Insulinomas are rare tumors with an estimated incidence of one per 250,000 person-years. Most insulinomas are benign with less than 10% demonstrating malignant behavior, the vast majority of which occur in adults. A systemic review of the literature revealed only nine cases of malignant insulinomas occurring in children. Herein, we present a case of metastatic malignant insulinoma in a 12-year-old child. The occurrence of this diagnosis in a child, its unusual pattern of metastases and the challenging management of severe hypoglycemia make this case worth reporting.
Thymic carcinoma arising within a thymolipoma has not been reported previously. The authors present a unique case of thymoma and undifferentiated thymic carcinoma arising within a thymolipoma in a 36-year-old woman. The bulk of the resected mass was composed of benign fatty tissue admixed with foci of unremarkable thymic tissue; however, it also harbored a central solid mass showing undifferentiated thymic carcinoma associated with a type B2 thymoma. The carcinoma cells were positive for cytokeratin AE1/AE3, cytokeratin 19, and cytokeratin 8/18. They were negative for vimentin, cytokeratin 7, cytokeratin 20, CD5, epithelial membrane antigen, CD30, placental alkaline phosphatase, carcinoembryonic antigen, CD99, leukocyte common antigen, Epstein-Barr virus, inhibin alpha, and protein gene product 9.5. Rare tumor cells showed positive staining for chromogranin and synaptophysin.
Background: Metaplastic breast cancer (MetBC) represents a therapeutic challenge. We evaluated the impact of clinicopathological characteristics and treatment modalities on outcomes among MetBC patients treated at our center. Methods: Women with stage I-III MetBC were reviewed from our database from 2005-2018. Kaplan-Meier method was used to calculate locoregional-failure-free survival (LRFFS), overall-survival (OS) and distant-metastases-free survival (DMFS). We assessed associations with survival outcomes by log-rank tests. Multivariate Cox proportional-hazards models were used to identify independent predictors of LRFFS, OS and DMFS. Results: 81 patients were eligible for the study. Median age at diagnosis was 48 years. 90.1% had G-III tumors, 64.2% were pathologically node negative and lympho-vascular invasion (LVI) was absent in 72.8%. 67.8% were triple negative, and 7.4% were HER2-neu positive. Most (66.7%) patients underwent mastectomy. Free margins were achieved in the entire cohort, however, 17.3% had close margin (<2 mm). Almost all patients received chemotherapy. 75.3% received radiotherapy, 23.5% received hormonal therapy and 6.2% received Trastuzumab. With a median follow-up of 54 months, 18.5% developed loco-regional recurrence and 34.6% relapsed distally. Five-year OS was 66.0%. On multivariate analysis: adjuvant radiotherapy correlated with better OS ( P < .0001), and tumor size >5 cm, nodal involvement and LVI correlated with worse OS, ( P = .019, P = .021, P = .028, respectively). There were no survival differences with respect to age, triple negativity, and morphologic subtype. Conclusion: We report the largest single institutional series on MetBC in the Middle East region. MetBC confers worse survival outcomes, and more aggressive local and systemic treatment strategies should be investigated.
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