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1.
J Med Case Rep ; 15(1): 514, 2021 Oct 11.
Artigo em Inglês | MEDLINE | ID: mdl-34635153

RESUMO

BACKGROUND: Ectopic adrenocorticotropic hormone secretion syndrome occurs in 10% of all patients with adrenocorticotropic-hormone-dependent hypercortisolism. It is usually associated with overt malignancies or with occult and indolent tumors. This study aims to confirm the source of ectopic adrenocorticotropic hormone in four patients with ectopic Cushing's syndrome over time. CASE PRESENTATION: A 38-year-old Iranian man with Cushing's syndrome underwent bilateral adrenalectomy since the source of ectopic adrenocorticotropic hormone secretion was not localized and pituitary imaging was normal. A whole-body scan revealed a right-lung tumoral mass with mediastinal lymph node metastasis. The mass was assumed a lung carcinoid tumor with mediastinal adenopathy. Right-lung mid-zone lobectomy and mediastinal lymphadenectomy were done. In a 47-year-old Iranian man with Cushing's syndrome, whole-body computed tomography scan revealed a pulmonary nodule in the posterior segment of the left lower lobe of the lung. The third case was a 25-year-old Iranian man who presented with symptoms and signs of Cushing's syndrome. Pituitary magnetic resonance imaging revealed a microadenoma 5 × 9 mm. Whole-body scan showed abnormal focal somatostatin receptors analog avid lesion in the posterior aspect of inferior third of right lung, highly suggestive of ectopic adrenocorticotropic-hormone-producing tumor. The last case was a 43-year-old Iranian woman with Marfan syndrome with a history of mitral and aortic valve replacement and chronic dissection of the aorta, who presented with symptoms and signs of Cushing's syndrome. She underwent bilateral adrenalectomy 1 year later owing to failure to locate ectopic adrenocorticotropic hormone syndrome. Whole-body scan showed abnormally increased radiotracer uptake in the midline of the skull base and posterior aspect of the middle zone of left hemithorax and bed of left lobe of thyroid. CONCLUSION: The clinical spectrum of ectopic adrenocorticotropic hormone secretion syndrome is wide, and distinguishing Cushing's disease from ectopic adrenocorticotropic hormone secretion syndrome is difficult. Initial failure to identify a tumor is common. Pulmonary carcinoid or occult source of ectopic adrenocorticotropic hormone secretion syndrome is usually the cause. In occult cases of ectopic adrenocorticotropic hormone in which the tumor cannot be localized, serial follow-up with serial computed tomography, magnetic resonance imaging, or scintigraphy is recommended for several years until the tumor can be localized and treated.


Assuntos
Síndrome de ACTH Ectópico , Síndrome de Cushing , Síndrome de ACTH Ectópico/cirurgia , Adrenalectomia , Adulto , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/cirurgia , Feminino , Seguimentos , Humanos , Irã (Geográfico) , Masculino , Pessoa de Meia-Idade
2.
Arch Esp Urol ; 74(8): 782-789, 2021 Oct.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34605409

RESUMO

OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare and heterogeneous disease, with challenging management and poor prognosis. Surgery withcurative intent is the preferred treatment option for localized disease, with a reported 5-year survival rate of 55% for complete resections. However, owing to the high risk of recurrence there is a need for adjuvant therapies, such as mitotane, an adrenolytic drug, or irradiation, while in advanced disease the standard of careis a combined chemotherapy scheme. The aim of this study was to report our experience in the surgical management and outcomes of ACC patients. MATERIALS AND METHODS: A retrospective observational study was performed in a cohort of ACC patients who had undergone surgical resection (open or laparoscopic approach) and were followed up at our tertiary hospital. Patients with localized or locally advanced disease were included in the analysis. All medical records, including clinical, surgical, pathologic, and follow-updata, were collected and analyzed. RESULTS: A total of 19 ACC patients were managed at our center between August 1990 and August 2013. The median age at diagnosis was 50.5 years (range 19-72), and most patients were males. Abdominal pain was the most common clinical presentation (n=9,47.4%). Abdominal contrast-enhanced computed tomography (CT) was performed in all cases. Only 3 tumors (15.8%) were functional and most were stage II at diagnosis (n=9, 47.4%). No patient presented metastasis. Of the 19 patients, 18 (94.7%) under went surgerywith curative intent, while one (5.3%) received adjuvant radiotherapy (ART). The open approach was used in 17 patients (89.5%), while the remaining 2 (10.5%) underwent laparoscopy. Postoperative complications occurred in 8 patients (42.1%); none were of grade IV or V. Median follow-up was 66 months (range 3-312). The majority of patients (n=15, 78.9%) were disease free with surgery alone. None received adjuvant mitotane therapy (AMT). Four patients (21%) experienced metachronous metastases and 3 (15.8%) local recurrence after a median time of 10.5 months (range 2-60) and 9.3 months (range 1.5-30), respectively. The 5-year overall survival rate was 47.4%. CONCLUSIONS: Our findings confirm both the unpredictable nature of ACC and the accepted primary role of surgery. The use of adjuvant therapy was less frequent in this series than is supported currently. However, a multidiscipinary approach should be the initial step in the management of this rare malignancy.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Carcinoma Adrenocortical/cirurgia , Adulto , Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
3.
Pan Afr Med J ; 39: 98, 2021.
Artigo em Francês | MEDLINE | ID: mdl-34466200

RESUMO

Bilateral macronodular adrenal hyperplasia (BMAH) is a rare cause of adrenal Cushing's syndrome, accounting for less than 1% of cases. We here report the case of a 48-year-old patient with diabetes and hypertension presenting with clinical signs of Cushing's syndrome. Etiological assessment enabled clinicians to retain the diagnosis of Adrenocorticotropic hormone (ACTH)-independent hypercortisolism associated with BMAH. Unilateral left adrenalectomy was performed based on noriodocholesterol scintigraphy results, with good outcome. However, given the risk of recurrence and cardiovascular complications, long-term monitoring was scheduled.


Assuntos
Doenças das Glândulas Suprarrenais/complicações , Adrenalectomia , Síndrome de Cushing/etiologia , Doenças das Glândulas Suprarrenais/diagnóstico , Doenças das Glândulas Suprarrenais/cirurgia , Hormônio Adrenocorticotrópico/metabolismo , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/cirurgia , Humanos , Hiperplasia , Masculino , Pessoa de Meia-Idade
4.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao ; 43(4): 653-658, 2021 Aug.
Artigo em Chinês | MEDLINE | ID: mdl-34494540

RESUMO

Primary aldosteronism is the most common cause of secondary hypertension.This review focuses on the procedures related to surgical treatment and summarizes the available evidence.We analyzed the impact of primary aldosteronism on the body,the advantages of surgical treatment,the choice of patients and surgical methods,perioperative management,and surgical efficacy evaluation.Finally,we put forward the prospect of scientific research in this field,with a view to providing reference for clinical work.


Assuntos
Hiperaldosteronismo , Hipertensão , Adrenalectomia , Humanos , Hiperaldosteronismo/cirurgia
6.
Zhonghua Yi Xue Za Zhi ; 101(34): 2662-2666, 2021 Sep 14.
Artigo em Chinês | MEDLINE | ID: mdl-34510871

RESUMO

Primary aldosteronism (PA) patients diagnosed with aldosterone-producing adenoma and unilateral adrenal hyperplasia are preferred for surgical treatment.The aim of the operation is to remove the hypersecretory aldosterone tissue of adrenal and to improve the prognosis.Current studies have shown that both partial adrenalectomy and total adrenalectomy can achieve clinical cure for PA, but the choice of surgical methods is still controversial. This paper discusses the efficacy and safety, postoperative changes in cortisol level, postoperative recurrence and surgical techniques of different surgical methods in the treatment of different PA subtypes, so as to find the best surgical methods for different PA subtypes and to maximize the benefits for patients.


Assuntos
Adenoma , Hiperaldosteronismo , Adenoma/cirurgia , Glândulas Suprarrenais , Adrenalectomia , Aldosterona , Humanos , Hiperaldosteronismo/cirurgia , Recidiva Local de Neoplasia
7.
Zhonghua Yi Xue Za Zhi ; 101(34): 2723-2727, 2021 Sep 14.
Artigo em Chinês | MEDLINE | ID: mdl-34510880

RESUMO

On the basis of existing laparoscopic transabdominal adrenalectomy, this article described a safe and reliable surgical method for the treatment of small adrenal lesions-laparoscopic anatomical adrenalectomy (LAA), and retrospectively analyzed the clinical data of 74 patients who had undergone LAA. All patients had no signs of recurrence on imaging. LAA has high safety and feasibility, clear intraoperative anatomical layers, good spatial operability, and low postoperative complications. LAA provides a more reliable option for the treatment of small adrenal diseases.


Assuntos
Neoplasias das Glândulas Suprarrenais , Laparoscopia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Humanos , Recidiva Local de Neoplasia , Complicações Pós-Operatórias , Estudos Retrospectivos
8.
BMJ Case Rep ; 14(9)2021 Sep 12.
Artigo em Inglês | MEDLINE | ID: mdl-34511410

RESUMO

Large adrenal pheochromocytomas encasing the renal artery are a rare entity. The management of such challenging cases is surgical resection. The involvement of renal tissue and renal artery may necessitate meticulous dissection and concomitant nephrectomy. Here, we present a case of 41-year-old man diagnosed with left adrenal pheochromocytoma with complete encasement of left renal artery and partial encasement of aorta. Open left adrenalectomy and nephrectomy was performed after adequate preoperative optimisation. The patient is doing well at 6-month follow-up. Large adrenal pheochromocytoma with renal involvement is a rare presentation and requires optimal preoperative imaging, adequate preoperative alpha and beta blockade and meticulous surgical technique.


Assuntos
Neoplasias das Glândulas Suprarrenais , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Adulto , Aorta , Humanos , Masculino , Nefrectomia , Feocromocitoma/diagnóstico por imagem , Feocromocitoma/cirurgia , Artéria Renal/diagnóstico por imagem , Artéria Renal/cirurgia
9.
J Vet Intern Med ; 35(5): 2159-2166, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34473866

RESUMO

BACKGROUND: Primary hyperaldosteronism caused by adrenal neoplasia has been well described in cats. Multiple corticosteroid abnormalities occur in a subset of affected cats, but characterizations of this syndrome are limited to several case reports. OBJECTIVES: To describe a series of cats with adrenal tumors secreting aldosterone and additional corticosteroids. ANIMALS: Ten cats with multiple corticosteroid secreting adrenocortical tumors. METHODS: Retrospective case series. Medical records of cats with adrenal tumors secreting both aldosterone and progesterone were identified. Data concerning historical findings, clinicopathologic features, treatments, and outcomes were retrieved from medical records. RESULTS: All 10 cats had diabetes mellitus in addition to biochemical features of hyperaldosteronism such as hypokalemia. High corticosterone concentrations were observed in all 3 cats in which this corticosteroid was measured. Ultrasound examinations revealed unilateral adrenal tumors in all 10 cases, and the contralateral adrenal gland was either atrophied or not identified in 5 cats. Three of 4 cats developed hypoadrenocorticism after surgical adrenalectomy. Three cats achieved diabetic remission after adrenalectomy. Two cats treated with adrenalectomy survived >1 year, 1 cat survived 6.5 months, and 1 cat was alive 5.5 months after diagnosis. Survival >1 year occurred in 2 of 4 cats treated with medical management alone. Two cats were not treated. CONCLUSIONS AND CLINICAL IMPORTANCE: The presence of multiple corticosteroid abnormalities should be considered in cats with aldosterone secreting adrenal tumors, especially those with concurrent diabetes mellitus. Both surgical and medical management can result in long-term survival, although diabetic remission was documented only in cats undergoing adrenalectomy.


Assuntos
Neoplasias das Glândulas Suprarrenais , Doenças do Gato , Hiperaldosteronismo , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/veterinária , Glândulas Suprarrenais/diagnóstico por imagem , Adrenalectomia/veterinária , Aldosterona , Animais , Gatos , Hiperaldosteronismo/cirurgia , Hiperaldosteronismo/veterinária , Progesterona , Estudos Retrospectivos
10.
BMJ Case Rep ; 14(8)2021 Aug 16.
Artigo em Inglês | MEDLINE | ID: mdl-34400431

RESUMO

A 55-year-old woman presented with a 4-month history of right-sided non-specific loin pain and 6 kg weight loss. A CT scan of the abdomen and pelvis showed an incidental 4.5 cm right-sided adrenal lesion which was not typical of an adrenal adenoma. This was further confirmed on MRI of the adrenals. Biochemical investigations to investigate for a functional adrenal lesion included serum catecholamines and metanephrines, an aldosterone to renin ratio and an overnight dexamethasone suppression test. These were all negative. A laparoscopic adrenalectomy was performed in view of the large size of the lesion. Histology was consistent with a phaeochromocytoma, which confirmed the diagnosis of a non-secreting phaeochromocytoma. Non-secreting phaeochromocytomas are rare and usually found in patients with known genetic mutations. Adrenal lesions not related to any mutations similar to our case are even rarer and reported even less in the literature.


Assuntos
Neoplasias das Glândulas Suprarrenais , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Adrenalectomia , Feminino , Humanos , Metanefrina , Pessoa de Meia-Idade , Dor , Feocromocitoma/diagnóstico por imagem , Feocromocitoma/cirurgia
11.
ACS Chem Neurosci ; 12(16): 2981-3001, 2021 08 18.
Artigo em Inglês | MEDLINE | ID: mdl-34339164

RESUMO

We explored sex-biased effects of the primary stress glucocorticoid hormone corticosterone on the miRNA expression profile in the rat hippocampus. Adult adrenalectomized (ADX) female and male rats received a single corticosterone (10 mg/kg) or vehicle injection, and after 6 h, hippocampi were collected for miRNA, mRNA, and Western blot analyses. miRNA profiling microarrays showed a basal sex-biased miRNA profile in ADX rat hippocampi. Additionally, acute corticosterone administration triggered a sex-biased differential expression of miRNAs derived from genes located in several chromosomes and clusters on the X and 6 chromosomes. Putative promoter analysis unveiled that most corticosterone-responsive miRNA genes contained motifs for either direct or indirect glucocorticoid actions in both sexes. The evaluation of transcription factors indicated that almost 50% of miRNA genes sensitive to corticosterone in both sexes was under glucocorticoid receptor regulation. Transcription factor-miRNA regulatory network analyses identified several transcription factors that regulate, activate, or repress miRNA expression. Validated target mRNA analysis of corticosterone-responsive miRNAs showed a more complex miRNA-mRNA interaction network in males compared to females. Enrichment analysis revealed that several hippocampal-relevant pathways were affected in both sexes, such as neurogenesis and neurotrophin signaling. The evaluation of selected miRNA targets from these pathways displayed a strong sex difference in the hippocampus of ADX-vehicle rats. Corticosterone treatment did not change the levels of the miRNA targets and their corresponding tested proteins. Our data indicate that corticosterone exerts a sex-biased effect on hippocampal miRNA expression, which may engage in sculpting the basal sex differences observed at higher levels of hippocampal functioning.


Assuntos
Corticosterona , MicroRNAs , Adrenalectomia , Animais , Corticosterona/farmacologia , Feminino , Hipocampo/metabolismo , Masculino , MicroRNAs/genética , Ratos , Receptores de Glucocorticoides/genética , Receptores de Glucocorticoides/metabolismo
12.
Pan Afr Med J ; 38: 367, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34367446

RESUMO

Adrenal unilateral macronodular hyperplasia is a rare cause of Cushing's syndrome. We discuss the case of two patients who present Cushing syndrome due to unilateral adrenal hyperplasia. They presented the signs of clinical hypercorticism as well as metabolic, cardiovascular and osteoporotic complications. Both patients presented clinical and laboratory signs of adrenocorticotropic hormone (ACTH)-independent Cushing syndrome with elevated urinary free cortisol (UFC) levels, adrenal computed tomography (CT) scan revealed the appearance of unilateral adrenal adenoma and normal contralateral adrenal gland. Adrenalectomy was performed under laparoscopic surgery; the resected mass was pathologically diagnosed as unilateral nodular adrenal hyperplasia. Unilateral adrenal hyperplasia is a very rare etiology of ACTH-independent Cushing syndrome, often mistaken for adenoma on CT and only pathological examination can confirm the diagnosis.


Assuntos
Doenças das Glândulas Suprarrenais/complicações , Adrenalectomia/métodos , Síndrome de Cushing/diagnóstico , Adenoma/diagnóstico , Doenças das Glândulas Suprarrenais/cirurgia , Hormônio Adrenocorticotrópico/metabolismo , Síndrome de Cushing/etiologia , Síndrome de Cushing/cirurgia , Feminino , Humanos , Hiperplasia , Laparoscopia , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
13.
Medicine (Baltimore) ; 100(31): e26838, 2021 Aug 06.
Artigo em Inglês | MEDLINE | ID: mdl-34397852

RESUMO

RATIONALE: Renal cell carcinoma (RCC) almost metastasizes to every organ, the possibility of adrenal metastasis is relatively low in patients that have undergone radical nephrectomy, only a few cases of bilateral adrenal metastasis are reported on literature. Although surgical treatment of metastases from RCC is preferred and contributes to the rate of survival, it is considered challenging to manage such cases due to the rarity of bilateral metastasis to the adrenal glands. PATIENT CONCERNS: A 64-year-old Manchus female presented with an incidental ultrasonic finding of a left adrenal mass 4 years after radical nephrectomy for left renal cell carcinoma. DIAGNOSIS: Abdominal contrast enhanced CT scan revealed bilateral adrenal masses, suggesting metastatic lesion. Examinations indicated neither local recurrence nor distant metastasis anywhere have been detected by whole body Positron Emission Tomography/Computed Tomography (PET/CT) scan except high radioactive uptake in bilateral adrenal glands. INTERVENTIONS: Metachronous bilateral adrenalectomy was taken and laparoscopic right adrenalectomy was first performed. She was discharged home on third postoperative day. Pathological examination revealed metastatic renal cell carcinoma. Two months later she was performed laparoscopic left adrenalectomy. OUTCOMES: The patient healed without obvious complications and no tumor recurrence. LESSONS: Bilateral metastatic adrenal recurrence from RCC is very rare. Early diagnosis of adrenal metastasis is challenging because they are usually silent both anatomically and functionally. Surgical intervention is a wise option for these patients that may improve survival, and metachronous bilateral adrenalectomy is proved to be safe and effective.


Assuntos
Neoplasias das Glândulas Suprarrenais , Adrenalectomia/métodos , Carcinoma de Células Renais , Neoplasias Renais , Metástase Neoplásica , Nefrectomia/métodos , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/cirurgia , Glândulas Suprarrenais/diagnóstico por imagem , Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/cirurgia , Carcinoma de Células Renais/patologia , Carcinoma de Células Renais/cirurgia , Feminino , Humanos , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Pessoa de Meia-Idade , Metástase Neoplásica/diagnóstico por imagem , Metástase Neoplásica/patologia , Metástase Neoplásica/terapia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Imagem Corporal Total/métodos
14.
Beijing Da Xue Xue Bao Yi Xue Ban ; 53(4): 808-810, 2021 Aug 18.
Artigo em Chinês | MEDLINE | ID: mdl-34393250

RESUMO

A 57-year-old male patient was referred to our department with complaints of his right adrenal gland occupancy and hypertension about 6 months. When admitted to the hospital, the blood pressure was about 160/100 mmHg, and the heart rate was 110 beats/min. He was no obvious obesity, acne, abnormal mood, without weakness of limbs, acral numbness, palpitation and headache. He presented with type 2 diabetes for more than 3 years, with oral administration of metformin enteric coated tablets and subcutaneous injection of insulin glargine to control blood glucose, and satisfied with blood glucose control. Enhanced CT showed that: the right adrenal gland showed a kind of oval isodense, slightly hypodense shadow, the edge was clear, lobular change, the size was about 5.8 cm×5.4 cm, uneven density, there were nodular and strip calcification, round lipid containing area and strip low density area, and the CT value of solid part was about 34 HU. Enhanced scan showed heterogeneous nodular enhancement in the solid part of the right adrenal gland, nodular enhancement could be seen inside. The CT values of solid part in arterial phase, venous phase and delayed phase were 45 HU, 50 HU and 81 HU, respectively. Considering from the right adrenal gland, cortical cancer was more likely. No obvious abnormality was found in his endocrine examination. After adequate preoperative preparation, retroperitoneal laparoscopic adrenalectomy was performed under general anesthesia. During the operation, the 6 cm adrenal tumor was closely related to the inferior vena cava and liver, and after careful separation, the tumor was completely removed and normal adrenal tissue was preserved. The operation lasted 180 min and the blood loss was 100 mL, and the blood pressure was stable during and after the operation. There was no obvious complication. The results of pathological examinations were as follows: the size of the tumor was 7.5 cm×6.0 cm×3.5 cm, soft, with intact capsule and grayish-red cystic in section. Pathological diagnosis: (right adrenal gland) cavernous hemangioma, secondary intravascular thrombosis, old hemorrhagic infarction with calcification and ossification. After 6 months of observation, no obvious complications and tumor recurrence were found. In summary, cavernous hemangioma of adrenal gland is a rare histopathological change. Its essence is a malformed vascular mass. Blood retention is the cause of thrombosis and calcification in malformed vessels. The imaging findings were inhomogeneous enhancement of soft tissue masses, and the adrenal function examination showed no obvious abnormalities. Retroperitoneal laparoscopic surgery is feasible after adequate preoperative preparation. It is difficult to diagnose the disease preoperatively and needs to be confirmed by postoperative pathology.


Assuntos
Neoplasias das Glândulas Suprarrenais , Diabetes Mellitus Tipo 2 , Hemangioma Cavernoso , Laparoscopia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Hemangioma Cavernoso/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia
15.
Eur J Endocrinol ; 185(3): 405-412, 2021 Aug 03.
Artigo em Inglês | MEDLINE | ID: mdl-34232123

RESUMO

Objective: Primary aldosteronism (PA) is the most common form of secondary and curable hypertension. Different germline and somatic mutations are found in aldosterone-producing adenoma (APA) and familial forms of the disease, while the causes of bilateral adrenal hyperplasia (BAH) remain largely unknown. Adrenalectomy is the recommended treatment for patients with APA; however, 6% of patients are not cured and show persistent PA after surgery suggesting BAH. The objective of this study was to analyze clinical data of patients with APA without biochemical success after adrenalectomy as well as the histological and genetic characteristics of their adrenal glands. Design and methods: Clinical data of 12 patients with partial and absent biochemical cure were compared to those from 39 PA patients with hormonal cure after surgery. Histological, morphological, and genetic characterization of the adrenals was carried out by CYP11B2 and CYP11B1 immunostaining and by CYP11B2-guided NGS. Results: Patients with absent hormonal cure displayed a longer duration of arterial hypertension and lower lateralization index of aldosterone production. In ten patients, APAs expressing CYP11B2 were identified. No difference in histological and morphological characteristics was observed between patients with or without a hormonal cure. Somatic mutations in APA driver genes were identified in all CYP11B2 positive APAs; CACNA1D mutations were the most frequent genetic abnormality. Conclusions: Patients with partial and absent biochemical cure were diagnosed later and exhibited a lower lateralization index of aldosterone production, suggesting asymmetric aldosterone production in the context of BAH. Somatic mutations in adrenal glands from those patients indicate common mechanisms underlying BAH and APA.


Assuntos
Doenças das Glândulas Suprarrenais/genética , Doenças das Glândulas Suprarrenais/patologia , Adrenalectomia , Hiperaldosteronismo/genética , Hiperaldosteronismo/patologia , Doenças das Glândulas Suprarrenais/cirurgia , Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/cirurgia , Adulto , Feminino , Humanos , Hiperaldosteronismo/cirurgia , Masculino , Pessoa de Meia-Idade , Mutação , Resultado do Tratamento
16.
Ann Ital Chir ; 92: 254-259, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34312325

RESUMO

INTRODUCTION: Pheochromocytoma is an endocrine tumour of chromaffin cells. It can be diagnosed either sporadically or in the context of hereditary syndromes (e.g. Von Hippel Lindau, Neurofibromatosis type 1 and multiple neuroendocrine neoplasia type 2). During pregnancy, its frequency is very low (about 0,007%). This tumour causes paroxysmal hypertension in 0,1-0,6% pregnant women, because of an overproduction of catecholamines. If undiagnosed and nontreated, it's associated with high maternal and fetal mortality (40-50%). We report the case of a 30-year-old female diagnosed with pheochromocytoma during pregnancy at week 31 of gestation. In a multidisciplinary team made of surgeons, gynaecologists, anaesthetists, geneticists and endocrinologists we evaluated the case and according to literature, we choose a surgical approach after childbirth: performing a laparoscopic right adrenalectomy. DISCUSSION: In pregnancy, pheochromocytoma is a rare clinical condition. Gold standard treatment is laparoscopic adrenalectomy. However, the optimum timing of surgery is a challenge. CONCLUSION: Timely diagnosis of pheochromocytoma in pregnant women with hypertension and appropriate therapeutic management can lead to improve maternal, fetal and neonatal outcomes. The multidisciplinary team is necessary to recognize the symptoms and to adopt the right pre - and post - operative treatment. Laparoscopic adrenalectomy after delivery is safe and feasible even though the surgical procedure should be performed by an experienced surgeon. KEY WORDS: Pheochromocytoma, Pregnancy, Laparoscopic adrenalectomy and pregnancy, Management of pheochromocytoma, Laparoscopic adrenalectomy, Adrenalectomy, Pregnancy and pheochromocytoma.


Assuntos
Neoplasias das Glândulas Suprarrenais , Adrenalectomia/métodos , Laparoscopia , Feocromocitoma , Complicações Neoplásicas na Gravidez , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Feminino , Humanos , Feocromocitoma/diagnóstico , Feocromocitoma/cirurgia , Gravidez , Complicações Neoplásicas na Gravidez/diagnóstico , Complicações Neoplásicas na Gravidez/cirurgia , Resultado da Gravidez , Resultado do Tratamento
17.
Ann Ital Chir ; 92: 293-298, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34312326

RESUMO

INTRODUCTION: Oncocytic adrenocortical tumors represent a subtype of the adrenal cortex neoplasms. These tumors can be divided into oncocytomas, oncocytic neoplasms of uncertain malignancy and carcinomas (OACs). To date, only 34 cases of OAC have been reported. CASE EXPERIENCE: We reported a case of a 54-year-old male patient with a history of laparoscopic right adrenalectomy for an OAC and subsequent chemotherapy for a recurrence. He was referred to our emergency room for diffuse abdominal pain, vomit, change in bowel habits, fever, asthenia. He underwent a laparotomy and a complete excision of the known bilobate lesion. The histopathological findings matched the features of a recurrent OAC. No chemotherapy was administered after surgery and the patient was disease-free after a follow-up of twenty-eight months. RESULTS - DISCUSSION: The most questionable issue in treating adrenocortical oncocytic neoplasms is the determination of malignancy. According to the Helsinki Score, which is the best prognostic system, the primary lesion was an OAC. We also implemented the score systems to the recurrent lesion, that seemed to be malignant. We believe that the adjuvant treatment can delay a recurrent lesion development, but finally, radical surgical excision is necessary. Moreover, we reported the longest survival after the primary adrenalectomy. CONCLUSIONS: This study described the first case of recurrent oncocytic adrenocortical carcinoma with the longest followup. Adrenocortical oncocytoma is an extremely rare tumor of the adrenal gland with variable biological behavior without definitive consensus about diagnostic criteria. This was also the first case in which different histopathological criteria have been implemented in a recurrence. KEY WORDS: Oncocytic adrenocortical carcinoma, Surgical oncology, Survival.


Assuntos
Adenoma Oxífilo , Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Recidiva Local de Neoplasia , Adenoma Oxífilo/diagnóstico , Adenoma Oxífilo/cirurgia , Neoplasias do Córtex Suprarrenal/diagnóstico , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Carcinoma Adrenocortical/diagnóstico , Carcinoma Adrenocortical/cirurgia , Humanos , Laparoscopia , Laparotomia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/cirurgia , Reoperação
18.
Arch Esp Urol ; 74(6): 587-591, 2021 Jul.
Artigo em Espanhol | MEDLINE | ID: mdl-34219061

RESUMO

OBJECTIVES: The aim of this paper is to describe the clinical features and the perioperative results of the bilateral laparoscopic adrenalectomy (BLA) of salvaje in 5 patients treated for an Ectopic Cushing Syndrome (CS) who were refractory to medical treatment. MATERIALS AND METHODS: We performed a retrospective analysis of the clinical records of our institution. Five BLAs were performed in patients with Ectopic CS refractory to medical treatment during the period from January 2009 and June 2017. All patients were evaluated by a multidisciplinary team that jointly decided the most appropriate time for surgery. The following protocol data were analyzed: age, sex, location of the secreting tumor, duration of hypercortisolism, reason for hospitalization, initial therapeutic management), biochemical data (pre-op serum cortisol, plasma ACTH and free urinary cortisol), surgical data (surgical approach, surgical time, type of surgery, need for conversion toopen surgery), perioperative data (post op surgical stay, post op complications according to the Clavien-Dindo scale 14, mortality, clinical cure rate and biochemical cure rate, follow up time). RESULTS: Five patients underwent BLA. The approach for the BLA was minimally invasive, either trans peritoneal (n=3) or posterior retroperitoneoscopic (n=2). Mean hospitalization time was 23.4 days (r=4-81). None of the patients died because of surgical complications, and all of them achieved biochemical and clinical remission of their hypercortisolism after surgery. CONCLUSIONS: BLA is an effective salvage alter native to control the symptoms associated with overproduction of corticosteroids in patients with ectopic CS refractory to medical treatment.


Assuntos
Síndrome de Cushing , Laparoscopia , Adrenalectomia , Síndrome de Cushing/cirurgia , Humanos , Estudos Retrospectivos , Resultado do Tratamento
19.
J Med Case Rep ; 15(1): 369, 2021 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-34253234

RESUMO

BACKGROUND: The presence of isolated metachronous adrenal metastasis in patients with esophageal cancer is rare. There is significant controversy regarding the management of such patients. Adrenal metastasectomy has been shown to be of benefit in some reports. Minimally invasive approach, although the gold standard for adrenalectomy, has not been used commonly in a postesophagectomy setting owing to the anticipated technical difficulties. We describe one such case wherein this approach helped in early recovery and long-term survival. CASE PRESENTATION: A 59-year-old male of Asian ethnicity presented with an isolated left adrenal nodule, 3 years after an Ivor Lewis esophagectomy for a lower esophageal adenocarcinoma. The biopsy of the nodule was suggestive of metastatic adenocarcinoma. The patient underwent laparoscopic excision of the left adrenal gland. CONCLUSION: Adrenal metastasectomy, in postesophagectomy patients can provide good oncological control. Laparoscopic approach, though technically challenging, can provide results equivalent to those of open surgery, albeit with less morbidity.


Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasias Esofágicas , Laparoscopia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Neoplasias Esofágicas/cirurgia , Esofagectomia , Humanos , Masculino , Pessoa de Meia-Idade
20.
BMC Cardiovasc Disord ; 21(1): 350, 2021 07 22.
Artigo em Inglês | MEDLINE | ID: mdl-34294029

RESUMO

BACKGROUND: Primary aldosteronism (PA), as a cause of secondary hypertension, can cause more serious cardiovascular damage than essential hypertension. The aldosterone-to-renin ratio (ARR) is recommended as the most reliable screening method for PA, but ARR screening is often influenced by many factors. PA cannot be easily excluded when negative ARR. CASE PRESENTATION: We report the case of a 45-year-old Chinese man with resistant hypertension. Three years ago, he underwent a comprehensive screening for secondary hypertension, including the ARR, and the result was negative. After that, the patient's blood pressure was still poorly controlled with four kinds of antihypertensive drugs, the target organ damage of hypertension progressed, and hypokalaemia was difficult to correct. When the patient was hospitalized again for comprehensive examination, we found that aldosterone levels had significantly increased, although the ARR was negative. An inhibitory test with saline was further carried out, and the results suggested that aldosterone was not inhibited; therefore, PA was diagnosed. We performed a unilateral adenoma resection for this patient, and spironolactone was continued to control blood pressure. After the operation, blood pressure is well controlled, and hypokalaemia is corrected. CONCLUSION: When the ARR is negative, PA cannot be easily excluded. Comprehensive analysis and diagnosis should be based on the medication and clinical conditions of patients.


Assuntos
Adenoma/complicações , Neoplasias das Glândulas Suprarrenais/complicações , Aldosterona/sangue , Hiperaldosteronismo/sangue , Hipertensão/etiologia , Renina/sangue , Adenoma/sangue , Adenoma/diagnóstico , Adenoma/cirurgia , Neoplasias das Glândulas Suprarrenais/sangue , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Anti-Hipertensivos/uso terapêutico , Biomarcadores/sangue , Pressão Sanguínea/efeitos dos fármacos , Humanos , Hiperaldosteronismo/diagnóstico , Hiperaldosteronismo/etiologia , Hipertensão/diagnóstico , Hipertensão/tratamento farmacológico , Hipertensão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
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