Carcinoma arising in ileal conduit or orthotopic ileal neobladder reconstruction: A 20-year single institute experience.

CONTEXT: Carcinomas found in urinary diversion specimens are uncommon, particularly new primary tumours. New primary tumours primarily occur when the large intestine is utilised, whereas the occurrence is infrequent with the use of the ileum. These tumours include both the recurrence of primary malignancy or the development of a new primary malignancy originating from the small intestine. DESIGN: A search was performed within the pathology laboratory system to identify cases of malignancies involving ileal conduit/reconstruction from 2002 to 2022. Data on demographics, clinical details, pathology and management was recorded. RESULTS: A total of 13 male patients, with a mean age of 67 years (range = 49-81 years) were included in the study. The initial procedure performed included cystoprostatectomy (n = 10, including one case with right nephroureterectomy) and cystectomy (n = 3, including one case for bladder exstrophy) for initial diagnoses including urothelial carcinoma (n = 11; conventional, 6; sarcomatoid, 1; glandular 1; plasmacytoid, 1; micropapillary, 2) and adenocarcinoma (n = 1). The initial management included radical surgery with neoadjuvant chemotherapy/immunotherapy (n = 1), adjuvant chemotherapy (n = 3), intravesical adjuvant BCG (n = 2) and intravesical adjuvant chemotherapy (n = 1). Malignancies in ileal conduit or orthotopic ileal neobladder included recurrent urothelial carcinoma (n = 10) and new secondary adenocarcinomas (n = 3), which developed as early as 3 months (usually recurrence) and up to 13, 33 and 45 years (new primary malignancy) following primary resection. CONCLUSIONS: Utilising the ileum as conduit/neobladder presents a viable alternative for urinary diversion with a reduced malignancy risk compared to using a segment of the large intestine. However, there remains a potential for malignancy, either tumour recurrence or a new primary malignancy. In our study, tumour recurrence occurred up to 4 years following the initial diagnosis and the development of a new primary malignancy occurred up to 45 years after the initial diagnosis. Consequently, it is crucial to prioritise long-term follow-up for these patients undergoing this procedure.

Successful Sequential Liver and Isolated Intestine Transplantation for Mitochondrial Neurogastrointestinal Encephalopathy Syndrome: A Case Report.

BACKGROUND Mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE) is an autosomal recessive disease caused by thymidine phosphorylase deficiency leading to progressive gastrointestinal dysmotility, cachexia, ptosis, ophthalmoparesis, peripheral neuropathy and leukoencephalopathy. Although liver transplantation corrects thymidine phosphorylase deficiency, intestinal deficiency of the enzyme persists. Retrospective chart review was carried out to obtain clinical, biochemical, and pathological details. CASE REPORT We present a case of liver and subsequent intestine transplant in a 28-year-old man with MNGIE syndrome with gastrointestinal dysmotility, inability to walk, leukoencephalopathy, ptosis, cachexia, and elevated serum thymidine. To halt progression of neurologic deficit, he first received a left-lobe partial liver transplantation. Although his motor deficit improved, gastrointestinal dysmotility persisted, requiring total parenteral nutrition. After exhaustive intestinal rehabilitation, he was listed for intestine transplantation. Two-and-half years after liver transplantation, he received an intestine transplant. At 4 years after LT and 20 months after the intestine transplant, he remains off parenteral nutrition and is slowly gaining weight. CONCLUSIONS This is the first reported case of mitochondrial neurogastrointestinal encephalomyopathy to undergo successful sequential liver and intestine transplantation.

Incidental findings during donor liver assessment: Single center experience.

Intraoperative consultation of donor liver is an important part of transplant evaluation and determination of liver eligibility. In this study, we describe incidental pathologic findings discovered during the pretransplant evaluation of liver donors in our Institution from 1/2010 to 12/2022. During this 13-year period 369 intraoperative consultations from 262 liver donors were performed. Of those cases, incidental findings were identified in 22 cases (5.9 %) from 19 donors (7.3 %); two donors had more than one lesion. The median age of this subset of patients was 53 years (range: 18-70) and females predominated (63 %). Sixteen of the donors had abnormal findings in the liver: 6 bile duct hamartoma (BDH), 5 hyalinized nodule with Histoplasma capsulatum, 5 focal nodular hyperplasia (FNH), 2 bile duct adenomas (BDA), 1 biliary cyst and 1 hemangioma. One donor had both FNH and a BDH. One BDH and 1 BDA case was misdiagnosed as malignancy during the frozen section evaluation. Three donors had extrahepatic pathologies: a pancreatic tail schwannoma, a low-grade appendiceal mucinous neoplasm, and a lymph node with metastatic endometrial endometrioid adenocarcinoma. Of the 19 livers, the final organ disposition was available for 9: 6 were transplanted (67 %) and 3 were discarded (33 %). Two of the 3 discarded organs were misdiagnosed BDH and BDA cases, and one was incorrectly reported as having 90 % microvesicular steatosis during the frozen assessment. We present the clinicopathologic characteristics of liver donors with incidental findings during the pre-transplant evaluation which could lead to unwarranted graft dismissal if misdiagnosed. Additionally, incidental fungal infections can have implications for immunosuppressive therapy and the decision to use or reject the graft.

Comparative Survival Benefits of Surgery and Adjuvant Chemotherapy in Neuroendocrine Carcinoma of the Gallbladder: A Population-Based Study with Insight into Future Personalized Therapeutic Approach.

BACKGROUND: Neuroendocrine carcinomas of the gallbladder (NECs-GB) are rare tumors, accounting for <0.2% of all neuroendocrine carcinomas of the gastrointestinal tract. They originate from the neuroendocrine cells of the gallbladder epithelium with associated intestinal or gastric metaplasia. The current study is the largest study from the SEER database on NECs-GB that aims to elucidate the demographic, clinical, and pathologic factors influencing the prognosis and comparative survival analysis of different treatment modalities. METHODS: The data from 176 patients with NECs-GB was abstracted from the Surveillance Epidemiology and End Result (SEER) database (2000-2018). Multivariate analysis, non-parametric survival analysis, and a chi-square test were used to analyze the data. RESULTS: NECs-GB had a higher incidence amongst females (72.7%) and Caucasians (72.7%). Most patients had surgery only (N = 52, 29.5%), (N = 40) 22.7% had chemotherapy only, and (N = 23) 13.1% had chemotherapy with surgery. Only (N = 17) 9.7% had trimodaltiy (surgery, chemotherapy, and radiation therapy), and for (N = 41) 23.3% the status of chemotherapy was unknown, and these cases had neither radiation nor surgery. CONCLUSION: NECs-GB more frequently affects Caucasian females after the 6th decade of life. The combination of surgery, radiation, and adjuvant chemotherapy was associated with better long-term (5 years) outcomes, while surgery alone was associated with better short-term (<2 years) outcome survival.

Comparison of endoscopic ultrasound-guided fine needle aspiration cytology versus endoscopic biopsy for the diagnosis of subepithelial lesions of the upper and lower gastrointestinal tract: A 10-year retrospective single institution analysis.

BACKGROUND: The aim of this study is to compare the diagnostic accuracy of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) versus endoscopic biopsy for the diagnosis of gastrointestinal (GI) subepithelial lesions (SELs) using surgical resection as the gold standard. METHODS: All patients who underwent EUS-FNA of upper and lower GI SELs over a 10-year period (2010 through 2019) were retrospectively reviewed. The medical records of all patients were reviewed and data extracted from the endoscopy, pathology, and surgical reports were analyzed. RESULTS: In total, 283 patients with ages ranging from 21 to 92 years underwent EUS-FNA for evaluation of GI SELs, 117 (41%) patients underwent endoscopic biopsy and 82 (29%) patients had concurrent surgical resection specimen. EUS-FNA was obtained from the stomach in 167 (59%) patients, duodenum in 51 (18%) patients, esophagus in 38 (13%) patients, and colorectum in 27 (10%) patients. It was found that the largest percentage of lesions originated in the muscularis propria (36%), followed by the submucosa (26%), deep mucosa (13%), and not specified in 21%. The concordance between EUS-FNA and endoscopic biopsy was good (correlation coefficient of 0.631, p < .001). EUS-FNA versus endoscopic biopsy in resected cases showed sensitivity and specificity of 78% versus 68% and 84% versus 100%, respectively. The EUS-FNA has an accuracy of 80% compared to 74% in biopsy. The diagnostic yield of EUS-FNA and endoscopic biopsy was 64% versus 55%. CONCLUSION: EUS-FNA is more sensitive and more accurate than endoscopic biopsy for diagnosing GI SELs with a good concordance between the two techniques.

Fine needle aspiration cytology of follicular dendritic cell sarcoma of the stomach masquerading as gastrointestinal stromal tumor: A case report of a unique entity with emphasis on cytomorphology.

Follicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm, postulated to arise from follicular dendritic cells, with approximately 343 reported cases. Less than 100 cases of FDCS were in the gastrointestinal tract, with only four cases described in the stomach, none of them diagnosed on fine needle aspiration (FNA) cytology. We report here the first case of FDCS of the stomach diagnosed on FNA. Our patient is a 31-year-old male who presented with several years history of intermittent abdominal pain prompting occasional emergency-room visits. Imaging showed a 10.6 cm mass arising from the stomach, concerning for gastrointestinal stromal tumor. FNA cytology was performed using five passes with a 22-gauge needle. The smears were moderately cellular consisting of sheets and large, loosely cohesive clusters of ovoid to spindle cells with indistinct cytoplasmic borders and abundant cytoplasm, peppered with numerous small mature lymphocytes. The nuclei of the tumor cells were oval with finely granular chromatin with frequent nuclear grooves, pseudoinclusions, and easily recognizable mitotic figures. The tumor cells were positive for FDCS markers (CD21, CD23, and CD35).

Clinicopathological and Treatment Patterns of Combined Small-Cell Lung Carcinoma with Future Insight to Treatment: A Population-Based Study.

BACKGROUND: Primary lung cancer is the most common cause of cancer-related mortality in the United States (US). Approximately 90% of lung cancers are associated with smoking and the use of other tobacco products. Based on histology, lung cancers are divided into small-cell lung carcinomas (SCLCs) and non-small-cell lung carcinomas (NSCLCs). Most SCLCs are of the pure subtype, while the rare combined SCLCs contain elements of both small-cell and non-small-cell morphologies. This study sought to evaluate the demographics, clinical factors, molecular abnormalities, treatment approaches, and survival outcomes with combined SCLC and NSCLCs. MATERIALS AND METHODS: Data on 2126 combined SCLC patients was extracted from the Surveillance Epidemiology and End Result (SEER) database from 2000 to 2018. Data extracted for analyses included age, sex, race, tumor size, tumor location, metastasis status, stage at diagnosis, treatment received, and treatment outcomes. Multivariate analysis was performed using Statistical Product and Service Solutions (SPSS) software. RESULTS: The patients had a median age of 68 years; 43.9% of the patients were female and 56.1% were male; 84.5% were White and 11.7% were African Americans. The majority of patients had a poorly differentiated disease at 29.6%; 17% were undifferentiated, 3.2% were moderately differentiated, and 0.8% were well differentiated. Chemotherapy was the most common treatment modality (45.3%); 17% underwent surgery only, 10.3% underwent surgery followed by adjuvant chemotherapy, and 10% underwent radiation after surgery. Five-year cancer-specific survival was 15.2% with surgery alone, and combined surgery and chemotherapy provided the highest percentages (38.3% and 34.7%, respectively). Females had significantly higher 1- and 5-year cancer-specific survival rates compared to males (59.3% and 29.9% vs. 48.0% and 23.7, respectively; p < 0.001). Well-differentiated tumors had significantly higher survival compared to other gradings (p < 0.001). Survival decreased as tumor staging moved distally from localized to regional to distant (p < 0.001). Metastasis to bone, liver, brain, and lung significantly decreased survival in comparison to patients who did not have any metastasis (p < 0.001). Females had significantly shorter survival compared to their counterparts when metastasis was to the bone, brain, or liver (p < 0.001). Multivariate analysis identified male sex (Hazard Ratio (HR) = 1.2), undifferentiated grade (HR = 1.9), regional extent of disease (HR = 1.7), distant extent of disease (HR = 3.7), and metastasis to liver (HR = 3.5) as variables associated with worse survival. CONCLUSION: Combined SCLC is overall very rare. However, the frequency of presentation with combined SCLC is on the rise, in part due to improvements in diagnostic techniques. Despite advances in therapies, treating combined SCLC is challenging, and novel therapies are not utilized, owing to low rates of targetable mutations. Combined SCLC has higher survival rates if well differentiated.

Sorghum Flour and Sorghum Flour Enriched Bread: Characterizations, Challenges, and Potential Improvements.

A Sorghum flour (SF) is a leading and prominent food source for humans in African countries. Recently extensive studies have been conducted on Sorghum bread (SB) or sorghum composite bread (SCB), covering various aspects. However, there are many technical challenges in the formation of SF and sorghum composite flour (SCF) that impact the quality of the bread and fail to meet the consumer's desires and expectations. This review primarily focuses on the characteristics of SF, SCF, SB, and SCB, with discussions encompassing the rheological and morphological properties of the dough, improvement strategies, and bread quality. Moreover, a comprehensive analysis has been conducted to investigate the behavior of SF and SCF along with a discussion of the challenges affecting bread quality and the strategies applied for improvement. The significant demand for nutrients-rich and gluten-free bread indicates that sorghum will become one of the most vital crops worldwide. However, further comprehensive research is highly demanded and necessary for an in-depth understanding of the key features of SF and the resulting bread quality. Such understanding is vital to optimize the utilization of sorghum grain in large-scale bread production.

Acute Intermittent Porphyria as a Rare Challenging Neuro-Metabolic Disease; a Case Report.

Porphyria is a challenging metabolic disease due to its heterogeneous presentation symptoms and its difficult diagnosis. Many affected individuals can complain of recurrent neuro-visceral attacks per year, some of which may be persistent and life-threatening, which is confusing if there is no established diagnosis. Although the motor manifestations, autonomic changes and seizure are highly suggestive, the diagnosis is often overlooked and needs confirmatory genetic testing. To the best of our knowledge, the acute intermittent porphyria (AIP) reported in this case, involving severe electrolyte disturbances and rapid severe weakness is a challenging neuro-metabolic case and is extremely rare worldwide. Here, we reported a case of AIP in a young girl who presented to the emergency department of Al-Araby international Hospital, Monufia, Egypt with severe abdominal pain, constipation, and headache which had started 10 days ago. It seems that the diagnosis of porphyria should be considered particularly in those patients with abdominal complaints associated with electrolyte disturbances, seizures, and severe progressive neuropathy.

Gastrointestinal histoplasmosis complicating pediatric Crohn disease: A case report and review of literature.

BACKGROUND: Infection with Histoplasma capsulatum (H. capsulatum) can lead to disseminated disease involving the gastrointestinal tract presenting as diffuse abdominal pain and diarrhea which may mimic inflammatory bowel disease (IBD). CASE SUMMARY: We report a case of 12-year-old boy with presumptive diagnosis of Crohn disease (CD) that presented with several months of abdominal pain, weight loss and bloody diarrhea. Colonoscopy showed patchy moderate inflammation characterized by erythema and numerous pseudopolyps involving the terminal ileum, cecum, and ascending colon. Histologic sections from the colon biopsy revealed diffuse cellular infiltrate within the lamina propria with scattered histiocytic aggregates, and occasional non-necrotizing granulomas. Grocott-Gomori's Methenamine Silver staining confirmed the presence of numerous yeast forms suggestive of Histoplasma spp., further confirmed with positive urine Histoplasma antigen (6.58 ng/mL, range 0.2-20 ng/mL) and serum immunoglobulin G antibodies to Histoplasma (35.9 EU, range 10.0-80.0 EU). Intravenous amphotericin was administered then transitioned to oral itraconazole. Follow-up computed tomography imaging showed a left lower lung nodule and mesenteric lymphadenopathy consistent with disseminated histoplasmosis infection. CONCLUSION: Gastrointestinal involvement with H. capsulatum with no accompanying respiratory symptoms is exceedingly rare and recognition is often delayed due to the overlapping clinical manifestations of IBD. This case illustrates the importance of excluding infectious etiologies in patients with "biopsy-proven" CD prior to initiating immunosuppressive therapies. Communication between clinicians and pathologists is crucial as blood cultures and antigen testing are key studies that should be performed in all suspected cases of histoplasmosis to avoid misdiagnosis and inappropriate treatment.

Tumor Genomic Profiling to Determine Tissue Origin of Cancers of Unknown Primary: A Single Institute Experience With its Utility and Impact on Patient Management.

Tumor genomic profiling represents a promising tool in diagnosis and management of cancer of unknown primary. We report our experience on the impact of genomic profiling in elucidating primary tumor site, correlation with pathologic findings and patient management. Tissue or cytology specimens from 22 cancers of unknown primary were referred for genomic profiling. Reports were available to review in 18 cases; 3 samples were inadequate for analysis. Of the remaining 15 cases, primary tumor site was suggested in 12 cases (80%), whereas it remained indeterminate in 3 (20%). Of the 12 cases, molecular profiling was concordant with light microscopy findings in 3 patients, whereas in 2 cases molecular testing identified a sarcoma, contradicting light microscopy and immunohistochemistry findings. The suggested primary was confirmed by additional immunohistochemistry in 1 case and by endoscopic biopsy in another. In 5 cases, follow-up biopsy or additional testing were not considered necessary for patient management. Three patients received palliative care and 12 received various chemotherapy regimens. Five patients died within a year, whereas 9 were alive more than a year after diagnosis, 3 of who were alive >3 years after diagnosis. In conclusion, genomic profiling helped confirm the original diagnosis and suggested primary sites in two third of our cases. Although many patients may be at a disease stage too advanced to withstand further investigations or underg aggressive therapy, molecular testing improves diagnostic accuracy and may thus assist in selection of the most appropriate therapy.

Effects of Bifidobacteria Fermentation on Physico-Chemical, Thermal and Structural Properties of Wheat Starch.

Lactic acid bacteria have been considered to be a very important species during sourdough fermentation. In order to explore the effects of bifidobacteria fermentation on thermal, physico-chemical and structural properties of wheat starch during dough fermentation, starch granules were separated from the fermented dough at different fermentation times, including 0 h, 2 h, 6 h, 9 h and 12 h. The results showed that the morphology of starch granules was destroyed gradually as the fermentation time increased, which appeared as erosion and rupture. With the increase in fermentation time, the solubility showed a significant increase, which changed from 8.51% (0 h) to 9.80% (12 h), and the swelling power was also increased from 9.31% (0 h) to 10.54% (12 h). As for the gelatinization property, the enthalpy was increased from 6.77 J/g (0 h) to 7.56 J/g (12 h), indicating a more stable thermal property of fermented starch, especially for the longer fermentation. The setback value was decreased with short fermentation time, indicating that the starch with a longer fermentation time was difficult to retrograde. The hardness of the gel texture was decreased significantly from 50.11 g to 38.66 g after fermentation for 12 h. The results show that bifidobacteria fermentation is an effective biological modification method of wheat starch for further applications.

Histopathologic correlation of pancreatic fibrosis with pancreatic magnetic resonance imaging quantitative metrics and Cambridge classification.

PURPOSE: To determine the correlation of the T1-weighted signal intensity ratio (T1 SIR, or T1 Score) and arterial-to-delayed venous enhancement ratio (ADV ratio) of the pancreas with pancreatic fibrosis on histopathology. METHODS: Sixty consecutive adult CP patients who had an MRI/MRCP study prior to pancreatic surgery were analyzed. Three blinded observers measured T1 SIR of pancreas to spleen (T1 SIR p/s), pancreas-to-paraspinal muscle (T1 SIR p/m), ADV ratio, and Cambridge grade. Histopathologic grades were given by a gastrointestinal pathologist using Ammann's fibrosis score. Statistical analysis included Spearman's correlation coefficient of the T1 SIR, ADV ratio, Cambridge grade with the fibrosis score, and weighted kappa for interobserver agreement. RESULTS: The study population included 31 female and 29 male patients, with an average age of 52.1 (26-78 years). Correlations between fibrosis score and T1 SIR p/s, T1 SIR p/m, and ADV ratio were ρ = - 0.54 (p = 0.0001), ρ = - 0.19 (p = 0.19), and ρ = - 0.39 (p = 0.003), respectively. The correlation of Cambridge grade with fibrosis score was ρ = 0.26 (p = 0.07). There was substantial interobserver agreement (weighted kappa) for T1 SIR p/s (0.78), T1 SIR p/m (0.71), and ADV ratio (0.64). T1 SIR p/s of ≤ 1.20 provided a sensitivity of 74% and specificity of 50% (AUC: 0.74), while ADV ratio of ≤ 1.10 provided a sensitivity of 75% and specificity of 55% (AUC: 0.68) to detect a fibrosis score of ≥ 6. CONCLUSION: There is a moderate negative correlation between the T1 Score (SIR p/s) and ADV ratio with pancreatic fibrosis and a substantial interobserver agreement. These parenchymal metrics show a higher correlation than the Cambridge grade.

Clinical, Pathologic, and Molecular-Genetic Aspects of Colorectal Polyps.

Most colorectal cancer arises from epithelial polyps. Polyps can be the result of acquired, germline, or inflammation-associated mutations in colonic stem cells (CSC). Their incidence and risk of progression are determined by factors that modify the baseline rate of spontaneous mutations occurring in CSC. In sporadic polyps, factors are primarily environmental; in individuals with germline mutations, it is the specific mutation, and in inflammation-associated polyps, it correlates with the extent, duration, and severity of the process. The different clinicopathologic and molecular genetic abnormalities underlying the different types of polyps are discussed.

Metastatic Liver Tumors in Surgical Pathology: Impact of Contemporary Diagnostic and Therapeutic Paradigms in a Tertiary Care Center.

We analyzed metastatic liver tumors received in the department of pathology in a tertiary care center over a 3-year period. There were 509 metastatic liver tumors; counterintuitively, there were as many resections (235 cases) as biopsies (274 cases). This unexpected finding reflects contemporaneous organ-specific paradigms for diagnosis and management of metastatic liver disease in oncologic practice, and the association of our practice with a National Cancer Institute-designated comprehensive cancer center with expertise and specialization in liver surgery. We receive a large number of resections for metastatic liver tumors because metastasectomy from a variety of primary tumors is associated with improved overall, and in many instances, disease-free, long-term survival. Metastatic colorectal carcinomas, metastatic neuroendocrine tumors, and metastatic gastrointestinal stromal tumors constituted 78% of resections because the largest body of literature and cumulative experience exists for these lesions. In contrast, breast carcinomas and pancreatic carcinomas, which are the next common metastatic liver tumors were biopsied but rarely resected, because metastasectomy is not the standard of care for these tumors. Immunohistochemistry was performed in less than a quarter of the total number of cases (23%), because the primary tumor site was known in the vast majority of cases. Of the 42 cases with unknown primary tumor, it was elucidated in 50% of the cases by immunohistochemical and clinical work-up. Of the cases with known primary tumor, immunohistochemistry was performed mostly in metastatic breast, colon, and lung carcinomas. In these cases, biomarker analyses provided additional information relevant to clinical management.

The Histopathologic Features of Sickle Cell Hepatopathy: A Multi-Institutional Study.

OBJECTIVES: Recent data on hepatic histopathology in patients with sickle cell disease (SCD) are lacking. METHODS: A total of 39 liver biopsies from SCD patients from 4 medical institutes were systematically evaluated. RESULTS: The average age of patients was 27 years; 23 were female. The majority of the patients had hemoglobin SS (33), 3 had hemoglobin SC, and 3 sickle cell trait. Elevated liver functional tests and evaluation for cirrhosis were the main indications for biopsy. At the time of biopsy, most had elevated liver transaminases or hepatomegaly. The most common histopathologic abnormalities were Kupffer cell erythrophagocytosis (76.9%), hemosiderosis (74.4%), sinusoidal dilatation (71.8%), and intrasinusoidal sickled red cells (69.3%). Portal inflammation, lobular inflammation, and bile duct injury were mild to minimal and present in a minority of cases. Advanced fibrosis was present in 28.2% of the cases. CONCLUSIONS: The typical histopathologic features seen in patients with SCD include Kupffer cell erythrophagocytosis, hemosiderosis, sinusoidal dilatation, and intrasinusoidal sickled red cells in a pauci-inflammatory or uninflamed background. Necrosis is less common than reported in older literature. Pathologists should be aware that significant portal and lobular inflammation, interface activity, and bile duct injury are unusual and may be suggestive of other etiologies.

Isolated Kaposi sarcoma of the upper aerodigestive tract in immunocompetent individuals, an underrecognized entity with favorable prognosis.

Kaposi sarcoma (KS) is a rare vascular neoplasm that most commonly arises in the setting of immunosuppression, in areas with high prevalence of Human Herpesvirus-8 infection, and when both situations coexist. Most cases affect the skin, isolated involvement of the upper respiratory tract without skin involvement is extremely rare with only a few cases reported in the literature. We present a case of isolated nasopharyngeal KS in an immunocompetent patient who achieved remission after multimodality therapy. Recent advances in KS-therapy are discussed.

Evaluating mismatch repair deficiency for solid tumor immunotherapy eligibility: immunohistochemistry versus microsatellite molecular testing.

While many landmark solid tumor immunotherapy studies show clinical benefits for solid tumors with high microsatellite instability (MSI-H) and mismatch repair deficiency (dMMR), the methodologies focus only on confirmatory polymerase chain reaction (PCR) testing for MSI-H. Because some tumors are either dMMR or MSI-H but not the other, clinicians must choose between two testing methods for a broad patient population. We investigated the level of correlation between MMR protein immunohistochemistry (IHC) and microsatellite PCR testing results in 62 cancer patients. Thirty-five of the 62 cases (56.5%) were MSI-H by PCR, whereas 35 (56.5%) were dMMR by IHC. MMR IHC results correlated well with MSI PCR in 32 co-positive cases (91.4%) and 24 co-negative cases (88.9%). Six discrepant cases (9.7%) were identified, among which three were MSI-H and MMR intact, and three were dMMR and microsatellite stable. The results of this study highlight the implications of dMMR/MSI testing strategies on precision oncology. Co-testing with both MMR IHC and MSI PCR may be an effective screening strategy for evaluating immunotherapy eligibility status for solid tumors.

The cytology of salivary gland neoplasms with globules of extracellular matrix: Case-based review of adenoid cystic carcinoma and its potential mimics.

Fine needle aspiration (FNA) cytology is an important tool for diagnosing salivary gland neoplasms and for guiding clinical management. The classic adenoid cystic carcinoma (AdCC) is a basaloid neoplasm with abundant extracellular matrix. The presence globules of extracellular matrix are quite characteristic of AdCC but not diagnostic. We selected from our files six FNA cases that contained at least some globules of amorphous matrix that are similar to the ones seen in AdCC. The aim of this case-based review is to discuss the pitfalls and some of the common differential diagnoses of AdCC in FNA cytology. By the end of this review, we hope to have shared with the readers the lessons we learned from these cases and to highlight the key criteria needed to make a correct diagnosis of AdCC based on cytomorphology. The importance of considering other entities, in addition to AdCC, whenever a salivary gland FNA presents with globules is emphasized.

Morphologic and Immunohistochemical Characteristics of Fluorescent In Situ Hybridization Confirmed TFE3-Gene Fusion Associated Renal Cell Carcinoma: A Single Institutional Cohort.

TFE3-fusion associated renal cell carcinoma (TFE3-RCC) accounts for up to 5% adults and 40% of childhood RCC. Their comprehensive immunohistochemical (IHC) profile in correlation to fluorescence in situ hybridization (FISH) testing and their role in the diagnostic approach are not well documented because of lacking published data. FISH confirmed TFE3-RCC between years 2010 and 2020 were identified from institutional electronic database and retrospectively reviewed. Eighty-five TFE3-RCC were identified. Seventy-six of 85 (89.4%) TFE3-RCC cases had positive TFE3 expression, with diffuse and strong/moderate TFE3 expression in 45 (54.2%). Three (3.5%) TFE3-RCC had negative TFE3 expression whereas 6 (7%) cases had equivocal TFE3 expression. On the other hand, positive TFE3-IHC expression was observed in 17/29 (58.6%) TFE3-FISH negative RCC cases, although only 8 (27.5%) had diffuse and moderate/strong TFE3 expression. Diffuse and strong TFE3-IHC expression was statistically significant in predicting TFE3-FISH positivity (P<0.0001) regardless of morphologic features. After univariate and multivariate analyses, TFE3-IHC was the only parameter with significant predictive value for detecting positive TFE3-FISH (P<0.0001). On univariate analysis, sex, classic morphology, age, negative AE1/AE3 or cytokeratin 7 were not predictive of TFE3-FISH positivity. Diffuse and strong nuclear TFE3-IHC expression is significantly associated with TFE3-FISH positivity and can be used as a surrogate marker to confirm translocation associated cases. TFE3-rearranged RCCs show variable histomorphologic features and TFE3-FISH should be performed in cases presenting at a younger age or, regardless of the age, tumors with unusual morphology. Despite previous reports, negative pancytokeratin and positive cathepsin K expression may not be reliable markers for TFE3-RCC.