Upregulation of HSP47 and collagen type III in the dermal fibrotic disease, keloid.

Keloid is a dermal fibrotic disease characterized by excessive accumulation of mainly type I collagen in extracellular matrix of the dermis. We have studied the expression levels of collagen types I and III, and its molecular chaperone HSP47 in keloid lesions and surrounding unaffected skin using Northern and Western blotting and immunohistochemical analyses. Collagen types I and III mRNA levels were found to be upregulated 20-fold in keloid tissues, contradicting previous reports of nearly normal type III collagen levels in this disease. HSP47 expression in keloid lesions was also highly upregulated; eightfold at mRNA level and more than 16-fold at the protein level. Strong upregulation of these three proteins in keloid was confirmed by immunohistochemical staining. These results suggest that accumulation of both type I and type III collagen is important for the development of keloid lesions, and that HSP47 plays a role in the rapid and extensive synthesis of collagen in keloid tissues.

Massive rectal bleeding due to ileal tuberculosis.

A patient with massive rectal bleeding due to ileal tuberculosis is reported. Technetium-99m labelled red blood cell scintigraphy indicated hemorrhage from the ileum, and laparotomy was then carried out. A 70-cm segment of ileum containing ulcers and erosions was resected, and epitheloid granuloma with Langhans-type giant cell was found in the resected specimen. Massive rectal bleeding is considered a rare presenting symptom of intestinal tuberculosis. Intestinal tuberculosis, including small intestinal tuberculosis, although uncommon, should be taken into consideration as a cause of rectal bleeding.

Prenatal diagnosis of prune belly syndrome at 12 weeks of pregnancy: case report and review of the literature.

We present a case of prune belly syndrome in a 12-week fetus whose previous anomaly scan at 10 weeks had been normal. The ultrasound diagnosis was based on the findings of a lower abdominal cystic echo caused by abnormal dilatation of the bladder. Termination was performed at 14 weeks and autopsy confirmed the distended bladder. In addition, there was bilateral hydronephrosis and an absence of abdominal muscles, liver, spleen and diaphragm. A review of the literature indicates that ours may be the earliest reported case of prune belly syndrome.

[A case of deep sylvian meningioma presenting temporal lobe epilepsy].

A rare case of deep sylvian meningioma is presented. A 62-year-old woman was admitted to our hospital because of one year history of temporal lobe epilepsy. She had no neurological deficit except for EEG abnormality. CT scans showed a small calcified mass in the left temporal lobe adjacent to the sylvian fissure with no enhancement by contrast medium. The mass was low-intense in both T1- and T2-weighted MR images. The T1-weighted image after the infusion of gadolinium revealed enhancement of the middle cerebral artery adjacent to the mass, similar to dural tail sign. Left external carotid angiography did not show any tumor stain nor the dilatation of the middle meningeal artery. Left internal carotid angiography disclosed enlarged middle cerebral artery without tumor stain. A left frontotemporal craniotomy was performed and the mass was totally removed. The tumor was located deep in sylvian fissure without any connection to the dura or ventricular system, which was firmly adherent to the middle cerebral artery. The histological examination of the surgical specimen revealed a psammomatous meningioma. Meningiomas are believed to originate from the arachnoid cap cells and can arise from various intracranial locations where arachnoid cap cells exist. The majority of them are attached to the dura, choroid plexus, or the tela choroidea. Only eleven cases of deep sylvian meningiomas have been presented in the literature. We have reviewed the clinical and radiological findings in such meningiomas. MR findings in deep sylvian meningioma have not been described.(ABSTRACT TRUNCATED AT 250 WORDS)

Lymphangioma of the duodenum: report of a case.

A case of lymphangioma of the duodenum with a successful preoperative diagnosis is reported herein. A 76-year-old man who complained of tarry stool was found to have a hemorrhagic polypoid tumor in the third portion of the duodenum. The tumor was diagnosed histologically as cavernous lymphangioma by endoscopic biopsy. Since such a tumor is essentially benign, a partial resection of the duodenum including the tumor was performed. Therefore, an endoscopic biopsy seems to be valuable in the diagnosis of duodenal lymphangioma.

E-selectin and vascular cell adhesion molecule-1 mediate adult T-cell leukemia cell adhesion to endothelial cells.

We studied the adhesion properties of peripheral blood leukemic cells from 10 patients with adult T-cell leukemia (ATL) to endothelial cells to better understand the mechanism of leukemic cell infiltration. ATL cells expressed lymphocyte function-associated antigen-1 (LFA-1), but the expression of very late antigen-4 (VLA-4) and sialyl-Lewisx (SLex) was variable. They did not express sialyl-Lewisa (SLea). Cell adhesion assays, which were performed in nine patients, showed marked adhesion of ATL cells to interleukin [IL]-1-activated human umbilical vein endothelial cells (HUVEC). A monoclonal antibody (MoAb) against E-selectin consistently inhibited ATL cell adhesion, and an MoAb against vascular cell adhesion molecule-1 (VCAM-1) or an MoAb against VLA-4 sometimes diminished it. In contrast, an MoAb against LFA-1 had a minor effect on freshly isolated ATL cell adhesion to HUVEC. The percentage of SLex+ cells in the cell population adherent to IL-1-activated HUVEC was slightly higher than that in unseparated cells. These results, together with the detection of E-selectin expression on the endothelium at ATL skin lesions, indicate that E-selectin-mediated adhesion is the major pathway for the adherence of ATL cells to endothelial cells. In addition, the ligand for E-selectin on ATL cells appears to differ from that on neutrophils.

Hepatic angiomyolipoma pre-operatively diagnosed by imaging.

An asymptomatic patient with pre-operatively diagnosed solitary angiomyolipoma is reported. The tumour was a well defined, fat density mass on computerized tomography (CT) and magnetic resonance imaging (MRI), and a hyperechoic nodule with a sharp margin on ultrasonography (US). The lesion was hypervascular on arteriography and CT during hepatic angiography. Chemical shift MRI confirmed the fat component within the lesion. Although a review of the literature shows this tumour to be rare and difficult to differentiate from a malignant lesion pre-operatively, the pre-operative diagnosis of angiomyolipoma is considered feasible when the characteristic findings as described here are present.

Carcinosarcoma of the esophagus: a report of two cases.

Among 137 cases of esophageal neoplasms surgically treated at Kobe City General Hospital from 1983 to 1990, there were two patients with a huge polypoid lesion identified as carcinosarcoma by light microscopic examination, both of whom underwent radical resection and esophagogastrostomy. Microscopic examination of the resected specimens revealed the tumors to be composed of carcinomatous and sarcomatous elements. Additional immunohistochemical examination disclosed keratin-positive cells in the carcinomatous element and vimentin-positive cells in the sarcomatous element. In case 1, keratin-positive cells were also found in the sarcomatous element, which suggested that the sarcomatous cells were derived from epithelial cells. Despite the huge size of the tumors, the depth of invasion to the esophageal wall was limited to the mucosal layer in case 1 and the submucosal layer in case 2, and there has been no evidence of recurrence in either case since surgery.

Des-gamma-carboxy prothrombin (PIVKA-II) and alpha-fetoprotein-producing IIc-type early gastric cancer.

We describe the case of a 56-yr-old man with primary gastric adenocarcinoma, who had an extremely high plasma level of des-gamma-carboxy prothrombin (2.45 AU/ml) and of serum alpha-fetoprotein (2810 ng/ml). Histopathologically, the gastric cancer was a IIc type of early cancer which consisted of a combination of a poorly differentiated adenocarcinoma and a well-differentiated tubular adenocarcinoma. The association of a hepatic tumor including hepatocellular carcinoma or liver metastasis was ruled out by ultrasonography, computed tomography, radiocolloid liver scan, magnetic resonance imaging, and angiography. Foci strongly resembling hepatocellular carcinoma (hepatoid differentiation) were noted in the gastric tumor. Localization of des-gamma-carboxy prothrombin and alpha-fetoprotein within the tumor cells, especially within the hepatoid differentiated foci, was demonstrated by the immunohistochemical staining of tissue obtained at biopsy and the resected specimen. This case seems to be the first case reported in which des-gamma-carboxy prothrombin was produced by the gastric cancer. This finding supports the theory of hepatoid differentiation of a gastric cancer.

Malignant meningioma with repeated multiple recurrence. A case report.

A case of malignant meningioma with repeated multiple recurrence was reported. A 22 year-old male first underwent the total removal of left parieto-occipital convexity meningioma in 1965. Although he had lived an uneventful life after the operation, he recognized motor weakness of the left lower limb in April 1985 when he was 41 years old and CT revealed a large tumor in the parieto-occipital parasagittal region. He underwent the total removal of the tumor and cranioplasty on May 23, 1985. After the second operation, repeated recurrence of multiple tumors was seen, which were in the frontal, parietal and occipital convexities, parasagittal regions and falx. He underwent further operations on January 23, 1986, December 11, 1986, March 30, 1987 and July 20, 1987 in addition to the first and second ones. Histological study on every operation indicated malignant meningioma with mitosis, hypercellularity and necrosis. Though radiotherapy (56 Gy whole brain irradiation) was conducted after the sixth operation, multiple tumors recurred and clinical symptoms and signs deteriorated gradually and he finally died September 9, 1989. In malignant meningioma such as our case, early aggressive radiotherapy and chemotherapy should be considered besides radical operations.

[Demonstration of PIVKA-II and AFP production by gastric cancer by indirect immunoenzymatic staining of paraffin sections].

The patient had elevated plasma PIVKA-II and serum AFP levels. However, no tumor was detected with an ultrasonography, angiography, abdominal computed tomography (CT), and magnetic resonance imaging (MR). Gastric endoscopic examination disclosed gastric cancer and then subtotal gastrectomy was done. Shortly after the surgery, both plasma PIVKA-II and serum AFP levels returned to each normal level. The extirpated tumor revealed histologically immature and mature cancer cells. It was pathologically diagnosed as IIc early cancer. The localization of PIVKA-II and AFP in gastric cancer cells was demonstrated by the immunostaining method using monoclonal antibody. Taken together, these results indicate that cancer cells may produce PIVKA-II or AFP in this patient.

Monostotic fibrous dysplasia of the left parietal bone--case report.

A 14-year-old female presented with a hard, painless mass, 5 x 5 cm, in the left parietal region. Skull x-rays showed a radiolucent skull tumor with a sclerotic margin in the parietal region. Computed tomography revealed an intradiploic multilocular mass separated by bony trabeculae. The outer table had thinned and protruded outward. The inner table was also thin and protruded inward slightly. External carotid angiography revealed a faint tumor stain and feeding from the middle meningeal artery. Bone scintigraphy revealed abnormal uptake in the lesion. Total removal of the skull tumor and cranioplasty were performed. The histological diagnosis was fibrous dysplasia. Fibrous dysplasia within the cranial vault is often expressed as painless bulging without neurological symptoms. Surgery is recommended when neurological symptoms and/or cosmetic problems are present. Histological confirmation of the diagnosis is also important.

Epidermoid tumor presenting with trigeminal neuralgia and ipsilateral hemifacial spasm: a case report.

A case of epidermoid tumor presenting with a painful tic convulsif was reported. A 35-year old male with trigeminal neuralgia and ipsilateral hemifacial spasm was diagnosed as having an epidermoid by CT and metrizamide CT cisternography and the symptoms were completely eliminated after the operation. In this case, metrizamide CT cisternography was very useful for preoperative diagnosis by demonstrating the characteristic findings of the epidermoid. It should be taken into consideration that there are some cases with trigeminal neuralgia and/or hemifacial spasm whose symptoms are due to brain tumors.