Doege-Potter syndrome associated to metastatic solitary fibrous tumor

ABSTRACT Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

Experience of treatment of prosthetic valve endocarditis: a retrospective single-center cross-sectional study

ABSTRACT BACKGROUND: The aim of this study was to describe the experience of treatment of early prosthetic valve endocarditis at a heart center. DESIGN AND SETTING: Retrospective single-center study on data collected from electronic medical records covering the period from January 2009 to December 2015. METHODS: Over the study period, 1,557 consecutive valve operations were performed on adult patients. The study population comprised 32 patients (2%) who were diagnosed with prosthetic valve endocarditis within 12 months after the index surgery. Medical records were retrieved from electronic hospital records, retrospectively. Descriptive clinical, echocardiographic, microbiological and treatment-type data were used. Risk factors for early mortality were studied through univariate and multivariate analyses. RESULTS: The main clinical manifestation of infective endocarditis was fever, and this was present in all patients. Most of the prostheses were affected in the aortic position (40.6% of cases). The most commonly cultured microorganisms were Staphylococcus epidermidis and Staphylococcus aureus. Twenty-six patients (81.3%) underwent surgical treatment and six (18.7%) underwent exclusive clinical treatment. The prevalence of postoperative complications was 31.3% and hospital mortality occurred in seven cases (21.9%). The mortality rate was 50% among the patients who underwent medical treatment and 15.4% among those who underwent surgery. There were no independent risk factors for mortality. CONCLUSION: Prosthetic valve endocarditis is an infrequent complication of valve replacement. Surgical treatment has mortality rates compatible with the severity of patients' conditions. Surgical indication should not be delayed when clinical treatment has been ineffective

What determines mortality in malignant pheochromocytoma? - Report of a case with eighteen-year survival and review of the literature

SUMMARY Pheochromocytoma (PCC) is a tumor derived from adrenomedullary chromaffin cells. Prognosis of malignant PCC is generally poor due to local recurrence or metastasis. We aim to report a case of malignant PCC with 18-year survival and discuss which factors may be related to mortality and long-term survival in malignant pheochromocytoma. The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors. Diagnosis of PCC was established biochemically and a tumor with infiltration of renal parenchyma was resected. No genetic mutation or copy number variations were identified in SDHB, SDHD, SDHC, MAX and VHL. Over 18 years, tumor progression was managed with 131I-MIBG (iodine-metaiodobenzylguanidine) and 177Lutetium-octreotate therapy. Currently, the patient is asymptomatic and presents sustained stable disease, despite the presence of lung, para-aortic lymph nodes and femoral metastases. Adequate response to treatment with control of tumor progression, absence of significant cardiovascular events and other neoplasms, and lack of mutations in the main predisposing genes reported so far may be factors possibly associated with the prolonged survival in this case. Early diagnosis and life-long follow-up in patients with malignant pheochromocytoma are known to be crucial in improving survival.