BCOR-rearranged sarcoma was classified by the World Health Organization in 2020 as a new subgroup of undifferentiated small round-cell sarcoma. It is known to occur very rarely in the kidney. This report presents the first case of a primary renal BCOR::CCNB3 sarcoma in a 22-year-old woman. An 8-cm cystic mass was identified in the left kidney by abdominal pelvic computed tomography. Histopathologic examination revealed the mass to be composed of small round to oval or spindle cells with fibrous septa and a delicate vascular network. A BCOR::CCNB3 fusion was detected by next-generation sequencing–based molecular testing. BCOR::CCNB3 sarcoma presents diagnostic difficulties, highlighting the importance of recognizing its histological features. Immunohistochemical markers are helpful for diagnosis, but genetic molecular testing is necessary for accurate diagnosis. These tumors have a very poor and aggressive prognosis, and an optimal therapeutic regimen has not yet been defined. Therefore, further studies are needed.

BCOR-rearranged sarcoma was classified by the World Health Organization in 2020 as a new subgroup of undifferentiated small round-cell sarcoma. It is known to occur very rarely in the kidney. This report presents the first case of a primary renal BCOR::CCNB3 sarcoma in a 22-year-old woman. An 8-cm cystic mass was identified in the left kidney by abdominal pelvic computed tomography. Histopathologic examination revealed the mass to be composed of small round to oval or spindle cells with fibrous septa and a delicate vascular network. A BCOR::CCNB3 fusion was detected by next-generation sequencing–based molecular testing. BCOR::CCNB3 sarcoma presents diagnostic difficulties, highlighting the importance of recognizing its histological features. Immunohistochemical markers are helpful for diagnosis, but genetic molecular testing is necessary for accurate diagnosis. These tumors have a very poor and aggressive prognosis, and an optimal therapeutic regimen has not yet been defined. Therefore, further studies are needed.

BCOR-rearranged sarcoma was classified by the World Health Organization in 2020 as a new subgroup of undifferentiated small round-cell sarcoma. It is known to occur very rarely in the kidney. This report presents the first case of a primary renal BCOR::CCNB3 sarcoma in a 22-year-old woman. An 8-cm cystic mass was identified in the left kidney by abdominal pelvic computed tomography. Histopathologic examination revealed the mass to be composed of small round to oval or spindle cells with fibrous septa and a delicate vascular network. A BCOR::CCNB3 fusion was detected by next-generation sequencing–based molecular testing. BCOR::CCNB3 sarcoma presents diagnostic difficulties, highlighting the importance of recognizing its histological features. Immunohistochemical markers are helpful for diagnosis, but genetic molecular testing is necessary for accurate diagnosis. These tumors have a very poor and aggressive prognosis, and an optimal therapeutic regimen has not yet been defined. Therefore, further studies are needed.

Undifferentiated pancreatic carcinoma with osteoclast-like giant cells (UC-OGC) is uncommon, accounting for only 1% of all pancreatic carcinomas. We report a case of a 39-year-old man with undifferentiated pancreatic carcinoma with osteoclast-like giant cells who underwent successful surgical resection and chemotherapy.

The use of biomarkers to guide patient and therapy selection has gained much attention to increase the scope and complexity of targeted therapy options and immunotherapy. Clinical trials provide a basis for discovery of biomarkers, which can then aid in development of new drugs. To that end, samples from cancer patients, including DNA, RNA, protein, and the metabolome isolated from cancer tissues and blood or urine, are analyzed in various ways to identify relevant biomarkers. In conjunction with nucleotide-based, high-throughput, next-generation sequencing techniques, therapy-guided biomarker assays relying on protein-based immunohistochemistry play a pivotal role in cancer care. In this review, we discuss the current knowledge regarding DNA and protein biomarkers for cancer immunotherapy

PURPOSE: The efficacy of nivolumab in metastatic renal cell carcinoma (mRCC) has been proven. However, the nivolumab experience in Korean patients with mRCC is still poorly reported. We report initial experiences with the efficacy and safety of nivolumab in patients with mRCC. MATERIALS AND METHODS: We retrospectively reviewed records for 25 patients with mRCC who had failed targeted therapy and were treated by nivolumab (2 mg/kg, every 2 weeks) at a single institution. The primary endpoint was objective response rate (ORR), and secondary endpoints were progression-free survival (PFS), safety profiles, and ORR in a programmed cell death receptor ligand 1 (PD-L1) expression subgroup. RESULTS: The median age was 60 years and 16 patients (64%) were male. Objective responses were achieved in 8 patients (32.0%) (complete response, 1; partial response, 7). Median PFS was 3.0 months (95% confidence interval, 1.46–4.53). Treatment-related adverse events (AEs) of any grade were observed in 19 patients (76.0%) with 6 (24.0%) experiencing grade 3 to 4 treatment-related AEs. In subgroups by PD-L1 expression levels classified as 1% or greater and less than 1%, ORR was 50% and 0%, respectively. CONCLUSIONS: This study showed the efficacy and safety of initial experiences with nivolumab in Korean patients with mRCC who had failed targeted therapy. Our results were comparable to recent clinical trials on nivolumab in mRCC.
Antigens, CD274 ; Carcinoma, Renal Cell ; Cell Death ; Disease-Free Survival ; Humans ; Male ; Neoplasm Metastasis ; Retrospective Studies ; Treatment Outcome

BACKGROUND: Meconium aspiration syndrome (MAS) is defined by respiratory distress requiring supplemental oxygen in a meconium-stained neonate. MAS is clinically subclassified as mild, moderate, and severe according to the oxygen requirement. The aims of this study were to compare the histological findings in the placentas of MAS neonates with those of meconium-stained but non-MAS neonates and to analyze the correlation between the severity of MAS and the grade of its histological parameters. METHODS: We collected 160 singleton term placentas from neonates with meconium staining at birth from a tertiary medical center, Seoul, Republic of Korea. We reviewed hematoxylin and eosin sections of tissue samples (full-thickness placental disc, chorioamniotic membranes, and umbilical cord). RESULTS: Funisitis was present more frequently in MAS than in non-MAS (p < .01), of which the stage was correlated with the severity of MAS (p < .001). The histological findings consistent with maternal underperfusion and chronic deciduitis were more frequent in MAS than in non-MAS (p < .05). There was a correlation between the degree of chorionic vascular muscle necrosis and the severity of MAS (p < .05). CONCLUSIONS: Our results suggest that fetal inflammatory response evidenced by funisitis occurs prenatally in MAS and that the stage of funisitis and of chorionic vascular muscle necrosis may be a predictive marker of the severity of MAS.
Chorioamnionitis ; Chorion ; Eosine Yellowish-(YS) ; Female ; Hematoxylin ; Humans ; Infant, Newborn ; Meconium Aspiration Syndrome* ; Meconium* ; Membranes ; Necrosis ; Oxygen ; Parturition ; Placenta ; Pregnancy ; Republic of Korea ; Seoul

Investigating neonatal deaths in the toilets is challenging for forensic pathologists. During the postmortem examination, they should evaluate whether the baby was alive or a stillbirth and determine any causes of death, such as prenatal cause, infection, anatomical abnormalities, birth or other blunt force injury, drowning, and asphyxia. We retrieved two cases of neonatal deaths in the toilets and reviewed their autopsy findings and circumstances. However, findings from the postmortem examination were insignificant. Their lung examinations revealed non-expanded alveoli, and hydrostatic tests were negative. However, the cases cannot be confirmed as stillbirths because of the possibility that they might be alive for a short period of time after birth and then exposed into the water in the toilet or to accidental or non-accidental asphyxia or that they might have died because of neglect. These cases illustrate that the death scene and the associated circumstances should be meticulously and carefully investigated.
Asphyxia ; Autopsy ; Cause of Death ; Drowning ; Forensic Pathology ; Humans ; Infant, Newborn* ; Lung ; Parturition ; Perinatal Death ; Stillbirth ; Water

PURPOSE: Cell division cycle and apoptosis regulator 1 (CCAR1) plays a dynamic role in regulation of cell growth and apoptosis by serving as a cofactor of steroid/thyroid nuclear receptors, β-catenin, and p53 in a variety of cell types including different cancer cells. However, whether CCAR1 protein is overexpressed in hepatocellular carcinoma (HCC) and the prognostic significance of CCAR1 protein expression in HCC have not been reported. MATERIALS AND METHODS: In 167 HCC patients with long-term follow-up, CCAR1 protein expression was examined by immunohistochemistry. RESULTS: High CCAR1 protein expression was observed in 149 of the 167 HCC cases (89.2%) and showed significant correlation with microvascular invasion, intrahepatic metastasis, higher American Joint Committee on Cancer (AJCC) T stage, and early recurrence. High CCAR1 expression showed an unfavorable effect on recurrence-free survival (RFS) (p=0.002). In subgroup analysis, among patients with α-fetoprotein ≤ 20 ng/mL (n=54) and patients with AJCC T stage 1 (n=62), significant differences in RFS were observed between high CCAR1 expression groups and low CCAR1 expression groups (p=0.015 and p=0.004, respectively). High CCAR1 expression tended to be an independent predictor of shorter RFS (p=0.054) and showed an unfavorable effect on overall survival (OS) (p=0.015). In subgroup analysis, among patients with α-fetoprotein ≤ 20 ng/mL (n=54), significant difference in OS was observed between high CCAR1 expression group and low CCAR1 expression group (p=0.046). CONCLUSION: CCAR1 protein could be a potential biomarker predicting RFS in HCC patients after curative hepatectomy. In addition, CCAR1 had prognostic values in HCC patients with normal serum α-fetoprotein levels or early stage HCC.
Apoptosis ; Carcinoma, Hepatocellular* ; Cell Cycle ; Follow-Up Studies ; Hepatectomy ; Humans ; Immunohistochemistry ; Joints ; Neoplasm Metastasis ; Prognosis* ; Receptors, Cytoplasmic and Nuclear ; Recurrence

IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.
Autoimmune Diseases ; Bile Ducts ; Biliary Tract ; Cholangiocarcinoma ; Cholangitis* ; Fibrosis ; Follow-Up Studies ; Humans ; Lymph Nodes ; Male ; Middle Aged ; Pancreas ; Plasma Cells