Suprarenal Mass Research Articles

7213 Severe IGF-2 Mediated Hypoglycemia In A Patient With Adrenocortical Carcinoma: A Case Report

Abstract Disclosure: M. Camelo Pereira: None. P. Itersky: None. S. Sternlieb: None. N. Manuel: None. J. Dendy: None. Background: Hypoglycemia has been rarely described as a complication of adrenocortical carcinoma (ACC). Tumor cells produce insulin-like growth factor II, causing a paraneoplastic syndrome with severe refractory hypoglycemia. We report the unusual case of a patient with known ACC presented with seizures and severe IGF-2 mediated hypoglycemia. Clinical case: A 21-year-old man presented to a regional hospital due to new onset seizures in September 2023. He had been diagnosed with ACC in July 2022, when he presented to the hospital with frank Cushing’s syndrome. CT scan of his chest and abdomen at that time revealed a large left suprarenal mass and multiple lung and liver masses; a biopsy from the liver lesion confirmed the diagnosis of ACC and he was started on treatment with mitotane, levoketoconazole and hydrocortisone. Oncology attempted treatment with 3 cycles of cisplatin and etoposide, with no significant response; subsequently, treatment with Keytruda initially decreased tumor size, but ultimately his CT scan showed progression of disease and mitotane was stopped in August 2023. When brought to the hospital in September 2023 due to seizures and altered mental status, the patient’s blood glucose (BG) was 25 mg/dL (70-110 mg/dL) on point of care testing, so he was started on IV 10% dextrose with improvement in BG and mental status. He underwent endotracheal intubation and was taken to the intensive care unit, showed improvement and was eventually transferred to the general medical floor, where the dextrose infusion was discontinued. However, the following day, he had another hypoglycemic episode complicated with a seizure and had to be reintubated, returning to intensive care. The patient was subsequently transferred to a large tertiary hospital. Evaluation showed undetectable point-of-care BG (< 5 mg/dL) as well as hypokalemia. Fasting labs showed undetectable BHB, undetectable insulin, C-peptide of 0.32 ng/mL (0.78 - 5.19 ng/mL), and negative insulin antibodies. IGF-2/IGF-1 ratio was elevated at 15.6, suggesting IGF-2 mediated hypoglycemia. He was started on IV glucose 20% and glucagon infusions. Mitotane was restarted aiming to decrease tumor burden and consequently IGF-2 levels. Given his hypokalemia, his levoketoconazole dose had to be increased; he was also treated with stress dose steroids. His BG levels improved, and he had partial neurologic recovery, however ultimately curative treatment was not possible given the extent of his disease, so he was transitioned to comfort-focused care. Conclusion: This case illustrates the importance of appropriate workup of IGF-2 mediated hypoglycemia, allowing for the best possible therapeutic approach. In our case, reducing tumor burden was not feasible since the patient had widely metastatic disease, leading to a poor outcome. Presentation: 6/3/2024

8563 Pheochromocytoma Screening In A Patient With Neurofibromatosis Type I And Medication-Resistant Hypertension

Abstract Disclosure: E. Mora: None. A. Bolduc, MD: None. G. Elshimy: None. Title: Pheochromocytoma Screening in a Patient With Neurofibromatosis Type I and Medication-Resistant Hypertension Background: Patients with neurofibromatosis type I (NF1) have a higher risk of pheochromocytoma and it may be missed if no screening done, thus placing the patient at risk of stroke and/or myocardial infarction. The prevalence of pheochromocytoma in patients with NF1 is 2.9% according to a retrospective cohort study by Gruber et al. We present a case of biochemically silent pheochromocytoma in a patient with NF1 and resistant hypertension Clinical case: A 74-year woman with NF1 presented with episodic headaches and labile systolic blood pressure (BP) in the 170-200s mm Hg on three different antihypertensives consistent with pheochromocytoma. Initial tests for renal artery stenosis and pheochromocytoma showed normal plasma MN (0.43 nmol/L, n< 0.50 nmol/L) and NM (0.86 nmol/L, n< 0.90 nmol/L), and normal flow in both renal hila by color Doppler and no large suprarenal masses identified on retroperitoneal US. Duplex imaging of the bilateral kidneys showed no evidence of renal artery stenosis. CT of abdomen/pelvis with and without IV contrast showed a 1.6 cm nodule on Right (RT) adrenal gland that measured 36 HU on unenhanced phase with an absolute washout of 67%. No discrete nodule in Lt adrenal gland. Dotatate PET/CT revealed a Rt adrenal nodule with elevated asymmetric activity (measured 28.1 standardized uptake values (SUV), n for liver: max 12.3, spleen: max 23.5) relative to the normal physiologic activity of the Leftt adrenal gland, which is concerning for pheochromocytoma. The patient was then referred to our endocrine clinic for further evaluation 6 months after the initial presentation. Labs showed normal aldosterone (5.8 ng/dL, n≤39.2 ng/dL), cortisol (7.66 mcg/dL, n: 3.09-22.40 mcg/dL), ACTH (20 pg/mL, n: 7.2-63 (AM collection), and DHEA-S (28 mcg/dL, n:5.3-124 mcg/dL). Normal repeat plasma MN (0.38 nmol/L, n< 0.50 nmol/L) and NM (0.46 nmol/L, n< 0.90 nmol/L). Repeat CT adrenal washout redemonstrated 1.4 cm Rt adrenal nodule that measured 39 HU on non- contrast image with an absolute washout of 68%. Patient will have a surgical resection of the Rt adrenal biochemically silent pheochromocytoma next week and she has been receiving an alpha-blocker prior to surgery to decrease risk for labile BP. Discussion and Conclusion: This case illustrates how CT and DOtatate scan findings can alert providers to the presence of a pheochromocytoma, even in biochemically negative patients. We recommend pheochromocytoma screening in all patients with NF1 to prevent delayed diagnosis and complications of untreated pheochromocytoma. Presentation: 6/1/2024

6701 Bilateral Adrenal Hematomas As Initial Presenting Sign Of Lung Adenocarcinoma

Abstract Disclosure: S. Gumireddy: None. R. Panta: None. Y. Shaikh: None. Introduction: Lung cancer typically presents with dyspnea, chest pain, hemoptysis, weight loss, or recurrent pulmonary infections. This case illustrates primary lung adenocarcinoma presenting with abdominal pain and bilateral adrenal hematomas. Clinical Case: 51-year-old male presented to the emergency department with left sided abdominal pain persisting for one week. CT abdomen showed a left suprarenal mass measuring 4.5 x 5 x 4.3cm indicating an acute adrenal hematoma. An MRI showed a partially solid left adrenal lesion that had hemorrhaged and a 1.3cm hemorrhagic nodule in the right adrenal gland. The patient remained hemodynamically stable. Hormonal evaluation done while patient was in the hospital showed renin level 0.417ng/mL/hr, aldosterone level <1.0ng/dL, epinephrine level 312pg/mL, norepinephrine level 333pg/mL, cortisol 11.3, ACTH level 13, plasma metanephrine levels 93pg/mL, plasma normetanephrine levels 111.7pg/mL, DHEA-S 151mcg/dL, potassium 4.1mmol/L, raising concern for hypersecretion. Patient was discharged with plans for outpatient follow-up but was readmitted in two months due to worsening abdominal pain. Repeat imaging revealed an increase in size of adrenal lesions, measuring 3.3 x 3.6 cm within the right adrenal gland and a 6.7 x 6.3 cm heterogeneously enhancing lesion within the left adrenal gland concerning for metastatic disease to bilateral adrenals and to the ileum causing small bowel obstruction. Chest imaging showed a 2.2cm irregular right apical nodule. Subsequent hormonal evaluation showed normalization of plasma metanephrine levels at 15.8pg/mL, renin level 3.314ng/mL/hr, aldosterone level 4.3ng/dL, deoxycorticosterone level 4.8ng/dL, androstenedione level 40ng/dL, plasma normetanephrines 146.4pg/mL. The patient underwent left adrenal gland mass biopsy which showed metastatic poorly differentiated adenocarcinoma of pulmonary origin. Tumor cells were positive for keratin cocktail, TTF1 and synaptophysin supporting the morphologic interpretation. Patient is currently receiving treatment with carboplatin, pemetrexed, and pembrolizumab. Conclusion: Adrenal masses can be unilateral (85%) or bilateral (15%). While most unilateral adrenal masses are benign and nonfunctional, bilateral adrenal masses are likely to be metastatic disease, hemorrhage, infiltrative disease, congenital adrenal hyperplasia, macronodular Cushing’s syndrome, or bilateral cortical adenomas. Although uncommon, our case highlights the importance of considering metastatic disease to the adrenal glands as a potential initial manifestation of primary cancers. The most likely primary cancers to metastasize to the adrenal glands are breast, lung, colon, kidney, and esophagus. Presentation: 6/1/2024

Delineating the role of laparoscopy in the treatment of large suprarenal masses.

Delineating the role of laparoscopy in the treatment of large suprarenal masses.

Outcome of radiotherapy for metastatic neuroblastoma

Background. Neuroblastoma (NB) is a prevalent solid extracranial tumor that primarily affects children. It is the third most common type of cancer in children, following leukemia and brain tumors. Aim. The study aimed to discuss the clinical outcomes after adjuvant radiotherapy to the primary site in case of metastatic NB after good response to chemotherapy. Methods. A retrospective clinical-dosimetric study of 25 patients metastatic NB treated with radiotherapy at Baghdad Radiotherapy and Nuclear Medicine Center, at Baghdad Medical City Complex, Baghdad, Iraq. Data collection begin from December 2023 and March 2024. Data were collected retrospectively with review of records. The following variables were studied: sex, age, date of diagnosis, site of primary tumor, site of metastasis, chemotherapy, surgery, RT, RT sites, doses of RT, fractions of RT, recurrence sites, date of relapse and survival outcome for each patient. Follow-up period include look up to metastasis, site of metastasis, recurrence, site of recurrence and survival rates. Results. In relation to sex, males (14, 56%) were more than females (11, 44%). The mean age of NB cases was 45.64±17.6 months. In NB, suprarenal masses were the most common site of primary in 21 (84%) of patients. The most common site of metastasis of NB was bone marrow in 12 (48). The multi sites of recurrence was the commonest presentation in 48%. The relapse-free survival (RFS) was 22.38 months. 5 out of 25 patients were alive whereas 20 (80%) of cases were dead. The median follow-up time was 6 years, and the OS of NB in this study after optimal treatment was 2.46 years (95%CI= 1.705-3.21) for survivors. The MS was 4.84 years. The OS was 6 yrs for those diagnosed at 2017, 2 yrs for those diagnosed at 2018, 1.5 yrs for those diagnosed at 2019, 1.75 yrs for those diagnosed at 2020, 3 yrs for those diagnosed at 2021 and 1.5 yrs for those diagnosed at 2022. There was a significant difference among doses (Log Rank (Mantel-Cox)= 11.425, p=0.044). Conclusions. This study is the first study in Iraq that examines the clinical results of radiotherapy on the primary site in cases of metastatic neuroblastoma following induction chemotherapy. The suprarenal masses of neuroblastoma are the most often occurring main site. The primary sites of metastases for neuroblastoma (NB) are the bone marrow and bones. Timely detection and proactive treatment of NB can improve overall results. Reoccurrence of skeletal issues and the infiltration of bone marrow are frequently observed. There were no significant differences observed in terms of relapse-free survival and overall survival based on factors such as sex, age, location of the main tumor, chemotherapy, radiation treatment, surgery, and radiation therapy dosages.

Diffuse pulmonary hemorrhage as a rare presentation of pheochromocytoma: a case report with review of the literature

A 37-year-old male patient with a past history of fluctuating hypertension and insulin-treated diabetes mellitus presented to our clinic with a 1-year history of sudden-onset cough, dyspnea, and frank hemoptysis. Examination showed asthenia, moderate anemia, uncontrolled blood pressure, and a past history of one admission for management of hemoptysis and cardiac failure. The evaluation showed mild anemia, azotemia, low ejection fraction, contrast-enhanced computed tomography (CECT) of the chest, and sputum suggestive of pulmonary hemorrhage. Investigation during current admission revealed proteinuria, left suprarenal mass on USG, and clear lung parenchyma on chest X-ray and CECT. Autoimmune workup for vasculitis was negative and 24-h urinary catecholamine levels were elevated with CECT abdomen confirming pheochromocytoma. His blood pressure was controlled and he underwent laparoscopic left adrenalectomy with confirmation on histopathology. Postoperatively he is doing well with minimal requirement of antihypertensive, and diabetes is controlled with diet and exercise only. This case highlights diffuse alveolar hemorrhage as a rare presentation of pheochromocytoma. This case also highlights that secondary cause of hypertension should always be ruled out in case of uncontrolled hypertension in young patients to prevent devastating complications.

Adrenal Cavernous Hemangioma: A Rare Incidental Finding from A Routine Ultrasound Assessment

Cavernous hemangioma of the adrenal gland is a very rare benign vascular tumour. Its rarity and non-specific presentation pose a challenge to the radiologists and surgeons in making the correct diagnosis. We report a case of adrenal cavernous hemangioma in a 76 years old gentleman, in which a large heterogeneous mass was incidentally found at his left hypochondriac region during a routine KUB and prostate ultrasound assessment for benign prostatic hyperplasia. A multiphasic CT study performed and revealed a large mass with a maximum diameter of 18 cm occupying the left suprarenal area. Considering the large size of the left suprarenal mass with mass effect to the surrounding structures, the patient undergone left adrenalectomy. His final histopathological diagnosis revealed a cavernous hemangioma. This case highlighted literature review and role of imaging in this extremely rare benign tumour as one of the differential diagnosis of an adrenal incidentaloma.

Hepatoid adenocarcinoma masquerading as adrenocortical carcinoma in an elderly male

Background: Hepatoid adenocarcinoma is a malignant lesion arising from an anatomic site other than the liver with histological features similar to hepatocellular carcinoma. Hepatoid adenocarcinoma has been reported from various anatomical sites, but adrenal hepatoid adenocarcinomas are extremely rare.Case Description: A 70-year-old male with well-controlled hypertension presented with chronic abdominal pain and was found to have a large left-sided supra renal mass. The lesion showed radiological features suggestive of malignancy with local invasion. There was biochemical evidence of primary aldosteronism and a non-suppressed overnight dexamethasone suppression test. He underwent open left-sided adrenalectomy: histologically the tumour demonstrated features typical of a hepatoid carcinoma with bile production and immunohistochemical staining for Hep Par-1 and CD10 demonstrating a hepatic canalicular pattern. Adrenocortical-specific immunohistochemical markers (Inhibin and melan A) and neuroendocrine markers (synaptophysin and chromogranin) were negative. He did not have clinical or biochemical evidence of cirrhosis, Hepatitis B or C infection. Triple-phase CT scanning of the abdomen before resection of the adrenal lesion and five months following surgery did not show any significant lesion in the liver suggestive of primary hepatocellular carcinoma, except an 8 mm non enhancing benign-appearing cystic lesion.Conclusion: Most hepatoid adenocarcinomas originate from the gastro-intestinal tract. We present a patient with hepatoid adenocarcinoma masquerading as adrenocortical carcinoma due to misleading imaging and biochemical evidence. Very little is known about their pathogenesis, especially those with an atypical site of origin such as the adrenal gland.

Atypical Radiology Imaging of Squamous Cell Lung Carcinoma: A Case Report and Narrative Review

Introduction: Multiple well-circumscribed round nodules, also known as “Cannonball” appearance in the lungs, are typically associated with specific pulmonary metastases. However, limited reports denote the finding, especially if correlated with primary squamous cell lung carcinoma in the younger population. This is the first case report in Indonesia of a 19-year-old male with primary squamous cell carcinoma in the lung and chest radiograph image mimicking a “Cannonball” appearance Case Presentation: A 19-year-old male was presented with a history of progressing hemoptysis for two weeks with no other abnormal respiratory symptoms but a reduction of vocal fremitus. The subject is an occasional smoker and had a history of lung tuberculosis in 2009 and a suprarenal or adrenal mass. X-ray imaging revealed multiple bilateral pulmonary nodules, mimicking the “Cannonball” appearance. A fine needle aspiration (FNA) biopsy of a nodule was done and interpreted to be primary squamous cell carcinoma. Symptomatic treatment was given and the subject was discharged on the fifth day. Conclusions: A “Cannonball” appearance or its mimic is not always typical to certain carcinoma based on what this case report finds. In the case of a tumor, radiograph findings should also be correlated with other supporting tests such as biopsy and clinical presentation.

PAEDIATRIC ADRENOCORTICAL CARCINOMA: A CASE REPORT

Adrenocortical carcinoma (ACC) is a relatively rare but an aggressive neoplasm that can have variety of manifes­tations. It can manifest in the form of virilization, hyperaldosteronism, cushingoid features or combination of that. Due to rapid appearance of signs and symptoms in children, these tumours can be diagnosed early however if delayed can bear worse prognosis as they tend to metastasize early in course of disease progression. We report a case of 4 years old female patient from Chitral, Pakistan who was referred to Endocrinology Department from Paediatric Department of Hayatabad Medical Complex, Peshawar Patient presented at paediatric OPD with com­plaints of weight gain, hirsutism, rashes over the face and acne. Patient had typical moon facies, buffalo hump and distended abdomen without striae as well as mass in the left hypochondrium on examination. On further evaluation clitoromegaly and pubic hair tanner stage 4 were found. Both Ultrasound and CT-Scan showed left supra-renal soft tissue mass invading the inferior venacava (IVC). She was further evaluated and her overnight dexamethasone suppression test (ODST) and serum testosterone levels were high although serum sodium and potassium were normal ruling out hyperaldosteronism. Next, biopsy was performed which was consistent with adrenocortical carcinoma. ACC although rare is highly aggressive tumor that requires early detection and diagnosis specially in children. If diagnosis is delayed prognosis may not be favourable. It therefore requires avid clinical suspicion to proceed onto diagnostic lines for patient’s benefit as well as physician’s satisfaction.

Suprarenal Mass with Hypertension: A Diagnostic Dilemma

Suprarenal mass with hypertension points towards endocrine etiology like phaeochromocytoma,primary aldosteronism and cushing’s disease. Here we report a 50 year old female who presented with adrenal mass and hypertension. On radiology and histopathology, she was reported as having benign adrenal myelolipoma. Her pre-operative and post-operative vanillylmandelic acid in 24 hrs urine were within normal limits. Rarely adrenal myelolipoma can be associated with catecholamine secretion. This case is reported here to highlight the importance of good clinical workup when faced with the diagnostic dilemma of a functional adrenal myelolipoma or pheochromocytoma as a differential to suprarenal mass associated with hypertension along with brief review of few rare case reports.

Adrenocortical tumors in children: Sri Lankan experience from a single center, and a mini review

BackgroundPediatric adrenocortical tumors include both benign adenomas and highly virulent malignant tumors. However, they are very rare among children. The aim of this study is to evaluate the clinicopathological data of children presenting with adrenocortical tumors and assess their survival in a South Asian population.Case presentationThis is a retrospective cohort study that includes patients diagnosed with adrenocortical tumors from August 2020 to August 2022 followed-up at Lady Ridgeway Hospital. Seven children were diagnosed with adrenal cortical tumors. Their ages ranged from 10 months to 6.5 years. Five of them were boys. All displayed signs of peripheral precocious puberty. One boy phenotypically had features of Beckwith–Wiedemann syndrome. The median time for diagnosis after the onset of symptoms was 4.4 months. The preoperative diagnosis was based on clinical manifestations, elevated dehydroepiandrosterone sulfate levels, and suprarenal masses on computed tomography. All five boys had right-sided suprarenal masses, while the two girls had them on the left side. All underwent surgery for tumor resection. The diagnosis was confirmed based on the histopathology of the adrenal specimens. Four children had a Wieneke score of 4 or more, suggesting the possibility of adrenocortical carcinoma; however, only two of them behaved as malignant tumors. To date, two children have developed local recurrences within a very short period.ConclusionAdrenocortical tumors are uncommon in children, and treatment options are limited. To identify early recurrences, routine clinical, radiological, and biochemical examinations at least once every 6–8 weeks is important.

Angiogenesis Imaging of Adrenocortical Carcinoma with Ga-68-NODAGA-RGD Positron Emission Tomography: Opening New Horizons in Multimodality Imaging from Theranostic Perspective.

A 53-year-old female, with a known case of adrenocortical carcinoma (ACC), underwent F-18 fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) for initial staging, which revealed FDG avid large left suprarenal mass contiguous with hypermetabolic tumor thrombus in the inferior vena cava (IVC) through the left renal vein. Thereafter, she underwent angiogenesis imaging using Ga-68-NODAGA-RGD PET/CT, which showed similar avid tracer uptake in both primary and IVC thrombus. Demonstration of RGD avidity in ACC in this case opens a new horizon for targeted radionuclide therapy (e.g., Lu-177 RGD) in selected patients, who may have limited therapeutic options.

FETAL BILATERAL ADRENAL HEMORRHAGE

Background. Fetal supra renal mass revealed incidentally by routine antenatal ultrasound is a great challenge for diagnosis and management by a surgeon. This is a matter of parental anxiety and diagnostic dilemma to a physician. Indeed, such masses turn out to be complicated by an intra-tumor hemorrhage in neuroblastoma or antenatally diagnosed adrenal hemorrhage. The first one needs intensive management and the latter needs watchful observation. Objective. A case of bilateral fetal adrenal mass revealed by routine fetal ultrasound examination at 28th week of gestation which turned out to be adrenal hemorrhage is presented. This is aimed to make awareness to ensure that clinicians always keep benign etiologies first and thoroughly investigate in case of incidentally detected fetal adrenal mass. Methods. The study is a single case report of incidentally revealed supra renal mass. This case report encompasses differentiating features between the two and investigations that aid the surgeon to avoid unnecessary intervention in a benign hemorrhage. Results. The baby was kept on follow up with serial ultrasound scans in the postnatal period and by the second scan in a month, the hemorrhage had resolved completely. Conclusion. In cases of benign looking masses like adrenal hemorrhage or spontaneously resolving neuroblastoma, appropriate antenatal assessment and close monitoring with serial ultrasound scans can avoid surgery.

Primary adrenal leiomyosarcoma

Primary adrenal leiomyosarcoma is a very rare mesenchymal tumor that arises from smooth muscle cells in the wall of the central adrenal vein or its branches (1). Less than 50 cases have been published in the English literature (2). The tumors are aggressive and often metastasize. This report describes a case of primary adrenal leiomyosarcoma that presented as intermittent left flank pain of 6 months duration in an otherwise healthy 58-year-old Caucasian female. The patient was initially imaged with an abdominal ultrasound, which revealed a left suprarenal mass. A follow-up CT of the abdomen and pelvis confirmed a malignant appearing left adrenal mass. A subsequent PET-CT demonstrated increased metabolic activity within the adrenal mass without evidence of metastasis. Biopsy proven metastasis eventually was detected on surveillance CT studies over the course of 2.5 years. Since this is such a rare malignancy, documenting its imaging findings with multiple modalities is of importance to add to the medical literature and help further characterize its imaging features.

Adrenal incidentaloma: A case report of Pheochromocytoma (PCC) with atypical radiological features

Abstract Introduction Adrenal incidentaloma (AI) is an adrenal lesion ≥ 1 cm discovered accidentally during an imaging technique not aimed to assess the adrenal gland (1). Most AIs are benign and nonfunctioning, about 20% are functioning in form of hypercortisolism, hyperaldosteronism, pheochromocytoma (PCC) or sex steroid overproduction (2). Computed Tomography (CT) scan is vital in differentiating adenoma from non-adenoma. Triphasic CT adrenal protocol (TCTAP) with absolute percentage washout (APW) has good sensitivity and specificity in diagnosis of AI. Here we present a case of PCC with atypical CT features. Clinical Case A 45 years old male patient presented with abdominal pain, abdominal US revealed a right suprarenal mass of 55 mm and referred to endocrinologist for further management. During assessment, the patient had attacks of hypertension, no cushingoid features, BMI was 35 kg/m2 and his blood pressure 180/120 mmHg. Normetanephrine was very high (1016.9 pg/mL), cortisol (10.1 μg/dL), suppressible serum cortisol (1.7 μg/dL), normal DHEA-S (110 μg/dL) and ACTH was 13 pg/mL. Aldosterone, renin, aldosterone/renin ratio (ARR), Metanephrine, K+ are normal. TCTAP showed a homogenous 51×46 mm right adrenal mass of a native Hounsfield units (HU) of 25, portal phase HU of 86, delayed phase (at 15 minute) HU of 36 and APW was 81%, which are features consistent with lipid poor adenoma (LPA) rather than PCC or carcinoma. The patient referred to surgery, right adrenalectomy had been done after good preparation and the diagnosis of PCC had been confirmed by histopathological (HP) and immunohistochemical (IHC) staining. TCTAP used to differentiate adenomatous from non-adenomatous AI with good sensitivity and specificity (3). PCC is an uncommon with variable clinical presentation ranging from severe symptoms to very mild and even sometimes discovered incidentally as AI with about 1.5–14% of all cases of AIs (1). PCC has heterogeneous texture, variable sizes, native HU > 10, avid enhancement and slow washout (4). The present AI was homogenous, has a native HU of 25 and has avid enhancement at portal phase reaching to 86 HU, but the APW was 81% which is atypical for PCC, making lipid poor adenoma at the top list of differential diagnoses. Hormonal assessment revealed the diagnosis of PCC which was confirmed by histopathological (HP) and immunohistochemical (IHC) staining. An American study showed that two of eight PCCs have an APW > 60%, but both of these cases have native HU< 10 and the APW had been calculated at 10 minutes (5). Coincided with our patient and our protocol of 15-minute APW calculation, Yoon et al presented a case of PCC with native HU of 37, portal phase HU of 127 and delayed phase HU of 62 HU with APW of 72% (6). Conclusion PCC is serious condition, could be asymptomatic and may be presented with a typical radiological feature, so biochemical and hormonal assessment is a cornerstone in management of AI.

A Rare Presentation of Adrenal Leiomyoma Arising in a Ganglioneuroma: A Case Report

Leiomyomas are benign tumours of smooth muscle origin and can occur in any part of the body with adrenal gland being an uncommon site. Ganglioneuromas of adrenal gland are benign neural crest-derived tumours. Both of these tumours are usually detected incidentally. Hereby, the authors report an unusual case of a 31-year-old woman, who presented with paroxysmal attacks of headache, palpitations and flushing pointing towards a hormone secreting adrenal tumour. However, functional adrenal markers were normal. Radiological investigations revealed 8×3 cm heterogeneously enhancing left suprarenal mass with calcification and extension to left renal vein. Patient underwent left nephrectomy and adrenalectomy with a high clinical suspicion of malignancy because of tumour extension to the renal vein. The histopathological examination revealed a leiomyoma arising in a background of ganglioneuroma which was later on confirmed using immunohistochemistry. Authors have presented the present case because of atypical presentation as a hormone secreting adrenal tumour with radiological features of malignancy and the unique histomorphology of the combined occurrence of leiomyoma and ganglioneuromatous area in the adrenal gland. To the best of author’s knowledge, this is the first case report of such an occurrence in the adrenal gland.

An Atypical Manifestation of Pheochromocytoma Crisis: Acute Delirium

Pheochromocytoma is a rare neuroendocrine tumor presenting with a classical triad of symptoms. In 10% of the cases the presentation can be atypical, and the diagnosis is often missed for several years. Most common neurological manifestations are haemorrhage, seizures, etc., but pheochromocytoma crisis presenting as delirium has been infrequently reported. We present a case of a middle-aged female who presented with hypertensive emergency and acute confusional state. Other causes of delirium were ruled out in the patient and a timely diagnosis of pheochromocytoma was made with CT abdomen showed a large right suprarenal mass which was confirmed on 123I-MIBG scan and supported by elevated plasma metanephrines. She responded to alpha and beta blockers following which her blood pressure spikes were controlled and subsequently her sensorium improved significantly. Patient underwent surgical resection of the tumor and was followed up after 2 weeks with her blood pressure controlled without any medications.

PSAT019 A Rare Case of Massive Bilateral Adrenal Myelolipomas in a Patient with Congenital Adrenal Hyperplasia

Abstract Background Myelolipomas are rare, benign tumors of the adrenal gland that consist of adipose and hematopoietic tissue. Most present unilaterally and rarely exceed 4 cm, but bilateral and large myelolipomas have been reported. The development of these neoplasms has been associated with congenital adrenal hyperplasia (CAH) (1). Clinical Case A 44-year-old woman with a history of simple virilizing CAH follows in endocrine clinic. She was diagnosed with CAH at birth and underwent vaginoplasty procedures at age 8 and 16. She has been on glucocorticoid therapy with hydrocortisone or prednisone throughout her life, but has had long gaps in treatment (sometimes up to a year) and reports often skipping doses due to concerns regarding facial swelling. She has not had a menstrual cycle for many years and reports shaving her facial hair daily. Her ACTH has been as elevated as 830 pg/mL, 17-alpha-OH-P has been as elevated as 17740 ng/dL, and testosterone as elevated as 401 ng/dL. She now presents for evaluation of an umbilical hernia. She did not have any abdominal or flank discomfort. CT abdomen and pelvis with contrast was obtained and showed bilateral, massive (approximately 25 cm and 18 cm in the largest dimensions on each side) suprarenal masses thought to most likely represent adrenal myelolipomas. She was referred to endocrine surgery and is undergoing discussions regarding possible adrenalectomy. Since the discovery of these adrenal masses, she has been consistently taking prednisone 5 mg daily. Conclusion This case illustrates the potential role of poorly-controlled CAH in the development of massive adrenal myelolipomas. Many hypothesize chronic ACTH stimulation can cause metaplasia of adrenocortical cells and lead to the development of myelolipomas. Review of the medical literature reveals no clear consensus on the management of these rare neoplasms (2). References (1) Al-Bahri S, Tariq A, Lowentritt B, Nasrallah DV. Giant bilateral adrenal myelolipoma with congenital adrenal hyperplasia. Case Rep Surg. 2014;2014: 728198. doi: 10.1155/2014/728198. Epub 2014 Jul 16. PMID: 25140269; PMCID: PMC4124659. (2) German-Mena E, Zibari GB, Levine SN. Adrenal myelolipomas in patients with congenital adrenal hyperplasia: review of the literature and a case report. Endocr Pract. 2011 May-Jun;17(3): 441-7. doi: 10.4158/EP10340.RA. PMID: 21324823. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

ODP059 Torn Between Two Adrenal Cancers: A Case Report

Abstract The adrenals are affected by Non-Hodgkin's lymphoma (NHL) in only 1 percent of cases. We report a rare case of NHL presenting as a large irregular mass at the adrenal gland in an elderly male. A 62-year-old male with hypertension and diabetes presented with 6-month history of vague right sided abdominal pain with recent extension to the left flank accompanied by body malaise and unintentional weight loss. Abdominal ultrasound revealed a 9-cm left suprarenal mass in addition to bilateral nephrolithiases and prostate enlargement. There was also left-sided minimal pleural effusion. CT scan and MRI contrast studies both confirmed a large left adrenal mass with infiltration of the spleen, pancreatic tail, and left kidney. High-Resolution CT scan of the chest confirmed left-sided pleural effusion with subsegmental atelectasis. Hormonal tests showed normal 24-hour urinary metanephrines, 24-hour urinary free cortisol, aldosterone-renin ratio, and serum DHEAS. Intact PTH and calcium were also normal. Multidisciplinary consultations involving endocrinology, medical oncology, hematology, pulmonology, surgical oncology, urology, thoracic cardiovascular surgery, anesthesiology, radiology, and pathology were done. He underwent exploratory laparotomy with resection of left adrenal mass and en bloc left nephrectomy, splenectomy and distal pancreatectomy. Histopathologic findings and immunostaining were consistent with diffuse large B-cell lymphoma. He completed R-CHOP chemotherapy and adjuvant radiotherapy with good clinical response and he has been on remission for two years. Presentation: No date and time listed