| 1. |
- Calissendorff, Jan, et al.
(författare)
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Characteristics, Treatment, Outcomes, and Survival in Neuroendocrine G1 and G2 Pancreatic Tumors : Experiences From a Single Tertiary Referral Center
- 2021
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Ingår i: Frontiers in Endocrinology. - : Frontiers Media S.A.. - 1664-2392. ; 12
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: Neuroendocrine tumors of the pancreas (Pan-NETs) are usually hormonally inactive with a capacity to metastasize. Since Pan-NETs are rare, more knowledge is needed.Methods: We reviewed all patients’ medical files with Pan-NET treated at a tertiary center (2006-2019). Grade 1 (G1) and grade 2 (G2) tumors were compared. The latter group was subdivided arbitrarily based on proliferation index into G2a (3-9.9%) and G2b (10-19.9%).Results: We found 137 patients (76 females, 61 males; G1 n=66, G2 n=42), the median age at diagnosis 61 years (interquartile range (IQR) 50–71), and tumor size 2 cm (1.3–5 cm). The initial surgery was performed in 101 patients. The remaining (n=36) were followed conservatively. Metastatic disease was evident in 22 patients (16%) at diagnosis while new lesions developed in 13 out of 22 patients (59%). In patients without previous metastatic disease, progressive disease was discovered in 29% of G1 vs. 55% of G2 patients (P=0.009), 47% of G2a vs. 75% of G2b patients (NS). Survival was poorer in patients with metastasis at diagnosis vs. those with local disease (P<0.001). During follow-up of 74 months, Pan-NET related death was found in 10 patients. Survival was not different between G1 vs. G2 or G2a vs. G2b, or if tumors were functional. Size ≤2 cm was associated with a better outcome (P=0.004). During the follow-up of small tumors (≤2 cm, n=36) two were resected.Conclusion: In small non-functional Pan-NETs, active surveillance is reasonable. Progressive disease was more common in G2, but survival was similar in G1, G2 and between G2 subgroups. Survival was poorer in patients with metastasis at diagnosis.
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| 2. |
- Juhlin, Carl Christofer, et al.
(författare)
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Highly proliferative anal neuroendocrine carcinoma : molecular and clinical features of a rare, recurrent case in complete remission
- 2020
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Ingår i: BMC Gastroenterology. - : BMC. - 1471-230X .- 1471-230X. ; 20:1
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Tidskriftsartikel (refereegranskat)abstract
- Background Poorly differentiated anal neuroendocrine carcinomas (ANECs) are rare lesions with poor prognosis, and the molecular etiology is only partially understood. Case presentation At our institution, we have treated and followed a patient with such a rare ANEC. He had primarily surgery followed by three rounds of repeated surgery for loco-regional recurrences. He also received three different combinations of chemotherapy and external beam radiation. At last follow-up 13 years since the primary diagnosis, the patient had been in complete remission for nine years. The patient's medical files were re-examined, including laboratory, radiology and clinical examinations. Histopathology was re-assessed, and expanded immunohistochemistry was performed from tissue specimens from the four surgical procedures. In addition, the molecular genetic status was evaluated through next-generation sequencing. The initial tumor was consistent with a 59 mm small cell neuroendocrine cancer with a Ki-67 index of 80%. Regional lymph node metastases were evident, and immunohistochemistry supported a neuroendocrine origin. A PCR screening detected human papilloma virus type 45 DNA (high-risk subtype), and focused next-generation sequencing found a missense mutation in thePhosphatidylinositol-4,5-Bisphosphate 3-Kinase Catalytic Subunit Alpha(PIK3CA) gene. In tissues representing subsequent recurrences, the Chromogranin A expression was lost, and the Ki-67 index increased to 90%. Conclusions For the first time, we report the detection of HPV45 in a case of ANEC. To our belief,PIK3CAmutations have also not been previously demonstrated in this tumor entity. In highly malignant ANECs, cure can in rare cases be achieved. Although speculative, expression of HPV45 and/or thePIK3CAmutation may have contributed to the favorable outcome.
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| 3. |
- Abraham-Nordling, Mirna, et al.
(författare)
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Incidence of hyperthyroidism in Stockholm, Sweden, 2003-2005
- 2008
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Ingår i: European Journal of Endocrinology. - 1479-683X. ; 158:6, s. 823-827
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Tidskriftsartikel (refereegranskat)abstract
- Objectives: To investigate the incidence of hyperthyroidism in Stockholm County in those patients who were diagnosed with hyperthyroidism for the First time during the years 2003-2005. Design: All new cases of hyperthyroidism >= 18 years of age were prospectively registered to calculate the total incidence of hyperthyroidism, as well as the incidence of the subgroups: Graves' disease (GD), toxic multinodular goitre and solitary toxic adenoma (STA). Eight specialized units/hospitals in Stockholm County participated in the registration. The participating physicians were all specialists in medical endocrinology. oncology, nuclear medicine or surgery. Results: Duringa 3-year period, 1431 new patients of hyperthyroidism were diagnosed in a well-defined adult population (>18 years of age) of in average 1 457 036 inhabitants. This corresponds to a mean annual incidence of hyperthyroidism of 32.7/100 000. The incidence of GD was 24.5/100 000 per year. toxic nodular goitre was 3.3/100 000 per year and STA was 4.9/100 000 per year. Conclusions: The total incidence of hyperthyroidism in Stockholm County was found to be 32.7/100 000 per year. of which 75% had GD. There were a higher percentage of smokers among the patients with hyperthyroidism compared with the overall population in Stockholm, but no difference in the frequency of smoking between patients with GD and toxic nodular goitre.
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| 4. |
- Abraham-Nordling, Mirna, et al.
(författare)
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Incidence of hyperthyroidism in Sweden
- 2011
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Ingår i: European Journal of Endocrinology. - 0804-4643 .- 1479-683X. ; 165:6, s. 899-905
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Tidskriftsartikel (refereegranskat)abstract
- Introduction: The incidence of hyperthyroidism has been reported in various countries to be 23-93/100000 inhabitants per year. This extended study has evaluated the incidence for similar to 40% of the Swedish population of 9 million inhabitants. Sweden is considered to be iodine sufficient country. Methods:All patients including children, who were newly diagnosed with overt hyperthyroidism in the years 2003-2005, were prospectively registered in a multicenter study. The inclusion criteria are as follows:clinical symptoms and/or signs of hyperthyroidism with plasma TSH concentration below 0.2 mIE/l and increased plasma levels of free/total triiodothyronine and/or free/total thyroxine. Patients with relapse of hyperthyroidism or thyroiditis were not included. The diagnosis of Graves' disease (GD), toxic multinodular goiter (TMNG) and solitary toxic adenoma (STA), smoking, initial treatment, occurrence of thyroid-associated eye symptoms/signs, and demographic data were registered. Results:A total of 2916 patients were diagnosed with de novo hyperthyroidism showing the total incidence of 27.6/100 000 inhabitants per year. The incidence of GD was 21.0/100 000 and toxic nodular goiter (TNG=STA+TMNG) occurred in 692 patients, corresponding to an annual incidence of 6.5/100 000. The incidence was higher in women compared with men (4.2:1). Seventy-five percent of the patients were diagnosed with GD, in whom thyroid-associated eye symptoms/signs occurred during diagnosis in every fifth patient. Geographical differences were observed. Conclusion:The incidence of hyperthyroidism in Sweden is in a lower range compared with international reports. Seventy-five percent of patients with hyperthyroidism had GD and 20% of them had thyroid-associated eye symptoms/signs during diagnosis. The observed geographical differences require further studies.
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| 5. |
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| 6. |
- Calissendorff, Jan, et al.
(författare)
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68Ga-DOTA-TOC-PET/CT detects heart metastases from ileal neuroendocrine tumors
- 2014
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Ingår i: Endocrine. - : Springer Science and Business Media LLC. - 1559-0100 .- 1355-008X. ; 47:1, s. 169-176
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Tidskriftsartikel (refereegranskat)abstract
- Metastases from ileal neuroendocrine tumors (NETs) to the myocardium are rare and generally seen in patients with widespread metastatic NET disease. The objectives of this investigation were to describe the frequency of intracardiac metastases in ileal NET patients examined by 68Ga-DOTA-TOC-PET/CT and to describe the cases in detail. All 68Ga-DOTA-TOC-PET/CT examinations performed at the Karolinska University Hospital since 2010 until April 2012 were reviewed. In all, 128 out of 337 examinations were in patients with ileal NETs. Four patients had seven myocardiac metastases, yielding a frequency of 4.3 % in patients with ileal NETs. One patient had cardiac surgery while three were treated with somatostatin analogs. The cardiac metastases did not affect the patients’ activity of daily life. 68Ga-DOTA-TOC-PET/CT is an established imaging modality in identifying cardiac metastases in ileal NETs. Prospective studies are needed to confirm the true clinical value of 68Ga-DOTA-TOC-PET/CT in detecting cardiac metastases in both ileal and non-ileal NETs.
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| 7. |
- Calissendorff, Jan, et al.
(författare)
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A Prospective Investigation of Graves' Disease and Selenium : Thyroid Hormones, Auto-Antibodies and Self-Rated Symptoms.
- 2015
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Ingår i: European thyroid journal. - : Bioscientifica. - 2235-0640 .- 2235-0802. ; 4:2, s. 93-98
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: In Graves' thyrotoxicosis tachycardia, weight loss and mental symptoms are common. Recovery takes time and varies between patients. Treatment with methimazole reduces thyroid hormone levels. According to previous research, this reduction has been faster if selenium (Se) is added.OBJECTIVE: The objective was to investigate whether supplementing the pharmacologic treatment with Se could change the immune mechanisms, hormone levels and/or depression and anxiety.METHODS: We prospectively investigated 38 patients with initially untreated thyrotoxicosis by measuring the thyroid-stimulating hormone (TSH), free thyroxine (FT4), free triiodothyronine (FT3), thyroid receptor antibodies and thyroid peroxidase auto-antibodies before medication and at 6, 18 and 36 weeks after commencing treatment with methimazole and levo-thyroxine, with a randomized blinded oral administration of 200 µg Se/day or placebo. The selenoprotein P concentration was determined in plasma at inclusion and after 36 weeks. The patients were also assessed with questionnaires about depression, anxiety and self-rated symptoms before medication was started and after 36 weeks.RESULTS: FT4 decreased more in the Se group at 18 weeks (14 vs. 17 pmol/l compared to the placebo group, p = 0.01) and also at 36 weeks (15 vs. 18 pmol/l, p = 0.01). The TSH increased more in the Se group at 18 weeks (0.05 vs. 0.02 mIU/l, p = 0.04). The depression and anxiety scores were similar in both groups. In the Se group, the depression rates correlated negatively with FT3 and positively with TSH. This was not seen in the placebo group.CONCLUSIONS: Se supplementation can enhance biochemical restoration of hyperthyroidism, but whether this could shorten clinical symptoms of thyrotoxicosis and reduce mental symptoms must be investigated further.
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| 8. |
- Calissendorff, Jan, et al.
(författare)
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Adrenal cysts : an emerging condition.
- 2023
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Ingår i: Nature Reviews Endocrinology. - : Springer Nature. - 1759-5029 .- 1759-5037. ; 19, s. 398-406
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Tidskriftsartikel (refereegranskat)abstract
- Adrenal cysts are rare lesions representing approximately 1-2% of adrenal incidentalomas. The majority of these rare lesions are benign. Rarely, phaeochromocytomas and adrenal malignant masses can present as cystic lesions and can occasionally be difficult to distinguish from benign cysts. Histologically, adrenal cysts are subdivided into pseudocysts, endothelial cysts, epithelial cysts and parasitic cysts. The radiological appearance of an adrenal cyst is generally similar to that of cysts in the kidney. They are thus well demarcated, usually rounded, with a thin wall and homogenous internal structure, low attenuating (<20 Hounsfield Units) on CT, low signalling on T1-weighted MRI sequences and high signalling on T2-weighted MRI sequences, and anechoic or hypoechoic on ultrasonography. Benign adrenal cysts have a slight female predominance and are usually diagnosed between the ages of 40 and 60. Most adrenal cysts are asymptomatic and are detected incidentally, although very large adrenal cysts can lead to mass effect symptoms, with surgery required to alleviate the symptoms. Thus, conservative management is usually recommended for asymptomatic cysts. However, when uncertainty exists regarding the benign nature of the cyst, additional work-up or follow-up is needed. The management of an adrenal cyst should preferably be discussed at an adrenal multidisciplinary team meeting.
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| 9. |
- Calissendorff, Jan, et al.
(författare)
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Adrenal myelolipomas
- 2021
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Ingår i: The Lancet Diabetes and Endocrinology. - : Elsevier. - 2213-8587 .- 2213-8595. ; 9:11, s. 767-776
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Forskningsöversikt (refereegranskat)abstract
- Adrenal myelolipomas are benign, lipomatous tumours with elements of myeloid cells, most of which present as adrenal incidentalomas and comprise 3·3-6·5% of all adrenal masses. Adrenal myelolipomas are usually unilateral (in 95% of cases), variable in size, most often found during midlife, and affect both sexes almost equally. On imaging, adrenal myelolipomas show pathognomonic imaging features consistent with the presence of macroscopic fat. Large adrenal myelolipomas can cause symptoms of mass effect, and can occasionally be complicated by haemorrhage. In the event of a concomitant adrenal cortical adenoma or hyperplasia, adrenal hormone excess might be detected in patients with adrenal myelolipoma. Patients with congenital adrenal hyperplasia exhibit a higher prevalence of adrenal myelolipomas than other patient groups, and are at risk of developing large and bilateral lesions. This Review discusses the pathogenesis, clinical presentation, and management of adrenal myelolipomas.
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| 10. |
- Calissendorff, Jan
(författare)
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Alcohol and the effect on some appetite-regulating hormones in man
- 2008
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Beverage containing alcohol has been used for centuries to stimulate appetite. Ingestion of a moderate amount of alcohol increase energy intake. Regulation of food intake is complex. Several factors cooperate such as neural impulses from sensory organs; sight, smell, gut distension, social setting, memory, current energy status and hormones. How alcohol affects these factors is not well understood. This research project has focused on how alcohol influences the peripheral secretion of hormones, known to convey information from the periphery to hunger-regulating centers in the brain about the prevailing caloric homeostasis. Leptin produced by adipocytes, and gut-derived peptide YY (PYY), are two such hormones which inhibit food intake, whereas ghrelin produced by cells in the upper part of the gastro intestinal tract, stimulates appetite. These hormones have central effects on hypothalamic neuropeptide Y (NPY) and on pro-opiomelanocortin (POMC) which stimulates and down-regulates hunger, respectively. Other gut hormones, having central effects as well as influence on intestinal motility, are glucagon-like peptide (GLP-1) and obestatin. Liver derived insulin-like growth factor-1 (IGF-1) and its binding protein insulin-like growth factor binding protein-1 (IGFBP-1) are also of interest in this context, as they are affected by nutritional status and could be factors which influence appetite-regulating centers directly or indirectly via peripherally produced hormones. Aim: To study the effect of alcohol on the secretion of peripheral hormones known to be involved in the regulation of food intake. Material and Methods: 51 healthy subjects (26F/25M) were included in the study. All were young, healthy, normal weight and free of medication. In five separate experiments subjects were investigated in groups of 7-12 individuals. In exp 1, 2 and 3 the effect of alcohol on various hormone levels in serum, was compared with the effect of drinking water. In exp 2 the alcohol effect on urinary excretion of catecholamines was determined with or without oral beta-receptor blockade (propranolol). In exp 5 alcohol influence on gastro-intestinal hormones was investigated with or without sucralfate gastroprotection. Results and discussion: A moderate amount of alcohol induced a significant inhibition of both diurnal and nocturnal secretion of leptin. Factors known to affect the secretion of leptin such as insulin, glucose, cortisol, testosterone, and catecholamines were not influenced by the drug. IGFBP-1 increased significantly after alcohol, contrasting IGF-1, which remained unchanged. This resulted in a low IGF-1/IGFBP-1 ratio and, as a consequence, in a decreased IGF-1 bioavailability. Alcohol had both acute and prolonged inhibitory effect on serum levels of both total and octanoylated ghrelin, but was without significant influence on serum concentrations of NPY, PYY, GLP-1 and obestatin. Gastro-protection with sucralfate did not change the alcohol-induced inhibition of leptin and ghrelin secretion. Conclusion: Acute ingestion of alcohol inhibits the secretion of leptin and ghrelin, induces a marked decline in the IGF-1/IGFBP-1 ratio, but leaves NPY, PYY, GLP-1 and obestatin unchanged. Previous studies suggest that leptin may have long-term rather than acute inhibitory effects on hunger. Therefore, the present findings do not lend strong support to the hypothesis that alcohol has acute stimulatory effect on appetite by influencing peripherally produced hormones. If alcohol has appetite-stimulating properties in humans it is more likely that this is an effect caused by direct influence on appetite-regulating neurons in the brain.
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