Osteopetrosis and Congenital Hypothyroidism Complicated by Slipped Capital Femoral Epiphysis

ObjectiveTo describe a 13-year-old girl with unilateral slipped capital femoral epiphysis (SCFE), who presented with an acute onset limp during follow-up for congenital hypothyroidism and osteopetrosis.MethodsWe present a case report detailing the patient’s history as well as clinical, laboratory, and imaging findings and discuss the related literature.ResultsThe patient had been diagnosed elsewhere with congenital hypothyroidism, and levothyroxine therapy was initiated when she was 20 days of age; however, adherence to the treatment was irregular. Both her weight and her height were below the 5th percentile, her breast development and pubic hair were consistent with Tanner stage 1, and she had mental retardation and atypical facies. Her gait was antalgic; no muscle atrophy or shortness in the affected leg was present. On laboratory investigation, thyroid function tests were concordant with primary hypothyroidism. Her bone age was estimated as 8 years. Dual-energy x-ray absorptiometry revealed increased bone mineral density. Radiographic studies disclosed striking opacity of the bones of the pelvis and sclerosis at the skull base. Computed tomography of the affected left lower limb showed a fragmented appearance of the capital femoral epiphysis and thickening and irregularities of the physis line on the left, consistent with SCFE.ConclusionWe underscore the possible facilitator role of osteopetrosis in the pathogenesis of SCFE, suggest the need to consider SCFE in the differential diagnosis when a lower extremity abnormality is detected in patients with congenital hypothyroidism or delayed puberty (or both), and emphasize this association with osteopetrosis.(Endocr Pract. 2010;16:646-649)     

Thyrotropin-Secreting Adenoma in a Patient with Primary Hypothyroidism

ObjectiveTo describe a patient who developed a thyrotropin (TSH)-Secreting adenoma in the setting of primary hypothyroidism.MethodsWe report the clinical, laboratory, and radiologic findings of a patient with a history of primary hypothyroidism who presented with headache, a bitemporal visual field deficit, and elevated TSH despite long-term levothyroxine therapy. We discuss the diagnostic challenges of this case and review the relevant literature.ResultsA54-year-old woman with a history of primary hypothyroidism presented with a 3-year history of headache and a week of worsening vision. Imaging revealed a heterogeneous sellar mass elevating the optic chiasm. Her serum TSH was 46.5 mIU/L and free thyroxine concentration was 0.1 ng/dL. The differential diagnosis included pituitary hyperplasia and a TSH-secreting adenoma in a patient with primary hypothyroidism. The pathologic characteristics of the tumor were consistent with the latter.ConclusionIn a patient with an elevated TSH concentration and a previous diagnosis of hypothyroidism, it is important to consider other entities besides medication noncompliance. TSH-secreting adenomas can also cause elevated levels of TSH. (Endocr Pract. 2011;17: e135-e139)     

Chorea--an unusual manifestation in a woman recovering from myxedema coma

ObjectiveTo report a case of reversible chorea in a woman with myxedema coma.MethodsWe describe the clinical course, imaging findings, and laboratory test results of a patient who initially presented with myxedema coma and then developed reversible chorea upon treatment.ResultsA 33-year-old woman with a known history of primary hypothyroidism presented with a 3-week history of lethargy, progressing to a precipitous decline in consciousness that required intubation. Physical examination revealed concurrent hypothermia and bradycardia. Laboratory investigations demonstrated a thyrotropin concentration greater than 100 mIU/L, a free triiodothyronine concentration of 1.9 pg/mL, and a free thyroxine concentration of 0.24 ng/dL, but no other metabolic abnormalities. She was treated with intravenous levothyroxine therapy on the first 2 days of hospital admission (200 mcg and 250 mcg, respectively). On day 2, she was obeying commands and she was extubated. She began exhibiting choreiform movements. Thyroid function test results revealed a normal free thyroxine concentration (1.10 ng/dL), but an elevated thyrotropin concentration (40.98 mIU/L) and a low free triiodothyronine concentration (1.9 pg/mL). Findings from computed tomography and magnetic resonance imaging of her brain and analysis of cerebrospinal fluid were normal. Her regimen was transitioned to oral levothyroxine, 88 mcg daily, and by day 4, her choreiform movements ceased.ConclusionsNeurologic manifestations of hypothyroidism include psychomotor slowing, memory deficits, and dementia, with myxedema coma at the extreme of this spectrum. Although chorea is a rare manifestation of hyperthyroidism, this is the first report of a patient with acquired, reversible choreiform movement disorder while still being severely hypothyroid and treated with levothyroxine. (Endocr Pract. 2012;18:e43-e48)     

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)     

Levothyroxine Pseudomalabsorption and Thyroxine Absorption Testing with Use of High-Dose Levothyroxine: Case Report and Discussion

ObjectiveTo report the case of a 55-year-old woman who had been prescribed a daily dose of 1, 000 μg of levothyroxine for the treatment of hypothyroidism but still had severe biochemical hypothyroidism and to discuss the use of thyroxine absorption testing to diagnose pseudomalabsorption.MethodsThe patient was admitted to the hospital for supervised thyroxine absorption testing. Baseline thyroid function tests were performed. An oral dose of 1, 000 μg of levothyroxine was administered while the patient had an empty stomach, and thyroid function tests were repeated at 2, 4, and 6 hours after administration. She was also given all her prescribed antihypertensive medications, and the blood pressure (which had been persistently high) was measured every 2 hours.ResultsAfter administration of 1, 000 μg of levothyroxine, a rapid improvement in the results of her thyroid function tests was noted. Similarly, a rapid decrease in her blood pressure was observed after supervised administration of her antihypertensive medications. A diagnosis of nonadherence to treatment (pseudomalabsorption of levothyroxine) was made. After reduction of her levothyroxine dosage to 100 μg daily, results of thyroid function tests showed improvement. The doses of her antihypertensive medications were likewise altered.ConclusionWe suggest that patients who are receiving doses of levothyroxine of more than 2 μg/kg of body weight, with persistently increased thyroid-stimulating hormone levels, should undergo testing for malabsorption and pseudomalabsorption of levothyroxine. Thyroxine absorption testing with use of high-dose levothyroxine is useful in diagnosing pseudomalabsorption but needs formal evaluation and validation. (Endocr Pract. 2010;16:1012-1015)     

Plasma Cell Granuloma of The Thyroid: Report of Case and Review of Literature

ObjectiveTo report the 12th case of a patient with a plasma cell granuloma of the thyroid, a rare cause of goiter and hypothyroidism.MethodsWe present a case report of a woman with a plasma cell granuloma of the thyroid. The clinical and pathologic features of the lesion are described, the differential diagnosis is discussed, and the relevant literature is reviewed.ResultsPlasma cell granulomas are uncommon benign lesions most typically located in the lung and only rarely identified in other organs. Only 11 cases of plasma cell granuloma of the thyroid gland have been reported previously. We describe the case of a 55-year-old woman with a long history of hypothyroidism and compressive symptoms from an enlarging neck mass. A thyroidectomy was performed. On gross examination, the thyroid had been replaced by firm, white, fibrotic tissue with a multinodular appearance. On microscopy, the infiltrate consisted predominantly of plasma cells that were polyclonal with the expression of both kappa and lambda light chains. A minor component of CD5- and CD20-positive (T and B) lymphocytes was observed. These features were important for establishing the diagnosis of a plasma cell granuloma and distinguishing the lesion from a plasmacytoma.ConclusionThis is the 12th reported case of plasma cell granuloma of the thyroid gland, a very rare cause of either a diffuse or a nodular goiter and hypothyroidism. The presence of a polyclonal plasma cell population with the expression of both kappa and lambda light chains helps to distinguish a plasma cell granuloma from a malignant plasmacytoma of the thyroid gland. (Endocr Pract. 2008; 14:611-617)     

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)     

Kwashiorkor and an Acrodermatitis Enteropathica-like Eruption after a Distal Gastric Bypass Surgical Procedure

ObjectiveTo describe a case of kwashiorkor and an acrodermatitis enteropathica-like eruption associated with zinc deficiency after a distal gastric bypass surgical procedure.MethodsA case report of a morbidly obese patient who underwent a gastric bypass operation is presented, including clinical, laboratory, and radiologic findings. In addition, the literature on potential nutritional deficiencies after bariatric surgical intervention is reviewed.ResultsA 43-year-old woman with a history of morbid obesity underwent a distal Roux-en-Y gastric bypass procedure at an outside institution. Six months later, she presented to our clinic because of abdominal pain, lower extremity edema, and a patchy maculopapular scaling rash. She had not adhered to a vitamin supplementation regimen prescribed postoperatively. Her symptoms progressively worsened, and she was hospitalized for management of severe malnutrition and dehydration. Laboratory tests revealed low levels of albumin, hemoglobin, vitamin A, vitamin D, copper, and zinc and elevated levels of liver enzymes. Anasarca and bowel wall edema were seen on an abdominal computed tomographic scan, and an upper endoscopy revealed a stomal ulcer and a stricture at the site of the gastrojejunal anastomosis. The patient was diagnosed as having kwashiorkor, zinc deficiency, and an acrodermatitis enteropathica-like eruption. Treatment was begun with total parenteral nutrition, which led to alleviation of her symptoms. Approximately 3 months later, she underwent gastric bypass revision but had numerous postoperative complications.ConclusionKwashiorkor and severe nutritional deficiencies were noted in this patient after a distal gastric bypass surgical procedure. This clinical presentation is uncommon and can be attributed to the increased malabsorption that occurs with distal gastric bypass, the development of mechanical complications, and the inadequacy of nutritional supplementation. After a bariatric operation, careful adherence to follow-up regimens and the involvement of a multidisciplinary team can improve the chances of a successful outcome. (Endocr Pract. 2007;13:277-282)     

Down syndrome associated with hypothyroidism and chronic pericardial effusion: echocardiographic follow-up

We present a 39-year-old male patient with Down syndrome who was evaluated for fatigue, palpitations and bouts of cyanosis. Physical examination showed features of trisomy-21(Down syndrome), with a slow pulse rate, distant cardiac sounds and absent apex beat. He had normal jugular venous pressure without pulsus paradoxus. The ECG showed QRS microvoltage and flattened P and T segments. The 48-hour ambulatory ECG depicted normal sinus rhythm with intermittent short PR interval without tachyarrhythmias. The chest Xray revealed cardiomegaly without pulmonary venous congestion. Although serial transthoracic echocardiographic examination demonstrated pericardial effusion with features of tamponade, there were no overt signs of clinical cardiac tamponade. Biochemically, the serum thyroxine of 3 pmol/l (normal 10 to 25) and thyroid-stimulating hormone of 160 mU/l (normal 0.20 to 4.20)) were compatible with hypothyroidism. The patient was treated with L-thyroxine sodium daily, which was gradually increased to 0.125 mg daily. Within a few months he lost weight and became more alert; furthermore, the symptoms of hypothyroidism and the pericardial effusion resolved. It can be concluded that Down syndrome may be associated with hypothyroidism and pericardial effusion. These were alleviated following hormone replacement. Regular evaluation of thyroid function tests is important in Down syndrome. (Neth Heart J 2007;15:67-70.)     

Graves Ophthalmopathy After Radiation Treatment of Thyroid Cancer

ObjectiveTo describe a patient with metastatic thyroid cancer who developed Graves ophthalmopathy after treatment with radioiodine (I 131) and external beam radiation.MethodsWe present a case report that includes clinical, laboratory, and radiologic findings and a brief review of the literature.ResultsA 49-year-old woman who had had a total thyroidectomy and neck dissection followed by I 131 treatment 5 years earlier for papillary thyroid cancer presented for follow-up management after recent neck dissection for recurrent disease. Because she had thyroglobulin antibodies, she was again treated with I 131 after preparation with recombinant human thyroid-stimulating hormone. A post-treatment scan revealed uptake in the right iliac crest. A fludeoxyglucose F 18 positron emission tomography/computed tomography fusion scan revealed osseous metastases in the right pelvis, and external beam radiotherapy was delivered to this area. Approximately 5 months later, she developed periocular swelling and excessive tearing. Magnetic resonance imaging of the orbits revealed enlargement of the extraocular muscles. Serum thyroid-stimulating immunoglobulins were greatly elevated.ConclusionThis case corroborates an earlier report to suggest that radiation-associated thyroid injury in a patient with thyroid cancer may be followed by Graves ophthalmopathy and appearance of thyroid-stimulating immune-globulins in the serum. (Endocr Pract. 2008;14:419-421)     

Repairing a "broken heart" with hormone replacement therapy: case report of cardiogenic shock due to undiagnosed pituitary insufficiency

ObjectivesTo indicate cardiogenic shock as a very rare but serious clinical consequence of untreated panhypopituitarism attributable to Sheehan syndrome; to emphasize the importance of eliciting a detailed endocrine and obstetric history in women presenting with idiopathic heart failure; to highlight the diagnostic shortcomings of screening for thyroid dysfunction solely with thyroidstimulating hormone determinations; and to report the reversibility of severe heart failure induced by longterm pituitary insufficiency.MethodsDescribed is a case report of a 35yearold woman who presented with severe congestive heart failure, hypotension, and confusion. Her 2dimensional echocardiogram revealed appreciable systolic and diastolic dysfunction. In screening for possible endocrine causes of heart failure, a normal thyroidstimulating hormone level of 0.72 mIU/L (reference range, 0.35 to 5.5) was unremarkable; however, a profoundly low free thyroxine level of 0.12 ng/dL (reference range, 0.9 to 1.8) led clinicians to pursue a workup of central hypothyroidism.ResultsEndocrine testing confirmed the presence of panhypopituitarism and adrenal insufficiency. Magnetic resonance imaging of the brain revealed empty sella syndrome. Further questioning of the patient revealed a history of extensive postpartum bleeding 15 years earlier, failure to lactate, and secondary amenorrhea—all consistent with undiagnosed Sheehan syndrome. In the hospital, the patient was treated with intravenously administered corticosteroids and levothyroxine. Her mental status and symptomatic heart failure improved dramatically. After 9 months of oral levothyroxine and glucocorticoid therapy, the patient remained asymptomatic, and repeated echocardiography indicated completely normalized cardiac function.ConclusionSevere heart failure and cardiogenic shock can be a very rare (but fortunately reversible) complication of longstanding panhypopituitarism resulting from undiagnosed Sheehan syndrome. (Endocr Pract. 2012;18:e26e31)     

Successful Treatment of Thyroid Storm with Plasmapheresis in a Patient with Methimazole-Induced Agranulocytosis

ObjectiveTo report a case of a patient with Graves disease presenting with agranulocytosis induced by methimazole, with subsequent thyroid storm and successful therapeutic use of plasmapheresis.MethodsThe clinical features and laboratory findings in a patient with agranulocytosis and thyroid storm are presented, and the available literature on utilization of plasmapheresis in the setting of thyrotoxicosis is reviewed.ResultsA 40-year-old Vietnamese woman with Graves disease was admitted with methimazole-induced agranulocytosis. Treatment with methimazole was discontinued, and therapy with antibiotics, granulocyte colonystimulating factor, and ibuprofen was initiated. During hospitalization of the patient, her clinical status deteriorated, with development of pericarditis, thrombocytopenia, and thyroid storm. Treatment with plasmapheresis yielded near-euthyroidism in 3 days. Subsequently, she underwent successful total thyroidectomy.ConclusionOur case highlights the effectiveness of plasmapheresis when clinical situations prohibit the use of traditional treatment methods for thyrotoxicosis or thyroid storm (or both). (Endocr Pract. 2010;16:673-676)     

An Unusual Case of Recurrent Hyperparathyroidism and Papillary Thyroid Cancer

ObjectiveTo report an unusual occurrence of recurrent hyperparathyroidism due to papillary thyroid carcinoma.MethodsWe describe the clinical history, physical examination findings, laboratory values, imaging findings, and pathologic findings of a woman who developed recurrent hyperparathyroidism 13 years after successful parathyroidectomy.ResultsA 59-year-old woman presented to our clinic with recurrent primary hyperparathyroidism. In 1994, she presented with nephrolithiasis and underwent resection of a right superior parathyroid adenoma that resulted in clinical and biochemical cure. Her clinical course had been followed at periodic intervals, and she had been symptom-free and normocalcemic. In 2007, she again developed nephrolithiasis and was documented to have recurrent hyperparathyroidism. Imaging studies suggested a parathyroid adenoma near the right inferior pole of the thyroid. The patient had reoperative neck exploration. No obvious parathyroid adenoma was found and a right thyroid lobectomy was performed, which resulted in normalization of intraoperative intact parathyroid hormone levels, and the incision was closed. Final pathology demonstrated no parathyroid adenoma, but instead, a 1-cm papillary thyroid carcinoma that stained positive for parathyroid hormone. More than 6 months after surgery, she remains clinically and biochemically cured.ConclusionsRecurrent hyperparathyroidism occurs secondary to multiple causes. This case demonstrates the challenge a surgeon faces in managing recurrent disease and highlights a rare phenomenon of papillary thyroid cancer causing recurrent hyperparathyroidism. (Endocr Pract. 2009;15:349-352)     

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)     

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

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

Reversible Mitral and Aortic Regurgitation Due to Pioglitazone

ObjectiveTo report the occurrence of pioglitazone induced reversible valvular regurgitant lesions.MethodsClinical, laboratory, and imaging data are reported on a patient with known type 2 diabetes mellitus, who was prescribed pioglitazone to achieve better glyce mic control.ResultsWe present a case report of a 50-year-old woman, in whom diabetes had been diagnosed 5 years pre viously, who developed severe mitral and aortic regurgita tion during 5 months of treatment with pioglitazone along with clinical and laboratory indications of fluid retention. Echocardiography 5 months after discontinued use of pio glitazone showed regression of regurgitant lesions and nor malization of pertinent laboratory variables.ConclusionFive months of treatment with pio glitazone could potentially induce major cardiac valvu lar dysfunction, which was reversible in our patient. This report emphasizes the importance of carefully monitoring patients during treatment with thiazolidinediones. (Endocr Pract. 2012;18:e32-e36)     

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)     

Nonfunctioning Parathyroid Carcinoma: Case Report and Review of Literature

ObjectiveTo report a case of nonfunctioning parathyroid carcinoma that was incidentally found during a thyroidectomy for multinodular goiter.MethodsWe present a case report, detailing the clinical course and histologic findings in a patient with a nonfunctional parathyroid carcinoma. The related literature is also reviewed.ResultsA 67-year-old woman presented with a 30-year history of a multinodular goiter that was symptomatic. A total thyroidectomy was performed. Histologic examination revealed not only a multinodular thyroid but also a mass in the left lobe, which was diagnostic of a parathyroid carcinoma. Serum calcium and parathyroid hormone levels were normal postoperatively. Eleven months after the initial operation, a suprasternal mass developed, and she underwent neck reexploration and subtotal resection of an invasive recurrent nonfunctioning parathyroid carcinoma. The serum parathyroid hormone and calcium levels were normal before and after the operation. Postoperatively, the patient underwent radiation therapy. Twenty-three months after the initial operation, a computed tomographic scan of the chest revealed an interval increase in size of a nodule in the left lower lobe of the lung, and 30 months after her initial operation, she underwent resection of an isolated, 1-cm (greatest diameter), metastatic parathyroid carcinoma in the left lower lobe of the lung. The patient is currently doing well without evidence of recurrent disease.ConclusionNonfunctioning parathyroid carcinomas are difficult to diagnose and to treat. Recurrent disease after operation is common, and radiation therapy may help stabilize tumor growth. Patients with nonfunctioning parathyroid carcinomas appear to have a poorer prognosis than do those with functioning parathyroid cancers. (Endocr Pract. 2007;13:750-757)     

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)     

Delayed treatment of papillary thyroid carcinoma arising from struma ovarii in a patient with history of bilateral salpingo-oophorectomy: a case report

ObjectiveWe present a case of papillary thyroid carcinoma arising from struma ovarii treated erroneously as ovarian adenocarcinoma for more than 3 years.MethodsWe report clinical, surgical, laboratory, and imaging findings of the study patient and review the relevant literature.ResultsA 64-year-old woman was treated for ovarian adenocarcinoma for more than 3 years before it was determined that she likely had papillary thyroid carcinoma arising from struma ovarii. This is the first reported case of thyroid carcinoma arising from struma ovarii in a patient with a history of bilateral salpingo-oophorectomy. Possible etiologies include residual ovarian tissue after oophorectomy, ectopic thyroid, or metastatic thyroid cancer.ConclusionsIt is important to include struma ovarii and thyroid carcinoma arising from struma ovarii in the differential diagnosis, even with a history of bilateral salpingo-oophorectomy. This case emphasizes the importance of effective communication among the pathologist, oncologist, and surgeon to ensure timely initiation of appropriate therapy and reduced patient morbidity. (Endocr Pract. 2012;18:e1-e4)