuss prophylactic total pancreatectomy but strategies to do so are lacking.
OBJECTIVE
To develop a shared decision-making programme for prophylactic total pancreatectomy using decision tables.
METHODS
Focus group meetings with patients were used to identify relevant questions. Systematic reviews were performed to answer these questions.
RESULTS
The first tables included hereditary pancreatitis and main-duct or mixed-type intraductal papillary mucinous neoplasm. No studies focused on prophylactic total pancreatectomy in these groups. In 52 studies (3570 patients), major morbidity after total pancreatectomy was 25% and 30-day mortality was 6%. After minimally invasive total pancreatectomy (seven studies, 35 patients) this was, respectively, 13% and 0%. Exocrine insufficiency-related symptoms occurred in 33%. Quality of life after total pancreatectomy was slightly lower compared with the general population.
CONCLUSION
The decision tables can be helpful for discussing prophylactic total pancreatectomy with individuals at high risk of pancreatic ductal adenocarcinoma.
Topics: Carcinoma, Pancreatic Ductal; Decision Making, Shared; Decision Support Techniques; Disease Progression; Exocrine Pancreatic Insufficiency; Humans; Pancreatectomy; Pancreatic Ducts; Pancreatic Neoplasms; Pancreatitis, Chronic; Postoperative Complications; Prophylactic Surgical Procedures; Quality of Life; Risk Assessment; Treatment Outcome
PubMed: 32703081
DOI: 10.1177/2050640620945534 -
Gut Jan 2021Recently, tumours with microsatellite instability (MSI)/defective DNA mismatch repair (dMMR) have gained considerable interest due to the success of immunotherapy in... (Comparative Study)
Comparative Study
OBJECTIVE
Recently, tumours with microsatellite instability (MSI)/defective DNA mismatch repair (dMMR) have gained considerable interest due to the success of immunotherapy in this molecular setting. Here, we aim to clarify clinical-pathological and/or molecular features of this tumour subgroup through a systematic review coupled with a comparative analysis with existing databases, also providing indications for a correct approach to the clinical identification of MSI/dMMR pancreatic ductal adenocarcinoma (PDAC).
DESIGN
PubMed, SCOPUS and Embase were searched for studies reporting data on MSI/dMMR in PDAC up to 30 November 2019. Histological and molecular data of MSI/dMMR PDAC were compared with non-MSI/dMMR PDAC and with PDAC reference cohorts (including SEER database and The Cancer Genome Atlas Research Network - TCGA project).
RESULTS
Overall, 34 studies with 8323 patients with PDAC were included in the systematic review. MSI/dMMR demonstrated a very low prevalence in PDAC (around 1%-2%). Compared with conventional PDAC, MSI/dMMR PDAC resulted strongly associated with medullary and mucinous/colloid histology (p<0.01) and with a / wild-type molecular background (p<0.01), with more common genes mutations. Data on survival are still unclear.
CONCLUSION
PDAC showing typical medullary or mucinous/colloid histology should be routinely examined for MSI/dMMR status using specific tests (immunohistochemistry, followed by MSI-PCR in cases with doubtful results). Next-generation sequencing (NGS) should be adopted either where there is limited tissue or as part of NGS tumour profiling in the context of precision oncology, acknowledging that conventional histology of PDAC may rarely harbour MSI/dMMR.
Topics: Carcinoma, Pancreatic Ductal; Databases, Factual; Humans; Microsatellite Instability; Pancreatic Neoplasms
PubMed: 32350089
DOI: 10.1136/gutjnl-2020-320726 -
Revista Espanola de Enfermedades... Feb 2020Perforation in colorectal cancer is an uncommon condition, and neoplastic invasion of the abdominal wall with local infection is even rarer. Our objective is to present...
Perforation in colorectal cancer is an uncommon condition, and neoplastic invasion of the abdominal wall with local infection is even rarer. Our objective is to present the case of an 84-year-old male with right colon cancer that manifested as an inguinal abscess, and also to perform a systematic review of the literature in PubMed, EMBASE, and Web of Science. A total of 59 cases in retrospective studies were found. Median age was 64 years, thirty cases were men and twenty-nine were women (51% and 49%, respectively). The most common location was the right colon with 27 cases (46%), followed by the left colon with 18 cases (31%), 12 cases in the transverse colon (20%), and 2 cases with colonic synchronous neoplasm (3%). Surgery was performed in two or more occasions for 33 cases (60%), and on one occasion for 21 cases (38%); medical treatment alone was administered in one case (2%). The most common histological type was adenocarcinoma (64%), followed by its mucinous variant (22%). There was recurrence in 33% of cases. Mortality at follow-up was 47%. As a limitation of our study, follow-up was heterogeneous, making it impossible to interpret long-term results regarding the influence of treatment on patient survival, also difficulted by the urgent nature of the condition and its exceptional incidence. Further studies are needed with prospective data collection on the management of colorectal cancer in the emergency setting, standardizing follow-up in order to facilitate an adequate analysis of the prognosis of the disease.
Topics: Abscess; Aged, 80 and over; Colonic Neoplasms; Female; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Prospective Studies; Retrospective Studies
PubMed: 31985253
DOI: 10.17235/reed.2020.5887/2018 -
Annals of Translational Medicine Nov 2019Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a cystic tumor with a disease spectrum ranging from low-grade dysplasia to invasive carcinoma. The... (Review)
Review
Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a cystic tumor with a disease spectrum ranging from low-grade dysplasia to invasive carcinoma. The evidence for adjuvant treatment in invasive IPMN is limited and mostly derived from studies in conventional pancreatic ductal adenocarcinoma (PDAC). We performed a systematic review focusing on all clinical studies concerning the efficacy of adjuvant therapy in patients with invasive IPMN. We identified 8 retrospective cohort studies, using either adjuvant chemotherapy alone (n=1), adjuvant radiotherapy alone (n=1) or adjuvant chemotherapy in combination with radiation (n=6). Adjuvant therapy was associated with a survival benefit in 7 out of the 8 studies. Specific survival benefit was noted for patients with node-positive disease, higher TNM stage, positive resection margins, poor differentiation and tubular subtype. We conclude that adjuvant therapy may be beneficial in invasive IPMN, but current data suggest that it should be given selectively based on individual tumor characteristics. Further prospective, randomized studies are warranted.
PubMed: 31930090
DOI: 10.21037/atm.2019.10.37 -
Romanian Journal of Morphology and... 2019Primary ovarian hydatid disease (HD) is a rare entity, produced by the larval stage of Echinococcus granulosus. HD commonly involves liver, lung, abdomen cavity, spleen...
Primary ovarian hydatid disease (HD) is a rare entity, produced by the larval stage of Echinococcus granulosus. HD commonly involves liver, lung, abdomen cavity, spleen and is unusually identified in pelvic organs. Based on our knowledge, the paper reviews 27 literature reports of ovarian HD, diagnosed during the last 20 years, providing a valuable database. Patients' ages ranged between 12-76 years, the gross appearance was that of 40-330 mm diameter hydatid cysts (HCs), 66.66% of them being primary. According to these reports, ovarian HD has non-specific clinical manifestations, such as abdominal or pelvic pain, nausea, dysmenorrhea or amenorrhea. The diagnosis may be achieved by abdominal ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI), serological exams, such as eosinophilia (in 10-30% of patients) or indirect hemagglutination and immunoglobulin (IgG) antibodies detection. Ovarian HC microscopic pattern is characterized by three layers: pericyst or adventitia (host origin), germinal layer (endocyst), and laminated membrane (ectocyst). The immunoreaction triggered by parasites is initially rich in macrophages and neutrophils, followed by eosinophils and lymphocytes, with numerous cluster of differentiation 8 (CD8)-positive T-cells in active lesions and progressive forms. Concomitant ovarian diseases are relatively rare, being represented by borderline tumors (n=2 cases), mucinous cystadenoma (n=1 case), hemorrhagic cyst (n=1 case), and serous adenocarcinoma (n=1 case). In conclusion, the ovarian location of HD should be considered in any differential diagnosis of a cystic lesion, while it does not exclude synchronous ovarian tumors. These cases reinforce the necessity of better measures of prophylaxis and screening of HD in endemic areas.
Topics: Adolescent; Adult; Aged; Child; Echinococcosis; Female; Humans; Immunohistochemistry; Middle Aged; Ovarian Cysts; Young Adult
PubMed: 31912083
DOI: No ID Found -
International Journal of Surgery... Nov 2019Previous studies have indicated that there may be a difference in tumor biology between intraductal papillary mucinous carcinoma (IPMC) and pancreatic ductal... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Previous studies have indicated that there may be a difference in tumor biology between intraductal papillary mucinous carcinoma (IPMC) and pancreatic ductal adenocarcinoma (PDAC). However, the data are still controversial. The aim of this systematic review and meta-analysis was to summarize and compare the outcome of IPMC and PDAC after surgical resection.
METHODS
Studies comparing IPMC and PDAC were identified using Medline and Embase search engines. Primary outcomes of interest were survival and recurrence. Secondary outcomes were clinicopathological characteristics. Meta-analysis of data was conducted using a random-effects model.
RESULTS
A total of 14 studies were included. Pooled analysis revealed an improved 5-year overall survival (OS) for IPMC compared to PDAC (OR 0.23, 95% CI 0.09-0.56). Both colloid and tubular IPMC showed improved 5-year OS compared to PDAC (OR 0.12, 95% CI 0.05-0.25 and OR 0.38, 95% CI 0.26-0.54, respectively). Median survival time ranged from 21 to 58 months in the IPMC group compared to 12-23 months in the PDAC group. No meta-analysis could be performed on recurrence or on time-to-event data. Descriptive data showed no survival difference for higher TNM stages. IPMC was more often found at a TNM-stage of 1 (OR 4.40, 95% CI 2.71-7.15) and had lower rates of lymph node spread (OR 0.43, 95% CI 0.32-0.57).
CONCLUSION
Available data suggest that IPMC has a more indolent course with a better 5-year OS compared to PDAC. The histopathological features are less aggressive in IPMC. The reason may be earlier detection. However, for IPMC with higher TNM stages the survival seems to be similar to that of PDAC.
Topics: Adenocarcinoma, Mucinous; Adenocarcinoma, Papillary; Aged; Breast Neoplasms; Carcinoma, Ductal, Breast; Carcinoma, Pancreatic Ductal; Female; Humans; Middle Aged; Neoplasm Staging; Pancreatic Neoplasms; Survival Rate
PubMed: 31546033
DOI: 10.1016/j.ijsu.2019.09.014 -
International Journal of Gynecological... Jul 2017Immunohistochemistry is widely used to support a pathology diagnosis of cervical adenocarcinoma despite the absence of a systematic review and meta-analysis of the... (Meta-Analysis)
Meta-Analysis Review
Tissue-based Immunohistochemical Biomarker Accuracy in the Diagnosis of Malignant Glandular Lesions of the Uterine Cervix: A Systematic Review of the Literature and Meta-Analysis.
Immunohistochemistry is widely used to support a pathology diagnosis of cervical adenocarcinoma despite the absence of a systematic review and meta-analysis of the published data. This systematic review and meta-analysis was performed to investigate the sensitivity and specificity of immunohistochemistry biomarkers in the tissue-based diagnosis of cervical adenocarcinoma histotypes compared with normal endocervix and benign glandular lesions. The systematic review and meta-analysis used a PICOT framework and QUADAS-2 to evaluate the quality of included studies. The literature search spanned 40 years and ended June 30, 2015. Abstracts of identified records were independently screened by 2 of the authors who then conducted a full-text review of selected articles. Sensitivity and specificity of immunohistochemistry expression in malignant glandular lesions of the cervix classified per WHO 2003 compared with 5 benign comparators (normal/benign endocervix, and benign endocervical, endometrioid, gastric, and mesonephric lesions) were calculated. Of 902 abstracts screened, 154 articles were selected for full review. Twenty-five articles with results for 36 biomarkers were included. The only biomarker with enough studies for a meta-analysis was p16 and the definition of positive p16 staining among them was variable. Nevertheless, any positive p16 expression was sensitive, ranging from 0.94 to 0.98 with narrow confidence intervals (CIs), for adenocarcinoma in situ (AIS) and mucinous adenocarcinomas in comparison with normal/benign endocervix and benign endocervical and endometrioid lesions. Specificity for AIS and mucinous adenocarcinomas was also high with narrow CIs compared with benign endocervical lesions. The specificity was high for AIS, 0.99 (0.24, 1.0), and mucinous adenocarcinoma, 0.95 (0.52, 1.0), compared with normal/benign endocervix but with wider CIs, and low with very wide CIs compared with benign endometrioid lesions: 0.31 (0.00, 0.99) and 0.34 (0.00, 0.99), respectively. Results from single studies showed that p16, p16/Ki67 dual stain, ProExC, CEA, ESA, HIK1083, Claudin 18, and ER loss in perilesional stromal cells were useful with high (≥0.75) sensitivity and specificity estimates in ≥1 malignant versus benign comparisons. None of the biomarkers had highly useful sensitivity and specificity estimates for AIS, mucinous adenocarcinomas, or minimal deviation adenocarcinoma/gastric adenocarcinoma compared with benign gastric or mesonephric lesions or for mesonephric carcinoma compared with normal/benign endocervix, benign endocervical, endometrial, or mesonephric lesions. Any expression of p16 supports a diagnosis of AIS and mucinous adenocarcinomas in comparison with normal/benign endocervix and benign endocervical lesions. The majority of studies did not separate mosaic/focal p16 staining from diffuse staining as a distinct pattern of p16 overexpression and this may have contributed to the poor performance of p16 in distinguishing AIS and mucinous adenocarcinomas from benign endometrioid lesions. Single studies support further investigation of 8 additional biomarkers that have highly useful sensitivity and specificity estimates for ≥1 malignant glandular lesions compared with ≥1 of the 5 benign comparators.
Topics: Adenocarcinoma; Adenocarcinoma in Situ; Adenocarcinoma, Mucinous; Biomarkers, Tumor; Cervix Uteri; Cyclin-Dependent Kinase Inhibitor p16; Female; Humans; Immunohistochemistry; Ki-67 Antigen; Sensitivity and Specificity; Uterine Cervical Neoplasms
PubMed: 27801764
DOI: 10.1097/PGP.0000000000000345