Introduction: We present two cases with symptoms of progressively worsening cough, dyspnea, decreased exercise tolerance and right-sided back pain in the first case and upper respiratory symptoms characterized by cough and a low grade fever in the second case. Methods: Report of two cases. Results:The initial chest X-ray in both the cases showed pleural effusion. Further imaging with computed tomography of the chest confirmed the effusion in both cases. Thoracentesis was done in both of them revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the diagnosis of primary pleural epithelioid hemangioendothelioma was made. Conclusions: Both the cases had progressive clinical deterioration despite chemotherapy with Taxol and Bevacizumab in one case and carboplatin, etoposide, and bevacizumab, in the second case. Both developed metastatic disease to lungs and died.Please cite this paper as: Lazarus A, Fuhrer G, Malekiani C, McKay S and Thurber J. Primary pleural epithelioid hemangioendothelioma (EHE) -two cases and review of the literature. Clin Respir J 2011; 5: e1-e5.
Familial adenomatous polyposis (FAP) is a dominantly inherited predisposition to the development of many hundreds to thousands of adenomatous polyps of the colon. The mean age of onset is around 15 years, symptoms may arise in the third decade, and the median age for the development of colonic cancer is 35-40 years. Prophylactic colectomy reduces the risk of death from colorectal cancer to such an extent that late sequelae such as upper gastrointestinal tumours have become the main cause of mortality in appropriately managed patients. The age at which colonic surveillance begins reflects the natural history of the disease. Onset of polyp formation and cancer in childhood is very unusual, but has recently been associated with a specific mutation at codon 1309 in exon 15 where a more severe phenotype is sometimes observed. The case histories of two families are reported in which there is childhood onset of polyps in the youngest generation and in one case a carcinoma, in whom mutations have been identified in exon 11 of the APC gene. Several other aVected relatives were diagnosed at ages ranging from 5-48 years, some already with a cancer at the time of first screening. Since the aim of screening for colonic polyps is prevention of colonic cancer, family members at risk should be oVered genetic assessment and direct mutation testing where this is possible, usually in the early teens. In the absence of a genetic test (the situation in about one third of families) or in a known gene carrier, annual colonoscopy examination is advised from the same age. Clinicians should take note of the family history and be prepared to consider much earlier intervention if symptoms occur in a child with a family history of FAP. Where childhood onset of polyps has occurred, other children at risk in the family must be oVered earlier genetic testing and endoscopic surveillance.
Background: Lung cancer remains the leading cause of cancer deaths in the United States, and lung cancer screening has been shown to decrease this mortality. Adherence to lung cancer screening is paramount to realize the mortality benefit, and reported adherence rates vary widely. Few reports address non-adherence to screening, and our study sought to understand the non-compliant patients in our military population. Methods: This Institutional Review Board approved retrospective review of patients enrolled in our screening program from 2013-2019 identified patients who failed to obtain a subsequent Low Dose CT scan (LDCT) within 15 months of their prior scan. Attempts were made to contact these patients and elucidate motivations for non-adherence via telephone. Results: Of the 242 patients enrolled, 183 (76%) patients were adherent to the protocol. Significant predictors of non-adherence versus adherence were younger age (P=0.008), female sex (P=0.005), and enlisted officer rank (P=0.03). There was no difference with regards to race, smoking status, pack-years, negative screens, lung-RADS level, or nodule size. 31 (52%) non-adherent patients were contacted, and 24 (77%) reported their reason for non-adherence was lack of follow-up for a LDCT. Twenty (64%) were interested in re-enrollment. Of the total screening cohort, 15 interventions were performed, with lung cancer identified in 5 (2%)-a 67% false positive rate. One stage IV lung cancer was found in a non-adherent patient who re-enrolled. Conclusions: Lack of perceived contact for follow-up was expressed as the primary reason for noncompliance in our screening program. Compliance is critical to the efficacy of any screening modality, and adherence rates to lung cancer screening may be increased through improved contact with patients via multiple avenues (i.e., phone, email, and letter). There is benefit in contacting non-adherent patients as high rates of re-enrollment are possible.
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