Bilateral Adrenal Enlargement Research Articles

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.

Adrenocortical hemorrhage following intravenous tetracosactide in a dog with hypercortisolism.

To summarize findings from a case of adrenocortical hemorrhage following tetracosactide injection during ACTH stimulation testing for monitoring of trilostane therapy in a dog. A 12-year old neutered male dog with adrenal-dependent hypercortisolism. 4 hours after ACTH stimulation testing, the patient developed vomiting, lethargy, and abdominal pain. Abdominal ultrasound was performed before and after an ACTH stimulation test. Following ACTH stimulation testing, there was progressive bilateral adrenal enlargement and free abdominal fluid had developed. This was considered to be caused by adrenocortical inflammation and hemorrhage secondary to the synthetic ACTH analog, tetracosactide, used during stimulation testing. A resting cortisol performed 5 hours after tetracosactide injection was not consistent with iatrogenic hypoadrenocorticism. The patient was managed with analgesia, IV fluids, and corticosteroids and made a full recovery. To the authors' knowledge, this was the first reported case of adrenocortical hemorrhage following administration of a synthetic ACTH analog in a dog. This should be considered as a rare potential complication of ACTH stimulation testing.

FRI226 Ectopic Cushing’s Syndrome Of Unknown Primary Origin With Etomidate Use

Abstract Disclosure: C. Ohammah: None. J. Lloyd: None. T. Abdulwahid: None. R. Tangri: None. C. Musgrove: None. K. Selk: None. Background: Ectopic Cushing's syndrome (ECS) is rare and requires adrenolytic agents to control hypercortisolism until a definitive plan can be made to treat the underlying cause. We present a case of ECS of unknown primary origin causing immunosuppression due to the hypercortisolism that responded with etomidate infusion. Case: A 61-year-old male who presented to the hospital after a few weeks of increasing weakness, dyspnea, and hemoptysis. In the months prior to his admission, he was found to have persistent hypokalemia, COVID pneumonia, and new onset diabetes mellitus. On admission, his examination was remarkable for proximal hip flexor weakness. Labs were significant for metabolic alkalosis, hyperglycemia (A1c 7.7%) and persistent hypokalemia requiring about 300 mEq of potassium daily. CT chest, abdomen and pelvis was remarkable for aspiration pneumonia, vertebral compression fracture, and bilateral adrenal enlargement. Blood cultures grew Staphylococcus Aureus (MSSA) (suspected lung source) and Candida albicans. Treatment with vancomycin and caspofungin was initiated and narrowed to cefazolin after speciation. His MSSA cleared rapidly however, fungemia was persistent after 1.5 weeks of treatment. MRI brain was unremarkable for causative lesion. Further testing revealed elevated morning cortisol (136.2ug/dL) confirmed on repeated testing and elevated ACTH (653pg/mL). Serum metanephrines were normal. A high-dose dexamethasone suppression was unremarkable. In the following days, he developed respiratory failure secondary to a spontaneous hemothorax requiring intubation and ICU admission. He was started on an etomidate infusion given abnormal liver enzymes and unavailability of metyrapone. The infusion was titrated every 6 hours based on cortisol levels. His liver enzymes improved and ketoconazole was added but later discontinued due to development of thrombocytopenia. His cortisol level decreased (21.5ug/dL) and fungemia cleared. Arrangement for bilateral adrenalectomy was made but the patient decompensated and developed shock requiring pressors. After a goals of care discussion, he was transitioned to comfort measures and eventually passed. Conclusion: ECS is rare, and the diagnosis is challenging and often delayed. Ectopic ACTH can result from various malignancies. Medical management with adrenolytic agents should be considered early in the management of ECS until a definitive plan is made. Etomidate, ketoconazole, metyrapone, and mitotane can be used to treat hypercortisolism to achieve medical adrenalectomy. Bilateral adrenalectomy should be considered in patients without adequate response to adrenolytic agents. This case describes the severity of hypercortisolism from an ectopic ACTH production causing refractory hypokalemia and immunosuppression resulting in fatal infections. We highlight the use of etomidate to control hypercortisolism. Presentation: Friday, June 16, 2023

THU518 Ectopic Cushing Syndrome In A Case Of Ewing Sarcoma

Abstract Disclosure: M. Fasen: None. S.E. Mayson: None. Background: Ewing sarcoma (ES) is a rare and aggressive neoplasm occurring in bone and soft tissue in children to young adults. It is part of a spectrum of neoplastic diseases known as the ES family of tumors that display varying degrees of neuroendocrine features. Ectopic Cushing’s syndrome (ECS) is also a rare but severe condition associated with unregulated ACTH or CRH secretion by neuroendocrine tumors (NETs) that can cause severe hypercortisolism. The most common tumors associated with ECS include small cell lung carcinoma, bronchial carcinoid and medullary carcinoma of the thyroid gland. Sarcomas causing ECS are extremely rare with only a handful of cases reported in the literature, mostly in children. Herein we detail what we believe to be the first reported case of ECS occurring in an adult male patient with ES. Clinical Case: A 23-year-old man with ES metastatic to pancreas, liver and bone was admitted to the hospital with acute on chronic back pain and lower extremity weakness, and subsequently diagnosed with cord compression and neutropenia from chemotherapy. New onset hypertension and refractory hypokalemia were observed prompting endocrinology consultation. Physical examination revealed facial fullness, central obesity and wide violaceous striae on his abdomen and axillae. Biochemical evaluation demonstrated blood glucose consistently over 300 mg/dL, serum potassium 2.5 mmol/L, renin 0.9 ng/ml/hr (0.2-1.6 ng/ml/hr), aldosterone renin ratio < 3.3, morning cortisol 73 ug/dL (4-22 ug/dL), and ACTH 1020 pg/mL (72-63 pg/mL). A 1 mg overnight dexamethasone suppression test yielded a serum cortisol of 90 ug/dL and 24-hour urine free cortisol was 6388 ug/d (<60 ug/d). MRI brain demonstrated a normal pituitary gland, and FDG PET-CT showed new areas of uptake in bone concerning for metastatic progression and new bilateral adrenal enlargement without nodularity. High dose dexamethasone suppression testing was deferred in the context of rapid neuropsychiatric deterioration characterized by somnolence and psychosis. The decision was made to treat immediately with spironolactone and ketoconazole, as well as insulin for hyperglycemia management. His mental status, blood pressure, serum potassium, glucose and cortisol improved following the initiation and titration of medical therapy. He was discharged home with addition of metyrapone and hospice support. Two months later the patient succumbed to his illness. Conclusion: The severity of hypercortisolism is a negative predictor of survival and prompt control should be the main therapeutic goal along with prevention of cortisol-induced complications in patients with ECS. ACTH and CRH immunostaining of the patient’s ES histopathology slides is pending. Presentation: Thursday, June 15, 2023

FRI220 Primary Bilateral Macronodular Adrenal Hyperplasia: A Rare Cause Of Cushing Syndrome

Abstract Disclosure: Y. Ortiz: None. A.M. Aviles Melendez: None. M.M. Mangual Garcia: None. J.M. Garcia-Mateo: None. Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a highly heterogenous entity whose pathophysiology remains largely unclear, representing less than 2% of Cushing syndrome (CS) cases, which leads to a challenging diagnosis. Many patients have a mild clinical picture and remain undiagnosed until abdominal imaging for unrelated reasons reveals bilateral adrenal enlargement. Hereby, we describe the case of a patient presented with abdominal pain and clinical hypercortisolism due to PBMAH. Case of a 42-year-old female with past medical history of arterial hypertension, referred for evaluation due to bilateral adrenal masses found on abdominal CT scan performed after patient presented with abdominal discomfort. On initial evaluation, patient refers a 50 pounds weight gain, increase in abdominal circumference, muscle weakness and sudden onset of facial and abdominal hair growth. Physical examination reveals dorsocervical fat pad, proximal muscle weakness, moon facies, facial and abdominal hirsutism. Abdominal CT scan with adrenal protocol showed bilateral adrenal lesions, with largest measuring 2.8 and 3.9 cm on the left side, with attenuation of 16 and 7.4 Hounsfield units. The absolute and relative washouts for the first were 76.6% and 61.6%, and for the second 73.5% and 67.1%, both consistent with adenomas. Laboratory results showed late night salivary cortisol levels of 4.60 ug/dl and 4.80 ug/dl, values which may be high or even normal due to ineffective steroidogenesis on PBMAH. Other labs consisted of low dose 1 mg dexamethasone suppression test: 4.80 ug/dl, and ACTH level: 9.60 pg/ml, which confirms clinical hypercortisolism, indeterminate for ACTH-independent cause. Desmopressin test was not performed, since levels may not be fully suppressed in PBMAH due to ectopic production by adrenal glands, leading to an erroneous diagnosis of ACTH-dependent CS. DHEA level of 618 ug/dl can also be increased, which could lead to a diagnosis of adrenocortical carcinoma. Considering risk of recurrence, risk for adrenal insufficiency, and lower documented complications, unilateral left-sided adrenalectomy was chosen as the best surgical treatment, despite bilateral adrenalectomy being the gold standard for diagnosis. Surgical pathology report showed Adrenocortical adenoma with associated myelolipomatous foci of 7.5 cm in greatest dimension. Despite limited data regarding unilateral adrenalectomy, it appears to convey clinical and biochemical benefits in most cases. Initial remission reported reaches more than 90% with low risk of recurrence between 10-15%. Adrenal insufficiency, despite the presence of residual hyperplastic adrenal tissue, occurs in about one-third of patients and is usually transient. Our case is a great example of the importance of individualization of management options based on therapy benefits, and decreased risks of future complications. Presentation: Friday, June 16, 2023

Hyperpigmentation on the dorsal tongue

•A 73-year-old man had hyperpigmentation on the dorsum of the tongue.•His-symptoms were consistent with addison's disease from metastatic adrenal tumors.•Hyperpigmentation in addison's disease can occur anywhere due to various stimuli.•Hyperpigmentation could indicate susceptibility to acute adrenal insufficiency. A 73-year-old man diagnosed with brain metastasis from non-small cell lung carcinoma, not otherwise specified (NSCLC-NOS), was hospitalized for 1 week due to anorexia. A computed tomography revealed a 16.5 mm irregular-shaped nodule in the right lung apex, lymphadenopathy of the mediastinum, mesentery, and peri-aorta, and bilateral adrenal enlargement (46.3 and 48.7 mm in diameter). About 1 week before admission, his systolic blood pressure, which was approximately 110 mmHg suddenly dropped to approximately 80 mmHg. Simultaneously, his fasting blood glucose, also decreased to approximately 100 mg/dL from 200 mg/dL with a fever of 38 °C; he had poorly controlled type 2 diabetes. Since he had an electrolyte disorder with hyponatremia of 131 mEq/L and a slightly high serum potassium level of 5.7 mEq/L, he was referred to our department. A physical examination revealed hyperpigmentation on the dorsum of his tongue, which he reported had been present for 3 months prior to admission (Fig. 1A). What is the diagnosis? The symptoms are consistent with Addison's disease due to metastatic bilateral adrenal tumors. His-early-morning fasting blood cortisol (9.98 µg/dL) was inappropriately low against a markedly high adrenocorticotropic hormone (ACTH) level (145 pg/mL). His-dehydroepiandrosterone-sulfate level (11 µg/dL) was disproportionately low. Additionally, his plasma aldosterone concentration was <4.0 pg/mL, which is inappropriately low against the active renin concentration of 14.26 pg/mL, suggesting that his bilateral adrenal metastases impaired adrenal steroidogenesis in all layers of the adrenal cortex. The comparatively lower blood glucose, hypotension, and hyperkalemia are compatible with fever-induced acute adrenal insufficiency, which requires hydrocortisone and fludrocortisone replacement. One month following treatment initiation, the tongue discoloration disappeared (Fig. 1B). The coupling of pro-opiomelanocortin (POMC) peptides, α–melanocyte-stimulating hormone, and ACTH with the melanocortin-1 receptor stimulates melanogenesis [[1]Buscà R. Ballotti R. Cyclic AMP a key messenger in the regulation of skin pigmentation.Pigment Cell Res. 2000; 13: 60-69https://doi.org/10.1034/j.1600-0749.2000.130203.xCrossref PubMed Scopus (664) Google Scholar,[2]Wolf Horrell E.M. Boulanger M.C. D'Orazio J.A Melanocortin 1 receptor: structure, function, and regulation.Front Genet. 2016; 7: 95https://doi.org/10.3389/fgene.2016.00095Crossref PubMed Scopus (136) Google Scholar]. In Addison's disease, decreased negative feedback to the hypothalamus and pituitary due to reduced adrenal cortisol secretion increases POMC peptides, causing hyperpigmentation, which tends to occur in areas exposed to ultraviolet, chronic friction, and pressure [[3]Dunlop D. Eighty-six cases of Addison's disease.Br Med J. 1963; 2: 887-891https://doi.org/10.1136/bmj.2.5362.887Crossref PubMed Scopus (153) Google Scholar]. Furthermore, the pigmentation generally occurs on the side of the tongue touching the teeth. The patient had consistently brushed his tongue with a toothbrush and believed the friction might have caused hyperpigmentation; thus, when hyperpigmentation is observed in high-risk patients, they should be informed that they are susceptible to acute adrenal insufficiency when under intense stress, such as fever or trauma.

Cushing’s Syndrome Presenting as Nonconvulsive Status Epilepticus: A Case Report and Literature Review (P9-1.011)

<h3>Objective:</h3> NA <h3>Background:</h3> Cushing’s Syndrome (CS) may present with neuropsychiatric manifestations that can mimic autoimmune encephalitis, potentially delaying diagnosis and treatment. We present a case of CS presenting with personality changes and status epilepticus and performed a literature review describing neurological manifestations of CS. <h3>Design/Methods:</h3> A male in his 50s presented with episodic word-finding difficulties, inappropriate smiling, fatigue, cognitive decline and one episode of generalized tonic clonic seizure on levetiracetam from an outside hospital. Workup revealed thrombocytopenia, hypokalemia, hypertension, normal MRI brain and sella. Continuous electroencephalogram revealed nonconvulsive status epilepticus. Lumbar puncture demonstrated elevated protein with normal white blood cell count. He was started on high-dose IV methylprednisolone followed by IVIg for presumed autoimmune encephalitis. Autoimmune encephalitis antibodies including anti-NMDA receptor were negative. CT PET scan showed bilateral adrenal enlargement, which in the context of hypokalemia, hyperglycemia, hypertension, and thrombocytopenia was concerning for severe Cushing’s Syndrome. Methylprednisolone and anti-seizure medications were stopped. Inferior petrosal sinus sampling and cerebral angiogram were unremarkable. The diagnosis was supported by elevated random and urine cortisol levels and managed with etomidate and ketoconazole. He underwent bilateral open adrenalectomy following which he was started on oral glucocorticoids. Repeat EEG showed no ictal activity and the patient remained seizure-free. <h3>Results:</h3> Literature review utilizing PubMed identified 17 articles describing CS presenting with neuropsychiatric symptoms. The most common symptoms cited were cognitive decline (23%), psychosis (18%) and seizure (6%). Additional isolated cases cited posterior reversible encephalopathy syndrome, hypogeusia and hyposmia. Most patients had significant improvement or full recovery once CS was treated. <h3>Conclusions:</h3> Cushing’s Syndrome can present with neuropsychiatric symptoms and status epilepticus. It can be an emergency requiring prompt endocrine and surgical evaluation. Serum cortisol levels should be checked in patients presenting with acute personality changes and seizures. <b>Disclosure:</b> Dr. Cameron has nothing to disclose. Dr. Venegas has nothing to disclose. Dr. Fahad has nothing to disclose. Dr. Mathew has nothing to disclose.

Stress-induced severe transient hypercortisolism with reversible bilateral adrenal enlargement after cardiogenic shock.

Acute illness-related stress can result in severe hypercortisolism and bilateral adrenal enlargement in certain patients. We report a case of stress-induced hypercortisolism and bilateral adrenal enlargement in a patient admitted for acute respiratory distress and cardiogenic shock. Bilateral adrenal enlargement and hypercortisolism found during hospitalization for acute illness resolved 3 weeks later following the resolution of acute illness. Acute illness can be a precipitating factor for stress-induced hypercortisolism and bilateral adrenal enlargement. We hypothesize that increased adrenocorticotrophic hormone mediated by corticotrophin-releasing hormone from physical stress resulted in significant adrenal hyperplasia and hypercortisolism. This mechanism is downregulated once acute illness resolves. Adrenal enlargement with abnormal adrenal function after stress is uncommon in humans; however, if present, it can have self-resolution after the acute illness is resolved. Stress induces enlargement of the adrenals, and the degree of cortisol elevation could be very massive. This process is acute, and the absence of cushingoid features is expected. Treatment efforts should be focused on treating the underlying condition.

PS-BPR01-9: A CASE OF CYCLIC CUSHING'S SYNDROME DIAGNOSED WITH HYPERTENSION AND LEG EDEMA

Background: Cushing's syndrome (CS) is associated with hypertension in approximately 80% of cases, which contributes to the increased mortality risk. Few cases of intermittent hypertension due to cyclic CS(CCS) have been reported. Case presentation: A 66-year-old woman with no specific medical history was referred to our hospital for evaluation of hypertension and leg edema. The Cardiovascular examination showed no abnormality in her cardiac function. Laboratory findings revealed hypokalemia, hyperglycemia, and metabolic alkalosis. She was tentatively initiated on calcium channel blocker (CCB) and insulin therapy. The endocrine examination revealed elevated ACTH and cortisol without circadian variation, which responded to neither the 8 mg dexamethasone suppression test nor CRH stimulation, leading to the diagnosis of ACTH-dependent CS. CT, FDG/PET CT, and Octreotide scan showed no obvious lesion other than bilateral adrenal enlargement. Enhanced MRI showed a normal pituitary gland with neither adenoma nor pituitary stalk deviation/tilt. Since the etiology of CS was not confirmed, metyrapone administration was initiated as symptomatic treatment. Her serum ACTH and cortisol fell rapidly after the initiation of metyrapone, and her blood pressure (BP) and blood glucose levels improved accordingly, even without the use of CCB and insulin. Since both ACTH and cortisol became normalized after discharge, metyrapone was tapered off after 4 months. Furthermore, CT showed normalization of the adrenal enlargement. For the next two months, her ACTH and cortisol remained within the normal limits, and her BP, serum potassium and blood glucose were well controlled. However, she visited our hospital because of re-elevated BP thereafter and was found to have re-elevated ACTH and cortisol with bilateral adrenal enlargement as well as re-decreased serum potassium. Since her BP and serum potassium levels were not controlled well even after she resumed taking metyrapone, the dose of metyrapone was increased, spironolactone was added, and CCB was resumed. Another two months later, her ACTH and cortisol normalized without metyrapone again, and her BP was well-controlled accordingly. This cyclical course of ACTH/cortisol elevation strongly suggested CCS. Conclusion: CCS is a clinical syndrome that exhibits a periodic or irregular increasing pattern in cortisol, which is a rare type of CS. Confirmation of CCS diagnosis is only possible during periods of relapse. In the present case, elevated BP was an indicator of hormonal fluctuations that could be appropriately treated. We report here a case of CCS that was diagnosed because of a good correlation between BP and hormone levels.

ODP016 A case of adrenal incidentaloma in a patient with latent tuberculosis

Abstract An adrenal incidentaloma can be either a nonfunctioning adrenocortical adenoma or may cause disease, such as adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis. However, less commonly, it can be a manifestation of infection such as tuberculosis. The case is a 40-year-old male who emigrated from China two years ago, referred to the endocrine clinic after he was found to have a 3 cm right adrenal macroadenoma on MRCP after hospitalization for acute cholecystitis. The patient was also found to have positive Quantiferon, but CXR was clear. He was started on isoniazid therapy for latent tuberculosis and was later switched to rifampin postoperatively. One month after hospital discharge, he was evaluated by Endocrinology. Vital signs, including BP were normal with no symptoms of dizziness or orthostatic hypotension. He had no symptoms of adrenal insufficiency or excess. Electrolytes, renal, liver function tests, and CBC were within normal. Albumin was 4.3g/dL(RR 3.4-5). Fasting 8 AM labs: ACTH 9 pg/mL (RR 0-47), cortisol 10.10 ug/dL (RR 5.3-22.5), DHEA S 130 mcg/dL (RR 80-560), plasma metanephrines and normetanephrines and midnight salivary cortisol were normal, aldosterone 10 ng/dL, renin 1.49 ng/mL/h with ARR of 6.7. Adrenal CT w/wo contrast showed right adrenal mass with absolute washout 78% and relative washout 53% consistent with adenoma, as well as bilateral subcentimeter lipid rich adenomas and marked mesenteric lymphadenopathy. Mycobacterium tuberculosis complex spreads to the adrenal glands hematogenously. Clinical manifestations of adrenal insufficiency in tuberculosis may take years to take effect, after 90% of gland destruction occurs. The majority of patients with active or recently acquired disease (&amp;lt;2 years) have bilateral adrenal enlargement, while calcification and atrophy is found with remote infection or inactive disease. The adrenals can be enlarged in patients with pulmonary tuberculosis even without active involvement of the glands solely due to stress and inflammation. The hypothalamic-pituitary-adrenal (HPA) axis can be activated or even under-activated in active pulmonary tuberculosis. Cortisol levels return to baseline after anti-tuberculous treatment. Rarely tuberculosis can cause adrenal incidentaloma. Adrenal biopsy is not necessary for primary adrenal insufficiency with bilateral adrenal enlargement in a patient with proven extra-adrenal tuberculosis. However, about 12% of patients with adrenal tuberculosis have no evidence of active extra-adrenal tuberculosis. Adrenal biopsy was refused but is generally necessary in these patients to prove adrenal involvement by tuberculosis. Our case highlights the fact that tuberculosis should be considered in the differential of adrenal masses. Reference: Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175(2): G1-G34. doi: 10.1530/EJE-16-0467. PMID: 27390021. Presentation: No date and time listed

Current perspective of adrenal histoplasmosis in India: A prospective study in a tertiary care hospital, Eastern India

PurposeAdrenal histoplasmosis (AH) is an uncommon form of disseminated histoplasmosis caused by the dimorphic fungus Histoplasma capsulatum. Though, India is considered to be a non-endemic area for histoplasmosis, a high rise of AH cases is reported currently from various parts of India. Our study aimed to evaluate the current perspective of adrenal histoplasmosis in India by reviewing its clinical course, differential diagnosis, treatment, and mortality of our eleven confirmed cases of AH along with the review of authentic reported AH cases from India. Material &methodClinical materials were taken from radiologically suspected all 15 cases either with unilateral or bilateral adrenal enlargement, referred between 2018 and 2020 for microbiological investigations. Fungal stain and fungal culture along with other tests for possible differential diagnosis with AH were conducted. ResultOut of fifteen incidentaloma detected by radio-imaging, eleven cases of AH had been diagnosed in our hospital with yield of Rhodotorula spp. in one mimicking case. Nine of them were male (82%) and all were HIV nonreactive, which corroborates with the literature review. All of them had nonspecific clinical presentation of chronic abdominal pain, fever, weight loss, and anorexia. Four developed primary adrenal insufficiency, which are similar to the literature review (41%). On treatment with itraconazole and/or amphotericin B, all patients survived except one lost in follow-up. ConclusionMale preponderance and non-compromised immune status are two special characteristics of most AH though reasons are ill understood. So, mycological investigations are to be done for every such case.

Refractory postoperative Staphylococcus hominis bacteremia in a patient with an ACTH-producing pancreatic neuroendocrine neoplasm: a case report

BackgroundStaphylococcus hominis (S. hominis) is an opportunistic pathogen that is often highly resistant to antibiotics and is difficult to treat. In patients diagnosed with an adrenocorticotropic hormone (ACTH)-producing tumor that compromises the immune system due to hypercortisolemia, cancer treatment and infection control should be considered simultaneously. This report presents a case of refractory postoperative S. hominis bacteremia requiring the prolonged administration of several antibiotics in a patient with an ACTH-producing pancreatic neuroendocrine neoplasm (pNEN).Case presentationA 35-year-old man visited a neighboring hospital for a thorough examination after experiencing weight gain and lower limb weakness for several months. Enhanced computed tomography revealed a pancreatic tail tumor and bilateral adrenal enlargement. Elevated plasma ACTH and serum cortisol were noted. Biopsy under endoscopic ultrasonography revealed the tumor as an ACTH-producing pNEN. The patient was transferred to our hospital for further treatment. Pneumocystis pneumonia was noted and treated with sulfamethoxazole and adjunctive glucocorticoids. Hypercortisolism was controlled with metyrapone and trilostane. Somatostatin receptor scintigraphy and ethoxybenzyl magnetic resonance imaging detected other lesions in the pancreatic head. A total pancreatectomy was performed given that the lesions were found in both the pancreatic head and tail. Plasma ACTH and serum cortisol levels decreased immediately after the resection. Pathological examination revealed that the pancreatic tail tumor was NEN G2 and T3N1aM0 Stage IIB and the pancreatic head lesions were SSTR-positive hyperplasia of the islet of Langerhans cells. On postoperative day 11, catheter-associated bacteremia occurred. Initially, meropenem hydrate and vancomycin hydrochloride were administered empirically. S. hominis was identified and appeared sensitive to these antibiotics according to susceptibility testing. However, S. hominis was repeatedly positive in blood cultures for more than one month, despite treatment with several antibiotics. Eventually, with the combined use of three antibiotics (meropenem hydrate, vancomycin hydrochloride, and clindamycin phosphate) for more than 3 weeks, the S. hominis-associated bacteremia improved. He was discharged 79 days after surgery.ConclusionsOur patient with an ACTH-producing pNEN was immunocompromised and needed meticulous attention for infectious complications even after successful tumor removal. Specifically, S. hominis bacteremia in such patients demands intensive treatments, such as with combinational antibiotics.

D2 MTE4 Case 11: Adult CAH, how do I optimize treatment?

28 years, female, born of non-consanguineous marriage, assigned female gender had genital ambiguity and seizures on day 10 of life. She presented to us at 9 years with genital ambiguity and hyperpigmentation. Tanner stage A3P4B2, external genitalia: phallic like structure 4.5 cm, posterior labial fusion, single opening, no palpable gonads. Unstimulated 17 OHP 192 ng/ml, testosterone and DHEAS elevated, low basal cortisol, other biochemical parameters normal, CT scan- bilateral adrenal enlargement, so a diagnosis of simple virilizing type of congenital adrenal hyperplasia was made and was started on T. Hydrocortisone and T. Fludrocortisone, after which pigmentation decreased. Gender role and identity as female. Underwent clitoral reduction and vaginal reconstruction. She had spontaneous puberty with pubarche at 7 years, thelarche at 10.5 years and menarche at 12.5 years. Her 17 OHP remained elevated and had early epiphyseal fusion leading to short stature. She had hirsutism at 16 years so she was started on T. Dexamethasone but was again changed to Hydrocortisone as she developed cushingoid features after 2 years. She is married for past 3 years and is eumenorrhic but now presents with primary infertility. Recent investigations revealed 17 OHP 50 ng/ml (Target: 4-12 ng/ml) and T 6.3 nmol/l. Currently on T. Hydrocortisone divided in 5 doses and T. Fludrocortisone 150 mcg. How to further manage fertility and long-term complications.?

Current Approach for Diagnosis and Treatment of Adrenal Tuberculosis-Our Experience and Review of Literature.

Addison's disease was first described by Thomas Addison in 1855. He demonstrated the destruction of bilateral adrenal gland by tuberculosis (TB) in six patients. Since then, the incidence of TB has declined in the Western world, but in developing countries, it is still the most common cause of adrenal insufficiency. Because of the introduction of antituberculous chemotherapy, the incidence of adrenal TB has been declined in the past decades. The most common symptoms are nonspecific; therefore, diagnosis is often delayed, and patients may first present with a life-threatening adrenal crisis. The most commonly identified organism for adrenal failure in adrenal TB is Mycobacterium tuberculosis infection. Adrenal TB involves bilateral adrenal glands more frequently than unilateral glands. Computed tomography (CT) scan and magnetic resonance imaging (MRI) are useful investigations to differentiate between tuberculous Addison's disease and the other causes of adrenal insufficiency. In CT scans or MRI, features of adrenal TB are bilateral adrenal enlargement and peripheral rim enhancement with or without calcifications. Antituberculous drugs, biochemical monitoring of adrenal function, and steroid therapy are essential for the management of adrenal TB and adrenal insufficiency. Here, we describe a case of adrenal TB with abscess formation followed by a review of the current literature of adrenal TB for better diagnosis and management of this condition.

Adrenal Histoplasmosis: An Uncommon Cause of Adrenal Crisis in an Immunocompetent Man

Abstract Background Histoplasmosis is a rare cause of primary adrenal insufficiency. Adrenal involvement is commonly encountered with disseminated disease in immunocompromised individuals though some have been reported in immunocompetent patients. Primary adrenal histoplasmosis where adrenals are the only organs with demonstrable disease is uncommon. Case Presentation A 33-year-old immunocompetent man presented with an adrenal crisis. Further evaluation of the underling etiology of primary adrenal insufficiency revealed bilateral adrenal enlargement that was confirmed to be histoplasmosis on adrenal biopsy. Conclusion The case highlights the importance of considering histoplasmosis as an underlying cause of primary adrenal insufficiency in patients from high endemic areas.

Out of Site, Out of Mind- A Rare Case of Adrenal Histoplasmosis from a Non Endemic Area

There has been a rising trend in the number of histoplasmosis cases reported in the recent years. However, in southern India, the number of cases is relatively less. This report is about a 46-year-old Indian male from Tamil Nadu who presented with decreased appetite, vomiting, and giddiness. In view of persistent hyponatremia and hypotension, a work-up for adrenal insufficiency was done, and serum cortisol was found to be low. Contrast Enhanced Computed Tomography (CECT) showed bilateral adrenal enlargement with peripheral rim enhancement and hypodense areas within. There was a diagnostic dilemma between tubercular and fungal aetiology. Fine Needle Aspiration Cytology (FNAC) of the adrenal gland showed features suggestive of histoplasmosis. The patient was treated with itraconazole along with steroid replacement therapy, leading to marked improvement in his overall condition. In immunocompetent individuals, disseminated histoplasmosis with adrenal gland involvement is a rare occurrence. As an unusual presentation of histoplasmosis, this case adds to the body of literature available from Tamil Nadu, which is a non endemic region for histoplasmosis, with only 15 cases being reported as of 2018.

Idiopathic bilateral adrenal hemorrhage presenting as acute abdomen in a 61-year-old male: A case report with review of literature

Introduction: Adrenal haemorrhage (AH) is a rare, life-threatening condition that requires prompt diagnosis and management. Case Report: A 61-year-old male presented with sudden-onset central abdominal pain, distended abdomen, and nausea. There was no history of trauma or any other predisposing factor for AH. The blood pressure was raised, while other vitals were normal. The patient was managed with intravenous Diclofenac, Tablet Amlodipine, and Ryle's tube insertion. Initial abdominal ultrasound revealed bilateral adrenal enlargement. However, non-contrast computed tomography of abdomen revealed changes suggestive of bilateral AH. These findings were confirmed on magnetic resonance imaging of abdomen. Though the serum cortisol and adrenocorticotropic hormone levels were normal, and the patients were not symptomatic of adrenal insufficiency, intravenous Methylprednisolone was initiated to prevent deterioration of adrenal function. All the known causes of AH were excluded, and a diagnosis of idiopathic bilateral AH was reached. On Day 7, an abdominal ultrasound revealed a non-progressive size of the bilateral adrenal lesions and the patient was discharged with well-controlled blood pressure. Conclusion: Idiopathic AH, in patients without any predisposing factors, should be included in the differential diagnosis of a patient with vague abdominal pain.

ARMC5-associated Bilateral Macronodular Adrenocortical Hyperplasia: A Novel Germline Variant Associated with Concomitant Papillary Thyroid Carcinoma and Meningioma

Abstract Introduction/Objective Germline mutations in the tumor suppressor gene Armadillo-containing repeat protein 5 gene (ARMC5) have been very recently recognized as a cause for a familial form of bilateral macronodular adrenocortical hyperplasia (BMAH), itself a rare cause of Cushing syndrome. In patients with ARMC5 mutations, scattered case reports have also shown an association with meningiomas and cancers of the pancreas, breast, colon, and thyroid. Methods/Case Report We present the case of BMAH, arising in a 61-year-old female with a history of metastatic papillary thyroid carcinoma and meningioma. The patient presented with bilateral but asymmetric adrenal enlargement (right greater than left) and Cushing syndrome. Given history of thyroid cancer and meningioma, genetics referral was ordered. Counseling revealed a pedigree without a strongly evident familial pattern of hereditary endocrine neoplasia characteristic of any of the more common inherited dispositions to endocrine neoplasia. Additionally, a targeted capture-based NGS germline genetic sequencing study for variants in 12 genes associated with associated with hereditary thyroid cancer was performed and negative. However, based on recent scholarship regarding ARMC5, follow-up germline NGS and Sanger sequencing studies encompassing the entire coding sequences of ARMC5 were ordered. These identified a germline, heterozygous, novel (not in ClinVar) but likely pathogenic variant in (c.802C&amp;gt;T, p.Arg268*), providing a likely explanation for the patient’s BMAH. In attempt to control the patient’s Cushing symptoms, right-sided adrenalectomy was performed, revealing a 220g adrenal gland with marked multinodular hyperplasia with solid, nested, and tubular architecture. Results (if a Case Study enter NA) NA Conclusion While case reports exist describing an association between other ARMC5 mutations and BMAH with concomitant meningiomas and/or malignancies, greater study is needed in order to better characterize the phenotypic spectrum of this disease. Our experience with this case not only reports a novel, apparently pathogenic mutation, but it documents its association with BMAH and, additionally, papillary thyroid carcinoma and meningioma.

Diagnostic Impact of Adrenal Vein Sampling in Adrenal Cushing's Syndrome

Adrenal Cushing's syndrome is characterized by ACTH-independent hypercortisolism. Adrenal vein sampling (AVS) is not routinely employed prior to management decisions, and few studies have investigated the value of AVS in this population. We assessed whether AVS provides a diagnostic benefit for treatment planning. Six patients with imaging and biochemical evidence of adrenal Cushing's syndrome undergoing AVS at our institution from 2015 to 2020 were analyzed retrospectively, including demographic and clinical characteristics. AVS lateralization index was determined by comparing the (cortisol/ipsilateral reference hormone) ratios of both adrenal veins. lateralization index of 2 or greater was considered diagnostic of unilateral disease. Post-management clinical improvement was defined as serum cortisol normalization, symptomatic improvement, or both. Cross-sectional imaging noted bilateral adrenal enlargement or nodules in three patients, and unilateral nodules in three patients. AVS results were discordant with imaging in three patients. Treatment included medical management in two patients, percutaneous radiofrequency ablation in one patient, and laparoscopic adrenalectomy in two patients. One patient was lost to follow up. AVS results aided management planning in five patients, definitively changing treatment from surgery to medical management in one patient. All five patients demonstrated clinical improvement. AVS offered useful information for determining appropriate management of adrenal Cushing's syndrome, especially distinguishing unilateral from bilateral disease. Even in bilateral disease, AVS may show a dominant gland, potentially allowing a staged unilateral adrenalectomy, before assessing the need for completion adrenalectomy or medical management. Larger studies are needed to better establish whether AVS offers significant benefit for this population.

A Pedigree and Clinical Analysis of 11β-Hydroxylase Deficiency with Homozygous Mutation of CYP11B1 Gene

Abstract Background To analyze the clinical features and the mutation of CYP11B1 gene in a family with 11β-hydroxylase deficiency. Methods The clinical and laboratory tests data of the patient were collected. The complete sequences of CYP11B1 gene in the proband and potential mutations in other seven family members were analyzed by polymerase chain reaction and direct sequencing technique. Results A 25-year female patient showed complicated clinical symptoms, comprising hypertension, heart failure, renal failure, and pulmonary infections. Laboratory results showed higher levels of 17α-hydoxy progesterone, dehydroepiandrosterone (DHS) and adrenocorticotropic hormone (ACTH), and lower levels of cortisol and potassium in serum. Additionally, a positive reaction in mid-dose dexamethasone suppression test and a significant enlargement of bilateral adrenal glands from computed tomography scan were exhibited in this patient. Furthermore, a homozygous c.1157C&amp;gt;T (p.Ala386Val) in exon 7 of the CYP11B1 gene was identified in this patient. Four family members (father, mother, maternal grandmother, and aunt of proband) were heterozygous of the same mutation. Conclusion 11β-hydroxylase deficiency in this patient is caused by homozygous c.1157C&amp;gt;T (p.Ala386Val) in the CYP11B1 gene.