Adrenal paracoccidioidomycosis / [Paracoccidiodomicosis suprarrenal].

Primary adrenal insufficiency is a defect in glucocorticoid, mineralocorticoid and sexual androgens production. Patients with this disorder have low cortisol levels and aldosterone deficiency with concomitant hyponatremia and hyperkalemia. The most common etiology of this disease is the production of antibodies against the enzyme 21 hydroxylase. Another common cause, particularly in low income countries, are infectious diseases. Several micro-organisms have been reported as a causal agent in adrenal insufficiency including Mycobacterium tuberculosis, Mycobacterium avium complex, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, cytomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. and Paracoccidioides brasiliensis. In this article, we present the computerized tomography and the adrenal biopsy of a patient with adrenal insufficiency. The final diagnosis was paracoccidioidomycosis.

La insuficiencia suprarrenal primaria es un defecto en la producción de glucocorticoides, mineralocorticoides y andrógenos sexuales. Los pacientes afectados por esta condición se caracterizan por concentraciones bajas de cortisol y deficiencia de aldosterona con hiponatremia e hiperpotasemia concomitantes. La etiología más común es el desarrollo de anticuerpos contra la enzima 21 hidroxilasa. Otra causa importante de la insuficiencia suprarrenal primaria son las enfermedades infecciosas, en especial en los países de bajos ingresos. Entre las causas infecciosas que se han descrito se encuentran: Mycobacterium tuberculosis, el complejo de Mycobacterium avium, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, citomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. y Paracoccidioides brasiliensis. En este artículo se presenta la imagen de la tomografía de un paciente que presentó falla suprarrenal, con masas en las glándulas suprarrenales, cuya biopsia permitió establecer el diagnóstico final de paracoccidioidomicosis.

Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with Bilateral Adrenal Hemorrhage in Two Caucasian Patients.

BACKGROUND Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome is a variant of idiopathic multicentric Castleman disease. Adrenal hemorrhage has rarely been reported in TAFRO syndrome, and previous cases have mainly been Asian patients. This report is of two Caucasian patients with TAFRO syndrome presenting with acute adrenal insufficiency due to bilateral adrenal hemorrhage. CASE REPORT Case 1 was a 19-year-old Caucasian man with no significant past medical history who was admitted with acute abdominal pain, vomiting, anorexia, and moderate weight loss. Case 2 was a 31-year-old Caucasian woman with no past medical history who was admitted to hospital with fever, dyspnea, thoracic and abdominal pain, polyarthralgia, and hypotension. Both patients had splenomegaly, mild lymphadenopathy, thrombocytopenia, acute kidney injury, and myelofibrosis. In both cases, lymph node biopsy histology showed mixed-type idiopathic multicentric Castleman disease. In both patients, a diagnosis of TAFRO was made, and they developed bilateral adrenal hemorrhage with adrenal insufficiency. Case 1 was treated with high-dose steroids, followed by tocilizumab infusion. Due to persistent thrombocytopenia, second-line treatment commenced with rituximab, but the patient relapsed two months later. Tocilizumab treatment was recommenced, which was followed by an immuno-allergic adverse event. He then had a good response to sirolimus. Case 2 died nine months after diagnosis due to acute respiratory distress. CONCLUSIONS Two cases of TAFRO syndrome presented with acute adrenal insufficiency due to bilateral adrenal hemorrhage. The symptoms were only partially controlled with tocilizumab, rituximab, and tacrolimus. Adrenal hemorrhage may be a specific manifestation of TAFRO syndrome.

Disseminated cytomegalovirus infection with bilateral adrenal pseudotumors masquerading as recurrent hematologic malignancy.

BACKGROUND: Mass-like lesions are an uncommon presentation of cytomegalovirus (CMV) disease. CASE: We report on a case of disseminated CMV disease with bilateral adrenal pseudotumors in a patient with a history of acute leukemia in remission. CONCLUSION: In the settings of advanced cancer therapy and organ transplantation, a high index of suspicion for CMV should be maintained for mass-like disease.

What is the role of medical therapy in adrenal-dependent Cushing's syndrome?

Medical therapy to control hypercortisolism in adrenal Cushing's syndrome is currently not the first-line therapy. However, in many clinical scenarios like pre-surgical treatment, in patients who are not suitable candidates for surgery or in patients with bilateral hyperplasia, medical therapy can be important representing the only viable treatment option. Adrenal steroidogenesis inhibitors and glucocorticoid receptor blockers have been used for many years: metyrapone, ketoconazole and mifepristone are in current use and effective. Mitotane can be used as well but is considered second-line therapy because of its high toxicity. Etomidate has a special position as emergency medication in severe hypercortisolism. New drugs are tested in prospective trials (levoketoconazole, osilidrostat and relacorilant) and might become effective alternatives to common drugs. Oher drugs - adrenal steroidogenesis inhibitors as well as glucocorticoid receptor antagonists - are currently tested in vitro.

Pneumocystis jirovecii infection of bilateral adrenal glands in an immunocompetent adult: a case report.

Pneumocystis jirovecii (PJ) infection is one of the most common opportunistic infections occurring in patients with HIV/AIDS and other immunocompromised states. It is not known to cause clinically significant illness in immunocompetent hosts. We report a 48-year-old HIV-negative, diabetic male who presented with fever and adrenal insufficiency. Abdominal sonography and PET-CT revealed bilateral enlarged adrenal glands with peripheral enhancement and central necrosis. An endoscopic ultrasound-guided fine-needle aspiration cytology of the left adrenal gland demonstrated well-defined, round cysts of PJ. There was no evidence of pulmonary involvement. The response to first-line treatment was poor and the patient responded to second-line treatment for Pneumocystis infection.

The first case of thrombocytopenia, anasarca, fever, renal impairment or reticulin fibrosis, and organomegaly (TAFRO) syndrome with unilateral adrenal necrosis: a case report.

BACKGROUND: TAFRO syndrome, which was first reported in 2010 in Japan, is a relatively rare disease characterized by thrombocytopenia, anasarca, fever, renal impairment, reticulin fibrosis, and organomegaly. Although this disease is considered similar to multicentric Castleman disease, some of the clinical features, such as thrombocytopenia, are different from typical cases of multicentric Castleman disease. In addition, the etiology of TAFRO syndrome remains unknown and controversial. There have only been a few cases of TAFRO syndrome complicated with adrenal gland lesions, and all of them have had hemorrhagic involvement. CASE PRESENTATION: This report describes the case of a 46-year-old Asian man who presented with fever, epigastric pain, and back pain for 1 month. A computed tomographic scan revealed ascites, mild lymphadenopathy, and left adrenal necrosis without hemorrhage. A blood test showed thrombocytopenia, anemia, and elevated C-reactive protein, alkaline phosphatase, and creatinine levels. Based on the edema, severe thrombocytopenia, fever, reticulin myelofibrosis shown by bone marrow biopsy, mild lymphadenopathy, and progressive renal insufficiency, we diagnosed this patient as having TAFRO syndrome. He was successfully treated by immediate administration of glucocorticoids and tocilizumab. CONCLUSIONS: There have been no previous reports of a case of TAFRO syndrome complicated with adrenal necrosis. Because the biopsy of the left adrenal gland revealed necrosis without any evidence of hemorrhage, we concluded that the unilateral adrenal necrosis in this case was caused by either ischemia from infarction or organomegaly itself under severe hypercytokinemia. This unusual clinical course is useful for further analysis of the etiology of TAFRO syndrome.

[Bilateral adrenal tuberculosis: about a case]. / Tuberculose surrénalienne bilatérale: à propos d'un cas.

Isolated adrenal tuberculosis accounts for less than 2% of adrenal incidentalomas. This is the most frequent infectious cause of adrenal insufficiencies. We report the case of a 53-year old patient with no previous medical history presenting with adrenal insufficiency with slow progression over six months. Physical examination didn't show any mass or hepatosplenomegaly. Blood pressure was 120/60 mmHg. Laboratory tests didn't show inflammatory syndrome. LDH level was normal. CT scan showed bilateral hypertrophy of the adrenal glands characterized by calcifications. Intradermo tuberculin reaction was positive at 25mm. The analyses to detect Koch's bacillus in the sputum and in the urine were negative. Quantiferon® test was positive. Trial antibacillar treatment was started with clinical improvement and 5kg weight gain in 12 months. Hormonal assays were low.

Bilateral Adrenal Hemorrhage in a Patient with Antiphospholipid Syndrome during Chronic Graft-versus-host Disease.

We present the case of a 56-year-old man with an upper respiratory infection followed by fatigue, hypotension, and hyponatremia. Bilateral adrenal hemorrhage was confirmed, based on T2-weighted magnetic resonance imaging. The patient had previously undergone allogeneic hematopoietic stem cell transplantation and had been diagnosed with antiphospholipid syndrome (APS) during the development of chronic graft-versus-host disease. A prompt diagnosis and steroid replacement, in addition to anticoagulant therapy, resulted in a favorable outcome. Once the diagnosis of APS has been confirmed, which might be the sign of bilateral adrenal hemorrhage, the initial manifestations of adrenal insufficiency should never be overlooked.

Alveolar echinococcosis of the right adrenal gland: a case report and review of the literature.

BACKGROUND: Extrahepatic manifestations of Echinococcus multilocularis are very rare, especially in the adrenal glands. To the best of our knowledge, only seven cases of adrenal alveolar echinococcosis have been reported, all from the Far East. All of these occurred exclusively in the right adrenal gland. CASE PRESENTATION: We report a rare case of an extrahepatic alveolar echinococcosis in an asymptomatic 78-year-old white man with an incidentaloma of his right adrenal gland. After surgical resection and medical treatment with albendazole no recurrence of the disease appeared at 1-year follow-up. CONCLUSIONS: As the occurrence of Echinococcus multilocularis in Europe increases, alveolar echinococcosis should be included in the differential diagnosis of cystic adrenal incidentalomas.

Post-transplant lymphoproliferative disorder of the adrenal gland after allogeneic hematopoietic stem cell transplantation: report of two cases and literature review.

Post-transplant lymphoproliferative disorder (PTLD) is one of the life-threatening complications after hematopoietic stem cell transplantation (HSCT) and solid organ transplantation (SOT), and it is associated almost exclusively with Epstein-Barr virus (EBV). We herein report 2 cases of EBV-associated PTLD after allogeneic HSCT localized in the adrenal gland. Both patients developed adrenal tumor within 3 months after HSCT and were successfully treated with rituximab or tapering immunosuppressive agents. Both remained alive without recurrence. A literature review revealed 12 reported cases of PTLD involving the adrenal gland, but the adrenal gland was involved as one of the lesions of advanced-stage PTLD after SOT. To the best of our knowledge, this is the first report to show cases of isolated EBV-associated adrenal PTLD after HSCT. PTLD should be recognized as one of the causes of isolated adrenal tumor after HSCT.

Increased Hair Cortisol Concentrations and BMI in Patients With Pituitary-Adrenal Disease on Hydrocortisone Replacement.

CONTEXT: Intrinsic imperfections and lack of reliable biomarkers preclude optimal individual dosing of hydrocortisone replacement in adrenal insufficiency (AI). However, the clinical relevance of optimal dosing is exemplified by frequently occurring side effects of overreplacement and the dangers of underreplacement. Cortisol in scalp hair has been identified as a retrospective biomarker for long-term cortisol exposure. We compared hair cortisol concentrations (CORT(hair)) of patients with primary or secondary AI on replacement therapy with those of patient controls with a pituitary disease without AI (PCs) and of healthy controls (HCs). METHODS: In this cross-sectional study, hair samples and anthropometric data were collected in 132 AI patients (52 males), 42 PCs (11 males), and 195 HCs (90 males). The proximal 3 cm of hair were used. CORT(hair) were measured using an ELISA. RESULTS: CORT(hair) were higher in AI patients than in HCs and PCs (P < .001), and hydrocortisone dose correlated with CORT(hair) (P = .04). Male AI patients demonstrated higher CORT(hair) than female patients (P < .001). AI patients had higher body mass index (BMI) than HCs (P < .001), and BMI correlated with CORT(hair) in the whole sample (P < .001). CONCLUSION: Physiological hydrocortisone replacement is associated with increased CORT(hair). The association between CORT(hair) and BMI could suggest a mild overtreatment that may lead to adverse anthropomorphic side effects, especially in males. CORT(hair) measurements may be a promising additional tool to monitor cumulative hydrocortisone replacement in AI.

[Unilateral adrenal tuberculosis: a case report].

Computed tomography (CT) performed for a 75-year-old man as a follow-up examination for deep vein thrombosis in October 2010 revealed a left adrenal mass (diameter, 8 mm). In December 2012, the adrenal mass increased to 28 mm in diameter, and he was referred to our department. Several blood examinations revealed that the adrenal mass was non-functioning, and only peripheral lesions were observed to be enhanced by using CT in the arterial phase. Malignancy was suspected due to the irregular shape and growth of the mass, and left adrenalectomy was performed in February 2013. The histopathological diagnosis was adrenal mycobacteriosis, and clinical diagnosis was adrenal tuberculosis. No other tuberculosis infection-related lesion was detected, and the patient was treated with multidrug antituberculous chemotherapy.

Extrahepatic primary adrenal alveolar echinococcosis: a review.

BACKGROUND: Primary echinococcosis, and especially primary alveolar echinococcosis (AE) is rare among adrenal lesions. METHODS: We report a case of primary AE in a 28-year-old male with low backache and occasional upper limb pain lasting for six months, and review the pertinent but sparse literature on this disorder. RESULTS: Evaluation of the patient revealed an abdominal mass as right adrenal AE. The adrenal gland is a rare extrahepatic site of occurrence of echinococcosis, and particularly of AE. Patients with adrenal echinococcosis usually have nonspecific clinical symptoms. Imaging examinations revealed clearly the features of AE and its surrounding pathology. Alveolar echinococcosis was seen as an inhomogeneous pseudotumor with irregular boundaries, an irregular central pseudocystic appearance, and infiltration into surrounding structures. On contrast-enhanced computed tomography, the periphery of the mass in AE may be enhanced and multicentric vesicles may be seen. Magnetic resonance imaging may show the multivesicular morphology of lesions. The definitive diagnosis of AE should be confirmed by pathologic or serologic examination. Radical surgical resection of the removable parasitic lesion, followed by anti-infective therapy with benzimidazoles, is the primary method for treating AE. CONCLUSION: Although primary adrenal AE is rare, it should be included in the differential diagnosis of adrenal masses, especially in regions where AE is endemic.

A report from the endocrine society's 94th annual meeting & expo (June 23-26 - Houston, Texas, USA).

At temperatures above 100 degrees Fahrenheit, but chilly air conditioning inside the George R. Brown convention center, Houston was the endocrine capital of America during June 2012, with the ENDO EXPO 2012 meeting calling attendees from all the states and abroad. Endocrinology is a highly varied specialty, ranging from hormonal disturbances of the pituitary (and in fact acromegaly and Cushing's syndrome were among the stars of this year's meeting therapy-wise) to diabetes, female hormonal disorders and endocrine-mediated malignancies, to mention only a few. New and investigational treatments for these conditions are summarized in the following report, based on the oral and poster presentations during the meeting.