Impact of a Routine Colorectal Endoscopic Submucosal Dissection in the Surgical Management of Nonmalignant Colorectal Lesions Treated in a Referral Cancer Center.

BACKGROUND: Recent data show an increasing number of abdominal surgeries being performed for the treatment of nonmalignant colorectal polyps in the West but in settings in which colorectal endoscopic submucosal dissection is not routinely performed. This study evaluated the number of nonmalignant colorectal lesions referred to surgical treatment in a tertiary cancer center that incorporated magnification chromoendoscopy and endoscopic submucosal dissection as part of the standard management of complex colorectal polyps. OBJECTIVE: The study aimed to estimate the number of patients with nonmalignant colorectal lesions referred to surgical resection at our institution after the standardization of routine endoscopic submucosal dissection and to describe outcomes for patients undergoing colorectal endoscopic submucosal dissection. DESIGN: Single-center retrospective study from a prospectively collected database of endoscopic submucosal dissections and colorectal surgeries performed between January 2016 and December 2019. SETTING: Reference cancer center. PATIENTS: Consecutive adult patients with complex nonmalignant colorectal polyps were included. INTERVENTIONS: Patients with nonmalignant colorectal polyps were treated by endoscopic submucosal dissection or surgery (elective colectomy, rectosigmoidectomy, low anterior resection, or proctocolectomy). MAIN OUTCOMES MEASURES: The primary outcome measure was the percentage of patients referred to colorectal surgery for nonmalignant lesions. RESULTS: In the study period, 1.1% of 825 colorectal surgeries were performed for nonmalignant lesions, and 97 complex polyps were endoscopically removed by endoscopic submucosal dissection. The en bloc, R0, and curative resection rates of endoscopic submucosal dissection were 91.7%, 83.5%, and 81.4%, respectively. The mean tumor size was 59 (SD 37.8) mm. Perforations during endoscopic submucosal dissection occurred in 3 cases, all treated with clipping. One patient presented with a delayed perforation 2 days after the endoscopic resection and underwent surgery. The mean follow-up period was 3 years, with no tumor recurrence in this cohort. LIMITATIONS: Single-center retrospective study. CONCLUSIONS: A workflow that includes assessment of the lesions with magnification chromoendoscopy and resection through endoscopic submucosal dissection can lead to a very low rate of abdominal surgery for nonmalignant colorectal lesions. See Video Abstract at http://links.lww.com/DCR/C123 . IMPACTO DE LA DISECCIN SUBMUCOSA ENDOSCPICA COLORRECTAL DE RUTINA EN EL MANEJO QUIRRGICO DE LESIONES COLORRECTALES NO MALIGNAS TRATADAS EN UN CENTRO ONCOLGICO DE REFERENCIA: ANTECEDENTES:Datos recientes muestran un número cada vez mayor de cirugías abdominales realizadas para el tratamiento de pólipos colorrectales no malignos en Occidente, pero no en los entornos donde la disección submucosa endoscópica colorrectal se realiza de forma rutinaria. El estudio evaluó el número de lesiones colorrectales no malignas referidas a tratamiento quirúrgico en un centro oncológico terciario, que incorporó cromoendoscopia de aumento y disección submucosa endoscópica como parte del manejo estándar de pólipos colorrectales complejos.OBJETIVO:Estimar el número de pacientes con lesiones colorrectales no malignas referidos para resección quirúrgica en nuestra institución, después de la estandarización de la disección submucosa endoscópica de rutina y describir los resultados para los pacientes sometidos a disección submucosa endoscópica colorrectal.DISEÑO:Estudio retrospectivo de un solo centro, a partir de una base de datos recolectada prospectivamente de disecciones submucosas endoscópicas y cirugías colorrectales realizadas entre enero de 2016 y diciembre de 2019.AJUSTE:Centro oncológico de referencia.PACIENTES:Pacientes adultos consecutivos con pólipos colorrectales no malignos complejos.INTERVENCIONES:Pacientes con pólipos colorrectales no malignos tratados mediante disección submucosa endoscópica o cirugía (colectomía electiva, rectosigmoidectomía, resección anterior baja o proctocolectomía).PRINCIPALES MEDIDAS DE RESULTADO:La medida de resultado primario fue el porcentaje de pacientes remitidos a cirugía colorrectal por lesiones no malignas.RESULTADOS:En el período, 1,1% de 825 cirugías colorrectales fueron realizadas por lesiones no malignas y 97 pólipos complejos fueron extirpados por. disección submucosa endoscópica. Las tasas de resección en bloque, R0 y curativa de disección submucosa endoscópica fueron 91,7%, 83,5% y 81,4%, respectivamente. El tamaño tumoral medio fue de 59 (DE 37,8) mm. Se produjeron perforaciones durante la disección submucosa endoscópica en 3 casos, todos tratados con clipaje. Un paciente presentó una perforación diferida 2 días después de la resección endoscópica y fue intervenido quirúrgicamente. El seguimiento medio fue de 3 años, sin recurrencia tumoral en esta cohorte.LIMITACIONES:Estudio retrospectivo de un solo centro.CONCLUSIONES:Un flujo de trabajo que incluye la evaluación de las lesiones con cromoendoscopia de aumento y resección a través de disección submucosa endoscópica, puede conducir a una tasa muy baja de cirugía abdominal para lesiones colorrectales no malignas. Consulte Video Resumen en http://links.lww.com/DCR/C123 . (Traducción-Dr. Fidel Ruiz Healy ).

Long-term outcomes of endoscopic submucosal dissection and transanal endoscopic microsurgery for the treatment of rectal tumors.

BACKGROUND AND AIM: Endoscopic submucosal dissection and transanal endoscopic microsurgery are good options for the treatment of rectal adenomas and early rectal carcinomas, but whether long-term outcomes of these procedures are comparable is not known. The aim of this study was to address this question. METHODS: A retrospective single-center study evaluating 98 consecutive procedures between June 2008 and December 2017 was performed in a tertiary cancer center. Consecutive patients who had undergone either endoscopic submucosal resection or transanal endoscopic microsurgery for rectal adenomas and early rectal carcinomas were evaluated, and long-term recurrence and complication rates were compared. RESULTS: Both groups were similar regarding sex, age, preoperative surgical risk, and en bloc resection rate (95.7% in the endoscopic and 100% in the surgical group, P = 0.81). Mean follow-up period was 37.6 months. Lesions resected endoscopically were significantly larger (68.5 mm) than those resected by transanal resection (44.5 mm), P = 0.003. Curative resections occurred in 97.2% of endoscopic resections and 85.2% of the surgical ones (P = 0.04). Comparing resections that fulfilled histologic curative criteria, there were no recurrences in the endoscopic group (out of 69 cases) and two recurrences in the transanal group (8.3% of 24 cases), P = 0.06. Late complications occurred in 12.7% of endoscopic procedures and 25.9% of surgical procedures (P = 0.13). CONCLUSIONS: In our experience, endoscopic submucosal resection seems to have advantages over transanal endoscopic microsurgery, with similar en bloc resection rate and lower rate of late complications and recurrences. Multicenter randomized controlled trials are needed to support our findings.

Prognostic Value of Tumor Regression Grade Based on Ryan Score in Squamous Cell Carcinoma and Adenocarcinoma of Esophagus.

PURPOSES: Tumor regression grade (TRG) of the primary tumor after neoadjuvant therapy is one of the most sensitive prognostic factors among patients with locally advanced esophageal cancer, although no TRG system is fully accepted. The Ryan score was proposed in 2005 to evaluate TRG in rectal cancer and could be adaptable for pathological evaluation of esophageal cancer. The objective of this study is to evaluate the prognostic value of the Ryan score for esophageal cancer in the setting of trimodal therapy. METHODS: We performed a retrospective cohort study in which patients with locally advanced esophageal cancer, submitted to neoadjuvant therapy followed by surgical resection, were selected. One hundred thirty-four patients were selected. All tissue specimens were assessed as per the TRG system proposed by Ryan et al. Survival curves were assessed by the Kaplan-Meier method and log-rank test. Chi-square test or likelihood-ratio test was used for absolute and relative variables. Kruskal-Wallis and analysis of variance tests were used to assess significant differences on a continuous dependent variable by a categorical independent variable. RESULTS: Of the 134 included patients, 94 (70.1%) had squamous cell carcinoma, and 40 (29.9%) adenocarcinoma. Ryan score was correlated with histological type (p < 0.001), and clinical (p = 0.044) and pathological (p < 0.001) staging. Mean follow-up was 31.1 months. Multivariate analysis showed that Ryan score can safely predict survival, and systemic and lymphatic recurrence (p < 0.05). CONCLUSIONS: Ryan score is an effective system to evaluate TRG and can predict risk for lymph node or distant metastasis, overall survival, and disease-free survival.

Mucinous cystic neoplasm of the liver with biliary communication: case report and surgical therapeutic option.

BACKGROUND: Mucinous cyst neoplasm of the liver (MCN-L) comprise less than 5% of all cystic liver lesions and is characterized by the presence of ovarian stroma and absence of bile duct communication. CASE PRESENTATION: Here, we discuss a 45-year-old woman who presented with symptomatic liver mass. Diagnostic workup detected a 4.2 × 3.6 cm septate cyst located in segments I, V, and VIII of the liver in communication with the right hepatic duct. An open right liver resection with total bile duct excision and hilar lymphadenectomy was performed. Pathology revealed a multiloculated cyst with lined mucinous epithelium and ovarian-like stroma, consistent with low-grade MCN-L. CONCLUSIONS: This case shows that unusual location and bile duct communication can be present in MCN-L.

Signet ring cell component, not the Lauren subtype, predicts poor survival: an analysis of 198 cases of gastric cancer.

AIM: Prognostic differences between major histologic gastric cancer groups, intestinal and diffuse are uncertain, since cellular components in each of them possibly have different behaviors. MATERIALS & METHODS: We reviewed 198 gastric cancer patients charts diagnosed from January 2003 to December 2015 in a tertiary hospital. Multivariate Cox proportional survival models were used to evaluate the impact of histologic groups on overall survival. RESULTS: About a third had the signet-ring cell carcinoma (SRCC). In a comparison of the different histologic subtypes, SRCC had the worst prognosis of all. The median durations of survival for patients with stage III and stage IV were 19.7 and 7.7 months, respectively. CONCLUSION: Signet-ring cell component seem to have a relevant role in defining prognosis for gastric cancer.

Microsatellite Instability and Clinical Use in Sarcomas: Systematic Review and Illustrative Case Report.

Sarcomas, a diverse group of malignancies, exhibit substantial heterogeneity in both biological behavior and microenvironment, influencing their response to immunotherapy. Although pembrolizumab is approved for deficient mismatch repair or microsatellite instability-high (dMMR/MSI-H) tumors regardless of histology, there is a paucity of data to support its effectiveness in dMMR/MSI-H sarcomas. This study presents a case of a metastatic undifferentiated pleomorphic sarcoma of the retroperitoneum with dMMR status demonstrating a sustained complete response to pembrolizumab. Our case revealed inconsistencies in identifying dMMR/MSI-H status through next-generation sequencing, immunohistochemistry, and polymerase chain reaction methods. Therefore, we conducted a systematic review to analyze various methods for assessing dMMR/MSI-H and to explore whether pembrolizumab's indications rely on this biomarker.

Gallbladder schistosomiasis.

Schistosomiasis is an infectious disease caused by parasitic flatworms of the genus Schistosoma. The species Schistosoma mansoni is associated with hepatosplenic disease. Schistosomiasis involving the gallbladder alone is highly unusual, with a few cases reported. Herein, we present the case of a woman from a region with endemic schistosomiasis who presented with a painless solid lesion and wall thickening of the gallbladder. She underwent an uneventful laparoscopic cholecystectomy. Microscopic examination of the surgical specimen revealed Schistosoma mansoni eggs associated with granulomatous reaction, leading to the diagnosis of schistosomiasis of the gallbladder, prompting subsequent treatment with praziquantel and follow-up. This case illustrates the importance of suspicion for this diagnosis in endemic areas, as it can be misdiagnosed with malignancy if not examined microscopically. Complications and treatment strategies are poorly characterized for the few cases of schistosomiasis; reporting this case can serve as a helpful reminder of a rare presentation of this disease.

Prevalence of germline variants in Brazilian pancreatic carcinoma patients.

We evaluated the prevalence of pathogenic/likely pathogenic germline variants (PGV) in Brazilian pancreatic adenocarcinoma (PC) patients, that represent a multiethnic population, in a cross-sectional study. We included 192 PC patients unselected for family history of cancer. We evaluated a panel of 113 cancer genes, through genomic DNA sequencing and 46 ancestry-informative markers, through multiplex PCR. The median age was 61 years; 63.5% of the patients presented disease clinical stages III or IV; 8.3% reported personal history of cancer; 4.7% and 16.1% reported first-degree relatives with PC or breast and/or prostate cancer, respectively. Although the main ancestry was European, there was considerable genetic composition admixture. Twelve patients (6.25%) were PGV carriers in PC predisposition genes (ATM, BRCA1, BRCA2, CDKN2A, MSH2, PALB2) and another 25 (13.0%) were PGV carriers in genes with a limited association or not previously associated with PC (ACD, BLM, BRIP1, CHEK2, ERCC4, FANCA, FANCE, FANCM, GALNT12, MITF, MRE11, MUTYH, POLE, RAD51B, RAD51C, RECQL4, SDHA, TERF2IP). The most frequently affected genes were CHEK2, ATM and FANC. In tumor samples from PGV carriers in ACD, BRIP1, MRE11, POLE, SDHA, TERF2IP, which were examined through exome sequencing, the main single base substitutions (SBS) mutational signature was SBS1+5+18, probably associated with age, tobacco smoking and reactive oxygen species. SBS3 associated with homologous repair deficiency was also represented, but on a lower scale. There was no difference in the frequency of PGV carriers between: (a) patients with or without first-degree relatives with cancer; and (b) patients with admixed ancestry versus those with predominantly European ancestry. Furthermore, there was no difference in overall survival between PGV carriers and non-carriers. Therefore, genetic testing should be offered to all Brazilian pancreatic cancer patients, regardless of their ancestry. Genes with limited or previously unrecognized associations with pancreatic cancer should be further investigated to clarify their role in cancer risk.

PANCREATIC SOLID-PSEUDOPAPILLARY NEOPLASM IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS.

BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.

Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort.

Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.

A rare sporadic pancreatic desmoid fibromatosis with splenic vein invasion diagnosed by CT scan-guided core needle biopsy: a case report with possible differential diagnosis from metastatic colorectal or renal cancer.

Desmoid tumor is a rare fibroblastic proliferation with a variable and often unpredictable clinical course that arises in the deep soft tissues and is characterized by infiltrative growth with tendency to local recurrence but not to metastasize. A 49-year-old man was referred for a second opinion regarding a pancreatic mass. With a personal neoplastic background of two different tumors, we considered as a high probability of being metastatic of his previous colorectal or renal cancers, in a peritoneal implant. Due to the unclear origin and nature of the mass, we opted for requesting a computed tomography (CT)-guided core needle biopsy that could eventually lead to a surgical and/or chemotherapy treatment. So far, this is the first case of pancreatic desmoid fibromatosis with splenic vein invasion diagnosed by CT scan-guided core needle biopsy. Surgery should be performed by an experienced surgeon as first-line therapy, provided expected surgical morbidity is limited.

Response to Pembrolizumab in Advanced Anal Squamous Cell Carcinoma With High TMB and PD-L1 and PD-L2 Amplification.

We report the case of a 44-year-old female with a prior diagnosis of Sjögren's syndrome who was treated for metastatic anal squamous cell carcinoma with second-line pembrolizumab and has achieved a sustained partial response after a follow-up of 13 months. Comprehensive genomic profiling was remarkable for PD-L1 and PD-L2 amplification and a high tumor mutational burden (19 mutations per megabase). To the best of our knowledge, we present the first report to correlate PD-L1 and PD-L2 amplification with good outcomes of immune checkpoint inhibition in metastatic anal squamous cell carcinoma.

Chronic pancreatitis with ductal stones in the pancreatic head treated by surgery: a case report.

Pancreatic duct stones are direct sequelae of chronic pancreatitis (CP) and can occur in ∼50% of patients. Selection of the appropriate treatment method for pancreatic duct stones depends on location, size and number of stones. We present a patient with upper abdominal pain and weight loss for the previous 3 months. Diagnostic workup detected a chronic inflammation of the pancreas with stone in the main pancreatic duct and a nodular lesion in the head of the pancreas. Endoscopic retrograde cholangiopancreatography was performed without success. Given the rise in incidence and prevalence of CP, the potential complications and high mortality rate, it is imperative that physicians understand the risk factors, disease process and management of this disease. Pancreaticoduodenectomy in patients with CP is a feasible option for the treatment of focal cystic lesions to the head of the pancreas associated to pancreatic stone in selected cases.

Synchronous gallbladder adenocarcinoma and gastric gastrointestinal stromal tumor: Case report and literature review.

INTRODUCTION: Synchronous occurrence of different types of neoplasms is not very frequent, representing around 6% of all cases of cancer. Usually there is a lack of information on how to treat these patients, especially when both types of cancers are also uncommon. No cases of synchronous gallbladder adenocarcinoma and gastric gastrointestinal stromal tumor have been published before. PRESENTATION OF CASE: We present the case, management and follow-up, of a 66-year-old female with incidental diagnosis of a pT2NxMx gallbladder adenocarcinoma after elective cholecystectomy that latter, during staging, was also diagnosed with GIST. Total gastrectomy, wedge resection of the liver and lymphadenectomy were performed due to the new findings. Adjuvant chemotherapy for 36 months was indicated. After 16 months of the treatment she has no signs of recurrence. DISCUSSION: Gastrointestinal stromal tumors (GISTs) had a turnaround in the end of the 20th century after the introduction tyrosine-kinase inhibitor to the adjuvant treatment and now the trend is to extend it up to 36 months in selected patients. Gallbladder adenocarcinoma is an uncommon cancer but the incidental diagnosis is increasing with the popularity of laparoscopic cholecystectomy and, thus, specific management should be offered for these patients, what frequently includes a complementary surgery. Although, GISTs may be associated with another synchronous tumor in 20% of the cases, the simultaneous occurrence with gallbladder cancer is incredibly rare. CONCLUSION: Simultaneous occurrence of gastric GIST and gallbladder adenocarcinoma has not been reported before and, thus, any information about it may help in the management of those patients.

Pancreatic solid-pseudopapillary neoplasm in patients with familial adenomatous polyposis / Neoplasia sólida pseudopapilífera pancreática em pacientes com polipose adenomatosa familiar

ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.

RESUMO RACIONAL: A neoplasia sólida pseudopapilífera do pâncreas é um tumor pancreático incomum, mais frequente em mulheres jovens. A polipose adenomatosa familiar, por sua vez, é uma condição genética associada a câncer colorretal e que também aumenta o risco de desenvolvimento de outros tumores. OBJETIVOS: Discutir a associação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera, que é bastante rara. MÉTODOS: Reportamos dois casos de pacientes com polipose adenomatosa familiar, um homem e uma mulher, que desenvolveram neoplasia sólida pseudopapilífera do pâncreas e foram submetidos a ressecção laparoscópica com preservação esplênica. RESULTADOS: As vias de sinalização da ß-catenina e Wnt tem um papel importante na tumorigênese da neoplasia sólida pseudopapilífera, e sua ativação constitutiva devido a inativação do gene adenomatous polyposis coli na polipose adenomatosa familiar pode explicar a relação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera. CONCLUSÕES: A ressecção do cólon deve ser priorizada, com preferência pela abordagem minimamente invasiva para minimizar o risco de desenvolvimento de tumor desmoide. A ressecção pancreática geralmente não requer linfadenectomia extensa para neoplasia sólida pseudopapilífera, portanto, a preservação esplênica é factível.

Liver Metastases in Pancreatic Acinar Cell Carcinoma Treated with Selective Internal Radiation Therapy with Y-90 Resin Microspheres.

BACKGROUND: Pancreatic acinar cell carcinoma (PACC) is a rare tumor. Surgical resection is the treatment of choice when feasible, but there are no clear recommendations for patients with advanced disease. Liver-directed therapy with Y-90 selective internal radiation therapy (SIRT) has been used to treat hepatic metastases from pancreatic tumors. We describe a case of PACC liver metastases treated with SIRT. CASE REPORT: 59-year-old man was admitted with an infiltrative, solid lesion in pancreatic tail diagnosed as PACC. Lymph nodes in the hepatic hilum were enlarged, and many metastatic liver nodules were observed. After partial pancreatectomy, the left and right lobes of the liver were separately treated with Y-90 resin microspheres. Follow-up imaging revealed that all hepatic nodules shrank by at least 50%, and 3 nodules disappeared completely. Lipase concentration was 8407 U/L at baseline, rose to 12,705 U/L after pancreatectomy, and declined to 344 U/L after SIRT. Multiple rounds of chemotherapy in the subsequent year shrank the hepatic tumors further; disease then progressed, but a third line of chemotherapy shrank the tumors again, 16 months after SIRT treatment. CONCLUSION: SIRT had a positive effect on liver metastases from PACC. In conjunction with systemic therapy, SIRT can achieve sustained disease control.

Leydig cell tumor of the testis with histological and immunohistochemical features of malignancy in a 1-year-old boy with isosexual pseudoprecocity.

The article reports the clinical, histopathological, and immunohistochemical findings of a 1-year-old boy presenting with isosexual pseudoprecocity attributable to a functioning Leydig cell tumor of the testis. The case appears to represent the youngest patient ever recognized with this well-known syndrome. Malignancy features were also for the first time initially assessed using criteria, retrospectively developed from the literature, for metastasizing Leydig cell tumor. All the following were found: infiltrative borders, cellular pleomorphism, high mitotic index (12-14/high-power field), high MIB-1 index (40%), P53 positivity in 50% of the cells, and bcl-2 positivity in 15% of the cells. Immunohistochemistry proved the cells of the tumor to be positive for inhibin, Melan-A, synaptophysin, cytokeratin, and calretinin and negative for S-100 and chromogranin A. Notably, lipochrome and crystals of Reinke were not found in the tumor cells. Although the neoplasm fulfilled the criteria for a potentially metastasizing Leydig cell tumor, there was no evidence of that event having occurred, perhaps as a result of early treatment or as indication that criteria developed for Leydig cell tumor of adults may not apply to children.

Evaluation and comparison of microvessel density using the markers CD34 and CD105 in regenerative nodules, dysplastic nodules and hepatocellular carcinoma.

PURPOSE: The progression of hepatocellular carcinoma (HCC) is multifactorial and angiogenesis plays a fundamental role, mainly because HCC is a highly vascularized tumor. METHODS: In this study, we determined microvessel density (MVD) using the immunohistochemical markers, CD34 and CD105 (Endoglin), in 44 hepatectomy specimens, encompassing 44 malignant nodules (HCC), 44 regenerative nodules (RN), and 15 dysplastic nodules (DN). The evaluation included the determination of MVD in all nodules. For statistical analysis, a descriptive analysis was carried out using measurements of position and dispersion for continuous variables; ANOVA was used to compare between groups, considering p < 0.05 as statistically significant. RESULTS: We observed a significant difference when comparing CD34 and CD105 immunoexpression in HCC, DN, and RN. CD105 was predominantly expressed in the peripheral regions in HCC, with mean MVD scores of 6.2 ± 4.1 and 10.7 ± 4.4 at the center and periphery of the nodules, respectively, with significant differences between groups (p < 0.0001). CD34 had higher mean MVD scores than CD105 in HCC, with a more uniform positivity pattern. CD105 immunoexpression in DN exhibited a pattern similar to HCC. However, in RN, CD105 exhibited a higher MVD score in the central portion of the nodules. CD105 was expressed in a subset of newly formed microvessels in HCC and demonstrated an elevated mean MVD in cirrhotic or regenerative nodules. CONCLUSIONS: MVD determined by CD34 and CD105 expression may be used as an additional parameter to distinguish benign from malignant liver nodules.

Hepatocellular carcinomas within the Milan criteria: predictors of histologic necrosis after drug-eluting beads transarterial chemoembolization.

PURPOSE: To evaluate pathologic, imaging, and technical predictors of therapy response in patients with hepatocellular carcinoma (HCC) within the Milan criteria undergoing doxorubicin drug-eluting beads transarterial chemoembolization (DEB-TACE) before orthotopic liver transplantation (OLT). METHODS: This prospective study included consecutive patients with HCC who underwent DEB-TACE before OLT. Tumor histologic necrosis on liver explants was utilized as the standard of reference to categorize treated HCCs as group 1 (>50 % necrosis) or group 2 (≤50 % necrosis). DEB-TACE technique, histological factors, and imaging evaluation utilizing the modified Response Evaluation Criteria in Solid Tumors (mRECIST) were compared between groups 1 and 2. RESULTS: Twenty-seven HCCs were identified in 23 patients. Group 1 comprised 18 HCCs (mean necrosis 86.2 %). Group 2 comprised 9 HCCs (mean necrosis 31.1 %). The mean time between the last DEB-TACE session and the OLT was 112 days. Lesion size was significantly larger in group 1 (mean 3.2 cm; 95 % confidence interval 2.55-3.85) than in group 2 (mean 2.1 cm; 95 % confidence interval 1.79-2.48) (p = 0.030). Group 1 also demonstrated a higher frequency of encapsulated lesions when compared to group 2 (78 % vs. 22 %; p = 0.0027). A significant linear correlation was found between the quantification of necrosis by imaging and pathology (p = 0.0011) using the mRECIST, with a poorer correlation index in group 2. CONCLUSION: Larger and encapsulated HCCS are associated with a higher percentage of necrosis. A significant linear correlation between the amount of necrosis by imaging and pathology was encountered when mRECIST was utilized.