Precocious Puberty Due to Rathke Cleft Cyst in a Child

ObjectiveTo report a case of a child with precocious puberty attributable to Rathke cleft cyst (RCC).MethodsThe clinical features, laboratory results, and findings on ultrasonography of the pelvis and magnetic resonance imaging of the pituitary gland are presented.ResultsA 16-month-old child had breast enlargement, height increase, and an increase in growth velocity. On examination, she was found to have Tanner stage 3 breast development, and her vaginal mucosa was estrogenized. Her height was above the 97th percentile. Biochemically, she was diagnosed as having central precocious puberty, and magnetic resonance imaging of her pituitary gland disclosed RCC. Treatment with leuprolide resulted in normalization of her growth rate and regression of the breast development; the vaginal mucosa also became unestrogenized.ConclusionAlthough RCC is a relatively common finding, it is a rare cause of precocious puberty. Magnetic resonance imaging of the pituitary gland should be performed in all children younger than 6 years of age who have precocious puberty, in an effort to detect any organic lesions. (Endocr Pract. 2009;15:134-137)     

A Rare Case of Central Precocious Puberty Due to Hypothalamic Hamartoma Diagnosed In Utero

ObjectiveTo report a rare case of central precocious puberty attributable to hypothalamic hamartoma that was diagnosed in utero.MethodsWe present the clinical, laboratory, and imaging data pertaining to our case and discuss the diagnostic features and recommended treatment of central precocious puberty in patients with hypothalamic hamartoma.ResultsA 3-month-old male child had had excessively rapid growth velocity and weight gain since birth. On investigation, the patient was diagnosed as having hypothalamic hamartoma with central precocious puberty. On inquiry, his mother described a history of prenatal ultrasonography and fetal magnetic resonance imaging suggesting the presence of a cystic lesion in his brain at 9 months of gestation. Because of continued rapid growth and acceleration of puberty during a 4-month observation period, we decided to treat the patient with leuprolide acetate. The patient responded well to treatment, with stabilization of growth.ConclusionTo the best of our knowledge, this patient is the youngest in the medical literature diagnosed to have central precocious puberty and also to receive treatment with leuprolide acetate. (Endocr Pract. 2010;16:237-240)     

Precocious Puberty and Large Multicystic Ovaries in Young Girls with Primary Hypothyroidism

ObjectiveTo describe 2 cases of primary hypothy-roidism, precocious puberty, large multicystic ovaries, possible diagnostic dilemma, unilateral oophorectomies, and subsequent response to levothyroxine replacement therapy.MethodsWe present the clinical, biochemical, radiologic, and histopathologic findings in 2 patients with rare cases of Van Wyk-Grumbach syndrome and megaovaries, who underwent unilateral oophorectomy.ResultsTwo patients, an 8-year-old girl and a 3-year-old girl (cases 1 and 2, respectively), were referred to our center. Both patients presented with precocious puberty and vaginal bleeding and had undergone unilateral oophorectomy before referral. In the first patient (case 1), the surgical intervention was a consequence of torsion of the left megaovary, necessitating emergency oophorecto-my. Oophorectomy in the second patient (case 2) was a result of initial diagnostic confusion, inasmuch as a sex-cord stromal tumor was suspected. A detailed history, physical examination, and laboratory results pointed toward primary hypothyroidism due to Hashimoto’s thy-roiditis and thyroid dysgenesis, respectively. Serial ultra-sound studies of the abdomen and pelvis revealed large multicystic ovaries, with progressive enlargement (includ-ing regrowth from an apparent ovarian “postsurgical remnant”). Both patients responded dramatically after initiation of levothyroxine replacement therapy, with no further vaginal bleeding and reversal of megaovary to normal size (in case 1).ConclusionIn a highly selected minority of children with untreated primary hypothyroidism, there is development of precocious puberty and progressively enlarging multicystic ovaries. The precise endocrine, neuroanatomic, and neurophysiologic bases for this phenomenon are unclear. Nevertheless, the entire clinicopathologic picture,including giant ovaries, dramatically reverts to normal status with the restoration of a euthyroid state by means of simple levothyroxine replacement therapy. (Endocr Pract. 2007;13:652-655)     

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)     

Pheochromocytoma and Tetralogy of Fallot: A Rare but Potentially Dangerous Combination

ObjectiveTo describe a case of pheochromocytoma (PHEO) with tetralogy of Fallot (TOF) and discuss the difficulties encountered during the management of this patient, with a review of the literature.MethodsWe report the clinical course, imaging, and management issues of our patient and review relevant literature.ResultsA 14-year-old female who was known to have TOF presented with classical paroxysmal symptoms and worsening dyspnea. She was diagnosed as having epinephrine-secreting PHEO based on biochemical, radiologic, and functional imaging. She was treated with an α-1 blocker for control of paroxysms but developed severe cyanotic spells. She required addition of a calcium-channel blocker for control of the paroxysms and underwent successful cardiac repair.ConclusionTreatment of the combination of cyanotic congenital heart disease (CCHD) and PHEO requires an individualized and multidisciplinary approach with judicious use of available medications. This is the first case of uncorrected TOF and epinephrine-secreting PHEO. Our case also reiterates the need for further studies to better understand the pathophysiologic link between PHEO/paraganglioma and CCHD. (Endocr Pract. 2014; 20:e80-e85)     

Bilateral Ovary Adrenal Rest Tumor in a Congenital Adrenal Hyperplasia Following Adrenalectomy

Objective:In contrast to the high incidence of testicular adrenal rest tumors in adult male patients with congenital adrenal hyperplasia (CAH), ovarian adrenal rest tumors (OARTs) in female CAH patients are rare. In this case report, we describe a case of bilateral OART in a female patient with CAH due to 21-hydroxylase deficiency.Methods:We present a detailed case report with the clinical, imaging, and laboratory findings of the patient. The pertinent literature is also reviewed.Results:A 17-year-old patient was known to have CAH due to 21-hydroxylase deficiency. Since the second month of her gestational age, her mother was treated with cortisone-replacement therapy. The patient was treated with hydrocortisone and fludrocortisone since the neonatal period. Her pertinent history included a bilateral adrenalectomy at the age of 13 years in 2006, and for 3 years she led a normal puberty life with no complaint with hormonal replacement therapy. Nevertheless, in 2009, she developed a virilizing syndrome. Subsequently, she underwent surgery in December 2009 for right adnexectomy. However, the regression of the masculinizing mass was not complete and worsened several months after the surgery. A new pelvic magnetic resonance image showed the activation of a contralateral ovarian mass, necessitating a left adnexectomy in August 2010.Conclusion:This case demonstrates some interesting features of OART that pose challenges to its management. If an OART is detected early enough and glucocorticoid therapy is received, it is possible that the OART will decrease in size following suppression of adrenocorticotropic hormone levels. (Endocr Pract. 2014;20:e69-e74)     

Bird's-Eye View of GnRH Analog use in a Pediatric Endocrinology Referral Center

Objective: Gonadotropin-releasing hormone analogs (GnRHa) are standard of care for the treatment of central precocious puberty (CPP). GnRHa have also been prescribed in other clinical settings with the hope of increasing adult stature, although evidence to support this practice is lacking. The degree to which GnRHa are being prescribed for indications other than CPP in routine clinical care has not been described. We sought to systematically examine GnRHa prescribing practices among the pediatric endocrinologists at our academic medical center.Methods: We reviewed medical records of children treated with GnRHa during a 6-year interval. Variables analyzed included gender, age at start of treatment, indication for therapy, and use of growth hormone as adjunctive treatment. Nonparametric analyses were utilized to compare treatment characteristics of those with CPP versus those without.Results: A total of 260 patients (82% female) aged 8.06 ± 2.68 years were identified. Of these, 191 (73.5%) were treated for CPP, whereas 69 (26.5%) were treated for normally timed puberty in the context of idiopathic short stature/poor predicted height (n = 37), growth hormone deficiency (n = 17), congenital adrenal hyperplasia (n = 10), primary hypothyroidism (n = 4), and developmental delay (n = 1). Of the 161 girls with CPP, GnRHa therapy was initiated at =8 years of age in 62 (39%).Conclusion: Whereas most patients were treated for CPP, ~27% were treated for other indications. Of girls with CPP, 39% were treated at an age when benefit in terms of height is unlikely. This highlights the need for rigorous studies of GnRHa use for indications beyond CPP.Abbreviations: CPP = central precocious puberty GnRHa = gonadotropin-releasing hormone analogs     

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)     

Hyperthyroidism due to Coexistence of Graves’ Disease and Struma Ovarii

ObjectiveTo report an unusual case of persistent thyrotoxicosis after treatment of Graves’ disease, because of coexistence of struma ovarii.MethodsWe report the clinical history, imaging studies, laboratory and pathologic data, and treatment in a patient with persistent hyperthyroidism after surgical treatment of Graves’ disease. In addition, we discuss some aspects of the pathogenesis of hyperthyroidism due to functioning struma ovarii.ResultsA 42-year-old woman underwent near-total thyroidectomy for treatment of Graves’ disease. Post-operatively, hyperthyroidism was still present. Methimazole was administered again, and performance of a ¹³¹I whole-body scan demonstrated a focus of intense uptake in the pelvis. Pelvic ultrasonography revealed a mass (11 by 8 by 7.1 cm) arising from the right ovary, with both solid and cystic components. Abdominal surgical exploration was performed, and the final histologic diagnosis was struma ovarii. The symptoms of hyperthyroidism diminished, and 3 weeks postoperatively, the thyroid hormone levels were in the hypothyroid range.ConclusionIn patients with refractory hyperthyroidism after thyroid surgical treatment, radioiodine scanning should be performed to diagnose or exclude the functioning profile of ovarian masses. (Endocr Pract. 2007;13:274-276)     

Granulomatous Adenohypophysitis after Interferon and Ribavirin Therapy

ObjectiveTo describe a case of granulomatous hypophysitis occurring after treatment with interferon alfa-2b and ribavirin for hepatitis C.MethodsClinical, radiologic, laboratory, and pathologic assessments of a woman with granulomatous hypophysitis and interferon-induced thyroiditis are presented.ResultsA 42-year-old woman with hepatitis C was treated with interferon alfa-2b and ribavirin for 5 months. She was referred after symptoms of thyrotoxicosis developed, in conjunction with laboratory and radiographic evidence of thyroiditis. During the initial evaluation, she was weak and hypotensive; biochemical evaluation showed undetectable plasma cortisol and corticotropin concentrations. Magnetic resonance imaging revealed diffuse enlargement of the pituitary gland, which encroached on but did not compress the optic chiasm. Treatment with supraphysiologic doses of prednisone resulted in clinical and radiographic improvement. Once physiologic doses of glucocorticoids were instituted, however, follow-up magnetic resonance imaging showed substantial progression of the diffuse pituitary enlargement and mild compression of the optic chiasm. Surgical debulking of the mass and histologic evaluation showed chronic, noncaseating gran-ulomatous hypophysitis. An extensive evaluation for secondary causes of granulomatous inflammation of the pituitary revealed only an elevated angiotensin-converting enzyme level; no organisms were identified. After 2 courses of high-dose glucocorticoids, she had radiographic evidence of decreased size of the pituitary lesion but continued to have multiple anterior pituitary hormone deficiencies.ConclusionGranulomatous hypophysitis and sarcoidosis of the pituitary are rare disorders. Hypophysitis should be considered in patients receiving interferon and ribavirin therapy who have symptoms consistent with pituitary dysfunction. (Endocr Pract. 2007;13:169-175)     

Osteomesopyknosis: A Case Report and Review Of Sclerosing Bone Disorders

ObjectiveWe present a case of osteomesopyknosis, a nonmalignant sclerosing bone dysplasia of the axial skeleton, with unknown etiology and unknown prevalence.MethodsWe studied a 49-year-old female who suffered from back and pelvic pain. Her history was obtained, a physical examination performed, and the laboratory results and imaging diagnostics were studied to describe her disease.ResultsA 49-year-old, perimenopausal female suffered excruciating, intermittent, dull back and pelvic pain for 1.5 years. Nonmalignant blastic bone lesions along her spine and pelvis were discovered on computed tomography (CT) and confirmed by magnetic resonance imaging (MRI) and bone scans. Other metabolic/endocrine conditions were ruled out. Her son also has similar symptoms, and corresponding changes were observed on his vertebrae MRI. Both were diagnosed with osteomesopyknosis.ConclusionOsteomesopyknosis is a rare, autosomal dominant condition characterized by nonmalignant osteosclerosis restricted to the axial skeleton. The disease may produce chronic low-grade back pain in the thoracic and lumbar regions. (Endocr Pract. 2014;20:e106-e111)     

Severe Hyponatre mia Due to Rosiglita zone Use in an Elderly Woman With Diabetes Mellitus: A Rare Cause of Syndrome of Inappropriate Antidiuretic Hormone Secretion

ObjectiveTo describe the first case of syndrome of inappropriate antidiuretic hormone secretion with lifethreatening hyponatremia due to rosiglitazone therapy.MethodsWe describe the clinical, laboratory, and imaging findings of the study patient.ResultsAn 89-year-old woman with a 5-year history of type 2 diabetes mellitus was admitted to the emergency department because of unconsciousness. She had reported generalized weakness for 15 days and nausea and vomiting for 3 days. Findings from laboratory analysis showed severe hyponatremia (sodium, 110 mEq/L). She had normal renal, cardiac, and adrenal function, and she did not have edema or volume depletion. The cause of hyponatremia was syndrome of inappropriate antidiuretic hormone secretion. We did not find any cause for her condition other than rosiglitazone, an antihyperglycemic drug that is increasingly being used in patients with type 2 diabetes mellitus. According to her medical history, rosiglitazone was prescribed 1 month previously after withdrawal of gliclazide. We stopped the rosiglitazone and administered hypertonic saline infusion to treat the hyponatremia. Saline infusion was stopped and blood sodium levels were stabilized in the normal range after 2 days. The patient’s plasma sodium concentration has remained in the reference range at follow-up visits.ConclusionsThis is the first reported case of syndrome of inappropriate antidiuretic hormone secretion as an adverse effect of rosiglitazone, and this drug should possibly be considered for addition to the list of drugs that cause this condition. (Endocr Pract. 2008;14:1017-1019)     

An Unusual Variant of Cushing Syndrome

ObjectiveTo discuss the initial clinical manifestations of primary pigmented nodular adrenocortical disease.MethodsWe present a case report of a 4-year-old boy who had the classic clinical features of Cushing syndrome. Results of hormonal investigations are reviewed, and histopathologic findings are illustrated.ResultsInvestigations revealed adrenocorticotropic hormone (corticotropin)-independent Cushing syndrome. Findings on magnetic resonance imaging of the pituitary gland and abdomen were within normal limits. The patient underwent bilateral adrenalectomy. The histopathologic features were consistent with primary pigmented nodular adrenocortical disease.ConclusionPrimary pigmented nodular adrenocortical disease should be suspected in patients with corticotropin- independent Cushing syndrome who have normal findings on adrenal imaging. (Endocr Pract. 2008;14:717-720)     

Case report of acute necrotizing pancreatitis associated with combination treatment of sitagliptin and exenatide

ObjectiveTo report the first postmarketing case of necrotizing pancreatitis in a patient on combination therapy of sitagliptin and exenatide.MethodsWe describe the patient’s clinical presentation, laboratory test results, imaging, and autopsy findings.ResultsA 76-year-old woman with a history of type 2 diabetes mellitus presented with severe abdominal pain, vomiting, and fever requiring hospital admission. She had been treated with exenatide for 3 years to manage her diabetes mellitus. A few weeks before presentation, sitagliptin was added, presumably to further optimize her glycemic control. Acute pancreatitis was diagnosed during hospital admission. At initial presentation, her serum amylase concentration was 1136 U/L (reference range, 10-130 U/L) and her lipase concentration was greater than 3500 U/L (reference range, 0-75 U/L). In addition, computed tomography of the abdomen and pelvis demonstrated extensive pancreatic parenchymal necrosis. She had undergone previous cholecystectomy, reported no alcohol consumption, and had a normal lipid profile. Although she had a long-standing history of diabetes mellitus, she had no history of pancreatitis or other risk factors that would have caused her to develop the underlying condition. After initial brief improvement, her symptoms worsened, and despite aggressive care, her clinical state deteriorated and she died. Autopsy findings demonstrated acute necrotizing pancreatitis with complete digestion of the pancreas.ConclusionsConsidering the temporal relationship of her symptoms to the addition of sitagliptin to her existing exenatide regimen, this case strongly suggests a possible causal link between exenatide or sitagliptin (or the combination of the 2 drugs) and the etiology of pancreatitis in this patient. (Endocr Pract. 2012;18:e10-e13)     

Transformation of a Pituitary Macroadenoma into to a Corticotropin-Secreting Carcinoma Over 16 Years

ObjectiveTo describe a case of a pituitary macroadenoma that differentiated into a corticotropin (ACTH)-secreting carcinoma with metastasis to the thigh.MethodsWe present a case report with a 16-year follow-up that includes anatomic and endocrine documentation of the history of an ACTH-secreting carcinoma.ResultsA 32-year-old woman presented for evaluation in 1989 because of visual feld defects. Magnetic resonance imaging revealed a locally invasive 3-cm macroadenoma. She had no clinical signs of cortisol excess. The patient underwent surgical debulking followed by a course of radiation directed to the pituitary. Results from retrospective immunohistochemical staining with antibodies against ACTH, prolactin, and MIB-1 were negative. Postoperatively, she could not be weaned from exogenous steroids without developing symptoms of adrenal insuffciency. In 1995, she developed left facial palsy and diplopia caused by tumor growth. In 1997, the patient developed progressive symptoms of cortisol excess, which continued after exogenous steroid replacement was discontinued. The patient’s clinical status continued to deteriorate because of local mass effect from tumor growth and uncontrolled hypercortisolism. She underwent bilateral adrenalectomy in 2003. The patient remained debilitated in a long-term care facility for 2 years when she was found to have a mass on her left hip. Biopsy results of the obturator muscle revealed metastatic tumor of neuroendocrine origin with strong reactivity to ACTH antibodies and MIB-1 labeling in 8% of tumor cell nuclei.ConclusionA pituitary tumor can transform into an ACTH-secreting carcinoma in an indolent manner. Patients with invasive pituitary adenomas require long-term surveillance to monitor for differentiation into pituitary carcinoma. (Endocr Pract. 2007;13:463-471)     

Slipped Capital Femoral Epiphysis In a Patient With Turner Syndrome Receiving Growth Hormone Therapy

ObjectiveTo report a case of slipped capital femoral epiphysis in a young patient with Turner syndrome (TS) receiving growth hormone therapy and to emphasize the importance of keeping this orthopedic condition in mind during management of this patient group.MethodsClinical, laboratory, and radiographic findings are presented, and risk factors for slipped capital femoral epiphysis are discussed.ResultsA child with TS presented for medical assessment because of a limp but with no history of trauma or febrile illness. Growth hormone therapy had been administered for 1 year because of her short stature. Physical examination and pelvic radiography of the patient showed the presence of bilateral slipped capital femoral epiphysis. She underwent bilateral pinning in situ, and growth hormone therapy was terminated. At follow-up after more than 2 years, no sequelae were noted.ConclusionPatients with TS are at high risk for developing certain orthopedic conditions, such as slipped capital femoral epiphysis. Furthermore, slipped capital femoral epiphysis is a known complication of growth hormone therapy in growing children. A limp, hip pain, knee pain, or thigh pain might be a symptom of slipped capital femoral epiphysis in patients with TS, especially those receiving growth hormone therapy. Prompt recognition and treatment of this condition are important for prevention of sequelae. (Endocr Pract. 2012;18:e135-e137)     

Risks and Benefits of Estrogen Therapy for a Male-to-Female Transsexual with a Prothrombin Gene Mutation

ObjectiveWe present the case of a male-to-female transsexual person in her 20s requesting hormone therapy in the setting of a history of a deep venous thrombosis and pulmonary embolus and carrying the prothrombin G20210A gene mutation.MethodsWe interviewed the patient and reviewed her medical records. We carefully weighed the risks and benefits of hormone therapy and took into account two important ethical principles: beneficence (to act in the patient’s best interest) and nonmaleficence (to avoid harm).ResultsOur patient presented to an outside facility with weight loss, generalized weakness, right lower extremity swelling, and chest pain. She was diagnosed with a pulmonary embolus and extensive deep venous thrombus by computed tomography (CT) scan and Doppler ultrasound, respectively. She was found to carry the pro-thrombin G20210A gene mutation. She was treated with anticoagulation therapy for 12 months, which was restarted prior to beginning therapy with transdermal estrogen.ConclusionWhile the exact risk of recurrent deep venous thrombosis and pulmonary embolus in our patient is unknown, we recommended that hormone therapy should only be given in conjunction with anticoagulation. We speculate that this strategy would allow the patient to experience the benefits to her overall well-being with hormone therapy while reducing the risks of venous thrombosis to acceptable levels. Prospective long-term follow-up of this patient is needed to verify the benefits and risk of the intervention chosen. (Endocr. Pract. 2013;19:e150-e153)     

Hürthle cell Thyroid Carcinoma Presenting as A “Hot” Nodule

ObjectiveTo report an unusual clinical scenario and a rare histopathologic finding of Hürthle cell thyroid carcinoma in a patient with an autonomous thyroid nodule.MethodsWe describe the presentation and clinical course leading to the surprising histopathologic diagnosis of Hürthle cell carcinoma in a pediatric patient who was diagnosed with hyperthyroidism presenting as a solitary toxic nodule.ResultsA 13-year-old white girl presented with a recent history of a palpable thyroid nodule during a routine primary care clinic visit. She was asymptomatic, and thyroid function tests revealed a suppressed thyrotropin concentration, high-normal free thyroxine concentration, and elevated triiodothyronine concentration. The patient underwent dedicated thyroid ultrasonography revealing a 3.5-cm complex mass in the left lobe with increased central vascularity. Iodine 123 imaging of the thyroid demonstrated homogenous, hyperintense activity in the left lobe. The right lobe was not visualized. A solitary toxic nodule was diagnosed, and, considering her age, she was referred for surgical management. The patient underwent a left lobectomy with isthmusectomy. Pathologic examination revealed a 5-cm, encapsulated, well-differentiated Hürthle cell carcinoma with negative margins and no lymphovascular invasion. The patient underwent subsequent completion thyroidectomy with no evidence of residual carcinoma in the right thyroid lobe.ConclusionsMalignancy in autonomously functioning thyroid nodules is rare. Most of the thyroid nodules presenting as “hot” on radioiodine scintigraphy are benign follicular adenomas. However, this case represents a rare clinical entity, and it highlights the need for clinicians to be vigilant and aware that occasionally carcinomas can masquerade as scintigraphic “hot” nodules. (Endocr Pract. 2011;17:e68-e72)     

A Case of Hypercalcemia Associated with Castleman Disease

ObjectiveTo present a case of hypercalcemia associated with Castleman disease.MethodsThe details of case presentation, evaluation, diagnosis, and treatment are reviewed in a 25-year-old woman with severe hypercalcemia, joint pain, conjunctival injection, and lymphadenopathy.ResultsEndocrinopathies such as primary hyperparathyroidism were ruled out. Infectious and rheumatologic laboratory evaluations revealed normal results. On a whole-body scan, the patient was noted to have diffusely increased osseous uptake in conjunction with increased periarticular uptake, consistent with a metabolic superscan. After extensive evaluation, the patient underwent a right axillary lymph node biopsy and was found to have multicentric Castleman disease. She was treated with high-dose corticosteroids and eventually immunomodulators to help control her disease.ConclusionTo the best of our knowledge, this is the first published case report demonstrating hypercalcemia in association with Castleman disease. The pathologic mechanism is likely bone turnover mediated by cytokines such as interleukin-6. (Endocr Pract. 2010;16:1007-1011)