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Benign adrenocortical masses: diagnosis with chemical shift MR imaging.

Benign adrenocortical masses often contain lipid; metastases and pheochromocytomas do not. Standard and lipid-sensitive (chemical shift) magnetic resonance (MR) images of the adrenal glands in 31 patients with 45 adrenal masses were reviewed to determine if simple visual analysis of these images would increase diagnostic specificity. Lipid was considered present if signal intensity of the adrenal mass relative to other tissues decreased on chemical shift images relative to comparable standard images. Both myelolipomas and 26 of 27 benign cortical masses displayed a loss of signal intensity on at least one chemical shift image; all 12 metastases, the three hemorrhages, and a cyst did not. Opposed-phase images were slightly more sensitive than fat-suppressed images in depicting lipid within benign cortical masses. All masses had higher signal intensity than that of the liver on standard T2-weighted MR images. Chemical shift MR imaging can demonstrate lipid within benign adrenocortical masses and thus increase specificity, potentially obviating biopsy and aggressive follow-up.

Adenoma↗

Predictive value of image-guided adrenal biopsy: analysis of results of 101 biopsies.

A retrospective study of 97 patients undergoing 101 image-guided adrenal biopsies (IGABs) was performed to analyze the effects of specific pathologic results on test characteristics. Three categories of pathologic results (benign adrenal tissue, malignant tissue, and nondiagnostic) were compared with outcomes. Diagnostic samples were obtained in 86% of cases. Among 72 patients with proved outcomes, IGAB had an accuracy of 96%, a sensitivity of 93%, and a negative predictive value of 91% (92% in patients with bronchogenic carcinoma). In this subset of patients, 33 had biopsy specimens that contained benign adrenal tissue. In these 33 patients, three masses (each smaller than 3 cm) proved malignant. In the 14 patients with nondiagnostic samples, two masses proved malignant. Obtaining benign adrenal tissue was highly predictive of benignity, even in the setting of lung cancer. The authors conclude that IGAB is an accurate procedure in both oncologic and nononcologic patients. However, when the biopsy specimen does not contain benign adrenal tissue or malignant cells, repeat percutaneous biopsy or surgery should be considered.

Adrenal Gland Diseases↗

Indeterminate adrenal mass in patients with cancer: evaluation at PET with 2-[F-18]-fluoro-2-deoxy-D-glucose.

PURPOSE: To evaluate the ability of positron emission tomography (PET) to characterize adrenal masses in patients with cancer. MATERIALS AND METHODS: Twenty-four adrenal masses (size range, 1.5-10.0 cm [mean, 2.8 cm]) in 20 patients (13 men and seven women) with cancer (lung [n = 10], colon [n = 3], lymphoma [n = 3], miscellaneous [n = 4]) were evaluated. PET was performed in fasting patients 45 minutes after infusion of 5-10 mCi (185-370 MBq) of 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG). PET images were correlated with findings at computed tomography (CT), surgery, and/or percutaneous biopsy. RESULTS: Fourteen adrenal masses were proved malignant at surgery (n = 4), biopsy (n = 7), or follow-up (n = 3). Ten lesions were proved benign at biopsy (n = 4) or follow-up CT (n = 6). PET helped correct differentiation of benign from malignant adrenal lesions in all patients. A statistically significant difference (P < .001) was seen between mean tumor-to-background ratios for malignant (mean, 7.4; range, 2.9-16.6; median, 6.9) versus benign (mean, 0.8; range, 0.2-1.2; median, 0.6) adrenal lesions. CONCLUSION: PET helped correct differentiation of benign from malignant adrenal lesions.

Adrenal Gland Diseases↗

Cystic adrenal lesions: CT features.

PURPOSE: To define computed tomographic (CT) features of cystic adrenal lesions that differentiate them from similar-appearing adenoma. MATERIALS AND METHODS: CT scans of 13 cystic adrenal lesions (four endothelial cysts, three benign pseudocysts, one nonspecific benign cyst, one carcinoma, one pheochromocytoma, and three stable lesions with no histologic diagnosis) were analyzed retrospectively. Reports in the literature of 26 benign adrenal cysts were also reviewed. RESULTS: Mean lesion diameter was 6.2 cm, and six lesions were less than 5.0 cm. Mean attenuation values was 21 HU, and in eight cases it was less than 15 HU. Partial adrenal involvement was noted in six cases. Ten lesions contained wall or septal calcification. Wall thickness was 3 mm or less in nine and exceeded 6 mm in three lesions; one of the latter was cystic carcinoma. Wall enhancement (but no intralesional enhancement) was found in two of six lesions. Of 37 reviewed benign cysts, 19 had mural and seven had central calcification, 28 were unilocular, and seven had high attenuation value. Wall thickness was 3 mm or less in 31 lesions. CONCLUSION: CT findings of a nonenhancing mass with or without wall calcification allow differentiation of adrenal cyst from adenoma. A small adrenal cyst with near-water attenuation and a thin (< or = 3-mm) wall is likely to be benign.

Adenoma↗

Myelolipoma: CT and pathologic features.

PURPOSE: To correlate clinical and pathologic patterns of myelolipoma with the computed tomographic (CT) appearance. MATERIALS AND METHODS: All cases of myelolipoma accessioned by the Armed Forces Institute of Pathology from 1981 through 1997 were reviewed. CT images were scored for the location and size of each myelolipoma and the presence of calcification, hemorrhage, fat, and pseudocapsule. Pathologic findings for the pattern of fat and bone marrow elements were correlated with CT findings. RESULTS: In 74 patients, 86 myelolipomas were found, of which 72 were in an adrenal gland (eight were bilateral), and 14 extra-adrenal masses were found in 10 patients. Four clinicopathologic patterns emerged: (a) isolated adrenal myelolipoma in 37 patients (fat evident at CT, no other disorders present); (b) myelolipoma with hemorrhage in nine patients (imaging features similar to those of isolated adrenal myelolipomas but larger [mean diameter, 14.2 vs 9.9 cm; P = .01]; (c) extra-adrenal myelolipoma in 10 patients (imaging findings similar to those of adrenal myelolipomas, found most often in the retroperitoneum); and (d) myelolipomatous foci within other adrenal pathologic conditions in 18 patients (smaller, lower fat content, more heavily calcified). CONCLUSION: Myelolipomas are adrenal or extra-adrenal masses, with hemorrhage more common in larger lesions (diameter, > 10 cm). The CT appearance of myelolipomatous foci, which can be found within other pathologic adrenal conditions, is different from that of isolated adrenal myelolipomas.

Adipose Tissue↗

Diagnostic and therapeutic dilemma with large prenatally detected cystic adrenal masses.

OBJECTIVES: The prenatal finding of a large cystic adrenal mass raises the dilemma of the differential diagnosis between adrenal hemorrhage and cystic neuroblastoma. The possibility of a neuroblastoma usually leads to surgical excision of such tumors. Nevertheless, an adrenal hemorrhage has to be recognized, so that unnecessary surgery may be avoided. METHODS: Three cases of large prenatally detected adrenal masses managed nonoperatively are reported. Data studied were: age at the diagnosis, prenatal and postnatal ultrasonographic consistency, and tumor marker levels. Size and sonographic evolution were also studied. RESULTS: In all 3 cases, a cystic mass, measuring more than 40 mm in size, was detected during the 3rd trimester of pregnancy. The sonographic appearance evolved from a sonolucent tumor to a heterogeneous mass with hyperechoic areas. The tumor marker levels were normal. All infants had a documented decrease in mass size at birth and were managed nonoperatively. All these tumors were considered adrenal hemorrhages. CONCLUSIONS: Prenatal ultrasonography rarely permits to distinguish an adrenal hemorrhage from a cystic neuroblastoma. The differential diagnosis, even in large masses, is based on close postnatal follow-up with serial sonography. Surgery is not mandatory, unless the size does not decrease. However, without pathologic proof, it is not possible to differentiate an adrenal hemorrhage from a spontaneously resolved neuroblastoma.

Adrenal Gland Diseases↗

Large adrenal pseudocyst presenting with epigastric distress and abdominal distention.

OBJECTIVE: We report a patient with a large adrenal pseudocyst presenting with epigastric distress and abdominal distention. CLINICAL PRESENTATION AND INTERVENTION: A 35-year-old man presented with a 1-year history of nonspecific epigastric distress and abdominal distention. Routine laboratory tests and endocrine function tests were within the normal ranges. Magnetic resonance imaging (MRI) revealed a large left adrenal cyst. Adrenal cyst showed low intensity on T1-weighted MRI images and high density on T2-weighted MRI images. The patient underwent surgery, and the cyst was completely removed. Histological examination showed that the cyst wall consisted of hyalinized fibrous tissue without an epithelial or endothelial lining, and a diagnosis of an adrenal pseudocyst was made. Symptoms resolved after pseudocyst removal. CONCLUSION: The report showed a patient with adrenal pseudocyst, a rare and uncommon condition that was diagnosed on MR images and confirmed by histology. The epigastric distress and the abdominal distention resolved after the pseudocyst was surgically removed.

Abdominal Pain↗

Clinical and subclinical ACTH-independent macronodular adrenal hyperplasia and aberrant hormone receptors.

ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a very rare cause of endogenous Cushing's syndrome (CS). In this review, the clinical characteristics, the pathophysiology, and the management of AIMAH are described. AIMAH typically presents with overt CS, but subclinical oversecretion of cortisol has been increasingly described. The diagnosis is suspected by adrenal nodular enlargement on conventional imaging following the demonstration of ACTH-independent hypercortisolism. Final diagnosis is established by histological examination of the adrenal tissue. Bilateral adrenalectomy is the treatment of choice but unilateral adrenalectomy has been proposed in selected cases. In patients with subclinical CS, the decision to treat should be individualized. The pathophysiology of this condition has begun to be elucidated in recent years. Diverse aberrant membrane-bound receptors expressed in a non-mutated form in the adrenal gland have been found to be implicated in the regulation of steroidogenesis in AIMAH. When systematically screened, most patients with AIMAH and CS or subclinical CS exhibit an in vivo aberrant cortisol response to one or various ligands suggesting the presence of aberrant adrenal receptors. A protocol designed to screen patients for the presence of these aberrant receptors should be undertaken in all patients with AIMAH. The identification of these receptors provides the potential for novel pharmacological therapies by suppressing the endogenous ligands or blocking the receptor with specific antagonists.

Adrenal Gland Diseases↗

Adrenalectomy in adrenal hypertension.

Between 1965 and 1983, a total of 138 patients with endocrine hypertension were treated at the Münster Surgical Clinic. 71 catecholamine-producing tumors were removed in 56 patients, 60 patients had primary aldosteronism; 32 patients with Cushing's syndrome received uni- or bilateral adrenalectomy. The operative access to the adrenals was by anterior abdominal incision which we consider superior to lateral or transthoracic approaches. 35 complications occurred. The operative management of these forms of secondary hypertension, with its low overall rate of complications, is currently seen as the most successful therapeutic approach.

Adenoma↗

Plasma levels of aldosterone precursors (including the 18-hydroxylated compounds in this synthesis pathway)--a tool for the analysis of adrenal disorders?

Some current ideas concerning the 18-hydroxylated corticosteroids (18-hydroxycorticosterone and 18-hydroxydeoxycorticosterone) and the other steroids of the mineralocorticosteroid pathway from deoxycorticosterone (DOC) are reviewed. Then, some recent findings from our own laboratory, obtained during the analysis of all aldosterone precursors from DOC in various adrenal disorders, such as enzyme deficiencies, and particularly adrenal masses detected in patients from various hospitals in France are discussed. The findings reported here show that these specialized assays can be valuable tools to determine adrenal function in complex cases.

18-Hydroxycorticosterone↗

Adrenocorticotropin receptor and adrenal disorders.

The ACTH receptor is the shortest G-protein-coupled receptor to date and consists of 297 residues with two putative glycosylation sites at the extracellular N terminus. In vitro studies have demonstrated upregulation of the ACTH receptor by its own ligand and by angiotensin II. Inactivating mutations of the ACTH receptor lead to the familial glucocorticoid deficiency (FGD) syndrome, a rare recessive autosomal disorder characterized by degeneration of the zona fasciculata/reticularis and unresponsiveness to exogenous ACTH. Interestingly, ACTH receptor mutations are not present in all patients with FGD and also not in the closely related "triple A' syndrome indicating that other mechanisms of ACTH resistance are still to be elucidated. Despite an extensive search, no activating ACTH receptor mutations have been found in adrenal tumors, excluding the ACTH receptor as a relevant oncogene for adrenal tumorigenesis. However, the ACTH receptor may play a role as a differentiation factor, as loss of heterozygosity for the ACTH receptor in adrenal tumors seems to be associated with an undifferentiated phenotype. ACTH receptor mRNA expression in benign adrenal tumors is strongly related to the expression of P-450 side chain cleavage enzyme mRNA indicating a close regulative relationship. However, this correlation is disrupted in adrenal carcinomas, an observation which may help in the difficult differential diagnosis between benign and malignant tumors. Surprisingly, the highest ACTH receptor mRNA expression was found in aldosteronomas, while it is low in non-functioning adenomas and carcinomas. No correlation between ACTH receptor mRNA expression and circulating ACTH levels has been found in patients with adrenal disorders casting doubts on the physiological significance of ACTH receptor upregulation by its own ligand in vivo.

Adrenal Gland Diseases↗

Neonatal adrenal abscesses.

Unilateral or bilateral suprarenal abscesses are rare in the neonate. We describe 2 cases, an 8-week-old Syrian female with a right, and a 6-week-old white female infant with a left adrenal abscess, both following adrenal hemorrhage. The etiology and treatment are discussed.

Abdominal Abscess↗