Individualized treatment guided by endoscopic ultrasound-guided fine-needle aspiration for adrenocortical oncocytoma:A case report

BACKGROUND Adrenocortical oncocytoma is a rare,mostly benign,nonfunctional tumor that is typically detected incidentally.Its diagnosis is challenging because of the absence of distinctive imaging characteristics,necessitating pathological validation.CASE SUMMARY We present a case report of a 35-year-old woman with an adrenal mass located on the left side,where endoscopic ultrasound-guided fine-needle aspiration(EUSFNA)was performed after comprehensive diagnostic assessment.Our results are consistent with those of previously documented cases regarding tumor demographics and anatomical location.Given the limited number of reports on this condition,long-term follow-up is crucial to enhance our understanding of its prognosis.CONCLUSION For patients with adrenocortical oncocytoma,EUS-FNA can enables collection of preoperative tissue specimens leading to suitable treatment strategies.

Oncocytic adrenocortical tumor with uncertain malignant potential in pediatric population: A case report and review of literature

BACKGROUND Oncocytic adrenocortical tumor(OACT)is rare,with few cases reported in the literature.No more than 20 cases in children have been reported.The clinical characteristics,diagnosis,treatment and prognosis of children with OACT are summarized based on a literature review,in order to improve the understanding of OACT in children.CASE SUMMARY We report a case of a 17-mo-old patient who was admitted to our hospital due to symptoms of odynuria and fever,which are clinical features consistent with a functional adrenocortical tumor.The patient was diagnosed with OACT of uncertain malignant potential.Computed tomography indicated a soft tissue giant tumor in the right adrenal region,approximately 4.3 cm×5.5 cm in size.Multiple nodular and speckled calcifications were observed in the lesion.The patient received robot-assisted laparoscopic right adrenal tumor resection.Postoperative pathological results were consistent with OACT,and immunohistochemical results showed cytokeratin+/-,chromogranin A+,synaptophysin-,neuronspecific enolase-,S100-,Ki67 about 10%,CD34-and D2-40-.After surgery,urinary tract ultrasonography was reviewed monthly,catecholamine hormone and sex hormone levels were examined every 2 mo and computed tomography was performed every 6 mo.To date,no tumor metastasis or recurrence has been identified in this patient.The levels of sex hormones and catecholamine hormones decreased to normal 1 mo after surgery.CONCLUSION OACT is rare in the pediatric population,with few cases reported in the literature.Although most pediatric OACTs are benign,malignant cases have been reported.Surgical resection is the preferred option in most patients.

Rare complication of acute adrenocortical dysfunction in adrenocortical carcinoma after transcatheter arterial chemoembolization:A case report

BACKGROUND Adrenocortical carcinoma(ACC)is a rare and highly invasive endocrine malignant tumor with a poor prognosis.Although surgical resection is the main treatment for ACC,postoperative recurrence and metastasis have become the important factors of death.Transcatheter arterial chemoembolization(TACE)is an important option for the treatment of advanced ACC with liver metastasis.However,due to the small number of patients treated for ACC,the safety of the operation is not completely clear and needs to be further studied.CASE SUMMARY A 47-year-old patient with ACC after surgery was admitted for reexamination by abdominal computerized tomography suggesting liver metastasis.Because the patient expressed reluctance to undergo surgery again,we treated her with TACE for the liver lesions.After treatment,symptoms of acute adrenal dysfunction such as decreased blood pressure,anorexia,and fatigue appeared,which were relieved after hydrocortisone treatment.To date,the patient's liver lesion is well controlled and no other metastases are observed.CONCLUSION We report a rare case of acute adrenal hypofunction after TACE.Glucocorticoid supplementation can alleviate the symptoms.

Risk factors and predictive model of adrenocortical insuffi ciency in patients with traumatic brain injury

BACKGROUND:Neuroendocrine dysfunction after traumatic brain injury(TBI)has received increased attention due to its impact on the recovery of neural function.The purpose of this study is to investigate the incidence and risk factors of adrenocortical insuffi ciency(AI)after TBI to reveal independent predictors and build a prediction model of AI after TBI.METHODS:Enrolled patients were grouped into the AI and non-AI groups.Fourteen preset impact factors were recorded.Patients were regrouped according to each impact factor as a categorical variable.Univariate and multiple logistic regression analyses were performed to screen the related independent risk factors of AI after TBI and develop the predictive model.RESULTS:A total of 108 patients were recruited,of whom 34(31.5%)patients had AI.Nine factors(age,Glasgow Coma Scale[GCS]score on admission,mean arterial pressure[MAP],urinary volume,serum sodium level,cerebral hernia,frontal lobe contusion,diff use axonal injury[DAI],and skull base fracture)were probably related to AI after TBI.Three factors(urinary volume[X4],serum sodium level[X5],and DAI[X8])were independent variables,based on which a prediction model was developed(logit P=-3.552+2.583X4+2.235X5+2.269X8).CONCLUSIONS:The incidence of AI after TBI is high.Factors such as age,GCS score,MAP,urinary volume,serum sodium level,cerebral hernia,frontal lobe contusion,DAI,and skull base fracture are probably related to AI after TBI.Urinary volume,serum sodium level,and DAI are the independent predictors of AI after TBI.

Giant nonfunctional ectopic adrenocortical carcinoma on the anterior abdominal wall: A case report

BACKGROUND Adrenocortical cancer(ACC) is an infrequent and often aggressive malignancy with a very poor prognosis. It can be classified as functional or nonfunctional.Nonfunctional ACC is hampered by the absence of specific signs or symptoms;only abdominal pain with or without incidental adrenal occupation is typically present.CASE SUMMARY We report a rare case of a patient with a 30 cm × 15 cm × 8 cm ectopic ACC on the anterior abdominal wall without organ adhesion. A 77-year-old male was admitted to our hospital because of a huge abdominal mass, which, by ultrasonography, had an unclear border with the liver. Computed tomography showed that the mass was not associated with any organ but was adherent to the anterior abdominal wall. The patient underwent tumor resection, and a postoperative pathology examination showed a neuroendocrine tumor, which was diagnosed as ACC. The patient was disease-free at the 9-mo follow up.CONCLUSION The anterior abdominal wall is a rare site of ACC growth.

Yttrium-90 microsphere selective internal radiation therapy for liver metastases following systemic chemotherapy and surgical resection for metastatic adrenocortical carcinoma

Adrenocortical carcinoma(ACC)is a rare malignancy with generally poor outcomes and limited treatment options.While surgical resection can be curative for early local disease,most patients present with advanced ACC owing to nonspecific symptoms.For those patients,treatment options include systemic chemotherapy and locoregional therapies including radiofrequency ablation and transarterial chemoembolization.We present the first reported case of utilizing yttrium-90 microsphere selective internal radiation therapy(SIRT)in combination with first line EDP-M(Etoposide,Doxorubicin,Cisplatin,Mitotane)chemotherapy and debulking surgical primary tumor resection for treatment of metastatic ACC.Stable complete radiologic response has been maintained after twelve months with resolution of clinical symptoms.These findings prompt the need for further consideration and studies to elucidate the role of SIRT in combination with systemic and surgical treatment for metastatic ACC.

Bilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing's syndrome: A case report and review of the literature

BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease,ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortical adenomas(BAAs) or carcinomas. BAAs causing ACTHindependent CS are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. Adrenal vein sampling(AVS) is a good way to diagnose ACTH-independent CS.CASE SUMMARY A 31-year-old woman had a typical appearance of CS. The oral glucose tolerance test showed impaired glucose tolerance and obviously increased insulin and Cpeptide levels. Her baseline serum cortisol and urine free cortisol were elevated and did not show either a circadian rhythm or suppression with dexamethasone administration. The peripheral 1-deamino-8-D-arginine-vasopressin(DDVAP)stimulation test showed a delay of the peak level, which was 1.05 times as high as the baseline level. Bilateral AVS results suggested the possibility of BAAs.Abdominal computed tomography showed bilateral adrenal adenomas with atrophic adrenal glands(right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). Magnetic resonance imaging of the pituitary gland demonstrated normal findings. A left adenomectomy by retroperitoneoscopy was performed first,followed by resection of the right-side adrenal mass 3 mo later. Biopsy results of both adenomas showed cortical tumors. Evaluations of ACTH and cortisol showed a significant decrease after left adenomectomy but could still not be suppressed, and the circadian rhythm was absent. Following bilateral adenomectomy, this patient has been administered with prednisone until now,all of her symptoms were alleviated, and she had normal blood pressure without edema in either of her lower extremities.CONCLUSION BAAs causing ACTH-independent CS are rare. AVS is of great significance for obtaining information on the functional state of BAAs before surgery.

Adrenocortical system activity in alloxan-resistant and alloxan-susceptible Wistar rats

In the dynamics of the disease development, diuresis and glycosuria increase in alloxan-susceptible rats, while in alloxan-resistant rats the increase in the values of these indices is expressed to a lesser extent, and they begin to decrease by day 8 of the disease. In alloxan-susceptible rats, the mass index of adrenal gland is increased, and that of thymus is decreased and corticosterone concentration in blood, adrenal gland and urine as well as alanine and aspartate aminotransferase activities in liver are increased;in alloxan-resistant rats the values of these indices do not differ from those of rats of the control group.

THE CHARACTERISTICS OF ENDOGENOUS OUABAIN SECRETION FROM CULTURED BOVINE ADRENOCORTICAL CELLS

Objective To compare the characteristics or endogenous ouabaln(EO) secretion with the other adrenocortical hormones and determine the effects of angiotensin I (Ang I ), and ad reno corticotrophin (ACTH ) on the secretion or EO. Methods EO was measured by radioimmunoassay from primary cultured bovine adrenocotical cells (BAC). Results oouabain was determined in the media or cultured BAC. Both EO and aldosterone secretion were decreased from the 6uter to inner layer or the cultured adrenal cortex, and the responses to Ang I and ACTH were hlgher than that in the mld layer (P <o. o5) and inner layer (P <o. o1 ). Cortisol secretion was activated by Ang I or ACTH was slgnificantly higher in the mid layer and in the inner layer than that in the outer layer. The tlme-course experlment showed that the gradually rising amounts or aldosterone and cortisol could be determined during the continuous incubation to 48h wlth or without Ang I or ACTH. However, EO did not increase continuously arter 24h or incubation in the basal secreting sltuation and arter 12h of lncubatiou in the stimulating situation by Ang,or ACTH. There were obvlous drops in aldosterone and cortisol secretlou from 3rd day during a 21 day-perlod cell culture, but the peak secretion of ouabain was in 7th day. Conclusion it suggests that the secretory mechanism might be different between EO and aldosterone or cortisol. Also, Ang I and ACTR might be involved in the regulation of Eo secretion.

Elucidating the clinical and immunological value of m6A regulatormediated methylation modification patterns in adrenocortical carcinoma

N6-methyladenosine methylation(m6A)is a common type of epigenetic alteration that prominently affects the prognosis of tumor patients.However,it is unknown how the m6A regulator affects the tumor microenvironment(TME)cell infiltration in adrenocortical carcinoma(ACC)and how it affects the prognosis of ACC patients yet.The m6A alteration patterns of 112 ACC patients were evaluated,furthermore,the association with immune infiltration cell features was investigated.The unsupervised clustering method was applied to typify the m6A alteration patterns of ACC patients.The principal component analysis(PCA)technique was taken to create the m6A score to assess the alteration pattern in specific malignancies.We found two independent patterns of m6A alteration in ACC patients.The TME cell infiltration features were significantly in accordance with phenotypes of tumor immune-inflamed and immune desert in both patterns.The m6Ascore also served as an independent predictive factor in ACC patients.The somatic copy number variation(CNV)and patients prognosis can be predicted by m6A alteration patterns.Moreover,the ACC patients with high m6A scores had better overall survival(OS)and higher efficiency in immune checkpoint blockade therapy.Our work demonstrated the significance of m6A alteration to the ACC patients immunotherapy.The individual m6A alteration patterns analysis might contribute to ACC patients prognosis prediction and immunotherapy choice.

Multidisciplinary team therapy for left giant adrenocortical carcinoma: A case report

BACKGROUND Adrenocortical carcinoma(ACC)is a rare malignant epithelial tumor originating from adrenocortical cells that carries a very poor prognosis.Metastatic or inoperable diseases are often considered incurable,and treatment remains a challenge.Especially for advanced cases such as ACC complicated with renal venous cancer thrombus,there are few cumulative cases in the literature.CASE SUMMARY The patient in this case was a 39-year-old middle-aged male who was admitted to the hospital for more than half a month due to dizziness and chest tightness.Computed tomography(CT)findings after admission revealed a left retroperitoneal malignant space-occupying lesion,but the origin of the formation of the left renal vein cancer thrombus remained to be determined.It was speculated that it originated from the left adrenal gland,perhaps a retroperitoneal source,and left adrenal mass+left nephrectomy+left renal vein tumor thrombus removal+angioplasty were performed under general anesthesia.Postoperative pathology results indicated a diagnosis of ACC.Postoperative steroid therapy was administered.At 3 mo after surgery,abdominal CT reexamination revealed multiple enlarged retroperitoneal lymph nodes and multiple low-density shadows in the liver,and palliative radiotherapy and mitotane were administered,considering the possibility of metastasis.The patient is currently being followed up.CONCLUSION ACC is a highly malignant tumor.Even if the tumor is removed surgically,there is still the possibility of recurrence.Postoperative mitotane and adjuvant chemoradiotherapy have certain benefits for patients,but they cannot fully offset the poor prognosis of this disease.

Giant androgen-producing adrenocortical carcinoma with atrial flutter: A case report and review of the literature

BACKGROUND Adrenocortical carcinoma(ACC),the second most aggressive malignant tumor,lacks epidemiological data worldwide;therefore,every new case can improve the understanding of the pathology and treatment of this malignancy.CASE SUMMARY We present the case of a 66-year-old Caucasian woman with a giant androgenproducing ACC(21 cm×17 cm×12 cm;2100 g),without metastases,which unusually presented with an acute onset of atrial flutter and congestive heart failure.The cardiac complications observed in our case support the hypothesis that androgen excess in women is a cardiovascular risk factor.Androgen excess in women can be a rare cause of reversible dilated cardiomyopathy,therefore a comprehensive approach to the patient is essential to improve the recognition of androgen-secreting ACC.The atrial flutter was remitted after initiation of drug treatment during admission.The severe heart failure was totally remitted at 6 mo after radical open surgery to remove the giant ACC.CONCLUSION Radical open surgery to remove a giant androgen-producing ACC was the firstline treatment to cure the excess of androgen,which determined the total remission of cardiac complications at 6 mo after surgery in the women of this case report.

Screening of biomarkers for the diagnosis and prognosis of adrenocortical carcinoma based on bioinformatics analysis

Objective:To explore the gene biomarkers related to the diagnosis and prognosis of adrenocortical carcinoma(ACC)by bioinformatics.Methods:GEPIA online analysis tool was used to screen differentially expressed genes for sequencing data from patients with ACC and normal adrenal cortex.The overall survival rate and disease-free survival method were used to conduct batch survival analysis on differentially expressed genes,and the top 100 genes with HR values calculated by the two methods were obtained respectively.The intersection method is used to obtain core genes that play a key role in both overall survival and disease-free survival.GEPIA online analysis tool was used again to explore the relationship between the above-mentioned survival-related genes and the pathological stage of ACC.Use UALCAN online analysis tool to verify the survival-related genes again and draw the Kaplan-Meier survival curve.Finally,GSE33371 chip dataset of the GEO database was used to evaluate the diagnostic value of the above-mentioned survival-related genes.Results:514 differentially expressed genes were obtained by limma method.Batch analysis of differential genes was performed to obtain the top 100 genes most related to overall survival and disease-free survival,of which 13 genes were closely related to overall survival and disease-free survival.9 hub genes including TP73,SNHG1,PDE6D,GPC2,SUV39H2,HELLS,CLK2,COPS7B and CEP164 were finally obtained by exploring the relationship between their expression levels and pathological stage and resurvival analysis.At the same time,the results of ROC analysis suggest that the above hub genes have high diagnostic value for patients with adrenocortical carcinoma.Conclusion:By using GEPIA,UALCAN and the gene chip retrieved from GEO database,combined with the bioinformatics method,we analyzed and verified the new biomarkers that can be used to evaluate the prognosis of patients with ACC and to differential diagnosis of ACC,and provided the theoretical support of bioinformatics for exploring the occurrence,development of molecular mechanism and potential target of treatment of ACC.

Aberrant Adrenocortical Tissue Associated with Resected Lipoma of Spermatic Cord in an Adult

Aberrant adrenocortical tissue in spermatic cord is rare in adult population. We report an unusual case of a 52-year-old male patient with an aberrant adrenocortical tissue which is incidentally resected within lipoma of spermatic cord and identified on microscopic examination. Although he was diagnosed with right inguinal hernia, there was no significant hernia orifice intraoperatively. When the 2nd operation by anterior open approach was undergone, there was a fat-like tissue adhered to the spermatic cord. It was removed and the histopathological finding first revealed aberrant adrenocortical tissue in the resected lipoma of spermatic cord (Figure 1). This abnormal tissue is usually found incidentally during inguinal operation. In general, most of the aberrant adrenal tissue becomes atrophic by adult life. Moreover, it is not clinically significant and also not associated with endocrine abnormalities.

Splenic Arteriogastric Fistula Resulting from Adrenocortical Carcinoma: A Rare Cause of Massive Upper Gastrointestinal Hemorrhage

Splenic arteriogastric fistula is a rare cause of upper gastrointestinal (GI) hemorrhage, only reported a handful of times in the literature. Herein, we present a case of a 49-year-old woman with metastatic adrenocortical carcincoma who developed a fistula between the splenic artery and gastric lumen as a result of local invasion of her primary tumor. This fistula led to several episodes of massive upper GI bleeding. Selective splenic artery embolization was successful in ceasing the GI bleed;however, the intervention was not timely enough for the patient to survive the hemorrhage. We outline several clinical and imaging findings to assist physicians with earlier detection of splenic arteriogastric fistulas, and advocate prophylactic selective splenic artery embolization when this rare but highly fatal entity is discovered.

Cushing Syndrome Revealing an Adrenocortical Carcinoma

Adrenocortical carcinoma is a malignant tumor of the adrenal gland, very rare in pediatric pathology. Authors presented a pediatric case of adrenocortical carcinoma which showed neurology symptoms at 1st visit and Cushing syndrome secondarily. The patient received a ketoconazole-based treatment. The outcome was fatal before the surgical excision of the tumor, because of delayed diagnosis. Early diagnosis and multidisciplinary management of adrenocortical carcinoma could improve the prognosis in children.

Expression of mTOR signaling pathway in adrenocortical tumour and its relationship with cell invasion and angiogenesis

Objective:To study the expression of mTOR signaling pathway in adrenocortical tumour and its relationship with cell invasion and angiogenesis.Methods:Adrenocortical malignant tumor tissues and normal adrenal cortex tissues surgically removed in the Third People's Hospital of Huizhou Guangdong Province between September 2014 and February 2018 were selected, the protein was extracted to determine the expression of mTOR signaling molecules, and the RNA was extracted to determine the mRNA expression of cell invasion molecules and angiogenesis molecules.Results: p-PI3K, p-AKT, p-mTOR, p-S6K1 and p-4E-BP1 protein expression as well as MMP2, MMP9, PKC , VEGF and nm23-H1 mRNA expression in adrenocortical malignant tumor tissue were significantly higher than those in normal adrenal cortex tissue whereas PCAF, E-cadherin and TSP1 mRNA expression were significantly lower than those in normal adrenal cortex tissue;Pearson correlation analysis showed that p-PI3K, p-AKT, p-mTOR, p-S6K1 and p-4E-BP1 protein expression in adrenocortical malignant tumor tissue were negatively correlated with PCAF, E-cadherin and TSP1 mRNA expression, and positively correlated with MMP2, MMP9, PKC , VEGF and nm23-H1 mRNA expression.Conclusion:The excessive activation of mTOR signaling pathway in adrenocortical malignant tumor can promote cell invasion and angiogenesis.

Case Report： Adrenocortical Carcinoma with a Solitary Fibrous Tumor

ACC （adrenocortical carcinoma） is an uncommon and aggressive malignancy with an overall five year survival rate from less than 50%. The majority of cases are accidentally diagnosed by imaging studies done for other reasons, so they are often diagnosed in the later stages. Due to the rarity of both neoplasms, the synchronous presentation and the lack of effective therapeutic options; we report a case concerning about a female patient with adrenal cancer with a low grade solitary fibrous tumor who underwent surgery, adjuvant radiotherapy and chemotherapy with good response.

SEMYXOID ADRENOCORTICAL CARCINOMA PRESENTING AS CUSHING SYNDROME:ONE CASE REPORT

Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.

Myxoid adrenocortical adenoma： a case report

Myxoid adrenocortical adenomas are extremely rare .with only 24 cases reported, The first case wasreported by Tang et al in 1979 which was diagnosed as a myxoid adrenocortical carcinoma. Nearly all the reports were of single case except Brown et al who described a group of 14 cases. We report here an additional case of myxoid adrenocortical adenoma with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.