Left Adrenal Tumor Research Articles

A case of adrenal cavernous hemangioma resected due to tumor growth accompanied by intratumoral hemorrhage

IntroductionWe describe a case of an adrenal cavernous hemangioma that was surgically resected because of tumor growth and intratumoral hemorrhage.Case presentationA 73‐year‐old woman presented with an enlarged adrenal tumor and intratumoral hemorrhage during the follow‐up of an incidental adrenal tumor. A computed tomography showed that the left adrenal tumor had grown from 23 to 44 mm over 1 year. Blood tests revealed a normal metabolic profile. Paragangliomas and metastatic tumors were suspected on imaging. Laparoscopic adrenalectomy was performed to prevent tumor rupture due to further bleeding. No adhesions or bleeding were observed around the tumor during surgery. Pathological diagnosis was adrenal cavernous hemangioma.ConclusionAdrenal cavernous hemangioma is difficult to distinguish preoperatively from other adrenal tumors, including malignant tumors. The intraoperative findings of this case suggest that laparoscopic adrenalectomy is a safe treatment option for relatively small adrenal cavernous hemangioma.

Intra-adrenal Paraganglioma and Paraaortic Paraganglioma in 15 Years Old Boys

Intra-adrenal paraganglioma (also called pheochromocytoma) is a chromaffin cell tumor that arises in the adrenal medulla and produces excess catecholamines. Paraganglioma can also be located in the extra-adrenal area. The incidence rate of intra-adrenal and extra-adrenal paraganglioma is about 1 case per 2.500-6.500 population, highest incidence at 40-50 years old, and distribution is the same in males and females. Intra-adrenal paraganglioma in pediatrics is more familial, bilateral, multifocal, and malignant. In children, the incidence rate of these tumors was 8.86%, the ratio of boys to girls was 2:1, and the average age of 11. This article reports a case, of a 15 years old boy with severe headaches, recurrent seizures, and a hypertension crisis (143/103-220/110 mmHg). MSCT scan of abdominal showed solid mass heterogeneous with central necrotic multiple in the supero-anterior of the left and right kidneys and on the right anterolateral aorta as high as CV L2, impressive adrenal gland mass and extra-adrenal, suspect pheochromocytoma. Performed surgery, routine histopathological examination, and Immunohistochemistry. Macroscopic examination showed an oval round shape mass of left and right adrenal tumors as well as the paraaortic, a smooth outer surface of the capsular, with yellowish brown color mass tumor at cut surface, supple consistency. Microscopic examination showing adrenal gland and paraaortic tissue with fibrous capsule contains proliferation of neoplastic cells forming a nested alveolar (Zellballen) pattern with a round oval to polygonal cells, granular eosinophilic cytoplasm, round oval nuclei, surrounded by sustentacular cells. Chromogranin A and S-100 are positive. The case was concluded by clinical findings, histopathological, and immunohistochemistry as intra-adrenal paraganglioma and paraganglioma of paraaortic.

Steroid metabolites producing adenoma: a case report

Hyperandrogenism is the most prevalent cause of menstrual cycle abnormalities and infertility in women. Here, we present a case of a 32-year-old woman with a 7-year history of menstrual irregularity and infertility. Laboratory test results revealed elevated 17-hydroxyprogesterone, progesterone 21-deoxycortisol. Abdominal computed tomography found a 3,9х2,9х2,6 cm left adrenal tumor. Non-classic congenital adrenal hyperplasia was diagnosed initially, however, treatment with supraphysiological doses of dexamethasone proved ineffective and progesterone and 17-hydroxyprogesterone levels remained high. Genetic testing revealed no 21-hydroxylase deficiency. Laparoscopic adrenalectomy was performed with subsequent pathological report being compatible with an adrenal cortical adenoma. 17-hydroxyprogesterone, progesterone 21-deoxycortisol levels returned to the normal range postoperatively and the patient’s menstrual cycle normalized without additional medication. Steroid metabolites producing adenomas are also extremely rare with only a few cases found in patients without previous CAH diagnosis. When a patient with clinically and biochemically diagnosed NCCAH demonstrate no typical features and shows poor response to steroid therapy, the patient should receive multisteroid LC–MS/MS assay for glucocorticoids and androgens, adrenal and ovarian imaging and undergo CYP21A2 gene mutation analysis.

FRI241 Late Diagnosis Of Non-classical CAH In An Elderly Male Previously Diagnosed With Estrogen Producing Adrenal Tumor

Abstract Disclosure: R. Kaval: None. J. Subauste: None. In 2013 a 66-year-old Caucasian male presented to the VA outpatient endocrinology clinic to establish care; the patient carried a diagnosis of estrogen producing left adrenal tumor in 1989 at the age of 42 at an outside facility. On review of records the patient was initially worked up for hypogonadism and was noted to have an elevated estrogen level and a “left adrenal tumor”. He subsequently underwent an unilateral left adrenalectomy for treatment and pathology reported with benign tumor findings. Following surgery, the patient was started on steroid and mineralocorticoid replacement for primary adrenal insufficiency (PAI) and topical testosterone gel for hypogonadism. The question was raised as to why this patient had PAI as he had an intact right adrenal gland on CT scan. On laboratory work-up in 2006 the patient had baseline cortisol of 3.4 ug/dl, ACTH was elevated at 99 pg/ml [7.2- 63.3], and adrenal antibodies (21-OH antibody] were negative. Renin was 2.35 ng/ml/hr [0.167-5.380], Estradiol was 41.8 pg/ml [7.6 - 42.6], LH was low at 0.20 mIU/ml [1.2-8.6] and total testosterone was low at 2.2 ng/ml [1.8-7.8]. Repeat testing yielded ACTH that was elevated at 686.7 pg/ml [7.2- 63.3], electrolytes including sodium and potassium were normal. On physical examination the patient had hyperpigmented skin, normal height, weight and otherwise unremarkable. On CT scan of the abdomen the right adrenal gland was mildly diffusely enlarged. In 2019, 30 years after his initial diagnosis the team questioned whether the adrenal tumor was truly an estrogen producing tumor. As a result, 17-OH progesterone was obtained, and the unstimulated value resulted as 3522 ng/dl [27-199]. With the elevated 17 OH >1000 ng/dL this was diagnostic for Non-classical CAH. The unilateral adrenalectomy likely unmasked the underlying impairment of cortisol production leading to primary AI that is seen with Non-classical CAH patients. The patient was lost to follow up during the pandemic, hence we were not able to perform and further testing. Presentation: Friday, June 16, 2023

SAT344 Takotsubo Cardiomyopathy Complicating Cholecystectomy In A Patient With Clinically Silent Pheochromocytoma

Abstract Disclosure: K.B. Huynh: None. T.A. Nguyen: None. J.P. Perdomo Rodriguez: None. Introduction: Pheochromocytomas (PCC) are responsible for 85 to 90 % of all catecholamine-secreting tumors, which originate from chromaffin cells of the adrenal medulla. Normotensive Incidental Pheochromocytoma (NIP) may be a distinct entity and has been reported in up to 55% in case series. Case description: A 48-year-old woman with a history of gestational hypertension presented to the hospital with acute cholecystitis. Shortly after cholecystectomy, she developed severe hypertension and flash pulmonary edema. The hospital course was complicated by an acute ischemic stroke secondary to cardiogenic emboli. She was diagnosed with Takotsubo cardiomyopathy, and a transthoracic echocardiology (TTE) revealed an ejection fraction (EF) between 20% and 45 %. CT scan of the abdomen/pelvis revealed a complex 8.3 x 6.2 x 0.6 centimeter left adrenal tumor. PET DOTATE has been declined. She was discharged on carvedilol 25 mg twice daily but not on ACE inhibitors due to soft blood pressure. She was referred to outpatient endocrinology for pheochromocytoma. Metanephrine level was elevated to 1023 pg/mL, normetanephrine was 2209 pg/mL and the total level was 3232 pg/mL. Adrenal steroids including aldosterone, cortisol, and DHEA sulfate were within normal limits. Chromogranin A was normal. Germline genetic analysis was negative. Hypertension was treated with prazosin titrated slowly to 8 mg three times daily, and carvedilol was continued. Home blood pressure readings ranged between 85/55 and 113/72 without orthostatic hypotension. Pulse ranged between 55 and 73 beats per minute. Weeks later, left adrenalectomy was aborted due to hypertension urgency and bradycardia. However, a TTE revealed normal EF. Carvedilol was discontinued, and treatment was changed to nifedipine and phenoxybenzamine, which was titrated to 40 mg three times daily until she became orthostatic over 4 weeks. She underwent successful left adrenalectomy. Discussion: Normotensive pheochromocytomas have been described in 13.5 % to 55% of incidental pheochromocytoma series. Moreover, hypertensive crises have been reported following surgery in patients with unsuspected pheochromocytoma. Hence, plasma metanephrines should be checked in all adrenal incidentalomas. While normotension is thought to mostly occur when the tumor is too small, a study by Haissaguerre et al found no correlation between size and biochemical parameters in NIP, and the NIP group was characterized by a global reduction in catecholamine and metanephrine secretion and a mean size of 52 +/- 6.7 millimeters. Our case was a much larger pheochromocytoma with 18- and 15-fold elevation in metanephrine and normetanephrine respectively. It has been described that normotensive pheochromocytoma exhibit down-regulation of different proteins involved in key processes of catecholamine metabolism that may lead to reductions in the storage and release of catecholamines. Presentation: Saturday, June 17, 2023

THU094 Multiple Endocrine Neoplasia Type 1 Tumor Recurrence After Loss To Follow Up

Abstract Disclosure: S.J. Sternlieb: None. K.G. Romo: None. R.S. Boothe: None. D. Englert: None. N. Manuel: None. Q.Z. Iqbal: None. Multiple Endocrine Neoplasia Type 1 Tumor Recurrence After Loss to Follow-up Sternlieb, S. J., Romo, K.G., Boothe, R., Manuel, N., Iqbal, Q.Z., Grissett, B., Englert, D. Background: MEN1 is a rare, autosomal dominant tumor disorder due to mutations in the MEN1 tumor suppressor gene. It is characterized by parathyroid, enteropancreatic and pituitary tumors. We present a young woman with MEN1 with prolactinoma, primary hyperparathyroidism and multiple pancreatic neuroendocrine tumors. Clinical Case: A 30-year-old woman with MEN1 presented with complaints of abdominal pain and GI bleeding (GIB). She was diagnosed with a prolactinoma at age 14 after presenting for primary amenorrhea and galactorrhea. She didn’t tolerate medical therapy with bromocriptine or cabergoline and required transphenoidal resection. At age 19, she presented with hypoglycemia and was found to have multiple pancreatic neuroendocrine tumors (NET) as well as a left (L) adrenal gland tumor. She underwent distal pancreatectomy and L adrenalectomy. Ten tumors in the pancreas stained variably for insulin, glucagon, gastrin and VIP. Multiple cortical adenomas were found in the adrenal gland. Post-operatively she developed a spinal cord hematoma related to placement of an epidural catheter for pain control, which led to permanent paraplegia. At age 21, she was diagnosed with primary hyperparathyroidism. She underwent left parathyroidectomy, then was lost to follow-up for 9 years. EGD showed multiple superficial duodenal ulcers. MRI brain identified a right (R) cavernous sinus mass inseparable from the R pituitary. CT abdomen showed a pancreatic head mass. Given the concern for neuroendocrine etiology, she underwent an EUS showing a pancreatic mass and a liver mass. Pancreatic mass pathology confirmed low-grade NET with Ki67 proliferation rate <1%, positive synaptophysin and chromogranin staining. After multidisciplinary discussion, the consensus was to continue PPI and sucralfate for GI ulcers, start lanreotide, and obtain somatostatin receptor scintigraphy. Surgery was not recommended unless significant GIB occurred on maximal therapy. She was referred to follow-up with GI, endocrinology, and NSGY. Since discharge, she has presented several times to other hospitals for abdominal pain, but has not adhered to follow-up. Conclusions: This case of recurrent neuroendocrine and pituitary tumors despite prior resections demonstrates the consequences of prolonged loss to follow-up with MEN1. Without routine surveillance, recurrence is likely. This patient lives in a rural area with lack of access to nearby subspecialty care, which is a barrier. More resources are needed to support patients with social barriers and progressive endocrinopathies. Key words: MEN1, prolactinoma, gastrinoma, NET, health care barriers Presentation: Thursday, June 15, 2023

FRI125 Recurrence Of Primary Aldosteronism Long-Term After Adrenalectomy For Aldosterone-Producing Adenoma With KCNJ5 Gene Mutation: A Two-Cases Report

Abstract Disclosure: K. Aiga: None. M. Kometani: None. D. Aono: None. S. Karashima: None. T. Yoneda: None. Primary aldosteronism (PA) is a major cause of secondary hypertension. PA is known to have higher prevalence of cerebral or cardiovascular complications, indicating the importance of early detection and treatment for PA. PA is caused by autonomous secretion of aldosterone in the adrenal glands. PA is characterized by high plasma aldosterone concentration, low plasma renin activity and hypokalemia. PA is classified into unilateral PA (aldosterone-producing adenoma [APA] or unilateral hyperplasia) and bilateral PA (bilateral adrenal hyperplasia). PA with aldosterone excess in bilateral adrenal glands is defined as bilateral PA. On the other hand, PA with aldosterone excess in unilateral adrenal gland is defined as unilateral PA. Strategies for PA treatment depends on the subtype of PA. Medication by mineralocorticoid receptor antagonists is a common treatment for bilateral PA. Adrenalectomy is the most efficient treatment for unilateral PA. In general, patients tend to maintain normal serum potassium level and blood pressure after adrenalectomy, and recurrence of PA is extremely rare. Recently, mutation in the gene named KCNJ5 was found to derive APA. Herein, we report two cases of PA recurrence more than 10 years after surgical treatment for APA. Somatic mutation in KCNJ5 was detected in the first occurrence of PA in both cases. First case, a 52-year-old woman was examined for hypertension 22 years after total adrenalectomy of the right adrenal gland. Recurrent PA was diagnosed based on high aldosterone-renin-ratio (ARR), identification of left adrenal gland tumor by computed tomography (CT), and a confirmatory test. Second case, a 65-year-old man was examined for hypertension 17 years after total adrenalectomy of the left adrenal gland. He had maintained his blood pressure using medication since the onset of hypertension 4 years after the surgery. A year later, a high ARR was observed. PA recurrence was determined by a right adrenal gland tumor noted on CT and a confirmatory test. Tissues of the adrenal gland were obtained from adrenalectomy in both cases. Histopathological analysis revealed presence of one adenoma in the first case, while two adenomas were confirmed in the second case. Somatic mutation in KCNJ5 gene was detected in both cases. To date, there are no specific guidelines established for the management of recurrent PA. Early detection is crucial for the prevention of severe cardiovascular diseases. Long-term follow-up is recommended after the treatment of PA. Presentation: Friday, June 16, 2023

FRI193 Early Presentation Of Pediatric Oncocytic Adrenal Tumor Associated With Germline TP53 Pathogenic Variant—Report Of Three Cases

Abstract Disclosure: R.R. Oppenheimer: None. L.P. Lins: None. C.P. Oliveira: None. I.P. Magalhaes: None. A. Latronico: None. B.B. Mendonca: None. M.Q. Almeida: None. A.W. Kuhn: None. H. Charchar: None. M.C. Fragoso: None. Oncocytic adrenal tumors (OAT) are uncommon and usually nonfunctional. In the pediatric age group, OAT are extremely rare and less than 20 cases have been reported. Herein we report the clinical presentation and follow up of 3 non-related children with Li-Fraumeni syndrome with a germline TP53 pathogenic variant (PV) (p.R337H) who developed secreting OAT. Case 1. A 19 month old male presented with moon face, weight gain, irritability and pubic hair (Tanner stage P3) for over 4 months. He had no family history of neoplasms and his parents were unrelated to each other. Hormonal data revealed serum cortisol 33 mcg/dL with a suppressed ACTH. Testosterone was elevated (242 ng/dL - RR <12 ng/dL) with low plasma gonadotropins. Androstenedione 3,08 (RR<0,5ng/mL). Abdominal CT revealed a left adrenal tumor (5.0 x 4.4 x 4.0cm). He underwent a left laparoscopic adrenalectomy and histopathologic analysis confirmed OAT Wieneke score 2/9. Immunohistochemistry was positive for inhibin, p53 and Ki67 (10-15%). The postoperative clinical course was uneventful. The TP53 p.R337H PV was inherited from his father. He is now prepubertal and there is no evidence of relapse in 14 months of monitoring. Case 2. An 18 month old female presented with acne, clitoromegaly, pubic hair (Tanner stage 2), axillary odor, axillary hair and weight gain. Hormonal study showed elevated total testosterone of 624.7 ng/dL, androstenedione, DHEA-S and prepubertal gonadotropins. 17OHP and cortisol were normal. Abdominal CT confirmed a heterogeneous solid nodule of 5.5 x 4.5 x 3.7 cm in the right adrenal topography. The patient underwent a right adrenalectomy and OAT was identified by histopathology (Wieneke score 3/9). Immunohistochemistry was positive for p53, inhibin and Ki67 (30%). The TP53 p.R337H PV was inherited from her mother. She had remission of secondary sexual characteristics and he is now prepubertal. Case 3. A 2 years and 8 months old girl was referred with precocious pubarche and clitoromegaly (2,6cm). She had a right flank palpable mass and tanner 1 breasts. Hormonal studies showed elevated testosterone (70ng/dL), androstenedione (3.81ng/mL) and DHEA-S (13490ng/mL), with LH 0.3IU/L and FSH 3.4IU/L. The cortisol after DST was 5.8mcg/dL and ACTH was suppressed. Abdominal MRI revealed a 5 cm tumor at the right adrenal gland. She underwent laparoscopic adrenalectomy. The histopathologic analysis revealed an OAT Wieneke score 1/9. Immunohistochemistry was positive for p53 and Ki67 (13%). The TP53 p.R337H PV was inherited from her father. Conclusion: Although rare, functional OATs have been reported in children. Herein we describe the first patients with the TP53 p.R337H PV to present with OATs. These are the youngest cases of OAT published so far. The clinical presentation, hormonal data and follow-up were not different from those of other pediatric adrenal tumors. So far there is no evidence of disease relapse during follow-up. Presentation: Friday, June 16, 2023

Expanded indication for EUS-guided radiofrequency ablation: management of adrenal tumors

EUS-guided radiofrequency ablation (EUS-RFA) has been performed as an alternative to surgery for the treatment of pancreatic tumors. The promising results obtained using this procedure suggest that the indication for EUS-RFA could be expanded to the management of extrapancreatic tumors. Here, we evaluate the feasibility, efficacy, and safety of EUS-RFA in the treatment of left adrenal tumors. This single-center, prospective study was conducted at Asan Medical Center between January 2016 and November 2021. A total of 11 patients with left adrenal tumors were enrolled in the study. The technical success rate, treatment response, and adverse events were evaluated. EUS-RFA was performed successfully in all patients (technical success rate, 100%). The indications for the procedure were an increase in tumor size (n= 8), and adrenal tumor with adrenal hormone excess (n= 3). After a median of 2 EUS-RFA sessions (range, 1-2), 73% of patients had a complete response, and 27% had a partial response. During follow-up, 5 patients experienced self-limiting mild abdominal pain; no moderate or severe adverse events were reported. EUS-RFA showed high technical feasibility, clinical success, and an acceptable safety profile in the treatment of left adrenal tumors. In patients at high surgical risk, EUS-RFA can be considered as an alternative therapeutic modality to surgery for the treatment of left adrenal tumors.

Adrenal Incidentaloma With the Diagnosis of Normotensive Pheochromocytoma: A Case Report

Background: Pheochromocytomas (PCCs) are catecholamine-producing neuroendocrine tumors that originate from the adrenal medulla. Their clinical presentations most commonly include hypertension, headache, palpitations, and sweating; however, PCCs are sometimes normotensive and clinically silent. Case Presentation: A female patient with abdominal pain as well as persistent and crushing left flank pain for the past six months was examined. The imaging studies revealed a mass in the upper pole of the left kidney indicative of a potential adrenal gland tumor; however, she had normal blood pressure (BP). Histopathologic examination of the mass from exploratory laparotomy showed that it was a PCC. Findings from sonography and computed tomography (CT) scan of the left adrenal tumor, along with elevated urinary normetanephrine level and positive iodine-123 metaiodobenzylguanidine (MIBG) scan led to preoperative diagnosis of PCC in our case. Conclusion: This study highlighted the importance of imaging and biochemical testing in diagnosing PCCs in patients with adrenal incidentalomas (AIs), even though they were normotensive and entirely asymptomatic.

PS-BPR01-5: DOPAMINE-SECRETING PHEOCHROMOCYTOMA: AN OVERLOOKED CAUSE OF INCIDENTALLY DISCOVERED ADRENAL TUMOR AND NORMAL BLOOD PRESSURE

A 64-year-old woman with a history of chronic thyroiditis and no family history of endocrine disease was incidentally determined to have a left retroperitoneal mass on abdominal ultrasonography. A subsequent abdominal computed tomography (CT) scan revealed a 5.6 × 5.1 × 10.6 cm left adrenal tumor (10–50 Hounsfield Units of CT attenuation value), which showed marginal irregularity and an internal cystic structure [Figure 1A]. She was admitted to our hospital for further investigation of a suspected adrenocortical carcinoma. On admission, she was normotensive and had no symptoms of catecholamine excess. The 24-hour urine catecholamine level showed normal norepinephrine (122.9 ug/day), epinephrine (24.3 ug/day), whereas markedly elevated dopamine (148,212.4 ug/day). 123I-MIBG scintigraphy revealed tumor uptake. After alpha-blockade as preoperative management, she successfully underwent laparoscopic left adrenalectomy and was finally diagnosed with an exclusively dopamine-secreting pheochromocytoma. The tumor was histologically comprised of small polygonal cells with high cellularity and was immunohistochemically positive for all three catecholamine synthesizing enzymes: tyrosine hydroxylase (very weak), dopamine beta-hydroxylase (heterogeneous), and phenylethanolamine N-methyltransferase (very weak). Electron microscopy revealed very few catecholamine-containing small vesicles with a few organelles, which reflected immature cells. No biochemical or imaging evidence of recurrence or metastasis was evident 1 year after the surgery. To our knowledge, this is the first case of an exclusively dopamine-secreting pheochromocytoma in which immunostaining of catecholamine synthetic enzymes and electron microscopy of tumor tissues were performed together to identify the mechanism underlying a dopamine-secreting PPGL. The purpose of this report was to identify the mechanism underlying a dopamine-secreting pheochromocytoma from the histopathological findings. We also discuss the reasons for the lack of catecholamine excess symptoms, whether preoperative management of alpha-blockade is needed, and the association between the prognosis and genetic mutation with an extensive literature review. JOURNAL/jhype/04.03/00004872-202301001-00862/figure1/v/2023-10-24T163949Z/r/image-jpeg

PS-R03-2: UNILATERAL PRIMARY ALDOSTERONISM AND CONTRALATERAL GROWING ADRENAL MASS: GROWING CONCERN FOR PHEOCHROMOCYTOMA

Concomitant unilateral primary aldosteronism (PA) and pheochromocytoma is extremely rare. A 52-year-old previously healthy woman with no family history of endocrine disease was admitted to our hospital with suspected PA based on high blood pressure, spontaneous hypokalemia, and a high aldosterone-to-renin ratio. She had no catecholamine excess symptoms other than hypertension. Abdominal computed tomography (CT) showed a right lipid-rich adrenal mass and a left lipid-poor adrenal mass. PA was diagnosed by the captopril challenge test. The 24-h urinary fractionated metanephrines were slightly elevated. Adrenal vein sampling (AVS) confirmed that the right adrenal gland was responsible for aldosterone hypersecretion. Medical therapy with eplerenone was started because the patient refused surgery. Five years later, she requested surgery for PA. The second AVS confirmed right unilateral hyperaldosteronism, as expected. Repeat abdominal CT showed the enlargement of the left adrenal mass. The 24-h urinary fractionated metanephrines had risen to the diagnostic level. 123I-MIBG scintigraphy showed a marked accumulation in the left adrenal tumor with no metastatic lesion. After preoperative management of alpha-blockade, laparoscopic left partial adrenalectomy was performed. Immunohistochemical examination of the tumor showed chromogranin A positivity leading to the diagnosis of left pheochromocytoma. One year after surgery, blood pressure and serum K levels remain well controlled. No surgical treatment for the right adrenal mass has been performed, and PHEO has not recurred. This case highlights the potential pitfalls in the treatment and diagnosis of unilateral primary aldosteronism with contralateral pheochromocytoma. In cases of unilateral PA with contralateral lipid-poor adrenal mass, clinicians should rule out if the contralateral adrenal mass is pheochromocytoma to proceed to undergo unilateral adrenalectomy for the treatment of PA.

PS-R03-1: A CASE REPORT OF NEUROFIBROMATOSIS TYPE 1 (VON RECKLINGHAUSEN'S DISEASE) WITH HYPERTENSION, ASSOCIATED WITH PHEOCHROMOCYTOMA

The patient is a 75-year-old man, who had multiple neurofibromas on the skin since the age 30 and was clinically diagnosed as neurofibromatosis type 1 (von Recklinghausen's disease) at the age 57, although there was no apparent family history. At the age 70, the patient had excerbated hypertension (home blood pressure 160/90 mmHg) and received amlodipine 5 mg/day. At the age 73, a 35 mm left adrenal tumor was incidentally noted on CT performed with an exacerbation of ulcerative colitis. He was diagnosed with left adrenal pheochromocytoma, due to high levels of urinary metanephrine and normetanephrine shown by urinalysis and an accumulation of 123I on the tumor site shown by 123I-MIBG scintigraphy. After adequate alpha blocking, left adrenal resection was performed. Pathology showed that PASS was 2 points, and MIB1 was 2–3%. Recently, two years after the operation, metanephrine and normetanephrine level has not been increased and keeps in normal range. Hypertension has been improved: Office blood pressure is about 130/70 mm Hg, and home blood pressure 120/70 mmHg, taking doxazosin 2 mg /day only, without amlodipine. Neurofibromatosis type 1 is an autosomal dominant genetic disorder caused by NF1 gene abnormality, characterized by cafe Ole spots and multiple neurofibromas. Pheochromocytomas have been clinically identified in 0.1 to 5.7% of patients with von Recklinghausen's disease (J Urol 1999; 162: 1582). When hypertension is observed in patients with neurofibromatosis type 1 (Recklinghausen's disease), it is important to screen for pheochromocytoma.

PS-BPR01-6: A CASE SERIES OF UNILATERAL PRIMARY ALDOSTERONISM TREATED BY RADIOFREQUENCY ABLATION

Background: In primary aldosteronism (PA), early and appropriate management based on its subtype is crucial to abolish an enhanced cardiovascular risk. Mineralocorticoid receptor antagonists (MRAs) are the mainstay of PA treatment, while a unilateral adrenalectomy (ADX) has been considered as the gold standard intervention for unilateral PA, mainly caused by an aldosterone-producing adenoma. The laparoscopic ADX is a less-invasive procedure than open surgery, whereas the procedure harbors a perioperative risk associated with general anesthesia. Herein, we report 3 PA cases where we employed radiofrequency ablation (RFA) as an alternate therapy for their aldosterone-producing tumors. In those cases, RFA successfully and safely led to remission of PA with more reduced physical burden, compared with the laparoscopic ADX. Case presentation: We performed RFA in 3 unilateral PA cases with aldosterone-producing tumors; Case 1, a 46-year-old man with a right adrenal tumor (14 mm); Case 2, a 65-year-old man with a right adrenal tumor (15 mm); Case 3, a 47-year-old man with a left adrenal tumor (19 mm). At baseline, their blood pressure levels were 124/87, 118/79 and 142/88 mmHg under 1, 2 and 2 antihypertensive agents, respectively. Plasma aldosterone concentrations and renin activity levels were as follows; 29.70, 25.7 and 48.6 ng/dL and 0.5, < 0.2 and < 0.2 ng/mL/hr, respectively. In all cases, we confirmed the presence and localization of PA with confirmatory tests and segmental adrenal venous sampling. Before the RFA procedure, we initiated and titrated MRAs to sufficiently block effects of hyperaldosteronism. Percutaneous RFA was guided by computed tomography under intravenous anesthesia without intubation. Complete tumor ablation was achieved within 1 hour, confirmed by enhanced adrenal imaging. No major complication was observed, and physical activity was almost recovered on the same day. Three months after RFA, all 3 patients showed normotension without any antihypertensives. For biochemical parameters, plasma aldosterone levels decreased to 2.47, < 0.40 and 1.41 ng/dL in Case 1, 2 and 3, respectively, followed by mild restoration of plasma renin activity (1.4, 0.5 and < 0.2 ng/mL/hr, respectively). Conclusion: This case series demonstrates that RFA is an alternate treatment for unilateral PA. Based on accurate localization of hyperaldosteronism, RFA could cause necrosis of only an adrenal-producing tumor and spare an ipsilateral adjacent adrenal gland, resulting in remission of PA with minimal physical burden.

Hyperandrogenism caused by a rare adrenocortical oncocytic neoplasm with uncertain malignant potential: a case report and review of the literature.

Hyperandrogenism is a state of androgen excess that can induce hirsutism and oligo/amenorrhea in women of reproductive age. Therapeutic strategies differ according to etiology. Hence, the differential diagnosis of hyperandrogenism is crucial. The adrenal gland is an important organ that produces androgens. One common cause of hyperandrogenism is androgen-secreting adrenal tumors; however, adrenocortical oncocytic neoplasms (ACONs) are rare. A 23-year-old woman presented with severe hirsutism and menstrual disorders for 2 years. Her Ferriman-Gallway hirsutism score was 15 at her first consultation. Her menstrual cycles were irregular, and her menstrual flow had diminished gradually over the past 2 years. She had a remarkable elevation of total testosterone, dehydroepiandrosterone sulfate and androstenedione. Pelvic ultrasonography showed normal morphology of the uterus and bilateral ovaries. Computed tomography revealed a giant left adrenal tumor with a diameter of 12 cm. The patient then underwent robotic-assisted adrenal tumor resection. Histopathological assessment indicated adrenocortical oncocytic neoplasm with uncertain malignant potential. After 4 years of follow-up, no recurrence of symptoms was noted, and this patient delivered a healthy infant on her due date in October 2021. This article reviews the clinical features, diagnosis, and treatment of ACONs and highlights the importance of differential diagnosis for hyperandrogenism in women.

A Left Adrenal Mass on Workup Which Turns Out To Be A Gastric Stromal Tumor at Surgery

A left adrenal mass was incidentally detected in a 61 years old male as he was evaluated for a dysuria. The man had a 20 years-long history of high blood pressure and used to snuff tobacco. All the adrenal hormones were normal levelled but that of the aldosterone. The aldosterone to renin ratio was low. However, the mass turned out to be an extra-adrenal one, typically a stromal tumor pedunculated into the gastric fundus. The urologists should be aware that a gastro-intestinal stromal tumor may misleadingly presents as a left adrenal tumor and consider it especially when an adrenal mass exhibits atypical hormone levels.

Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors– comprehensive adrenal evaluation aiding perfect management: a case report

BackgroundCoexistence of a catecholamine-secreting tumor and an adrenal cortical tumor is quite rare which makes both diagnosis and management challenging. The purpose of this article is to describe the presence of this condition, share a stepwise approach for preoperative evaluation, and review the related literature.Case presentationA 44-year-old male patient had a history of hypertension and aggravating hypokalemia for years. Abdominal computed tomography incidentally found concomitant bilateral adrenal and left para-aortic tumors. Comprehensive adrenal hormone tests revealed a high aldosterone renin ratio and mildly elevated 24-h urine vanillylmandelic acid and norepinephrine levels. Subsequently, a metaiodobenzylguanidine scan showed uptake over the left para-aortic tumor, and NP-59 adrenal scintigraphy showed uptake over the left adrenal tumor. Further confirmatory tests, including captopril suppression, irbesartan suppression, and saline infusion, all confirmed the diagnosis of hyperaldosteronism. Adrenal venous sampling following 2 months of preparation with an alpha blocker demonstrated a left aldosterone-producing adrenal adenoma. Combining hormonal analysis, imaging studies, and adrenal venous sampling, the patient was diagnosed with left adrenal aldosteronoma, right adrenal nonfunctional tumor, and left para-aortic paraganglioma (PGL). Accordingly, laparoscopic left adrenalectomy and left PGL excision were performed smoothly under alpha blocker maintenance. The pathology report confirmed left adrenal cortical adenoma and left para-aortic PGL. Postoperatively, the blood pressure, biochemical tests, and adrenal hormone assays returned to normal, and related symptoms disappeared and were relatively stable during the follow-up period of two years.ConclusionsThis is the first case of left para-aortic PGL coexisting with an ipsilateral aldosterone-producing adenoma presenting as a left para-aortic tumor associated with bilateral adrenal tumors. Awareness of the rarity of this coexistence can avoid unexpected disasters during the process of evaluation and management.

ODP017 A case of an infected pheochromocytoma presenting as an acute abdomen: case report.

Abstract This is a case of a 41 years old gentleman who had presented with a 3 day history of severe left sided abdominal pain associated with vomiting 6 years prior in 2015. He had a CT abdomen at that time which showed acute cholecystitis and a left adrenal lesion arising from the lateral limb with a size of 2.8×2.9cm with a HU of 139 and less than 60% washout. He was treated with antibiotics for 3 days however he took an AOR discharge once he felt better. A 24 hour urine cathecolamines were sent during that admission. Subsequently he had defaulted his follow up and presented again 6 years later in May 2021 to another hospital with severe sharp left hypochondriac pain of 2 weeks duration with a pain score of 6/10 associated with vomiting. He was hypertensive at 190/100mmHg, tachycardic at 117 beats/min, febrile at 40 degrees Celcius and had associated hyperglycemia with a blood glucose of 28mmol/L with no metabolic acidosis. His blood investigations revealed a raised white cell count of14.7×10 9 /L with a normal renal and liver profile. He was started on a intravenous antibiotics, variable rate insulin infusion and kept nil by mouth. An urgent CT abdomen done showed a large mass of 9.8×10.1×12.5cm with a mean HU of 25 suggestive of solid cystic mass arising from the left adrenal. His previous cathecolamines from year 2015 was traced and the epinephrine level was 2x elevated at 46mcg/day. He was started on oral Phenoxybenzamine 10mg BD and further titrated to 10mg TDS. Propanolol 40mg BD was given as well. He was only given oral Phenoxybenzamine for 3 days (usually needing 1 week) as an emergency left adrenalectomy was done due to worsening pain not relieved by analgesics and ileus. He had an open left adrenalectomy, splenectomy and distal pancreatectomy. Intraoperative findings weregeneralized pus contamination, stomach, small and large bowel grossly dilated without any perforation, infected left adrenal tumour 10×8cm with no clear plane with distal pancreas including splenic vein due to desmoplastic reaction. During surgery his blood pressure was ranging between 110-140mmHg (systolic) and 60-80mmHg (diastolic). Intra – op, his blood pressure was supported by a low dose noradrenaline which was discontinued within a few hours post surgery. Histopathalogy examination showed neutrophilic infiltrates forming microabscesses with necrosis amounting 60% of the total tumour. The ragged area of the adrenal gland shows mainly inflammation and fibrosis with no evidence of tumour. A final diagnosis of infected pheochromocytoma was given. After surgery, he recovered well and did not need antihypertensives and was discharged with oral glucose lowering agents. Presentation: No date and time listed

TAKOTSUBO SYNDROME REVEALING PHEOCHROMOCYTOMA

Objective: We report the case of 57-year old woman with no previously known cardiovascular disease. Design and method: Her conventional cardiovascular risk factors were type 2 diabetes (HbA1c< 6.5%), hypertension and active smoking. She presented to the ER for thoracic pain, headache, sweating and tachycardia (150 bpm). Results: Blood analysis showed an elevation of cardiac biomarkers (Troponin 2035ng/l; BNP 541ng/l, Ddimers 19500ug/l), and acute renal impairment (creatinin 158umol/l) while EKG showed inferior NSTEMI. Echocardiography visualized typical takotsubo aspect with apical hypokinesia. Thoraco-abdominal CT-scan showed an important left adrenal tumor. Plasmatic and urinary metanephrine and normetanephrine were high. The patient benefited from adrenal surgery with an optimal result on blood pressure control. Conclusions: Although the association TTS-pheochromocytoma has already been described, it remains uncommon. Catecholamine excessive secretion mediates TTS cardiomyopathy having a negative impact essentially on apical segments increasing wall stress and pressure, but different ventricular segments may be concerned as individual adrenoreceptors distribution patterns vary. TTS diagnosis should legitimate pheochromocytoma screening when associated with labile hypertension.

CUSHINGS SYNDROME WITH BILATERAL ADRENAL MASSES: WHAT MANAGEMENT?

ACTH independent Cushings syndrome (CS) with bilateral adrenal masses is an extremely rare entity. Its management constitutes a challenge to clinicians due to difficulty of exact location of the functional lesions. We herein report a case of a 42-year-old patient with a heavy smoking history who presented a CS for 2 years. Serial examinations revealed ACTH independent CS and bilateral adrenal masses on adrenal computed tomography the largest was on the left measuring 30 x 27 mm. Then the patient underwent a laparoscopic adrenalectomy for left adrenal tumor which diagnosed as an adrenal cortical adenoma with a Weiss Score <2 by the pathological reports. The immediate post-operative course was marked by a corticotroph deficiency that was substituted. The removal of the largest adrenal lesion could represent a therapeutic alternative in the management of bilateral adrenal masses when functional and / or invasive exploration isnt available.