Carcinoma adrenocortical: Presentación y desenlaces en una institución oncológica / Adrenocortical carcinoma: Presentation and outcomes at an oncological institute

Resumen Introducción: El carcinoma adrenocortical es una neoplasia endocrina infrecuente pero con un comportamiento altamente agresivo y pobre pronóstico. Dado su baja prevalencia, la experiencia de los centros de referencia es fundamental para aumentar el conocimiento de esta entidad. Métodos: Se elaboró una serie de casos de pacientes con carcinoma adrenocortical, tratados en una institución oncológica de referencia entre enero de 2007 y diciembre de 2017. Se describieron las características clínicas e histopatológicas de los pacientes. Se estimó el tiempo de supervivencia libre de progresión y el tiempo de supervivencia global (SG) de forma gráfica y con funciones de tiempo al evento mediante la función de Kaplan-Meier. Resultados: Se identificaron 19 pacientes, 14 de los cuales fueron mujeres con edad media al diagnóstico de 43.4 años (rango 20 - 65). El 58% de los pacientes tuvo secreción hormonal, siendo el síndrome de Cushing el predominante. 7 pacientes tuvieron compromiso metastásico al momento del diagnóstico. Todos los pacientes fueron llevados a adrenalectomía y el estado postquirúrgico en 10 pacientes fue R0. Al final del periodo de estudio, 11 pacientes estaban vivos. La mediana de supervivencia libre de progresión fue de 18 meses +/- 7.86 y la mediana de supervivencia global fue de 30 meses +/-19.80. Conclusión: En la población de pacientes analizada, se encontraron desenlaces de supervivencia libre de progresión y supervivencia global similares a lo reportado en centros de alta experiencia en patología adrenal.

Abstract Introduction: Adrenocortical carcinoma is a rare endocrine neoplasm, but with highly aggressive behavior and a poor prognosis. Given its low prevalence, the experience of reference centers is essential to characterize the factors associated with this disease. Methods: It is a case series of patients with adrenocortical carcinoma, treated at a reference oncology institute between January 2007 and December 2017. The clinical and histopathological characteristics of patients are described. Progression-free survival and overall survival (OS) were estimated graphically and with time-to-event data using the Kaplan-Meier function. Results: 19 patients were identified; 14 of them were women with a mean age at diagnosis of 43.4 years (range 20-65). 58% of the patients had hormone secretion, with Cushing's syndrome being the predominant one. 7 patients had metastatic compromise at the time of diagnosis. All patients underwent adrenalectomy, and R0 was the post-surgical status in 10 of them. At the end of the study period, 11 patients were alive. The median progression-free survival was 18 months +/- 7.86, and the median overall survival was 30 months +/- 19.8. Conclusion: In the analyzed patient population, outcomes of progression-free survival and overall survival were similar to that reported at centers with extensive experience in adrenal disease.

One case of heterotopic adrenal cortical adenocarcinoma / 中华内分泌外科杂志

Adrenal cortical adenocarcinoma is a rare and often aggressive malignant tumor. For ACC that originated outside the adrenal gland, there are few reports at home and abroad. We report a case of adrenocortical adenocarcinoma that originated from kidney, introduce its diagnosis, treatment and follow-up results in order to provide help for clinical diagnosis and treatment.

A case of adrenal cortical carcinoma with inferior vena cava thrombus extending into the right atrium: a two staged procedure

We present a case of adrenal cortical carcinoma (ACC) with tumor thrombus involving the inferior vena cava (IVC) and right atrium in a morbidly obese, middle-aged female. ACC is a rare type of cancer with a poor outcome. Most cases present with metastasis at the time of initial presentation. This patient presented with breathing difficulty and flank pain. With endocrinology evaluation and collaborative effort of multidisciplinary teams, the patient successfully underwent staged procedures of atrial clot evacuation after thoracotomy with cardiopulmonary bypass (CBP) along with inferior vena cava thrombectomy (IVCT) and left radical nephroadrenalectomy. The two staged procedure reduces the mortality when compared with a single staged procedure. The patient underwent atrial clot evacuation, performed by cardiothoracic surgery team under CBP and deployment of IVC filter by interventional radiologist as a first staged procedure. At six months of follow-up, the patient had locoregional disease spread but with good functional status. This case report highlights that even a high-volume disease, with proper planning by an experienced surgical team, can be operated successfully with an acceptable post-surgery quality of life for the patients.

Pre-operative 8 am plasma total cortisol level predicts survival for adrenal cortical carcinoma after surgery / 中华泌尿外科杂志

Objective To investigate the effects of preoperative 8 am plasma total cortisol level (PTC) on the prognosis of adrenocortical carcinoma (ACC).Methods A total of 44 patients from January 2009 to October 2017 with ACC were included,PTC level within one week before surgery,clinical and pathological data were collected.The mean age of the patients was (45.2 ± 15.2) years.There were 27 females and 17 males.The mean tumor diameter was (63.6 ± 20.7) mm.There were 20 cases with functional ACC,in which 18 patients presented symptoms associated with Cushing syndrome.According to ENSAT stage,9 patients were staged as stage Ⅰ,and 35 were staged as stage Ⅱ.The median pre-operative 8 am PTC was 572.6 nmol/L,range from 89.3 to 1 118.7 nmol/L.Open adrenalectomy was employed in 23 patients and laparoscopic approach in 21 patients.The optimal cut-off value of pre-operative PTC was determined by receiver operating characteristic curve (ROC) and patients were divided into two groups with high PTC and low PTC values.Kaplan-Meier method was used to draw the survival curve,univariate and multivariate Cox regression were used to explore factors influencing the prognosis of ACC patients.Results The median follow-up duration was 42 months,range from 3-104 months.The optimal cut-off value was determined as 476.2 nmol/L,baseline and clinic-pathologic characteristics differences between two groups were not statistical significant (P ＞ 0.05).The overall survival (OS) in the high PTC group was shorter than that in the low PTC group [median 18months (20-104 months) vs.40 months (5-98 months),P =0.014],and the recurrence free survival (RFS) of patients in the high PTC group was also shorter than that in the low PTC [median 26 months (0-104 months) vs.50 months (5-98 months),P =0.028).In univariate analysis,age,gender,tumor stage,preoperative PTC,and symptoms were correlated with postoperative OS and RFS (P ＜ 0.05).After adjusting for age,gender,tumor stage,symptoms,multivariate Cox regression showed that pre-operative high PTC was an independent prognostic factor associated with a decreased OS (HR =2.086,95 ％ CI 1.495-2.287,P =0.014) and decreased RFS (HR =2.234,95％ CI 1.546-2.334,P =0.045).Conclusion The preoperative morning PTC is an independent risk factor for the prognosis of ACC.

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.

Analysis of risk factors of herpes zoster after renal transplantation in a single center / 器官移植

Objective To investigate the clinical characteristics and risk factors of the incidence of herpes zoster after renal transplantation. Methods Clinical data of 830 recipients undergoing renal transplantation for the first time in the Organ Transplantation Research Institute of the 309th Hospital of Chinese People's Liberation Army from March 2009 to March 2012 were retrospectively analyzed. Univariate and multivariate Logistic regression analyses were performed to identify the risk factors of the incidence of herpes zoster after renal transplantation. Results Among 830 patients, 42 (5.1%) suffered from herpes zoster postoperatively. Clinical manifestations of herpes zoster mainly included varicella-zoster rash in the head, face, trunk and limbs. No patient died from herpes zoster. Post-herpetic neuralgia (PHN) was the most common complication of herpes zoster. Univariate Logistic regression analysis revealed that advanced age and adrenal cortical hormone (hormone) shock therapy could increase the risk of herpes zoster viral infection after renal transplantation (OR=2.414, P=0.016; OR=2.936, P=0.003). Multivariate Logistic regression analysis demonstrated that advanced age and hormone shock therapy were the independent risk factor of the incidence of herpes zoster following renal transplantation (OR=2.238, P=0.030; OR=2.755, P=0.005). Conclusions Herpes zoster after renal transplantation is clinically manifested with varicellazoster rash. Advanced age and hormone shock therapy are the independent risk factor of the incidence of herpes zoster after renal transplantation.

Effect of adriamycin on the characteristics of colony derived from human adrenal cortical carcinoma cells SW-13 / 实用医学杂志

Objective To explore the effect of adriamycin on the characteristics of colony derived from human adrenal cortical carcinoma cells (ACC) SW-13.Methods Treatment with Adriamycin (ADM) was used in BALB/c-nude mouse tumor xenograft model established using the ACC cell line SW-13.The characteristic of colony was assessed for the formation rates,the percentagc of three colony types and growth curve of single cell.Hoechst33342 dyeing test was used to test drug resistance.Results The Single-cell colony formation rate of experimental group were significantly higher than control group (P ＜ 0.05),and the holoclone percentage of experimental group were significantly higher than control group (P ＜ 0.05).In the Hoechst33342 dyeing tcst,the fluorescence intensity of control was higher than experimental group.Conclusion The treatment of ADM in vivo is beneficial for the colony formations of ACC cell and the formations rate of holoclone,and can improve the ability of drug resistance of ACC cell SW-13.

Diffuse alveolar hemorrhage syndrome after renal transplantation:one case report and literature review / 器官移植

Objective To analyze and summarize clinical characteristics and treatment of diffuse alveolar hemorrhage syndrome (DAHS)complication after renal transplantation.Methods Clinical data of one patient,admitted to the 309 th Hospital of People's Liberation Army in December 201 2, who was complicated with DAHS after renal transplantation,were obtained.The incidence,diagnosis and treatment courses of DAHS were retrospectively analyzed.Literature review was conducted to summarize clinical experience.Results The patient was clinically manifested with respiratory failure,progressive aggravation of hemoptysis and anemia.Imaging examination revealed that diffusive infiltration of bilateral lungs was aggravated.After the diagnosis of DAHS was confirmed,adrenal cortical hormone (hormone)shock and anti-infectious medication therapies were timely delivered to actively prevent and treat relevant complications.The patient was successfully healed.Until the submission date,the patient presented with normal renal function and no pulmonary complications were noted.Conclusions DAHS is a rare and fatal complication after renal transplantation.Early diagnosis, active anti-infection therapy and timely administration of large-dose hormone shock treatment determine the success of clinical treatment.

Changes of adrenal cortical function in children with growth hormone deficiency before and after treatment with recombinant human growth hormone / 临床儿科杂志

Objective To observe the changes of adrenal cortical function in children with growth hormone deficiency before and after treatment with recombinant human growth hormone. Methods Seventy-two children diagnosed with GHD who received rhGH treatment for no less than 6 month were included, among whom there were 32 children who were accompanied with adrenal cortical hormone (ACTH) deficiency. The changes of fasting plasma cortisol (COR) and ACTH levels before and 3 and 6 months after treatment with rhGH were retrospectively analyzed. Results Thirty-two children with ACTH deficiency were treated with rhGH after COR level reached normal by the supplementing exogenous hydrocortisone. There was negative correlation between COR level before treatment and the HC dose that make the COR reach normal lowest limit level (r?=?-0.899, P??0.05). The level of COR was obviously decreased after treatment with rhGH, and there was statistical difference compared with that before treatment (P?

A pedigree of multiple endocrine neoplasia type 1 with the proband initially presenting adrenal cortical carcinoma / 中华内分泌代谢杂志

[Summary]_ This is a pedigree of multiple endocrine neoplasia type 1(MEN1). The proband pursuit medical assistance because of hypertension and weakness. Adrenal cortical carcinoma with possible Cushing's syndrome was diagnosed after a series of tests. During this process, the proband was found to have hypercalcemia, and he was diagnosed as primary hyperparathyroidism. Adrenal carcinoma plus primary hyperparathyroidism suggested MEN1, which was confirmed by MEN1 gene 400_401insC mutation. Pedigree investigation found six additional patients, including one with high parathyroid hormone level and two without clinical evidence of any MEN1 diseases. The proband died of metastatic malignancy 7 months after diagnosis while the other 3 patients with clinically confirmed MEN1 tumor responded well to surgery, including one with adrenal cortical carcinoma.

Adrenal cortical carcinoma in Thailand: A review on the previous reported case.

Background: Adrenal cortical carcinoma (ACC) is an uncommon cancer. Materials and Methods: The author performed a literature review on the reports of ACC in Thailand in order to summarize the characteristics of this rare cancer among Thai patients. Results: According to this study, there have been at least seven reports in the literature of nine individual cases of ACC, of which none was lethal directly to ACC. Conclusions: Here, the ACC is sporadically reported in Thailand. The diagnosis of ACC is usually by detection adrenal mass using imaging technique and the confirmation for the nature of cancer is usually by histopathology. Nevertheless, among the Thai population, the prognostic outcome is usually good.

Adrenal Medullary Hyperplasia with Coexisting Adrenal Cortical Adenoma / 대한내분비외과학회지

Adrenal medullary hyperplasia is an increase in the mass of the adrenal medullary cells. We report a case of a 38-year-old man presenting with pheochromocytoma-like symptoms who was preoperatively misdiagnosed with pheochromocytoma. Hypertension was associated with an intracranial hemorrhage evident in a brain computed tomography scan, in which no obvious pituitary gland enlargement was detected. An abdominopelvic CT revealed a solitary tumor in the right adrenal gland with no obvious enlargement of the contralateral adrenal gland or sympathetic chains. Lab results showed increased levels of urinary metanephrines. Based on clinical data, the patient underwent a laparoscopic right adrenalectomy bases on a diagnosis of pheochromocytoma. The patient was finally diagnosed with adrenal medullary hyperplasia with coexisting ipsilateral non-functioning adrenal cortical adenoma. Postoperatively, blood pressure and lab results were maintained in the normal range and the patient was symptomatically free during the follow-up period.

Functional Adrenocortical Oncocytoma: A Case Report of Rare Neoplasm of Uncertain Malignant Potential

Adrenocortical oncocytoma is a rare adrenal neoplasm with only 25 cases having been reported in the English medical literature, of which only seven were functional tumors. Since these adrenal tumors are usually nonfunctional, they are mostly incidentally detected, and most of them are benign. Herein, we report on a rare case of a functional adrenocortical oncocytoma of an uncertain malignant potential and this tumor was located in the left adrenal gland in a 59-year-old woman who presented with hypertension. The tumor size was large with foci of necrosis in the cut surface and it exclusively had oncocytic histologic features.

An Adrenal Cortical Adenoma with Adrenal Medullary Hyperplasia / 대한내과학회지

The relationship between the adrenal cortex and medulla has been studied since the 1960s. Rarely, a patient with an adrenal cortical adenoma presents with the findings of pheochromocytoma. However, there has been no report of a case with the clinical features of pheochromocytoma showing the pathological features of an adrenal cortical adenoma with medullary hyperplasia on histological examination. We report a 59-year-old-man who was shown to have an adrenal cortical adenoma, with medullary hyperplasia, during a diagnostic work up for pheochromocytoma.

Adenoma cortical suprarrenal, hipercortisolismo, cardiomiopatía y bazo accesorio intrapancreático en un lactante / Cortical adrenal adenoma, hypercortisolism, cardiomyopathy, and intra-pancreatic accessory spleen in a 3-month-old infant

Introducción. El síndrome de Cushing es el resultado de concentraciones elevadas de glucocorticoides en la circulación, su presentación en lactantes es poco común. Caso clínico. Presentamos un caso de hipercortisolismo endógeno en un niño de 3 meses de edad, secundario a un adenoma suprarrenal congénito. El paciente manifestó polifagia, aumento de peso y alteraciones en el patrón del sueño; al examen físico se observó hirsutismo frontal, cara en "luna llena", abultamiento visible en región cervico-dorsal ("giba de búfalo"), hipertensión arterial; el cortisol plasmático fue de 163 pg/dL, y el ultrasonido abdominal reveló tumoración suprarrenal izquierda. Durante el período de hospitalización, el niño cursó con dificultad respiratoria y taquicardia; la radiografía de tórax mostró cardiomegalia. Después de un procedimiento quirúrgico simple (venodisección), el paciente desarrolló choque cardiogénico y falleció. En la autopsia se encontró, además de la tumoración suprarrenal, cardiomiopatía hipertrófica y bazo accesorio intrapancreático. Conclusión. La causa más común del síndrome de Cushing en todos los grupos de edad, excepto en lactantes, es hipercortisolismo exógeno; la asociación de patologías aquí presentadas es muy raro.

Introduction. Cushing syndrome is associated with high levels of glucocorticoids in the circulation and is infrequently in infants. Case report. We present a case of congenital cortical adrenal adenoma-associated endogenous hypercortisolism in a 3-month-old infant. The patient manifested polyphagia, weight gain, and changes in sleep patterns. During physical examination we found a full-moon face, bulkiness in the cervico-dorsal "buffalo-hump" region, high blood pressure, and serum cortisol of 163 pg/dL. Abdominal ultrasound revealed left adrenal tumor. During hospitalization, the patient experienced respiratory difficulty and tachycardia, and thoracic X- ray revealed cardiomegaly. After a simple surgical procedure (venous dissection), the patient developed cardiogenic shock and died. At autopsy, adrenal tumor was found in addition to hypertrophic cardiomyopathy and intrapancreatic accessory spleen. Conclusion. The most common cause of Cushing syndrome in all age groups is exogenous hypercortisolism, except in infants. The associated pathologies described in this article are rare.

Expressions of multiple proteins in adrenal tumors and the clinical significance / 中华泌尿外科杂志

Objective To investigate the expressions of Ki67,p53,CKL,EMA,S-lOO,NSE,CgA,Syn,CEA and nm23 in adrenal tumors and their clinical significance.Methods Clinical data from 157 cases of adre-nal tumor patients were retrospectively reviewed including the clinical informa-tion and pathology data.Expressions of Ki67,p53,CKL,EMA,S-100,NSE,CgA,Syn,CEA and nm23 proteins were studied by immunohistochemistry(SP method)using monoclonal antibodies,and the relationship of their expressions with histopathologic type and clinical imformation was analyzed with SAS v6.12 software.A P value of＜0.05 was considered statistically significant.Results A increase of the expression rate of CKL,S-100,NSE,CgA,Syn and nm23 in adrenal tumors was obsered(P＜0.05).For univariate analysis,the expression of S-100,CgA,Syn was in connection with histopathologie types(P＜0.05).The expression of S-100,CgA,Syn had positive correlation with each other.The expression of CKL,S-100,NSE,CgA,Syn,nm-23 was no difference between adrnalbenign tumors and malignant tumors(P＞0.05),but it was much higher than in normal adrenal tissues(P＜0.05).The expression of Syn in adrenal cortical adenomaa was higher than in adrenal cortical cancers(P＜0.05),the expression of Ki67 in adrenal cortical adenomas was much lower than in adrenal cortical cancers(P＜0.05).The expression of EMA、CKL in adrenal cortical tumors were higher than in adrenal medullary tumors(P＜0.05),the expression of S-100,Syn,NSE,CgA in adrenal cortical tumors were lower than in adrenal medullary tumors(P＜0.05).Conclusions CKL,S-100,NSE,CgA,Syn and nm23 were good markers for adrenal tumors,they could be use for the adrenal tumors diagnosis.Detect Syn and Ki67 simutaneously was helpful to the diagnosis of adrenal cortical tumors.Detect EMA,CKL,S-100,Syn,NSE and CgA simultaneously and combine with clinical data was helpful to diagnosis between adrenal cortical tumors and adrenal medullay tumors.In malignant tumors,blood pressure had positive correlation with the expression of CgA,the size of tumor had neg-ative correlation of blood pressure,no prognostic factor was found.

Estrogen Producing Adrenocortical Carcinoma / 대한비뇨기과학회지

An estrogen producing adrenocortical carcinoma is a rare, highly malignant tumor. Fewer than 100 cases of feminizing adrenocortical carcinomas in men have been reported. These tumors are usually large in size and show high levels of serum total estrogen and estradiol. Recently, two cases of estrogen producing adrenocortical carcinoma were experienced by our selves during in year. A unilateral adrenalectomy, with mass excision, was performed in both patients, with an ipsilateral nephrectomy performed in just one. Mitotane or cisplatin based adjuvant chemotherapy was also performed.

A Case of Cushing's Syndrome in Pregnancy Secondary to an Adrenal Cortical Adenoma

Cushing's syndrome in pregnant women is rare and difficult to be diagnosed because of the syndrome's association with oligomenorrhea or amenorrhea and the changes in cortisol metabolism during normal pregnancy. Cushing syndrome in pregnancy is usually confused with complicated pregnancy, such as preeclampsia or gestational diabetes, and its rarity leads to a low degree of clinical suspicion, often delaying diagnosis. We experienced a case of Cushing's syndrome in pregnancy, which had been considered as the severe preeclampsia and gestational diabetes due to uncontrolled hypertension and hyperglycemia. The pregnancy was terminated with an emergency cesarean operation at 30 weeks of gestation because of severe preeclampsia. In consequence of the evaluation about the Cushing's syndrome after delivery, the adrenal cortical adenoma of right adrenal gland was diagnosed and laparoscopic adrenalectomy was performed.

Conn's Syndrome Associated with Behcet's Disease

A 39-yr-old woman, who had been treated for Behcet's disease for 4 yr, was admitted for further investigation of recently identified hypokalemia and hypertension. Suppressed plasma renin activity with elevated plasma aldosterone concentration and an anomalous postural decrease in plasma aldosterone were observed. An abdominal CT scan revealed a right adrenal mass. The patient was diagnosed with Conn's syndrome. The association of Conn's syndrome with Behcet's disease was thought to be coincidental. To our knowledge, this is the first case of Conn's syndrome associated with Behcet's disease.