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)     

Variations In Clinical And Imaging Findings By Time Of Diagnosis In Females With Hypopituitarism Attributed To Lymphocytic Hypophysitis

Objective: To describe the various patterns of presentation, including assisting analyses, associated with the timing of diagnosis of females with hypopituitarism and suspected clinical diagnosis of lymphocytic hypophysitis.Methods: A retrospective study of 9 consecutive females with pituitary dysfunction developed during or after pregnancy. All subjects were treated in our clinics between 2008 and 2014. Data were collected on clinical characteristics, pituitary hormone levels, and imaging findings.Results: The study group included 9 patients with a mean age 33.7 ± 7.8 years at delivery. The probable cause of disease was lymphocytic hypophysitis. Headache or specific symptoms/signs of hypopituitarism appeared within 1 year of delivery. Five patients had headache, and 8 had difficulty breastfeeding or amenorrhea. Laboratory findings included central hypocortisolism (8/9 patients), hypogonadotropic hypogonadism (8/9), and central hypothyroidism (6/7). Insulin-like growth factor-1 (IGF-1) levels were low in 8/8 patients. Prolactin levels were low in 3/9 patients, and 1 patient had diabetes insipidus. Seven patients were diagnosed less than 1 year from symptom onset; 4 (57%) complained of headaches, and 5 (71%) had panhypopituitarism. Two patients were diagnosed later. Both had difficulty breastfeeding and amenorrhea, and one also had headaches. Both had panhypopituitarism and reduced pituitary volume. None of the patients fully recovered pituitary function. Normalization of the thyrotroph axis occurred in 3 patients, gonadotroph function in 3, the corticotroph axis in 2, and IGF-1 normalized in 1 subject.Conclusion: Hypopituitarism attributed to lymphocytic hypophysitis may present during pregnancy or early postpartum period with a clear clinical picture, or later, with indolent and nonspecific symptoms and signs.Abbreviations:ACTH = adrenocorticotropic hormoneDI = diabetes insipidusFSH = follicle-stimulating hormoneGH = growth hormoneIGF-1 = insulin-like growth factor-1LH = luteinizing hormoneLT4 = levothyroxineMRI = magnetic resonance imagingT4 = thyroxineTSH = thyroid stimulating hormone     

Hypophysitis

ObjectiveHypophysitis is considered a rare inflammatory disease of the pituitary gland. For a long time, primary autoimmune hypophysitis has stood out as the most relevant type of hypophysitis. However, with the advent of immunotherapy for the treatment of malignancies and identification of hypophysitis as an immune-related adverse event, hypophysitis has garnered increasing interest and recognition. Therefore, awareness, early recognition, and appropriate management are becoming important as the indication for immunomodulatory therapies broaden.MethodsIn this review, we discuss the epidemiology, diagnosis, and treatment of hypophysitis with a focus on recent data and highlight subtypes of particular interest while recognizing the gaps in knowledge that remain.ResultsRegardless of cause, symptoms and signs of hypophysitis may be related to mass effect (headache and visual disturbance) and hormonal disruption that warrant prompt evaluation. In the vast majority of cases, a diagnosis of hypophysitis can be made presumptively in the appropriate clinical context with radiologic findings consistent with hypophysitis and after the exclusion of other causes.ConclusionAlthough subtle differences currently exist in management and outcome expectations between primary and secondary causes of hypophysitis, universally, treatment is aimed at symptom management and hormonal replacement therapy.     

Perioperative Management of a Patient with a Nonresectable Pheochromocytoma

ObjectiveTo describe the clinical course of a patient with a nonresectable pheochromocytoma during urgent hip surgery.MethodsTo describe the clinical management and postoperative outcome of the patient and review the relevant literature.ResultsAn 85-year-old male with a nonresectable pheochromocytoma required urgent hip surgery following a traumatic hip fracture. He was perioperatively managed with phenoxybenzamine, metyrosine, and metoprolol to avoid potential pheochromocytoma-related complications. He remained hemodynamically stable and recovered from the surgery without complications.ConclusionsThis case illustrates the successful management of a patient requiring urgent surgery in the setting of a nonresectable pheochromocytoma, which is rarely described in the literature. (Endocr Pract. 2013;19: e74-e76     

Recurrent Neutropenia Due to Dopamine Agonists in a Patient with Microprolactinoma

ObjectiveThe objective of our case report is to increase the awareness of neutropenia as a rare but potentially serious adverse outcome of commonly used dopamine agonists for the treatment of prolactinomas.MethodsThis report reviews the clinical history, diagnosis, investigations, and drug treatment of our patient.ResultsNeutropenia was recurrent after changing to various different dopamine agonists necessitating surgical treatment of a prolactinoma.ConclusionThis case serves a reminder of this adverse drug reaction. Considering the significant impact and potentially a life threatening outcome, we advocate routine monitoring of full blood count prior to and during the treatment with dopamine agonists. (Endocr. Pract. 2013;19:e122-e123)     

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)     

Active Crohn Disease and Hypercalcemia Treated With Infliximab: Case Report and Literature Review

ObjectiveTo report a case of 1,25-dihydroxyvitamin D–mediated hypercalcemia caused by active Crohn disease that improved with infliximab therapy.MethodsWe present the clinical and laboratory findings and describe the clinical course of a patient who had hypercalcemia during Crohn disease exacerbations. The literature is reviewed regarding 1,25-dihydroxyvitamin D production in Crohn disease, and the 3 cases of hypercalcemia in individuals with Crohn disease reported in the literature are described.ResultsA 50-year-old man with long-standing Crohn disease treated with multiple bowel resections presented for take-down ileostomy. He was hypercalcemic and had suppressed parathyroid hormone and parathyroid hormone–related peptide levels. Histopathology of the resected ileostomy site and adjacent small bowel indicated active Crohn disease. Hypercalcemia promptly resolved after a few days of treatment with intravenous glucocorticoids. One month later, hypercalcemia recurred in the presence of an inappropriately high 1,25-dihydroxyvitamin D level and increased urinary calcium and serum angiotensin-converting enzyme levels. The serum and urinary calcium levels became normal with infliximab therapy. Three previous reports of hypercalcemia caused by active Crohn disease describe effective treatment with glucocorticoids. This is the first report of successful response to infliximab in this setting.ConclusionHypercalcemia mediated by 1,25-dihydroxyvitamin D in the setting of Crohn disease may respond to glucocorticoid-sparing immunomodulators. (Endocr Pract. 2008;14:87-92)     

Enfermedad de Erdheim-Chester: primer caso pediátrico reportado en Colombia

The Erdheim-Chester’s disease is extremely rare in children. We present the case of a 12-year-old girl with histological and radiological diagnosis of this disease and mutation of the BRAF gene, who developed multisystemic compromise requiring treatment with dabrafenib. We identified 22 reports of this condition among children worldwide and this is the second pediatric case in Latin America. Diagnostic imaging is critical to confirm Erdheim-Chester disease and for the surgical planning of the biopsy. Additionally, we identified the parasellar dark sign, which has previously been reported on lymphocytic hypophysitis.This report contributes to the current practice as it shows the clinical presentation and the diagnostic workout of this disease in pediatrics.     

Erlotinib-Associated Exacerbation of Hypothyroidism with Pericardial Tamponade

ObjectiveTo report a case of erlotinib-associated exacerbation of hypothyroidism complicated by pericardial tamponade.MethodsWe describe the patient’s clinical presentation, biochemical workup, and clinical course.ResultsNon-small cell lung cancer was diagnosed in a 54-year-old woman. After cisplatin and radiation therapy, she was noted to have subclinical hypothyroidism that did not necessitate treatment. The tyrosine kinase inhibitor erlotinib, 150 mg once daily, was prescribed. Three months later, the patient was documented to have severe hypothyroidism. Levothyroxine was prescribed, but she continued to experience shortness of breath, fatigue, and chest and back pain, which resulted in an emergency department visit. Inpatient workup revealed cardiac tamponade with a large pericardial effusion and a right ventricular diastolic collapse. Pericardiocentesis was performed.ConclusionsThis is the first case report linking erlotinib use and thyroid disease. (Endocr Pract. 2012; 18:e111-e113)     

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)     

A Case of Hypoglycemia,Lactic Acidosis,and Hematologic Malignancy

ObjectiveTo report a case of chronic and persistent hypoglycemia and lactic acidosis in a 74-year-old military veteran.MethodsThe clinical, laboratory, radiologic, and cytogenetic details of the case are presented, followed by a discussion of the related literature.ResultsThe patient was treated for septicemia without evidence of infection. Mitochondrial dysfunction was explored because of the possibility of environmental exposures during military service. On bone marrow biopsy, he was found to have immature B-cell lymphoma and myelodysplasia. There have been 28 previously reported cases of non-Hodgkin lymphoma-induced lactic acidosis in adult patients (11 with hypoglycemia), which has been associated with a high mortality rate.ConclusionOur case is unique because, to our knowledge, it is the first reported case of immature B-cell lymphoma/leukemia and myelodysplasia with a complex karyotype based on extensive cytogenetic studies in a patient presenting with hypoglycemia, lactic acidosis, and central nervous system involvement by lymphoma. (Endocr Pract. 2010;16:241-243)     

Apoplexy of A Microprolactinoma During Pregnancy: Case Report and Review of Literature

ObjectiveTo report a rare case of apoplexy in a microprolactinoma during pregnancy.MethodsWe present the initial clinical manifesta tions, laboratory results, radiologic findings, and man agement in a patient who had pituitary apoplexy during early pregnancy. The pertinent literature and management options are also reviewed.ResultsA 37-year-old woman with a history of a microprolactinoma presented during the 16th week of her first pregnancy with a sudden onset of severe headache, nausea, vomiting, and blurred vision. Magnetic resonance imaging showed a sellar heterogeneous mass with supra sellar extension and contact with the optic chiasm, com patible with adenoma apoplexy. The patient’s visual fields were normal. Conservative management was followed by rapid clinical improvement and a notable regression of the sellar mass after 5 weeks of cabergoline therapy. Uneventful pregnancy resulted in the delivery of a healthy baby. Repeated magnetic resonance imaging was per formed 1 week after delivery and showed resolution of the microadenoma. At 3 months after delivery, the patient had recovered regular menses and had sustained normal prolac tin levels without treatment.ConclusionThis case illustrates a rare occurrence of apoplexy in a microprolactinoma during pregnancy, which was managed conservatively and led to a complete regression of the pituitary tumor. 3Endocr Pract. 2012;18:e147-e150)     

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 Interesting Case of Peripheral Vascular Disease,Vascular Reperfusion,and Subsequent Development of Pain Due to Paget's Disease of Bone

ObjectiveTo present a case of Paget’s disease of bone that was unmasked after vascular reperfusion.MethodsIn this case study, we review the presentation, evaluation, diagnosis, and management of a patient with Paget’s disease and peripheral vascular disease.ResultsA 79-year-old-woman with a history of coronary artery heart disease and recent finding of a T5 compression fracture was hospitalized for evaluation of right lower extremity claudication. Angiography demonstrated a focal complete occlusion of the distal right femoral and popliteal arteries. A self-expanding stent was placed in the distal femoral and popliteal arteries. Approximately 48 hours after the procedure, the patient developed severe, right lower leg pain. On endocrine evaluation, the patient was found to have clinical signs suggesting Paget’s disease of bone, which was subsequently confirmed by imaging.ConclusionThis patient’s development of severe pain following reperfusion of distal femoral and popliteal arteries is in keeping with the known and aforementioned hypervascularity of pagetic bone. The finding of increased warmth over an area of skeletal deformation should always raise the possibility of Paget’s disease of bone.(Endocr Pract. 2013;19:e61-e63)     

A Case of Occult Ectopic Adrenocorticotropic Hormone-Secreting Tumor: Diagnostic and Management Dilemmas

ObjectiveTo report a case of ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient whose tumor was not localized by radiographic or biochemical means and to discuss the difficulties inherent in this patient’s care, illustrative of the challenges encountered by clinicians faced with similar cases.MethodsWe describe the clinical presentation of our case and discuss its management.ResultsA 49-year-old woman presented with symptoms and physical findings strongly suggestive of Cushing syndrome. Findings on biochemical evaluation were consistent with ectopic ACTH syndrome. Radiographic imaging did not clearly identify a discrete tumor. Bilateral inferior petrosal sinus sampling and whole-body selected venous sampling were unsuccessful in localizing the source of ACTH secretion. Surgical exploration was undertaken with use of intraoperative ultrasonography. Both a primary tumor and metastatic disease were identified, and the patient underwent distal pancreatectomy and splenectomy, as well as sequential bilateral adrenalectomy. The primary lesion was a neuroendocrine tumor that did not stain positive for ACTH. Chemotherapeutic agents were used to control bulky hepatic metastatic lesions until the patient’s demise 2½ years after her initial presentation.ConclusionThis case illustrates the difficulties encountered in the assessment and management of a patient with ectopic ACTH syndrome when conventional methods of tumor localization fail to identify the source of hormone secretion. (Endocr Pract. 2008;14:588-591)     

Hemorrhagic Shock As the Initial Manifestation of Pheochromocytoma: Report of A Sequential Management Strategy

ObjectiveTo describe a patient presenting with hemorrhagic shock attributable to bleeding pheochromocytomas and the sequential management strategy used for treating this patient.MethodsWe summarize the clinical presentation,diagnostic work-up, surgical management, and pathologicfeatures of our patient and review the pertinent literature.ResultsA 38-year-old man with multiple endocrineneoplasia type 2A and bilateral pheochromocytomas presented initially with nearly fatal retroperitoneal and intraperitoneal hemorrhage rather than the characteristic hypertensive paroxysms. After lifesaving operative interventionand a 5-month period of rehabilitation and convalescence,the patient underwent bilateral retroperitoneoscopic adrenalectomy as definitive treatment. Thus, the abdomen thathad been operated on multiple times because of hemorrhage was left undisturbed, and the patient had a successfulrecovery.ConclusionNear-fatalintraperitonealhemorrhageis a very rare initial manifestation of pheochromocytoma.Our current patient with bilateral pheochromocytomaspresented in this dramatic manner. This case shows that a sequential management strategy of damage-control surgical treatment followed by future resection of the tumorsafter appropriate a-adrenergic blockade is a safe and effective therapeutic option. (Endocr Pract. 2012;18:e81-e84)     

Spontaneous Hemorrhage of a Parathyroid Adenoma Into the Mediastinum

ObjectiveTo describe the case of a previously healthy 56-year-old woman, who presented with acute, nontraumatic pain in the left side of the neck and mild dysphagia.MethodsWe report the results of the physical examination, imaging studies, and clinical laboratory studies. In addition, we describe the patient’s hospital course after surgical intervention.ResultsA patient who sought medical attention because of acute, nontraumatic neck pain and dysphagia was subsequently found to have acute extracapsular hemorrhage of a parathyroid adenoma. Computed tomography and magnetic resonance imaging studies revealed a mass effect beginning in the neck and extending into the mediastinum. Surgical exploration of the neck and histopathologic evaluation confirmed the diagnosis of spontaneous rupture of a parathyroid adenoma with associated hemorrhage.ConclusionExtracapsular hemorrhage of a parathyroid adenoma is rare and necessitates a high index of clinical suspicion for diagnosis. This diagnosis should be considered in a patient who presents with acute anemia, hypercalcemia, and a neck mass. (Endocr Pract. 2012;18: e57-e60)     

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)