Histological Subtype Research Articles

Maintenance treatment with trofosfamide in patients with advanced soft tissue sarcoma – a retrospective single-centre analysis

Background: The prognosis of patients with advanced soft tissue sarcoma (STS) remains dismal. Trofosfamide (TRO) has been proposed as a well-tolerated oral maintenance therapy. This retrospective analysis aims to determine the value of this therapy. Methods: Fifty-nine patients with advanced STS who received TRO maintenance therapy between 2016 and 2022 were reviewed and analysed regarding clinical parameters and outcomes. Results: The median age was 48 years; the most common histological subtype was synovial sarcoma (n = 22, 37%), and 71% of patients (n = 42) presented with metastatic disease. No radiological evidence of disease (NED) before the start of maintenance was reported in 36% of patients (n = 21). The median follow-up was 38.2 months with a median maintenance duration of 9.0 months. The median event-free survival (EFS) and overall survival (OS) were 9.5 and 33.2 months, respectively. In metastatic patients achieving NED before the initiation of TRO, the median EFS was 29.4 months, while the median OS was not reached. In metastatic patients with anthracycline + ifosfamide (AI) as first-line induction therapy without prior metastasis-directed local therapy, the median EFS and OS from the start of AI were 13.9 and 26.8 months, respectively. Multivariate analysis of the overall cohort demonstrated that NED before the start of maintenance was significantly associated with a prolonged EFS (p = 0.024, hazard ratio [HR] = 0.26), and G2 histology correlated with longer OS (p = 0.030, HR = 0.16, reference: G3). Interpretation: Oral maintenance therapy with TRO appears to improve outcomes in patients with advanced STS. Metastatic patients who achieve NED through prior metastasectomy may particularly benefit from TRO maintenance.

Spatial distribution and subtype-specific expression patterns of Nectin-4 in muscle-invasive bladder cancer.

To investigate the expression patterns of Nectin-4, the target molecule of the antibody-drug conjugate enfortumab vedotin (EV), in relation to histological and molecular subtypes of urothelial bladder cancer (UBC). We assessed the protein expression patterns of Nectin-4 in a spatially organised tissue microarray containing 1386 tissue cores from 314 consecutive patients with UBC who underwent radical cystectomy (2005-2018). Results were correlated with clinicopathological and follow-up data, as well as with different spatial locations (tumour central vs tumour-normal interface and primary tumour vs lymph node [LN] metastases). Additionally, we correlated Nectin-4 expression levels with histological and molecular subtypes. Finally, we assessed the value of Nectin-4 expression for predicting the efficacy of platinum therapy in the peri-operative setting. Nectin-4 expression was observed in 63% of primary tumours and 87% of LN metastases, with significantly higher levels in LNs. Of the histological subtypes, the micropapillary (58%) and pure urothelial histologies (30%) were associated with the highest Nectin-4 positivity, while the sarcomatoid (17%), squamous (15%) and small/cell-neuroendocrine (0%) subtypes exhibited the lowest. Nectin-4 immunopositivity rates were significantly higher in luminal (urothelial-like [42%] and genomically unstable [34%] Lund subtypes) compared to the basal (5%) or mesenchymal (0%) molecular subtypes. Higher Nectin-4 expression levels were associated with lower tumour stage but showed no association with overall survival. Finally, patients with low Nectin-4 expression tended to derive more benefit from platinum-based chemotherapy in both adjuvant and neoadjuvant settings (P < 0.001, P = 0.067). Our results revealed a low spatial heterogeneity of Nectin-4 expression within the primary tumour. In contrast, differential Nectin-4 expression was found in the context of histological and molecular subtypes. Nectin-4-expressing tumours may show varying sensitivity to both EV and platinum-based chemotherapy.

TRIM36 Inhibits the Development of AOM/DSS-Induced Colitis-Associated Colorectal Cancer by Promoting the Ubiquitination and Degradation of GRB7.

Colorectal cancer (CRC) is among the most common cancer types for both sexes. Tripartite motif 36 (TRIM36) has been reported to be aberrantly expressed in several cancer types, suggesting its involvement in cancer progression. However, the role of TRIM36 in the colorectal carcinogenesis remain unknown. In our in vivo experiments, we investigated the role of TRIM36 in AOM/DSS-induced colitis-associated carcinogenesis using TRIM36-knockout (TRIM36 KO) mice. Subsequently, we overexpressed and knocked down TRIM36 expression in two CRC cell lines to further confirm the role of TRIM36 in vitro. The UALCAN database revealed a significant decrease in TRIM36 levels in CRC tissues, including colon adenocarcinoma and rectum adenocarcinoma. A significant correlation was observed between TRIM36 levels and the histological subtype, individual cancer stage, and nodal metastasis status. The downregulation of TRIM36 in CRC tissues was further confirmed using our own collected clinical specimens. Low expression of TRIM36 was found to be associated with unfavorable overall survival and recurrence-free survival in CRC. TRIM36 KO promoted inflammation, inhibited autophagy, and facilitated the development of AOM/DSS-induced CRC. TRIM36 overexpression inhibited proliferation, migration, and invasion, while activated autophagy in CRC cells. TRIM36 directly bound to and regulated the ubiquitination of GRB7 protein. The tumor-suppressive role of TRIM36 in CRC cells was mediated by GRB7. The TRIM36/GRB7 axis may represent a promising therapeutic target for the treatment of CRC.

Comparative evaluation of PD-L1 expression and tumor immune microenvironment in molecular subtypes of muscle-invasive bladder cancer and its correlation with survival outcomes.

Immune checkpoint inhibitors have revolutionized treatment of platinum-refractory advanced bladder cancer, offering hope where options are limited. Response varies, however, influenced by factors such as the tumor's immune microenvironment and prior therapy. Muscle-invasive bladder cancer (MIBC) is stratified into molecular subtypes, with distinct clinicopathologic features affecting prognosis and treatment. This study assessed the expression of programmed cell death 1 ligand 1 (PD-L1) and other immune markers in MIBC, categorized by molecular phenotype. Using GATA3 and CK5/6 immunohistochemistry, 90 neoadjuvant chemotherapy-naive MIBC cases were classified into luminal andnon-luminalsubtypes. The immune microenvironment was characterized through immunostaining for PD-L1, CD4, and CD8. We applied PD-L1 positivity thresholds of 1% or greater for tumor cells and 5% or greater for immune cells. Tumors were examined for PD-L1 expression, histologic subtypes, and immune cell infiltration. Varied expression of PD-L1 and T-cell subtype densities were observed among MIBC subtypes. The double-negative subtype displayed the highest PD-L1 immune cell expression and stromal CD4 and CD8 T-cell densities, indicating an active immune profile. The basal subtype exhibited the highest PD-L1 positivity in tumor cells. In contrast, the luminal type showed the lowest PD-L1 tumor and immune cell expression, with high intratumoral CD4 T-cell density. Although PD-L1 expression in tumor or immune cells did not independently affect survival, patients with basal and double-negative tumors had poorer overall survival. This study highlighted the immune diversity of MIBC in the context of molecular subtypes. Distinct molecular and immune profiles could guide the development of predictive signatures for enhanced immunotherapy response in advanced bladder cancer.

Utility of Polygenic Risk Scores (PRSs) in Predicting Pancreatic Cancer: A Systematic Review and Meta-Analysis of Common-Variant and Mixed Scores with Insights into Rare Variant Analysis

Pancreatic adenocarcinoma is the most common histological subtype of pancreatic cancer, representing approximately 85% of all cases [...]

PET radiomics for histologic subtype classification of non-small cell lung cancer: a systematic review and meta-analysis.

To systematically review the literature and perform a meta-analysis of PET radiomics for histologic subtype classification in non-small cell lung cancer (NSCLC). PubMed, Embase, Scopus, and Web of Science databases were systematically searched in English on human subjects for studies on distinguishing adenocarcinoma (ADC) from squamous cell carcinoma (SCC) using PET radiomics published from inception until November 2024. The Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool and the Radiomics Quality Score (RQS) were utilized to assess the methodological quality of the included studies. The area under the receiver operating characteristic curves (AUC) was pooled to estimate predictive performance. An overall effect size was estimated using a random-effects model. Statistical heterogeneity was evaluated by the I2 value. Subgroup analyses were conducted to explore sources of heterogeneity. Twelve studies were included in the analysis, yielding a pooled AUC of 0.92 (95% confidence interval [CI]: 0.89-0.94). Despite this promising result, the studies showed limitations in both study design and methodological quality, as evidenced by a median RQS of 11/36. A significant degree of heterogeneity was observed among the studies, with an I2 of 92.20% (95% CI: 89.01-95.39) for sensitivity and 89.29% (95% CI: 84.48-94.10) for specificity. This meta-analysis highlights the potential utility of PET radiomics in distinguishing ADC from SCC. However, the observed high heterogeneity indicates substantial methodological variability across the included studies. Future research should focus on standardization, transparency, and multicenter collaborations to improve the reliability and clinical applicability of PET radiomics for histologic subtype classification in NSCLC.

Analysis of Pleiotropic Effects of Nivolumab in Patients with relapsed Pleural Mesothelioma: A Single center retrospective study.

In August 2018, the Japanese PMDA approved nivolumab, an immune checkpoint inhibitor (ICI), for previously treated, unresectable, advanced, or recurrent pleural mesothelioma (PM) based on the MERIT trial, a phase II study of 34 cases. However, concerns regarding limited evidence persist. We retrospectively analyzed 83 patients with previously treated, unresectable, advanced, or recurrent malignant pleural mesothelioma (MPM) treated with nivolumab from August 2018 to May 2022. Efficacy was evaluated using overall response rate (ORR), progression-free survival (PFS), and overall survival (OS) per modified RECIST criteria. Safety was assessed by treatment-related adverse events (TRAEs) according to CTCAE v5.0. PD-L1 expression was analyzed with the anti-PD-1 antibody (22C3). The median age was 73 years. Histological subtypes included epithelioid (60), sarcomatoid (15), biphasic (6), and unknown (2). Lines of treatment were 2nd (62), 3rd (13), and 4th or later (8). Partial response (PR) was seen in 16 patients, stable disease (SD) in 30, progressive disease (PD) in 29, and not evaluable (NE) in 8, with an ORR of 19.3% and a disease control rate of 55.4%. Median PFS and OS were 5.1 and 12.4 months, respectively. TRAEs occurred in 45 patients (54.2%), with grade ≥3 in 6 (7.2%) and one treatment-related death. PFS correlated with male gender, TRAEs, and good performance status (PS: 0-1), while OS correlated with PS. Nivolumab demonstrated efficacy and safety in clinical practice, supporting its use in patients with good PS, even in later lines.

Comprehensive Overview of Molecular, Imaging, and Therapeutic Challenges in Rectal Mucinous Adenocarcinoma

Rectal cancer is one of the most frequent malignancies worldwide. The most common histological type is adenocarcinoma, followed by mucinous adenocarcinoma. The outcome is less favorable for the mucinous type, yet the treatment course is the same. The aim of this systematic literature review is to assess existing information in order to improve survival in rectal mucinous adenocarcinoma (RMA) and establish a starting point for future research. A systematic search of PubMed, Google Scholar, and Web of Science online libraries was performed in October 2024, evaluating studies regarding clinicopathological and genetic features in connection with targeted treatment and survival outcomes in RMA, using the terms “rectal cancer”, “rectum”, “mucinous adenocarcinoma”, or a combination of the terms. We selected 23 studies, 10 of them regarding the diagnostic implications and 13 discussing the treatment strategies and prognosis of this histological subtype. There were six studies addressing the imaging aspects, highlighting the distinct features of mucinous histology in MRI. The molecular specifics were detailed in four studies, outlining the molecular footprint. The prognosis and treatment course were addressed in 12 studies. The inflammation index prognosis, complete response to neoadjuvant chemotherapy, and surgical aspects were addressed individually in each study. We encapsulated the molecular and clinicopathological characteristics of RMA, as well as diagnostic and treatment approaches, to establish a baseline of references for the benefit of daily practice and further research.

Epidemiology and survival analysis according to the histologic subtype of pancreatic cancer: a population-based cohort study

Epidemiology and survival analysis according to the histologic subtype of pancreatic cancer: a population-based cohort study

Prognostic factors in patients with localized and metastatic alveolar rhabdomyosarcoma. A report from two studies and two registries of the Cooperative Weichteilsarkom Studiengruppe CWS.

The histologic classification of rhabdomyosarcoma (RMS) as alveolar (aRMS) or embryonal (eRMS) is of prognostic importance, with the aRMS being associated with a worse outcome. Specific gene fusions (PAX3/7::FOXO1) found in the majority of aRMS have been recognized as markers associated with poor prognosis and are included in current risk stratification instead of histologic subtypes in localized disease. In metastatic disease, the independent prognostic significance of fusion status has not been definitively established. The objective of this analysis was to evaluate survival outcomes of patients with localized and metastatic aRMS and its association with fusion status and subtype (PAX3/7::FOXO1, FOXO1 break), and clinical prognostic factors. A total of 470 patients with aRMS ≤21 years of age enrolled in two CWS-trials and two registries was eligible for the analysis. The 5-year event-free survival (EFS) and overall survival (OS) rates for all patients with localized vs. metastatic tumors were: 56% and 65% vs. 18% and 22%, respectively. Of the 368 (78%) tumors tested, specific fusion was found in 330 (90%), considered "fusion positive" FP (PAX3::FOXO1 in 280, PAX7::FOXO1 in 49, FOXO1 break in 59 tumors). In patients with localized tumors, univariate analysis revealed that clinical group, tumor invasiveness (T1 vs.T2), regional lymph node involvement (N0 vs. N1) and FOXO1 fusion were significantly associated with EFS and OS, tumor size and PAX variant with OS only. In patients with metastatic aRMS, age, bone/marrow (B/BM) metastases, FOXO1 fusion and PAX variant were associated with EFS and OS, T status with OS only. Multivariate analysis identified PAX3::FOXO1 fusion as an independent adverse prognostic factor for EFS in patients with localized disease and for EFS and OS in patients with metastatic disease, B/BM metastases for EFS. PAX3::FOXO1 fusion should replace FOXO1 fusion as an adverse prognostic factor in risk stratification. The prognostic relevance of PAX7::FOXO1-positive and FOXO1 fusion negative aRMS, along with the clinical factors described in this report, allows further refinement of risk assessment of patients with localized and metastatic aRMS.

Galectin-3 marker expression in renal cell carcinoma and correlation with patient's clinicopathologic factors: A cross-sectional study.

Renal Cell Carcinoma is one of the most common malignancies worldwide. To date, multiple attempts had been made to accurately diagnose it and predict its behavior. One of the most intriguing biomarkers that have been assessed since 90s, is galectin-3. This study aimed to increase the prior knowledge of galectin-3 expression association with patient's clinicopathologic factors. In this single-center cross-sectional study, 71 patient samples from hospital archive were assessed for galectin-3 expression by immunohistochemistry assay. Pathologic slides were evaluated for histologic subtype, grade, stage, tumor size, presence of necrosis, and invasion of the renal vein. By adding cytoplasmic staining score to the color intensity score, a final score was recorded as galectin-3 positivity score. 88 pathological slides of patients with confirmed RCC were screened and 71 were finally assessed. The mean age of the patients was 58.52 years (lowest 30 and highest 87). 67.6% were males and 32.4% were females. 68% of tumors were clear cell carcinoma, and only one oncocytoma was present. All 9 chromophobe cases showed a strong galectin-3 expression. Except for female gender (47.8% vs 18.8% in men; P=0.01), no statistically significant association was found between patient age, tumor grade, tumor size, tumor stage, and renal vein invasion with the level of galectin-3 expression. Considering the contradictory findings between this study and other similar studies, it can be concluded that the physiological role of galectins is very complex and the need for larger and more comprehensive evaluations is felt.

Intriguing tumor, synovial sarcoma: hard to diagnose, harder to treat.

Synovial sarcoma (SS) is classified as an aggressive high-grade soft tissue sarcoma that predominantly affects the extremities. Despite its prevalence in the extremities (up to 80%), diagnostic and treatment challenges persist. This study aims to address these challenges by providing a comprehensive analysis of SS in extremities, focusing on diagnostic accuracy and treatment outcomes. The central questions of this study are: What are the diagnostic and treatment challenges associated with SS? How do tumor volume and histologic subtype influence prognosis? What role do immunohistochemistry and genetic markers play in SS diagnosis and management? We hypothesize that larger tumor volume and poor histologic differentiation are associated with worse survival outcomes in patients with SS. This retrospective study analyzed data from 63 patients diagnosed with SS between 2005 and 2020 at a single center. Patients with complete records of pathology, radiology, and surgery, and a minimum follow-up of 12 months were included. Tumor characteristics, treatment modalities, and follow-up data were reviewed. he study included 63 patients and 65 tumors. The mean age was 38 ± 17 years. There were 31 females (49%) and 32 males (51%). SS predominantly affected the extremities (n = 63, 97%), especially the lower limbs (n = 49, 75%). Tumor volume, with a mean of 110 ± 176 cm³, was a significant factor, with tumors >30 cm³ associated with higher rates of metastasis (p = 0,006) and reduced survival (p = 0,027). Histologically, 25 (38%) were monophasic, 24 (37%) were biphasic, and 16 (25%) were poorly differentiated, with poorer survival linked to poorly differentiated subtypes. Immunohistochemistry showed high positivity rates for Bcl-2 (89%, 17/19), EMA (88%, 52/59), and TLE1 (87%, 13/15). SS18-SSX fusion gene detected in 73% of cases (8/11). Metastasis occurred in 27 (42%) patients and recurrence in 24 (37%). 15 (23%) patients died from the disease. Accurate diagnosis of SS is crucial for effective management. Clinicians should be aware of negative predictive factors, including tumor volume >30 cm³ and poor histologic differentiation, when making treatment decisions. The study highlights the importance of extended follow-up due to the risk of late recurrence. IV.

Association of Histologic Subtype with Radiation Response and Survival Outcomes in Synovial Sarcoma

Association of Histologic Subtype with Radiation Response and Survival Outcomes in Synovial Sarcoma

BCL-2 Protein Expression in Epithelial Ovarian Cancer: Association with Age and Histopathological Features in Uganda

Background: Globally, ovarian cancer accounted for 314,000 new cases and 207,000 deaths in 2020. In Uganda, 626 cases were documented over 25 years, with an average incidence rate of 7.1 per 100,000 women. Epithelial ovarian carcinoma is the predominant subtype, and BCL-2, a 26-kDa anti-apoptotic protein, has been associated with chemotherapy resistance. However, no study has assessed BCL-2 expression in epithelial ovarian cancer in Uganda. Objective: This study aimed to determine the prevalence of BCL-2 protein expression in epithelial ovarian cancer and its association with age and histopathological features in Uganda. Methods: A retrospective cross-sectional study was conducted using randomly sampled archived tissue blocks from 113 patients diagnosed with epithelial ovarian cancer. Samples underwent hematoxylin and eosin (H&amp;E) and immunohistochemistry (IHC) staining to evaluate BCL-2 expression. Results: The mean patient age was 55 years, with the largest proportion (36.3%) aged 50–59 years. BCL-2 expression was observed in 36.3% of cases. There was no statistically significant association between BCL-2 expression and age (p = 0.396), histological subtype (p = 0.520), or tumor grade (p = 0.833). High-grade serous carcinoma was the predominant histological subtype (46%), and well-differentiated carcinoma was the most common grade (53%). Conclusion: BCL-2 expression was prevalent in epithelial ovarian cancer in Uganda but showed no significant association with age, histological grade, or subtype. These findings underscored the need for further research to explore the prognostic and therapeutic implications of BCL-2 in ovarian cancer management.

Treatment Approach for Rare Histologic Subtypes of Breast Cancer: A Review

Rare histological subtypes of breast cancer introduce unique challenges to treatment and require a more personalized approach. Subtypes with favorable prognoses, such as mucinous, tubular, and cribriform carcinomas, generally respond well to less aggressive treatments such as endocrine therapy. However, certain triple-negative breast cancer subtypes that are typically considered aggressive also demonstrate unexpectedly better outcomes in specific cases. Conversely, more aggressive subtypes, including invasive micropapillary carcinoma and high-grade metaplastic breast cancer, often present greater treatment challenges due to their resistance to conventional therapies. Recent advancements in targeted treatments, such as immune checkpoint inhibitors, have shown promise in improving outcomes in some of these difficult cases. This review emphasizes the need for ongoing research and a tailored approach that considers both molecular and histological characteristics to improve the management and survival outcomes of patients with these rare and complex breast cancer subtypes.

SH2D5 promotes lung adenocarcinoma cell metastasis and triggers EMT via activating AKT signaling pathway.

Lung adenocarcinoma (LUAD) is the most common histological subtype of lung cancer, characterized by a high incidence in late stages, high mortality rate, and poor prognosis. Src Homology 2 Domain Containing Protein 5 (SH2D5) is a mammalian-specific, uncharacterized scaffolding protein, and its role in LUAD remains unclear. In the present study, we investigated the function and potential mechanisms of SH2D5 in the progression of LUAD. We found aberrant expression of SH2D5 in LUAD tissues and cells, and its high expression is closely associated with poor prognosis in LUAD patients. Through loss-of-function and gain-of-function experiments, we revealed that overexpression of SH2D5 promotes the proliferation and migration abilities of lung adenocarcinoma cells. Gene set enrichment analysis (GSEA) revealed that SH2D5 positively regulates the epithelial-mesenchymal transition (EMT) process in lung adenocarcinoma cells. Additionally, we found that regulating the expression of SH2D5 influenced the phosphorylation levels of AKT, and the rescue experiments with AKT pathway activators/inhibitors partially reversed the tumor progression and EMT processes induced by SH2D5. In summary, our study demonstrated that SH2D5 promotes the migration and EMT process of LUAD cells through the AKT signaling pathway, suggesting that SH2D5 may serve as a crucial potential target for the treatment of metastatic LUAD.

Hypoxia drives the formation of lung micropapillary adenocarcinoma-like structure through hypoxia-inducible factor-1α

Micropapillary adenocarcinoma (MPC) is an aggressive histological subtype of lung adenocarcinoma (LUAD). MPC is composed of small clusters of cancer cells exhibiting inverted polarity. However, the mechanism underlying its formation is poorly understood. Here we show that hypoxia is involved in MPC formation. Hypoxia induced the formation of MPC-like structures (MLSs) in a three-dimensional culture system using A549 human LUAD cells, and HIF-1α was indispensable for MLS formation. RNA sequencing analysis demonstrated that A549 cells forming MLSs exhibited a gene expression signature similar to that of lung MPC. Moreover, MLS formation enhanced the resistance of A549 cells to natural killer cell cytotoxicity. Our findings suggest that hypoxia drives lung MPC formation through HIF-1α and that immune escape from natural killer cells might underlie the aggressiveness of MPC.

Impact of Positive Surgical Margins on Recurrence and Overall Survival Following Partial Nephrectomy: A Systematic Review and Meta-Analysis.

Positive surgical margins (PSM) following partial nephrectomy (PN) for renal cell carcinoma (RCC) are a concern due to potential implications for recurrence and survival. This systematic review and meta-analysis assess the impact of PSM on recurrence rates and progression-free survival in RCC patients. We conducted a systematic search of PubMed, Embase, Scopus, Cochrane, and Web of Science databases from inception through July 2024. Studies examining recurrence and survival outcomes in RCC patients with and without PSM post-PN were included. A random-effects model was applied to calculate pooled hazard ratios (HR) and 95% confidence intervals (CI) for recurrence and survival. Thirty studies met the inclusion criteria. Our analysis showed that PSM was significantly associated with a higher risk of local recurrence (HR = 2.13, 95% CI: 1.67-2.72) and a lower recurrence/progression-free survival (HR = 1.70, 95% CI: 1.40-2.07) compared to negative surgical margins. Subgroup analyses indicated consistent results across study designs and RCC histologic subtypes. The presence of PSM following PN for RCC is associated with a 2.13-fold increase in local recurrence and a 1.7-fold reduction in progression-free survival, emphasizing the need for precise margin management during surgery. These findings highlight the importance of optimizing surgical techniques and considering adjuvant treatment strategies for patients with PSM to improve oncologic outcomes.

Risk prediction criteria for the primary hepatic perivascular epithelioid cell tumour family, including angiomyolipoma: analysis of 132 cases with a literature review.

The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors. In total, 132 patients were included. Clinical data and histopathological slides were assessed along with the p53 and Ki-67 immunohistochemistry. Targeted next-generation sequencing was performed in three cases. Based on the histologic subtypes, 7 (10%), 36 (51%), 13 (18%), and 15 (21%) patients were classified as inflammatory angiomyolipoma, conventional angiomyolipoma, epithelioid angiomyolipoma, and PEComa not otherwise specified (NOS), respectively, among 71 patients who underwent surgical resection. We proposed the risk prediction criteria after defining primary tumour size ≥7 cm, infiltrative border, mitotic rate >1/10 mm2, necrosis, vascular invasion, and PEComa NOS as worrisome features, as follows: high-risk: ≥3 worrisome features; intermediate-risk: 1-2 features; low-risk: none of the features. Applying these criteria, 4 (6%), 31 (44%), and 36 (51%) patients were classified into high-, intermediate-, and low-risk groups, respectively. One patient each in the high-risk (25%) and intermediate-risk (3%) groups developed peritoneal metastases and intrahepatic recurrence, respectively, whereas none in the low-risk group showed disease progression. A literature review of clinically malignant hepatic PEComa family tumours was conducted, and upon application of our criteria, 62% (16/26) of the patients were classified as high-risk and 35% (9/26) as intermediate- or high-risk. Our risk prediction criteria can effectively predict the clinical outcomes in primary hepatic PEComa.

Autoimmune Pancreatitis: Approach to Diagnosis and Treatment

Autoimmune pancreatitis (AIP) is a chronic inflammatory disease of the pancreas that has classically been divided into distinct histologic and clinical subtypes [1]. Type I AIP, previously referred to as lymphoplasmacytic sclerosing pancreatitis, is the pancreatic manifestation of IgG4-related disease, which often occurs in the context of multi-organ involvement. In this context, type 1 AIP is most typically associated with IgG4-related cholangitis. Type II AIP, or idiopathic duct-centric pancreatitis, is typically a pancreas-isolated disease although approximately 30% of cases are associated with inflammatory bowel disease (IBD). More recently, type 3 AIP has been described as an immunological sequelae of immune-checkpoint inhibitor therapy [2]. This review will focus on the more classic subtypes of AIP, type 1 and 2. Although AIP is rare, prompt recognition is crucial as early intervention and disease control can prevent complications related to irreversible pancreatic damage such as exocrine pancreatic insufficiency and diabetes mellitus.