Fine Needle Aspiration of Adrenal Myelolipoma: A Case Report

A case of an adrenal myelolipoma in a 50-year-old woman with endometrial carcinoma is described. The diagnosis was established by computed tomography-guided fine needle aspiration, which is particularly useful for the pre-operative evaluation of adrenal tumours in asymptomatic, high-risk or cancer patients. The criteria used to distinguish extra-adrenal myelolipomas from mass-forming extramedullary haematopoiesis are discussed.     

Adrenal Myelolipomas in Patients with Congenital Adrenal Hyperplasia: Review of the Literature and A Case Report

ObjectiveTo review the association between congenital adrenal hyperplasia (CAH) and adrenal myelolipomas and report a case of bilateral, giant adrenal myelolipomas in a patient with untreated CAH due to 21-hydroxylase deficiency.MethodsWe describe the patient’s clinical presentation, imaging findings, and laboratory test results and review the relevant English-language literature concerning patients with both CAH and myelolipomas.ResultsA 45-year-old man with untreated CAH due to 21-hydroxylase deficiency presented with increasing abdominal girth and abdominal pain. Computed tomography of the abdomen demonstrated very low-density adrenal masses (22 × 11 cm on the left side and 6 × 5.5-cm on the right side) consistent with adrenal myelolipomas. The left adrenal myelolipoma was resected (24.4 × 19.0 × 9.5 cm; 2557 g). The mass was composed of mature adipose tissue with areas of hematopoietic cells of myeloid, erythroid, and megakaryocytic cell lines. Islands of adrenal cortical cells were scattered between the adipose and hematopoietic tissue. Including the present case, we identified 31 patients with both CAH and myelolipomas who have been described in the English-language literature. The details of these cases were reviewed.ConclusionsPersons with CAH may be at increased risk of developing adrenal myelolipomas, particularly if their CAH is poorly controlled. How and whether chronic exposure of the adrenal glands to high corticotropin levels increases the risk of developing myelolipomas remains a matter of speculation. (Endocr Pract. 2011;17:441-447)     

Testicular Adrenal Rest Tumors in Congential Adrenal Hyperlasia: A Case Report and Literature Overview

ObjectiveIn this paper, the etiology, differential diagnosis, and therapy of testicular adrenal rest tumors (TARTs) is presented based on a patient presentation and an overview of the most recent literature concerning this subject.MethodsRetrospective analysis of the clinical and biochemical data of an 18-year-old male patient diagnosed at the age of 2 weeks with the classic salt-wasting form of congenital adrenal hyperplasia that has been monitored in the pediatric and adolescent outpatient department since the age of 4 years.ResultsThe results of adrenal hormone tests (17-hydroxyprogesterone, 17-ketosteroids, and pregnanes) had been unsatisfactory, particularly over the previous 5 years. Scrotal ultrasound detected TARTs bilaterally. After increasing the dose of hydrocortisone and introducing dexamethasone, considerable regression of the tumors was noted.ConclusionLack of complete regression of the lesions is caused by fibrosis and is probably due to decreased sensitivity of adrenocorticotropic hormone and angiotensin II receptors in this tissue. (Endocr Pract. 2014;20:e219-e224)     

X-Linked Adrenal Hypoplasia Congenita: A Case Report and Ethical Dilemma

ObjectiveOur objective is to present the first case report of X-linked adrenal hypoplasia congenita in a child conceived by a donated egg and which also presented atypically, with initial mineralocorticoid deficiency.MethodsCase report with literature review.ResultsA late preterm fraternal twin male, conceived by in vitro fertilization of donated eggs, presented shortly after birth with feeding intolerance, hyponatremia, and hyperkalemia. Testing revealed a low aldosterone level, high plasma renin activity, normal cortisol level, and normal 17-hydroxyprogesterone level. He was diagnosed with 18-hydroxylase deficiency based on low 18-hydroxycorticosterone levels and was treated with mineralocorticoid successfully for 17 months. At age 18 months, he presented with dehydration secondary to herpetic gingivostomatitis and was found to be hypoglycemic, hyponatremic, hyperkalemic, and acidotic, with a low serum cortisol level. An adrenocorticotropic hormone (ACTH) stimulation test revealed low levels of all adrenal cortex products, with an elevated ACTH level. He was started on glucocorticoids. Genetic testing confirmed X-linked adrenal hypoplasia congenita (AHC). His asymptomatic fraternal twin underwent genetic testing and the results were negative. The fertility center records indicated that the mother had donated eggs to other families, but none of the children were known to have this disorder. The egg donor was informed but did not pursue genetic testing.ConclusionWe report a case of X-linked AHC presenting in the context of extraordinary ethical considerations. Our case raises a question unique to the era of assisted reproduction: should routine genetic screening of gamete donors be done for rare but potentially life-threatening conditions? (Endocr Pract. 2014;20:e126-e129)     

Solitary Plasmacytoma of the Parotid Gland with Crystalline Inclusions: A Case Report

Background  

Solitary extramedullary plasmacytoma of the parotid gland is a rare condition. Intracytoplasmic Crystalline inclusions in the tumor are even rarer and have been reported only once in the parotid gland.     

m-Synephrine: Normal Occurrence in Adrenal Gland

Abstract: Gas chromatography-mass spectrometry-selected ion monitoring has been used to identify m-synephrine (phenylephrine) in bovine adrenal gland. After ion exchange chromatography, m-synephrine was converted to its N-trifluoroacetyl-O-trifluoroacetoxy derivative and identified by retention time and relative intensities of the two characteristic ions at m/e 140 and m/e 455. Deuterated m-synephrine was synthesized and used as an internal standard for quantitative analysis. Bovine adrenal gland contains 83 ng g-¹ (range, 33-131) of endogenous m-synephrine.     

Incidentally Identified Adrenal Lipoma: Case Report and Review of Related Literature

ObjectiveTo report a case of an incidentally identified adrenal lipoma in a 68-year-old man with flank pain and hypertension.MethodsThe clinical, computed tomographic, and magnetic resonance imaging findings in this patient are described, and the pathologic features of the tumor are characterized. Moreover, the related literature is reviewed and summarized.ResultsThe patient had undergone coronary artery bypass grafting, had a prosthetic heart valve, and required systemic anticoagulation. Computed tomography showed a well-circumscribed left adrenal lesion with evidence of scattered foci of macroscopic fat; these findings were confirmed on magnetic resonance imaging. After laparoscopic left adrenalectomy, pathologic examination revealed a well-circumscribed, fatty, 7-cm adrenocortical-based tumor, which weighed 135 g. Histologic sampling disclosed mature adipose tissue without evidence of myeloid heterotopy or features of malignant involvement, and adrenal lipoma was diagnosed. On the basis of our review of the English-language literature, this tumor is the 11th reported case of adrenal lipoma. The vast majority of these tumors have been asymptomatic and identified incidentally on imaging or at autopsy. A minority of patients have had a history of hypertension.ConclusionWith the increased utilization and sensitivity of abdominal imaging techniques, identification of nonfunctional adrenal tumors has become more frequent. Adrenal lipoma should be included in the differential diagnosis of lipomatous adrenal tumors. (Endocr Pract. 2008; 14:209-212)     

Asymptomatic Bilateral Giant Adrenal Myelolipomas: Case Report and Review of Literature

ObjectiveTo describe an unusual case of bilateral giant adrenal masses caused by a primary adrenal myelolipoma.MethodsWe present the clinical, laboratory, and pathologic findings in a 32-year-old man with bilateral adrenal masses. The previous reports of bilateral myelolipomas also were reviewed.ResultsDuring a routine examination, a 32-year-old Thai man was found to have an asymptomatic abdominal mass. A computed tomographic scan of the abdomen disclosed bilateral adrenal masses; the one on the left was approximately 27 by 24 by 12 cm, and the one on the right side was 9 by 5 by 5 cm. The computed tomographic scan characteristics showed that both masses consisted mainly of low-density tissues (-30 to -90 Hounsfield units), suggestive of fatty component. An endocrinologic evaluation revealed no evidence of adrenal cortical or medullary functional abnormalities. Bilateral adrenalectomy was performed because of the large size of the lesions and the inability to rule out malignant involvement.ConclusionMyelolipoma is a relatively rare benign tumor of the adrenal glands composed of adipose cells and mature hematopoietic elements. Most such lesions are small, asymptomatic, and unilateral; giant or bilateral myelolipomas are quite rare. To our knowledge, our current case may represent the largest bilateral lesions in the literature. (Endocr Pract. 2007;13:667-671)     

阑尾杯状细胞类癌合并回盲部混合型腺神经内分泌癌1例病例报道并文献复习

目的:报道1例极为罕见的阑尾杯状细胞类癌(goblet cell carcinoid,GCC)合并回盲部混合型腺神经内分泌癌(mixed adenoneuroendocrine carcinoma,MANEC),以提高临床医师对本病的认识。方法:回顾性分析1例阑尾GCC合并回盲部MANEC患者的临床、病理特征、免疫组化及术后情况并进行文献复习。结果:该病例经病理切片会诊明确诊断为GCC,行右半结肠切除术后进一步诊断为回盲部MANEC,行1次FOLFOX化疗后,一般情况良好。结论:阑尾GCC合并回盲部MANEC是具有高度侵袭性的异质性恶性肿瘤,免疫组化局部Cg A和局部Syn阳性、Tang分类C组、临床分期IV期和非根治性手术为不良预后的危险因素。     

Bronchoalveolar Lavage In the Diagnosis of Bronchiolo-Alveolar Carcinoma. A Case Report

Bronchiolo-alveolar carcinoma is usually localized to the terminal bronchioles and alveoli, and may present on chest X-ray as interstitial pulmonary disease with diffuse reticulonodular infiltrates. The cytological diagnosis is often difficult to obtain. This case demonstrates that bronchoalveolar lavage can be useful in the diagnostic evaluation of this type of malignancy, in conjunction with transbronchial lung biopsy.