Analysis of adrenalectomy for the treatment of adrenal diseases performed by the Public Health Service in São Paulo between 2008 and 2019 / Análise da adrenalectomia para tratamento de doenças adrenais realizadas pelo Serviço Público de Saúde de São Paulo entre 2008 e 2019

ABSTRACT Introduction: treating benign (hormonally active or nonfunctional) and malignant adrenal cancer includes adrenalectomy. The expertise of surgeons and surgery performed by high-volume surgeons were associated with fewer complications and lower cost. We aimed to describe and compare the number of surgeries, mortality rate, and length of hospital stay for adrenalectomies performed between 2008 and 2019 in the public health system of São Paulo. Methods: this was an ecological study. The data were collected using the TabNet Platform of the Unified Health System Department of Informatics. Outcomes analyzed included the number of surgeries performed, mortality rate during hospital stay, and length of hospital stay. Public hospitals in Sao Paulo were divided into three subgroups according to the surgical volume of adrenalectomies performed as well as hospitals with and without a residency program in Urology, and the results were compared among them. Results: a total of 943 adrenalectomies were performed in Sao Paulo between 2008 and 2019. Mortality rates during hospital stay according to hospital surgical volume were no reported deaths in low-volume, 0.015% in intermediate-volume, and 0.004% in high-volume hospitals. The average length of the ICU stay was 1.03 days in low-volume, 2.8 in intermediate-volume, and 1.12 in high-volume hospitals (analysis between intermediate and high volume centers with statistical significance, p=0.016). Conclusions: despite no statistically significant differences among the groups analyzed, mortality rates were very low in all groups. ICU stay was shorter in high-volume centers than in intermediate-volume centers.

RESUMO Introdução: o tratamento do câncer de adrenal benigno (hormonalmente ativo ou não funcional) e maligno inclui a adrenalectomia. A experiência dos cirurgiões e a cirurgia realizada por cirurgiões de alto volume foram associadas a menos complicações e menor custo. O objetivo do estudo foi descrever e comparar o número de cirurgias, a taxa de mortalidade e o tempo de internação para adrenalectomias realizadas entre 2008 e 2019 na rede pública de saúde de São Paulo. Métodos: trata-se de um estudo ecológico. Os dados foram coletados da Plataforma TabNet do Departamento de Informática do Sistema Único de Saúde. Os hospitais foram divididos em três subgrupos de acordo com o volume cirúrgico e hospitais com e sem programa de residência médica em Urologia. Os resultados foram comparados entre os grupos. Resultados: no período estudado, 943 adrenalectomias foram realizadas em São Paulo. As taxas de mortalidade durante a internação de acordo com o volume cirúrgico hospitalar foram: não foram relatados óbitos em hospitais de baixo volume; 0,015% em hospitais de volume intermediário e 0,004% em alto volume. O tempo médio de permanência na UTI foi de 1,03 dias nos hospitais de baixo volume; 2,8 nos de médio volume e 1,12 nos de alto volume (análise entre centros de médio e alto volume com significância estatística, p=0,016). Conclusão: as taxas de mortalidade foram muito baixas em todos os grupos. A permanência na UTI foi menor em centros de alto volume do que em centros de volume intermediário.

Ganglioneuroma suprarrenal. Reporte de un caso / Adrenal ganglioneuroma. A case report / Ganglioneuroma suprarrenal. Relato de caso

Resumen: Introducción: el ganglioneuroma suprarrenal es un tumor benigno excepcional originado en las células nerviosas derivadas de la cresta neural. El objetivo de este trabajo es comunicar un caso clínico de un ganglioneuroma suprarrenal derecho sintomático. Caso clínico: paciente de 37 años, sexo femenino, que consultó por dolor abdominal inespecífico que luego de la valoración imagenológica y estudio funcional hormonal, se diagnosticó una tumoración suprarrenal derecha no funcionante. Con dicho diagnóstico se realizó la adrenalectomía por abordaje convencional retroperitoneal y el estudio anatomopatológico de la pieza diagnosticó un ganglioneuroma. Discusión: los ganglioneuromas habitualmente son asintomáticos y no funcionantes, por lo que su diagnóstico es incidental en un alto porcentaje de casos. Otros se presentan con una sintomatología inespecífica. Su diagnóstico etiológico preoperatorio raramente es realizado y su confirmación es anatomopatológica. Tienen indicación quirúrgica los sintomáticos, los que superan los 6 cm, o persisten dudas diagnósticas de malignidad. El pronóstico es bueno, siendo excepcional la recidiva.

Summary: Introduction: adrenal ganglioneuroma is an exceptional benign tumour which originates from neural crest cells. The study aims to inform about a clinical case of asymptomatic right adrenal ganglioneuroma. Clinical case: 37-year old patient, female, who consulted for non-specific abdominal pain which, after imaging assessment and functional and hormone test was diagnosed with non-functioning tumour of the right adrenal gland. Upon this diagnosis, adrenalectomy using the conventional retroperitoneal approach was performed, and the pathology study of the piece confirmed the diagnosis of ganglioneuroma. Discussion: ganglioneuromas are usually asymptomatic and non-functioning, so diagnosis is incidental in a large percentage of cases. Others present non-specific symptoms. Preoperative etiological diagnosis is rare, and confirmation is usually after pathology study. Symptomatic tumours over 6cm long have an indication of surgery, as well as those suspicious of malignancy. Prognosis is good, relapses being exceptional.

Resumo: Introdução: o ganglioneuroma suprarrenal é um tumor benigno excepcional originado nas células nervosas derivadas da crista neural. O objetivo deste trabalho é descrever o caso clínico de um ganglioneuroma suprarrenal direito sintomático. Caso clínico: paciente de 37 anos, sexo feminino que consultou por dor abdominal inespecífica que foi diagnosticado como uma tumoração suprarrenal direita não funcionante depois da avaliação de estudos de imagem e funcional hormonal. Com este diagnóstico realizou-se adrenalectomia por abordagem convencional retroperitoneal e o laudo anatomopatológico diagnosticou um ganglioneuroma. Discussão: os ganglioneuromas geralmente são assintomáticos e não funcionantes por isso seu diagnóstico é acidental em uma alta proporção dos casos. Outros se apresentam com sintomatologia inespecífica. O diagnóstico etiológico pré-operatório raramente é realizado e sua confirmação é feita pela anatomia patológica. Quando são sintomáticos, têm mais de 6 cm ou se persistem dúvidas sobre sua malignidade têm indicação cirúrgica. O prognóstico é bom, e a recidiva é excepcional.

Analysis of influencing factors of adrenal hemorrhage and necrosis in critically ill children / 中国医师杂志

Objective To investigate the adrenal histopathology damage in critically ill non-survival children,and the incidence of adrenal damage,and to explore the risk factors for the adrenal hemorrhage and necrosis in critically ill children.Methods A total of 141 critically ill non-survival children was admitted in this study in Hunan Children's Hospital from January 1,2005 to December 30,2012.Clinical data in children were systematically collected,including age,sex,sodium,potassium,blood gas analysis,liver and kidney function,blood clotting function,etiology,treatment and pediatric clinical illness score (PCIS) or neonatal critical illness score (NCIS),and pathological data.All data were analyzed by SPSS 18.0.Results Logistic regression analysis showed the factors of adrenal hemorrhage and necrosis of critically ill non-survival children were sepsis,PCIS score ＜ 80 critically ill children are risk factors for adrenal hemorrhage necrosis (P ＜ 0.05),ORwas 3.659 (95％ CI:1.344-9.965),and 2.325 (95％ CI:1.028 -5.258).Intravenous corticosteroids were protective factors for critically ill non-survival children with adrenal hemorrhage necrosis (P ＜ 0.05),and ORwas 0.377 (95％ CI:0.163-0.875).Conclusions There were two significant risk factors of adrenal hemorrhage and necrosis:sepsis and critical illness score less than 80 points.Intravenous corticosteroid is a protective factor in critically ill children.

Fetal adrenal area space-occupying lesions:prenatal diagnosis on ultrasound and prognosis / 中华超声影像学杂志

Objective To explore the prenatal ultrasonic types ,sonographic features of malformation , ultrasonic diagnosis and prognosis of fetuses with adrenal area space‐occupying lesions . Methods Forty‐four fetuses with adrenal area space‐occupying lesions were retrospectively analyzed . Compared with postpartum neonatal special inspection results or the anatomical and pathological examination results of the corpses after induced labor ,the ultrasound types ,sonographic feature ,the key points of differential diagnosis were summarized . The newborns were followed up to 6 months after birth ,tracking the prognosis . Results Totally 44 fetuses among 166 471 fetuses(166 471 pregnant women) were detected with adrenal area space‐occupying lesions , 41 cases after delivery , including 24 cases adrenal hematoma ( 2 cases prenataly misdiagnosed as neuroblastoma) ,14 cases adrenal cyst and 3 cases neuroblastoma ,were accurately diagnosed through color Doppler or MRI or CT or pathological examination results . Three cases after induced labor , including 1 case adrenal neuroblastoma ,1 case mullerian duct cyst and 1 case adrenal hematoma( prenataly misdiagnosed as neuroblastoma ) were confirmed through the anatomical and pathological examination results of the corpses . So the conformity rate of adrenal area space‐occupying lesions was 90 .9% (40/44) . The postnatal ultrasound showed that the tumor disappeared in twenty‐four cases of adrenal hematoma without surgery ,and the longest time of the tumor disappearance was 1 year . The size of 14 cases adrenal cyst had no obvious change . One case of adrenal neuroblastoma was operated at 2 months ,2 cases were operated at 2 years of age . Conclusions Ultrasound can be used as qualitative diagnosis and differential diagnosis for fetal adrenal area space‐occupying lesions . The majority of the children with adrenal area space‐occupying lesions have a good prognosis .

Hemorragia suprarrenal que se manifiesta como hematoma escrotal en el recién nacido: A propósito de un caso / Adrenal hemorrhage presenting as a scrotal hematoma in the newborn: A case report

La hemorragia suprarrenal neonatal es poco frecuente. Se presenta en el 0,2% de los recién nacidos. El 10% de los casos son bilaterales. Puede estar asociada a un traumatismo al nacimiento, peso alto al nacer o un curso neonatal complicado por hipoxia, asfixia, hipotensión o coagulopatía. El hematoma escrotal es una manifestación extremadamente rara de la hemorragia suprarrenal neonatal. La mayoría de los pacientes presentan tumefacción escrotal con coloración azulada. En los recién nacidos, la tumefacción escrotal, con o sin coloración azulada, puede deberse a diferentes causas. Comunicamos un caso inusual de hemorragia suprarrenal neonatal secundaria a asfixia perinatal, que se manifestó con un hematoma escrotal. El diagnóstico de hemorragia suprarrenal neonatal y hematoma escrotal fue ecográfico; el tratamiento conservador evitó la exploración quirúrgica innecesaria.

Neonatal adrenal hemorrhage is uncommon. It is present in 0,2% of newborns. Ten percent of the cases occur bilaterally. It can be associated with birth trauma, large birth weight, or neonatal course complicated by hypoxia and asphyxia, hypotension, or coagulopathy. Scrotal hematoma is an extremely rare manifestation of NAH. Most patients present scrotal swelling with bluish discolouration. Scrotal swelling with/without bluish discoloration in newborns may result from different causes. We report an unusual case of neonatal adrenal hemorrage secondary to perinatal asphyxia, associated with SH. Neonatal adrenal hemorrhage and scrotal hematoma were diagnosedby ultrasonography and treated by conservative treatment, avoiding unnecessary surgical exploration.

Differential diagnosis value of enhanced multi-slice spiral CT scan on adrenal adenoma from metastases in patients with malignant tumor / 国际肿瘤学杂志

Objective To evaluate the differential diagnosis value of enhanced multi-slice spiral CT (MSCT) scan on adrenal adenoma and metastases in patients with malignant tumor.Methods Thirty-nine malignant tumor patients complicated with adrenal nodules were chosen, and all patients underwent MSCT plain scan and enhanced scan.Features of adrenal adenomas and metastases of MSCT enhanced were analyzed.Results Forty-nine adrenal gland nodules were found in 39 patients, and 35 adrenal metastasis were found in 25 patients.They were shown quasi-circular, oval or irregular shaped nodules.The average diameter was (2.6 ± 0.7) cm.Part of them were uneven density, and the CT value of the solid part was (32.8 ± 6.1) Hu.The solid part of tumor in enhancement scanning arterial phase was underwent mild to moderate strengthening, and the CT value was (49.5 ±6.9)Hu.The solid part of tumor was underwent further strengthen scanning in the venous phase, and the CT value was (74.9 ±8.0)Hu.The average CT value of solid part in after 3 min scanning tumor was (72.4 ± 7.6) Hu.Fourteen adrenal adenomas were found in 14 patients.CT value was (19.6 ± 4.5) Hu, and tumor diameter was (1.8 ± 0.4) cm.Enhanced scanning the tumors showed mild to moderate homogeneous enhancement in arterial phase, the CT value was (43.8 ± 8.1) Hu.Venous phase enhanced obviously, the average of CT value was (67.7 ±9.2)Hu.The strong degree in the delay period was decreased significantly, the average value of CT was (55.9 ± 8.8) Hu.The adrenal metastasis tumor diameter (t =4.006, P ＜ 0.001), CT value of plain scan (t =7.320, P ＜ 0.001), CT value of arterial phase enhanced scan (t =2.486, P =0.017) , venous phase enhanced scan (t =2.727, P =0.009) and CT value of the delay period (t =6.653, P ＜ 0.001) were higher than those in adrenal adenoma.Conclusion Enhanced MSCT scan can reflect the hemodynamic changes of adrenal lesions, and provide the bases for the differential diagnosis of enhanced MSCT scan on adrenal adenoma and metastases in patients with malignant tumor.

Tumores adrenocorticais na criança: da abordagem clínica à avaliação molecular / Childhood adrenocortical tumors: from a clinical to a molecular approach

Tumores do córtex adrenal (TCA) são mais frequentes em crianças, mas podem ocorrer em qualquer faixa etária. São classificados como funcionantes, não funcionantes (predominam no adulto), e mistos. O diagnóstico é baseado na avaliação clínica, hormonal e exames de imagem. Em crianças, o método de escolha para diferenciar entre benigno ou maligno é a classificação baseada no estadiamento do tumor. Alguns marcadores moleculares merecem destaque: além de mutações inativadoras no gene supressor tumoral TP53, há evidências de envolvimento do IGF2 em 90 por cento de TAC malignos, e mutações no éxon 3 do gene CTNNB1 foram encontradas em 6 por cento dos TAC pediátricos. Além disso, microRNAs podem atuar como reguladores negativos da expressão gênica e participar da tumorigênese adrenocortical. Métodos para análise da expressão gênica permitem identificar TCA com prognóstico bom ou ruim, e espera-se que esses estudos possam facilitar o desenvolvimento de drogas para tratar pacientes de acordo com as vias de sinalização específicas que estiverem alteradas.

Adrenocortical tumors (ACT) are more frequent during childhood, but they can appear at any age. ACTs can be classified in functioning, nonfunctioning (mainly observed in adults) and mixed. The diagnosis is based on clinical, biochemical findings and imaging. In children, in order to classify ACT as benign or malignant, tumor staging classification is recommended. Regarding molecular markers some studies should be taken into account: besides TP53 mutations, previous studies have also provided evidences of IGF2 involvement in 90 percent of the malignant ACT. Mutations altering exon 3 of CTNNB1 gene have been found in 6 percent of childhood ACTs. In addition, microRNAs can act as negative regulators of gene expression by targeting mRNA controlling cell growth, differentiation and apoptosis and have been implicated in adrenal tumorigenesis. High-throughput methods to analyze genome-wide expression have been developed over the last decade and identified a subset of tumors with good or poor prognosis. In the future, these studies can provide the basis of specific drug development, which can treat patients according to specific altered signaling pathway.

Scrotal hematoma as a sign of adrenal hemorrhage in newborns / Hematoma escrotal como um sinal de hemorragia da adrenal no recém-nascido

CONTEXT: Bluish discoloration and swelling of the scrotum in newborns can arise from a number of diseases, including torsion of the testes, orchitis, scrotal or testicular edema, hydrocele, inguinal hernia, meconium peritonitis, hematocele, testicular tumor and traumatic hematoma. Forty-two cases of scrotal abnormalities as signs of neonatal adrenal hemorrhage were found in the literature. CASE REPORT: We present a case of scrotal hematoma due to adrenal hemorrhage in a newborn. Conservative treatment with clinical follow-up was adopted, with complete resolution within 10 days. The possible differential diagnoses are reviewed and discussed.

CONTEXTO: Edema e coloração azulada do escroto do recém-nascido podem sugerir uma série de doenças, incluindo torção dos testículos, orquite, edema escrotal e testicular, hidrocele, hérnia inguinal, peritonite meconial, hematocele, tumor testicular e hematoma traumático. Quarenta e dois casos de alterações escrotais como sinal de hemorragia da glândula adrenal foram encontrados na literatura. RELATO DE CASO: Apresentamos um caso de hematoma escrotal devido a hemorragia da glândula adrenal em um recém-nascido. O tratamento adotado foi conservador, com acompanhamento clínico, com resolução completa em 10 dias. Os possíveis diagnósticos diferenciais são revisados e discutidos.

Value of sonography in diagnosis of neonatal adrenal hemorrhage / 中华超声影像学杂志

Objective To investigate the ultrasonographic character of neonatal adrenal hemorrhage (NAH). Methods Forty-four hospitalized NAH were retrospectively analyzed. Doppler ultrasonography detected their shape and supply. Results Forty-one cases were diagnosed by ultrasonography in 44 cases of NAH,3 cases were misdiagnosed, accompanying with scrotal hematoma in 5 cases. Ultrasonography demonstrated mixed, anechoic, hyperechoic, hypoechoic adrenal mass, no color Doppler signal in all of NAH. Conclusions NAH can be diagnosed by color Doppler ultrasound in early stage and it is the first choice method.

Ultrnsonographic observation of fetal adrenal cystic lesions / 中华超声影像学杂志

Objective To evaluate the value of ultrasound in diagnosing fetal adrenal cystic lesions. Methods Twenty one hospitalized fetuses with adrenal cystic lesions were retrospectively studied. All fetuses were followed up by ultrasound during neonatal period, and all lesions were finally confirmed by pathology after operation or ultrasound-guided biopsy. Results In 21 cases of fetal cystic lesions, the ultrasonic characteristics of 15 cases were anechoic and 6 cases were flake and mixed echoic. Nevertheless, the characteristics of these lesions were changed in neonatal period, 10 cases were presented anechoic and 11 cases were presented mixed echoic. The pathologic results: I0 cases were simple cysts, 5 cases were cysts with hemorrhage and the other 6 cases were spontaneous hemorrhage of adrenal glands. Conclusions The ultrasonic characteristics of adrenal cystic lesions in the stage of pregnancy can provide useful diagnostic information for clinic practice.

Transperitoneal Laparoscopic Adrenalectomy: A Single Surgeon Experience / 대한비뇨기과학회지

PURPOSE: We present a single surgeon experience of 52 cases of transperitoneal laparoscopic adrenalectomy for various adrenal diseases. MATERIALS AND METHODS: Transperitoneal laparoscopic adrenalectomy (n= 52) for various adrenal diseases was performed by a single surgeon from February 1998 to December 2004. Clinical diagnosis of the subjects included 17 cases of primary aldosteronism, 11 of adrenal adenoma causing Cushing's syndrome, 3 of pheochromocytoma and 21 of nonfunctioning adenoma. The 52 patients were divided into 3 groups according to the time when operations were performed: early group of 18 patients, intermediate group of 17 patients, and late group of 17 patients. The mean operative time, mean estimated blood loss (EBL) and complication of each group were investigated to analyze the single surgeon's learning curve. RESULTS: All laparoscopic adrenalectomies were successfully completed in all 52 patients. The mean operative time, mean EBL, mean tumor size, and mean postoperative hospital stay were 124 minutes (60-360), 56cc (30-300), 2.7cm (1-4.7), and 3.8 days (3-8), respectively. The mean time to solid diet and ambulation were 1.5 days and 1 day, respectively. There was no serious intraoperative or postoperative complication. Statistically, the mean operative time and estimated blood loss showed a significant reduction in the intermediate and late group compared with the early group. CONCLUSIONS: Our results showed that transperitoneal laparoscopic adrenalectomy is a safe and effective surgical treatment modality in adrenal diseases.

Adrenogenital syndrome in children / 中华泌尿外科杂志

Objective To discuss the clinical features and therapeutic effects of adrenogenital syndrome in children. Methods Clinical and follow up data were summarized for 42 children with adrenogenital syndrome, including 30 cases of congenital adrenal hyperplasia (CAH) and 12 cases of adrenal cortex tumor. Results Endocrine function examination in 15 cases of CAH showed that 24h urine 17 KS was elevated in 10 cases and normal in 5.There was obvious clitorism in all 30 CAH children and urogenital sinus malformation in 28.Of the 12 cases of adrenal cortex tumor 7 had sexual abnormality and hypercortisolism simultaneously.Clitoris shortening or clitoridectomy was performed in CAH cases plus vaginal vestibule plasty simultaneously.Of the 12 cases of adrenal cortex tumor 10 underwent complete tumor resection;1 partial tumor resection and the remaining 1 declined treatment.During the follow up 8 case of cortex tumor,4 cases of adenoma survived tumor free with an average survival time of 7.5 years;2 cases of cortex carcinoma survived tumor free for 3 and 4 years;while the other 2 cases died soon after discharge. Conclusions The diagnosis can be established based on clitorism,labium confluence or male sexual precocity,increase in urine 24h 17 KS and blood testrone.Cortex hormone should be taken early,properly,life long in CAH patients,and appropriate perineoplasty should be performed.Early detection and resection of tumor is the key point for treating adrenogenital syndrome caused by cortex tumor.

ADRENAL HEMATOMA: A REPORT OF 4 CASES AND REVIEW OF CHINESE LITERATURE / 解放军医学杂志

To investigate the cause, clinical features, differential diagnosis and treatment of adrenal hematoma, 4 cases were studied retrospectively. Chinese literature was reviewed. There were 4 cases diagnosed as adrenal hematoma pathologically among 282 cases of adrenalectomy in Chinese PLA General Hospital. Two cases were suspected as adrenal adenoma and the others were found incidentally during nephrectomy. Forty six cases of hematoma were reported in 7 Chinese articles since 1979. 26 1%(12/46)were misdiagnosed as adenoma for surgical treatment and 73 9%(34/46)were followed up. It is very important to have sound knowledge to make a correct diagnosis of adrenal hematoma. It is helpful to inquire the case history in detail and to do B type ultrasound repeatedly, CT scanning or MRI with contrast. Follow up is reasonable if it is not suspected to have malignant or hyperfunctioning adenoma.

ANALYSIS OF 98 CASES OF ADRENAL INCIDENTALOMAS / 解放军医学杂志

In order to explore the clinical feature, diagnosis and differential diagnosis of adrenal incidentalomas, we reviewed 98 cases of adrenal incidentalomas for which adrenalectomy was performed in our hospital from 1993 to 2002. It was found that the average age of the patients was 48 years old, 16 cases (16 3%) had no specific symptoms, and others were incidentally diagnosed. Of the 98 cases, 18 (18 4%) were malignant lesions, including 8 metastatic cancers, 8 adrenal carcinomas and 4 lymphangioma, and 80 were benign lesions. Twenty one cases were functioning adrenal adenoma. Ultrasound, CT scan, MRI or MIBG were effective methods to detect adrenal incidentalomas.