Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Introduction: Ectopic Cushing`s syndrome (ECS) secondary to neuroendocrine tumours (NETs) of the lung and mediastinum are rarely encountered. In this study, we present our experience in Iran on 15 patients with ECS secondary to lung and mediastinal tumours over a period of 27 years. Material and methods: Since 1985, 15 patients with ECS secondary to lung and mediastinal tumours have been diagnosed and prospectively followed by the endocrinology team of Taleghani Hospital, Tehran, Iran. The clinical signs and symptoms, laboratory findings, radiological features, immunohistochemical characteristics, management strategies and outcome data are here presented. Results: There were six women and nine men, aged 26–70 years, all presenting with typical features of Cushing’s syndrome. Based on histopathologic evaluations, four patients had small cell lung cancer (SCLC) and seven patients had pulmonary NETs, one patient had a thymic NET, and one case was diagnosed as a lung tumourlet. The mean ± SD serum cortisol, 24h urine free cortisol and plasma ACTH were 47.2 ± 20.5 μg/dL, 2,702 ± 5,439 μg/day, and 220 ± 147 pg/mL, respectively. Pulmonary lesions ranged in diameter from 1.1 to 4 cm (mean 1.9 ± 1.1 cm). One patient had a 10 cm mediastinal mass. The duration of follow up in these cases was between one month and seven years (mean 29.9 ± 27.5 months). The four patients with SCLC died within three months of diagnosis. Conclusion: Our data demonstrates the protean clinical and laboratory manifestations of ECS secondary to lung and mediastinal tumours, the problems encountered in diagnosis, and the need for a multidisciplinary approach. This study confirms other series from Western Europe and North America that, unlike the SCLC patients who show a poor outlook, ECS secondary to lung carcinoids has a more favourable prognosis. Wstep: Ektopowy zespół Cushinga (ectopic Cushing`s syndrome, ECS), który rozwija się u pacjentów mających nowotwory neuroendokrynne (neuroendocrine tumours, NET) śródpiersia lub płuc, jest zaburzeniem rzadko spotykanym. Niniejsza publikacja przedstawia doświadczenia autorów z ośrodka w Iranie, którzy w okresie 27 lat prowadzili 15 pacjentów z zespołem Cushinga wtórnym do nowotworów śródpiersia lub płuc. Materiał i metody: W okresie od 1985 zespół endokrynologów ze szpitala Taleghani Hospital w Teheranie zidentyfikował i prowadził w sposób prospektywny 15 pacjentów z objawami ECS uwarunkowanymi obecnością nowotworów śródpiersia lub płuc. Niniejsza publikacja przedstawia obraz kliniczny i objawy podmiotowe, wyniki badań laboratoryjnych i radiologicznych oraz badań immunohistochemicznych materiału tkankowegoz guzów nowotworowych, a także informacje na temat wdrożonego leczenia i jego wyników. Wyniki: W badanej grupie znalazło się 6 kobiet i 9 mężczyzn, w wieku 26–70 lat. U wszystkich pacjentów stwierdzono typowe cechy kliniczne zespołu Cushinga. Na podstawie badań histopatologicznych wycinków z guzów nowotworowych postawiono rozpoznanie drobnokomórkowego raka płuca (SCLC, small cell lung cancer) u 4 pacjentów, neuroendokrynnego nowotworu płuca (NET) bliżej nieokreślonego typu u 7 pacjentów, NET grasicy u jednej osoby oraz rozpoznanie zmiany typu tumorlet w płucu u jednej osoby. Średnie stężenie kortyzolu w surowicy ( ± odchylenie standardowe) wyniosło 47,2 ± 20,5 μg/dl, stężenie wolnego kortyzolu w moczu ze zbiórki dobowej 2.702 ± 5.439 μg/dobę, a stężenie ACTH w osoczu 220 ± 147 pg/ml. Zmiany nowotworowe w płucach miały wielkość od 1,1 do 4 cm (średni wymiar guza 1,9 ± 1,1 cm). Guz śródpiersia (u jednej osoby) miał średnicę 10 cm. Czas obserwacji wyniósł od 1 miesiąca do 7 lat (średnia 29,9 ± 27,5 miesięcy). Czterech chorych na SCLC zmarło w okresie do 3 miesięcy od postawienia rozpoznania. Wnioski: Przedstawione dane wskazują na różnorodność obrazu klinicznego i danych laboratoryjnych u pacjentów z objawami ECS w przebiegu choroby nowotworowej śródpiersia lub płuc. Konstelacja ta wymaga więc wielospecjalistycznej opieki już od momentu wdrożenia postępowania diagnostycznego. Przedstawione wyniki są zbieżne z danymi z publikacji pochodzących z Europy Zachodniej i Stanów Zjednoczonych i wskazują na stosunkowo korzystne rokowanie u pacjentów mających objawy ECS w związku z rakowiakiem płuca, w przeciwieństwie do pacjentów mających zmiany typu SCLC

Post-Intubation Tracheoesophageal Fistula; A Nine-Year Experience

Introduction: Tracheoesophageal fistula (TEF) is a rare condition, which could be life-threatening if diagnosed late or mismanaged. Post-intubation TEF is the most common form of acquired, non-malignant TEF and is usually associated with tracheal stenosis, which makes the treatment more challenging. Here, we present our experience of managing 21 patients with post-intubation TEF.   Materials & Methods: Twenty one patients including seven women and fourteen men with mean age of 38.05 years, who had post-intubation TEF were managed in our center (Massih Daneshvari Hospital, Tehran, Iran) during 2004-2013. None of the patients were operated before weaning from mechanical ventilation. Single division and closure of the fistula was performed in one patient who did not have accompanying tracheal stenosis. One-stage surgical repair including tracheal resection, anastomosis, primary closure of the esophageal defect, and muscle flap Interposition was the main treatment method in all other cases. Patients were followed up for at least two years. Results: Excellent and good results achieved in 85.7% of our patients. Major complications including permanent vocal cord paralysis and recurrence of tracheal stenosis necessitating T-tube insertion occurred in two patients (9.5%). Severe cachexia and sepsis secondary to sputum retention resulted in one mortality (4.8%). Conclusion: Surgery might provide the best treatment results along with low mortality and morbidity rates in post-intubation TEFs if performed within the proper time

Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome

Abstract Background STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. Method Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. Result Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. Conclusion Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies