Non-functioning Adrenocortical Carcinoma Research Articles

Clinical analysis of prognosis of adrenocortical carcinoma

Objective To study the clinical factors affecting the prognosis of adrenocortical car-cinoma (ACC) in order to guide the diagnosis and treatment of ACC. Methods The clinical data of 40 cases of ACC were retrospectively analyzed. The patients were diagnosed as ACC by histopatholo-gical examination after operation or biopsy, including 19 cases of nonfunctional ACC and 21 cases of functional ACC. The functional ACC included 14 cases of Cushing's syndrome, 4 cases of sexual ab-normality (3 cases of androphany and one case of male sexual precosity) and 3 cases of primary aldo-steronism. The clinical stage included 3 cases in stage Ⅰ , 10 cases in stage Ⅱ , 7 cases in stage Ⅲ, and 21 cases in stage Ⅳ. Eighteen cases underwent complete excision, 10 cases underwent palliative excision, 12 cases underwent expectant treatment (2 cases underwent arterial embolism). All patients were followed up for 2 to 121 months. The total life span and survival rate were calculated, and the dependability between life span and certain indices (tumor size, function, clinical stage and surgical treatment, etc) was compared. The results were statistically analyzed by utilizing the SPSS 11.5 soft-ware. Rank-sum test was used for the two independent samples, one-factor analysis of variance was used for multiple samples and Pearson correlation analysis was used for the correlation of two vari-ances. Results There were no statistical correlations between gender, age, sides and survival time (P>0.05). The survival time of nonfunctional ACC patients was 37.0 months, of which 11 dead ca-ses survived 11.0 months and 8 survival cases survived 73.5 months. The survival time of functional ACC patients was 11.5 months, of which 19 dead cases survived 12.2 months and 2 survival cases survived 4.8 months. The survival time of nonfunctional ACC was significantly longer than that of functional ACC (P 0. 05]. Con-clusions The most important influencing prognostic factors of ACC are function and clinical stage. For ACC in stage Ⅰ and Ⅱ , tumor resection is the most effective treatment, and second surgical oper-ation is recommended for local recurrence. For ACC in stage Ⅲ, radical or extensive surgical operation is recommended, and for ACC in stage Ⅳ, surgical operation has no effect on the prognosis. Key words: Adrenocortical carcinoma; Surgical procedures,operative; Prognosis; Stage

Investigation of BRAFand CTNNB1 activating mutations in adrenocortical tumors

Activating mutations of the BRAF oncogene play a central role in the development of various cancer types, but their role in human adrenocortical tumors is unknown. At variance, activating mutations of another oncogene, CTNNB1, which encodes beta-catenin, have been shown to be common events in both benign and malignant adrenocortical tumors. To investigate the prevalence of BRAF and CTNNB1 activating mutations in sporadic adrenocortical tumors. Tissue samples from 15 adrenocortical carcinomas and 41 adrenocortical adenomas were investigated for the presence of BRAF and CTNNB1 activating mutations by PCR amplification and direct sequencing. An advanced invasive non-functioning adrenocortical carcinoma carried a somatic heterozygous BRAF V600E mutation, while 4 functioning and 4 non-functioning adenomas and 3 functioning carcinomas carried different CTNNB1 activating mutations. Activating BRAF somatic mutations may be occasionally found in advanced adrenocortical carcinomas, while CTNNB1 activating mutations are early and common events in adrenal tumorigenesis.

A Case of Non-Functioning Huge Adrenocortical Carcinoma Extending Into Inferior Vena Cava and Right Atrium

Primary adrenocortical carcinoma (ACC) is a rare tumor and its usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%). However, intracaval invasion extending into the right atrium is very rare and spontaneous regression of tumor burden in adrenal carcinoma is also rare. We report a case of ACC with direct invasion of the inferior vena cava and right atrium. A 34-yr-old male patient presented with progressive dyspnea, weight loss, and poor oral intake over 3 months. Non-functioning ACC with direct invasion of the inferior vena cava and right atrium was confirmed by imaging, pathologic, and hormonal study. Chemo-radio-therapy was attempted. However, tumor burden was not changed, but rather toxic hepatitis and thrombocytopenia were developed. His subjective symptoms and general conditions were improved after 1 month of conservative management and the patient was discharged. During clinical follow-up, this tumor showed spontaneous regression.

Adrenocortical tumors in children

Background/Purpose: Etiopathogenesis and management of pediatric adrenocortical tumors (ACTs) is still obscure because of the limited number of cases. The aim of this study is to present a clear picture of the entire spectrum of pediatric ACTs by reviewing one of the largest noncollected pediatric series treated in a single medical center. Methods: Records of children treated for ACTs in our unit between 1970 and 1999, inclusive, were reviewed. Information recorded for each patient included age, sex, clinical characteristics, diagnostic methods, stage of disease, treatment, pathologic findings, and outcome. The patients were subdivided into 2 groups: group I, patients with adrenocortical carcinoma (ACC) and group II, patients with adrenocortical adenoma (ACA). These groups were analyzed with regard to parameters mentioned above. Results: There were 30 children treated for ACTs in the study period with a mean age of 6.7 ± 4.2 years (range, 2.5 to 13 years). Of these, 20 had ACC, and 10 had ACA. The tumors were right sided in 22 patients, left sided in 6 and bilateral in 2. Analysis of each group with regard to age and site of tumor showed no significant difference. Endocrine dysfunction was noted in 83% of the patients and virilization was the most common presentation followed by Cushing's syndrome. The most striking difference between 2 groups was the prepondarance of virilization in group II and Cushing's syndrome in group I. In the latter, 14 patients presented with palpable abdominal mass and 3 patients with distant metastases. The mean time from initial symptoms to diagnosis was 8.1 ± 0.2 months, and this interval was similar in 2 groups, in functional and nonfunctional tumors, and in both sexes. Ultrasound scan, computerized tomography, magnetic resonance imaging, intravenous pyelography, and angiography were used for the diagnosis. All patients with ACA had localized disease, whereas 80% of the patients with ACC had regional or metastatic disease. Total excision was done in all patients with ACA, but only in 13 patients with ACCs. Of the latter, 2 patients underwent ipsilateral nephrectomy, and 1 patient had right hepatic lobectomy plus nephrectomy. Adjuvant chemotherapy consisting of mitotane (n = 12), mitotane plus cisplatin and etoposide (n = 2) was commenced. Seven patients with ACC had distant metastases postoperatively. The presence of regional disease at presentation was associated with a significantly shorter disease-free interval. All patients presenting with nonfunctional ACC (n = 4), functional ACC that have been totally resected (n = 4), and partially resected (n = 3) died of disease within the first 2.5 years after diagnosis. There was no significant difference between the functional and nonfunctional ACCs with regard to survival rate. All patients who had distant metastases postoperatively and who had partial excision died. Of the surviving 9 patients with ACC, there are 6 known long-term survivors who are still alive. Conclusions: ACAs are treated by total excision satisfactorily without any complication. For the time being, the most important aspect of therapy for ACCs is early diagnosis and total excision. Partial excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy in ACTs. Because of the steadily increasing incidence of precancerous genetic syndromes of adrenal glands and poor prognosis of ACCs, childhood patients of endocrine disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults. Patients with ACTs should be entered into multi-institutional trials to adequately assess effective chemotherapy and radiotherapy protocols and molecular mechanisms of oncogenesis. J Pediatr Surg 36:549-554. Copyright © 2001 by W.B. Saunders Company.

Bilateral adrenocortical carcinoma showing loss of heterozygosity at the p53 and rb gene loci

Presented is a rare case of nonfamilial, hormonally nonfunctional adrenocortical carcinoma with synchronous bilateral adrenal involvement. We investigated adrenal and metastatic tumors for loss of heterozygosity affecting four genetic loci containing the tumor suppressor genes p53, RB, DCC, and APC, using polymerase chain reaction and restriction fragment length polymorphism assay. Allelic losses at the p53 and RB loci were detected in all tumor samples, suggesting that the p53 and RB genes are involved in the tumorigenesis of adrenocortical carcinoma.

Spontaneous Regression of Pulmonary Metastasis from Nonfunctioning Adrenocortical Carcinoma after Removal of the Primary Lesion: A Case Report

A 46-year-old man presented for general fatigue. Abdominal computerized tomography (CT) obtained by his physician showed a tumor in the left adrenal gland but no other lesions. Physical examination demonstrated no abnormality. Laboratory evaluations revealed the elevation of serum carcinoembryonic antigen (80 ngJml., normal less than 5) and slight anemia but no pulmonary infection. CT of the chest showed an irregular 8.9 X 6.9 cm. mass in the upper lobe of the left lung (fig. 1,A). Levels of urinary 17-ketosteroids, 17-hydroxycortico-sterone, vanillylmandelic acid, serum cortisol, and serum and urinary catecholamine and aldosterone were within normal limits. Transtracheal biopsy of the lung tumor revealed that the tumor had metastasized from adrenocortical carcinoma without inilammation. Endoscopy of the gastrointestinal tract, and bone and gallium scintigraphy did not show any abnormal findings suggesting metastatic tumor except in the lung and left adrenal. Recently complete resection of metastatic and recurrent tumor combined with adjuvant mitotane (0, p'-DDD) therapy has been recommended,' and the metastatic lesion in our case seemed to be resectable. Therefore, surgical resection of the primary and metastatic tumors was performed (fig. 2, A). Initially the patient underwent en bloc resection of the tumor, which was well encapsulated. The left kidney, to which the tumor was adhering, was also resected but the tumor had not invaded beyond the capsule. Microscopic examination of the resected tumor revealed solid clusters of viable tumor cells with pleomorphic nuclei. The diagnosis was clinically and biochemically nonfunctional adrenocortical carcinoma (fig. 2, B). CT of the chest demonstrated partial regression of the lung tumor (fig. 1, B). The tumor gradually decreased to 10% of its size at initial presentation 2 months after removal of the left adrenal tumor and left kidney. Segmental resection of the upper lobe of the left lung was then performed. No swollen regional lymph nodes were noted at either surgery. Major parts of the lung tumor showed necrosis on microscopy. Some areas consisted of large ple

METASTATIC ADRENOCORTICAL CARCINOMA TREATED BY REPEATED RESECTION: A CASE REPORT OF LONG‐TERM SURVIVAL OVER 18 YEARS

A case of recurrent and metastatic nonfunctioning adrenocortical carcinoma in a 40-year-old woman is reported. The patient received 4 laparotomies and 1 thoracotomy for recurrent and metastatic disease after removal of the primary adrenal tumor. She has been alive for over 18 years following multiple surgery for diseases after the first adrenalectomy. In selected patients with recurrence and/or metastasis, repeated surgical resection offers the possibility of a cure or extended palliation.

Adrenal rest carcinoma in hilumof kidney

Adrenocortical rests are uncommon in adults and infrequentlyundergo malignant changes. Most of the published cases are those of functional tumors allowing preoperative diagnosis on the basis of the resulting endocrinopathy. Most nonfunctioning tumors were found accidentally at operation or autopsy. A case is presented of a parapelvic nonfunctioning adrenocortical rest carcinoma situated in the renal hilus in a fifty-year-old woman. No such tumor has been described previously.

A RESECTED CASE OF NON-FUNCTIONAL ADRENOCORTICAL ADENOMA

Aresected case of non-functional adrenocortical adenoma that was accidentally discovered during ultrasound examinaiton of the abdomen is reported. The case is a 41-year-old female who underwent a thorough medical examination at a local hospital owing to hypochondralgia and symptoms of cold, and a right adrenal tumor was accidentally discovered by ultrasanography, . She had non-hormonal clinical symptoms and normal serum and urine endocrinologic findings. Computed tomography of the abdomen revealed a tumor with low density from anterior, posterior and lower segment of the right hepatic lobe extending to the right upper pole of the kidney, and angiography of the right suprarenal artery displayed a hyper-vascular tumor of the adrenal gland. The resected tumor was covered with capsule showing elastic soft, even solid yellow tone, measuring 9.2×9.0×7.4cm in size, weighing 325g and was diagnosed histologically as benign adrenocortical adenoma. Recent widespread use of ultrasonography or CT has increased an opportunity to detect an adrenal tumor, but only a total of 45 cases of non-functional adenocortical adenoma has been reported. Preoperative differential diagnosis of this disease from non-functional adrenocortical carcinoma is sometimes difficult. Therefore, particularly in small tumors, operative indication must be carefally considered.

Acute Budd-Chiari Syndrome as First Manifestation of Adrenocortical Carcinoma

We report the case of a young woman in whom investigations for acute Budd-Chiari syndrome disclosed an hormone-secreting but clinically nonfunctioning adrenocortical carcinoma. We supply a very brief review of the literature.

Prolonged Fever as a Presenting Symptom in Adrenal Tumors

Four patients with adrenal tumors in whom prolonged fever was the foremost symptom are presented. Two of the patients had nonfunctional adrenocortical carcinoma, one had a pheochromocytoma without adrenergic hyperactivity, and in the fourth, an aldosteronoma was found. Only in 1 case was the tumor (pheochromocytoma) resectable and this patient became afebrile post surgery. A review of the literature revealed that fever is encountered in 6-10% of cases with adrenal tumors, sometimes as the first manifestation of disease. However, a survey of the large series of fever of unknown origin revealed no mention of adrenal tumors in that group of solid tumors which many cause prolonged fever.

Primary nonfunctioning carcinoma of adrenal cortex

Two cases of nonfunctioning adrenocortical carcinomas are presented. The clinical features, operative findings, and histopathologic appearances are discussed. It is believed that these tumors are difficult to diagnose because of their vague presentation. Intravenous urography with nephrotomography is the most valuable method of detecting these tumors, and surgery remains the best treatment.