Bilateral Adrenal Lesions Research Articles

Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening

Adrenal tumors are a common manifestation of the multiple endocrine neoplasia type 1 (MEN-1) syndrome. Prevalence in recent studies varies between 9 and 45%. A genotype-phenotype correlation has been described as well as the development of adrenocortical carcinomas. Long-term prospective data are still lacking. Thirty-eight MEN-1 patients with proven germline mutations have been prospectively observed in a regular screening program in our hospital. Adrenal glands have been screened by biochemical analysis and either by endoscopic ultrasound (EUS) or computed tomography (CT) or both. Median follow-up was 48 months (12-108 months). Age at diagnosis of MEN-1, type of adrenal tumor, genotype, therapy, and clinical characteristics have been analyzed. In 21 (55%) patients, adrenal involvement of the disease was detected. Adrenal lesions were detected in average 6.9 years after the initial diagnosis of MEN-1. Median tumor size was 12 mm (5-40 mm). Tumor size smaller than 10 mm was observed in 11 patients. Twelve patients had unilateral while nine had bilateral adrenal lesions. EUS detected all adrenal tumors, whereas CT failed in seven cases. In three patients, functioning tumors (one pheochromocytoma, one bilateral Cushing adenoma, and one adrenocortical carcinoma) and one nonfunctioning adenoma were diagnosed by histology and biochemical assessment. Two laparoscopic adrenalectomies and one laparoscopic subtotal resection were performed. Nonfunctioning adrenal lesions, not characterized by histology yet, were found in 18 patients. There was no statistical difference with regard to adrenal involvement between patients with germline mutations in exons 2 and 10 (12/21) and those with mutations in exons 3-9 (6/11). MEN-1-associated adrenal tumors are mostly small, benign, and nonfunctioning and much more common than previously reported. EUS was the most sensitive imaging procedure. The genotype-pheotype correlation previously suggested by our group could not be confirmed.

Role of multidetector row computed tomography in the assessment of adrenal gland injuries

Objective To determine the prevalence of adrenal injuries in a group of patients submitted to multidetector row CT evaluation after blunt trauma and to assess the impact of CT findings on clinical management decisions. Materials and method During a 4-year period, 2026 emergency CT examinations were performed in the setting of major blunt trauma. A total of 82 patients were retrospectively identified as having adrenal gland traumatic lesions. At multidetector row CT the following findings were considered specific of adrenal injury: round or oval hematoma expanding the adrenal gland, irregular hemorrhage obliterating the gland, uniform adrenal gland swelling, active extravasation of contrast material from the adrenal vessels and adrenal gland rupture. Associated CT findings were: stranding of the periadrenal fat, diffuse hemorrhage in the adjacent retroperitoneum and compression of the adrenal gland by adjacent traumatic lesions. Results We identified 82 patients (46 males and 36 females, age ranging from 15 to 86 years) with adrenal injuries. The right adrenal gland was injured in 60/82 patients, while the left adrenal gland was injured in 21 cases; in 1 patient bilateral adrenal gland traumatic lesions occurred. In 76 patients with non-isolated adrenal injuries concomitant injuries to the liver (49 cases), ipsilateral kidney (18 cases) and spleen (9 cases) were observed. Round or oval hematoma expanding the adrenal gland (61 cases), irregular hemorrhage obliterating the gland (14 cases), stranding of the periadrenal fat (9 cases) and diffuse hemorrhage in the adjacent retroperitoneum (8 cases) were the more frequent findings detected at CT. Six patients underwent surgical intervention for the presence of major injuries to the spleen (three cases), to the liver (one), to the right kidney (one), to the left kidney (one). Seventy-six patients were conservatively treated. Conclusion Blunt adrenal injuries typically present as part of a multiorgan trauma. Familiarity with characteristic CT findings of adrenal trauma is essential for the radiologist to avoid misdiagnosis.

Mutations of p53, c-kit, K-ras, and beta-catenin gene in non-Hodgkin's lymphoma of adrenal gland.

Malignant lymphoma of the adrenal gland is a rare disease, usually with diffuse large cell morphology and B‐cell immunophenotype, and often associated with Epstein‐Barr virus infection. In this study, mutations of p53, c‐kit, K‐ras, and β‐catenin gene were analyzed in 17 cases (13 males and four females with ages ranging from 25 to 84 years) of such lymphomas by polymerase chain reaction‐single strand conformation polymorphism followed by direct sequencing. Selected exons in each gene, representing hot spots, were analyzed. All 44 mutations detected were single‐nucleotide substitutions and 33 were missense mutations. Nineteen mutations were detected in exon 5 and/or 7 of the p53 gene in nine of 17 cases (52.9%) and 21 in exon 11 and/or 17 of the c‐kit gene in 10 of 14 cases (71.4%). Bilateral adrenal lesions in one case who had not received any adjuvant therapy showed different mutational patterns of the p53 and c‐kit genes, suggesting different clonal evolution of lymphoma between the left and right sides. Mutation at codon 13 of the K‐ras gene was detected in one of 14 cases (7.1%), and in exon 3 of the β‐catenin gene in three of 12 cases (25%). All but one mutation were transition mutations, indicating that some endogenous mutagens act in lymphomagenesis in the adrenal gland. Our results suggest that p53 and c‐kit gene mutations might play a role in adrenal lymphomagenesis.

Scintigraphic imaging of the adrenal glands

This article presents the well established scintigraphic imaging techniques of the adrenal glands. Both adrenocortical scintigraphy with [ 131]6β-iodomethylnorcholesterol (NP-59) and adrenomedullary imaging with 131I or 123I-labelled metaiodobenzylguanidine (MIBG) as function-dependant imaging techniques provide functional metabolic information for lesion characterization. They enable the distinction between unilateral and bilateral adrenal lesions. Furthermore, they have the advantage of giving the possibility of a whole body evaluation with a single administration of the tracer without additional radiation dose. On the other hand, radiological imaging modalities provide excellent anatomical details which is essential for planning therapy. Both radiological and scintigraphic imaging methods of the adrenal glands are necessary and should, therefore, be considered complementary.

Twenty-Five-Year Surgical Experience with Pheochromocytoma in Children

Our objective was to analyze the presentation, diagnostic localization, operative management, histology, and long-term outcome of a single center's experience with pheochromocytomas in children. A chart review was done to identify all operatively managed pheochromocytomas in patients age 18 years or younger. Open and laparoscopic cases were included. We reviewed the presentation, diagnostic imaging, localization, operative management, pathology, and postoperative outcome of these patients. Clinic visits, contact with the tumor registry, and telephone interviews were used for follow-up. From 1973 through 1999, there were 11 children (four males and seven females) with 14 pheochromocytomas. Two (18.2%) patients had bilateral adrenal lesions and one patient had both adrenal and extra-adrenal tumors. Six (54.5%) patients had extra-adrenal lesions. The average age at operation was 14.7 years (range 9-18 years). Nine (82%) patients had significant hypertension at presentation. CT was used to localize the tumor in eight patients and urine catecholamine levels were used to confirm the diagnosis. Two of the cases were associated with inherited syndromes (multiple endocrine neoplasia 2A and von Hippel-Lindau). Ten patients underwent an open operation and one patient had a laparoscopic resection. The average patient follow-up was 9.2 years (range 9 months to 25 years). There were no operative complications and all patients were alive and well at the time of last follow-up. Three patients (27.2%) had tumors with microscopic malignant features. No tumors recurred or had evidence for metastatic spread. We conclude that peak incidence of pheochromocytomas in children is in early adolescence. Resection can be carried out safely with minimal morbidity and mortality. Current best management of this entity includes establishment of a biochemical diagnosis, adequate preoperative blockade, appropriate imaging, and an individualized operative approach based on tumor location and size.

Retrospective review of the ultrasonographic features of adrenal lesions in 21 ferrets.

Clinical signs and follow-up information were recorded. Histopathologic diagnoses were obtained for 25 adrenal glands in 21 ferrets. Adrenal lesions included ten adenocarcinomas, nine adenomas, one hyperplasia and one cortical cyst. Four adrenal glands (all right-sided) were diagnosed as unspecified adrenal tumors but lacked a definite histopathologic diagnosis (adenoma vs. adenocarcinoma) due to incomplete surgical resection and consequent small sample sizes. Bilateral adrenal lesions were identified in 4 ferrets (19%). Adrenal shape, size, echogenicity, laterality, and the presence of vascular invasion were evaluated with ultrasound. Size and shape were variable and not specific to lesion type. Both benign and malignant adrenal tumors (adenomas, adenocarcinomas) appeared most often as masses with increased thickness and a normal length (11/23), less frequently as larger masses with increased thickness and length (4/23) or as nodules focally deforming the normal adrenal shape (6/23). The only cortical cyst appeared as a nodule. Three adrenal glands had a normal size and shape and were diagnosed as adenomas (2) or hyperplasia (1). Therefore treatment may be warranted based solely on clinical signs if adrenal glands are ultrasonographically normal. Vascular invasion was not identified ultrasonographically. However, focal absence of periglandular fat resulting in contact of 8 adrenal glands with either caudal vena cava (6), aorta (1) or liver (1) identified ultrasonographically, correlated with incomplete surgical resectability (6/8) and histopathologic diagnoses of carcinoma (4/8) or unspecified tumors (4/8). Therefore, a focal absence of periglandular fat between the adrenal gland and the large vessels or liver, deviation or compression of the large vessels by the adrenal lesion may indicate malignancy. Adrenal tumors (benign and malignant) were often associated with a prominent uterus, uterine stump or prostate with or without prostatic cysts.

Tc-99m MIBI Scintigraphic Detection of Metastatic Insular Thyroid Carcinoma

Thallium-201 and, recently, Tc-99m MIBI have been used in conjunction with I-131 scintigraphy for follow-up of patients with well-differentiated thyroid cancer. Insular carcinoma of the thyroid is a fairly aggressive thyroid neoplasm that is believed to arise from follicular cells and usually concentrates I-131. The authors report a patient with recurrent insular thyroid carcinoma in whom bilateral adrenal and lung metastatic lesions developed 3 years after ablative I-131 therapy for cervical lymph node and skeletal metastases. Tc-99m MIBI planar and SPECT images demonstrated these new lesions better than pretherapy I-131 scintigraphy and affords an imaging technique for post-I-131 therapy follow-up that does not require withholding thyroid hormone suppression.

Spectrum of pheochromocytoma in the 131I-MIBG era.

Abstract131I‐metaiodobenzylguanidine (131I‐MIBG) scintigraphy allows for both functional diagnosis and anatomical localization of pheochromocytoma. The spectrum of pheochromocytoma since the routine use of preoperative131I‐MIBG scan was studied.From 1980 to 1986, a total of 34 patients were primarily diagnosed and treated at the University of Michigan Medical Center, Ann Arbor, Michigan, U.S.A. There were 16 males and 18 females. The mean age was 38 years and 4 patients (11.8%) were under 18 years of age. Six patients (17.6%) had family history of pheochromocytoma or multiple endocrine neoplasia (MEN) II syndrome. The presenting symptoms were hypertension in 29 patients (85.3%); attacks of headache, palpitation, sweating, and flushing in 4 (11.8%), and 1 patient presented with a neck mass.Plasma catecholamines were elevated in 97% of patients while urinary catecholamines and metabolites were elevated in 93.5%.131I‐MIBG was accurate in 82.3%, partly positive in 11.8%, and false‐negative in 5.9% of patients. CT scan was accurate in 80%, partly positive in 10%, but failed to show the tumor in another 10% of patients.At operation, extraadrenal lesions were found in 38.2% of the patients and among these, one‐third were extraabdominal. Multiple tumors occurred in 5 (14.7%), and bilateral adrenal lesions occurred in 4 patients (11.8%). Malignancy was diagnosed in 3 patients (8.8%) after an average follow‐up period of 2 years.We conclude that the use of routine preoperative131I‐MIBG scanning improves localization of pheochromocytoma and earlier diagnosis is possible in patients with MEN II syndrome. Multiple tumors, extraadrenal and extraabdominal lesions occur more often than commonly believed. The low rate of confirmed malignancy is probably related to the short period of follow‐up.