Metabolic effects of growth hormone replacement in two pediatric patients with growth without growth hormone.

Growth without growth hormone (GH) has occasionally been described in patients who have had tumors removed in the hypothalamic-pituitary area. Most of these patients have metabolic abnormalities such as obesity, dyslipidemia and fatty liver. This report describes the metabolic beneficial effects of GH replacement in pediatric patients with growth without GH. Two children in whom the growth without GH phenomenon occurred after therapy for brain tumors participated in this study. Case 1 is a 15-yr-old Japanese girl, diagnosed as having Langerhans cell histiocytosis with multiple intracranial lesions at the age of two. She showed a slight body fat increase, dyslipidemia and fatty liver. Case 2 is a 10-yr-old Indonesian boy, diagnosed with craniopharyngioma at the age of three. He was obese and had low bone mineral density (BMD). In both cases, GH replacement therapy was started at 0.042 mg/kg/week for 12 months. Body composition, BMD, and visceral abdominal area were measured every 3 months. Serum fasting blood glucose, insulin, ALT, lipid profile, leptin, and adiponectin levels were also measured every 3 months. Case 1 showed improvement of transaminase (ALT from 64 to 16 IU/L) and triglyceride (from 239 to 129 mg/dL) over 12 months, but did not show a decrease in visceral fat area or of body fat percentage. Case 2 showed a decrease in body fat percentage and visceral fat area, accompanied by elevated serum adiponectin and decreased leptin levels. In conclusion, twelve months GH replacement therapy improves metabolic abnormalities in pediatric patients with growth without GH.

Spontaneous regression of isolated neurohypophyseal langerhans cell histiocytosis with diabetes insipidus.

In pediatric and adolescent patients, the most common causes for a thickened pituitary stalk with central diabetes insipidus are germ cell tumors, lymphocytic infundibuloneurohypophysitis (LIN), and Langerhans cell histiocytosis (LCH). We describe here a 13-year-old girl who had an abrupt onset of polyuria and polydipsia. Magnetic resonance imaging of the brain revealed thickening of the pituitary stalk, and loss of the physiological hyperintense signal of the posterior pituitary gland. Based on a histopathology, she was diagnosed as having LCH. Another LCH lesion was not detected. The prognoses for LCH patients with single-system and single-site are generally good so we decided on only simple observation. The lesion spontaneously regressed 3 months later, resembling a typical self-limiting course of LIN. In conclusion, the present case suggests that 1) radiological differential diagnosis between LIN and LCH is so difficult that histological confirmation is crucial for correct diagnosis, 2) some past cases of histologically-unconfirmed LIN can include LCH, 3) solitary neurohypophyseal LCH can shrink spontaneously up to near remission level.

Preoperative depiction of cavernous sinus invasion by pituitary macroadenoma using three-dimensional anisotropy contrast periodically rotated overlapping parallel lines with enhanced reconstruction imaging on a 3-tesla system.

OBJECTIVES: Three-dimensional anisotropy contrast (3DAC) magnetic resonance (MR) imaging provides clear depiction of neuronal fibers. The aim of this study was to identify intracavernous cranial nerves in patients with pituitary macro-adenoma and in healthy volunteers by using 3DAC MR imaging on a 3-tesla system and to preoperatively predict cavernous sinus invasion by pituitary macroadenoma. METHODS: Thirty-three patients (cavernous sinuses in 66 sides) with pituitary macroadenomas and 25 healthy volunteers (50 sides) participated in this study. Coronal 3DAC MR images constructed from diffusion weighted images, acquired with periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) sequences, and T2-weighted reverse images were obtained at the same anatomical locations using a 3-tesla MR imaging system. Attempts were made to identify the cranial nerves. RESULTS: The oculomotor and ophthalmic/maxillary nerves were preoperatively identified in all sides (66 sides in patients and 50 sides in healthy volunteers) on 3DAC MR images. In the 33 patients, cavernous sinus invasion was revealed in 10 (12 [18.2%] of 66 sides) by intraoperative endoscopic observation. Coronal 3DAC MR images revealed that the oculomotor nerves were half surrounded with adenoma in all 12 of these sides, and the ophthalmic/maxillary nerves were half encapsulated with tumor (sensitivity/specificity: 100%/100% and 83%/100%, respectively). CONCLUSIONS: Preoperative evaluation of pituitary macroadenomas using 3DAC PROPELLER MR imaging on a 3-tesla system is likely to be a powerful noninvasive method of detecting cavernous sinus invasion, which can potentially dominate the therapeutic strategy for these lesions.

3D-CT Epidurography Can Detect Cerebrospinal-Fluid Leakage in a Patient with Spontaneous Intracranial Hypotension: A Case Report.

Spontaneous intracranial hypotension presents with many symptoms including orthostatic headache, dizziness, and nausea due to cerebrospinal-fluid (CSF) leakage from the spinal dural sac. Although CSF leakage can be estimated by radioisotope (RI) cisternography or computed tomography/magnetic resonance imaging myelography, it is not easy to detect the leakage point using these modalities. Here, we describe a patient with spontaneous intracranial hypotension in whom three-dimensional computed tomography (3D-CT) performed just after an epidural blood patch (EBP) containing contrast medium detected leakage point. The contrast medium injected into the epidural space at the L3/4 level migrated into the intradural space at the lower cervical spine level. RI cisternography performed before EBP did not show the CSF leakage point or any intracranial extension of the tracer. The rostral extension of the RI may be blocked by the collapse of the CSF space due to a large amount of CSF leakage, and due to the compression of the intradural CSF space by epidural CSF. 3D-CT epidurography may be useful to detect the fistula of a CSF leakage even in patients where other modalities including MRI, CT, or RI cisternography cannot specify the leakage point.

Synchronized multiple regression of diagnostic radiation-induced rather than spontaneous: disseminated primary intracranial germinoma in a woman: a case report.

INTRODUCTION: Examples of the spontaneous regression of primary intracranial germinomas can be found in the literature. We present the case of a patient with disseminated lesions of primary intracranial germinoma which synchronously shrunk following diagnostic irradiation. We will discuss whether this regression was spontaneous or radiation-induced. CASE PRESENTATION: A 43-year-old Japanese woman presented to our hospital complaining of memory problems over a period of one year and blurred vision over a period of three months. Following magnetic resonance imaging, she was found to have a massive lesion in the third ventricle and small lesions in the pineal region, fourth ventricle, and in the anterior horn of the left lateral ventricle. Prior to an open biopsy to confirm the pathology of the lesions, she underwent a single cranial computed tomography scan and a single cranial digital subtraction angiography for a transcranial biopsy. Fourteen days after the first magnetic resonance image - 12 and eight days after the computed tomography scan and digital subtraction angiography, respectively - a pre-operative magnetic resonance image was taken, which showed a notable synchronous shrinkage of the third ventricle tumor, as well as shrinkage of the lesions in the pineal region and in the fourth ventricle. She did not undergo steroid administration until after a biopsy that confirmed the pathological diagnosis of pure germinoma. She then underwent whole craniospinal irradiation and went into a complete remission. CONCLUSIONS: In our case report, we state that diagnostic radiation can induce the regression of germinomas; this is the most reasonable explanation for the synchronous multiple regression observed in this case of germinoma. Clinicians should keep this non-spontaneous regression in mind and monitor germinoma lesions with minimal exposure to diagnostic radiation before diagnostic confirmation, and also before radiation treatment with or without chemotherapy begins.

Occipital transtentorial approach and combined treatments for pineal parenchymal tumors.

The deep-seated location of pineal parenchymal tumors (PPTs) and their associations with critical structures make their surgical resection technically challenging; further, the rarity of PPTs and repeated changes in their histopathological diagnostic criteria makes the study of their biological behavior and clinical outcomes difficult. Here, we describe the surgical techniques and results of an occipital transtentorial approach for PPTs together with the results in the clinicopathological study of PPTs. Since 1982, we have treated 93 patients with pineal region tumors, including 17 PPTs, with the occipital transtentorial approach using the lateral semiprone position. The infrasplenial approach is helpful in separating the internal cerebral veins from the tumor, particularly when the tumor is tightly adherent to the veins. Permanent homonymous hemianopsia occurred in 1 of the 17 patients with PPTs. Permanent ocular movement disorders were not encountered. Extensive removal of the tumor significantly prolongs survival at least in patients with pineocytomas and PPT of intermediate differentiation (PPTIMD). Despite extensive resection and adjuvant radiochemotherapy, the prognosis of the patients with pineoblastomas is extremely poor. Although the proliferative potentials of pineocytomas and PPTIMD were significantly lower than those of pineoblastomas, there was no such difference between pineocytomas and PPTIMD.

Relationship between insulin-like growth factor-I and brain natriuretic peptide in patients with acromegaly after surgery.

BACKGROUND: Increased cardiac insulin-like growth factor (IGF)-I production is associated with physiological cardiac hypertrophy in athletes, and IGF-I has been recognized as a cardioprotective agent in experimental animal studies. On the other hand, acromegaly which is characterized by an excess of IGF-I has been linked to impaired cardiac function. METHODS AND RESULTS: Both the relationship between the serum levels of IGF-I and brain natriuretic peptide (BNP), which is released from the cardiac ventricles in response to ventricular stress, and that between IGF-I and the concentrations of the plasma amino-terminal propeptide of procollagen type III (P-III-P), which is associated with myocardial fibrosis, were evaluated in 19 patients after surgical treatment for acromegaly. Echocardiography revealed that left ventricular systolic function and dimensions were within normal range in all patients. Significant inverse correlations were found between IGF-I and the BNP (r=-0.5, p=0.02) and P-III-P levels (r=-0.62, p=0.005). CONCLUSION: We observed an inverse significant relationship between IGF-I and both the BNP and P-III-P value in surgically treated acromegaly patients. These observations suggest that appropriate levels of IGF-I have beneficial cardioprotective effects after surgery in patients with acromegaly.

Basal ganglion hamartoma in a patient presenting with precocious puberty.

We report a basal ganglion hamartoma in a 10-year-old boy in whom the major clinical feature was precocious puberty. Endocrinological evaluation showed a gonadotropin reaction to luteinizing hormone-releasing hormone with no elevation of the human chorionic gonadotropin beta-subunit level in the serum or cerebrospinal fluid. Neuroimaging studies showed a small, calcified, nonenhanced mass lesion with some cystic components in the right basal ganglion. Histopathological examination of specimens removed by stereotactic needle biopsy revealed disorganized neuronal and glial elements in the calcified gray matter-like lesion. In addition, although the presence of microcalcifications was not conspicuous, similar neuroglial lesions of various sizes were scattered in the surrounding white matter. Immunostaining for Ki-67 antigen (MIB-1) showed very low proliferative potential of the glial cells in all the lesions. To our knowledge, this is the first reported occurrence of an intracranial hamartoma located in a site other than the hypothalamus and causing precocious puberty. The possible mechanisms underlying the development of precocious puberty in this patient are discussed.