RESUMO
Diffuse idiopathic pulmonary neuroendocrine cell (DIPNECH syndrome) remains unfamiliar to most clinicians even though it was first described almost 30 years ago. Diagnosis is usually confirmed histopathologically after lung biopsy, but often, a diagnosis or suspected diagnosis can be made radiographically. In this paper, we present a case report of a 68-year-old female with shortness of breath and fleeting pulmonary nodules observed on chest CT scan. She was initially misdiagnosed with asthma based on an abnormal pulmonary function test which revealed an obstructive ventilatory defect. The classic radiographic findings of DIPNECH syndrome and the typical patient demographics that should arouse suspicion of a DIPNECH diagnosis were also illustrated. DIPNECH syndrome is a clinicopathological syndrome whereas focal NECH is a pathological diagnosis that is often made incidentally on histological examination and is encountered in a variety of settings, including in resected carcinoid tumors, in the context of reactive changes concomitant with infection, in metastatic cancer, radiation pneumonitis, intra-lobar sequestration, smokers, interstitial lung disease, and lung adenocarcinoma. There are no proven treatments for DIPNECH syndrome. In patients with obstructive ventilatory symptoms, bronchodilators with inhaled steroids are usually prescribed. Some severe cases may require parenteral steroids. Somatostatin analogs (SSA) have also been used in some cases with mixed results. Rapamycin has been used in several cases based on the purported activation of the mammalian target of rapamycin (mTOR) in DIPNECH. Some patients with large carcinoid tumors may benefit from resection.
RESUMO
Detection of Epstein-Barr virus (EBV) may be achieved by various methods, including EBV-encoded RNA (EBER) in-situ hybridization (ISH) and immunohistochemistry (IHC) for latent membrane protein (LMP-1). We compared novel automated ISH and IHC techniques in pediatric lymphoproliferative disorders with results obtained by manual ISH. Thirty-seven pediatric cases previously studied by manual EBER ISH (including 18 EBER-positive, 15 EBER-negative, and 4 EBER-equivocal cases) were used for the study. Automated EBER ISH and automated LMP-1 IHC were performed using the BondMax autostainer and prediluted EBER probe and EBV cell surface 1 to 4 at 1:50 dilution, respectively. Results of each of the automated techniques for EBV detection were compared with results by manual EBER ISH. Compared with manual EBER ISH as the gold standard, automated ISH had a sensitivity and specificity of 94% and 69%, respectively, accuracy of 83%, positive predictive value (PPV) of 79%, and negative predictive value (NPV) of 90%. Automated IHC had a sensitivity of 44%, specificity of 93%, accuracy of 67%, PPV of 88%, and NPV of 59%. Automated ISH and IHC correlated significantly (P < 0.045). Automated ISH is useful for diagnosis of EBV-related pediatric neoplasms, being easy to perform and interpret and requiring only the technologist's time to set up and having a high sensitivity and NPV The automated IHC protocol is of too low sensitivity for routine use, although results show high specificity and PPV.
