Rhabdoid Tumor of the Kidney Is a Component of the Rhabdoid Predisposition Syndrome 

The rhabdoid predisposition syndrome (RPS) is characterized by pedigrees in which two or more individuals carry germline mutations of the hSNF5/INI1 tumor suppressor gene. The tumors associated with the syndrome include atypical teratoid/rhabdoid tumor (AT/RT), choroid plexus carcinoma, medulloblastoma, and extrarenal rhabdoid tumor. Rhabdoid tumor of the kidney (RTK) has not been described as part of the RPS. We report a case of a 7-month-old boy with RTK whose sister had a malignant cerebellar tumor followed by a malignant lung and pleural tumor of childhood with typical rhabdoid histology. Molecular genetic analysis of the RTK and tissue from the pleural tumor revealed in both cases identical nonsense mutations of the hSNF5/INI1 gene on chromosome 22q11.2, where thymidine was substituted for cytosine in base 472. The proband had an identical germline mutation. This is the fifth genetically analyzed RPS pedigree and the first to include an RTK.

title: Test and teach. Number fifty-three. Diagnosis: Calcifying fibrous pseudotumour.

title: Fibrosarcoma of the ovary arising in a fibrothecomatous tumor with minor sex cord elements. A Case report and review of the literature.
abstract: We report the case of a 69-year-old woman who presented with postmenopausal bleeding. Endometrial curettings showed complex atypical hyperplasia with focal well-differentiated adenocarcinoma. A computed tomographic scan of the abdomen revealed a right ovarian mass. Histologically, the right ovarian tumor was a fibrothecoma with minor sex cord elements showing focal fibrosarcomatous change. Fibrosarcoma of the ovary is a rare tumor that is considered to arise de novo or secondary to benign fibromatous tumors. Fibrothecoma of the ovary with minor sex cord elements is also a rare entity. To the best of our knowledge, this is the first reported case of a fibrosarcoma arising in a fibrothecoma with minor sex cord elements.

title: Primary acinic cell carcinoma of the lung with lymph node metastasis.
abstract: We report the case of a 30-year-old woman who presented with a subpleural mass in the lower lobe of the right lung, which was found incidentally on a routine chest radiograph. A wedge biopsy followed by lobectomy and lymph node staging revealed an acinic cell carcinoma with involvement of a hilar lymph node. The subcarinal and mediastinal lymph nodes were free of tumor. Perineural invasion was identified at the periphery of the tumor. There was no evidence of origin from salivary gland or involvement of any other organ. The patient was well 12 months postoperatively. To our knowledge, this is the second case report featuring lymph node metastasis in an acinic cell carcinoma of the lung and the first to demonstrate perineural invasion.

title: One-pot three-component tandem metathesis/Diels-Alder reaction.
abstract: [reaction: see text] A tandem enyne, diene-ene metathesis reaction followed by Diels-Alder reaction accomplished a stereoselective three-component reaction protocol with four stereocenters.

title: Mixed-type paratesticular rhabdomyosarcoma--a case report.
abstract: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood; about 80% of cases occur before the age of 21 with the remaining 20% evenly spread throughout the remaining decades. A primary paratesticular site is considered to have a good prognosis in comparison with other RMS sites. Histologically, any subtype of RMS, including alveolar, pleomorphic, embryonal, and mixed type, may occur in the paratesticular region, but only a relatively small number of cases are mixed and this variant has a poor prognosis. We report a case of paratesticular RMS (mixed embryonal and alveolar type) in a 16-year-old boy.

title: A stereoselective enyne cross metathesis.
abstract: [reaction: see text] Intermolecular enyne metathesis reaction of alkynes with olefins catalyzed by second-generation Grubbs catalyst (1) proceeded stereoselectively under ethylene atmosphere to produce 1,3-disubstituted butadienes with E stereochemistry.

title: Volume-Based Parameter of 18F-FDG PET/CT in Malignant Pleural Mesothelioma: Prediction of Therapeutic Response and Prognostic Implications
abstract: BackgroundTo evaluate the importance of assessing volume-based parameter of 18F-FDG PET/CT in patients with malignant pleural mesothelioma (MPM) for the prediction of response and patient outcome early in the course of treatment.Materials and MethodsPatients (n = 13; M:F = 9:4, mean age, 54 years) with histopathologically proven MPM, all of whom were scheduled to undergo curative extrapleural pneumonectomy (EPP) or palliative chemotherapy, were included in this study. They were evaluated using integrated 18F-FDG PET/CT at baseline. Maximum standardized uptake value (SUVmax), average SUV (SUVavg), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) were measured using PET/CT data. Relationships between tumor progression and PET parameters were statistically analyzed.ResultsOf the 13 patients, 8 (62%) developed disease recurrence after surgery or tumor progression after chemotherapy (median follow-up, 329 days; range, 28–536 days). Between subgroups with and without tumor progression, significant differences were noted in MTV (P = 0.045). On ROC curve analysis, MTV (AUC = 0.850, 95% confidence interval [95% CI] 0.550–0.977) and TLG (AUC = 0.800, 95% CI 0.494–0.960) showed good predictive performance for tumor progression. Multivariate analysis adjusted for treatment modality showed that MTV (HR 1.003, P = 0.025) and TLG (HR 1.001, P = 0.031) were independent factors associated with tumor progression. Time to tumor progression was shorter in patients with a high volume-based parameter of PET than in those with a low value.ConclusionsVolume-based parameters of 18F-FDG PET/CT have the potential to provide prognostic information in MPM patients who are receiving surgery or palliative chemotherapy.

title: New CT response criteria in non-small cell lung cancer: proposal and application in EGFR tyrosine kinase inhibitor therapy.
abstract: We aimed to devise new CT response criteria (new response criteria, NRC) in patients with non-small cell lung cancer (NSCLC) and to evaluate the efficacy of the criteria for stratifying patient responses and predicting patient survival compared to that of the traditional size-based criteria RECIST version 1.1. Our institutional review board approved this study with a waiver of informed consent. We enrolled 80 NSCLC patients as an experimental arm and treated them with epidermal growth factor receptor tyrosine kinase inhibitors (EGFR TKIs). Two blinded, independent radiologists assessed CT images for tumor response using the NRC, which were also validated in a separate arm (75 NSCLC patients). Tumor responses evaluated by RECIST 1.1 and the new criteria were compared from each other and correlated with patient survival. For statistical analyses, Kaplan-Meier method and kappa statistics were used. In the experimental arm (n=80), interobserver agreements for the assessment of patient response were excellent for both RECIST and NRC. Sixteen RECIST nonresponding patients achieved a designation of partial response according to NRC. In the validation arm (n=75), patients of optimal response (partial response) with the new criteria had median overall survival of 18.4 months compared with 8.5 months in patients with poor response (P=.04). However, RECIST failed to show survival difference between the two response groups. In NSCLC patients treated with EGFR-TKIs, new CT criteria reflecting additional morphological characteristics of target lesions are reproducible and have statistically significant association with overall survival.