Síndrome de Cushing por carcinoma adrenocortical en lactante de 5 meses

RESUMEN Introducción: Las causas adrenales del síndrome de Cushing como el carcinoma son las etiologías comunes en el niño, si bien su incidencia es baja. Objetivo: Describir los procedimientos diagnósticos y terapéuticos seguidos en el caso de una lactante de cinco meses con síndrome de Cushing como consecuencia de un carcinoma adrenocortical. Presentación del caso: Lactante femenina de cinco meses consultada por aumento excesivo de peso a partir del segundo mes de nacida a pesar de no alimentarse adecuadamente, además de presentar un retraso en el crecimiento. Se constató obesidad centrípeta y facie pletórica, ligero eritema facial y livedo reticularis, tensión arterial de 130/80 mmHg (>95 percentil). Presentaba agenesia de labios menores, sin vello axilar o pubiano. En el estudio de la función suprarrenal se encontró cifras elevadas de cortisol plasmático. En la tomografía computarizada abdominal se observó imagen hiperdensa compatible con tumor suprarrenal. Se diagnosticó síndrome de Cushing de etiología suprarrenal. Se le realizó adrenalectomía unilateral y se tomó muestra para biopsia posoperatoria y se emitió el diagnóstico de carcinoma adrenocortical. La paciente presenta una evolución desfavorable y finalmente fallece a consecuencia de un fallo multiorgánico al mes de realizada la cirugía. Conclusiones: Ante la sospecha clínica de síndrome de Cushing en la lactante se realizaron las determinaciones hormonales pertinentes y precisó la etiología mediante pruebas de imagen, confirmada por biopsia posquirúrgica. La cirugía constituye la terapia más adecuada ante un paciente con síndrome de Cushing de causa tumoral adrenal, aunque estos pacientes por lo general cursan con mal pronóstico.

ABSTRACT Introduction: The adrenal causes of Cushing's syndrome such as carcinoma are the common etiologies in the child, although their incidence is low. Objective: Describe the diagnostic and therapeutic procedures followed in the case of a five-month-old infant with Cushing's syndrome as a result of adrenocortical carcinoma. Case presentation: Five-month-old female infant consulted due to excessive weight gain from the second month of birth despite not eating properly, in addition to presenting a stunted growth. Centripetal and facial plethora obesity, slight facial erythema and livedo reticularis, blood pressure of 130/80 mmHg (>95 percentile) were found. She had agenesis of labia minora, without axillary or pubic hair. In the study of adrenal function, elevated plasma cortisol levels were found. Abdominal CT scan showed hyperdense image compatible with adrenal tumor. Cushing's syndrome of adrenal etiology was diagnosed. Unilateral adrenalectomy was performed and a sample was taken for postoperative biopsy and the diagnosis of adrenocortical carcinoma was made. The patient presented an unfavorable evolution and she finally died as a result of a multi-organ failure a month after the surgery. Conclusions: Given the clinical suspicion of Cushing's syndrome in the infant, the pertinent hormonal determinations were made and the etiology was specified by imaging tests, confirmed by post-surgical biopsy. Surgery is the most appropriate therapy for a patient with Cushing's syndrome with an adrenal tumor cause, although these patients usually have a bad prognosis.

Safety and efficacy of domestic single-port robotic surgery system for extraperitoneal urological surgery / 中华泌尿外科杂志

Objective:To investigate the safety and efficacy of the novel single-port surgical robotic surgical system in extraperitoneal urological surgery.Methods:From February to April 2022, patients was prospectively enrolled who required laparoscopic radical prostatectomy, partial nephrectomy and adrenal tumor resection in urology department. Inclusion criteria were: age ≥ 18 years old; BMI 18.5-30 kg/m 2; American Society of Anesthesiologists (ASA) physical status classification system grades 1 to 3; can cooperate with the completion of the visits and related examinations stipulated in the plan, and participate voluntarily clinical trials, and consent or the guardian agrees to sign the informed consent form; tumor indicators meet one of the following surgical treatment indications: kidney tumor T 1 stage, single, maximum tumor diameter ≤ 4 cm; prostate cancer, stage ≤ T 2b, preoperative PSA ≤ 20 ng /ml; Gleason score ≤ 7; adrenal tumor diameter ≤ 7 cm, for non-functioning adrenal adenoma, tumor diameter ≥ 3 cm. Exclusion criteria were: patients with other malignancies or a history of other malignancies and the investigators believe that they are not suitable for inclusion in this researcher; patients who have received the same type of urological surgery in the past and are not suitable for participating in this study as assessed by the investigators; included Those who have undergone other major surgery within the first 3 months and during the trial period, or who cannot recover from the side effects of any such surgery; syphilis, hepatitis B, HIV infection and carriers; long-term use of anticoagulants or blood system diseases; Unable to use effective contraception during the trial period and other conditions that the investigators deem inappropriate to participate in this trial. All operations were performed by a novel single-port robotic surgical operating system, and all surgical procedures were performed through an extraperitoneal approach. Surgical method: the surgical system is mainly composed of a remote console including a high-definition display, a surgical equipment trolley, a surgical execution system that accommodates a serpentine robotic arm, and a bendable serpentine robotic arm. In this study, the extraperitoneal approach was used. For radical prostatectomy, the patient was placed in a supine position, a longitudinal incision of about 3 cm was made below the umbilicus, the anterior rectus sheath was incised, the extraperitoneal space was separated, and an operating sheath was placed. A 12 mm trocar is placed between the right McBurney point and the umbilicus as an auxiliary hole. For partial nephrectomy and adrenal tumor resection, the patient is placed in the lateral position, and an 3cm incision is made 2 cm above the iliac crest on the midaxillary line as the main operating hole. The skin, subcutaneous tissue, and muscle were incised to the retroperitoneal cavity, and a 12mm trocar was placed at the level of the anterior superior iliac spine on the anterior axillary line as an auxiliary hole. The operation was performed after connecting each robotic arm. After the operation, the specimen was placed in the specimen bag, and a drainage tube is placed in the auxiliary hole, the specimen was taken out, and the incision was closed in turn. Preoperative basic information, operation time, blood loss, incision size, postoperative complications, preoperative and postoperative PSA score, eGFR index, postoperative pathological information and other perioperative information were collected. Results:A total of 17 patients were included in this study, including 6 with prostate cancer, 8 with renal tumor, and 3 with adrenal tumor. There were 9 males and 8 females, with an average age of (56.7±14.6) years and a BMI of (23.3±3.4) kg/m 2. The mean operation time of radical prostatectomy was (244.6±35.1) min, the mean operating time of the chief surgeon was (184.0±39.0) min, and the mean blood loss was (36.6±23.8) ml. Postoperative positive margin was found in 2 cases. The average operation time of partial nephrectomy was (189.6±49.4) minutes, the average operating time of the chief surgeon was (115±39.7) minutes, the average blood loss was (12.7±8.3) ml, and the average warm ischemia time was (23.1±10.8) minutes. There was no significant difference in the eGFR index before and after the operation ( P>0.05). The average operation time of adrenalectomy was (177.6±26.9) min, the average operating time of the chief surgeon was (99±20.4) min, and the average blood loss was (11.6±6.2) ml. The overall average operation time of the three surgical methods was (206.9±50.1) min, the overall average operating time of the chief surgeon was (136.5±51.1) min, the overall average blood loss was (21.0±9.2) ml, and the overall average incision size was (3.5±0.5) cm, all added a 12 mm auxiliary channel, and the overall average hospital stay was (8.1±2.7) days. All operations were successfully completed, and there was no conversion to open surgery during the operation, and no operation holes were added. There was no Clavien-Dindo≥grade 3 complication after operation. Conclusions:The novel single-port robot could safely and effectively perform radical prostatectomy, partial nephrectomy and adrenalectomy which are common in urology through extraperitoneal approach.

Robot-assisted laparoscopic surgery for treatment of adrenal tumors in children / 中华实用儿科临床杂志

Objective:To explore the feasibility, efficacy and limitation of robot-assisted laparoscopic surgery in the treatment of adrenal tumors in children.Methods:The clinical data of 5 children with adrenal tumors treated with robot-assisted laparoscopic surgery in Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science & Technology from October 2020 to March 2021 were retrospectively analyzed. All patients were males aged between 2 years and 4 months to 13 years and 1 month (median: 7 years and 7 months). One case had left adrenal adenomas and 4 cases had right adrenal adenomas.The maximum diameter of tumors was 3.0-6.0 cm (median: 4.8 cm).Results:Robot-assisted laparoscopic adrenalectomy was performed successfully on all 5 patients.The ope-ration time was 215-325 min (median: 275 min). The intraoperative bleeding was 10-50 mL (median: 28 mL). The indwelling time of the drainage tube after surgery was 3-5 days (median: 4 days). The postoperative hospital stay was 9-13 days (median: 11 days). The hospitalization cost was 51 268-58 157 yuan (median: 53 485 yuan). No complications occurred during or after the operation.Postoperative pathological tests suggested adrenal cortex adenocarcinoma in 1 case, pheochromocytoma in 1 case, gangliocytoma in 2 cases, and ganglioneuroblastoma in 1 case.Conclusions:Robot-assisted laparoscopic surgery is safe and feasible in children with adrenal tumors, but there are some limitations.This approach needs to be verified by further clinical research.

Series of rare diagnosis and presentations of abdominal mass in children: a great learning experience

Abdominal mass is a common finding in children, either palpable or radiologically evident surprisingly. Some of them are rare tumors. Functional active tumors are rarely palpable but present with varied arrays of symptoms. In this series authors are discussing few rare cases with atypical presentations such as- teratoma arising from adrenal gland, teratoma presenting with hypertension, fetus in fetu (Girl and Boy child), adrenocortical tumor presenting as precocious puberty and adrenal pheochromocytoma with features of cushing's syndrome. These atypical presentations may have pose a challenge in diagnosis and management for the treating team with first time occurrence specifically if they are handling them for first time.

Functioning adrenocortical carcinoma causing virilisation: a case report

Adrenocortical carticnoma (ACC) is an uncommon tumor with an incidence of 1-2 cases/million/year. It has two peak incidences; the first one in the first decade and the second one in the fourth decade. Most patients present with features of steroid hormone excess or abdominal mass effects, but about 15% of ACC are diagnosed incidentally. It is hormonally functional in 80 - 100% patients and the predisposing lesions include congenital adrenal hyperplasia and adenoma. ACC has significant syndromic and genetic association. Surgery offers the best chance of cure, especially in localized disease. Here, we present the case of virilization in a young female child secondary to a functioning ACC. The child had classical hormonal and imaging features of functioning ACC and underwent resection of the tumor (Adrenalectomy) with good outcome.

Oncocitoma suprarrenal / Oncocytic adrenocortical carcinoma

El oncocitoma suprarrenal es un tumor extremadamente infrecuente, con apenas unos 150 casos descriptos. Paciente de sexo masculino, de 48 años, con adrenalectomía derecha por incidentaloma suprarrenal de 7 cm, no funcionante. El oncocitoma suprarrenal no puede distinguirse desde el punto de vista clínico-imagenológico de otros tumores adrenales más frecuentes, por lo que su confirmación es histológica. Rara vez son malignos, y el tratamiento aceptado es la suprarrenalectomía, idealmente por vía laparoscópica. En nuestro caso, la presencia de dos criterios histológicos menores lo clasifican como un tumor con potencial maligno.

Oncocytic adrenocortical carcinoma is an extremely rare tumor, with only 150 cases reported in the literature. A 48-year-old male patient, with right adrenalectomy due to a 7-cm non-functional adrenal incidentaloma. From a clinical imaging point of view, oncocytic adrenocortical carcinoma cannot be distinguished from other more common adrenal tumors; therefore, the diagnostic confirmation is histological. It is rarely malignant, and the accepted treatment is laparoscopic adrenalectomy. In our case, two minor histological criteria classify it as a potentially malignant tumor.

Diagnóstico diferencial y cirugía mínimamente invasiva de un tumor suprarrenal de hallazgo antenatal / Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.

Abstract: Introduction: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. Objective: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. Case report: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. Conclusions: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.

Analysis of contrast-enhanced ultrasound performance of adrenal cortical adenoma and cortical hyperplasia nodules / 上海交通大学学报（医学版）

Objective • To explore the features of contrast-enhanced ultrasound (CEUS) image of adrenal cortical adenoma and cortical hyperplasia nodules (size greater than 10 mm) and its diagnostic value. Methods • Fifty cases of adrenal cortical adenoma and 22 cases of cortical hyperplasia nodules which were confirmed by pathology were retrospectively analyzed. The conventional ultrasound and CEUS features were recorded, including the size, border, arrival time, peak time, wash-out time, enhancement degree [hypo, hyper (iso-)], enhancement pattern (peripheral to central, overall, and nonspecific), etc. Results • There was no significant differences between two groups among conventional ultrasonic features. Multi-parameters of CEUS images analysis results showed that the wash-out time of cortical adenomas were significantly longer than the cortical hyperplasia nodules (P=0.002), the under area of the receiver operating characteristic (ROC) curve was 0.694, had the 75.7% sensitivity and 53.3% specificity with the cutoff of 42 s. Conclusion • There is no significant difference between the adrenal cortical adenoma and cortex hyperplasia nodules in CEUS pattern, but it still can provide certain information for differentiation.

Analysis of contrast-enhanced ultrasound performance of adrenal cortical adenoma and cortical hyperplasia nodules / 上海交通大学学报（医学版）

Objective · To explore the features of contrast-enhanced ultrasound (CEUS) image of adrenal cortical adenoma and cortical hyperplasia nodules (size greater than 10 mm) and its diagnostic value. Methods · Fifty cases of adrenal cortical adenoma and 22 cases of cortical hyperplasia nodules which were confirmed by pathology were retrospectively analyzed. The conventional ultrasound and CEUS features were recorded, including the size, border, arrival time, peak time, wash-out time, enhancement degree [hypo, hyper (iso-) ], enhancement pattern (peripheral to central, overall, and nonspecific), etc. Results · There was no significant differences between two groups among conventional ultrasonic features. Multi-parameters of CEUS images analysis results showed that the wash-out time of cortical adenomas were significantly longer than the cortical hyperplasia nodules (P=0.002), the under area of the receiver operating characteristic (ROC) curve was 0.694, had the 75.7％ sensitivity and 53.3％ specificity with the cutoff of 42 s.Conclusion · There is no significant difference between the adrenal cortical adenoma and cortex hyperplasia nodules in CEUS pattern, but it still can provide certain information for differentiation.

Diagnosis,treatment and prognosis of adrenal tumor in children / 中华实用儿科临床杂志

Objective To analyze the prognostic factors of adrenal tumor in children under 12 years of age. Methods A total of 90 children with 97 adrenal tumors admitted from June 2006 to August 2017 were selected in Children's Hospital of Nanjing Medical University.The age distribution,tumor type,biochemistry and tumor indicators, treatment,stage classification and prognosis were analyzed.Results There were 46 males and 44 females in 90 cases. Ages ranged from 4 days to 11 years and 1 month,with an average of (38.1 ± 31.3)months.The main clinical mani-festations were abdominal mass,fever and abdominal pain.Eighty cases (82.5%)underwent surgery,while 17 cases (17. 5%)did not.Open resection was performed in 48 cases,open partial resection in 11 cases,laparoscopic surgery in 10 cases,and just biopsy in 11 cases.The pathological examination showed 43 cases with neuroblastoma,13 cases with ganglioneuroblastoma,8 cases with ganglioneuroma,5 cases with adrenocortical carcinoma,3 cases with teratoma,1 case with pheochromocytoma,1 case with malignant rhabdoid tumor.Statistical analysis revealed that neuron－specific eno-lase(NSE)value of neuroblastoma and lactate dehydrogenase(LDH)value of cortical cancer increased significantly. The age was correlated with tumor stage,and patients had older age on stage Ⅳ.Complete resection in surgery was correlated with the stage of the tumor,as tumor in lower tumor stage seemed easier to be completely removed.Fifty－three cases (58.9%)were followed up for 2 months up to 11 years and 4 months.Forty－four cases survived and 9 ca-ses died.Higher tumor stage predicated worse prognosis.Conclusions Adrenal gland tumors need early diagnosis and active treatment.Earlier onset of age,complete surgical resection with patients have better prognosis.Complete resection of the disease was a key factor in the prognosis.

Differential diagnosis of adrenal metastasis and lipid-poor adrenal adenoma with CT / 中华内分泌外科杂志

Objective To study the CT characteristics of adrenal metastasis (AM) and lipid-poor adrenal adenoma (Lp-AA).Methods We retrospectively analyzed clinical and CT dates of AM (n=25) and Lp-AA (n=61) which were pathologically or clinically confirmed.The size,location,number,contour,cystic degeneration,calcification,hemorrhage and the clinical data were analyzed,as well as,CT value of tumor were measured and calculated.Receiver operating characteristic (ROC) curves were employed to assess the significant continuous variables to tell AM from Lp-AA.Results 34 adrenal lesions were detected for 25 cases of AM (9 case showed bilateral with 18 lesions).There were 65 lesions for 61 cases of Lp-AA (3 cases showed bilateral and 1case showed two lesions in unilateral side).Sex and age had significant difference between the two groups (P＜0.05).On the categorical variables of CT examination,number,cystic degeneration and contour had significant difference between the two groups (P＜0.05),while tumor location,hemorrhage and calcification had no significant difference (P＞0.05).For the continuous variables,plain scan CT value,enhancement amplitude,relative percentage washout (RPW) and tumor size had significant difference between the two groups (P＜0.05).Area under ROC curve analysis showed that when the plain scan CT value＞30.4 HU,area under ROC curve,sensitivity and specificity were 0.878,91.2％,and 72.3％,respectively.When RPW was ＞-33.4％,the area under ROC curve,sensitivity and specificity were 0.643,79.4％,64.7％,respectively.When the maximum and minimum diameter of tumor were ＞2.9 cm and 2.2 cm respectively,the area under the curve,sensitivity,specificity were 0.762,73.5％,75.4％ and 0.706,64.7％,69.2％,respectively.When venous phase increase and the largest increase were ＜37.7 HU and 37.3 HU respectively,the area under the curve,sensitivity,specificity were 0.805,89.2％,64.7％ and 0.833,95.4％,64.7％ respectively.Conclusion Tumor size,number,contour,cystic degeneration,CT measured and calculated value,as well as the patients' clinical data are of great value in differentiating AM and Lp-AA.

Experience of 243 cases in transperitoneal robotic-assisted laparoscopic surgery for adrenal diseases / 中华泌尿外科杂志

Objective To summarize our experience in robotic-assisted laparoscopic surgery for adrenal diseases Methods The clinical data of 243 patients with adrenal tumor treated by robotic-assisted laparoscopic surgery from March 2010 to February 2017 were retrospectively reviewed.There were 99 men and 144 women.The mean age was 51.6 years (range, 19-84).Tumors located at left adrenal in 140 cases, right in 97 cases,and both sides in 6 cases.The average diameter was 3.32 cm (range, 0.8-12 cm).However, there were 41 cases whose tumor diameter were greater than 5 cm.Results There were 2 cases of conversion during operation, 1 case converted to open surgery and the other to the traditional laparoscope surgery.The mean operative time was 35 min (range, 20-130 min).Estimated blood loss was 80 ml (range,20-1 200 ml).Blood transfusion was needed in 6 cases.The mean postoperative hospital stay was 5d (range, 3-20 d).The pathological diagnosis included 37 cases of pheochromocytoma, 149 cases of cortical adenoma, 3 cases of cortical carcinoma, 5 cases of metastatic tumor, 9 cases of adrenal myelolipoma, 3 cases of adrenal cyst, 2 cases of bronchogenic cyst, 25 cases of adrenal nodular hyperplasia,2 cases of angiomyolipomas, 1 case of mature teratoma, 1 case of diffuse large B-Cell lymphoma, 1 case of angioma, and 4 cases of neurofibromatosis.Conclusions Robotic-assisted laparoscopic adrenalectomy was safe and effective.Robotic-assisted laparoscopic surgery has the advantages over laparoscopic surgery in treatment of complicated adrenal tumor, such as large adrenal tumors, pheochromocytoma, bilateral adrenal diseases, overweight and obese patients with adrenal diseases.

Analysis of two cases of huge adrenal tumor / 基础医学与临床

Objective To investigate the clinicopathological characteristics, diagnosis and treatment of huge adrenal tumor.Methods Retrospectively analysed the clinical data of the 2 patients with huge adrenal tumor more than 10 cm in diameter, who were admitted in our hospital from September 2013 to January 2015.Clinical manifestations, treatment and prognosis were evaluated.Results Two patients were both suspected of adrenocortical carcinomas.Case 1 was a 28-year-old female.Laparoscopic surgery was performed at first but was conversed to open palliative adrenalectomy due to tumor rupture and bleeding.The postoperative pathology was adrenocortical carcinoma.The patient died of gastrointestinal bleeding caused by tumor metastasis 10 months later.Case 2 was a 39-year-old female, who underwent laparoscopic adrenolectomy with tumor excision completely.The postoperative pathology showed neoplasm of malignant potential, and no tumor recurred in 15 months follow-up.ConclusionsOpen surgery should be the priority if the diameter of adrenal tumor is more than 10 cm and diagnosis as suspicious of malignancy.Huge adrenocortical carcinoma is related to poor therapeutic response,and multimodality therapy may improve the prognosis.

Diagnosis and minimally invasive treatment of adrenal tumors / 中国肿瘤临床

Minimally invasive approach has been the gold standard therapy for adrenal tumor since 1992. The improvements of endo-crine test and radiographic options that are used to evaluate adrenal lesion relevance ratio have promoted the diagnosis and treat-ment. Future research may focus on secondary hypertension screening, subclinical situation of functional adrenal tumor, and differen-tial diagnosis through imaging tests and other methods. Subclinical hypercortisolism and the relationship between incidentaloma and metabolism should be given particular importance. Laparoscopic adrenalectomy has become the gold standard approach for adrenal benign tumor treatment. The indication for usage of this approach has extended and included adrenal carcinoma (tumor size ranging within 6-10 cm) and pheochromocytoma larger than 6 cm. Partial adrenalectomy mainly depends on surgeon's experience. In addi-tion, the perioperative period of minimally invasive surgery need further research. This paper reviews related studies.

Diagnosis and treatment of adult adrenal neuroblastoma / 局解手术学杂志

Objective To investigate the clinical characteristics of adult neuroblastoma,and improve the diagnosis and treatment level. Methods The clinical characteristics,imaging findings and pathological features of a 20-year-old patient with adult adrenal neuroblastoma in our hospital was analyzed with related reference.Results The frozen section was misdiagnosed as lymphoma for the large tumor with impor-tant vessels around,the cytoreductive surgery was performed,which was comfirmed as neuroblastoma by postoperative pathology.Conclusion Neuroblastomas are extremely rare in adults.These tumors often have peculiar features and behave differently than those in infants and chil-dren.Adjuvant chemotherapy and radiotherapy should be considered due to their aggressive nature.

The clinical analysis of adrenal tumor combined with renal carcinoma in 42 patients / 中华内分泌代谢杂志

Objective To summarize the clinical characteristics of adrenal tumors combined with renal carcinoma and to extend the clinical understanding of the situation.Methods To analyze the data of 1 100 patients with adrenal tumors hospitalized in Peking University First Hospital from Jan.1994 to Mar.2012 retrospectively,in the series there were 42 combined with renal carcinoma.Results There were 27 males,15 females,aged 29 to 83 years,mean 60.5 years old.In 42 renal carcinomas,there were 29 (69.0％) clear cell carcinomas,5 (11.9 ％) cystic renal carcinomas,2 (4.8％)clear cell with granulosa cell hybrid carcinomas,1 renal lymphoma,1 (2.4％) spindle cell tumor,1 acidophilic cell adenoma,and 1 undifferentiated type of renal cell carcinoma and pathologically not clear in 2 cases (4.8％).Clinical manifestations were not specific.In 42 adrenal tumors,20 situated in the left,and 17 right,and 5 were bilateral.35 cases were considered primary adrenal tumors,in which 3 were diagnosed primary hyperaldosteronism,32 were non-functional tumors.Other 7 were non-functional matastatic carcinomas.25 patients underwent resection of adrenal tumors,and pathology revealed 22 (88.0％) were primary,in which 12 (54.6％) were cortical adenomas,7 (31.9％) hyperplasia,1 (4.5 ％) pheochromocytoma,1 (4.5 ％) ganglioneuroma,1 (4.5 ％)adenoma with hyperplasia;2 (8.0％)metastatic tumors,and 1 (4.0％) non-Hodgkin' s lymphoma.The mean diameter of primary adrenal tumors was 1.9 cm,while that of metastatic carcinoma was 3.84 cm.25 underwent abdominal ultrasonography,40 underwent computed tomography (CT) scan,6 underwent magnetic resonance imaging (MRI) scan,positive rate was 72.0％,97.5％,and 100.0％ respectively.25 patients who underwent surgery were followed up,17 survived for 8 months-6 years,4 died,in which 1 metastatic carcinoma died of intraoperative bleeding,1 died of adrenal lymphoma,2 died because of other disease,4 were lost to follow-up.Conclusions The most common type of renal carcinoma combined with adrenal tumors was clear cell carcinoma,while majority of the adrenal tumors were primary with non-specific clinical manifestations and function.The adrenal tumors might be located in the same or the opposite side of renal carcinoma,also might be bilateral which were more common in metastatic carcinomas and always bigger than primary ones.CT/MRI scans were important in judging the nature of the tumors and pathology was the gold standard.The non-functioning primary adrenal tumors could be safely followed up,and the isolated metastatic carcinoma should be resected.Prognosis depends mainly on that of renal carcinoma.

Clinical study of 3D laparoscopic surgery for adrenal pheochromocytoma / paraganglioma / 中华泌尿外科杂志

Objective To investigate the efficacy and safety of 3D-laparoscopic surgery in adrenalpheochromocytomas /paragangliomas.Methods From December 2012 to July 2014,23 cases of adrenal pheochromocytomas/paragangliomas were treated by 3D-laparoscopic surgery.Among them,7 cases were male and 16 cases were female,whose mean age are 47 (32-68) years old.Their clinical symptoms including sustained hypertension in 11 cases,intermittent hypertension in 8 cases,sustained hypertension with intermittent progression in 2 cases and asymptom in 2 cases.All tumors were diagnosed by endocrine examination,image test and nuclear medicine.Mean diameter of tumors are 8 cm,ranged from 3-14cm.In order to adjust the blood pressure,patients took α-receptor blocker for 2-4 weeks preoperatively.Under the general anesthesia,19 patients received the 3D-laparoscopic surgery via retroperitoneal approach and 4 cases received the procedure via peritoneal approach.Results All operations were completed successfully.There were no major intraoperative complications.Mean operating time was (78 ± 21) min and mean estimated blood loss was (54.8 ± 36.3) ml.Mean hospital stay after operation was (3.8 ± 1.4) days.The pathological diagnosis included pheochromocytomas in 15 cases and paragangliomas in 8 cases.During the 3 to 18 months following up,no recurrence or metastasis was found in this study.Conclusions 3D-laparoscopic surgery has obvious advantage in spatial location and the sense of depth for adrenal pheochromocytomas /paragangliomas,which shortens the operation time and increase the safety obviously.

Conducta ante un incidentaloma suprarrenal / Behavior to be adopted to face adrenal incidentaloma

El incidentaloma suprarrenal en la actualidad es uno de los dilemas diagnóstico-terapéuticos mas frecuentes en la Endocrinología. Tiene un pico de aparición entre los 50 y los 70 años de edad, y se asocia a una mayor frecuencia de obesidad, diabetes mellitus e hipertensión arterial. La etiología es diversa, pero alrededor del 80 por ciento son adenomas benignos no funcionantes. A pesar de ello, la aparición de un carcinoma (por su agresividad) y disfunción hormonal (por la repercusión desfavorable sobre algunos factores de riesgo cardiovascular y el sistema óseo), pueden ensombrecer el pronóstico. La conducta estará basada en las definiciones mencionadas: naturaleza y función, y las 2 modalidades más empleadas son la cirugía adrenal (de preferencia laparoscópica, excepto si el tumor tiene criterios imagenológicos de malignidad); y la conservadora, que conlleva un seguimiento de, al menos, 5 años. El conocimiento de sus características, puede favorecer el mejor tratamiento a los pacientes con diagnóstico de un tumor suprarrenal incidental, y la garantía de una mejor calidad de vida(AU)

Adrenal incidentaloma is at present one of the commonest diagnostic/therapeutic dilemmas in endocrinology. It mainly occurs in the 50 to 70 years age group and is associated to higher frequency of obesity, diabetes mellitus and blood hypertension. Its etiology is varied, but around 80 percent of them are benign non-functioning adenomas. Despite the above-mentioned, the occurrence of carcinoma (based on aggressiveness) and hormonal dysfunction (negative impact on some cardiovascular risk factors and on the osseous system) can make prognosis gloomy. The behavior to be adopted will consider the stated definitions: nature and function, and the most used variants are adrenal surgery (mainly laparoscopic, except for a tumor with imaging-based malignancy criteria) and conservative surgery that implies at least five-year follow-up of the patient. Knowledge about the characteristics of adrenal incidentaloma may support a best treatment for patients suffering incidental adrenal tumor and assure them a better quality of life(AU)

Actualización en el diagnóstico por imágenes de los tumores adrenales / Update on imaging diagnosis of adrenal tumors

Cada vez es más frecuente el diagnóstico por imágenes de los tumores adrenales. La caracterización y diferenciación de los adenomas benignos y las lesiones malignas es muy importante, sobre todo, en el paciente con enfermedad maligna o funcionante. La tomografía axial computarizada, la resonancia magnética nuclear, y la tomografía con emisión de positrones acoplada a tomografía axial computarizada, permiten, con gran precisión, caracterizar y diferenciar, de forma no invasiva, las masas adrenales. En esta revisión se explican los principios fisiológicos en que se basan estas técnicas, sus ventajas y sus limitaciones. Se describen las principales características de imagen de los tumores suprarrenales más frecuentes, y se propone un algoritmo para el uso de estos medios diagnósticos(AU)

Imaging diagnosis of adrenal tumors is increasingly frequent. Characterization and differentiation of benign adenomas and malignant injures is very important, mainly in the patient with malignant or functioning disease. Computed tomography, magnetic resonance imaging and positron emission tomography/computed tomography allowed greater precision in characterizing and differentiating the adrenal masses in a non-invasive way. This review explained the physiological principles supporting these techniques, its advantages and restrictions. The main characteristics of the most frequent adrenal tumor images were described, in addition to submitting an algorithm for the use of these diagnostic means(AU)

Feocromocitoma maligno: comunicación de 2 casos tratados con cirugía de mínima invasión / Malignant pheochromocytoma treated with minimally invasive surgery

Introducción: El Feocromocitoma es un tumor de células cromafines productor de catecolaminas. Su variedad maligna es muy poco frecuente. Objetivo: Describir 2 casos de Feocromocitoma maligno tratados con cirugía mínimamente invasiva. Casos clínicos: Presentamos 2 pacientes de sexo femenino, de 31 y 51 años respectivamente. La primera con historia de hipertensión arterial de larga data y uso de múltiples fármacos. La segunda paciente debuta con dolor abdominal. En ambos casos los estudios radiológicos y metabólicos confirmaron el diagnóstico de Feocromocitoma. A la primera paciente se le realizó un procedimiento laparoscópico puro, mientras que a la segunda paciente se le realizó un procedimiento laparoscópico asistido por robot. En ambos casos fue necesario realizar adrenalectomía en bloque con el riñón, por compromiso del hilio renal. No hubo complicaciones severas intra ni post-operatorias, como tampoco conversión a cirugía abierta. La histopatología confirmó el diagnóstico de Feocromocitoma maligno con metástasis ganglionares regionales y márgenes quirúrgicos negativos. Ambas se mantienen en control, sin evidencia de recurrencia. Conclusión: El Feocromocitoma maligno puede ser manejado de forma mínimamente invasiva, con morbilidad y resultados oncológicos comparables a cirugía abierta.

Introduction: Pheochromocytoma is a tumor derived from chromaffin cells that produces cathecolamines. Malignant pheochromocytomas are extremely rare. Aim: To describe 2 cases of malignant Pheochromocytoma treated with pure laparoscopic and robot assisted surgery. Clinical cases: Two female patients, 31 and 51 years of age, presented with a history of high blood pressure and abdominal pain. Radiologic and metabolic studies confirmed the diagnosis of Pheochromocytoma in both patients. A pure laparoscopic approach was performed in the first patient while a robotic assisted laparoscopy was done in the second patient. In both cases, adrenalectomy with in bloc nephrectomy was performed due to compromise of the renal sinus. There were no complications or conversion to open surgery. Histopathology confirmed the diagnosis of malignant Pheochromocytoma with metastasis to regional nodes and negative surgical margins. At follow-up, there is no evidence of recurrence. Conclusion: Malignant Pheochromocytoma can be treated with minimally invasive surgery, with morbidity and oncological outcomes similar to open approach.