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

Hypercalcemia and huge splenomegaly presenting in an elderly patient with B-cell non-Hodgkin's lymphoma: a case report

<p>Abstract</p> <p>Introduction</p> <p>Hypercalcemia is the major electrolyte abnormality in patients with malignant tumors. It can be due to localized osteolytic hypercalcemia or elaboration of humoral substances such as parathyroid hormone-related protein from tumoral cells. In hematological malignancies, a third mechanism of uncontrolled synthesis and secretion of 1-25(OH)₂D₃from tumoral cells or neighboring macrophages may contribute to the problem. However, hypercalcemia is quite unusual in patients with B-cell non-Hodgkin's lymphoma.</p> <p>Case presentation</p> <p>An 85-year-old Caucasian woman presented with low grade fever, anorexia, abdominal discomfort and fullness in her left abdomen for the last six months. She was mildly anemic and complained of fatigability. She had huge splenomegaly and was hypercalcemic. After correction of her hypercalcemia, she had a splenectomy. Microscopic evaluation revealed a malignant lymphoma. Her immunohistochemistry was positive for leukocyte common antigen, CD20 and parathyroid hormone-related peptide.</p> <p>Conclusion</p> <p>Immunopositivity for parathyroid hormone-related peptide clearly demonstrates that hypersecretion of a parathyroid hormone-like substance from the tumor had led to hypercalcemia in this case. High serum calcium is seen in only seven to eight percent of patients with B-cell non-Hodgkin's lymphoma, apparently due to different mechanisms. Evaluation of serum parathyroid hormone-related protein and 1-25(OH)₂D₃can be helpful in diagnosis and management. It should be noted that presentation with hypercalcemia has a serious impact on prognosis and survival.</p

Abdominal Inflammatory Myofibroblastic Tumor: Report on Four Cases and Review of Literature

Background:The Abdominal Inflammatory Myofibroblastic Tumor (AIMT) is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT. Cases Presentation: We herein present four cases of AIMT in different ages (range: 3.5 to 13 years) and in different organs (stomach, periduodenal, mesenteric, and colon). There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years. Conclusion: As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up

Postpartum Hypercalcemia Secondary to a Neuroendocrine Tumor of Pancreas; a Case Report and Review of Literature

Parathyroid hormone-related protein producing pancreatic neuroendocrine tumors have been infrequently reported. Herein, we report a case of an Iranian woman who had such a tumor during pregnancy, and gave birth to a female neonate with esophago-tracheal fistula and imperforated anus. Hypercalcemia was diagnosed at postpartum because of elevated serum calcium levels in the neonate and neurologic deterioration of the mother. Extensive literature review revealed 42 cases with pancreatic neuroendocrine tumors and hypercalcaemia. The clinical and laboratory findings of such patients are reviewed in this manuscript.<

Beyond Spirometry: Linking Wasted Ventilation to Exertional Dyspnea in the Initial Stages of COPD

AbstractExertional dyspnea, a key complaint of patients with chronic obstructive pulmonary disease (COPD), ultimately reflects an increased inspiratory neural drive to breathe. In non-hypoxemic patients with largely preserved lung mechanics – as those in the initial stages of the disease – the heightened inspiratory neural drive is strongly associated with an exaggerated ventilatory response to metabolic demand. Several lines of evidence indicate that the so-called excess ventilation (high ventilation-CO2 output relationship) primarily reflects poor gas exchange efficiency, namely increased physiological dead space. Pulmonary function tests estimating the extension of the wasted ventilation and selected cardiopulmonary exercise testing variables can, therefore, shed unique light on the genesis of patients’ out-of-proportion dyspnea. After a succinct overview of the basis of gas exchange efficiency in health and inefficiency in COPD, we discuss how wasted ventilation translates into exertional dyspnea in individual patients. We then outline what is currently known about the structural basis of wasted ventilation in “minor/trivial” COPD vis-à-vis the contribution of emphysema versus a potential impairment in lung perfusion across non-emphysematous lung. After summarizing some unanswered questions on the field, we propose that functional imaging be amalgamated with pulmonary function tests beyond spirometry to improve our understanding of this deeply neglected cause of exertional dyspnea. Advances in the field will depend on our ability to develop robust platforms for deeply phenotyping (structurally and functionally), the dyspneic patients showing unordinary high wasted ventilation despite relatively preserved FEV1