Atypical Glomus Tumor in the Mediastinum: A Case Report with Immunohistochemical and Ultrastructural Studies

A case is reported of atypical glomus tumor occurring in the posterior inferior mediastinum of a 26-year-old woman complaining of severe back pain. The tumor was composed of atypical small, round tumor cells with scattered mitotic figures. In addition to sheet-like, diffuse proliferation of the tumor cells, some areas of the tumor contained small “glo-moid” cells arranged in organoid and hemangiopericytoma-like patterns. Immunohistochemically, many tumor cells were positive for muscle-type actins and a few cells were focally positive for desmin. Ultrastructural studies revealed smooth muscle features of tumor cells, that is, pinocytotic vesicles, external laminas, dense plaques, and occasional thin filaments with dense bodies. The patient remained well for 5 years and 4 months after the operation without additional radiation and chemotherapy. The tumor was diagnosed as an atypical, or low-grade malignant, glomus tumor morphologically. It seems important to recognize the presence of this type of tumor in sites other than extremities and to differentiate it from other malignant small, round cell tumors.     

Ullrich-Turner syndrome and anorexia nervosa

We report on a 6-year-old girl with Ullrich-Turner syndrome and anorexia nervosa. The diagnosis was made at 6 years and she became anorectic at 14 years. She had been treated with low doses of estrogen just before the onset of anorexia. In spite of remarkable decrease in food intake, her body weight was in the normal range compared to standard weight. Rohrer indices were also normal, probably due to abnormal habitus in individuals with the syndrome. The pathogenetic relationship between this disorder and the hormone treatment in the onset of anorexia nervosa is discussed.     

Hypofluorescent spots in indocyanine green angiography of peau d‘orange and fundus

Purpose. Hypofluorescent spots were seen inindocyanine green (ICG) angiography of peau dorangefundus in eyes with angioid streaks. Origin of the hypofluorescentspots were examined with attention to their correlationwith a peau dorange appearance of the central fundususing a computer-assisted image comparison system. Methods. ICG angiography was performed in 5 patientshaving peau dorange appearance of fundus using ascanning laser ophthalmoscope (SLO) and a digitalvideo-fundus camera. The same central fundus areas corresponding to hypofluorescent spots in an ICGangiogram were then digitally identified in afluorescein angiogram and in a red-free picture in all10 eyes of the 5 patients. Monochromatic lightobservation was also performed with a dark fieldobservation using a SLO to see subretinal orintrachoroidal pigment clumping. Results. In no patient, the areas identified withhypofluorescent spots did show relevant changes ina fluorescein angiogram or a red-free picture. SLOexamination revealed not perfusion defect at the sameareas. The dark field observation showed no pigmentclumping at the peripapillary and papillomacularbundle regions where hypofluorescent spots were seen.Conclusions: Hypofluorescent spots seen in ICGangiograms did not show exact consistency with peau dorange changes in their location and shape. Perfusion defects or blocking by pigments were not acause of hypofluorescent spots. The scatteredhypofluorescent spots were considered to be relevantwith irregular affinity of the fundus to ICG dye.     

Microsomal Cytochrome P-450 Monooxygenase System and Its Drug-metabolizing Activity after Partial Portal Vein Ligation in the Rat

RID=" ID=" <E5>Correspondence to:</E5> K. Izuishi, M.D.     

A case of female alcoholic who developed liver cirrhosis with small amounts of alcohol by the common use of contraceptive agent]

A case of female alcoholic who developed liver cirrhosis with small amounts of alcohol by the common use of contraceptive agent was reported. A case was a 33-year-old female who had complained of systemic edema and jaundice. She had been drinking alcohol, while she had been taking the contraceptive agent from 20-year-old. On admission, she had a large amount of ascites with jaundice. She was diagnosed as liver cirrhosis and hepatic failure by CT scanning of abdomen and laboratory data. Her condition was temporarily improved by the abstinence and the treatment. Since she drank under hospitalization, she had to change the hospital and died after 2 months. She had been drinking for only 10 years. Her cumulative alcohol intake was also very small. She may have developed alcoholic cirrhosis with small amount of alcohol because of common use of contraceptive agent with drinking.     

Gene Amplification of int-2 and erbβ in Human Esophageal Cancer: Relationship to Clinicopathological Variables

Gene amplification is a relatively frequent event in human malignant tumors and is believed to play an important role in tumor progression. The int-2 and erbB genes are amplified more frequently than any other genes in human esophageal cancer. In order to investigate the correlation between these two proto-oncogenes and the clinical behavior of esophageal cancer, we examined DNA amplification of int-2 and erbB and analyzed their relationship to clinicopathological variables. Genomic DNA was extracted from 21 esophageal squamous carcinomas and normal esopfiageal mucosa, as well as from 4 metastatic tumors. We used Southern blot analysis for detection of gene amplification. Amplification of int-2 was observed in 13 of 21 cases (62%) and in all the metastatic tumors (4/4; 100%). We found a significant correlation between amplification of int-2 and the length of the primary lesion. Amplification of erbB was detected in 3 of 18 patients (17%). All patients who showed amplification of erbB also demonstrated amplification of int-2. These results suggest that amplification of int-2 or neighboring genes on 11q may participate in tumor progression and metastasis in patients with esophageal squamous cancer.     

No Improvement of Adult Height in Non-growth Hormone (GH) Deficient Short Children with GH Treatment

It is still in doubt whether the standard-dose growth hormone (GH) used in Japan (0.5 IU/kg/week, 0.167 mg/kg/week) for growth hormone deficiency is effective for achieving significant adult height improvement in non-growth hormone deficient (non-GHD) short children. We compared the growth of GH-treated non-GHD short children with that of untreated short children to examine the effect of standard-dose GH treatment on non-GHD short children. GH treatment with recombinant human growth hormone (rhGH) was started before the age of 11 yr in 64 boys and 76 girls with non-GHD short stature registered at the Foundation for Growth Science who have now reached their adult height. In 119 untreated boys and 127 untreated girls whose height standard deviation score (SDS) was below –2 SD at the age of 6 yr, height growth was followed until 17 yr. Height SDS was significantly lower before GH treatment in the GH-treated group than at the age of 6 yr in the untreated group, in both sexes. Adult height and adult height SDS were significantly greater in the untreated group than in the GH-treated group, in both sexes, although the change in height SDS did not differ significantly. Height SDS was significantly lower before GH treatment in the GH-treated group than at the age of 6 yr in the untreated group, so 57 boys and 57 girls whose height SDS at the age of 6 yr in the untreated group closely matched the height SDS before GH treatment in the GH-treated group were chosen for comparison. Height SDS did not differ significantly between the GH-treated group before GH treatment and the untreated group at the age of 6 yr, nor were there differences between these subgroups in adult height, adult height SDS, or height SDS change, in either sex. The effect of GH treatment is reported to be dose-dependent and doses over 0.23 mg/kg/week are reported to be necessary to improve adult height in non-GHD short children. Currently, the GH dose is fixed at 0.175 mg/kg/week in Japan, and we expected to find, and indeed concluded, that ordinary GH treatment in Japanese, non-GHD short children does not improve adult height.     

Left upper lobe bronchus reimplantation for nonpenetrating thoracic trauma

Trauma to the tracheobronchial tree has been diagnosed and treated with increasing frequency over the last several decades. However, most reports have dealt with management of injuries to the trachea and main stem bronchi, as approximately 80% of blunt tracheobronchial injuries occur within this area. With few exceptions, injury to the lobar bronchi has resulted in thoracotomy and lobectomy. We describe a patient with an injury to the left upper lobe bronchus who presented with delayed obstruction of the airway by fibrogranulation tissue. A successful segmental resection of the bronchial occlusion with reimplantation was performed, thereby preserving the patient's otherwise normal left upper lobe. This case demonstrates that resection and reimplantation of an injured lobar bronchus are feasible, even in a delayed setting.     

A dose-finding study of glycosylated G-CSF (Lenograstim) combined with CHOP therapy for stem cell mobilization in patients with non-Hodgkin's lymphoma

BACKGROUND: Peripheral blood stem cell (PBSC) reinfusion has been widely used for hematopoietic reconstitution after high-dose chemotherapy. However, the optimal dose of granulocyte colony-stimulating factor (G-CSF) for PBSC mobilization in combination with chemotherapy for autograft remains unknown. METHODS: To find the optimal dose of glycosylated G-CSF (lenograstim) for PBSC mobilization in combination with chemotherapy for aggressive non-Hodgkin's lymphoma (NHL), we conducted a dose-finding study on 43 newly diagnosed patients who had unfavorable prognostic factors. They received four to six courses of cyclophosphamide, doxorubicin, vincristine and prednisolone combined with lenograstim every 2 weeks (biweekly CHOP therapy). PBSC apheresis was started after the third course of biweekly CHOP therapy. Lenograstim was given daily from day 3 until the day of the last apheresis. The optimum dose of lenograstim was assessed based on mobilization efficacy and safety profiles at a daily single dose of 2, 5 and 10 microg/kg for eight patients in each level. RESULTS: The collected number of CD34+ cells in the first apheresis products was higher in the 5 microg/kg group than in the 2 microg/kg group (median, 4.22 x 10(6) vs 2.49 x 10(6) CD34+ cells/kg, P = 0.051). The highest dose of 10 microg/kg (median, 2.99 x 10(6) CD34+ cells/kg) failed to show a dose dependence in PBSC mobilization. The efficacy and safety of the 5 microg/kg dose were further confirmed in an additional 19 patients. CONCLUSIONS: The present study suggests that the recommended dose of lenograstim for PBSC mobilization with CHOP therapy in untreated NHL is 5 microg/kg.     

Synthesis and photophysical properties of photostable 1,8-naphthalimide dyes incorporating benzotriazole-based UV absorbers

We developed a series of blue-emitting 1,8-naphthalimide dyes covalently attached to 2-(2-hydroxyphenyl)-2H-benzotriazoles that retard photodegradation of the fluorophore. The dyes displayed weaker fluorescence emissions than the parent 1.8-naphthalimide. Quantum chemical calculations suggested that the decreased fluorescence was caused by the nonradiative deactivation promoted through the excited state intramolecular proton transfer (ESIPT) in benzotriazole components. The dyes'' phosphorescences in a degassed solution at 77 K were more efficient than that of the parent 1.8-naphthalimide, indicating a possible deactivation pathway through intersystem crossing. PMMA films doped with these dyes showed higher resistance against photoaging than the film doped with an equimolar mixture of constituent 1.8-naphthalimide and the benzotriazole derivatives. Thus, the covalently linked benzotriazole units slow fluorophore degradation not only by preferential absorption of harmful UV light, which is found in the film with a simple mixture of two components, but also by the nonradiative deactivation involved in benzotriazole units.

Highly photostable blue fluorescence dyes were developed by hybridization of 1,8-naphthalimides with benzotriazole-based UV absorbers enabling a non-radiative energy dissipation process of excited-state intramolecular proton transfer (ESIPT).