Diagnostic Challenges in Adrenocortical Carcinoma: Recommendations for Surveillance after Surgical Resection of Selected Adrenal Nodules

ObjectiveTo discuss challenges in the diagnosis of adrenocortical carcinoma and to suggest surveillance measures after removal of selected adrenal nodules.MethodsWe present the case of a 65-year-old man with worsening hypertension and new-onset hypokalemia attributed to primary hyperaldosteronism due to a 3-cm right adrenal nodule.ResultsA laparoscopic right adrenalectomy was performed, and the histologic diagnosis was a benign adenoma. The patient’s hypertension and hypokalemia improved postoperatively but recurred 8 months later, and florid Cushing’s syndrome developed. Magnetic resonance imaging showed an 8-cm mass in the right adrenal bed and multiple hepatic metastatic lesions. Fine-needle biopsy confirmed the presence of adrenocortical carcinoma.ConclusionDespite a comprehensive biochemical, radiologic, and histologic assessment, the diagnosis of adrenocortical carcinoma can be missed. Particularly, we caution against undue reliance on the initial tumor size. We recommend that abdominal imaging be performed every 3 months for the first year and every 6 months for the second year after surgical removal of selected adrenal nodules. (Endocr Pract. 2007;13:636-641)     

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)     

Clinical Outcomes in Adrenal Incidentaloma: Experience From one Center

Objective: To investigate the outcome in patients with adrenal incidentaloma (AI).Methods: A retrospective evaluation of 637 patients with AI referred to a tertiary center over 8 years. Radiologic and hormonal evaluations were performed at baseline. Follow-up imaging was carried out if necessary, and hormonal evaluation was performed at 24 months according to national guidelines.Results: The mean age was 62.7 ± 11.6 years, and the mean AI size was 25.3 ± 17.0 mm at presentation. Hormonal evaluation revealed that 85.4% of all tumors were nonfunctioning adenomas, 4.1% subclinical Cushing syndrome (SCS), 1.4% pheochromocytoma, 1.4% primary hyperaldosteronism, 0.8% Cushing syndrome, 0.6% adrenocortical carcinoma, 0.3% congenital adrenal hyperplasia, 2.2% metastasis to adrenals, and 3.8% other lesions of benign origin. Bilateral tumors were found in 11%, and compared to unilateral tumors, SCS was more prevalent. Only 2 cases were reclassified during follow-up, both as SCS, but neither had had a dexamethasone suppression test performed at initial work-up. In patients diagnosed with an adrenal metastasis, 92.9% were deceased within 2 years. Excluding those with malignant tumors, 12.9% of patients died during the study period of up to 11 years due to other causes than adrenal.Conclusion: Most AIs were benign, but a small fraction of tumors were functional and malignant. The prognosis of patients with adrenal metastasis was extremely poor, but otherwise, the mortality rate was similar to that for the general population. Follow-up of AIs <4 cm with an initial nonfunctional profile and benign radiologic appearance appears unwarranted, but screening for congenital adrenal hyperplasia should be considered.Abbreviations: 17OHP = 17-hydroxyprogesterone ACC = adrenal cortical carcinoma ACTH = adrenocorticotropic hormone AI = adrenal incidentaloma CAH = congenital adrenal hyperplasia CT = computed tomography CS = Cushing syndrome DST = dexamethasone suppression test HPA = hypothalamic-pituitary-adrenal axis MRI = magnetic resonance imaging SCS = subclinical Cushing syndrome T2DM = type 2 diabetes mellitus UFC = urinary free cortisol     

Glucocorticoid- and Androgen-Secreting Black Adrenocortical Adenomas: Unique Cause of Corticotropin-Independent Cushing Syndrome

ObjectiveTo describe the unique association of corticotropin-independent Cushing syndrome caused by cortisoland androgen-secreting black adrenal cortical adenomas with myelolipomatous change.MethodsWe report the clinical, laboratory, radiologic, and pathologic findings from 2 patients who presented with androgen excess and typical signs and symptoms of Cushing syndrome.ResultsEndocrine investigations showed high serum cortisol concentrations that lacked diurnal rhythm, undetectable plasma corticotropin concentrations, and absence of serum cortisol suppression after overnight dexamethasone suppression tests. Serum levels of adrenal androgens were elevated. Computed tomography of the abdomen revealed unilateral adrenal masses (largest lesional diameters 4.0 and 3.1 cm). On the basis of the plurihormonal hypersecretion and the imaging characteristics, adrenocortical carcinoma was considered as a possible diagnosis. However, histopathologic analysis in both patients revealed black adrenal cortical adenomas with myelolipomatous change. After surgery, adrenal androgens normalized, and the signs and symptoms of Cushing syndrome and androgen excess resolved. There was no evidence of recurrent disease at last follow-up.ConclusionsA unique form of corticotropin-independent Cushing syndrome is described: cortisoland androgen-secreting black adrenal cortical adenomas with myelolipomatous change. Although most patients with corticotropin-independent Cushing syndrome associated with androgen excess prove to have adrenocortical carcinoma, the clinician should be aware of the possibility of benign, black adrenal adenomas in this clinical setting. (Endocr Pract. 2011;17:e73-e78)     

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)     

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     

Diagnosis by Serendipity: Cushing Syndrome Attributable to Cortisol-Producing Adrenal Adenoma as the Initial Manifestation of Multiple Endocrine Neoplasia Type 1 Due to a Rare Splicing Site Men1 Gene Mutation

ObjectiveTo report a case that highlights the potential for Cushing syndrome to be the first manifestation of multiple endocrine neoplasia type 1 (MEN 1) syndrome and to describe the rare underlying genetic mutation and the heterogeneous manifestations of the syndrome within the same family.MethodsWe present a case report including biochemical and radiologic findings, review family data, and discuss the results of genetic analyses.ResultsA 16-year-old girl who was not known to have any medical illness and had no known family history of MEN 1 syndrome presented with Cushing syndrome attributable to a cortisol-producing adrenal adenoma. During her evaluation, she was found to have primary hyperparathyroidism and a pituitary microprolactinoma. These findings raised the possibility of MEN 1 syndrome. She did not have clinical, biochemical, or radiologic evidence of islet cell pancreatic tumors. Family screening showed that her father had evidence of primary hyperparathyroidism, mild hyperprolactinemia, normal findings on magnetic resonance imaging of the pituitary, and a 1.2- cm nodule in the tail of the pancreas in conjunction with slight elevation of serum insulin and normal gastrin levels. The patient’s 5 siblings had evidence of primary hyperparathyroidism, and 2 of them also had mild hyperprolactinemia. Genetic screening confirmed the presence of a MEN1 gene missense G to A mutation in the patient, her father, and her siblings at the splicing site of intron 6 (IVS6 + 1G > A). This mutation leads to frameshift and truncation of the MEN1 gene.ConclusionIn MEN 1, Cushing syndrome is an extremely rare and usually late manifestation. Most cases are due to corticotropin-producing pituitary adenomas. Although Cushing syndrome generally develops years after the more typical manifestations of MEN 1 appear, it may be the primary manifestation of MEN 1 syndrome. There is considerable heterogeneity in the manifestations of MEN 1, even within a family having the same genetic mutation. (Endocr Pract. 2008;14:595-602)     

Case report: schwannoma arising from the unilateral adrenal area with bilateral hyperaldosteronism

Background

We report a rare case of a juxta-adrenal schwannoma that could not be discriminated from an adrenal tumor before surgical resection and was complicated by bilateral hyperaldosteronism. To the best of our knowledge, this is first case in which both a juxta-adrenal schwannoma and hyperaldosteronism co-existed.

Case presentation

A 69-year-old male treated for hypertension was found to have a left supra-renal mass (5.8?×?5.2 cm) by abdominal computed tomography. His laboratory data showed that his plasma aldosterone concentration (PAC) was within the normal range, but his plasma renin activity (PRA) was reduced, resulting in an increased aldosterone/renin ratio (ARR). Load tests of captopril or furosemide in the standing position demonstrated autonomous aldosterone secretion and renin suppression. Adrenal venous sampling (AVS) with ACTH stimulation indicated bilateral hypersecretion of aldosterone. A left supra-renal tumor was resected because of the possibility of malignancy and was found to be a benign schwannoma arising from the juxta-adrenal region together with an adrenal gland. The dissected left adrenal gland was morphologically hyperplastic in the zona glomerulosa, but was immunohistochemically negative for CYP11B2 (aldosterone synthase). Multiple CYP11B2-positive adrenocortical micronodules were detected in the adrenal gland, indicating micronodular hyperplasia. Although bilateral aldosteronism was indicated by AVS before the operation, the PRA, PAC and ARR values were within their respective reference ranges after resection of the unilateral tumor, suggesting that the slight increase in hormone secretion from the remaining right-sided lesion could not be detected after resection.

Conclusion

A clinical and morphologic diagnosis of juxta-adrenal schwannoma is difficult, particularly in a case of hyperaldosteronism, as shown in this case. These data suggest the complexity and difficulty diagnosing adrenal incidentaloma.

     

Pituitary Carcinoma in Situ

ObjectivePituitary carcinomas are extremely rare tumors associated with poor prognosis despite surgery, radiation, and chemotherapy. The hallmark of diagnosis implies subarachnoid, brain, or systemic tumor spread.MethodsWe report a case of rapid transformation of atypical nonfunctioning pituitary adenoma to a carcinoma.ResultsA 64-year-old woman presented with sudden onset of ophthalmoplegia. Magnetic resonance imaging (MRI) scan showed a pituitary macroadenoma (2.2 × 2.1 cm) with invasion of the right cavernous sinus. Biochemical data was consistent with a nonfunctioning pituitary adenoma. Pathology showed a pituitary adenoma with negative immunohistochemistry for pituitary hormones. The patient returned a month later with weakness, lethargy, and a dilated nonreactive right pupil. MRI showed an invasive large mass (5 × 4.7 cm). After an emergent second transsphenoidal surgery, histopathologic examination revealed a widely infiltrative neoplasm invading the overlying mucosa and showing a high mitotic activity and necrosis and a very high Ki-67 (MIB-1) proliferation index (80%). MIB-1 retrospectively performed on the first specimen was also elevated (30%). Soon after the second surgery, MRI showed a 7.9 × 8.0 cm mass that metastasized to dura mater and extended into the right orbit, right middle cranial fossa, nasopharynx, clivus, posterior fossa, and along the right tentorium cerebelli, resulting in significant compression of the brainstem.ConclusionDevelopment of a pituitary carcinoma from an adenoma is an exceptional occurrence and predictors of such course are currently lacking. A very high Ki-67 proliferation index should raise concern of a pituitary carcinoma in situ or premetastatic carcinoma. (Endocr Pract. 2013;19:e69-e73)     

Intravascular Lymphoma: an Unusual Diagnostic Outcome of an Incidentally Detected Adrenal Mass

ObjectiveTo describe a rare diagnosis of intravascular lymphoma in a patient presenting with an incidentally discovered adrenal mass.MethodsWe describe the patient’s clinical history and the findings from biochemical evaluation, radiologic studies, and surgical pathology and review the relevant literature.ResultsA 43-year-old woman developed sudden onset of flank pain associated with a flushing sensation and presented to the emergency department where computed tomography showed a 5-cm left adrenal mass. She had normal electrolytes, and serum and urinary test results were negative for pheochromocytoma. A 24-hour urinary cortisol level was minimally elevated, and the midnight salivary cortisol value was within the reference range. Magnetic resonance imaging revealed a 6.5 × 5.8-cm left adrenal lesion that demonstrated moderate T2-weighted signal and gradual delayed enhancement with no drop in signal on out-of-phase images. Since the lesion lacked high intensity and the biochemical testing results did not suggest a pheochromocytoma, it was deemed likely that the mass was a malignant lesion of the left adrenal gland. A laparoscopic left adrenalectomy was performed. Morphologic and immunohistochemical findings were consistent with a large B-cell lymphoma, which by virtue of its near exclusive distribution in vascular spaces, was consistent with the diagnosis of intravascular large B-cell lymphoma.ConclusionIntravascular large B-cell lymphoma should be included in the differential diagnosis of an incidentally detected adrenal mass even though the diagnosis is rare. (Endocr Pract. 2008;14:884-888)     

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: 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)     

Diagnostic Challenge of Pheochromocytoma in a Patient Receiving Levodopa for Parkinson's Disease

ObjectiveThe diagnosis of pheochromocytoma in patients receiving levodopa is challenging because the standard diagnostic biochemical tests may be confounded by dopaminergic therapy. We aim to showcase our experience with the diagnosis of pheochromocytoma in a patient with a known case of Parkinson’s disease who was receiving levodopa.MethodsWe present the case of an elderly male who was diagnosed as having pheochromocytoma while receiving dopaminergic therapy for Parkinson’s disease.ResultsA 75-year-old man presented with vague abdominal symptoms. Computed tomography revealed a 3.5 × 3.2 cm right adrenal mass with a well-defined margin. As revealed by magnetic resonance imaging, the mass was hypointense on T1-weighted and hyperintense on T2-weighted images. Biochemical tests revealed elevated levels of urinary dopamine, which was considered to be caused by levodopa therapy. However, concurrent elevation in urinary adrenaline and his metanephrine and vanillylmandelic acid levels suggested an underlying case of pheochromocytoma. An ¹²³I-metaiodobenzylguanidine (¹²³I-MIBG) scintigraphy scan performed under levodopa therapy showed positive tracer uptake in the right adrenal gland. Histopathology of the adrenalectomy specimen confirmed the diagnosis of pheochromocytoma.ConclusionOur experience with the present case indicates that although the standard diagnostic biochemical tests for pheochromocytoma may be confounded by dopaminergic therapy, ¹²³I-MIBG scintigraphy has diagnostic value for confirming pheochromocytoma even in patients receiving dopaminergic therapy. (Endocr Pract. 2014;20:e112-e115)     

Mixed Corticomedullary Carcinoma of the Adrenal Gland: A Case Report

ObjectiveTo report the case of a 78-year-old woman with mixed corticomedullary carcinoma of the adrenal gland, and to review other reported lesions that exhibit clinical and/or histopathologic features of both adrenal cortical and medullary differentiation.MethodsWe describe the patient’s clinical findings and laboratory test results, as well as the gross and histopathologic features of her tumor. We also review the literature pertaining to mixed corticomedullary adenomas and cortical tumors with clinical features of pheochromocytoma, and vice versa.ResultsA 78-year-old woman with a 10-cm left adrenal mass was hospitalized for management of hypertensiveurgency. Laboratory workup revealed elevated urinary metanephrine excretion and elevated serum dehydroepiandrosterone sulfate levels. She underwent left adrenalectomy. Pathologic examination of the lesion showed mixed cortical and medullary histologic characteristics, as well as gross and microscopic evidence of malignancy. Including the present case, we identified 17 cases of neoplasms that exhibit features of mixed corticomedullary differentiation.ConclusionsThis report represents the first documented case of mixed corticomedullary carcinoma. Several benign lesions combine clinical, biochemical, and/or histopathologic evidence of both adrenal cortical and medullary differentiation, including mixed corticomedullary adenomas and corticotropin-secreting pheochromocytomas. The differential diagnosis of a lesion with mixed cortical and medullary features should also include a malignant neoplasm. (Endocr Pract. 2012;18:e37-e42)     

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)     

Unexpected Clinical Course During Treatment of a TSH-Secreting Pituitary Adenoma

ObjectiveTo report the rare occurrence of a patient with thyrotropinoma that transitioned into a secretory thyro-somatotroph adenoma during medical treatment with somatostatin analogue.MethodsWe report the case of a patient with a thyrotroph pituitary adenoma who developed de novo evidence of growth hormone cosecretion following one year of successful medical treatment.ResultsA 78-year-old woman was diagnosed with a thyroid stimulating hormone (TSH) secreting pituitary macroadenoma (TSHoma) based on classical clinical and biochemical features. There was no clinical or biochemical evidence of growth hormone (GH) cosecretion. She declined surgical resection and was treated with primary medical therapy, octreotide long acting repeatable (LAR), to which she had an antitumor and antisecretory response; however, following 12 months of successful medical treatment she developed de novo hypersecretion of growth hormone despite involution of the tumor mass. TSH-secreting pituitary adenomas may rarely become plurihormonal during apparently successful medical treatment. This may represent an unusual form of secondary resistance to somatostatin analogue or the rarer phenomenon of tumor transformation into a secretory thyro-somatotroph adenoma.ConclusionThe unexpected clinical course of this case highlights the need for careful long-term surveillance in patients with TSH secreting pituitary adenomas. (Endocr. Pract. 2013;19:e88-e91)     

Plurihormonal Pituitary Adenoma Immunoreactive for Thyroid-Stimulating Hormone,Growth Hormone,Follicle-Stimulating Hormone,and Prolactin

ObjectiveTo describe the case of a patient with an unusual plurihormonal pituitary adenoma with immunoreactivity for thyroid-stimulating hormone (TSH), growth hormone, follicle-stimulating hormone, prolactin, an α-subunit.MethodsWe report the clinical, laboratory, imaging, and pathology findings of a patient symptomatic from a plurihormonal pituitary adenoma and describe her outcome after surgical treatment.ResultsA 60-year-old woman presented to the emergency department with headaches, blurry vision, fatigue, palpitations, sweaty hands, and weight loss. Her medical history was notable for hyperthyroidism, treated intermit with methimazole. Magnetic resonance imaging disclosed a pituitary macroadenoma (2.3 by 2.2 by 2.0 cm), and preoperative blood studies revealed elevated levels of TSH at 6.11 mIU/L, free thyroxine at 3.6 ng/dL, and free triiodothyronine at 6.0 pg/mL. She underwent an uncomplicated transsphenoidal resection of the pituitary adenoma. Immunostaining of tumor tissue demonstrated positivity for not only TSH but also growth hormone, follicle-stimulating hormone, prolactin, and α-subunit. The Ki-67 index of the tumor was estimated at 2% to 5%, and DNA repair enzyme O6-methylguanine-DNA methyltransferase immunostaining was mostly negative. Electron microscopy showed the ultrastructural phenotype of a glycoprotein-producing adenoma. Postoperatively, her symptoms and hyperthyroidism resolved.ConclusionThyrotropin-secreting pituitary adenomas are rare. Furthermore, recent reports suggest that 31% to 36% of adenomas may show evidence of secretion of multiple pituitary hormones. This case emphasizes the importance of considering pituitary causes of thyrotoxicosis and summarizes the clinical and pathology findings in a patient with a plurihormonal pituitary adenoma. (Endocr Pract. 2012;18:e121-e126)     

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)     

Large Adrenal Oncocytoma with Uncertain Malignant Potential: Case Report and Review of Literature

ObjectiveTo report a case of oncocytoma, a relatively rare adrenal tumor, which most commonly is detected as an adrenal incidentaloma.MethodsWe present a case report, including laboratory, imaging, and pathologic findings, of a 47-year-old obese woman who had hypertension and an incidentally found large, left adrenal mass.ResultsOn the basis of the hormonal evaluation, this mass was a nonsecreting adrenal tumor, which histologically proved to be an oncocytoma with borderline malignant characteristics. A collective analysis of the few cases of adrenal oncocytoma published in the medical literature showed that our case corresponded to the previously published cases in preponderant location (left side) as well as the general size (11.4 cm in the largest dimension) and weight (372 g).ConclusionAdrenal oncocytoma should be included in the differential diagnosis of adrenal incidentalomas, especially if large tumors are detected. In addition, a longterm follow-up is suggested because there are no certain clues about the true potential of this tumor. (Endocr Pract. 2010;16:641-645)