Vincristine, Irinotecan, and Temozolomide as a Salvage Regimen for Relapsed or Refractory Sarcoma in Children and Young Adults / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#No standard salvage regimen is available for relapsed or refractory sarcoma. We investigated the efficacy and toxicity of the vincristine, irinotecan, and temozolomide combination (VIT) for relapsed or refractory sarcomas of variable histology in children and young adults. @*Materials and Methods@#We retrospectively reviewed data from the relapsed or refractory sarcoma patients who were treated with VIT. The VIT protocol was given every 3 weeks as follows: vincristine, 1.5 mg/m2 intravenously on day 1, irinotecan, 50 mg/m2/day intravenously on days 1-5, and temozolomide, 100 mg/m2/day orally on days 1-5. @*Results@#A total of 26 patients (12 males) with various sarcoma histology were included in the study. Most common diagnosis was rhabdomyosarcoma (n=8) followed by osteosarcoma (n=7). Median age at the start of VIT was 18.5 years (range, 2.0 to 39.9). VIT was delivered as 2nd to 7th line of treatment, with 4th line most common (9/26, 34.6%). Median number of VIT courses given was 3 (range, 1 to 18). Of the 25 evaluable patients, there was two partial response (PR) and 11 stable disease (SD) with an overall control rate (complete remission+PR+SD) of 52%. PR was seen in one (50%) of the two evaluable patients with Ewing sarcoma and one (14.3%) of the seven patients with osteosarcoma. Overall survival and progression-free survival rates were 79.3% and 33.9% at 1 year, and 45.5% and 25.4% at 2 years, respectively. There was no treatment-related mortality. @*Conclusion@#The VIT regimen was effective and relatively safe in our cohort of sarcoma patients.

Tumors of the Pleura and Lung Developed 17 Years after Allogeneic Bone Marrow Transplantation for Childhood Acute Myelomonocytic Leukemia:Synovial Sarcoma Mimicking Malignant Mesothelioma / 임상소아혈액종양

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

Sequential Treatment with an Immune Checkpoint Inhibitor Followed by a Small-Molecule Targeted Agent Increases Drug-Induced Pneumonitis / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Immune checkpoint inhibitors (ICI) and targeted small-molecule drugs are mainstay elements of lung cancer chemotherapy. However, they are associated with development of pneumonitis, a rare, but potentially life-threatening event. We analyzed lung cancer patients treated with ICI to evaluate the effect of sequential therapeutic administration on the incidence of pneumonitis. @*Materials and Methods@#In this retrospective study, 242 patients were included. Serial radiologic findings taken during and immediately after ICI treatment were reviewed. Factors that increased pneumonitis and the relationship between peri-ICI chemotherapy and the development of pneumonitis were evaluated. @*Results@#Pneumonitis developed in 23 patients (9.5%); severe pneumonitis (grade ≥ 3) occurred in 13 of 23 patients (56%); pneumonitis-related death occurred in six. High-dose thoracic radiation (≥ 6,000 cGy) revealed a tendency toward high risk of pneumonitis (odds ratio, 2.642; 95% confidence interval, 0.932 to 7.490; p=0.068). Among 149 patients followed for ≥ 8 weeks after the final ICI dose, more patients who received targeted agents within 8-weeks post-ICI experienced pneumonitis (3/16, 18.8%) compared with patients who received cytotoxic agents (4/54, 7.4%) or no chemotherapy (4/79, 5.1%) (p=0.162). Targeted therapy was associated with earlier-onset pneumonitis than treatment with cytotoxic agents (35 vs. 62 days post-ICI, p=0.007); the resulting pneumonitis was more severe (grade ≥ 3, 100% vs. 0%, p=0.031). @*Conclusion@#Sequential administration of small-molecule targeted agents immediately after ICI may increase the risk of severe pneumonitis. The sequence of chemotherapy regimens that include ICI and targeted agents should be carefully planned to reduce the risk of pneumonitis in lung cancer patients.

The Clinical Impact of Capmatinib in the Treatment of Advanced Non–Small Cell Lung Cancer with MET Exon 14 Skipping Mutation or Gene Amplification / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Capmatinib, an oral MET kinase inhibitor, has demonstrated its efficacy against non–small cell lung cancer (NSCLC) with MET dysregulation. We investigated its clinical impact in advanced NSCLC with MET exon 14 skipping mutation (METex14) or gene amplification. @*Materials and Methods@#Patients who participated in the screening of a phase II study of capmatinib for advanced NSCLC were enrolled in this study. MET gene copy number (GCN), protein expression, and METex14 were analyzed and the patients’ clinical outcome were retrospectively reviewed. @*Results@#A total of 72 patients were included in this analysis (group A: GCN ≥ 10 or METex14, n=14; group B: others, n=58). Among them, 13 patients were treated with capmatinib (group A, n=8; group B, n=5), and the overall response rate was 50% for group A, and 0% for group B. In all patients, the median overall survival (OS) was 20.2 months (95% confidence interval [CI], 6.9 to not applicable [NA]) for group A, and 11.3 months (95% CI, 8.2 to 20.3) for group B (p=0.457). However, within group A, median OS was 21.5 months (95% CI, 20.8 to NA) for capmatinib-treated, and 7.5 months (95% CI, 3.2 to NA) for capmatinib-untreated patients (p=0.025). Among all capmatinib-untreated patients (n=59), group A showed a trend towards worse OS to group B (median OS, 7.5 months vs. 11.3 months; p=0.123). @*Conclusion@#Our data suggest that capmatinib is a new compelling treatment for NSCLC with MET GCN ≥ 10 or METex14 based on the improved survival within these patients.

Tumors of the Pleura and Lung Developed 17 Years after Allogeneic Bone Marrow Transplantation for Childhood Acute Myelomonocytic Leukemia:Synovial Sarcoma Mimicking Malignant Mesothelioma / 임상소아혈액종양

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

A Rare Case of Phyllodes Tumor Metastasis to the Stomach Presenting as Anemia / Journal of the Korean Cancer Association, 대한암학회지

Metastasis of a phyllodes tumor to the stomach is an extremely rare condition with important clinical implications. A 44-year-old woman was initially diagnosed with a phyllodes tumor in her right breast in 2008, and subsequently presented to an out-patient clinic with dizziness on December 16, 2013. We found that she had severe anemia (hemoglobin levels, 6.7 g/dL), and we quickly performed esophagogastroduodenoscopy to identify the cause. This procedure revealed large ulcerofungating masses with active bleeding in the stomach. Histopathological examination revealed that the masses were consistent with phyllodes tumor metastases. In patients with a metastatic phyllodes tumor presenting as anemia, gastric metastasis should be considered as one of the differential diagnoses because overlooking the possibility might have dire consequences if cytotoxic chemotherapy were administered.

A Rare Case of Phyllodes Tumor Metastasis to the Stomach Presenting as Anemia / Journal of the Korean Cancer Association, 대한암학회지

Metastasis of a phyllodes tumor to the stomach is an extremely rare condition with important clinical implications. A 44-year-old woman was initially diagnosed with a phyllodes tumor in her right breast in 2008, and subsequently presented to an out-patient clinic with dizziness on December 16, 2013. We found that she had severe anemia (hemoglobin levels, 6.7 g/dL), and we quickly performed esophagogastroduodenoscopy to identify the cause. This procedure revealed large ulcerofungating masses with active bleeding in the stomach. Histopathological examination revealed that the masses were consistent with phyllodes tumor metastases. In patients with a metastatic phyllodes tumor presenting as anemia, gastric metastasis should be considered as one of the differential diagnoses because overlooking the possibility might have dire consequences if cytotoxic chemotherapy were administered.

Low Grade Chondrosarcoma Presenting as Progressive Valgus Limb Deformity in a Growing Period / 대한골관절종양학회지

A femoral bone tumor causing a valgus deformity by affecting the growth plate was found. Long intramedullary diaphyseal tumor was separated by septum at the metapysis. Low grade chondrosarcoma was confirmed diagnosed by pathologists. Progressive limb deformity can be a sign of bone tumor in growing period.

Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in an Adolscence, Manifested as Isolated Cervical Mass / 임상소아혈액종양

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PPNET) arises from bone or soft tissue of many sites of body, but ES/PPNET, as primary lesion, in the soft tissue of neck is very uncommon. The authors experienced a case of ES/PPNET in a 17-year-old girl, which was manifested as isolated cervical mass in the soft tissue without any other suspected primary lesion or metastasis. We report the case with a brief review of the literature.

Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in an Adolscence, Manifested as Isolated Cervical Mass / 임상소아혈액종양

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PPNET) arises from bone or soft tissue of many sites of body, but ES/PPNET, as primary lesion, in the soft tissue of neck is very uncommon. The authors experienced a case of ES/PPNET in a 17-year-old girl, which was manifested as isolated cervical mass in the soft tissue without any other suspected primary lesion or metastasis. We report the case with a brief review of the literature.

A Spongiform Mass in the Stomach: Pyloric Gland Adenoma with a Transition to Adenocarcinoma / 대한소화기학회지

No abstract available.

Mucinous Tubular and Spindle Cell Carcinoma of Kidney Occurring in a Patient with Pulmonary Adenocarcinoma

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of kidney tumor that has only been recently described. Furthermore, a case of MTSCC associated with a simultaneous lung cancer in the same patient has never been reported in the literature. In this paper, we describe a kidney tumor that was detected during staging work-up in a 72-year-old lung cancer patient. The kidney tumor was removed and shown to exhibit histological and immunophenotypic features of MTSCC, completely distinct from the pulmonary adenocarcinoma. In addition, this case was unique because it was characterized by neuroendocrine differentiation as well as p53 and Ki-67 overexpression in tumor cells. Therefore, we report a case of MTSCC diagnosed in a patient with pulmonary adenocarcinoma and describe the detailed histologic and immunohistochemical features of MTSCC.

Acinar Cell Cystadenoma of the Pancreas: Report of a Case with Metaplastic Ossification

Acinar cell cystadenoma (ACA) is a very rare cystic lesion of the pancreas. The lining epithelium of ACA is morphologically identical to acinar cells of the pancreas. It is uncertain whether ACA is a benign neoplasm or cystic transformation of acinar glands but it is worthy to consider ACA in the differential diagnosis of other cystic neoplasms of the pancreas. We report here a 25-year old-woman who was operated on for a cystic mass of the pancreas. Grossly, a multilocular cystic mass containing clear serous fluid was observed. There was no communication between the cysts and the pancreatic ducts. Microscopically, cysts of various size were lined by columnar, cuboidal or flattened epithelial cells with a few foci of pseudostratification. The cells had granular apical cytoplasm and basally located nuclei with minimal atypia, the same as normal acinar cells. Metaplastic ossification was noted in the stroma. Immunohistochemically, the lining epithelium was positive for cytokeratin 7, antitrypsin and antichymotrypsin.

Methylation Profiles of CpG Island Loci in Major Types of Human Cancers

Several reports have described aberrant methylation in various types of human cancers. However, the interpretation of methylation frequency in various human cancers has some limitations because of the different materials and methods used for methylation analysis. To gain an insight into the role of DNA hypermethylation in human cancers and allow direct comparison of tissue specific methylation, we generated methylation profiles in 328 human cancers, including 24 breast, 48 colon, 61 stomach, 48 liver, 37 larynx, 24 lung, 40 prostate, and 46 uterine cervical cancer samples by analyzing CpG island hypermethylation of 13 genes using methylation-specific PCR. The mean numbers of methylated genes were 6.5, 4.4, 3.6, 3.4, 3.1, 3.1, 3.1, and 2.1 in gastric, liver, prostate, larynx, colon, lung, uterine cervix, and in breast cancer samples, respectively. The number of genes that were methylated at a frequency of more than 40% in each tumor type ranged from nine (stomach) to one (breast). Generally genes frequently methylated in a specific cancer type differed from those methylated in other cancer types. The findings indicate that aberrant CpG island hypermethylation is a frequent finding in human cancers of various tissue types, and each tissue type has its own distinct methylation pattern.

Twist Expression in Upper Urinary Tract Urothelial Carcinoma Affects Patients Disease Free Survival and is Associated with Tumor Grade

BACKGROUND: Epithelial-mesenchymal transition (EMT) is critical for morphogenesis during embryonic development and is also implicated in the conversion of early-stage tumors into invasive malignancies. Recently, Twist has been identified to play an important role in EMTmediated metastatic progression of several types of human cancer. The present study examined the expression of Twist and evaluated its clinicopathologic significance in urothelial carcinoma of upper urinary tract. METHODS: Immunohistochemical staining for Twist expression was performed on 70 upper urinary tract urothelial carcinomas (UUT-UCs) using tissue microarray. RESULTS: Immunohistochemical staining for Twist was positive in 31/70 cases (44.3%) of UUT-UCs. Twist expression was associated with high-grade and advanced-stage (ISUP grade, p<0.01; stage, p=0.045). The patients with Twist positive-tumors revealed lower disease free survival rate than those with Twist negative-tumors (p<0.01). The overall survival for patients with Twist positive-tumors was slightly worse than the patients with Twist negative- tumors, but the difference was not statistically significant (p=0.12). CONCLUSION: Our results suggest that Twist is a novel marker for advanced UUT-UC.