The efficacy and safety comparison of transperitoneal laparoscopic adrenalectomy and retroperitoneal laparoscopic adrenalectomy for adrenocortical carcinoma / 中华泌尿外科杂志

Objective:To compare the efficacy and safety of retroperitoneal laparoscopic adrenalectomy (RLA) and transperitoneal laparoscopic adrenalectomy (TLA) in the treatment of localized adrenocortical carcinoma (ACC).Methods:The data of 22 patients with stage Ⅰ/Ⅱ ACC underwent laparoscopic adrenalectomy in our institution from January 2009 to December 2018 were retrospectively analyzed. According to the different surgical approaches, these patients were divided into RLA and TLA groups. Eleven patients underwent RLA and 11 patients underwent TLA. There were no significant differences between the RLA group and the TLA group in terms of age at first diagnosis[44 (35, 54) vs. 46(41, 55) years, P= 0.793], sex (male/female: 3/8 vs. 4/7, P = 1.00), secreting tumor ratio (3/11 vs. 4/11, P = 1.00), tumor location (left/right: 6/6 vs. 7/4, P = 1.00), with hypertension or diabetes mellitus (4/11 vs. 3/11, P = 1.00). However, RLA has significantly smaller tumor size [3.0(2.5, 8.4) cm vs. 7.7(5.2, 8.4)cm, P= 0.001], and more stage Ⅰ patients [90.9%(10/11) vs. 18.2%(2/11), P=0.002], compared with those in TLA group. The perioperative indicators and oncology prognosis outcomes were collected and compared between the two groups. The Kaplan-Meier method was performed to calculate the overall survival (OS) and disease-free survival (DFS). Results:Compared with TLA, RLA had shorter operation time[90(70, 100) vs. 110 (90, 120) min, P = 0.005] and postoperative drainage tube removal time [2 (2, 3) vs. 3 (2, 6) day, P = 0.002), and the difference was statistically significant. In the TLA group, one patient was converted to open operation due to intraoperative tumor capsule rupture. For postoperative complications, one patient in the TLA group suffered with wound infection. There were no perioperative deaths in either group. All postoperative pathological examinations confirmed ACC, and there was no significant difference in Ki-67 index between the two groups [10%(3%, 35%) vs. 10%(9%, 25%), P = 0.484]. The median follow-up was similar in the two groups [48(26, 98) vs. 31(18, 49) months, P=0.237]. The local recurrence and metastasis rates were 36.4% for RLA group and 63.6% for TLA group ( P = 0.395). Survival analysis showed no statistically significant difference in DFS [5-year DFS rate: 33.6% vs. 73.2%, P = 0.118] between the two groups. The 5-year OS rates for RLA group versus TLA group were 58.3% vs. 45.5% ( P=0.485). Conclusions:For localized (stage Ⅰ/Ⅱ) ACC, both RLA and TLA seem safe and feasible, based on the similar long-term oncological prognosis. However, compared with TLA, RLA has the advantage of shorter operation time and postoperative drainage tube removal time. Due to the small number of cases included in this study, further multi-center, large-sample studies are required to demonstrate clear benefit of one surgical approach in the future.

Carcinoma de corteza suprarrenal. Presentación de un caso y revisión de la literatura

RESUMEN El carcinoma suprarrenal es un tumor raro pero devastador, esto se debe fundamentalmente a que en la mayoría de los casos se encuentra en estadios avanzados en el momento del diagnóstico. Tiene una incidencia de 0,5-2 casos por un millón habitantes al año. Entre el 40 y el 70 % de los pacientes tienen metástasis cuando se inicia el estudio. Se presenta de manera general en adultos, aunque también afecta a los niños. La mediana de edad en el momento del diagnóstico es 46 años, siendo más frecuente en el sexo masculino. Se presenta el caso de una paciente con antecedentes de hipertensión arterial de un año de evolución, ante los síntomas presentados se le realizan estudios por imágenes y de laboratorio diagnosticándose un tumor suprarrenal izquierdo. Se le realizó supraadrenalectomía izquierda obteniéndose como resultado histopatológico un carcinoma adenocortical suprarrenal. Se presenta este caso porque el carcinoma suprarrenal representa una entidad poco frecuente y es relevante exponer esta experiencia para el manejo de este tipo de neoplasia.

ABSTRACT Adrenal carcinoma is a rare devastating tumor, mainly because in most cases it is in advanced stages at the time of diagnosis. It has an incidence of 0.5-2 cases per one million inhabitants per year, in which 40 to 70 % of patients have metastases when the study begins. It occurs mainly in adults, although it also affects children. The median age at the time of diagnosis is 46 years, being more frequent in males. The case of a patient with a history of arterial hypertension of one year of evolution is presented. In view of the symptoms presented, imaging and laboratory studies are performed, diagnosing a left adrenal tumor. A left supraadrenelectomy was performed, obtaining an adenocortical adrenal carcinoma as a histopathological result.

Anesthesia management for resection of adrenal cortical carcinoma in children / 中华麻醉学杂志

The medical records of 11 pediatric patients undergoing resection of adrenal cortical carcinoma from January 2012 to January 2019 in our hospital were collected.Anesthesia management for resection of adrenal cortical carcinoma in children was analyzed and investigated.Anesthesia was induced by intravenously injecting atropine 0.01 mg/kg, dexamethasone 2-5 mg, propofol 2-3 mg/kg, sufentanil 0.3-0.5 μg/kg or fentanyl 1-2 μg/kg, rocuronium 0.5 mg/kg or cis-atracurium 0.1-0.2 mg/kg.Radial artery catheterization and femoral vein catheterization were performed under ultrasound guidance.Arterial blood pressure was continuously monitored.The esophageal thermometers probe was placed to continuously monitor body temperature.The catheter was placed to monitor urine volume.Intermittent positive pressure ventilation was performed after endotracheal intubation with the inspiratory oxygen fraction set 60%-100%, oxygen flow rate 2-3 L/min, tidal volume 7-10 ml/kg, ventilation frequency 20-26 times/min, inhalation/respiration ratio 1∶(1.5-2.0) and airway pressure 16-20 cmH 2O, and the end-tidal pressure of carbon dioxide was maintained at 35-45 mmHg.Anesthesia was maintained by inhaling 2%-4% sevoflurane and/or intravenously infusing propofol 0.10-0.15 mg·kg -1·min -1, and continuously infusing remifentanil 0.2-0.5 μg·kg -1·min -1.Hemodynamics was maintained within the normal range, and the bispectral index was maintained at 40-60 during the surgery.Before the tumor was completely removed, 5-10 mg/kg sodium hydrocortisone succinate was intravenously infused.At the end of the operation, sufentanil 0.75-1.00 μg·kg -1·d -1 or fentanyl 7.5-10.0 μg·kg -1·d -1 was continuously infused for postoperative analgesia until 48 h after operation.Operation was smoothly completed with stable anesthesia in all the pediatric patients.The tracheal tube was removed successfully after the operation.All children in this group were discharged from hospital and no death occurred.Anesthesia management for resection of adrenal cortical carcinoma required an appreciation of the clinical characteristics and perioperative pathophysiological changes.Paying attention to the changes in hormone levels during perioperative period and timely adjusting the children′s internal environment to maintain the stability of anesthesia and reduce the stress response were the keys to anesthesia management.

Late recurrence of adrenocortical carcinoma and metastatic disease. Case report / Recurrencia tardía de carcinoma adrenocortical con compromiso metastásico. Reporte de un caso

Abstract Introduction: Adrenocortical carcinoma (ACC) is a malignancy with an annual incidence of 0.72 cases per million people. It is a rare tumor that is associated with high mortality in late stages, as well as with a 5-year survival of 13% in stage IV patients, and 61% in stage II lesions. Nevertheless, tumor recurrence occurs in up to 54% at 23 months with predominance of locoregional involvement, being striking the late presentation of tumor recurrence with extensive involvement. Case presentation: This is the case of a 52-year-old male patient with a history of resection of an ACC of 6cm five years earlier, who was admitted for decompensated heart failure. A chest x-ray was taken and metastasis was suspected. After conducting biochemical studies and a CT scan of the abdomen, ACC with metastases to the liver and lung was diagnosed. The patient decided not to receive palliative chemotherapy. Conclusion: This unfortunate outcome is related to the lack of follow-up after the initial procedure. Clinical, hormonal, and imaging evaluation is recommended every 3 months for 2 years, and then at least every 6 months.

Resumen Introducción. El carcinoma adrenocortical (CAC) es una neoplasia que reporta incidencia anual de 0.72 casos por cada millón de personas. Se trata de un tumor infrecuente que se asocia con una mortalidad elevada en estadios avanzados y una supervivencia a 5 años del 13% de pacientes en estadio IV y del 61% para lesiones en estadio II; sin embargo, la presencia de recurrencia tumoral es hasta de 54% a los 23 meses con predominio de compromiso locoregional, siendo llamativa la presentación tardía de la recurrencia tumoral con compromiso extenso. Presentación del caso. Paciente masculino de 52 años con antecedente de resección de un CAC de 6cm de tamaño 5 años atrás, quien ingresa por falla cardíaca descompensada, encontrando en la radiografía de tórax una imagen sugestiva de metástasis. Tras estudios bioquímicos y tomografía de abdomen se diagnostica CAC con compromiso metastásico a hígado y pulmón. El paciente opta por no recibir manejo quimioterapéutico paliativo. Conclusión. Este desenlace desafortunado tiene relación con la ausencia de seguimiento tras el procedimiento inicial. Se recomienda evaluación clínica, hormonal e imagenológica cada 3 meses por 2 años y luego al menos cada 6 meses.

Clinical analysis of 11 cases of primary adrenocortical carcinoma / 中国基层医药

Objective@#To improve the diagnosis and treatment of primary adrenal cortical carcinoma.@*Methods@#The clinical and pathological data of 11 patients who diagnosed as primary adrenal cortical carcinoma by pathology were retrospectively analyzed.The median age was 43 years, including 6 males, 5 females.7 cases(63.6%) got the tumor on the left.9 cases(81.8%) presented the maximum diameter of tumor more than 6 cm, and the average maximum diameter was 12.9 cm.All cases had CT or MRI imaging examination.Among most of them, CT showed mixed density lesions, or MRI showed mixed signal.Only 3 cases(27.3%) were considered adrenal cortical cancer through preoperative examinations.@*Results@#11 patients were treated with surgical treatment.Of them, 9 cases(81.8%) had complete resection of tumor.3 cases were lost to follow-up, while other 8 cases were followed up for 5-58 months.3 cases presented tumor-specific death at 5-15 months after surgery, with each 1 case in AJCC stage Ⅱ, Ⅲ and Ⅳ, respectively.Survive were in 5cases, and 3cases of them were tumor-free survival, while the other 2 cases of them had local recurrence.@*Conclusion@#Adrenal cortical carcinoma is very difficult to diagnose preoperatively.Adrenal cortical cancer should be considered when tumor size more than 4cm and tumor presenting inhomogeneous enhancement.Then, the surgical treatment should be performed as soon as possible, and as appropriate, chemotherapy and other comprehensive treatment should be given subsequently.Clinical and basic research such as genes test may provide more treatment options for advanced tumors in future.

Correlation of ultrasound findings with clinical pathological types of adrenal cortical tumors / 中华全科医师杂志

Objective To investigate the correlation between ultrasound findings and clinical pathological types of adrenal cortical tumors(ACT).Methods The clinical data and ultrasonographic finding of 138 ACT patients undergoing surgical treatment in Affiliated Hospital of Qingdao University from January 2006 to December 2015 were retrospectively reviewed.The correlation between ultrasonic signs and pathological types of ACT was analyzed with Spearman method.Results There were 65 males and 73 females aged 18-71 years old.Among 138 cases,90 patients were characterized by hypertension,Cushing signs,Coon signs and abnormal sexual character, with the clinical diagnosis of adrenal cortical hyperplasia or adenoma;while 48 asymptomatic patients were first detected by ultrasonography.The coincidence rate of ultrasound and clinical pathological diagnosis was 92.0%(127/138),and 11 cases were misdiagnosed.The coincidence rate of ultrasound and surgical diagnosis was 94.2%(130/138), and 8 cases were misdiagnosed.The coincidence rate for no functional adenoma was 95.8%(46/48), for cortisol cortical adenoma was 94.1%(32/34),for aldosterone cortical adenoma was 93.6%(29/31),for cortical cysts was 9/10,for no functional adenocarcinoma was 5/6,for sexual abnormal cortical adenocarcinoma was 3/4,for cortisol cortical adenocarcinoma was 2/3,for mucous cortical adenoma was 1/2.The diameter of ACT was 0.6-11.5 cm.The low echo nodules of the adrenal region with a diameter <2.0 cm were associated with aldosterone cortical adenomas(r=0.485, P=0.01); the low echo or isoechoic nodules with a diameter 2.0-5.0 cm were associated with cortical adenomas or non-functional adenomas(r=0.567,0.452,P=0.01),and the circular non-echo nodules were associated with cortical cysts(r=0.483, P=0.01); the irregular nodules with a diameter >5.0 cm were associated with cortical adenocarcinoma(r=0.595, P=0.01).Conclusion The ultrasound image of ACT is related to different clinical pathological types,which can provide important information for clinical diagnosis and selection of surgical modalities.

Time-series analysis of expression profile of adrenocortical carcinoma in children for screening related targets / 天津医药

@#Objective To explore the potential pathogenic mechanism of adrenocortical carcinoma (ACT), and screen out genes that may be related to biological targets. Methods In this study, the gene expression datasets of ACT were obtained from the Gene Expression Omnibus (GEO) with the accession number of GSE75415. Through R programming software, the microarray preprocessing and differential expression analysis of 18 ACT tissue samples (experimental group) and 7 normal adrenocortical tissue samples (control group) were conducted to identify potential biomarkers for ACT in different stages. Besides, through functional enrichment and Kaplan-Meier analysis, several more reliable biomarkers for ACT were identified. At the same time, the two generation sequencing data of the TCGA database, including 79 ACT samples were analyzed, and the genes that can affect the survival of ACT patients were screened. Results There were 248, 334, 315 and 561 differentially expressed genes in stage1-4 respectively. There were 73 overlapping genes (OLDEGs) among the different grading samples. Central genes HSPA13, GARS, STXBP1, AKIRIN1 and TUBB3, were up-regulated in all of stages of ACT samples compared with those of normal samples, while, central genes ADH1B, DCN, RASSF2, PDGFRA, PLAT, C3 and FOS were down-regulated in ACT samples. They were found to be significantly associated with pathways of immune response, cell cycle, phosphorylation and cruor, which were all closely related with ACT progression. Besides, Kaplan-Meier analysis of 73 OLDEGs in 79 ACT samples from TCGA database identified several genes, including XPO1, RACGAP1, PDGFD, NR4A2, MXRA5, VPS51, TMED3, NDFIP1 and CDKN1C, which were significantly associated with ACT overall survival. Conclusion Differentially expressed genes and survival related genes in all of stages can serve as new targets for ACT therapy, and which should be helpful for the understanding of its pathogenesis and prognosis.

Análise da expressão e do silenciamento do receptor tipo 1 do fator de crescimento semelhante à insulina em tumores adrenocorticais humanos / Analysis of expression and silencing of insulin-like growth factor 1 receptor in human adrenocortical tumors

Introdução O sistema dos fatores de crescimento semelhantes à insulina (IGF) desempenha importante papel no crescimento e desenvolvimento celular normal. Hiperexpressão do gene IGF1R tem sido demonstrada em diversos tumores, sugerindo que a expressão deste receptor represente um pré-requisito fundamental para transformação celular. Nosso grupo de pesquisa demonstrou o aumento de expressão de IGF1R em tumores adrenocorticais pediátricos. Objetivos: Induzir o silenciamento do gene IGF1R por siRNA na linhagem de tumor adrenocortical humano NCI H295R, bem como avaliar os efeitos in vitro por meio da análise de proliferação celular e apoptose desta linhagem celular. Adicionalmente, avaliar a expressão de IGF-1R e de microRNAs relacionados a sua transcrição em tumores adrenocorticais humanos. Pacientes e métodos: A linhagem celular de carcinoma adrenocortical humano NCI H295R foi cultivada e submetida ao tratamento com 2 siRNAs específicos para IGF-1R. Todos os experimentos foram realizados em quatro grupos: (1) células não tratadas com siRNA, (2) células tratadas com siRNA # 1, (3) células tratadas com siRNA # 2 e (4) células tratadas com o siRNA controle negativo. A expressão gênica e proteica de IGF-1R foram determinadas por meio das técnicas de PCR em tempo real e Western Blot, respectivamente. Os efeitos do silenciamento de IGF-1R in vitro foram avaliados por ensaios de proliferação celular e análise de atividade de caspases. Além disso, 202 pacientes com tumor adrenocortical foram selecionados para o estudo de expressão proteica de IGF-1R por imunohistoquímica. Para avaliação de expressão de microRNAs relacionados à expressão de IGF-1R (miR-100, 375, 145 e 126) por PCR em tempo real foram selecionados 32 pacientes dos 202 disponíveis. Resultados: A expressão de IGF-1R foi significantemente diminuída nas células tratadas com siRNA # 1 e siRNA # 2. Os valores relativos de RNA mensageiro de IGF1R diminuíram aproximadamente 50% e as análises de Western Blot...

Introduction: The insulin-like growth factor (IGF) system plays a key role in normal cell growth and development. IGF1R overexpression has been demonstrated in several tumors suggesting that its expression is a prerequisite for cell transformation. We demonstrated IGF1R overexpression in pediatric adrenocortical tumors. Objectives: To induce IGF1R silencing by siRNA in a human adrenocortical cell line NCI H295R and evaluate its effects on cell proliferation and apoptosis. Additionally, evaluate the expression of IGF-1R protein and microRNAs related to its transcription in human adrenocortical tumors. Patients and methods: The human adrenocortical tumor cell line NCI H295R was cultured and treated with 2 specific IGF1R siRNA. All experiments were carried out in four groups: (1) untreated NCI H295R cells, (2) NCI H295R cells transfected with specific IGF1R siRNA # 1, (3) NCI H295R cells transfected with specific IGF1R siRNA # 2 and (4) NCI H295R cells transfected with a negative control. IGF-1R gene and protein expression was determined by the techniques of real-time PCR and Western blot, respectively. We assessed the effects of IGF-1R silencing on cell proliferation and apoptosis. Moreover, 202 patients with adrenocortical tumors were selected for the study of IGF-1R protein expression by immunohistochemistry. In the analysis of microRNAs that are related to IGF1R (miR-100, 375, 145 e 126) by real time PCR, 32 out 202 patients were selected. Results: IGF-1R levels were significantly decreased in cells that were treated with IGF-1R siRNA # 1 and siRNA # 2. The relative values of IGF1R mRNA decreased approximately 50% and Western blot analysis revealed a 30% of reduction in IGF-1R protein. Downregulation of this gene was accompanied by a reduction in 40% of cell growth in vitro and an increase in 45% of apoptosis. The analysis of microRNAs demonstrated that IGF1R expression is not correlated with the expression of these small RNAs. Additionally, the analysis of...

A Boy with Adrenocortical Tumor Who Progressed to Central Precocious Puberty after Surgery

Adrenocortical tumors in children are rare and show various clinical symptoms. We present an 8-year-old boy with peripheral precocious puberty caused by adrenocortical tumor. He showed elevated serum DHEA-S and 17-hydroxyprogesterone, and computed tomography revealed an abdominal mass. After surgical resection, he developed central precocious puberty. We report a rare case that showed progression from peripheral precocious puberty to central precocious puberty.

Neuroendocrinology of adrenalcortical tumors: A primary study / 第二军医大学学报

Objective: To investigate the neuroendocrine properties of adrenalcortical tumors. Methods: We retrospectively reviewed the clinical data of 99 adrenalcortical tumor patients, who were treated in Changzheng Hospital form June 1999 to June 2005. Expression of neuron specific enolase (NSE), chromogranin A (CgA) and synaptophysin (Syn) proteins were examined by immunohistochemistry (S-P method) using monoclonal antibodies. The general data of patients, including the age, symptoms, laboratory findings, and pathological types, were collected and subjected to statistical analysis with SAS v6. 12 software. Results: The expression of all the above 3 proteins was found in adrenalcortical adenoma tissues, with the positive rate of NSE being 80%, the positive rate of CgA being 48.9%, and the positive rate of Syn being 75.6%; the positive rates in the adrenalcortical carcinoma tissues were 77.8%, 22.2%, and 77.8%, respectively; and those in the normal adrenal tissues was 20%, 0%, and 10%, respectively. The positive rates of 3 proteins in adrenalcortical tumors was significantly higher than those in the normal adrenal tissues (P

Virilizing Adrenocortical Oncocytoma in a Child: A Case Report

Functioning adrenocortical oncocytomas are extremely rare and most reported patients are 40-60 yr of age. To our knowledge, only 2 cases of functioning adrenocortical oncocytomas have been reported in childhood. We report a case of functioning adrenocortical oncocytoma in a 14-yr-old female child presenting with virilization. She presented with deepening of the voice and excessive hair growth, and elevation of plasma testosterone and dehydroepiandrosterone sulfate. She had an adrenalectomy. The completely resected tumor composed predominantly of oncocytes without atypical mitosis and necrosis. A discussion of this case and a review of the literature on this entity are presented.

Myxoid adrenocortical adenoma:a case report and literature review / 中华泌尿外科杂志

Objective To investigate the clinical-pathological feature and treatment of mvxoid adrenocortieal adenoma. Methods The clinical features of a Myxoid adrenocortical adenoma were re-viewed with its clinical manifestation,imaging,pathology and therapy.The patient was a 43-vear-old woman.The patient complained of recurrent headache,fatigue with hypertension and hypokalemia for 10 years.B-ultrasound examination revealed a 4.1 crux 3.4 cm hypoechoic solid mass in the left adre-nal gland. Relative literatures were reviewed. Results The patient was operated successfully by 1ap-aroscope.The section of tumor showed gray-like flour with yellow jelly-like substance.Hemorrhage was seen in some area. Under light microscope,the tumor cells were similar in size with boundary.There was no obvious heteromorphism and mitotic feature.There was full of mucoid substance.Im-munohitochemical staining showed that the tumor cells were positive for Vimentin,Melan-A and AB/ PAS staining,which confirmed the diagnosis of myxoid adrenocortical adenoraa. Blood pressure andbiochemical indicators returned to normal after surgery.There was no sign of recurrence after 6 month follow-up. Conclusions Myxoid adrenocortical adenoma is a rare adrenal tumor.Path010gy is the key to diagnosis.Surgical removal of tumor could be the preferred therapeutic choice.

A Case of Adrenocortical Tumor in a 16-Month-Old Boy Presenting with Virilization / 대한소아내분비학회지

A 16-month-old boy had signs of virilization such as penile enlargement and pubic hair since 13 months of age. Hormone evaluation indicated increased levels of 17-OH-progesterone, dehydroepiandrosterone- sulfate (DHEA-S) and testosterone. Initially he was diagnosed as simple virilizing type of congenital adrenal hyperplasia and hydrocortisone was given to him. After 2 months, his penile enlargement continued and we did not find gene mutation for 21-hydroxylase deficiency. Adrenal magnetic resonance imaging revealed a 3.5x2.7x4.4 cm size of adrenal mass consistent with an adrenocortical tumor. The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormone levels. Childhood adrenocortical tumors are rare and are not well characterized. Surgery is the definitive treatment for adrenocortical tumors and prognosis is often poor. We report a case of adrenocortical tumor in a 16-month-old boy presenting with virilization with review of literature.

Malignant adrenal tumors (report of 50 cases) / 中华泌尿外科杂志

Objective To improve the diagnosis and treatment for malignant adrenal tumors. Methods Clinical data of 50 cases of malignant adrenal tumors were retrospectively analyzed.Of them 7 cases of corticoadrenal carcinoma with endocrine function presented with relevant symptoms and endocrine abnormality.Among 23 cases of malignant pheochromocytoma high blood pressure existed in 19 and 18 had a high level of urinary catecholamine.Fifteen cases had corticoadrenal carcinoma without endocrine function and 5 cases developed adrenal metastic carcinoma.All of the patients underwent endocrinological and image examination before operation. Results Among the 27 cases of corticoadrenal carcinoma or metastasis,25 were confirmed by pathology and 2 were confirmed during follow-up.Of the 23 patients who were diagnosed as malignant pheochromocytoma,11 were confirmed to have extensive tumorous infiltration or metastasis by imaging procedures and operations and 12 were detected by follow-up to have metastasis.Tumors were resected in 14 cases of corticoadrenal carcinoma,8 of whom survived for 1～5 years.Twelve cases of malignant pheochromocytoma underwent radical operations with a survival of 1～14 years. Conclusions Most cases of corticoadrenal carcinoma can be diagnosed by pathology or image findings of metastasis.Pheochromocytoma is more likely to be malignant when it recurs,expecially for the extro-adrenal pheochromocytoma.The diagnosis of malignant pheochromocytoma must depend on the evidence of tumorous infiltration or invasion of the tissue without para ganglions found by imaging procedures or pathology.The prognosis of the late-stage corticoadrenal carcinoma is poor.For the cases of late-stage malignant pheochromocytoma dibenzyline or 131 I-MIBG treatment can extend their lives.

Adrenal cortical carcinoma (report of 12 cases) / 中华泌尿外科杂志

Objective To discuss the diagnosis and management of adrenal cortical carcinoma. Methods 12 cases of adrenal cortical carcinoma were evaluated with regard to clinical,pathologic characteristics and prognosis.Of the 12 patients,6 were male and 6 were female with an average age of 37.The tumor was functional in 4 and nonfunctional in 8.The tumor was on the right side in 10 and on the left in 2.The diameter of the tumor was 3～30 cm.3 cases were Sullivan stage Ⅰ,3 stage Ⅱ,2 stage Ⅲ and 4 stage Ⅳ. Results Tolal ablation of the tumor has been conducted in 11 and biopsy only in 1.Estrogen receptor expression was positive in 5 out of 7 as deteted by immunohistochemistry assay.Five patient died within 1.5 years after operation whereas the other 7 are still surviving including 5 administered with tamoxifen. Conclusions Adrenal cortical carcinoma may be an estrogen-dependant tumor,so tamoxifen is an effective adjuvant drug in the treatment.Tumor size and stage appear to be strongly correlated with prognosis,larger size and higher stage are associated with a poor prognosis.

Clinical diagnosis and management of adrenocortical carcinoma in children / 中华泌尿外科杂志

Objective To study the clinical features of adrenocortical carcinoma in children. Methods From 1983 to 1999, 9 cases of adrenocortical carcinoma were reviewed retrospectively. Results Six cases were surgically treated, complete tumor resection in four cases and palliative tumor resection in two.All were female. All the operated cases were followed up for an average of 2 years and 3 months.The 2 year survival is 4 out of 6. Conclusions Early diagnosis and complete tumor resection is the only means to achieve good survival in children with adrenocortical carcinoma.

Neuroendocrinology of adrenalcortical tumors:a primary study / 第二军医大学学报

Objective:To investigate the neuroendocrine properties of adrenalcortical tumors.Methods: We retrospectively reviewed the clinical data of 99 adrenalcortical tumor patients,who were treated in Changzheng Hospital form June 1999 to June 2005.Expression of neuron specific enolase(NSE),chromogranin A(CgA) and synaptophysin(Syn) proteins were examined by immunohistochemistry(S-P method) using monoclonal antibodies.The general data of patients,including the age,symptoms,laboratory findings,and pathological types,were collected and subjected to statistical analysis with SAS v6.12 software.Results: The expression of all the above 3 proteins was found in adrenalcortical adenoma tissues,with the positive rate of NSE being 80%,the positive rate of CgA being 48.9%,and the positive rate of Syn being 75.6%;the positive rates in the adrenalcortical carcinoma tissues were 77.8%,22.2%,and 77.8%,respectively;and those in the normal adrenal tissues was 20%,0%,and 10%,respectively.The positive rates of 3 proteins in adrenalcortical tumors was significantly higher than those in the normal adrenal tissues(P

Partial remission with transarterial embolization in a case of metastatic adrenal cortical carcinoma

A case of metastatic adrenal cortical carcinoma in which partial remission was achieved with transarterial embolization is presented as probably the first reported case in the literature to date. A 29-year-old woman was admitted because of adrenal cortical carcinoma which had not responded to mitotane. A left adrenalectomy with segmentectomy of the involved liver had been done previously. Abdominal computerized tomography demonstrated multiple large metastatic tumors in the liver. Transarterial embolization with Gelfoam and 20 mCi of 131I-labeled lipiodol was performed and resulted in a decrease in tumor size and biochemical parameters. Transarterial embolization can be one of the therapeutic modalities for metastatic adrenal cortical carcinomas.