Achievement of Fertility in an Infertile Man With Resistant Macroprolactinoma Using High-Dose Bromocriptine and a Combination of Human Chorionic Gonadotropin and an Aromatase Inhibitor

ObjectiveTo describe the achievement of fertility in an infertile man with a resistant macroprolactinoma by using high-dose bromocriptine and a combination of human chorionic gonadotropin (hCG) and an aromatase inhibitor.MethodsWe present historical features and results of clinical, laboratory, and imaging evaluation in a man with secondary infertility attributable to a resistant macroprolactinoma.ResultsWe report a case of macroprolactinoma in a 36-year-old infertile man who failed to attain a normal serum testosterone level and fertility with use of either bromocriptine or cabergoline treatment. Testosterone replacement or hCG therapy in this patient resulted in an increase in serum prolactin levels, which declined after discontinuation of this therapy. The combination of high doses of bromocriptine, hCG, and an aromatase inhibitor facilitated near-normalization of serum prolactin levels, shrinkage of the macroprolactinoma, recovery of serum testosterone levels, sexual function, and sperm count, and achievement of fertility.ConclusionAn aromatase inhibitor may facilitate successful testosterone replacement therapy in male patients with prolactinoma. (Endocr Pract. 2010;16:669-672)     

Bone Marrow Hypoplasiaresponsive to Testosterone Therapyin a Patient with Panhypopituitarism:Need for Adherence to Androgen Replacement

ObjectiveTo describe the case of a young Saudi male patient with long-term panhypopituitarism and pancytopenia attributable to poor adherence to androgen replacement therapy, which resolved after institution of testosterone treatment and recurred after another interval of poor adherence to recommended therapy.MethodsWe present the clinical and laboratory data before and after treatment with testosterone. In addition, the corresponding histologic changes in the bone marrow are illustrated.ResultsAfter resection of a hypothalamic glioma, panhypopituitarism developed in a 14-year-old Saudi boy. At age 22 years, he had shunt-related meningitis. He was then noted to have pancytopenia, with a platelet count of 54 × 10³/μL, a hemoglobin concentration of 6.9 g/dL, and a leukocyte count of 2.7 × 10³/μL. After treatment of sepsis, the pancytopenia persisted. No underlying cause was detected. Bone marrow biopsy showed a hypocellular marrow with dysplastic megakaryocytes. The patient’s family indicated that he had not been taking his testosterone therapy. Testosterone decanoate (250 mg) was administered intramuscularly daily for 3 days. His platelet count increased to 74 × 10³/μL. Maintenance therapy with testosterone once weekly for 3 weeks and then once every 3 weeks resulted in improved hematologic findings. Repeated bone marrow biopsy after 6 weeks showed normocellular marrow, with disappearance of the megakaryocytic dysplasia. The patient again discontinued his testosterone treatment, and the hematologic abnormalities recurred but were again corrected after supervised testosterone therapy.ConclusionThis case emphasizes the importance of androgen replacement therapy in patients with hypopituitarism, not only for sexual potency, bone strength, and quality of life but also for normal bone marrow function. (Endocr Pract. 2008;14:229-232)     

Pulmonary Atypical Carcinoid Tumor with Metastatic Involvement of the Pituitary Gland Causing Functional Hypopituitarism

ObjectiveTo report a unique case of a peripherally located pulmonary atypical carcinoid tumor with metastatic involvement of the pituitary, manifesting with evidence of functional hypopituitarism and compressive symptoms of dysarthria, bitemporal loss of vision, and ataxia.MethodsWe present a case report, including detailed laboratory, radiologic, and pathologic findings in a 50-year-old woman with a peripherally located pulmonary atypical carcinoid tumor and a lesion metastatic to the pituitary gland. The pertinent literature is also reviewed.ResultsA 50-year-old woman with a medical history of metastatic pulmonary atypical carcinoid tumor presented with symptoms of bitemporal hemianopia, ataxia, and dysarthria. Laboratory evaluation revealed functional hypopituitarism, and magnetic resonance imaging of the brain with use of gadolinium contrast demonstrated a pituitary lesion measuring 3.5 by 2.5 by 2.5 cm. Visual field testing revealed bitemporal superior quadrantanopia, consistent with a pituitary lesion of the chiasm. Computed tomographic angiography excluded the presence of an aneurysm of the internal carotid artery. Subsequently, the patient underwent subtotal transsphenoidal hypophysectomy. Findings on pathology examination were consistent with a lesion metastatic from the primary pulmonary atypical carcinoid tumor. The patient subsequently received monthly octreotide injections as well as chemotherapeutic regimens of capecitobine and temozolomide. At 18-month follow-up, disease had not recurred.ConclusionThis case of metastatic involvement of the pituitary gland from a peripherally located pulmonary atypical carcinoid tumor manifesting with evidence of functional hypopituitarism is highly uncommon. To our knowledge, such a case has not been previously reported. The presentation of an advanced pituitary metastatic lesion can be particularly dramatic, and the course can be potentially complicated. Therefore, heightened awareness of the possibility of a pituitary lesion metastatic from a pulmonary atypical carcinoid tumor is important. (Endocr Pract. 2007;13:291-295)     

High Prevalence of Nonalcoholic Fatty Liver Disease Among Adolescents and Young Adults With Hypopituitarism due to Growth Hormone Deficiency

ObjectiveTo investigate the prevalence of nonalcoholic fatty liver disease (NAFLD) in adolescents and young adults with hypopituitarism and to examine the associations of growth hormone (GH) deficiency with the occurrence of NAFLD.MethodsA cross-sectional study for the determination of NAFLD prevalence included 76 patients with childhood-onset hypopituitarism and 74 controls matched by age and body mass index (BMI). We investigated the prevalence of NAFLD in adolescent and young adult patients with hypopituitarism as well as the age- and BMI-matched controls. Among patients with hypopituitarism, anthropometric, clinical, and biochemical assessments using transient elastography and magnetic resonance imaging were performed. Logistic regression was used to identify the factors associated with NAFLD.ResultsThe adolescents and young adults with hypopituitarism exhibited higher prevalence of NAFLD than the age- and BMI-matched controls. Among patients with hypopituitarism, obesity and obesity-related metabolic derangements were significantly associated with liver steatosis and fibrosis, whereas lower insulin-like growth factor (IGF)-I standard deviation score (SDS) and IGF-I/IGF-binding protein 3 molar ratios were associated with steatosis. In regression analyses adjusted for BMI SDS, steatosis was found to be associated with a lower IGF-I SDS and IGF-I/IGF-binding protein 3 molar ratios, whereas liver fibrosis was found to be associated with a lower IGF-I SDS.ConclusionOur results suggest that GH deficiency contributes to the occurrence of NAFLD, along with obesity and obesity-related metabolic changes. Because NAFLD occurs early in patients with hypopituitarism, the surveillance, weight control, and timely replacement of deficit hormones, including GH, are essential.     

Diabetes Insipidus Associated with Hehmophagocytic Lymphohistiocytosis: First Case Report

ObjectiveTo report the first case of central diabetes insipidus associated with hemophagocytic lymphohistiocytosis.MethodsWe describe the clinical course of a 75-yearold woman who developed febrile illness 1 month after receiving the H1N1 influenza vaccination. Shortly thereafter, she developed central diabetes insipidus, which responded to treatment with intranasal desmopressin acetate. She was then hospitalized with another febrile illness and diagnosed with hemophagocytic lymphohistiocytosis.ResultsMagnetic resonance imaging of the pituitary gland was significant for plaquelike meningeal enhancement and thickening surrounding the infundibulum, which has been previously reported as a manifestation of histiocytosis.ConclusionWe believe this is the first case of central diabetes insipidus associated with hemophagocytic lymphohistiocytosis. (Endocr Pract. 2011;17:e118-e122)     

Substantial Shrinkage of Adenomas Cosecreting Growth Hormone and Prolactin with use of Cabergoline Therapy

ObjectiveTo present 2 cases of patients with acromegaly and severe hyperprolactinemia whose primary therapy with cabergoline resulted in hormonal normalization and a considerable reduction in the size of their somatotroph macroadenomas.MethodsWe summarize the clinical presentation and the pertinent laboratory findings in 2 patients with acromegaly, as well as their clinical response to the therapy with cabergoline. A review of the literature regarding the use of cabergoline in acromegaly is also presented.ResultsA 48-year-old man (case 1) and a 26-year-old woman (case 2) were found to have acromegaly associated with very high levels of serum prolactin (2,700 and 5,250 ng/mL, respectively). These patients received first-line therapy with cabergoline that resulted not only in clinical improvement and normalization of growth hormone, prolactin, and insulin-like growth factor-I levels but also in a substantial reduction in the size of their somatotroph macroadenomas. By 6 months after the patients began to take cabergoline, tumor shrinkage of 94% (in case 1) and of 70% (in case 2) was demonstrated by magnetic resonance imaging.ConclusionOur findings demonstrate that cabergoline should be considered for medical treatment of adenomas cosecreting growth hormone and prolactin, even in the presence of large tumors with appreciable suprasellar extension, because substantial tumor shrinkage is possible with this therapy. (Endocr Pract. 2007;13:396-402)     

Association of Dengue Hemorrhagic Fever With Multiple Risk Factors for Pituitary Apoplexy

ObjectiveTo describe pituitary apoplexy that developed during the course of dengue hemorrhagic fever.MethodsWe describe the clinical findings, laboratory test results, imaging findings, and clinical course of the study patients.ResultsPatient 1 was a 40-year-old man who developed clinical signs and symptoms of dengue, which was confirmed by serologic testing. He presented with thrombocytopenia and developed severe headache and vomiting. During hospitalization, acromegaly was suspected because of the characteristic disease phenotype. Magnetic resonance imaging confirmed the diagnosis of pituitary apoplexy. Subsequently, the biochemical diagnosis of acromegaly was confirmed, and the patient underwent transsphenoidal surgery. Histopathologic examination showed signs of recent bleeding. Patient 2 was a 38-year-old man with a macroprolactinoma, who had been treated with cabergoline for 10 weeks and had shown improvement on laboratory testing and imaging. The patient then presented with clinical symptoms of dengue (confirmed serologically) and thrombocytopenia. He developed bilateral hemianopsia, and magnetic resonance imaging showed enlargement of the pituitary adenoma with signs of intratumoral bleeding. The patient underwent transsphenoidal surgery, and histopathologic examination documented a pituitary adenoma diffusely infiltrated by blood cells.ConclusionsWe describe dengue as a probable novel condition for pituitary apoplexy because it may be associated with multiple risk factors for pituitary infarction or bleeding. Physicians should suspect pituitary apoplexy in patients with dengue hemorrhagic fever who develop a rapid onset of severe headache and vision defects, even in those without known pituitary adenomas. (Endocr Pract. 2012;18:e97-e101)     

Xanthoma Disseminatum: Case Report and Literature Review

ObjectiveTo report a case of a young normolipidemic woman with mucocutaneous xanthomas who developed neurogenic diabetes insipidus and hyperprolactinemia because of an inflammatory pituitary stalk lesion.MethodsThe clinical features, laboratory results, magnetic resonance imaging, and pathology findings are presented. In addition, the pertinent literature is reviewed.ResultsA 23-year-old woman presented with a 9-month history of polydipsia, polyuria, galactorrhea, secondary amenorrhea, and weight gain. Her previous medical history included chronic anemia and widespread mucocutaneous xanthomas. Laboratory tests showed hyperprolactinemia, normal electrolytes, and a normal lipid profile. The results of a water deprivation test were compatible with neurogenic diabetes insipidus, and cerebral magnetic resonance imaging showed pituitary stalk thickening. Histologic findings on a skin biopsy specimen supported the diagnosis of non-Langerhans histiocytosis. Treatment was initiated with cabergoline, nasally administered desmopressin, radio-frequency ablation of facial skin lesions, and surgical excision of other accessible lesions.ConclusionXanthoma disseminatum is a rare, benign proliferative disorder characterized by extensive cutaneous and mucous membrane xanthomas in normolipidemic patients. Central nervous system involvement is rare and usually occurs in the systemic variety. Pituitary stalk disease commonly causes hyperprolactinemia, diabetes insipidus, and various degrees of hypopituitarism. The natural history of xanthoma disseminatum usually is benign, but lesions in critical anatomic sites may result in morbidity and mortality. (Endocr Pract. 2010;16:1003-1006)     

Diagnosing the Unrecognized Systemic Absorption of Intra-Articular and Epidural Steroid Injections

ObjectiveTo present a case series of patients who were misdiagnosed with endocrine disorders because of failure to recognize the systemic absorption of intra-articular and epidural steroids and to discuss the utility of performing a urine screen to detect synthetic glucocorticoids.MethodsIn this case series, we describe the clinical, laboratory, and imaging findings of 3 patients referred to our clinic, each with a presumed endocrine disorder.ResultsPatient 1 was a 54-year-old woman with weakness, loss of appetite, and a hormonal profile suggestive of hypopituitarism. Patient 2 was a 49-year-old woman with chronic fatigue and history of physical abuse, whose history and test results were compatible with growth hormone deficiency secondary to head trauma. Patient 3 was a 46-year-old woman who was diagnosed with endogenous Cushing syndrome despite normal 24-hour urinary cortisol excretion. In each case, we subsequently elicited a history of intra-articular or epidural glucocorticoid injections, and a urine screen documented the presence of synthetic glucocorticoids. Systemic absorption of the injected steroids was thus determined to be the cause of the symptoms and abnormal laboratory findings in each case.ConclusionsThe potential for harm from intra-articular and epidural glucocorticoid administration is underrecognized by physicians, leading to expensive investigation, false diagnoses, and unnecessary treatment. A urine screen for synthetic glucocorticoids is a valuable adjunct towards appropriate diagnosis. (Endocr Pract. 2009;15:225-228)     

Pituitary Apoplexy Secondary to Thrombocytopenia Due to Dengue Hemorrhagic Fever: A Case Report and Review of the Literature

Objective:To present a case of pituitary apoplexy secondary to thrombocytopenia associated with dengue hemorrhagic fever (DHF).Methods:In this case study, we review the presentation, evaluation, diagnosis, and management of a case of pituitary apoplexy in the setting of DHF. We also searched the literature for cases of pituitary apoplexy associated with thrombocytopenia and review their clinical presentation, management, and outcome.Results:A 53-year-old man presented with fever, lethargy, and worsening headache. Routine investigations revealed thrombocytopenia secondary to dengue fever. He developed symptoms of a cavernous sinus lesion the next day. Urgent magnetic resonance imaging revealed pituitary apoplexy in a pituitary macroadenoma. A transsphenoidal surgery was done and histology was consistent with apoplexy in a prolactin/follicle-stimulating hormone macroadenoma. Subsequently, the patient developed permanent deficits of anterior pituitary hormones. We review 8 other cases of pituitary apoplexy associated with thrombocyto-penia reported in the literature.Conclusion:Thrombocytopenia due to various causes may be a predisposing factor for pituitary apoplexy in a patient with underlying pituitary disease. In view of the tendency for bleeding associated with thrombocytopenia, the risks of surgical intervention have to be carefully weighed against the potential benefits. Indications for surgery would include progressive alteration of consciousness, visual disturbances, and opthalmoplegia despite conservative management. Patients with underlying pituitary macroadenomas with optic chiasm compression have a worse prognosis, and the chances of recovery, even with early surgery, are limited.     

Pituitary Macroadenoma in a Patient with Poems Syndrome in Conjunction with Castleman Disease: First Report of a Case and Review of Related Literature

ObjectiveTo describe the first reported case of a patient with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) in conjunction with the endocrinologic manifestation of panhypopituitarism due to a large clinically nonfunctioning pituitary adenoma.MethodsWe present the clinical, laboratory, and radiologic details of the case and review the relevant updated literature.ResultsA 48-year-old man with hypopituitarism and progressive polyneuropathy presented to an outside hospital with confusion and diaphoresis. He also had diffuse lymphadenopathy, monoclonal gammopathy, and skin lesions consistent with a diagnosis of POEMS syndrome. Cytopathologic study of a lymph node showed findings consistent with Castleman disease. A large suprasellar mass was found to be the cause of the hypopituitarism.ConclusionPOEMS syndrome is a rare paraneoplastic condition, commonly associated with Castleman disease, that manifests with progressive distal polyneuropathy and a monoclonal plasma cell disorder, often accompanied by endocrinopathy, organomegaly, skin changes, sclerotic bone lesions, ascites, erythrocytosis, and thrombocytosis. Our current patient had all 5 classic features of POEMS syndrome along with some diagnostic elements of Castleman disease, sclerotic bone lesions, and thrombocytosis. To our knowledge, this is the first known reported case of a patient whose endocrinologic manifestation of POEMS syndrome was panhypopituitarism attributable to a large clinically nonfunctioning pituitary adenoma. (Endocr Pract. 2010;16:97-101)     

Endocrine Disturbances in Empty Sella Syndrome: Case Reports and Review of Literature

ObjectiveTo report 5 cases of empty sella syndrome (ESS) manifesting with various degrees of pituitary dysfunction.MethodsWe describe the initial manifestations in 5 patients with primary ESS and in previous cases of ESS reported in the English language literature.ResultsReview of our recent medical records identified 5 patients referred for evaluation of pituitary deficiencies in whom ESS was diagnosed. Glucocorticoid replacement was required in 3 patients, 2 of whom presented initially with symptoms of severe glucocorticoid deficiency. In each case, magnetic resonance imaging of the brain demonstrated an empty sella.ConclusionOur cases suggest that endocrine abnormalities are not rare as the initial manifestation of ESS and that, contrary to many studies in the literature, the endocrine abnormalities may be quite severe. (Endocr Pract. 2005;11:120-124)     

Hypothalamic Germinoma Masquerading as Superior Mesenteric Artery (Sma) Syndrome

ObjectiveTo report a case of superior mesenteric artery (SMA) syndrome secondary to hypothalamic germinoma.MethodsWe describe the clinical presentation, diagnostic work-up, management, and clinical course of a patient admitted with SMA syndrome who was subsequently found to have a hypothalamic germinoma.ResultsAn adolescent boy was admitted to the surgical ward with progressive weight loss over a 2 year period and postprandial vomiting. He was diagnosed with SMA syndrome based on evidence of proximal duodenal dilatation, extrinsic compression of the distal duodenum, and a narrowed aortomesenteric angle (16°). Investigations performed to exclude thyrotoxicosis unexpectedly revealed secondary hypothyroidism and further evaluation demonstrated evidence of pan-hypopituitarism. Psychiatric evaluation excluded anorexia nervosa and bulimia. Magnetic resonance imaging (MRI) of the brain revealed a heterogeneously enhancing hypothalamic lesion, but a normal pituitary gland. Hormone replacement with hydrocortisone, desmopressin, testosterone, and thyroxine resulted in weight gain and resolution of gastrointestinal symptoms. A transventricular endoscopic biopsy subsequently confirmed a hypothalamic germinoma and he was referred to an oncologist.ConclusionSMA syndrome secondary to severe weight loss is an uncommon cause of upper gastrointestinal obstruction. While there have been reports of poorly controlled diabetes mellitus and thyrotoxicosis manifesting as SMA syndrome, there are no published reports to date of SMA syndrome secondary to hypothalamic/pituitary disease. Management of SMA syndrome is conservative, as symptoms of intestinal obstruction resolve with weight gain following treatment of the underlying cause. Awareness of this uncommon presentation of endocrine cachexia/hypothalamic disease will prevent unnecessary laparotomies and a misdiagnosis of an eating disorder. (Endocr Pract. 2013;19:e29-e34)     

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)     

A Case of Gestational Thyrotoxicosis Associated With Wernicke’s Encephalopathy

ObjectiveTo present a case of gestational thyrotoxicosis and hyperemesis gravidarum associated with Wernicke’s encephalopathy.MethodsWe present a detailed case report with the clinical, imaging, and laboratory findings of the patient and review the pertinent literature.ResultsA 36-year-old woman at 14 weeks of gestation was admitted to the hospital for management of severe hyperemesis gravidarum (HG). While hospitalized, she developed low-grade fever, tachycardia, hypotension, and altered mentation. Laboratory tests were diagnostic of hyperthyroidism. Physical examination revealed a confused, lethargic woman with a normal-size thyroid and pendular nystagmus in primary and lateral gaze. She was treated empirically for thyroid storm with methimazole and other measures. A brain magnetic resonance imaging (MRI) study done later showed hyperintense abnormal signals in bilateral thalamic regions, consistent with Wernicke’s encephalopathy (WE). She was immediately started on intravenous thiamine and her mental status improved considerably within 3 to 4 days. Within 2 weeks, the patient’s thyroid-function tests normalized and methimazole was discontinued. A repeat brain MRI 6 months later showed marked reduction of signal intensity in both thalamic regions.ConclusionThis case demonstrates that gestational thyrotoxicosis in a patient with HG can precipitate acute WE, which may mimic thyroid storm and thus delay appropriate management of this neurologic disorder. We conclude that prophylactic thiamine administration may be considered before caloric replacement in patients who present with HG and acute neurologic dysfunction. (Endocr Pract. 2014;20:e237-e240)     

A Case of Riedel’s Thyroiditis Treated with Tamoxifen: Another Successful Outcome

ObjectiveTo report a case of Riedel’s thyroiditis, which was successfully treated with tamoxifen.MethodsWe present the clinical, laboratory, and imaging findings and describe the clinical course of a patient with Riedel’s thyroiditis.ResultsA 40-year-old woman presented with hypothyroidism and a large goiter, which was unresponsive to hormone replacement therapy. Magnetic resonance imaging confirmed the presence of an enlarged thyroid gland, more pronounced on the right than on the left. The patient had progressive discomfort attributable to compressive symptoms in the neck. Surgical exploration of the neck disclosed a hard, immobile thyroid mass, which could not be resected because of adherence to surrounding structures. Biopsy of the thyroid and of the muscles of the neck revealed Riedel’s thyroiditis. Treatment with tamoxifen, in a dosage of 20 mg twice a day for more than 1½ years, completely resolved the neck mass (substantiated by follow-up magnetic resonance imaging) and relieved the signs and symptoms of compression of the neck.ConclusionTamoxifen treatment is effective in resolving the mass and compression in Riedel’s thyroiditis. (Endocr Pract. 2004;10:483-486)     

Thyroid: an Unusual Hideout for Sarcoidosis

ObjectiveTo present a case of dysphagia secondary to a progressively increasing nontoxic multinodular goiter caused by sarcoidosis.MethodsWe summarize the clinical presentation and pertinent pathology in a patient with sarcoidosis involving the thyroid gland. A review of literature regarding this topic is also presented.ResultsA 54-year-old man was noted to have asymptomatic nontoxic thyromegaly. Biopsy of right thyroid nodule was benign while the biopsy from the isthmus nodule was nondiagnostic. He presented with acute onset of dysphagia two months later and the work-up for gastrointestinal causes was negative. Chest imaging showed left-sided lymphadenopathy, and biopsy of a lymph node showed sarcoidosis. Two years after the initial presentation a repeat biopsy of the isthmus nodule was again reported as nondiagnostic. Because he had persistent dysphagia, he underwent total thyroidectomy with resolution of dysphagia. Histopathological examination of the thyroid revealed non necrotizing granulomas consistent with sarcoidosis.ConclusionThis case brings to light this uncommon etiology of a nontoxic multinodular goiter. Involvement of the thyroid gland by sarcoidosis is very rare. It has been reported in 4.2 to 4.6% of patients with sarcoidosis. In patients with pulmonary or extrapulmonary sarcoidosis and associated thyromegaly, possible involvement of the thyroid by this process should be considered. (Endocr Pract. 2013;19:e40-e43)     

Association of Low Prostate-Specific Antigen Concentration and Panhypopituitarism

ObjectiveTo report the association of undetectable or very low prostate-specific antigen (PSA) concentrations with hypopituitarism.MethodsWe present a case series of 4 patients with low or undetectable PSA concentrations and associated panhypopituitarism and summarize the clinical presentation and pertinent laboratory and radiologic findings. We review related literature and discuss possible mechanisms explaining the described association.ResultsFour men with PSA values below the second percentile of a healthy population had hypopituitarism. In 3 men, low PSA concentrations were noted before large pituitary tumors were diagnosed. No man had headaches, visual problems, or other symptoms that were severe enough to prompt a search for tumor. All 4 patients had undetectable total testosterone levels. Luteinizing hormone and follicle-stimulating hormone levels were low to low-normal in 3 of the 4 patients. Baseline growth hormone level was low in 1 patient and undetectable in the other 3. The insulinlike growth factor 1 concentration was less than 73 ng/mL in each patient.ConclusionsWe believe that the association of low PSA concentrations and hypopituitarism is not incidental and that the extremely low PSA values in this case series were a consequence of both gonadal and growth hormone deficiency. We suggest that low PSA is a marker of combined profound testosterone and growth hormone deficiency in men with panhypopituitarism. This marker is important because PSA is frequently measured during routine health care visits; a low concentration may be the first clue to the presence of hypopituitarism in an otherwise asymptomatic patient. (Endocr Pract. 2008;14:432-436)     

American Association of Clinical Endocrinologists and American College of Endocrinology Disease State Clinical Review: A Neuroendocrine Approach to Patients With Traumatic Brain Injury

Objective: Traumatic brain injury (TBI) is now recognized as a major public health concern in the United States and is associated with substantial morbidity and mortality in both children and adults. Several lines of evidence indicate that TBI-induced hypopituitarism is not infrequent in TBI survivors and may contribute to the burden of illness in this population. The goal of this article is to review the published data and propose an approach for the neuroendocrine evaluation and management of these patients.Methods: To identify pertinent articles, electronic literature searches were conducted using the following keywords: “traumatic brain injury,” “pituitary,” “hypopituitarism,” “growth hormone deficiency,” “hypogonadism,” “hypoadrenalism,” and “hypothyroidism.” Relevant articles were identified and considered for inclusion in the present article.Results: TBI-induced hypopituitarism appears to be more common in patients with severe TBI. However, patients with mild TBI or those with repeated, sports-, or blast-related TBI are also at risk for hypopituitarism. Deficiencies of growth hormone and gonadotropins appear to be most common and have been associated with increased morbidity in this population. A systematic approach is advised in order to establish the presence of pituitary hormone deficiencies and implement appropriate replacement therapies.Conclusion: The presence of traumatic hypopituitarism should be considered during the acute phase as well as during the rehabilitation phase of patients with TBI. All patients with moderate to severe TBI require evaluation of pituitary function. In addition, symptomatic patients with mild TBI and impaired quality of life are at risk for hypopituitarism and should be offered neuroendocrine testing.Abbreviations: CBG = corticosteroid-binding globulin DI = diabetes insipidus GH = growth hormone IGF-1 = insulin-like growth factor 1 SIADH = syndrome of inappropriate antidiuretic hormone T₄ = thyroxine TBI = traumatic brain injury TSH = thyroid-stimulating hormone     

Ectopic Acromegaly due to A Pancreatic Neuroendocrine Tumor Producing Growth Hormone-Releasing Hormone

ObjectiveTo present a case of acromegaly due to ectopic growth hormone-releasing hormone (GHRH) secretion from a pancreatic neuroendocrine tumor in the context of multiple endocrine neoplasia type 1 (MEN 1).MethodsWe describe the clinical, imaging, and pathologic findings of the study patient.ResultsA 46 year old woman presented with clinical and biochemical findings diagnostic of acromegaly. Magnetic resonance imaging showed a 1.2-cm sellar mass. Following resection of the macroadenoma, serum insulinlike growth factor 1 (IGF-1) and growth hormone (GH) levels remained unchanged. Pathologic examination revealed adenomatous changes, including a nonsecretory focus and a prolactin immunopositive area (GH stain negative in both). Octreotide long-acting release was ineffective. Search for an ectopic tumor included normal octreoscan and abdominal computed tomography. GHRH was greater than 1000 pg/mL. Repeated abdominal computed tomography documented a 6.2-cm mass in the tail and body of the pancreas. Distal pancreatectomy revealed a pancreatic neuroendocrine tumor that stained positive for GHRH. Postoperatively, serum GHRH and IGF-1 normalized. Re-evaluation of the initial pituitary pathologic specimen revealed additional somatotroph hyperplasia of the adjacent, normal pituitary gland. Primary hyperparathyroidism was diagnosed, and multigland parathyroid hyperplasia was noted at surgery. Genetic testing was positive for a mutation in the MEN1 gene.ConclusionThis patient’s acromegaly was resistant to somatostatin analogue therapy, reflecting the negative octreoscan imaging. In addition, this case is novel because the patient presented with pituitary adenomatous changes, which were presumably associated with MEN 1 and/or possibly the elevated GHRH levels. (Endocr Pract. 2011; 17:79-84)