Ectopic adrenal tissue in the kidney: A systematic review

Introduction: Ectopic adrenal tissue in the kidney, including “Ectopic adrenal tissue” and “Adrenal-renal fusion”, is a rare event with a specific behavior which may be difficult to distinguish clinically from renal neoplasms. We performed a systematic review on ectopic adrenal tissue variants reported in the literature underlining its clinical aspects. Methods: Manuscripts which presented a case report or case series of ectopic adrenal tissue in the kidney were included even if published in original articles, reviews, or letters to the editor. A specific search on SCOPUS®, PubMed®, and Web of Science® database was performed. Only English language papers published in a period ranging between August 1991 and April 2020 were considered. Additionally, a case we had at our institution is described, and its characteristics are included. Data on clinical presentation, type of adrenal anomaly, location, anatomopathological and immune-histotype characteristics were collected. Results: We identified 888 manuscripts. Among these 29 were included in this systematic review. Overall, 39 patients with renal adrenal fusion or adrenal ectopia were considered. In most cases, the diagnosis was made incidentally, or following investigation for flank pain, abdominal pain, or endocrinological disorders. CT scan frequently identified a solid vascularized lesion that was difficult to distinguish from renal neoplasm. Adrenal fusion was mostly located at the level of the upper pole. Adrenal rest was found in the renal parenchyma, renal hilum, or retroperitoneum in close proximity to the renal peduncle. Often these ectopic adrenal tissue lesions follow a benign behavior and can be classified as functioning or non-functioning adenomas. Rarely, they may experience neoplastic degeneration. The most frequently positive markers were inhibin, vimentin, melan-A, synaptophysin and anti-p450 scc. Conclusions: Ectopic adrenal tissue in the kidney is a rare event with specific clinical characteristics that need to be identified in order to arrive at a correct diagnosis and carry out appropriate treatment management.

Ectopic Adrenocortical Tissue Attached to the Inguinal Sac in an Adult

Inguinal localization of ectopic adrenal tissue is a rare condition in literature. We report an unusual case of a 28-year-old male patient with ectopic adrenocortical tissue, which was noticed coincidentally as a orangeyellowish nodule during surgery of inguinal hernia and resected for patological examination.

MON-LB042 A Rare Case of Adrenocortical Carcinoma Arising From Ectopic Adrenal Tissue in the Mesentery

Background: ACTH independent Cushing’s syndrome usually arises from benign or malignant tumors of the adrenal gland. Ectopic adrenal tissue can undergo malignant transformation resulting in the development of adrenocortical carcinoma (ACC) with normal adrenal glands. We present a unique case of ACTH independent Cushing’s syndrome from a cortisol and androgen producing ACC arising from the mesentery. Clinical Case: A 72-year-old woman presented with a 6-month history of progressive weakness, lower extremity edema, worsening hypertension and uncontrolled DM. She had moon facies, hirsutism, multiple bruises, and proximal muscle weakness. Labs revealed hypokalemia (2.6 mmol/L, N 3.5 -5.1 nmol/L), elevated random cortisol (51.3 mcg/dL, Nl- 5-23), suppressed ACTH (<1 pg/mL, Nl-7.2-63.3) abnormal 8 mg dexamethasone suppression test (58.1 mcg/dL, N <1.8 mcg/dL), elevated DHEAS (538mcg/dL, N 10-90 mcg/dL), elevated testosterone (590.2 ng/dL, N <75 ng/dL) and elevated 11-deoxycortisol (4650 ng/dL N-<32 ng/dL). 24 hour urinary free cortisol was 2810mcg/mL (<45mcg/dL). MRI of pituitary was normal. CT scan of abdomen/pelvis showed normal bilateral adrenal glands and innumerable enhancing masses throughout the abdomen with the largest mass near the distal ileum and cecum. Biopsy of right lower abdominal mass revealed adrenocortical morphology with immunohistochemical staining positive for inhibin, synaptophysin and calretinin. Ki-67 index was 10-15%, suggestive of low-grade adrenocortical carcinoma. A CT scan done one and half years prior noted a 4.4 cm soft tissue mass in the right lower mesentery supporting origin of the tumor from the mesentery. Hypercortisolism was controlled with Metyrapone 250 mg BID. Mitotane 1000 mg bid was initiated but patient developed peritoneal carcinomatosis within 1 month. Conclusions: Our case is remarkable for the development of a metastatic ACC from an ectopic adrenal tissue with normal bilateral adrenal glands. Ectopic ACC is very rare with only a handful of cases reported in the literature. This is the first reported case of ACC arising from the mesentery. Ectopic adrenal tissue can be found close to the adrenal glands, along the path from gonads to adrenal glands or in association with the gonads. In the setting of ACTH independent Cushing’s syndrome with normal adrenal glands, physicians should direct their search to a functioning ectopic adrenocortical tissue. Concomitant DHEAS secretion suggests ectopic ACC. If surgery is not an option due to metastatic disease, a multidisciplinary approach should be adopted to control tumor growth and associated symptoms. In such cases, control of the hypercortisolemia can be achieved with adrenolytic medications such as Metyrapone, Ketoconazole or Mitotane. Adjuvant chemotherapy (Mitotane and combination of cytotoxic drugs) might be considered for metastatic ACC treatment.

SUN-LB36 Primary Aldosteronism Due Tosimultaneous Occurrence of Aldosteronoma in the Left Adrenal Gland and Ectopicaldosteronoma in the Liver: A Case Report

Rationale: Ectopic adrenal tissue is the adrenal rests along the path from gonads to adrenal glands during embryogenesis. Ectopic aldosteronoma is a rare disease presented with over-production of aldosterone by the ectopic adrenocortical tissue. Diagnosis is a clinical challenge with simultaneous occurrence of ectopic aldosteronoma. To our knowledge this is the first reported case of simultaneous occurrence of aldosteronoma in the adrenal gland and ectopic aldosteronoma in the liver based on literatures. Patient concerns: A 33-year-old woman presented with resistant hypertension and severe hypokalemea for 3 years. 5 months ago, the patient was diagnosed as aldosteronoma in left adrenal gland and underwent right adrenalectomy. The histopathological examination of the resected sample suggested adrenal cortical adenoma. The patient still had symptoms of hypertension and hypokalemia after operation, but the blood potassium level was higher than that before operation (minimum blood potassium level rose from 1.8 mmol/L to 2.6 mmol/L). Diagnosis: The saline load test, captopril test, and plasma aldosterone/renin ratio were indicative of primary aldosteronism (PA). The computed tomographic scan (CT) was suggestive of a low-density mass (2.9×2.2 cm) in the liver which was very near to the right adrenal area. Magnetic resonance imaging (MRI) further confirmed that the lesion was located in the liver. PET-CT eliminated the possibility of metastasis to other parts of the body. Ultrasound guided biopsy confirmed that the tumor was ectopic adrenal tissue in the liver. Interventions: Ultrasound-guided percutaneous radiofrequency ablation was performed to the tumor in the liver.Outcomes: The patient’s blood potassium level was 3.8 mmol/L on the third day after the ablation without any potassium supplementation treatments. On follow-up of 2-weeks duration, the patient has g good control over her blood pressure of around 126/74 mmHg and blood potassium of 4.55 mmol/L, without taking any medications. Lessons: The patient was diagnosed with PA due to simultaneous occurrence of aldosteronoma in the left adrenal gland and ectopic aldosteronoma in the liver, which is very rare. Ultrasound-Guided Percutaneous Radiofrequency Ablation is a safe and effective treatment for ectopic aldosteronoma in liver.

Adrenal-Renal Fusion with Adrenal Cortical Adenoma and Ectopic Adrenal Tissue, Presenting as Suspected Renal Mass: A Case Report

Adrenal-renal fusion with adrenal cortical adenoma is a rare anomaly with only a few cases described in the literature. Imaging-based identification of this anomaly remains a diagnostic challenge, making it difficult to differentiate upper pole renal malignancy from adrenal cortical adenoma. We describe a case of a 62-year-old woman with an upper pole cystic renal mass on imaging, who underwent robotic partial nephrectomy. Intraoperatively the renal mass was found to be an adrenal-renal fusion anomaly, with ectopic adrenal tissue. Adrenal-renal infusion of an adrenal cortical adenoma was confirmed on final pathology. Due to lack of imaging-based diagnosis, this condition should be considered in the differential for upper pole renal masses.

Pheochromocytoma arising from an ectopic adrenal tissue in multiple endocrine neoplasia type 2A

Summary A 21-year-old woman was referred to our hospital to treat bilateral pheochromocytomas (PCCs) after a diagnosis of multiple endocrine neoplasia type 2A (MEN2A). We performed bilateral laparoscopic adrenalectomy. One year after the operation, urinary fractionated metanephrines in 24-h urine increased. MRI showed a 30 mm tumor on the interaortocaval region and 123I-MIBG concentrated in this area. We excised the tumor and performed para-aortic lymphadenectomy. Histopathologic examination confirmed a PCC arising from ectopic adrenal tissue. Urinary fractionated metanephrines in 24-h urine declined to basal levels immediately after the operation. We detected no recurrence of paraganglioma or PCC for 5 years after the treatment. Learning points: Most ectopic adrenal tissue is associated with no symptoms and contains only the adrenal cortex. Adrenocortical tumors sometimes arise from ectopic adrenal tissues similarly to in the normal adrenal gland. PCC arising from ectopic adrenal tissue occurs infrequently. MEN2-related PCC is accompanied by adrenal medullary hyperplasia, which might be part of tumorigenesis.

Histologic evaluation of parovarian nodules in the cat

Objectives A notable, although relatively uncommon, finding during feline ovariohysterectomy is the presence of parovarian nodules, located proximal to the ovary, near or within the ovarian vascular bundle. They are usually 2–3 mm in diameter, white-to-tan in color and glandular in appearance. The objective of this study was to either decisively reinforce the existing findings reported in the literature, which suggest that these parovarian nodules are incidental adrenocortical nodules that are clinically and surgically insignificant, or capture any samples that were not ectopic adrenal tissue in order to characterize them histologically. Methods Ninety-one formalin-preserved tissue sections containing parovarian nodules were collected during routine feline elective ovariohysterectomy and evaluated histologically. Results Definitive histologic information was obtained from 73/91 (80.2%) submitted samples. Fifty-two of 73 (71.2%) samples were determined to contain accessory adrenal gland cortex. Twenty of 73 (27.4%) samples were determined to contain residual mesonephric structures. While accessory adrenocortical nodules were found bilaterally in cats of all ages, mesonephric structures were predominantly right-sided, and only found in one cat over the age of 1 year. Ectopic or accessory ovarian tissue was not found in any of the samples. Conclusions and relevance This study adds to the existing body of data which suggest these nodules are incidental structures that do not need to be removed during surgical sterilization. However, further in vivo studies with larger sample sizes and years of follow-up would be required to more definitively prove this theory.