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OncoReview
|
2014
|
vol. 4
|
issue 1
A18-25
EN
Pheochromocytoma and paraganglioma derive from chromaffin tissue that secretes catecholamines, which determine the characteristic clinical picture of the disease. Serious and potentially fatal cardiovascular complications require rapid diagnosis and treatment. The available biochemical tests are recommended not only to patients with characteristic clinical symptoms, but also to all patients with incidentally identified adrenal tumours. There is a growing incidence of diagnosed germline mutations associated with familial pheochromocytoma and paraganglioma in tumours originally identified as the sporadic form, and therefore current recommendations ever more often indicate the need for genetic testing in all patients with diagnosed pheochromocytoma or paraganglioma. Surgical treatment, which is the treatment of choice, brings very good results in the case of benign tumours, and even the malignant ones, provided they are operational. Unfortunately, there is no sufficiently good and efficacious alternative treatment for patients with unresectable or metastatic malignant tumours.
|
2019
|
vol. 1
|
issue 2
72-76
EN
Hereditary hemochromatosis (HH) is a rare genetic disorder, developing secondary to the accumulation of iron in tissues, which may lead to multiple organ failure. If untreated, it may result in liver cirrhosis or cardiomyopathy. The damage to the pancreas and the anterior pituitary, on the other hand, leads to a decreased production and secretion of hormones that are essential to life. Common symptoms of HH, that are distressing for patients, include joint pain, particularly involving hands and wrists, as well as the chronic fatigue syndrome. Iron overload affects the skeletal system, leading to osteoporosis. The pathological accumulation of iron in the anterior pituitary impairs the gonadotropin synthesis, resulting in reduced serum levels of testosterone in men and estrogens in women. This, however, contributes to lower bone mass. In vivo tests have also revealed that abnormal iron accumulation is related to an increased activity and number of osteoclasts, as well as the influence on the differentiation and activity of osteoblast-lineage cells. Based on a systematic review of literature, hereditary hemochromatosis (HH) will be presented as a chronic disease, affecting most of the endocrine glands.
EN
Hereditary hemochromatosis (HH) is a rare genetic disorder, developing secondary to the accumulation of iron in tissues, which may lead to multiple organ failure. If untreated, it may result in liver cirrhosis or cardiomyopathy. The damage to the pancreas and the anterior pituitary, on the other hand, leads to a decreased production and secretion of hormones that are essential to life. Common symptoms of HH, that are distressing for patients, include joint pain, particularly involving hands and wrists, as well as the chronic fatigue syndrome. Iron overload affects the skeletal system, leading to osteoporosis. The pathological accumulation of iron in the anterior pituitary impairs the gonadotropin synthesis, resulting in reduced serum levels of testosterone in men and estrogens in women. This, however, contributes to lower bone mass. In vivo tests have also revealed that abnormal iron accumulation is related to an increased activity and number of osteoclasts, as well as the influence on the differentiation and activity of osteoblast-lineage cells. Based on a systematic review of literature, hereditary hemochromatosis (HH) will be presented as a chronic disease, affecting most of the endocrine glands.
EN
Hyperparathyroidism (pHPT) is a relatively frequent endocrinopathy, however, the molecular mechanisms of its etiology remain poorly understood. This disorder is mainly associated with benign tumours (adenoma) and hyperplasia of the parathyroid, hence, the focus is directed also to genes that are likely to be involved in carcinogenesis. Among such genes are ErbB/Her family genes already used in diagnosis of other tumours (e.g., breast carcinoma) and reported also to play a role in development of endocrine lesions. So far, ErbB-1/Her-1/EGFR expression has been detected in pHPT-associated adenomas and hyperplasia as opposed to no expression in normal parathyroid tissue. Moreover, losses or gains of the fragments of chromosomes where ErbB/Her genes are located have been reported. In this study, the gene dosage of ErbB/Her family genes were determined for the first time in parathyroid adenomas, hyperplasia and morphologically unchanged tissue in order to establish their putative role in the development of the disease. Genomic DNA was isolated from 33 patients with sporadic hyperparathyroidism and the gene copy numbers were assessed using real-time PCR. The ErbB/Her genes' profile was unaltered in most of the examined samples. Two low-level amplifications of ErbB-1/Her-1/EGFR gene, two deletions of ErbB-2/Her-2, and six deletions of ErbB-4/Her-4 were found. The ErbB-3/Her-3 gene remained unaffected. No correlation with clinical parameters was found for any gene. Both the low number of alterations and a lack of their associations with clinical parameters exclude the prognostic value of the ErbB/Her genes family in parathyroid tumourigenesis. Nevertheless, the ErbB-4/Her-4 deletions seem to be interesting for further investigations, especially in the context of PTH secretion.
EN
Orbital tumor is a rare presentation of lymphoma. Unspecific symptoms, local infiltration, chronic progression may mimic other more common orbital diseases and often make the diagnosis difficult. We report a case of orbital non-Hodgkin lymphoma initially diagnosed as Graves’ disease. A 65-year-old woman was admitted to the Department of Endocrinology with a diagnosis of a left eye tumor. On admission, apart from the tumor, exophthalmos and the upper eyelid proptosis were present. The lesion had been observed for two years before hospitalization. Due to the muscle infiltration, as detected on computed tomography scanning and magnetic resonance imaging, Graves’ disease was suggested. The thyroid function was normal. Further diagnosis performed during hospitalization revealed lymphoplasmacytic lymphoma. Lymphoma may manifest as a localized orbital tumor without extraorbital or constitutive symptoms. Rare orbital diseases, among others lymphoproliferations, should be taken into account in the differential diagnosis of exophthalmos.
6
71%
EN
Management of patients with neuroendocrine tumors (NETs) of the pancreas causes considerable controversy because rarity of this neoplasm.The aim of the study was to present our results of treatment of patients with NETs and to sum up our experience in surgical management.Material and methods. Thirty four patients with neuroendocrine tumors of the pancreas were treated in Department of General, Endocrine and Transplant Surgery of Medical University in Gdańsk (24 inulinomas and 10 nonfunctioning neuroendocrine tumors). Insulinoma was present in the head of the pancreas in 3 cases, in the body in 8 cases, and 10 patients had lesion situated within the tail.Results. Localization of the tumor in patients with organic hyperinsulinism was possible in 21 out of 24 operated patients (17 patients with use of preoperative imaging studies, 4 patients with Intraoperative ultrasonography). In 3 remaining patients the localization of the pathologic mass was impossible with use of pre- and intraoperative techniques.Conclusions. Treatment of choice of patients with neuroendocrine tumors of the pancreas is surgery. Management of patients with islet cells adenomatosis is still difficult clinical problem.
EN
Adrenal tumors are common neoplasms and majority of them are small, benign, hormonally inactive adrenocortical adenomas. Whereas adrenal cancer (ACC) is a rarely occurring (5% of adrenal tumors) but highly aggressive neoplasm. The early diagnosis and complete surgical resection is the only effective treatment option. Laparoscopic adrenalectomy is the gold standard for small and medium tumors. Whereas for large tumors classic adrenalectomy is considered a procedure of choice with a proven better oncological outcome. We herein report a case of a 57-year-old female diagnosed with a large, advanced left adrenal tumor with invasion of vena cava. It was diagnosed in CT and proven in core biopsy. Open adrenalectomy with thoracotomy was conducted to completely resect the tumor by an interdisciplinary team.
EN
Introduction: Vitamin D deficiency is a worldwide public health problem. The objective of this survey was to assess the undergraduate students’ vitamin D status and knowledge about this vitamin. Materials and methods: An online multi-choice survey was designed and launched in Northern Poland (Gdańsk region). The first part of the survey assessed diet, supplementation, UV radiation exposure (UVE) and general health of respondents. The second part was a vitamin D knowledge test (vitamin D optimal level, deficiency-related diseases). 1766 student volunteers responded to the survey: 369 male and 1397 female. Data was divided according to the respondents’ sex and university affiliation. Appropriate parametric or non-parametric statistical tests were used with statistical significance set at p<0.05. Results: Regular consumption of vitamin D-rich food was high, except for fish (only 18; 22%). High number of participants did not declare any type of supplementation (43; 44%) and only occasionally were exposed to UV (77%; 80%). The most frequently recognized disease linked to vitamin D was osteoporosis. Medical University students obtained higher test scores (4,55), however this did not correlate with healthy vitamin D habits. Conclusion: Undergraduate medical and non-medical students have unsatisfactory vitamin D status and poor understanding of its function and impact on health, which implies the need for changes in the educational program.
EN
Adrenal tumors are common neoplasms and majority of them are small, benign, hormonally inactive adrenocortical adenomas. Whereas adrenal cancer (ACC) is a rarely occurring (5% of adrenal tumors) but highly aggressive neoplasm. The early diagnosis and complete surgical resection is the only effective treatment option. Laparoscopic adrenalectomy is the gold standard for small and medium tumors. Whereas for large tumors classic adrenalectomy is considered a procedure of choice with a proven better oncological outcome. We herein report a case of a 57-year-old female diagnosed with a large, advanced left adrenal tumor with invasion of vena cava. It was diagnosed in CT and proven in core biopsy. Open adrenalectomy with thoracotomy was conducted to completely resect the tumor by an interdisciplinary team.
EN
Introduction Vitamin D deficiency is a worldwide public health problem. The objective of this survey was to assess the undergraduate students’ vitamin D status and knowledge about this vitamin. Materials and methods An online multi-choice survey was designed and launched in Northern Poland (Gdańsk region). The first part of the survey assessed diet, supplementation, UV radiation exposure (UVE) and general health of respondents. The second part was a vitamin D knowledge test (vitamin D optimal level, deficiency-related diseases). 1766 student volunteers responded to the survey: 369 male and 1397 female. Data was divided according to the respondents’ sex and university affiliation. Appropriate parametric or non-parametric statistical tests were used with statistical significance set at p<0.05. Results Regular consumption of vitamin D-rich food was high, except for fish (only 18; 22%). High number of participants did not declare any type of supplementation (43; 44%) and only occasionally were exposed to UV (77%; 80%). The most frequently recognized disease linked to vitamin D was osteoporosis. Medical University students obtained higher test scores (4,55), however this did not correlate with healthy vitamin D habits. Conclusion Undergraduate medical and non-medical students have unsatisfactory vitamin D status and poor understanding of its function and impact on health, which implies the need for changes in the educational program.
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