Xanthogranuloma as an Unsuspected Cause of Idiopathic Central Diabetes Insipidus

ObjectiveTo demonstrate that xanthogranuloma is a rare cause of idiopathic central diabetes insipidus in the early phase of the disease and that it presents as a suprasellar mass at a later stage. In addition, we emphasize the importance of identifying the cause of idiopathic central diabetes insipidus and review the literature concerning endocrine disturbance in central xanthogranuloma.MethodsReview of recently published case reports of central xanthogranuloma with endocrine disorders. The case of a 35-year-old man who presented with a very large suprasellar mass is also reported. The patient was diagnosed with idiopathic central diabetes insipidus 20 years ago with normal brain magnetic resonance imaging.ResultMost cases of this disease present as supra-or parasellar masses with endocrine involvement, the most common of which (in approximately 75% of patients) is sex hormone deficiency. Diabetes insipidus was found in 65% of patients.ConclusionXanthogranuloma should be in the differential diagnosis of idiopathic central diabetes insipidus and sellar and parasellar masses. A detailed skin examination is very important in making the diagnosis of central diabetes insipidus. (Endocr Pract. 2014;20:e42-e46)     

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)     

Chordoid glioma of the third ventricle: Report of a case with cytologic features and utility during intraoperative consultation

BACKGROUND: Chordoid glioma is a rare, low grade neoplasm with a unique chordoid appearance as well as distinct clinicopathologic and immunohistochemical features. Its cytologic features have not been described. CASE: A 42-year-old woman with recent-onset amnesia and confusion underwent magnetic resonance imaging, which revealed a 5-cm mass lesion arising in the third ventricle. Intraoperative squash smears showed cellular sheets as well as nests and strands of epithelioid tumor cells with bland nuclei and polygonal to elongated cytoplasm in a mucinous background. Binucleation was commonly seen. The tumor was intimately admixed with a benign lymphoplasmacytic infiltrate and scattered Russell bodies. Histologically, the tumor cells were arranged in a syncytium with prominent lymphoplasmacytic infiltrates and scattered small foci of necrosis in a mucinous matrix. The foremost differential diagnosis was chordoid meningioma. Immunohistochemically, the tumor cells were positive for glial fibrillary acid protein (GFAP), vimentin, epithelial membrane antigen, CD34, neuron-specific enolase and CK-7 and negative for synaptophysin, S-100 protein, neurofilament, and estrogen and progesterone receptors. CONCLUSION: Intraoperative smear cytology in this case of chordoid glioma revealed distinctive cytologic features, reflecting the unique histologic pattern. Cytologic features, such as binucleation, absence of intranuclear pseudoinclusions and GFAP immunoreactivity, are particularly helpful in differentiating chordoid glioma from chordoid meningioma.     

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)     

Clinical Characteristics and Pituitary Dysfunction in Patients with Metastatic Cancer to the sella

ObjectiveMetastatic disease to the sella is uncommon and there are limited available data regarding the clinical aspects of this disease. We therefore sought to characterize the clinical demographics of sellar metastases.MethodsRetrospective chart review of adults treated at Stanford University Medical Center from 1980 to 2011 with metastatic disease to the sella.ResultsA total of 13 subjects were identified (9 females). The mean age at diagnosis was 55 years (range, 25 to 73 years). A total of 6 patients (46%) had breast car-cinoma, 3 (23%) had renal cell carcinoma, 2 (15%) had squamous cell carcinoma of the head and neck, 1 had bronchoalveolar carcinoma of the lung, and 1 had nodular sclerosing Hodgkin’s lymphoma. The most common presenting signs and symptoms were headache (58%), followed by fatigue (50%), polyuria (50%), visual field defects (42%), and ophthalmoplegia (42%). Seventy-five percent of patients presented with at least one pituitary hormone insufficiency, which included 6 patients (50%) with diabetes insipidus (DI). Eight (67%) subjects had secondary hypothyroidism and 5 (45%) had secondary adrenal insufficiency. Of the patients with stalk involvement, 86% had DI. All patients had a prior diagnosis of malignancy, with a mean duration of 95 months.ConclusionThe most common neoplastic sources to the sella were breast and renal cell carcinomas. Secondary hypothyroidism was the most common endocrine abnormality, followed by DI and adrenal insufficiency. Newonset central hypothyroidism and DI along with known malignancy in a patient with a sellar lesion should raise the suspicion of a metastatic source. (Endocr Pract. 2013; 19:914-919)     

Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus

Magnetic resonance imaging (MRI) has revealed isolated pituitary stalk thickening (PST) in certain cases of idiopathic or secondary central diabetes insipidus (DI) due to infiltrative processes. Twenty-six children with DI and PST underwent cerebral MRI at the age of 8 +/- 4 years and were followed (n = 25) by clinical and MRI evaluation for 5.5 +/- 3.6 and 3.0 +/- 2 years, respectively, but given no treatment other than hormonal substitutive therapy. Patients were subdivided into groups according to the etiology of the DI: germinoma (n = 4), Langerhans' histiocytosis (n = 5) or 'idiopathic' DI with PST (n = 17). Complete anterior pituitary evaluation in 24 of the 26 patients revealed that 14 children were suffering from associated growth hormone deficiency and 7 had multiple hormone deficiencies. At the first MRI evaluation, pituitary stalk enlargement varied from 2.2 to 9.0 mm. The anterior pituitary gland was found to be normal (n = 12), small (n = 8) or enlarged (n = 6). At the final evaluation, a change in MRI features had occurred in 16 patients: morphological and/or signal changes in the PST (n = 16; 6 of whom showed an increase in PST) and changes in anterior pituitary gland size (n = 8; 3 of whom had increased and 5 had decreased). The presence of a growing suprasellar mass with a progressively enlarging pituitary stalk was demonstrated in the 6 patients who had shown increased pituitary stalk enlargement 1.8 +/- 1.6 years after the first MRI. In 4 of these patients, a diagnosis of germinoma was made 1.3 +/- 0.6 years after PST identification by MRI at the onset of DI, but the other 2 patients showing a suprasellar mass were still categorized as 'idiopathic' at the final clinical evaluation performed 7.8 and 12.3 years after DI onset. In 10 patients (all but 1 with Langerhans' histiocytosis, showing 'idiopathic' DI) the pituitary stalk enlargement was diminished after 2.0 +/- 1.9 years of MRI follow-up, and there was a complete reversal of pituitary stalk enlargement in 5 patients. We conclude that the natural history of 'idiopathic' isolated central DI with PST is unpredictable. Although germinoma should always be considered during the first 3 years of follow-up in patients presenting isolated DI with PST by repeated investigations every 3-6 months, it remains an infrequent etiology, affecting only 15% of cases in our study.     

Incidence of Central Diabetes Insipidus in Children Presenting with Polydipsia and Polyuria

Objective: Polydipsia and polyuria are common reasons for referral to the Pediatric Endocrine clinic. In the absence of hyperglycemia, diabetes insipidus (DI) should be considered. The objectives of the study were to determine the prevalence of central DI (CDI) in a group of children presenting for evaluation of polydipsia and polyuria, and to determine if predictive features were present in patients in whom the diagnosis of DI was made.Methods: The study was a retrospective chart review of children presenting to the endocrine clinic with complaints of polydipsia and polyuria over a 5-year period.Results: The charts of 41 patients (mean age 4.9 ± 3.7 years, 28 males) were reviewed. CDI was diagnosed in 8 (20%) children based on abnormal water deprivation test (WDT) results. All but one patient had abnormal magnetic resonance imaging (MRI) findings, the most common being pituitary stalk thickening. Children with DI were older (7.86 ± 4.40 vs. 4.18 ± 3.20 years, P = .01) and had a higher propensity for cold beverages intake and unusual water-seeking behaviors compared to those without DI. Baseline WDT also revealed higher serum sodium (Na) and osmolality.Conclusion: The incidence of CDI in children presenting with polydipsia and polyuria is low. Factors associated with higher likelihood of pathology include older age, propensity for cold beverage intake, and higher baseline serum Na and osmolality on a WDT.Abbreviations:BMI = body mass indexCDI = central diabetes insipidusDI = diabetes insipidusNa = sodiumWDT = water deprivation test     

Apathetic Thyrotoxicosis Secondary to Atypical Subacute Thyroiditis

ObjectiveTo report a case of apathetic thyrotoxicosis with an etiology of subacute thyroiditis.MethodsWe describe the patient’s clinical findings, laboratory findings, and clinical course.ResultsAn 85-year-old woman with no history of thyroid disease presented with severe obtundation and altered mental status. Laboratory testing documented elevated free thyroxine and free triiodothyronine concentrations and a suppressed thyrotropin concentration. Thyroid antibodies were absent. A radioactive iodine study revealed severely diminished uptake, suggestive of thyroiditis. After a short course of steroids, the patient’s mental status returned to baseline. Follow-up laboratory testing showed normalizing thyroid function.ConclusionEven in the absence of a history of thyroid disease, we recommend considering thyroid dysfunction in the differential diagnosis of patients who present with altered mental status, particularly in the elderly population. (Endocr Pract. 2012;18:e127-e129)     

A Case of Rapid-Onset Obesity with Hypothalamic Dysfunction,Hypoventilation, Autonomic Dysregulation,and Neural Crest Tumor: Rohhadnet Syndrome

ObjectiveRapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) is a rare disorder that mimics both common obesity and genetic obesity syndromes along with several endocrine disorders during early childhood. We aim to present the clinical features, laboratory and imaging results, and treatment outcomes of a patient with ROHHAD syndrome.MethodsIn this case report, we describe a 26-month-old boy who was admitted to our emergency department with dyspnea and cyanosis and was suspected to have ROHHAD syndrome due to his rapid-onset obesity and alveolar hypoventilation.ResultsA thoracal and abdominal magnetic resonance imaging was performed to demonstrate a possible accompanying neural crest tumor and it provided a yet asymptomatic retroperitoneal ganglioneuroblastoma. Based on these findings, the patient was diagnosed as ROHHADNET syndrome.ConclusionBecause of the high prevalence of cardiorespiratory arrest and probability of accompanying tumors, early recognition of ROHHAD syndrome is important. To prevent presumptive mortality and morbidity, ROHHAD syndrome should be considered in all cases of rapid and early-onset obesity associated with hypothalamic-pituitary endocrine dysfunctions. (Endocr Pract. 2013;19:e13-e16)     

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)     

Cushing’s Syndrome Due to a Corticotropin-releasing Hormone- and Adrenocorticotrophic Hormone-producing Neuroendocrine Pancreatic Tumor

Objective: To our knowledge, only 2 cases of pancreatic neuroendocrine tumors have been described as the source of corticotropin-releasing hormone (CRH) in Cushing’s syndrome. Here, we describe a case of ectopic adrenocorticotrophic hormone (ACTH-) and CRH-production caused by a pancreatic neuroendocrine tumor.Methods:We analyzed and summarized the patient’s medical history, physical examination results, laboratory data, imaging studies, and histopathologic results.Results: An endocrinologic workup revealed massive ACTH-dependent hypercortisolism. Pituitary magnetic resonance imaging (MRI) showed no pathologic findings and led to extensive imaging in search of the suspected ectopic lesion. Ketoconazole treatment was initiated. Rapid deterioration of the patient’s clinical condition due to escalating cortisol levels and resulting sepsis required an emergency adrenalectomy to control the hypercortisolism. A positron emission tomography-computed tomography (PET-CT) scan revealed a hepatic lesion, which was biopsied. Histology of the lesion showed a well-differentiated endocrine tumor. Subsequent scintigraphy with octreotide (a somatostatin [SMS] analog) detected a pancreatic tumor, which was endosonographically confirmed. The initiated SMS therapy was followed by a distal splenopancreatectomy and a right hemihepatectomy. Immunostaining of the specimen showed positive expression for CRH and ACTH.Conclusion: We conclude that SMS-scintigraphy did have an additional diagnostic benefit compared to PET-CT. In hypercortisolemic patients, rapid endocrinologic evaluation is crucial to prevent rapid deterioration and a possible fatal outcome. (Endocr Pract. 2014;20:e53-e57)     

Endocrine Involvement in Systemic Amyloidosis

ObjectiveTo present an overview of the published data on endocrine involvement and endocrine dysfunction in patients with systemic amyloidosis.MethodsWe conducted a review of the medical literature using MEDLINE data sources, including clinical trials, in vitro studies, and case reports on pituitary, thyroid, parathyroid, pancreatic, adrenal, and gonadal involvement in systemic amyloidosis.ResultsReports of endocrine involvement in systemic amyloidosis seem to consist primarily of small-samplesize clinical trials or case reports, probably because of the rarity of the disease itself. Systemic amyloidosis mainly involves and causes functional impairment in the thyroid and testes in the endocrine system. Evaluation of adrenal function necessitates special consideration because amyloid infiltration of the adrenal glands resulting in failure may be a life-threatening condition. Amyloid deposition commonly seen in the pituitary gland and the pancreas of patients with Alzheimer disease and type 2 diabetes mellitus, respectively, is generally classified as local amyloidosis and should not be confused with systemic involvement. Additionally, detection of amyloid deposition in the thyroid and testes may have a diagnostic role in patients with suspected systemic or renal amyloidosis.ConclusionPublished data suggest that systemic amyloidosis frequently involves the endocrine system, and endocrine dysfunction seems to be not as rare as previously thought. A rapidly growing goiter or symptoms and signs of adrenal or gonadal dysfunction should raise suspicion of amyloid infiltration. Involvement of pituitary, parathyroid, and pancreatic sites in systemic amyloidosis still remains to be clarified. Further studies with larger sample sizes are needed for complete characterization of the effect of systemic amyloidosis on the endocrine system. (Endocr Pract. 2010;16:1056-1063)     

Pituitary Dysfunction in Patients with Intracranial Germ Cell Tumors Treated with Radiotherapy

Objective:To evaluate the endocrine abnormalities in intracranial germ cell tumors (iGCTs) treated with radio-therapy (RT), and to discuss the effects of RT on pituitary functions.Methods: Seventy-seven patients diagnosed with iGCTs who had received RT and endocrine follow-up in Huashan Hospital between January 2010 and July 2017 were retrospectively analyzed, consisting of 49 germinomas and 28 NGGCTs. The median follow-up period was 50.0 months. Fifty-one patients had radiologically proved suprasellar/sellar lesions.Results: The male to female ratio was 62/15. The median endocrine follow-up period was 19 (4, 42) months. The median age at the last endocrine visit was 18 (16, 20) years old. The 5-year overall and recurrence-free survival were both 98.7%. The overall prevalence of central adrenal insufficiency (CAI), central hypothyroidism (CHT), central hypogonadism (CHG), hyperprolactinemia, and central diabetes insipidus (CDI) was 57.3%, 56%, 56.6%, 35.3%, and 52.1%, respectively, after RT. Patients having suprasellar/sellar lesions showed significantly higher post-therapeutic prevalence of hypopituitarism than those who didn’t. Compared to that before RT, CAI, CHT, and CHG weren’t significantly improved while the levels of prolactin and the prevalence of CDI declined significantly (P =.03 and.001). The radiation doses to pituitary and hypothalamus between those with and without CAI, CHT, and CHG weren’t significantly different.Conclusion: The prevalence of hypopituitarism was high in iGCTs, especially in those with suprasellar/sellar involvement. The levels of prolactin and the prevalence of CDI declined significantly after RT. The hypopituitarism in iGCTs was mainly induced by tumor effects, and RT showed no additional damage to pituitary functions in our study.Abbreviations: AFP = alpha-fetoprotein; CAI = central adrenal insufficiency; CDI = central diabetes insipidus; CHG = central hypogonadism; CHT = central hypothyroidism; CT = computed tomography; DA = dopamine; GH = growth hormone; βHCG = beta-human chorionic gonadotropin; HPA = hypothalamus-pituitary-adrenal; HPG = hypothalamus-pituitary-gonadal; HPL = hyperprolactinemia; HPT = hypothalamus-pituitary-thyroid; iGCT = intracranial germ cell tumor; IGF-1 = insulin-like growth factor 1; NGGCT = nongerminomatous germ cell tumors; OS = overall survival; PFS = progression-free survival; PRL = hypothalamus-pituitary-prolactin; RT = radiotherapy     

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)     

Development of Thyroid Storm After Surgical Resection of A Thyrotropin-Secreting Pituitary Adenoma

ObjectiveTo describe a patient with a thyrotropinsecreting pituitary adenoma in whom postoperative thyroid storm developed.MethodsWe present a case report with details of the initial presentation, laboratory evaluation, surgical and pathologic findings, and subsequent course in a patient with a thyrotropin (thyroid-stimulating hormone or TSH)- secreting adenoma and postoperative thyroid storm.ResultsAn 18-year-old male patient presented with severe headaches and was found to have a large suprasellar tumor and a mildly elevated level of TSH. Thyroid storm developed immediately after surgical resection of the pituitary mass. Results of laboratory evaluation undertaken preoperatively became available after the patient had undergone the surgical procedure and revealed thyroid hormone levels 2 to 3 times the upper limit of normal. Propylthiouracil and β-adrenergic blocking agents controlled the postoperative thyrotoxicosis and were subsequently discontinued as his TSH and thyroid hormone levels normalized.ConclusionThis case demonstrates the rare case of a TSH-secreting adenoma in a young patient, which was complicated by the development of postoperative thyroid storm. In addition, this case emphasizes the importance of preoperative pituitary hormonal evaluation and treatment of hormonal abnormalities in all patients presenting with sellar or suprasellar tumors. (Endocr Pract. 2008;14:732- 737)     

Pituitary Hormone Dysfunction After Proton Beam Radiation Therapy in Children with Brain Tumors

Objectiveo characterize endocrine dysfunction in pediatric patients with brain tumors who received proton beam (PB) radiation therapy and to compare those treated with PB radiotherapy only versus combined conventional and PB irradiation.MethodsA retrospective review of medical records of patients ≤ 18 years of age who received PB radiation therapy for a brain tumor between 2000 and 2008 was performed. Variables analyzed included patient demographics, tumor type, therapeutic modalities, radiation doses, and types and timing of endocrine dysfunction.ResultsThirty-eight patients were identified, of whom 31 (19 boys and 12 girls; mean age, 11.9 ± 3.3 years) had undergone endocrine evaluation. Of these patients, 19 received PB radiotherapy only and 12 received conventional plus PB irradiation. Before irradiation, a cranial surgical procedure was performed in 28 study subjects, and 22 received chemotherapy. The mean duration of follow-up after radiation therapy was 1.8 ± 0.8 years. Nine patients (47%) in the PB only group and 4 (33%) in the conventional plus PB group developed endocrine dysfunction (no significant difference) after cranial irradiation. Children with endocrine sequelae treated with PB irradiation alone received fewer cobalt gray equivalents than those treated with conventional plus PB irradiation (5,384 ± 268 versus 5,775 ± 226, respectively; P < .02), and pituitary hormone deficiencies were detected later during follow-up in those who received PB radiotherapy only versus conventional plus PB irradiation (1.17 ± 0.4 years versus 0.33 ± 0.11 year, respectively; P < .01).ConclusionA high rate of endocrine sequelae was seen in our study. Children with brain tumors treated with conventional plus PB irradiation developed endocrine dysfunction faster and received a higher radiation dose than those receiving PB radiotherapy only. Prior surgical treatment and chemotherapy were additional risk factors. Large prospective studies are needed to evaluate further the incidence of endocrine sequelae after PB irradiation in children. (Endocr Pract. 2011;17:891-896)     

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)     

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)     

Idiopathic Sporadic Tumoral Calcinosis of the Hip: Successful Oral Bisphosphonate Therapy

ObjectiveTo report the case of a woman with idiopathic sporadic tumoral calcinosis treated successfully with orally administered bisphosphonates.MethodsWe report the clinical, laboratory, and imaging findings and describe the clinical course of tumoral calcinosis. The literature was reviewed for the pathophysiologic features and strategies for treatment of tumoral calcinosis. In addition, we specifically reviewed the use of bisphosphonates in tumoral calcinosis and the possible pharmacologic basis for the therapeutic benefit.ResultsA 45-year-old woman presented with a 6-week history of left-sided pain in the hip in conjunction with stiffness after a trivial fall 4 weeks before the onset of symptoms. The findings on conventional radiology of the hip joint were consistent with tumoral calcinosis of the left hip. The biochemical profile of the patient was unremarkable. Oral treatment with alendronate, 70 mg once a week, yielded alleviation of symptoms within 12 weeks. Radiology of the left hip repeated after a period of 15 months revealed notable regression of the calcified lesion.ConclusionBisphosphonate therapy may be considered as an alternative to surgical treatment in patients with idiopathic sporadic tumoral calcinosis. (Endocr Pract. 2007;13:182-186)