Bilateral Adrenal Infarctions as an Initial Manifestation of TAFRO Syndrome: A Case Report and Review of the Literature

TAFRO syndrome is a systemic inflammatory disorder resembling multicentric Castleman disease; it is characterized by thrombocytopenia, anasarca, a fever, reticulin fibrosis, and organomegaly. Involvement of the adrenal glands, including adrenal infarction, hemorrhaging, and adrenomegaly, has recently been reported in several cases and been considered a characteristic early-stage symptom. We herein report a case of TAFRO syndrome initially presenting with bilateral adrenal infarctions and review the literature on TAFRO syndrome related to adrenal involvement. This case suggests that adrenal abnormalities as an early clinical feature of TAFRO syndrome may be useful for the early diagnosis.     

The left adrenal vein: An important direction for right adrenal venous sampling

This study aimed to evaluate the guiding role of left adrenal vein (LAV) for right adrenal venous sampling (AVS). A total of 347 patients who were diagnosed with primary aldosteronism (PA) and underwent successful AVS procedures from January 2020 to July 2021 were retrospectively analyzed. According to the different quadrant position of the orifice of right adrenal vein (RAV), the area where the orifice of RAV is located is divided into three areas: A, B, and C and the area A is further subdivided into A1, A2, and A3 areas. By counting the area where the orifice of RAV is located, the guiding role of the LAV on the RAV is determined. Most of the orifice of RAV are located in area A, and the proportions of areas A, B, and C was 96.8%, 1.4%, and 1.7%, respectively. In area A, areas A1, A2, and A3 account for 80.9%, 17.0%, and 2.1%, respectively. High body mass index, female and smaller the angle between the LAV and horizontal line was associated with the closer positional relationship between the LAV on the RAV. These findings suggest that most of the horizontal position of the RAV orifice is close to the horizontal position of the most distal end of the LAV, which indicate that the LAV location can play an important role on the guiding for right AVS. Additionally, body mass index, sex, and the angle of the LAV was largely related to location of the orifice of the RAV.     

Non Hodgkin's lymphoma involving the adrenal glands and the central nervous system (CNS): a particular evolution after chemotherapy

Adrenal lymphoma is extremely rare. The prognostic depends on involvement of other organs (such as the central nervous system) responsible for lower median survival. We report the case of a 51-year-old man with non Hodgkin's Diffuse Large B Cell Lymphoma (DLBCL) involving the central nervous system (CNS) and the adrenal glands simultaneously. The endocrine exploration revealed a partial adrenal insufficiency and ruled out a pheochromocytoma. Computerized tomographic (CT) scan directed needle biopsy of the adrenal gland allowed the diagnostic of non-Hodgkin lymphoma (NHL). CNS biopsies showed similar histopathologic lesions. After aggressive polychemotherapy and methotrexate intrathecal injection, a dissociated therapeutic response was observed with a decrease of the cerebral lesion and an increase of the adrenal mass. This result may be explained by the efficacy of corticosteroid therapy on cerebral edema. The prognosis was poor with tumor infiltration of the leptomeninges and death 16 months after diagnosis.     

Subclinical Cushing's Syndrome

Clinically inapparent adrenal masses, or adrenal incidentalomas, are discovered inadvertently in the course of workup or treatment of unrelated disorders. Cortical adenoma is the most frequent type of adrenal incidentaloma accounting for approximately 50% of cases in surgical series and even greater shares in medical series. Incidentally discovered adrenal adenomas may secrete cortisol in an autonomous manner, that is not fully restrained by pituitary feed-back, in 5 to 20% of cases depending on study protocols and diagnostic criteria. A number of different alterations in the endocrine tests aimed to assess the function of the hypothalamic-pituitary-adrenal axis has been demonstrated in such patients. This heterogeneous condition has been termed as subclinical Cushing's syndrome, a definition that is more accurate than preclinical Cushing's syndrome since the evolution towards clinically overt hypercortisolism does occur rarely, if ever. The criteria for qualifying subclinical cortisol excess are controversial and we presently do not have sufficient evidence to define a gold standard for the diagnosis of subclinical Cushing's syndrome. An increased frequency of hypertension, central obesity, impaired glucose tolerance, diabetes and hyperlipoproteinemia has been described in patients with subclinical Cushing's syndrome; however, there is not evidence-based demonstration of its long-term complications and, consequently, the management of this condition is largely empirical. Either adrenalectomy or careful observation associated with treatment of metabolic syndrome has been suggested as treatment options because data are insufficient to indicate the superiority of a surgical or nonsurgical approach to manage patients with subclinical hyperfunctioning adrenal cortical adenomas. This work was partially supported by grants of the University of Turin (ex-60% funds).     

Role of Neurotransmitters and Neuropeptides in the Regulation of the Adrenal Cortex

Reviews in Endocrine and Metabolic Disorders -     

Adrenal incidentaloma--experience of a standardized diagnostic programme in the Swedish prospective study

OBJECTIVE: To report the 5-year experience of a diagnostic programme for adrenal incidentaloma with special emphasis to diagnose hormonally active and malignant lesions. DESIGN: A prospective study in which new cases of adrenal incidentalomas in Sweden have been evaluated by a standardized diagnostic protocol between January 1996 and July 2001. SETTING: Thirty-three different Swedish hospitals have contributed with cases. SUBJECTS: A total of 381 patients (217 females, 164 males) with adrenal incidentalomas were studied. INTERVENTIONS: Diagnostic procedures were undertaken according to a standardized programme. Operation was recommended if the incidentaloma had a size of more than 3-4 cm or if there was a suspicion of a hypersecreting tumour. MAIN OUTCOME MEASURES: The size of the incidentaloma, clinical characteristics of the patients and results of biochemical diagnostic tests were registered. RESULTS: The median age of the patients was 64 years (18-84 years), and the median size of the incidentalomas was 3 cm (1-20 cm). A total of 85(22%) patients were operated. Twenty of these patients were diagnosed with a benign hypersecreting tumour and 14 with a malignant tumour. Fourteen of 15 operated patients with diagnosed pheochromocytoma had elevated 24-h urinary noradrenaline and all of the patients operated because of a biochemical suspicion of aldosterone or cortisol hypersecretion (n = 6) were found to have adrenal adenomas. Of the 14 operated patients with malignant diseases, 10 were adrenal carcinomas (median size 10 cm; range 4-16 cm). In a multiple logistic regression model, incidentaloma size was significantly associated with the risk of a malignant tumour (P = 0.009), and there was a tendency of an association between age/male sex and the risk of a malignancy (both, P = 0.07). CONCLUSION: In this Swedish multicentre study of 381 cases with adrenal incidentalomas, 5% had benign hypersecreting tumours and nearly 4% had malignant tumours. The results of the biochemical diagnostic tests used had a high compatibility with the histological diagnosis found at operation in the patients with hypersecreting tumours. Tumour size, male gender and high age were predictive for the risk of a malignant tumour. A follow-up of the patients is warranted in order to establish whether there are undiscovered cases of malignant or hypersecreting tumours amongst the nonoperated patients.     

Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma

Testicular adrenal rest tumours are frequently associated with congenital adrenal hyperplasia (CAH). These ACTH-dependent tumours cannot be easily distinguished histologically from Leydig-cell tumours. We report the case of a 30-year-old man who was explored for infertility, azoospermia and unilateral testicular tumour. High levels of 17-OH progesterone and ACTH, low cortisol and undetectable gonadotropins levels, associated to bilateral adrenal hyperplasia, led to the diagnosis of CAH by 21-OH deficiency with a composite heterozygoty. The testicular tumour was first considered as adrenal rest. However, histological analysis of this unilateral painful tumour showed a steroid-hormone-secreting cell proliferation with atypical and frequent mitosis. To discriminate between a benign adrenal rest tumour and a possible malignant leydigioma, tumoral expression of specific gene products was analyzed by RT-PCR. No 11-beta-hydroxylase nor ACTH receptor mRNAs could be found in the tumour, which did not behave like usual adrenal rest cells. For this unilateral testicular tumour, the lack of adrenal-specific markers associated with a high rate of mitosis and pleiomorphism supported a leydigian origin with malignant potential. However, lack of tumoral LH-R mRNA expression and a tumour-free 3-year follow-up led us to retain the diagnosis of adrenal rest tumour with loss of adrenal gene expression and progressive autonomous behaviour.     

Oral Contraceptive Disturbed the Recovery of the Adrenal Function after Adrenalectomy in Cushing Syndrome

Estrogen is known to increase exogenous corticosteroid levels. In this case, a 27-year-old Japanese woman was referred to our hospital for examination of an adrenal tumor and was diagnosed with Cushing syndrome. Resection of the tumor resulted in secondary adrenal insufficiency. She also developed microcytic anemia due to hypermenorrhea, which was masked by Cushing syndrome. An oral contraceptive was administered for the treatment of hypermenorrhea, but this led to a marked increase in serum cortisol and the reduction of plasma adenocorticotropic hormone, disturbing the recovery of the adrenal function. Attention is required when oral contraceptives are used to treat hypermenorrhea masked by Cushing syndrome.     

Management of primary aldosteronism in patients with adrenal hemorrhage following adrenal vein sampling: A brief review with illustrative cases

The authors describe the clinical investigation of two cases of primary aldosteronism with adrenal hemorrhage (AH) following adrenal vein sampling. A literature review was conducted regarding the medical management of primary aldosteronism in patients with AH following adrenal vein sampling. Guidelines on the management of primary aldosteronism with AH following adrenal vein sampling are lacking. The two patients were followed with serial imaging to document resolution of AH and treated medically with excellent blood pressure response. Resolution of AH was achieved, but a repeat adrenal vein sampling was deferred given the increased morbidity risk associated with a repeat procedure.     

Emergence of Ectopic Adrenal Tissues-What are the Probable Mechanisms?

Ectopic adrenal tissue, defined as the formation of adrenal tissue in an abnormal anatomical location, is not a rare entity and may have clinical significance. Even though the mechanism for their emergence has not been fully understood, numerous cases of ectopic adrenal tissue have been reported, mostly in the vicinity of the original location of adrenal gland, such as in kidneys and gonads. In these cases, most authors attributed their emergence to a probable migration defect. However, this mechanism does not simply explain the ectopic tissues in remote locations, such as in the hypophysis or lungs. This review summarizes these reports, describing many different locations in which ectopic adrenal tissues were encountered, together with their suggested mechanisms.