Ga-67 scintigraphy in a case of mitochondrial myopathy.
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Biomedical subjects
Publications and source records attributed to M Shintaku.
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A müllerian carcinosarcoma or malignant mesodermal mixed tumor (MMMT) originated from the retroperitoneum of a 51-year-old woman; only two previous similar cases have been reported. The 1,040 gm tumor was found in the left retroperitoneal space; the center of the tumor was extensively liquified. The uterus, fallopian tubes, and ovaries were unremarkable. The histopathological features of the tumor were typical of MMMT, homologous type. The adenocarcinomatous component of the tumor was immunohistochemically positive for cytokeratin 7 and negative for cytokeratin 20. MMMTs arising in extragenital sites are rare, and most of them arise from the peritoneum. The histogenesis of extragenital MMMTs remains speculative, but the origin from the "secondary mullerian system" is most likely.
To examine the role of cyclic ADP-ribose (cADP-ribose) as a second messenger downstream of angiotensin II (Ang II) receptor activation in the heart, ADP-ribosyl cyclase activity was measured in a crude membrane fraction of ventricular myocytes. Ang II at 10-100 nM increased ADP-ribosyl cyclase activity by 40-90% in the ventricular muscle of neonatal (2-4-day-old) rats, but not in fetal or adult hearts. This increase was inhibited by the Ang II antipeptide. Stimulation of ADP-ribosyl cyclase was reproduced by GTP and guanosine 5'-[gamma-thio]triphosphate, and prevented by guanosine 5'-[beta-thio]diphosphate. Prior treatment of the rats with cholera toxin A and B subunits also blocked the Ang II-induced activation. The density of Ang II receptors detected as [(3)H]Ang II binding was higher in neonatal than adult rats. These results demonstrate the existence of a signalling pathway from Ang II receptors to membrane-bound ADP-ribosyl cyclase in the ventricular muscle cell and suggest that the Ang II-induced increase in cADP-ribose synthesis is involved in the regulation of cardiac function and development.
Malignant lymphoma is rarely seen in the hand. We present a case of malignant lymphoma of the hand with Ga-67 citrate and MR images, and discuss the usefulness of Ga-67 citrate scintigraphy for diagnosing this condition.
A male patient, who was born with congenital cutis laxa characterized by cutaneous laxity due to the degeneration of elastic fibers, presented with an arrest of mental and motor development at the age of 3 years. The progressive decline of the psychomotor abilities led to the patient's death at the age of 4 years and 9 months. An autopsy revealed extensive white matter degeneration, characterized by the formation of numerous neuroaxonal spheroids and a diffuse loss of axons and myelin sheaths. The centrum semiovale and the cerebellar white matter were the most severely affected. The ultrastructure of the spheroids was consistent with a dystrophic type of axonal swelling. Neurons of the cerebral cortex, cerebellar cortex, and some brain stem nuclei were lost in moderate to severe degrees, and there were relatively few neuroaxonal spheroids in the gray matter. The pallidum and substantia nigra were well preserved. Neuroaxonal leukodystrophy, in which the spheroid formation predominantly affects the white matter, is the rarest variant of primary neuroaxonal dystrophies, and there are very few reports of autopsied cases. Among the reported cases, two Japanese siblings had congenital skin lesions similar to those of our case. The unique association of neuroaxonal leukodystrophy and congenital cutis laxa may form a distinct variant in this disease category.
A case of mucus-producing papillary adenocarcinoma of the renal pelvis associated with multiple renal calculi in a 16-year-old male is reported. The majority of the tumor cells contained large, round or cone-shaped, vimentin-positive intracytoplasmic inclusions, which exhibited some morphological and immunohistochemical resemblance to inclusions found in malignant rhabdoid tumor of the kidney. An intracytoplasmic accumulation of mucus was also noted in some tumor cells. The patient is free from recurrence and metastasis after a nephrectomy.
Infection of the cerebral cortical neurons with JC virus (JCV) with possible dysplastic ganglion-like alteration of the infected neurons found in a case of progressive multifocal leukoencephalopathy (PML) is described. The patient was a 21-year-old man with common variable immunodeficiency who died of PML after a 9-month clinical course. At autopsy, the white matter of the cerebrum, brainstem, cerebellum, and spinal cord exhibited extensive demyelination and necrosis. Numerous inclusion-bearing oligodendrocytes and bizarre astrocytes were found. In the occipital and temporal cortex, thick band-like aggregates of dysplastic ganglion-like cells (DGLCs) were found. These DGLCs showed immunohistochemical properties of neurons, and nuclei of some DGLCs were immunoreactive for large T antigen of SV40/JCV and p53, but not for capsid protein JCV VP1. In situ hybridization for mRNA of JCV large T antigen revealed positive signals in the nuclei of some DGLCs. These results indicate that JCV infected neurons and it is suggested that binding of the large T antigen with cellular proteins could have resulted in the dysplastic, ganglion cell-like change of the infected neurons, although the possibility that the aggregates of DGLCs represent a pre-existent malformative lesion of the cortex cannot be excluded completely.
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A case of adenocarcinoma of the uterine cervix that showed choriocarcinomatous and hepatoid differentiation was encountered in a 65-year-old woman. She presented with genital bleeding and had multiple metastatic nodules in the lungs. At operation, a large, hemorrhagic, and necrotic tumor was found in the uterine cervix. The major portion of the tumor consisted of typical choriocarcinoma admixed with minor areas of hepatoid carcinoma and endocervical adenocarcinoma. Human chorionic gonadotropin and alpha-fetoprotein were detected in tumor cells in the choriocarcinomatous and hepatoid areas, respectively. The patient died of pulmonary metastasis 4 months after the operation. The coexistence of choriocarcinomatous and hepatoid carcinoma in an endocervical adenocarcinoma has not been reported previously. Both heterotopic components were probably derived from aberrant differentiation (or neometaplasia) of the somatic epithelial cells of the endocervical adenocarcinoma.
A surgical case of glioblastoma which showed a pronounced "adenoid" (or "epithelioid") appearance was reported. The patient was an 81-year-old woman, who presented with unsteady gait. Neuroradiological examination revealed three discrete mass lesions located in the 1-frontal, 1-parieto-occipital, and r-occipito-temporal lobes. Despite the subtotal removal of two of the three lesions and postoperative chemotherapy and radiotherapy, the patient died 21 months after the onset of illness. Histopathological examination of the resected tumors revealed typical features of glioblastoma in the peripheral region of the tumor. In the central region, the tumor cells were arranged in a papillary fashion or formed solid, sheet-like cell nests and were surrounded by fibrous connective tissue septa. Although the histopathological appearance of the tumor closely resembled metastasis of adenocarcinoma, the immunohistochemical and ultrastructural studies of the tumor failed to detect evidence of a definite differentiation towards epithelial cells.
Investigation of the leaves of Fraxinus americana led to the isolation of five secoiridoid glucosides, demethylligstroside, (2"R)- and (2"S)-2"-hydroxyoleuropeins, fraxamoside and frameroside, together with 18 known compounds. Their structures were determined on the basis of spectroscopic studies and chemical evidence.
Investigation of the leaves of Fraxinus americana led to the isolation of five secoiridoid glucosides, demethylligstroside, (2"R)- and (2"S)-2"-hydroxyoleuropeins, fraxamoside and frameroside, together with 18 known compounds. Their structures were determined on the basis of spectroscopic studies and chemical evidence.
BACKGROUND: Chemotherapy with methotrexate (MTX), vinblastine, doxorubicin, and cisplatin (M-VAC) is reported to be the most effective regimen for urothelial carcinoma. Complete response (CR) is observed in many cases. However, to the authors' knowledge there is no alternative therapy for nonresponders. Thus, the authors attempted high dose chemotherapy (HDC) supported with peripheral blood stem cells (PBSCs) collected after a modified method of mobilization to yield sufficient PBSCs for the HDC regimen employed. METHODS: PBSCs were collected from ten patients, all of whom had recurrent and/or refractory transitional cell carcinoma. They were treated by modified M-VAC (with pirarubicin in place of doxorubicin) for PBSC harvest. Seven micrograms per kilogram of body weight of granulocyte-colony stimulating factor was injected subcutaneously daily from Day 10 of treatment to the end of the harvest. Harvest was initiated from the day when peripheral leukocyte counts exceeded 10,000/microL and usually continued for 3 consecutive days. Each patient received two courses of HDC. Therefore, 20 courses of HDC comprised of 300 mg/body of MTX, 1500 mg/m(2) of etoposide, and high dose carboplatin (CBDCA) were given to these 10 patients. The dose of CBDCA was determined by the formula of Calvert et al., in which the target area under the concentration versus time curve of CBDCA was adjusted to 21 mg. minute/mL. RESULTS: Sufficient PBSCs were collected for myeloablative chemotherapy in all patients. No patient responded to the treatment with modified M-VAC. Response to HDC was observed in nine of ten patients. CR was achieved in seven patients and a partial response was noted in two patients. A patient with multiple bone metastases showed no response. All patients rapidly recovered after PBSC transplantation. No patient died of treatment-related toxicity. CONCLUSIONS: HDC supported by PBSC transplantation was found to have a remarkable response against refractory urothelial carcinoma, for which there was no alternative therapy.
A surgical case of gliosarcoma in which the mesenchymal component showed angiosarcomatous features is reported. The neoplasm was extirpated from the right cerebral hemisphere of a 76-year-old man who presented with left hemiparesis and headache, and it was composed of both anaplastic astrocytomatous and sarcomatous elements. The sarcomatous element was composed of a proliferation of atypical endothelial cells that had pleomorphic, mitotically active nuclei and formed vascular lumina of irregular shapes or solid cellular sheets, and was accompanied by the deposition of collagenous matrix. The cytoplasm of some of the atypical endothelial cells was immunohistochemically positive for vimentin, factor VIII-related antigen, CD 34, and thrombomodulin. The MIB-1 labeling index of the sarcomatous component was higher than that of the astrocytomatous component.
67Ga-citrate and 99mTc(V)-DMSA images were obtained in a patient with recurrent extraabdominal fibromatosis of the left calf. The 67Ga-citrate image showed abnormal uptake in the left calf lesion and right calf, which was considered due to probable myositis caused by overexertion. On the other hand, the 99mTc(V)-DMSA image showed abnormal uptake only in the left calf lesion. Surgical and radiation therapy was performed. Two years after the treatment, two lesions of the recurrent extraabdominal fibromatosis followed, and a 99mTc(V)-DMSA image could demarcate the two lesions. 99mTc(V)-DMSA images were considered to be useful in following up of recurrent extraabdominal fibromatosis.
We present a case of neuropsychiatric lupus erythematosus with granular calcification in the basal ganglia and cerebral white matter on CT. Histopathologically, these were identified as perivenous necrotising lesions, with loss of axons and myelin sheaths and prominent dystrophic calcification.
The patient, a 22-year-old woman who had been treated for systemic lupus erythematosus (SLE) for 10 years, was hospitalized for arthralgia, melena, and difficulty in walking. CT examination of the brain showed grain-like high-density lesions scattered throughout the cerebral white matter and basal ganglia. At autopsy, multiple perivenous, well-demarcated foci of brownish discoloration were seen scattered throughout the cerebral white matter and basal ganglia. Histopathologically these lesions consisted of foci of coagulation necrosis surrounding the veins. The veins in the foci showed fibrous thickening of the walls, but there were no indications of vasculitis. At the periphery of the lesions, the axons were better preserved than their myelin sheaths. The neuropathological findings in the present case closely resemble those of acute disseminated (perivenous) encephalomyelitis, although an inflammatory cell infiltration had apparently already subsided. Although its pathogenesis remains unclear, this finding should not be regarded as an incidental complication but rather as a rare subtype of central nervous system lesion occurring with SLE.