[Multiple myeloma with hyperammonemia].
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Biomedical subjects
Publications and source records attributed to A Horie.
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We report a 69-year-old woman who presented with dystonic movement in the left upper limb. She also had left hemiparesis and sensory disturbance in the right face and the left half of the body, pseudoathetosis in the left hand, and hotness, swelling, and lead-pipe rigidity in the left upper limb. The dystonic movement was presented mainly in the proximal part of the left upper limb, and was induced by voluntary movements; for example, when she was ordered to shake hands, the left shoulder always abducted with the flexion of the left elbow. Brain MRI showed a fresh hemorrhage in the dorsal part of the right middle pons including the base and the tegmentum, old infarctions in the right postero-lateral thalamus, putamen, and right parietal lobe. The dystonic movement persisted for 2 weeks, and hotness and swelling in the left upper limb lasted for 2 months, while the rigidity and pseudoathetosis persisted for 7 months or more despite medication.
In this morphological study the authors investigated whether spinal cord cavitation, produced in young mongrel dogs that had been rendered hydrocephalic by cisternal injection of kaolin, consists of a dilated central canal or intramedullary cavities. Hydrocephalus was noted in 50 of 56 dogs treated with kaolin. Of the 50 hydrocephalic young dogs, 29 were shown to have central canal dilation that was prominent at the thoracic level and 21 to have cervical intramedullary cavities in the posterior column and/or the posterior horn. In 11 dogs from the latter group these cavities were demonstrated to have no communication with the central canal. This finding could not be explained by the hydrodynamic theory. On histopathological examination, myelomalacia and hemorrhagic infarction following ventricular shunting were noted adjacent to the cervical cavities, which suggested vascular impairment. A perfusion study revealed insufficient blood flow within the cervical cord at the level of the intramedullary cavities. A close correlation between the vascular insufficiency of the cervical cord and the pressure cone resulting from significant hydrocephalus was observed. The latter may cause cervicomedullary compression at the foramen magnum, affecting the venous drainage of the cervical cord below that level, resulting in intramedullary cavitation. Accordingly, vascular impairment was thought to play a significant role in the development of cervical syrinx formation in our kaolin model. The current results may provide a reasonable explanation for the formation of noncommunicating cervical syringomyelia in Chiari I malformation.
We have previously reported that a novel enzyme, L-2,4-diaminobutyrate decarboxylase (DABA DC), which is responsible for the formation of 1,3-diaminopropane, occurs in two Acinetobacter species. The present study extends this observation to additional Acinetobacter species and strains (6 reference strains and 30 clinical isolates). Furthermore, the DABA DC protein was detected in every strain by Western blot analysis with the antiserum against the enzyme purified from A. baumannii ATCC 19606. However, only the DABA DCs in the A. calcoaceticus and Acinetobacter genospecies 3 in addition to A. baumannii strains strongly cross-reacted with the antiserum, suggesting antigenic heterogeneity among the DABA DC proteins in Acinetobacter species. Therefore, immunological testing of the DABA DC protein may provide an additional method for differentiating and identifying Acinetobacter strains.
A cholangiocarcinoma of the hepatic hilus with an element of giant cell tumor that occurred in a 59-year-old man is reported. His medical history included systemic cholelithiasis and repeated operations on the biliary passages. Four years after the last operation, which was a hepatic segmentectomy, he was readmitted because of persistent fever. A computed tomography scan showed a low-density area and stones in the hepatic hilus. He died of hepatic failure approximately 1 month later. At autopsy, a fist-sized tumor and gallstones were found at the hepatic hilus. Histologically, the tumor mainly showed sarcomatoid features and some tubular adenocarcinoma. An element of giant cell tumor consisting of many osteoclast-type giant cells also was noted. The results of immunohistochemical studies showed a positive reaction to cytokeratin and vimentin in some of the spindle-shaped sarcomatoid cells. Sarcomatoid bile duct carcinomas are rare, as are those with osteoclast-type giant cells. The authors also discuss the histogenesis of these giant cells.
One hundred and thirty-five cases of basal cell carcinoma (BCC) were investigated, focusing upon factors determining a postoperative prognosis. Out of the 135, nine tumors on the face recurred. All of these nine tumors were insufficiently extirpated at the initial operation, and showed micronodular or infiltrative patterns with stromal fibrosis. Dividing the degree of dermal invasion into four levels, all tumors with recurrence reached levels 3 and 4, the two deeper groups. The immunohistochemical study using anti-laminin and anti-type IV collagen antibodies showed various changes of staining pattern around tumor cell nests, such as attenuation, disruption, and thickening of basement membrane, in contrast with the normal thinly continuous staining around nontumorous control epidermis. The disruption of basement membranes was remarkable around the tumor cells showing a micronodular growth pattern, although the discontinuity of basement membrane was observed in all types of BCCs to a greater or lesser degree. Ultrastructural thickening, multiplication, or discontinuity of basement membranes were found in all 19 cases examined with a greater or lesser degree, although they were most frequently observed around the cell nests showing micronodular growth patterns. It was concluded that deep dermal and marginal invasions were the most ominous signs of recurrence of BCCs. Although the disruption of basement membranes might participate in the local aggressiveness of BCCs, especially in the tumor cells showing micronodular infiltrative growth, other factors may concern the recurrence of BCCs.
A rare gastric carcinoma containing diverse components, that is, neuroendocrine (small cell carcinoma), squamous and gland-like elements in an 82 yr old woman is described. Radiologic examination revealed a large ulcerated tumor, and a Borrmann type II tumor, 6.5 x 5 cm, was found in the resected stomach. Histologically, the tumor was mainly composed of small cells with hyperchromatic nuclei and scant cytoplasm. Argyrophilic granules were seen in these cells. There were also scattered foci of large cells with features of squamous cells, and many intermediate cells with oncocytic cytoplasm. The small cancer cells were positive for chromogranin A and neuron specific enolase. Squamous cell nests were positive for high molecular cytokeratin (CK), and intermediate cells were positive for low molecular CK. Electron microscopic examination revealed secretory granules in the small cells and tonofilaments in the squamous cells. This tumor might have originated from the pluripotential stem cell in the gastric epithelium.
The morphological mechanism of the reconstitution of shunted mantle was studied histopathologically in 22 kaolin-treated hydrocephalic puppies. A remarkable attenuation of cerebral mantle to less than 1 cm in thickness was seen on computerized tomography (CT) scans of four animals sacrificed 1 to 2 months after kaolin treatment (preshunt group). Ventricular shunting resulted in successful recovery of the mantle on repeated CT scans obtained 1 to 2 months after shunting in seven animals (postshunt group). In the remaining 11 animals the cerebral mantle, which had been reduced to 4 mm in thickness prior to shunting, failed to recover even 2 months after the procedure (shunt-refractory group). On gross inspection, the preshunt specimens showed marked thinning of the white matter, with the cortical ribbon well preserved, while the postshunt specimens consisted predominantly of thickened white matter. Histopathological examination of the attenuated white matter of the preshunt specimens showed decreased nerve-fiber density, myelin destruction with myelin regeneration and/or repair of myelin sheaths, and reactive astrocytosis, which were prominent especially in the periventricular white matter. The main findings in the reconstituted white matter of the postshunt specimens were extensive myelin regeneration of residual axons and remarkable astroglial proliferation with mesenchymal reaction, particularly at capillaries. No clear evidence of increased numbers of nerve fibers or axonal regeneration was observed. The shunt-refractory specimens showed remarkable attenuation of cortex, in which reduced numbers of neurons and loss of cortical lamination were noted, with vestigial white matter. The results indicate that astroglial proliferation with mesenchymal reaction and myelin regeneration contribute to the reconstitution of the cerebral mantle volume following ventricular shunting in this model. It is suggested that the critical factor for mantle reconstitution in chronic hydrocephalus is whether cortex is preserved.
Transmission and scanning electron microscopic examinations were performed to evaluate the fine-structural differences in sinusoids of hepatocellular carcinoma, adenomatous hyperplasia of the liver, and fetal livers. In cancerous sinusoids, thickened endothelial cells with loss of sieve plates were conspicuous features. In the sinusoids of adenomatous hyperplasia, scanning electron microscopy showed apparent sieve plates, and basement membrane formation (capillarization) was less conspicuous than in carcinoma. In fetal livers, occasional large gaps as well as small fenestrations in the endothelium were noted. Basement membrane materials were not seen. It was concluded that the sinusoids of hepatocellular carcinoma lost the fine-structural nature of normal sinusoids and showed much more prominent capillarizations than the sinusoids of adenomatous hyperplasia, and that the fine structures of cancerous sinusoids were also different from those of the sinusoids in fetal livers.
We report the case of a 65-yr-old woman with hyalinized hemangioma of the liver which, on radiological examination, resembled primary or metastatic carcinoma of the liver. She had undergone a partial colectomy for a sigmoid adenocarcinoma, followed by the diagnosis of a hepatic tumor with ultrasonic echogram 5 months later. The tumor was depicted as a low-density mass on plain computed tomography (CT), and an enhancement at the peripheral portion was noted by contrast CT. Hepatic angiography disclosed a faint pooling of contrast medium in segment 8. A subsementectomy of the liver was performed under the diagnosis of metastatic adenocarcinoma or hepatocellular carcinoma. Histologically, the tumor was composed of dense collagenous tissues with marked hyalinization and scattered sclerotic vessels. Elastic fibers were distributed concentrically around the vessels. Totally hyalinized sclerosis of hemangioma is uncommon, and can be erroneously diagnosed as carcinoma by radiologic examination. This unusual hemangioma is reported, with pertinent literature.
Many papers have reported that Dieulafoy's ulcer is one of the notorious causes of gastric hemorrhage. Three cases of shallow subfundic ulcers with massive bleeding are reported. The resected specimens have demonstrated that elevated caliber-persistent artery (CPA), a branch of the left gastric artery with few anastomoses, in the base of the ulcer has tortuous penetration from the serosa to submucosa, showing patchy, eccentric intimal fibroelastosis. These findings of CPA are almost the same in both anterior and posterior walls, namely both the ruptured and contralateral sides. Thus, morphogenesis of the ulcer may have originated from anatomical deviation, which is related to regional hypertension aggravated by longterm peristalsis, as well as aging.
Six cases of primary hepatic carcinomas with a significant amount of sarcomatoid elements were examined by using immunohistochemical stainings. Four of the six cases were associated with ordinary hepatocellular carcinoma (HCC), one with cholangiocellular carcinoma (CCC), and one with mixed HCC and CCC. Alpha-fetoprotein and alpha-1-antitrypsin were negative in sarcomatoid cells of all cases; vimentin stained positively in sarcomatoid tumor cells in two of the six cases; and cytokeratin (CK8) was detected in five cases. The CK8 was not detected in tumor cells of two cases of hepatic angiosarcoma, two of metastatic leiomyosarcomas, and one of metastatic fibrosarcoma, although vimentin stained positively in all these true sarcomas. It was concluded that sarcomatoid dedifferentiation of liver carcinomas might derive from both HCC and CCC. In addition CK8 might be an excellent marker to make a differential diagnosis of sarcomatoid cancers from true metastatic or primary sarcomas of the liver.
Giant cell carcinomas of the lung have been notorious in fulminant clinical courses. Thus, this report describes two exceptionally favorable cases among six cases of giant cell carcinoma of the lung. Their histopathological features are a sharply-demarcated tumor of Stage I, absence of vascular permeation of the cancer cells, prominent lymphoid and plasma cell infiltration in the tumor tissue, and lymph follicle formation in the surrounding tissues. Another case with a Stage II tumor showed the same histopathological findings as the above two cases with the exception of lymphatic permeation of the cancer cells. This patient expired about one year after undergoing an operation. As conventional controls, the remaining three cases with Stage III tumors showed an alveolar extension of tumor cells and vascular permeation. There was a fulminant course after the operation. Notwithstanding similar intervals from their clinical onset to operation in the 4 cases other than Cases 4 and 6, their stages showed considerable variations. Hence, each histopathological feature might have substantiated the different clinical courses following the operation. Electron microscopy of three of the cases indicated double-membrane-bound blisters with intermediate junctions in the bizarre giant cells, and cancer cell differentiation toward both glandular and squamous directions.
Malignant fibrous histiocytoma (MFH) can be classified as a pleomorphic mesenchymal sarcoma which occurs most commonly in the extremities and retroperitoneum. We report a very rare case of MFH presumably originating in the pulmonary artery and resembling recurrent pulmonary thromboembolism. MFH was suggested by transbronchial biopsy and confirmed by open lung biopsy. Obstruction of the right main pulmonary artery and stenosis of the left main pulmonary artery was confirmed by pulmonary angiography.
CT and US findings of 7 cases of splenic metastases are described and the prevalence of splenic metastases at autopsy in 641 cases with malignant tumors were evaluated. Metastatic foci in spleen appeared mostly as poorly-defined low density masses on CT. Iodinated contrast material was administered in 2 cases, but no contrast enhancement was observed. US showed both hypoechoic and hyperechoic patterns. These appearances were nonspecific, but were similar to those of metastatic lesions in the liver which were often visible on CT associated with splenic metastases. At autopsy splenic metastases were found in 34 of 641 cases (5.3%). Gastric, colon, lung and ovarian cancers were most common primary tumors. However, the rate of splenic metastasis per tumor was highest in ovarian cancer (50.0%), followed by malignant melanoma (33.3%), colon cancer (16.2%) and gastric cancer (8.2%). Hepatoma which had the biggest number of autopsy cases in this series showed the lowest rate of splenic metastasis (0.8%).
We present a case of a 71-year-old female with actinomycosis in the liver, which is a rare region to be primarily affected with actinomycosis. The diagnosis was done histopathologically with a partially resected liver specimen taken during surgery for choledocholithiasis. There were no clinical signs of Actinomyces infection before surgery. The hepatic lesion was a 2 cm sized nodule with histological appearances of abscess-forming suppurative inflammation with fibrosis, in which eosinophilic radiate granules with peripheral clubs were found. The Brown-Brenn stain showed Gram-positive branched filamentous bacilli, which were revealed as acid-fastness by the Ziehl-Neelsen method of Putt's modification. These findings were considered to be consistent with actinomycosis of the liver.
Twenty-four male rats of the Wistar strain divided into four groups were injected s.c. with a dose of 0.8, 1.5, and 3.0 mg Cd/kg body wt as CdCl2 in saline, and saline alone to the control rats, three times a week for 3 wk. Cadmium levels of whole kidney homogenate, supernatant (cytosol), precipitate, and metallothionein (MT) fraction were measured. Histological changes of the renal proximal tubules were investigated by optical and electron microscopy. In the kidneys, Cd levels were increased with the increment of Cd dosage; 80-90% of Cd was contained in cytosol, and 55-75% was in MT fraction. Non-MT-Cd reached a maximum in the 1.5 mg Cd group, whereas that of the 3.0 mg Cd group showed some decline. With increasing Cd doses, the size of nuclei and nucleoli in the cells of proximal tubule showed significant enlargement and also an increase in the number of nucleoli on light microscopy. At higher doses, chromatin condensation of the tubular nuclei and vacuolar degeneration of the tubular cells were evident. On electron microscopy, perichromatin granules of the proximal tubular nuclei were increased in number, especially in the rats of Cd 0.8 mg and 1.5 mg/kg groups. As the Cd doses increased, ring-shaped nucleoli were increased in number and nucleolar segregation was observed more clearly. Moreover, in the 3.0 mg/kg Cd group, nuclear indentation and nucleoli containing compact dense granules were observed. In the cytoplasm, there was an increase of lysosomes, myelin bodies, ring-shaped mitochondria, and vesiculation; ultimate changes were degeneration and cell necrosis. The injured cells were heterogenously distributed in each nephron and this heterogeneity was attributed in the difference in Cd content and cell cycle in each cell of the nephron.
A case of an extremely rare cystic lesion of the pancreas is presented. The multilocular cyst was found adjacent to the upper border of the pancreatic body, and the cyst contained bean curd lees-like substances. Histologically, the cyst wall consisted of mature keratinizing squamous epithelium and surrounding lymphoid tissue stroma, and the cyst was filled with keratinized materials. A histopathologic diagnosis of typical lymphoepithelial cyst of the pancreas, proposed by Truong et al (Am J Surg Pathol 11:899-903, 1987), was made. Its histogenesis is still unknown; however, we hypothesize that it might arise from a benign epithelial inclusion of a peripancreatic lymph node, followed by squamous metaplasia of the epithelial inclusion. We recently found a retropyloric lymph node with a squamous epithelial inclusion, which might support this hypothesis regarding the histogenesis of the cyst.