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)     

Adrenal Incidentalomas: Diagnostic Evaluation and Long-Term Follow-Up

ObjectiveTo evaluate the cause and the clinical and laboratory features of adrenal incidentalomas (AI) in 52 patients and to assess the evolution of nonsurgically treated lesions during long-term follow-up.MethodsWe retrospectively analyzed the medical records of 52 patients with AI undergoing routine followup in 2 Brazilian endocrine centers.ResultsIn our study group, nonfunctioning adenomas were the most frequent cause of AI (42%), followed by cortisol-secreting adenomas (15%), metastatic disease (10%), pheochromocytomas (8%), myelolipomas (6%), cysts (6%), carcinomas (4%), lymphomas (4%), tuberculosis (4%), and aldosteronoma (2%). Only 13 lesions (25%) were functioning (8 cortisol-secreting adenomas, 4 pheochromocytomas, and 1 aldosteronoma). Carcinomas were the largest adrenal masses (mean diameter, 11.7 ± 1.3 cm). With the exception of 1 pheochromocytoma, 1 cyst, and 1 myelolipoma, all AI larger than 6 cm were carcinomas. During follow-up of 21 patients with nonsurgically treated AI for 6 to 36 months (mean, 24.8 ± 8.9), no patient had tumor reduction or disappearance. After 12 months of follow-up, however, a 45-year-old woman had adrenal mass enlargement from 3.2 cm to 4.4 cm; the excised lesion proved to be an adenoma. Moreover, evidence of cortisol hypersecretion developed after 24 months of follow-up in a 30-year-old man with a 3.5-cm adenoma in the left adrenal gland.ConclusionOur findings demonstrate that most AI are nonfunctioning benign lesions and emphasize the need for long-term follow-up of patients with conservatively managed lesions, in light of the potential for evolution to hormonal hypersecretion or tumor growth. (Endocr Pract. 2008;14:269-278)     

Bilateral giant myelolipoma in the adrenal of a cotton-top tamarin (Saguinus oedipus)

Abstract: A rare case of bilateral adrenal myelolipomas in a female cotton-top tamarin is reported. Large bilateral masses in the adrenal glands were composed of mature adipose cells containing varying amounts of hematopoietic cells of the myeloid, erythroid, and megakaryocyte series. The gross and histologic features of this case closely resemble human “giant” adrenal myelolipomas.     

Association of Pheochromocytoma and Ganglioneuroma: Unusual Finding in Neurofibromatosis Type 1

ObjectiveTo report a rare case of association of pheochromocytoma and ganglioneuroma in an asymptomatic patient with neurofibromatosis type 1 (NF1) and to discuss the importance of annual biochemical and imaging studies.MethodsWe present the clinical, laboratory, and pathology findings in a 41-year-old woman with NF1 and review the pertinent literature.ResultsA 41-year-old woman with NF1 presented for a routine gynecologic examination, at which time a right adrenal mass (4 by 3 cm) was discovered by abdominal ultrasonography and confirmed by abdominal computed tomographic scans and magnetic resonance imaging. The patient was normotensive and complained only of discrete essential tremors. Biochemical studies showed a serum epinephrine level of 195 pg/mL (normal,<100) and a 24-hour urine epinephrine excretion of 55 μg (normal,<20), findings consistent with pheochromocytoma. Metaiodobenzylguanidine scintigraphy revealed uptake in the right adrenal gland, with no evidence of metastatic lesions. Before surgical treatment, the patient received an α-adrenergic antagonist for 30 days. Laparoscopic excision of the right adrenal gland yielded excellent postoperative results. Surgical pathology revealed a multinodular mass composed of pheochromocytoma and ganglioneuroma. In patients with NF1 (von Recklinghausen’s disease), a tumor consisting of pheochromocytoma and ganglioneuroma is rare and may be more aggressive than pheochromocytoma alone. An asymptomatic catecholamine-producing tumor may cause substantial morbidity and mortality, especially in patients who are undergoing surgical intervention or are under other stressors.ConclusionThe current guidelines for managing patients with NF1 are an annual history and physical examination. Because of the increased prevalence of pheochromocytoma and ganglioneuroma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies. (Endocr Pract. 2007;13:647-651)     

Multiple Unilateral adrenal Adenomas in a Patient with Primary Hyperaldosteronism

ObjectiveTo report a rare case of multiple unilateral adrenal adenomas in which immunohistochemistry results confirmed primary hyperaldosteronism in each of 3 adenomas.MethodsWe present the clinical, laboratory, radiographic, and pathologic findings of a case of multiple unilateral adrenal adenomas.ResultsAlthough multiple nodules in both adrenal glands are fairly common in patients with bilateral hyperplasia, multiple unilateral nodules are extremely rare. A 45-year-old woman with a long-standing history of severe hypertension was found to have biochemical parameters consistent with primary hyperaldosteronism, multiple unilateral adrenal adenomas, and immunohistochemical test results confirming primary hyperaldosteronism arising from each of 3 adrenal nodules (measuring 2.2 × 2.2 cm, 1.7 × 0.7 cm, and 0.5 × 0.5 cm).ConclusionThis case illustrates the rare presentation of primary hyperaldosteronism as multiple unilateral adrenal adenomas in which immunohistochemistry results can confirm the suspected preoperative diagnosis as suggested by biochemical and radiographic evidence. (Endocr Pract. 2008;14:76-79)     

Retroperitoneal Cystic Teratoma Masquerading as an Incidentally Discovered Adrenal Mass

ObjectiveTo report a case of a retroperitoneal cystic teratoma that obscured and compressed the adrenal gland, mimicking a primary adrenal tumor.MethodsThe presenting manifestations, radiographic characteristics, gross and microscopic pathologic features, and results of surgical therapy and long-term follow-up are described.ResultsA 50-year-old African American woman with a 2-year history of low back pain and night sweats had a computed tomographic scan of the abdomen, which revealed an incidental 8 by 4 by 3.5-cm left adrenal mass without a clear plane between the mass and the left crus of the diaphragm. Laboratory studies excluded a functioning adrenal tumor. The tumor was resected laparoscopically. It was compressing but not involving the adrenal gland, nor was it involving the diaphragm. Microscopic evaluation revealed a benign mature cystic teratoma characterized by cystic spaces lined by respiratory epithelium with cartilage, bone, lymphoid tissue, smooth muscle, and ganglionic tissue in the cyst wall. The patient had an uneventful postoperative course and is free of recurrence after 18 months of follow-up.ConclusionAlthough rare, a mature cystic teratoma of the retroperitoneum that compresses the normal adrenal gland may masquerade as a primary adrenal tumor and should be included in the differential diagnosis of a nonfunctioning adrenal incidentaloma. (Endocr Pract. 2011; 17:e130-e134)     

Adrenal Incidentalomas, 2003 to 2005: Experience After Publication of the National Institutes of Health Consensus Statement

ObjectiveTo determine the clinical outcomes and follow-up imaging characteristics for incidentally discovered adrenal nodules at a large metropolitan health care center, as well as compliance with National Institutes of Health guidelines for the evaluation of these lesions.MethodsBetween October 2003 and June 2005, computed tomographic (CT) scanning performed at Harvard Vanguard Medical Associates detected 165 adrenal nodules (incidentalomas) in 129 patients without extra-adrenal malignant lesions or evidence of adrenal hyperfunction. Nodule characteristics, including size at baseline, change in size at follow-up, CT attenuation values, biochemical data, and clinical outcomes, were analyzed.ResultsOf the 165 adrenal incidentalomas, 96% were smaller than 4 cm. When attenuation values were determined, 65% were low (< 10 Housfield units). Ninetyeight patients (76%) with 142 nodules (86% of all incidentalomas) had at least 1 follow-up CT scan. Of these 142 lesions, 20 (14%) changed significantly in size (grew or became smaller by 0.5 cm or more). Growth was detected within 12 months in 5 of the 7 lesions (71%) that grew. For 95% of patients, the adrenal nodule did not necessitate intervention, including change in medication or surgical treatment. Biochemical studies for adrenal hyperfunction were done in 39 of the overall 129 patients (30%), but in only 20 of 109 patients (18%) not seen by endocrinologists.ConclusionMost adrenal incidentalomas are benign, nonfunctional tumors. Growth typically occurs within 12 months after detection. Despite the 2002 National Institutes of Health guidelines, 82% of patients at our medical center with adrenal nodules who were not seen by endocrinologists did not have hormonal testing. More physician education about the evaluation of incidentalomas should be provided, and prospective studies to establish the appropriate duration of follow-up should be conducted. (Endocr Pract. 2008;14:279-284)     

Virilizing Ovarian Leydig Cell Tumor in A Woman with Subclinical Cushing Syndrome

ObjectiveTo report the case of a patient with a virilizing ovarian Leydig cell tumor and subclinical Cushing syndrome attributable to an adrenal adenoma.MethodsDetailed clinical, laboratory, radiologic, and pathologic findings are presented, and the pertinent literature is reviewed.ResultsA 49-year-old woman was referred for evaluation of a left adrenal mass (3.0 by 2.4 cm), which had been diagnosed by computed tomographic scan 4 years previously during a work-up for hirsutism. On examination, she had central obesity, facial hirsutism, and male pattern baldness. Work-up showed elevated total and free testosterone levels of 196 ng/dL (reference range, 20 to 70) and 24 pg/mL (1 to 9), respectively. Other results (and reference ranges) were as follows: dehydroepiandrosterone sulfate, 7.5 μg/dL (10 to 221); corticotropin, 12 pg/mL (5 to 50); morning cortisol, 1.4 μg/dL after a 1-mg overnight dexamethasone suppression test; and urine free cortisol, 48.8 μg/24 h (20 to 100). The testosterone level decreased by 14% after a 2-day low-dose dexamethasone suppression test. Findings on transvaginal ovarian ultrasonography and a computed tomographic scan of the pelvis were normal. A laparoscopic adrenalectomy revealed an adrenal adenoma. On the first day postoperatively, the cortisol level was less than 1.0 μg/dL; however, the testosterone level remained elevated. At 6 months postoperatively, a normal result of a cosyntropin stimulation test indicated recovery of the hypothalamic-pituitaryadrenal axis. Bilateral oophorectomy revealed a 1.3-cm right ovarian Leydig cell tumor. Postoperatively, the testosterone level declined to less than 20 ng/dL.ConclusionTo our knowledge, this is the first case report of a virilizing ovarian Leydig cell tumor in a patient with subclinical Cushing syndrome. (Endocr Pract. 2008;14:358-361)     

Concomitant Secretion of Glucocorticoid,Androgens, and Mineralocorticoid by an Adrenocortical Carcinoma: Case Report and Review of Literature

ObjectiveTo present a case of concomitant secretion of cortisol, androgens, and 11-deoxycorticosterone (DOC) by an adrenocortical carcinoma and review the literature in an attempt to identify similar cases.MethodsThe patient’s medical history, physical examination, laboratory data, computed tomographic scan, and histopathologic results were analyzed and summarized in a case report, and an extensive review of the literature was performed.ResultsEndocrinologic data showed excess cortisol production, substantially elevated testosterone and androstenedione levels, and profoundly increased DOC in the setting of suppressed aldosterone. An abdominal computed tomographic scan showed a left adrenal tumor. A left adrenalectomy was performed, and the histopathologic diagnosis was stage II adrenocortical carcinoma. The review of the pertinent literature revealed the absence of any identical cases in the past.ConclusionOur patient presented with a rare case of cosecretion of cortisol, testosterone, androstenedione, and DOC by an adrenocortical carcinoma, resulting in a clinical picture consistent with Cushing’s syndrome, hyperandrogenism, and primary hypermineralocorticoidism. We recommend the routine performance of a DOC assay in the setting of mineralocorticoid excess in association with low plasma aldosterone levels. (Endocr Pract. 2007;13: 408-412)     

Hyperandrogenism Due to a Testosterone-Secreting Sertoli-Leydig Cell Tumor Associated With a Dehydroepiandrosterone Sulfate-Secreting Adrenal Adenoma in a Postmenopausal Woman: Case Presentation and Review of Literature

ObjectiveTo report a case of hyperandrogenism attributable to the presence of an adrenal adenoma secreting dehydroepiandrosterone sulfate (DHEA-S) and an ovarian Sertoli-Leydig cell tumor secreting testosterone in a postmenopausal woman.MethodsThe laboratory, radiologic, and pathologic findings in our case are described. In addition, the pertinent literature is reviewed.ResultsA 56-year-old woman presented with a history of gradual increase in facial and body hair, scalp hair loss, male pattern baldness, and deepening of her voice, beginning a few years after spontaneous menopause at age 49 years. She had hypertension, obesity, and type 2 diabetes mellitus. Laboratory tests showed elevated levels of total testosterone (348 ng/dL) and DHEA-S (2,058 μg/dL), and a left adrenal tumor (3 by 4 cm) was detected on abdominal computed tomographic scan. Laparoscopic left adrenalectomy was performed, and the pathologic diagnosis was adrenal adenoma. The DHEA-S returned to normal levels, but the serum testosterone concentration remained elevated. Transvaginal ultrasonography disclosed an ovarian tumor. Bilateral oophorectomy was performed, and an ovarian Sertoli-Leydig cell tumor was diagnosed. The hormonal and clinical picture normalized after this surgical intervention.ConclusionAfter extensive review of the literature, we believe that this is the first reported case of a coincidental DHEA-S-secreting adrenal adenoma and a testosterone-secreting ovarian Leydig cell tumor causing signs of virilization. (Endocr Pract. 2009;15:149-152)     

Giant Pancreatic Tumor With Clinical Characteristics of Insulinoma But Without Common Pathologic Features

ObjectiveTo report a case of a large pancreatic tumor that had clinical characteristics of an insulinoma without classic pathologic features.MethodsWe describe a 58-year-old woman who presented with a 3-month history of symptomatic hypoglycemic episodes, which were characterized by confusion. The laboratory, imaging, and pathologic findings are summarized, the current literature on giant insulinomas is reviewed, and the distinction between clinical and pathologic diagnosis of neuroendocrine tumors is discussed.ResultsThe biochemical diagnosis of insulinoma was established with concomitant low fasting blood glucose concentrations and inappropriately high insulin levels. An abdominal computed tomographic scan revealed a mass (10 by 11.7 by 9.7 cm) in the head and body of the pancreas, which was resected. Pathologic examination revealed a massive neuroendocrine tumor (13.5 by 11 by 8 cm) without immunohistochemical evidence of insulin expression. Nevertheless, tumor resection resulted in decreased blood insulin levels and resolution of the patient’s hypoglycemia.ConclusionAlthough more than 95% of insulinomas are smaller than 3 cm, this case is unique in that the extremely large pancreatic tumor had clinical characteristics of an insulinoma but did not have the classic pathologic findings. Because of the extensive pancreatic resection, the patient is dependent on both insulin and orally administered pancreatic enzymes but remained free of symptoms and disease recurrence at 1-year follow-up. (Endocr Pract. 2011;17:e12-e16)     

Osteogenic Sarcoma of the Sella After Radiation Treatment of a Pituitary Adenoma

ObjectiveTo report an uncommon case of osteogenic sarcoma of the sella turcica after radiation treatment of a pituitary adenoma.MethodsWe present the clinical history, physical findings, laboratory data, imaging studies, and pathologic findings in a patient found to have osteogenic sarcoma of the sella after radiation therapy for a nonfunctioning pituitary adenoma.ResultsSix years after transsphenoidal resection and postoperative fractionated radiation therapy for a nonfunctioning pituitary adenoma that extended to the cavernous sinus, a 45-year-old man presented with a sinus infection, diplopia, and ophthalmoplegia of the right eye. A computed tomographic scan of the head showed a mass in the sella with involvement of the optic chiasm and right cavernous sinus. Transsphenoidal resection and debulking of the tumor revealed an osteogenic sarcoma. The patient was discharged from the hospital with residual diplopia and ophthalmoplegia. He was treated with levothyroxine, testosterone, and hydrocortisone. Six weeks later, the patient was readmitted after he was found unresponsive, and computed tomographic scans disclosed a massive cerebrovascular accident. He died a few days later.ConclusionOsteogenic sarcoma is a rare, late complication of radiation treatment of pituitary adenoma. Although radiotherapy remains an effective adjunctive treatment in patients with pituitary adenomas, particularly those with residual or recurrent tumor, potential complications must be acknowledged. (Endocr Pract. 2004;10: 335-338)     

Composite Pheochromocytoma-Ganglioneuroma:A Rare Experiment of Nature

ObjectiveTo present a rare case of composite pheochromocytoma-ganglioneuroma (Pheo-GN) of the adrenal medulla, review the related literature, and discuss the clinical features, pathologic findings, behavior, and management of such tumors.MethodsA case report of a patient with composite Pheo-GN of the adrenal gland is presented. Using the online database PUBMED, we searched and analyzed all cases of composite pheochromocytoma reported in the English-language literature during the past 70 years.ResultsOn computed tomography, a 61-year-old man was incidentally found to have a 3.8-cm nonadenomatous right adrenal lesion. Adrenalectomy revealed a 5-cm mass consistent with composite Pheo-GN. To date, 45 cases of composite pheochromocytomas have been reported during the past 70 years, 71% of which coexisted with ganglioneuromas. These tumors occurred with approximately equal frequency in male and female patients, the majority of whom were from 40 to 60 years old. Only 14 cases have been reported in the United States. Bilateral tumors were found in 3 cases. The mean size was 4 to 6 cm. Preoperatively, functional evidence was found in 76.3% of all composite pheochromocytomas (and in 67% of Pheo-GN). Only one Pheo-GN was found to have liver metastatic lesions at the time of autopsy; the rest were not aggressive.ConclusionTo our knowledge, this is the first literature review describing the characteristics and behavior of all reported cases of composite pheochromocytomas, with an emphasis on those with ganglioneuromas. Composite pheochromocytoma is a rare variant of a relatively uncommon disease diagnosed by pathologists only. Fortunately, the treatment of such an entity remains the same as for any pheochromocytoma. (Endocr Pract. 2010;16:291-299)     

The Potential Clinical Application of a Lower Bilateral Adrenal LIMB Width Ratio (L/RW) in Patients with Bilateral Primary Hyperaldosteronism

Objective: This study investigated the characteristics of the adrenal limbs of primary aldosteronism (PA) patients and evaluated the value of the adrenal limb width measurement for the differentiation of unilateral PA from bilateral PA.Methods: A total of 122 PA patients (93 unilateral PA, ages ranged from 23 to 72 years; 29 bilateral PA, ages ranged from 30 to 68 years) who had undergone successful adrenal venous sampling (AVS) and adrenal gland computed tomography (CT) scan were retrospectively included. The maximum width of each adrenal gland limb (normal area on CT images) was measured, the left adrenal limb width to right adrenal limb width ratio (L/Rw) was calculated, and its potential value in the differentiation of unilateral PA and bilateral PA was analyzed.Results: The mean widths of the left adrenal limbs and the right adrenal limbs were 0.52 ± 0.10 cm and 0.43 ± 0.09 cm in unilateral PA patients, versus 0.52 ± 0.10 cm and 0.49 ± 0.12 cm in bilateral PA patients. The L/Rw ratio was 1.22 ± 0.24 in unilateral PA patients and 1.11 ± 0.23 in bilateral PA patients (P<.05). In the subgroup of PA patients over 55 years of age, compared with AVS, the sensitivity and specificity of the L/Rw ratio at 1.06 for subtype classification were 75% and 82%, respectively.Conclusion: A lower L/Rw ratio, referring to the ratio of the left adrenal limb width to the right adrenal limb width, may be a predictor of bilateral PA, especially in PA patients over 55 years of age.Abbreviations: APA = aldosterone-producing adenoma; AVS = adrenal venous sampling; BAH = bilateral adrenal hyperplasia; BMI = body mass index; CT = computed tomography; L/Rw = ratio of left adrenal limb width to right adrenal limb width; PA = primary aldosteronism     

Humoral Hypercalcemia of Malignancy in a Patient with Large Cell Carcinoma of the Lung: Report of Case and Review of Literature

ObjectiveTo report a case of hypercalcemia associated with parathyroid hormone-related protein (PTHrP) in large cell carcinoma of the lung.MethodsWe present a case of PTHrP-mediated hypercalcemia in a patient with a large cell carcinoma of the lung and review the related literature.ResultsA 43-year-old African American man required medical attention because of lethargy, confusion, and poor oral intake. He had bullous emphysema attributable to a 50-pack-year smoking history. On physical examination, vital signs were normal, he was oriented to place and person but not time, and he had cachexia. Breath sounds were decreased in the left lower lung field. Findings on cardiac and abdominal examination were normal. Results of laboratory studies (and corresponding reference ranges) were as follows: calcium 12.1 mg/dL (8.5 to 10.5), albumin 2.0 g/dL (3.5 to 5.0), phosphorus 2 mg/dL (2.5 to 4.5), alkaline phosphatase 68 U/L (40 to 150), intact parathyroid hormone 5 pg/mL (10 to 60), PTHrP 7.0 pmol/L (0.0 to 1.5), 1,25-dihydroxyvitamin D 20.8 pg/mL (25.1 to 66.1), and 25-hydroxyvitamin D 3.7 ng/mL (10 to 60). Computed tomographic scans of the chest showed a large complex lesion in the left lower hemithorax, a small right pleural effusion, and extensive pulmonary emphysema bilaterally. Open lung biopsy revealed a large cell undifferentiated carcinoma. Abdominal and pelvic computed tomographic scans showed no evidence of metastatic involvement. A bone scan was negative for osseous metastatic lesions.ConclusionAlthough the finding is rare, patients with large cell carcinoma of the lung and hypercalcemia may have humoral hypercalcemia mediated by PTHrP. (Endocr Pract. 2007;13:389-395)     

A Case of Adrenal Cavernous Hemangioma Presenting with Progressive Enlargement and Apparent Hormonal Hypersecretion

ObjectiveTo report the case of a man with an adrenal cavernous hemangioma presenting as a progressively enlarging adrenal mass with apparent hormonal hyper-secretion.MethodsWe report the clinical, laboratory, imaging findings, and clinical course of this patient, and we highlight the important atypical features of this case. The literature is reviewed for the typical presentations of adrenal cavernous hemangiomas.ResultsA 59-year-old man presented with an adrenal incidentaloma that had an imaging phenotype suggestive of a pheochromocytoma or an adrenal carcinoma. The hormonal profile also suggested a state of aldosterone and catecholamine hypersecretion. Surgery, however, proved the diagnosis to be an adrenal cavernous hemangioma.ConclusionAlthough adrenal cavernous hemangioma is a rare entity, it should be considered in the differential diagnosis of an adrenal incidentaloma. Its radiologic features are not specific, and the presence of hormonal hypersecretion does not exclude the diagnosis. (Endocr Pract. 2008;14:104-108)     

Adrenal Cortical Carcinoma With Late Pulmonary Metastases Causing Clinicical Cushing’s Syndrome: Case Report With Immunohistochemical Analysis Of Steriodogenic Enzyme Production

ObjectiveTo present a case of pulmonary metastases from adrenocortical carcinomas (ACC) that were secreting fully-functional cortisol resulting in clinical Cushing’s syndrome and to compare the steroidogenic enzyme expression in the primary tumor and lung.MethodsWe analyzed and summarized the patient’s medical history, physical examination results, labora tory data, imaging studies, and histopathologic results. The original tumor and the pulmonary metastases were then immunohistochemically evaluated for steroidogenic enzymes.ResultsInitial endocrinological workup revealed hyperandrogenism and adrenocorticotropic hormone (ACTH) independent Cushing’s due to a 4 cm left adrenal mass. The patient was initially diagnosed with an adrenal adenoma. Four years later, the patient developed recurrent Cushing’s syndrome. Repeat magnetic resonance imaging (MRI) showed no adrenal masses; however, chest computed tomography (CT) showed multiple bilateral lung nodules and biopsy revealed metastases of adrenal origin. Upon immunohistochemical analysis, side chain cleavage, 17α hydroxylase, 3β hydroxysteroid dehydrogenase, and 21 hydroxylase immunoreactivity were detected in both the original and pulmonary metastatic lesions with patterns of disorganized steroidogenesis. Dehydroepiandrosterone sulfotransferase (DHEA-ST) immunoreactivity was detected in the original tumor but not in the lung metastases.ConclusionThis case demonstrates some inter esting features of ACC that pose challenges to its management, including the difficulties in establishing the pathologic diagnosis, the potential for fullyfunctional steroidogenesis even in late metastases, and the plasticity of steroidogenic potential in tumor cells. (Endocr Pract. 2012;18:e138-e143)     

Reversibility of Fibrotic Appearance of Lungs with Thyroxine Replacement Therapy in Patients with Severe Hypothyroidism

ObjectiveTo present 2 cases of hypothyroidism with hypoxia associated with computed tomographic (CT) features suggestive of pulmonary fibrosis that resolved with correction of the hypothyroidism.MethodsClinical case histories are described, comparative radiologic pulmonary images before and after treatment are provided, and the pertinent literature regarding possible pathologic mechanisms is reviewed.ResultsOur first patient, a 68-year-old woman, presented with symptomatic severe hypothyroidism associated with respiratory failure. A CT scan of her lungs showed appearances suggestive of pulmonary fibrosis. Replacement therapy with levothyroxine led to correction of hypoxia and radiologic abnormalities. Our second patient, a 26-year-old man, presented with symptoms suggestive of obstructive sleep apnea that persisted despite use of positive pressure ventilation. Biochemical evaluation revealed severe hypothyroidism, and a CT scan disclosed pulmonary appearances consistent with fibrosis. His symptoms and radiologic abnormalities also improved after correction of hypothyroidism with levothyroxine therapy.ConclusionRadiologic pulmonary abnormalities suggestive of fibrotic disease are associated with severe hypothyroidism. Invasive investigations such as lung biopsy should be deferred until the clinical and radiologic responses to thyroxine replacement therapy have been assessed. (Endocr Pract. 2009;15:720-724)     

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.