The role of leptin, soluble leptin receptor, resistin, and insulin secretory dynamics in the pathogenesis of hypothalamic obesity in children.

INTRODUCTION: In this study, we have investigated the role of leptin, soluble leptin receptor(sOb-R), resistin, and insulin secretory dynamics in the development of hypothalamic obesity. MATERIALS AND METHODS: Children who had hypothalamo-pituitary tumor were divided into two groups. First group included obese-overweight (hypothalamic obese = HOB group, n = 23) and second group included non-obese children (hypothalamic non-obese = HNOB group, n = 16). Exogenously obese-overweight children (OB group, n = 22) were included as controls. Basal and second-hour serum glucose and insulin in oral glucose tolerance test (OGTT), basal serum leptin, sOb-R, resistin levels, and homeostasis model assessment (HOMA) indexes were compared between the groups. RESULTS: Age, sex, and pubertal status were similar in study groups. Median and interquartile ranges of body mass index (BMI) z scores were similar in HOB and OB groups (2.0 (1.5-2.1) and 2.1 (1.8-2.3), respectively). Serum leptin levels corrected for BMI were highest and total leptin/sOb-R ratios (free leptin index (FLI)) tended to be higher in HOB than HNOB and OB groups, indicating leptin resistance (leptin/BMI, 4.0 (1.6-5.2), 1.5 (0.8-3.1), and 2.5 (1.8-3.5); FLI, 2.0 (0.8-3.5), 0.6 (0.3-1.2), and 1.5 (1-2.3) in HOB, HNOB, and OB groups; respectively). Serum resistin levels were similar in groups (2.6 (1.9-3.1), 2.8 (1.7-3.4), and 3.0 (2.2-3.5) ng/ml in HOB, HNOB, and OB groups, respectively). Basal serum glucose, basal and second-hour insulin levels in OGTT, and HOMA index were higher in OB group than the HOB and HNOB groups, indicating insulin resistance in simple obesity; however, increment of insulin to same glycemic load in OGTT was highest in the HOB group indicating insulin dysregulation (p < 0.05). CONCLUSION: Hypothalamic obesity seems to be related to both dysregulated afferent (leptin) and efferent (insulin) neural outputs through the autonomic nervous system resulting in energy storage as fat.

Germ cell tumors of the basal ganglia and thalamus.

The clinicopathological findings in 6 patients with germ cell tumors originating in the basal ganglia and thalamus are presented. Clinical, biological and diagnostic features were somewhat different from germ cell tumors in the pineal region. Early and comprehensive treatment is recommended because of the possible presence of nongerminomatous germ cell components.

Early experience with the Gill Thomas Locator for computed tomography-directed stereotactic biopsy of intracranial lesions.

The Gill Thomas Locator is a stereotactic adaptor for the Brown-Roberts-Wells and Cosman-Roberts-Wells systems. It is a noninvasive device that relies on temporary fixation to the maxillary teeth. A series of 20 patients have had stereotactic biopsies with this system. A diagnostic biopsy was obtained in 19 cases. The frame was well tolerated, accurately relocatable, and allowed computed tomographic scanning and surgery to be conducted at different times.

[Osteosarcoma developing after radiation and chemotherapy for primary mediastinal seminoma].

This report presented an osteosarcoma arising from the same field after radiation and chemotherapy without surgery for primary mediastinal seminoma. A 45-year-old man had received from June 1979 to August 1981 irradiation and chemotherapy for the anterior-mediastinal seminoma. Since then he was been without evidence of disease for five years. In March 1987, he had suffered from an advanced tumor extending from the neck and the mediastinum to the right anterior chest wall. Further irradiation combined with thermotherapy were performed but without improvement. He was thereafter seen and admitted July 23 1987 to our hospital with complaint of back pain. Needle biopsy specimen revealed chondrosarcoma. Despite chemotherapy with CDDP, he died four months later after admission. Pathological finding of his autopsy revealed a widely invading osteosarcoma extending to extra- and intrathorax and the neck without evidence of germ cell component. To explain the pathogenesis of the secondarily developing osteosarcoma, two hypotheses are offered; 1. Malignancy of mesenchymal component in germ cell tumor. 2. Radiation-induced osteosarcoma. The latter hypothesis appears to be the most probable pathogenesis from his autopsy. These findings suggest that a long survival patient with malignant germ cell tumor after therapy should be never considered as being free from a potential risk of secondarily developing malignancy.

Differential diagnosis of testicular mass by cytological examination of seminal fluid collected by ejaculation or prostatic massage.

The cytological examination of the seminal fluid of 3 patients with painless testicular masses was performed using an ordinary conventional technique. Neoplastic cells appeared in the ejaculate or fluid from prostatic massage of all patients. The final pathologies were seminoma, choriocarcinoma and mixed teratocarcinoma. Malignant cells were no longer found in the seminal fluid after orchiectomy. This sample, noninvasive technique plus further flow cytometric study of cellular DNA contents is a great help in the preoperative differential diagnosis of testicular masses.

Testicular seminoma. Results of a program of megavoltage irradiation.

Between April 1962 and March 1984, 61 patients with a diagnosis of testicular seminoma were seen at Hahnemann University Hospital. Of these patients, 52 had long-term follow-up after definitive treatment with megavoltage irradiation. In 30 patients, disease was classified as stage I, in 21 patients, stage II, and in one patient, stage III. Treatment techniques are described. Overall relapse-free survival rate for all stages was 92% with an adjusted survival rate of 96%. Treatment policies for each stage are presented.

[A case of thalamic germinoma with crossed aphasia in a dextral (author's transl)].

We present a rare case of thalamic germinoma with crossed aphasia in a dextral. A patient, 17-year-old righat-handed male, was admitted to Nippon Medical School Hospital with chief complaints of headache, abnormality of visual field and speech disturbance. There were pigmentations on the back of hand, foot and the perineum. Neurological examination revealed left homonymous hemianopsia, right slight degree of ptosis, left facial palsy, a mild paresis of the left upper extremity and motor aphasia. Right carotid angiography showed marked unrolling and midline shift of right anterior cerebral artery. CT scan revealed ring-like high density area in the right thalamic region, which was enhanced after constant infusion. Brain scintigraphy also showed an abnormal accumulation at the same site. The hen-egg sized tumor of 40 g. weight was almost totally removed by the right fronto-parietal craniotomy. The tumor was characterized histologically by the so-called two cell pattern with teratomatous components. As postoperative treatment local injection of adriamycine, irradiation and immunotherapy with picibanil were performed, and then left hemiparesis was markedly improved without sign of recurrence. Language evaluation was performed after operation. There were dysarthria, remarkable word amnesia, paraphasia and perseveration. Repetition was also impaired. His speech function was concluded to be a mixed type aphasia mainly composed of Broca's aphasia. The speech function of thalamus and crossed aphasia with dextrales were discussed.

[Cerebral tumors of primitive germinal origin]. / Les tumeurs cerebrales primitives d'origine germinale.

The authors attempt to justify the term cerebral tumour of primitive germinal origin from four of their own cases and a review of the literature. They emphasise the specific features. The tumours are more common in boys and involve the pineal, the walls of the third ventricle, the hypothalamus and the posterior pituitary. Several histological types may be distinguished by the degree of differentiation but the stromal reaction, which is partly responsible for the symptoms, is always present. The clinical course of the illness is biphasic. The first is manifest by endocrine disorders and is of relatively long duration. Water homeostasis is always affected and may be associated with other hypothalamic disorders. In the second phase, neurological symptoms and raised intracranial pressure appear. Surgical removal is not always possible, but radiotherapy improves the outlook.