After cisplatin-based chemotherapy became the standard treatment for metastatic urothelial cancer (mUC), very little progress has been made in the treatment landscape of this condition until recently. With increased knowledge about the molecular biology of mUC and advances in the field of cancer immunobiology, there has been an explosion in the number of clinical trials for mUC, and systemic treatment of mUC is rapidly changing. Despite the availability of several novel therapeutic agents, cisplatin-based cytotoxic chemotherapy remains the standard, first-line treatment option. Immune checkpoint inhibitors (ICIs), including programmed death-1 and programmed death ligand-1 inhibitors, are preferred second-line treatment options that are also used in first-line cisplatin-ineligible settings. For patients with actionable fibroblast growth factor receptor 2 (FGFR2) or FGFR3 genomic alterations, erdafitinib can be considered after platinum-based treatment. Enfortumab vedotin, a monoclonal antibody targeting nectin-4 conjugated to monomethyl auristatin E, has been approved for patients who do not respond to both cytotoxic chemotherapy and ICIs. In this review, we address the clinical trial data that have established the current standard treatments and ongoing clinical trials of various agents with different mechanisms as well as provide a brief overview of current practice guidelines and recommendations in patients with mUC.

PURPOSE: The incidence of gastrointestinal stromal tumors (GISTs) harboring platelet-derived growth factor receptor alpha (PDGFRA) mutations is low, therefore further investigation of the efficacy of imatinib in this subgroup was needed. MATERIALS AND METHODS: Patients with PDGFRA-mutant GISTs who received imatinib as primary therapy for advanced disease between January 2000 and June 2012 were identified from the GIST registry of Asan Medical Center, Seoul, Korea. RESULTS: KIT and PDGFRA genotyping in 823 patients identified 18 patients (2%) with PDGFRA mutations who were treated with first-line imatinib. Exon 18 D842V substitution, non-D842V exon 18 mutations, and exon 12 mutations were detected in nine (50%), four (22%), and five (28%) patients, respectively. Objective response rate differed significantly between patients with the D842V mutation and those with non-D842V mutations (0% [0/5] vs. 71% [5/7], p=0.03). In all patients, median progression-free survival (PFS) and overall survival (OS) was 24.8 months (95% confidence interval [CI], 0.0 to 57.2) and 51.2 months (95% CI, 37.1 to 65.3), respectively. Significantly, poorer PFS was observed for patients with D842V-mutant GISTs than those with non-D842V PDGFRA-mutant GISTs: median 3.8 months (95% CI, 1.4 to 6.3) versus 29.5 months (95% CI, 18.3 to 40.7) (p < 0.001). Patients with the D842V mutation had poorer OS than those with non-D842V PDGFRA mutations: median 25.2 months (95% CI, 12.7 to 37.8) versus 59.8 months (95% CI, 43.0 to 76.5) (p=0.02). CONCLUSION: Imatinib is active against non-D842V PDGFRA-mutant GISTs, whereas GISTs harboring the D842V mutation are primarily resistant to imatinib.
Chungcheongnam-do ; Disease-Free Survival ; Exons ; Gastrointestinal Stromal Tumors ; Humans ; Incidence ; Korea ; Platelet-Derived Growth Factor* ; Receptors, Platelet-Derived Growth Factor* ; Seoul

OBJECTIVE: Efficacy or long-term result of metastasectomy for recurrent or metastatic biliary tract carcinoma (BTC) is not well established. We conducted a retrospective review of the outcomes of metastasectomy for recurrent or metastatic BTCs. METHODS: The clinicopathological features and outcomes of consecutive patients with BTCs who underwent surgical resection for primary and metastatic disease at a tertiary referral hospital from 2003 to 2013 were reviewed retrospectively. RESULTS: We found 19 eligible patients. Median age of patients was 57 years old (range, 27 to 68 years old), and 11 patients (58%) were female. Primary sites were gallbladder cancer (seven patients, 37%), intrahepatic cholangiocarcinoma (five patients, 26%), distal common bile duct cancer (three patients, 16%), proximal common bile duct cancer (two patients, 11%), and ampulla of Vater cancer (two patients, 11%). Eight patients (42%) had synchronous metastasis, while 11 (58%) had metachronous metastasis. The most common metastatic site was liver (nine patients, 47%), lymph node (nine patients, 47%), and peritoneum (three patients, 16%). Nine patients (47%) achieved R0 resection, while four (21%) and six (32%) patients had R1 and R2 resection, respectively. With a median follow-up period of 26.7 months, the estimated median overall survival (OS) was 18.2 months (95% confidence interval [CI], 13.6 to 22.9 months). Lower Eastern Cooperative Oncology Group performance status (P=0.023), metachronous metastasis (P=0.04), absence of lymph node metastasis (P=0.009), lower numbers of metastatic organs (P<0.001), normal postoperative carbohydrate antigen 19-9 level (P=0.034), and time from diagnosis to metastasectomy more than one year (P=0.019) were identified as prognostic factors for a longer OS after metastasectomy. CONCLUSION: For recurrent or metastatic BTCs, metastasectomy can be a viable option for selected patients.
Ampulla of Vater ; Biliary Tract Neoplasms* ; Biliary Tract* ; Cholangiocarcinoma ; Common Bile Duct ; Diagnosis ; Female ; Follow-Up Studies ; Gallbladder Neoplasms ; Humans ; Liver ; Lymph Nodes ; Metastasectomy* ; Neoplasm Metastasis ; Peritoneum ; Prognosis ; Retrospective Studies ; Tertiary Care Centers

Gastrointestinal stromal tumors (GISTs) are rare, but are the most common mesenchymal neoplasm of the gastrointestinal tract. The most common sites of metastasis are liver and peritoneum, while bone metastasis is rare. We report on a patient with skull metastasis after seven years of treatment with imatinib for metastatic GIST. She underwent metastasectomy consisting of craniectomy with excision of the mass, and cranioplasty and continued treatment with imatinib and sunitinib, without evidence of cranial recurrence. She died of pneumonia sepsis one year after metastasectomy. Skull metastasis of GIST is a very rare presentation, and an aggressive multidisciplinary approach should be considered whenever possible.
Gastrointestinal Stromal Tumors* ; Gastrointestinal Tract ; Humans ; Imatinib Mesylate ; Liver ; Metastasectomy ; Neoplasm Metastasis* ; Peritoneum ; Pneumonia ; Recurrence ; Sepsis ; Skull*

raumatic shoulder dislocations are one of the most common major dislocations in the general population. Injury to major vessels is rarely reported as a complication of shoulder dislocations. This case report presents the traumatic dissection of the axillary artery after a simple shoulder dislocation that was managed successfully with the placement of a self-expanding stent. With the clinical manifestations of a brachial plexus injury and progressive vascular compromise in the affected arm, a major vascular injury was detected on an angiogram, and a self-expanding stent was deployed. Through immediate diagnosis and prompt intervention, serious complications, such as hypovolemic shock and even death, were averted, ultimately achieving a favorable patient outcome.

BACKGROUND: Primary testicular diffuse large B-cell lymphoma (DLBCL) is a rare but aggressive extranodal lymphoma, and its relapse in the central nervous system (CNS) is a major concern during treatment. Despite this, the role of intrathecal prophylaxis in primary testicular DLBCL remains controversial. METHODS: We retrospectively reviewed the medical records of 14 patients with primary testicular DLBCL diagnosed between November 2000 and June 2012, and analyzed the CNS relapse rate in patients treated without intrathecal prophylaxis. Survival curves were estimated using the Kaplan-Meier method. RESULTS: The median age at diagnosis was 57 years (range, 41-79 years). Unilateral testicular involvement was observed in 13 patients. Nine patients had stage I, 1 had stage II, and 4 had stage IV disease. The international prognostic index was low or low-intermediate risk in 12 patients and high-intermediate risk in 2 patients. Thirteen patients underwent orchiectomy. All the patients received systemic chemotherapy without intrathecal prophylaxis, and prophylactic radiotherapy was administered to the contralateral testis in 12 patients. The median follow-up period of surviving patients was 39 months (range, 10-139 months). Median overall survival was not reached and the median progression-free survival was 3.8 years. Four patients experienced relapse, but CNS relapse was observed in only one patient (7.1%) with stage IV disease, 27 months after a complete response. CONCLUSION: Even without intrathecal prophylaxis, the rate of relapse in the CNS was lower in the Korean patients with primary testicular DLBCL compared to prior reports.
Central Nervous System ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Follow-Up Studies ; Humans ; Lymphoma ; Lymphoma, B-Cell* ; Medical Records ; Orchiectomy ; Radiotherapy ; Recurrence ; Retrospective Studies ; Testis

Splenosis refers to the heterotropic autotransplantation of splenic tissue. Sometimes splenosis after surgical resection is difficult to differentiate from recurrence or metastasis of cancer. A 49-year-old male patient was diagnosed with clear cell renal cell carcinoma of left kidney. As there was no evidence of metastasis, he underwent radical nephrectomy with splenectomy. On surveillance computed tomography, masses at nephrectomy site and pleura were found and both were initially considered to be recurrence. After several cycle of pazopanib administration, pleural mass decreased in size while mass at nephrectomy site did not respond at all. Spleen scan showed increased uptake of the mass and therefore the mass was revealed to be splenosis. To avoid unnecessary treatment and planning optimal treatment, considering the possibility of splenosis is important and spleen scan can be helpful.
Autografts ; Carcinoma, Renal Cell* ; Humans ; Kidney ; Male ; Middle Aged ; Neoplasm Metastasis ; Nephrectomy* ; Pleura ; Radionuclide Imaging ; Recurrence* ; Spleen ; Splenectomy ; Splenosis* ; Transplantation, Autologous

The role of metastasectomy for recurrent disease in patients with adenoid cystic carcinoma (ACC) is not defined clearly yet. A 52-year-old woman found two hepatic metastatic nodules 3 years after the completion of treatment for primary ACC of the trachea. After confirming the absence of other lesions, metastasectomy was performed on the two metastatic nodules. Regular follow-up for more than 24 months showed no evidence of recurrent disease after the hepatic metastasectomy. Therefore, we suggest metastasectomy as an option for certain cases of metastatic ACC.
Adenoids ; Carcinoma, Adenoid Cystic ; Female ; Follow-Up Studies ; Humans ; Metastasectomy ; Middle Aged ; Neoplasm Metastasis ; Trachea

Purpose: Evidence in favor of adding docetaxel in treatment of metastatic hormone-sensitive prostate cancer (mHSPC) has led to docetaxel in conjunction with androgen deprivation therapy (ADT) as standard therapy. The aim of this study was to examine the effectiveness of docetaxel with ADT for Korean patients with mHSPC in real-world practice. Materials and Methods: A retrospective cohort study was performed at six Korean hospitals for patients with mHSPC treated with docetaxel plus ADT. Patients were treated every 3 weeks for up to six cycles with 75 mg/m 2 of docetaxel. The primary endpoint was time to castration resistant prostate cancer (CRPC). Results: This study included 46 eligible patients from June 2016 to February 2021. Median age was 68.5 years (range, 52–84) and all patients present with de novo M1 with high-volume disease. The median prostate-specific antigen (PSA) level at ADT initiation was 205.4 (7.7–1933) ng/mL, and time from ADT to docetaxel was 2.4 months (0–5.3). All six planned cycles of docetaxel were delivered in 36 patients (78%), 7 patients (15%) discontinued treatment due to adverse events, and 3 patients (7%) discontinued due to progression. At the time of the analysis, CRPC had developed in 34 patients (74%), and the median time to CRPC was 18.0 (95% confidence interval, 14.1–21.9) months. PSA <0.2 ng/mL was achieved in 11 patients (24%) after 6 months of ADT and in 10 patients (22%) after 12 months. At last follow-up, 35 patients (76%) were alive; the median overall survival was not reached. Conclusion The effect of docetaxel combined with ADT for Korean patients with mHSPC is comparable with prior results in Western studies.

Background: Hereditary leiomyomatosis and renal cell cancer (HLRCC) is an autosomal dominant cancer predisposition syndrome. HLRCC is characterized by the development of cutaneous leiomyomas, early-onset uterine leiomyomas, and HLRCC-associated renal cell cancer (RCC) and caused by germline fumarate hydratase (FH) deficiency. We investigated the genotypic and phenotypic characteristics of Korean patients with HLRCC. Methods: We performed direct sequencing analysis of FH in 13 patients with suspected HLRCC and their family members. A chromosomal microarray test was performed in female patients with negative sequencing results but highly suspected HLRCC. In addition, we analyzed the clinical characteristics and evaluated the genotype–phenotype correlations in Korean patients with HLRCC. Results: We identified six different pathogenic or likely pathogenic FH variants in six of the 13 patients (46.2%). The variants included two nonsense variants, two splicing variants, one frameshift variant, and one missense variant. Of the six variants, two (33.3%) were novel (c.132+1G > C, and c.243dup). RCC and early-onset uterine leiomyoma were frequently observed in families with HLRCC, while cutaneous leiomyoma was less common. No significant genotype–phenotype correlation was observed. Conclusions We describe the genotypic and phenotypic spectrum in a small series of Korean patients with HLRCC. Our data reveal the unique characteristics of Korean patients with HLRCC and suggest a need for establishing an optimal diagnostic approach for them.