Bilateral Adrenal Masses Research Articles

Extranodal Mantle Cell Lymphoma Presented as Bilateral Adrenal Masses on 18F-FDG PET/CT and 68Ga-DOTATATE PET/CT.

Mantle cell lymphoma (MCL) is a rare type of B-cell non-Hodgkin lymphoma that commonly presents with constitutional symptoms, lymphadenopathy, and laboratory abnormalities. Although less common, extranodal involvement of MCL may be seen at initial presentation and most commonly occurs in the spleen, liver, bone marrow, and gastrointestinal tract. Adrenal involvement in MCL is extremely rare, and only a few cases have been reported in the literature. Herein, we present 18F-FDG PET/CT and 68Ga-DOTATATE PET/CT findings of a unique case of primary extranodal MCL with gastric and adrenal involvement.

A Decade of Insights into Bilateral Adrenal Masses: Epidemiology, Clinical Profiles, and Therapeutic Approaches

Bilateral adrenal masses (BAMs) are rare, accounting for 10-15% of adrenal masses. Their diagnostic and therapeutic approaches differ from those of unilateral masses. This study aimed to analyze the epidemiological, clinical, paraclinical, and therapeutic profiles of BAMs. It is based on a retrospective analysis of 30 patient records from the Endocrinology, Diabetology, and Nutrition Department at CHU Hassan II in Fez, over a 12-year period. The average age of the patients was 53 years, with a male predominance (53.3%). BAMs were symptomatically revealed in 46.7% of cases, incidentally discovered in 23.3%, identified during tumor staging in 20%, and associated with a genetic syndrome in 10% of cases. Clinical examination revealed hypertension in 23.3% of patients, an abdominal mass in 10%, signs of adrenal insufficiency in 10%, hypercortisolism in 6.67%, and hyperandrogenism in 16% of cases. Biological assessment showed elevated urinary or plasma metanephrine derivatives in 9 patients, hypercortisolism in 3 patients, adrenal insufficiency in 3 patients, 11-β-hydroxylase enzyme block in 3 patients, and primary hyperaldosteronism in 1 patient. Adrenal computed tomography (CT) was performed in 29 patients. A staging workup, including PET scan, was requested in 20% of cases. MIBG scintigraphy was performed in 23.3% of patients. The investigations revealed 53% of BAMs were secreting and 47% non-secreting. There were 9 cases of pheochromocytomas, including 5 associated with a syndrome (3 MEN2a, 1 MEN2b, 1 VHL), 8 cases of non-secreting adenomas, 3 cases of congenital adrenal hyperplasia due to 11-β-hydroxylase enzyme block, 2 cases of adrenal metastases, 2 cases of adrenocortical carcinoma, 2 cases of adrenal cysts, 1 case of Conn’s adenoma, 1 case of cortisol-secreting adenoma, 1 case of primary adrenal lymphoma, and 1 case of adrenal hematoma. Treatment included the management of emergencies and preoperative preparation for patients eligible for surgery. Bilateral adrenalectomy was performed in 33.3% of patients, and unilateral adenectomy in 1 case of cortisol-secreting adenoma. Medical treatment was initiated in 16.6% of cases, and palliative treatment in 13.33% of cases. Surveillance alone was implemented in the remaining 33.3%. Prognosis depends on the type of adrenal mass, with outcomes marked by the death of one case of malignant adrenocortical carcinoma and one case of primary adrenal lymphoma. Our study, involving 30 cases of BAMs, highlights their clinical, biological, radiological, therapeutic, and prognostic characteristics. Data in the literature are limited compared to unilateral adrenal masses. Future larger studies are desirable for optimal management.

6606 Connshing Syndrome: An Uncommon Presentation Of Bilateral Adrenal Mass

Abstract Disclosure: C.S. Botero Suarez: None. N. Meda: None. K. Lamar: None. S.K. Suryanarayanan: None. Introduction: Mild autonomous hypersecretion of cortisol in the underlying setting of hyperaldosteronism has been described before and coined the term Connshing Syndrome. Even though it was described in 1979, recently W. Arlt et al in 2017 revived interest in the topic of Connshing Syndrome represents a distinct entity among PA (primary hyperaldosteronisms). We present a case of bilateral adrenal mass associated with mild autonomous hypersecretion of cortisol as well as the development of primary aldosteronism. Case Description: A 62-year-old male with a medical history of hyperlipidemia, hypertension, spontaneous hypokalemia, and T2DM was referred to the clinic due to concerns for hyperaldosteronism. Exam was unremarkable for signs of hypercortisolism. Blood pressure in the clinic was 143/88 mmHg, pulse 81 bpm, weight 105.4 Kg (231.8 lbs.), and a BMI of 28 kg/m2. A non-contrast abdominal CT performed, showed a 2.9 x.1.4 cm right adrenal lipid-rich adenoma with -3.7 HU, as well as a left adrenal lipid-rich adenoma of 1.8 x 1.3 x cm and -7.7 HU. A hormonal workup showed normal plasma metanephrines, an aldosterone of 39 ng/dL, plasma renin activity 0.16 ng/mL/h, and aldosterone/PRA ratio of 243. The patient underwent 1 mg dexamethasone suppression test which resulted in an ACTH <5 pg/mL, dexamethasone level of 288 ng/dL, and cortisol of 1.9 mcg/dL. AVS with Cosyntropin was performed (see Table 1). Right sided adrenalectomy is being considered but the identification of possible lateralization of cortisol is being considered. Discussion: Surgical management is usually preferred treatment modality for both unilateral PA and MACS, but identification of lateralization of both cortisol and aldosterone can be difficult. Although identifying lateralization of aldosterone with Cosyntropin-stimulated AVS is well understood, lateralization of cortisol production is not well understood. Johnson et. all have suggested the use of adrenal venous sampling with dexamethasone suppression as well as measurement of epinephrine levels to identify the source of hypercortisolism. They demonstrated identification of cortisol lateralization with adrenal vein sampling with specificity of 95 – 100% for unilateral disease utilizing a AV/IVC cortisol ratio > 9 and a cortisol lateralization ratio (CLR) of > 2.3. Although our case had a Cosyntropin-stimulated AVS showing clear lateralization of aldosterone production to the right adrenal, the lateralization of cortisol production is yet unknown and AVS with dexamethasone suppression is being considered. Although the treatment considerations are traditionally utilizing the guidelines for both PA and Cushing Syndrome guidelines respectively, very little is known when there are divergence of autonomous secretion amongst the bilateral adrenal masses and more studies are needed to clarify the long-term treatment considerations. 1 Presentation: 6/3/2024

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

7777 The Adrenal Tumor Volumes Help Predict Surgical Indications And Postoperative Recurrence Of PMAH

Abstract Disclosure: S. Suzuki: None. K. Ishiwata: None. Y. Ruike: None. K. Igarashi: None. S. Watanabe: None. M. Sugo: None. M. Fujimoto: None. H. Koide: None. K. Yokote: None. Introduction: Primary macronodular adrenal hyperplasia (PMAH) is characterized by benign bilateral enlarged adrenal masses, causing Cushing's syndrome (CS). Unilateral adrenalectomy of the largest gland has been proposed as a treatment for PMAH. However, there are cases of recurrence or persistence of hypercortisolism after unilateral adrenalectomy, and remaining tumor volumes might be associated. Objective: This study aimed to analyze whether tumor volume helps select treatment and predict postoperative recurrence. Patients and methods: We conducted a retrospective study of thirteen patients (five males and eight females, mean age 59 years) with CS due to PMAH in a single center. Eight patients with PMAH underwent unilateral adrenalectomy, but five PMAH were not. Long-term clinical and biochemical outcomes were analyzed in operated and non-operated patients (112±57 months after unilateral adrenalectomy and 144±49 months after initial diagnosis, respectively). Results: Eight PMAH patients who underwent unilateral adrenalectomy had total tumor volumes greater than 1500 mm2. After unilateral adrenalectomy, when the size of the remaining adrenal tumor was greater than 1000 mm2 (1053, 1268, and 1910, respectively), recurrence occurred along with enlargement of the residual adrenal tumor in two cases, and diabetes worsened in one case. However, when the size of the remaining adrenal tumor was less than 500 mm2 (18.2, 201, 214, 327, and 446, respectively), CS resolved. Among five PMAH patients who did not receive surgery, three PMAH patients with total tumor volume less than 1500 mm2 (856, 1038, and 1496, respectively) were biochemically controlled. However, in two PMAH patients with a total volume greater than 1500 mm2 (1610 and 1650), Cushing's signs, including buffalo hump and central obesity, appeared and worsened metabolic complications during follow-up. Conclusions: Our data suggest that adrenalectomy should be considered in PMAH with a total tumor volume greater than 1500 mm2. Unilateral adrenalectomy might have a risk of recurrence if the remaining adrenal tumor is over 1000 mm2. Further large-scale examination is required. Presentation: 6/3/2024

7648 Bilateral Adrenal Hemorrhage After Laminectomy: A Rare Complication

Abstract Disclosure: M. Rizk: None. S. Sharma: None. H. Qadeer: None. R. Paudel: None. C. Sherpa: None. Introduction: Bilateral Adrenal Hemorrhage (BAH) is an exceedingly uncommon complication of laminectomy, with few cases reported in the literature. In patients with history of any surgical procedure who present with unexplained symptoms of abdominal pain, back pain, hypotension, fever, confusion, or electrolyte abnormalities especially hyponatremia, acute adrenal insufficiency (AI) should be highly suspected. We herein describe a case of BAH following laminectomy. Clinical Case: 63-year-old male with past medical history of hypertriglyceridemia was admitted for post traumatic L1 burst fracture and was treated with T11-L3 fusion and T12-L3 laminectomy. The patient had normal bilateral adrenal glands after the fall on CT thoracic/lumbar spine imaging. The course was complicated by thrombocytopenia initially thought to be induced by therapeutic heparin since heparin antibody was positive. Serotonin-release assay returned negative weeks later ruling out heparin induced thrombocytopenia. Two weeks later, he developed unexplained tachycardia and CT angiogram was done which showed segmental pulmonary embolism and bilateral adrenal nodules measuring at least 3.9 cm on right side and 3.3 cm on left side. He was treated with Eliquis and was discharged home. Two weeks later, he was readmitted with weight loss of 30 lbs, weakness and altered mental status. His vital signs were significant for hypotension and tachycardia. Initial work up showed profound hyponatremia (sodium 121 mEq/L), hyperkalemia (potassium 6.1 mEq/L), hypoglycemia (Glucose 58 mg/dL), hypercalcemia (calcium 11.7 mg/dL) and acute kidney injury (creatinine 2.58 mg/dL). Repeat CT scan of chest/abdomen/pelvis without contrast showed bilateral hyperdense 4 cm adrenal mass/hematoma. Since initial CT of thorax/spine done after the fall showed normal adrenal glands, the new bilateral hyperdense adrenal masses noted post procedure is suggestive of adrenal hemorrhage/hematoma. Undetectable cortisol and high ACTH confirmed AI. Patient was treated with stress dose hydrocortisone followed by slow taper. MRI of pituitary gland showed partial empty Sella however since ACTH was high, empty sella was only an incidental finding and not the cause of AI. He is maintained on hydrocortisone 15 mg in the morning and 5 mg in the evening plus fludrocortisone 0.1 mg daily. His fatigue has improved, he is gaining weight and electrolytes are normal. Conclusions: This case report emphasizes the significance of timely diagnosis and management of BAH, which leads to primary AI. BAH is a rare complication following laminectomy. Clinicians should be aware of this possibility and consider it in the differential diagnosis of postoperative abdominal pain and electrolyte abnormalities, especially hyponatremia. Timely recognition and high index of suspicion should prompt empiric glucocorticoid replacement to decrease mortality. Presentation: 6/1/2024

8365 Bilateral Adrenal Hemorrhage Unveiling Adrenal Metastases In A Patient With Retroperitoneal Sarcoma

Abstract Disclosure: R.J. Shah: None. A.C. Goldberg: None. S. Jasim: None. Introduction: Metastatic disease to the adrenal gland is the most commonly occurring malignancy of the adrenal glands. Adrenal hemorrhage (AH) however is a rare but potentially fatal manifestation of malignancy. This case report highlights the unusual presentation of retroperitoneal sarcoma, manifesting as bilateral adrenal hemorrhage in the context of underlying metastases. Case Presentation: A 53-year-old man presented with a 4-week history of fatigue, intermittent abdominal pain radiating to the back, and vomiting. CT revealed bilateral adrenal masses measuring up to 8.5cm on the left side, with Hounsfield units (HU) 27, and 5.9cm on the right, HU 27, consistent with large areas of bilateral adrenal hemorrhage later confirmed on MRI. Biochemical evaluation included ACTH-stimulated cortisol, which was borderline at 17.4mcg/dl (recommended above 18-20mcg/dl), with a significantly elevated ACTH level of 103.8pg/nl (reference range 7.0-63.0pg/ml). Plasma metanephrines, electrolytes, aldosterone, and DHEA-S were normal. Although hemodynamically stable, he was started on prednisone 5mg daily as he was at high risk for primary adrenal insufficiency (AI). He was referred to endocrine surgery and further workup with PET/CT demonstrated a new FDG-avid periaortic retroperitoneal mass, with an interval increase in the size of the adrenal masses and development of hypoattenuating lesions in the kidneys and pancreas favoring aggressive metastatic disease. He underwent biopsy of the retroperitoneal mass, and pathology was consistent with high-grade sarcoma. Oncology started treatment with dexrazoxane and doxorubicin. His adrenal masses remained unchanged in size, and the patient died related to rapid progression of disease. Discussion: Spontaneous bilateral adrenal hemorrhage is rare with a reported incidence of 0.14 to 1.8%. Although most cases have been associated with sepsis, anticoagulation, hematologic disorders and trauma, it is important to maintain a high level of suspicion for underlying adrenal tumors and metastatic disease. AH can be a diagnostic challenge because of its non-specific presentation, and imaging is vital for timely recognition. Patients with bilateral AH should be managed with corticosteroids to avert AI-related fatalities. Presentation: 6/1/2024

6728 Bilateral Adrenal Hemorrhage: Cause Or Consequence?

Abstract Disclosure: M.N. Rayan: None. R. Horner: None. A. Valenciaga: None. L.E. Ryan: None. Introduction: Before the advent of the CT scan, bilateral adrenal hemorrhage was primarily discovered post-mortem in patients who had succumbed to sepsis, burns, or anticoagulant therapy. Today, non-traumatic hemorrhage is recognized as an infrequent, yet potentially lethal diagnosis causing clinical deterioration in acutely ill patients. Clinical Case: Our patient is a 64-year-old-male with a medical history of Myelodysplastic Syndrome initially presented to an external ED with abdominal pain. CT abdomen revealed hepatomegaly, splenomegaly, and new bilateral adrenal masses—absent in an MRI conducted a year earlier. He was treated conservatively and subsequently discharged. Two weeks later, he returned to the hospital with severe right sided flank pain. His hemoglobin and platelet counts dropped from 11.5 g/dL and 309 K/uL during the previous ED visit to 7.7 g/dL and 80 K/uL, respectively. Additionally, his PT/INR, PTT, fibrinogen, and D-Dimer levels were elevated. A repeat CT scan indicated a significant enlargement of the right adrenal mass, raising suspicion of adrenal hemorrhage. He was appropriately transfused, and a BM biopsy was performed to evaluate for acute transformation of underlying MDS. Cortisol levels were monitored daily for adrenal insufficiency. A complicated hospital course ensued marked by worsening anemia, thrombocytopenia, and coagulopathy, accompanied by high fevers and new-onset atrial fibrillation. Mixing studies showed absence of a factor inhibitor ruling out acquired forms of Hemophilia. Infectious workup including fungal and viral serologies returned negative. A PET scan revealed several small hypermetabolic hypodense liver lesions, and hypermetabolic activity diffusely in the spleen, and adrenal glands leading to suspicion of metastasis. Results of the BM biopsy returned revealing hypercellularity, with aggregates of hemosiderin-laden macrophages replacing the bone marrow space with associated necrosis. Worsening abdominal pain with hematochezia prompted an endoscopy, confirming a diagnosis of metastatic melanoma. Concurrently, seizure activity and altered mentation led to a head CT, revealing new metastatic lesions that confirmed the rapid progression of the disease. Subsequently, the patient was transitioned to comfort care and passed away. Clinical Lesson: It is crucial to review the various causes of adrenal hemorrhage, understand the challenges associated with interpreting adrenal imaging, emphasize the need to monitor adrenal function, and consider the occasional necessity for tissue sampling. In this clinical scenario, metastases with hemorrhage were the most likely etiology. The causes of adrenal hemorrhage are not always apparent; in this presentation, the consequence of the underlying disease state ultimately led to the initial presenting symptom of abdominal pain. Presentation: 6/2/2024

Approach to the Patient With Bilateral Adrenal Masses.

Bilateral adrenal masses, increasingly encountered in clinical practice, manifest across diverse contexts, including incidental discovery, malignancy staging, and targeted imaging after hormonal diagnosis of adrenal disorders. The spectrum encompasses various pathologies, such as cortical adenomas, macronodular adrenal disease, pheochromocytomas, myelolipomas, infiltrative disorders, and primary and secondary malignancies. Notably, not all masses in both adrenal glands necessarily share the same etiology, often exhibiting diverse causes. Recently, the European Society of Endocrinology and the European Network for the Study of Adrenal Tumors updated guidelines, introduced a 4-option schema based on imaging, aiding in targeted hormonal testing and management. This "Approach to the Patient" review delves into the latest advancements in imaging, biochemical, and genetic approaches for the diagnostic and management nuances of bilateral adrenal masses. It provides insights and a contemporary framework for navigating the complexities associated with this clinical entity.

Bilateral adrenal histoplasmosis in a Malaysian tertiary hospital: Report of four cases

Rationale: Adrenal histoplasmosis can present as bilateral adrenal enlargement with constitutional symptoms and/or adrenal insufficiency. Often these patients are initially investigated as secondary adrenal metastases before eventually their diagnosis established by histopathological examination of the adrenal tissues. Patients concerns: We report 4 cases of patients aged 55-78 who presented with hypocortisolism and bilateral adrenal masses. Diagnoses: Bilateral adrenal histoplasmosis. Interventions: Three patients received antifungal treatment for at least one year while 1 patient passed away before the initiation of antifungal treatment. Outcomes: Two patients’ adrenal function recovered not requiring any steroid replacement, while 1 patient still requires long-term steroid replacement. Lessons: Adrenal histoplasmosis should be excluded in patients from endemic areas presenting with bilateral adrenal masses and adrenal insufficiency. Usually patients have an exposure to bird droppings, bat guano or soil contaminated with histoplasmosis fungi. Histopathological examination of biopsied adrenal tissues is still the gold standard investigation as serology and molecular diagnostic methods are still not widely available in most centers in Malaysia.

Surgical Technique and Perioperative Outcomes Following Single-Port Robotic Adrenalectomy: A Single Institutional Experience.

Objective: To describe a single institution's experience with single-port robotic adrenalectomy (SP-RA) and report perioperative outcomes. Materials and Methods: This is a retrospective, single-center study of adult patients who underwent SP-RA between January 2019 and April 2022 by a single surgeon using an IRB-approved institutional database. Patient demographics, perioperative data, surgical pathology, and postoperative outcomes were assessed. Results: Nineteen patients were identified who underwent SP-RA by a single surgeon over the period reviewed. One patient underwent bilateral SP-RA for bilateral adrenal masses, totaling 20 SP-RA procedures performed. Mean operative time was 80.7 ± 22.9 minutes and mean length of stay was 33.1 ± 27.9 hours. Two minor complications (Clavien-Dindo <IIIa) occurred, and one major complication (Clavien-Dindo ≥IIIa) occurred at a mean follow-up of 107.8 ± 159.0 days after surgery. Among 20 surgical specimens, mean mass diameter was 5.2 ± 3.9 cm with one positive margin reported on final pathology (5%). Conclusion: SP-RA provides an effective approach for the surgical management of select adrenal masses with low risk for perioperative complications. Additional studies with increased sample size are needed to validate our findings and to determine the costs associated with performing SP-RA.

Clinical features and imaging manifestations for intravascular large B-cell lymphoma.

Intravascular large B-cell lymphomas are a rare group of extranodal non-Hodgkin's lymphomas with no apparent specificity of symptoms and often a poor prognosis. This study aims to investigate the clinical features and imaging manifestations of intravascular large B-cell lymphoma, to improve the awareness of clinicians and imaging physicians and to reduce the underdiagnosis and misdiagnosis of this disease. Five patients with pathologically confirmed intravascular large B-cell lymphoma diagnosed between December 2011 and February 2022 at Xiangya Hospital of Central South University were retrospectively analyzed for clinical features and imaging manifestations. The age range for the 5 patients in this paper was 44-72 years at an average of (55.4±11.2) years, 2 were male and 3 were female. There were 4 patients with fever, 3 patients with cough, 3 patients with fatigue, and 2 patients with neurological symptoms. The primary sites of lesions were bilateral adrenal glands (2 cases), liver (1 case), central nervous system (1 case), lung (1 case). Imaging of the corresponding site was performed in 5 cases. When the primary site is the adrenal gland, it appears as bilateral adrenal masses or bilateral adrenal thickening with clear contours. When the primary site is the liver, it appears as a hypodense lesion in the right lobe of the liver with "vascular floating sign". When the primary site is the central nervous system, it appears as multiple tumor-like lesions in the frontal-parietal-occipital lobe, right temporal lobe and bilateral cerebellar hemispheres with multiple hemorrhages within the lesions. When the primary site is the lung, it appears as multiple ground glass shadows and solid changes in both lungs with localized lobular septal thickening. The final pathological diagnosis was intravascular large B-cell lymphoma in all 5 patients, one of whom had a good prognosis after chemotherapy, 2 of whom died within 3 days of discharge, and two of whom were lost to follow-up. Intravascular large B-cell lymphoma can involve multiple tissues and organs, and its prognosis is often poor, and its imaging manifestations possess certain specificity. When this disease is highly suspected, a biopsy of the corresponding organ should be performed as soon as possible to clarify the diagnosis.

Niemann-Pick Disease With Bilateral Adrenal Mass

Abstract Niemann–Pick disease (NPD) is a heterogeneous group of lysosomal storage disorders with autosomal recessive inheritance pattern. There are 4 types of NPD. Patients with NPD type B generally have better prognosis, allowing them to survive into adulthood. They have a widespread clinical presentation, affecting multiple organs but rarely neurological involvement. Here, we describe the case of a 38-year-old woman with unexplained hepatosplenomegaly and young hypertensive intracranial bleed. Evaluation for young hypertension further revealed a nonfunctional bilateral adrenal mass and gross hepatosplenomegaly. NPD was confirmed through dried blood spot investigation. It showed low activity of acid sphingomyelinase and genetic testing also detected 2 pathogenic mutations. She is being managed by a multidisciplinary team for supportive treatment that includes regular symptoms monitoring and genetic counseling.

An Unusual Cause of Bilateral Adrenal Incidentaloma: A Case Report of Primary Adrenal Lymphoma

Primary adrenal lymphoma (PAL) is an extremely rare among the causes of adrenal incidentaloma. Most were diagnosed with adrenal insufficiency and B symptoms (unexplained weight loss, night sweats, fever). This article presented a 57-year-old woman who was investigated for bilateral adrenal masses found incidentally on computed tomography (CT). Physical examination and laboratory tests revealed no evidence of adrenal insufficiency or B symptoms. Only 24-hour urinary metanephrine and normetanephrine excretion were increased. Tumour F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET/CT) scan showed the greatest dimension was 14 cm in the left adrenal mass and the maximum standardized uptake value (SUV max) was 26.1 (relative to mean SUV in the normal liver parenchyma, which was 2). An adrenal biopsy was performed after taking adequate precautions against the possibility of a catecholamine crisis. Histopathology revealed high-grade B-cell lymphoma. Bone marrow involvement and brain metastasis were not observed. She received the R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) regimen and intrathecal methotrexate therapy as central nervous system prophylaxis. The patient responded well to treatment, and close clinical follow-up continues. PAL should always be considered when a bilateral adrenal mass is detected.

Primary metaplastic breast carcinoma or breast metastasis of a poorly differentiated squamous cell carcinoma from the lung? Distinction not always clear

Diagnosing primary triple-negative breast cancer (TNBC) with lung metastases or primary TTF1negative lung carcinoma can be difficult due to lack of a TNBC standard immunoprofile.We report a rare case of a woman with an age range between 50 and 55 years, who presented with a mass of the left breast evolving for 2 months. The core needle biopsy concluded to a TNBC. Complete staging with a thoraco-abdomino-pelvic and cerebral computed tomography revealed a huge lung mass associated with laterotracheal and subcarineal adenomegaly next to spinal lytic lesions and bilateral adrenal masses consisting with metastasis. The lung biopsy revealed a poorly differentiated squamous cell carcinoma. The patient was then scheduled to chemotherapy.Pathological and immunohistochemistry studies are limited to establish the formal diagnosis between primary metaplastic carcinoma that metastasize to the lung and primary lung poorly differentiated squamous cell carcinoma. Confrontation with radiology and clinical presentation is highly important to resolve the problem.

FRI179 Etiology And Morphology Of Adrenal Calcifications: Single Center Retrospective Study Of 540 Patients

Abstract Disclosure: A. Kittithaworn: None. P. Dogra: None. L. Rahimi: None. R. Sandooja: None. I. Bancos: None. Background: Adrenal calcification is discovered incidentally on imaging performed for reasons other than adrenal pathology and represents &amp;lt;1% of benign adrenal lesions. Data on the etiology and morphology of adrenal calcifications are scarce. Objective: To determine the etiology and imaging characteristics of adrenal calcification. Methods: We conducted a single-center retrospective study of patients with adrenal calcification on abdominal computed tomography between January 1st,2017 and December 31st, 2021. Medical records were reviewed for demographics, discovery mode, etiology and morphology. Subgroup analysis of patients with calcification/s associated with an adrenal mass was performed. Results: Adrenal calcification was reported in 540 patients (245, 45% women, median age 65 years (IQR 54-74). Only a minority of patients with adrenal calcifications were evaluated by an endocrinologist (167, 31%). Adrenal calcifications were discovered incidentally in 472 (87%) patients, during cancer staging imaging in 66 (12%), and evaluation of other symptoms in 2 (&amp;lt;1%). The etiology of adrenal calcification was most commonly idiopathic (390, 72%), followed by adrenal mass (112, 21%), hemorrhage (20, 6%), and granulomatous disease (8, 1.5%). Adrenal calcifications were bilateral in 34 (6%) patients and unilateral in 506 (94%), with a similar left and right distribution. Unilateral calcifications were more frequently single (399, 79%) with punctate (60%), coarse (28%), linear/curvilinear (9%), or eggshell (3%) morphology. Bilateral adrenal calcifications presented more frequently with multiple lesions (53% vs 21% in unilateral calcifications, p &amp;lt;0.0001), and were coarse (59%), punctate (29%), or linear (9%). When associated with either unilateral or bilateral adrenal mass, calcifications were either central (50, 45%), peripheral (44, 39%), septal (10, 9%), or both central and peripheral (7, 7%) in relation to the adrenal mass. Adrenal masses associated with adrenal calcifications were: adrenal adenomas in 63 (56%), myelolipomas or cysts in 28 (23%), metastases in 12 (11%), adrenal carcinomas in 4 (4%), pheochromocytomas in 2 (2%), and other rare masses in 3 (3%) patients. Patients with adrenal calcifications associated with an adrenal mass were younger at the time of diagnosis (median of 63 vs 66 years, p=0.014), and had a higher proportion of women (61% vs. 41%, p=0.0002), and were more likely to be discovered during cancer staging imaging (21% vs. 10%, p=0.003). Adrenal mass-associated calcifications were more frequently multiple rather than single lesions (46% vs. 17%, p&amp;lt;0.0001). Conclusion: Adrenal calcifications are usually unilateral single punctate or coarse lesions of unclear etiology. The majority of the adrenal masses that demonstrated calcifications were benign, however adrenal malignancy or pheochromocytomas were diagnosed in 17% of cases. Presentation: Friday, June 16, 2023

FRI195 Tuberculosis and Adrenal Glands: The Great Forgotten

Abstract Disclosure: I. Vina: None. R. Sánchez: None. I.C. Fernández: None. C. Vázquez: None. Background: Tuberculous adrenalitis is an extremely rare cause of Addison disease in developed countries. Since more than 80% of the primary adrenal insufficiency cases in the developed world are caused by autoimmune disease, tuberculous adrenalitis often go unnoticed. The aim of this case report is to remember that, even if it is not frequent, TB cannot be overlooked as a cause of longstanding primary adrenal insufficiency, even without evidence of pulmonary or extra glandular TB disease. Herein, we reported a case of bilateral tuberculous adrenalitis, wherein the patient initially presented with gastrointestinal symptoms, hyperpigmentation, bilateral adrenal masses, and no evidence of extra-adrenal tuberculosis. Case report: A 57-year-old woman from Madrid with no medical relevant history, was admitted to our hospital with 4 months of evolution of asthenia, weight loss (-8kg) and nausea that in recent days has been accompanied by fever and anorexia. He also refers progressive hyperpigmentation for the past 3 years. Denies recent travel abroad. On physical examination hypotension (80/53 mmHg) and generalized hyperpigmentation predominantly in the oral mucosa stand out. Blood test: Glu: 41 mg/dL, Na+: 122 mmol/L, K+: 4.8 mmol/L, ACTH: 922 pg/mL, Cortisol: 4.63 ug/dL, Anti-Hydroxylase Ab and Anti-Adrenal glands Ab: negative A computed tomography scan of the abdomen with intravenous contrast revealed bilaterally enlarged adrenal glands with small bilateral calcifications (12-18mm). TB QuantiFERON test: positive. She was initially treated with saline serum and intravenous hydrocortisone causing her symptoms to improve significantly in a few days and was subsequently discharged on hydrocortisone and fludrocortisone and proper treatment for TB Adrenalitis and follow-up in outpatient clinics. Conclusions: Although not frequent, infectious causes should always be kept in mind, such as the etiology of adrenal insufficiency in a patient with long-term compatible symptoms, even though it is a developed country, given world globalization, which makes infectious diseases highly transmissible between countries and continents. Presentation: Friday, June 16, 2023

SAT531 Thyroid Metastasis From Non-small Cell Lung Cancer: An Unusual Case Presentation

Abstract Disclosure: E.Y. Ibrahim: None. I. Guillen Alvarez: None. I. Hulinsky: None. D. Regelmann: None. C. Janowiecki: None. Background: Cases of thyroid tumors metastasizing from the lungs are rare, and their diagnosis is often challenging and can be mistaken for primary thyroid tumors or subacute thyroiditis. Thyroid function tests are typically normal and not helpful in establishing the diagnosis. Ultrasonography commonly shows focal or diffuse hypoechoic lesions. Correspondingly, heterogeneous hypodense areas with mild contrast enhancement are seen on CT scans. Clinical Case: Our patient is a 61-year-old male with a history of diabetes mellitus and 40 years tobacco use, who presented with a 1-week history of right-sided weakness and 3-weeks of dry cough. The weakness of his leg progressed to the point he could not ambulate. Initially, he was evaluated for a stroke and underwent CT imaging showing extensive lesions of the bilateral frontal, temporal, and parietal lobes. These findings were suspicious, and confirmed by MRI, to reflect multifocal metastatic lesions with vasogenic edema. Initial physical examination revealed a palpable thyroid nodule in the right lobe with bilateral cervical lymphadenopathy. CT of the neck demonstrated lymphadenopathy as well as multiple bilateral hypoechoic thyroid nodules, the largest of which measured 0.8 cm in the right thyroid lobe. TSH was 0.34 (0.48-4.17mU/L) with Free T4 at 1.39 (0.8-1.90ng/dL). The patient had denied symptoms of heat intolerance, tremor, or palpitations. A CT chest with contrast found a 3.5 cm cavitary lesion in the right upper lobe concerning for a primary lung neoplasm, as well as multiple bilateral lung nodules, mediastinal and hilar lymphadenopathy, and bilateral adrenal masses. Subsequent ultrasound-guided biopsy of the thyroid tumor revealed histologic features of a poorly differentiated non-small cell carcinoma, with features of a primary lung origin. These features were also present on a concurrent fine needle biopsy of an enlarged cervical lymph node. Immunohistochemistry staining showed the tumor was highly expressive for PDL1, focally positive for p40, pancytokeratin AE1/AE3, and negative for TTF-1 and thyroglobulinDue to the vasogenic cerebral edema, he was given dexamethasone with good response, and his weakness improved to the point that patient was able to ambulate. Treatment also included radiation therapy with subsequent pembrolizumab upon completion of radiotherapy. Conclusion: Several thyroid lesions representing metastases may go unnoticed because the physician’s attention toward lesions at other, more common sites, and detailed thyroid examination can be commonly overlooked. Patients with newly diagnosed malignancies should also be screened for thyroid involvement, including detailed physical, radiographic, and histologic examination of pathologic findings. Presentation Date: Saturday, June 17, 2023

SAT341 Even Mild Cortisol Excess Is Unsafe: Use of Mifepristone In Mild Autonomous Cortisol Secretion

Abstract Disclosure: A. Figueroa Cruz: None. M. Torres Torres: None. A.M. Santiago Carrion: None. Y. Ortiz Torres: None. A. Aponte Velez: None. I.A. Rivera Nazario: None. M.M. Mangual Garcia: None. Cortisol is released from the adrenal glands and works through the glucocorticoid receptor to mediate stress, metabolism, immune and inflammatory processes. Is known as our stress hormone and have manifestations in almost every system as its receptor is present in nearly every tissue. Therefore, even mild cortisol overproduction can lead to serious complications. Mild autonomous cortisol secretion (MACS) is prevalent in nearly half of adults with benign adrenal tumors and can increase the risk of hypertension and type 2 diabetes. It may go undiagnosed if not evaluated properly. There is controversial data on how we should treat this entity. Herein we present a case of MACS denoting the importance of its detection and management. 55yo female comes for evaluation after been found with bilateral adrenal masses in an MRI perform for back pain. Patient with PMHx of diabetes, hypertension, dyslipidemia, and osteopenia refers worsening hyperglycemia and blood pressure. She was previously well controlled with less medications and has needed frequent changes in therapy without achieving treatment goals. Physical exam was negative for discriminatory findings of Cushing’s syndrome. However, she recently noted increase in weight after starting sulfonylurea and insulin for blood glucose control. A CT scan confirmed the presence of adrenal masses measuring 2.5x2.9cm on the left and 2.0x2.4cm on the right with HU 10 and washout 82%. MACS was confirmed with a post Dexamethasone Suppression Test cortisol level of 10.8 ug/dL. Normal cortisol levels were found on Late Nigh Salivary Test done twice. In view of these findings, we can argue about how mild cortisol hypersecretion can be when patient A1c is 10.7% despite multiple therapies for blood glucose and uncontrolled blood pressure despite 3 medications in maximal doses. Considering surgery in this setting is controversial due to bilateral masses with minimal size difference. Since hypercortisolism needed to be addressed it was decided to start medical therapy with the glucocorticoid antagonist Mifepristone, although its use for MACS is considered off label. After mifepristone therapy A1c decreased to 8.0%, prandial insulin was discontinued, and basal insulin decreased. Current regimen for hypertension includes spironolactone to avoid hypokalemia related to mineralocorticoid stimulation of cortisol with mifepristone therapy. Patient symptoms and quality of life has improved significantly. Benign adrenal incidentalomas can cause MACS in up to 35% of cases. Untreated hypercortisolism can lead to complications, and increased mortality risk even when is “mild”. We just need the right amount of cortisol, and minimal excess need to be identified, for which high level of suspicion is needed and more education on this entity and its available treatment options is essential. Presentation: Saturday, June 17, 2023

FRI235 Primary Adrenal Insufficiency As Presenting Symptom Of Disseminated Histoplasmosis And Cryptococcus Co-Infection In Immunocompetent Patient

Abstract Disclosure: A. Thompson: None. Background: Autoimmune adrenalitis remains the most common cause of primary adrenal insufficiency in the United States. Infectious etiologies from fungal sources are much less commonly seen. Below we discuss a case of disseminated histoplasmosis and cryptococcus that initially presented with primary adrenal insufficiency. Case: A 74 y/o F with history of Type 2 DM presented to the hospital with 3 months of fatigue and weight loss. She was initially found to be in DKA and shock requiring vasopressor support. Following the resolution of DKA, she remained hypotensive without obvious cause. A random cortisol was found to be 8.2 [6-25 ug/dL]. She then underwent an ACTH stimulation test and her cortisol level at 30 minutes was 5.9 ug/dL and at 60 minutes was 4.2 ug/dL. An ACTH level was obtained and found to be elevated at 69 [7.2-63 pg/mL]. She was subsequently started on Hydrocortisone and her shock resolved. CT adrenal glands were notable for bilateral adrenal masses measuring 4.5 cm on the right and 4.6 cm on the left with HU of 27. No other suspicious lesions were noted on imaging. An infectious work up was initiated, which was notable for fungal serologies positive of Histoplasmosis with titer of 1:8 (&amp;lt;1:8). Histoplasmosis urine antigen was positive as well. She was tested for Cryptococcus, which was positive with a titer of 1:8 (&amp;lt;1:8). She started treatment with Amphotericin B for 4 weeks followed by Itraconazole for maintenance therapy. Discussion: Primary adrenal insufficiency from invasive fungal infection remains uncommon and is found in less than 10% of cases. Disseminated fungal infections are identified as causes like histoplasmosis and paracoccidiomycosis. Disseminated cryptococcus is an exceedingly rare cause of adrenal insufficiency. These invasive infections can lead to bilateral enlargement of the adrenal glands and calcifications are seen on radiographic imaging. Such cases can also lead to perivasculitis and cause thrombosis and subsequent infarction of the adrenal glands. Disseminated fungal infections are more commonly seen in patients with underlying immunodeficiency such as HIV or those receiving immunosuppressant therapy. This case highlights that these infections can still be seen in those without a compromised immune system. Diagnosis can be challenging given the vague symptoms of adrenal insufficiency but should be considered in patients with electrolyte disturbances, hypoglycemia, and hypotension. There is potential for adrenal recovery following treatment with antifungal therapy. Conclusion: Although autoimmune adrenalitis is the most common cause of primary adrenal insufficiency in the US, this case highlights the importance of evaluating for other potential causes such as infectious sources, which can be treated and potentially have adrenal recovery. Presentation: Friday, June 16, 2023