A case of Behçet's disease with occlusion of both caval veins and "downhill" esophageal varices.

A case of Behçet's disease (BD) with occlusion of both caval veins and "downhill" esophageal varices is reported. A 59-year-old male patient had esophageal varices, established as the "downhill" type by endoscopy, venography, and contrast-enhanced computed tomography (CE-CT) scan. Venography via both jugular veins did not lead to the visualization of the superior vena cava (SVC), but revealed numerous collateral veins, which formed esophageal varices en route. Venography via both femoral veins did not lead to the visualization of the inferior vena cava (IVC), but revealed numerous collateral veins, which flowed into the portal vein together with the jugular flow. CE-CT scan revealed the disappearance of the SVC and the lower part of the IVC, below the hepatic vein. The patient was diagnosed as having BD, based on his history of recurrent oral and skin lesions over the past 26 years.

Direct effect of danazol on endometrial hyperplasia in adenomyotic women: treatment with danazol containing intrauterine device.

It is well known that danazol has a direct effect on endometriosis tissue and cell. We have been treating adenomyotic women with danazol containing intrauterine device (D-IUD) from June 1993 to August 2000 and significant decrease of dysmenorrhea and serum CA-125 levels were observed. Of fifty-nine adenomyotic women, eight women were also diagnosed by endometrial biopsy as endometrial hyperplasia and one woman was diagnosed as atypical endometrial hyperplasia. In these endometrial hyperplastic patients, endometrial tissues were obtained before insertion and at the time of removal or exchange of D-IUD and examined pathologically. In all of the 9 women, histopathological findings of endometrial hyperplasia disappeared after D-IUD treatment. In particular, in one patient, findings of atypical endometrial hyperplasia also disappeared after D-IUD treatment. She is now closely observed at our clinic using D-IUD. By these evidences, we postulate that D-IUD is one of the treatment choices of endometrial hyperplasia given exposure of the endometrium to such an extraordinary high concentration of danazol released by D-IUD and avoidance of adverse effects of oral danazol or general administration of GnRH and progesterone. In particular, in atypical endometrial hyperplasia case, its mechanisms might give great benefit to patient. However, mechanisms of direct effect of danazol on endometrial hyperplasia remain to be elucidated in the future study.

Cowden's disease diagnosed through mucocutaneous lesions and gastrointestinal polyposis with recurrent hematochezia, unrevealed by initial diagnosis.

A 51-year-old man was admitted to our hospital because of hematochezia and painful keratotic plaques involving both hands. He had gastrointestinal polyposis, and a history of liver hemangiomas and thyroid tumor. Numerous papules on the face and papillomatosis on the oral mucosa were present. A diagnosis was made as a typical case of Cowden's disease according to the criteria proposed by Salem and Steck (J Am Acad Dermatol 8: 686, 1983). The patient was not correctly diagnosed initially in spite of typical manifestations of Cowden's disease, mainly due to his concomitant manifestations which occurred chronologically.

Takayasu's arteritis in a 69 year-old woman.

Takayasu's arteritis and temporal arteritis share many clinical and pathological features. The most discriminatory feature between the two diseases is the age at onset; the mean age at onset of the disease was reported as being 26 years for Takayasu's arteritis and 69 years for temporal arteritis. Here we report a 69-year-old woman who presented with a weak right radial artery pulse. The ethnic background and the presence of vascular insufficiency of the right upper extremity and the absence of clinical signs such as shoulder stiffness and tender scalp indicate that her diagnosis is Takayasu's arteritis. It must be emphasized that the two conditions could be differentiated based on the clinical findings even in a patient as old as 69 years old.

Primary adenosquamous carcinoma of the liver which produces granulocyte-colony-stimulating factor and parathyroid hormone related protein: association with leukocytosis and hypercalcemia.

A 55-year-old man was admitted to our hospital with fever and vomiting. Abdominal computed tomography (CT) revealed multiple low density masses in the liver. A diagnosis of primary adenosquamous carcinoma of the liver was confirmed by histological examination of a necropsy specimen. The present case showed leukocytosis and hypercalcemia with high levels of serum granulocyte-colony-stimulating factor (G-CSF) and parathyroid hormone related protein (PTHrP). Recent studies have shown that G-CSF and PTHrP are responsible for the paraneoplastic syndromes with leukocytosis and hypercalcemia. The tumor cells demonstrated positive cytoplasmic immunohistochemistry staining with anti-G-CSF and anti-PTHrP antibodies. This result suggested that the tumor produced G-CSF and PTHrP.

Primary biliary cirrhosis associated with painless thyroiditis.

A case of anti-mitochondrial antibody (AMA)-negative primary biliary cirrhosis (PBC) associated with painless thyroiditis is reported in a 47-year-old woman who diagnosed as PBC based on her elevated serum gamma-glutamyl transpeptidase and immunoglobulin M levels, as well as histological findings of destroyed bile ducts surrounded by mononuclear infiltrates in the biopsied liver. She was negative for AMA and had a depressed level of thyroid-stimulating hormone accompanied by increased free thyrosine, thyroxine and triiodothyronine levels and low titers of anti-microsomal and anti-thyroid peroxidase antibodies. Her thyroid disorder corresponded with painless thyroiditis. An association between PBC and hyperthyroidism is rare. Furthermore, an association between AMA-negative PBC and hyperthyroidism due to painless thyroiditis has not previously been reported.

A seminal vesicle cyst complicated with a tumor like nodular mass of benign proliferating prostatic tissue: a case report with ultrastructural and immunohistochemical studies.

We report a seminal vesicle cyst complicated with a tumor-like nodular mass of benign proliferating prostatic tissue. The patient was a 53-year-old Japanese man. A cyst of approximately 4.5 cm in diameter was discovered at the left seminal vesicle area. In the inner part of the cyst, a papillary nodular mass of 0.7 cm in diameter was seen. Under the clinical diagnosis of a seminal vesicle cyst with a tumorous mural nodule, the patient underwent resection of the seminal vesicle cyst to rule out the possibility that the nodular mass in the cyst was a neoplasm of an especially malignant nature. Microscopic examination of the excised specimen revealed a small dome-like nodular mass on the luminal surface of the cyst consisting of nodular proliferation of benign tubular gland tissue with various configurations. Conventional histologic, immunohistochemical, and ultrastructural analysis showed the proliferating cells in the nodular mass consisted of the benign prostate type. It is extremely important to differentiate between a benign proliferation and a malignant one, when the nodular mass is found in the seminal vesicle cyst.

A unique early gastric tubular adenocarcinoma arising from a pre-existent carcinoid tumor in a patient with a more than 20-year history of type A gastritis: an immunohistochemical and ultrastructural study.

A unique early gastric tubular adenocarcinoma developed from a pre-existent carcinoid tumor in a patient with a more than 20-year history of type A gastritis, multiple endocrine cell micronests, hypergastrinemia, and a high level of serum antiparietal cell autoantibody. The patient was a 60-year-old Japanese man. The background gastric mucosa around the tumor showed marked atrophy with intestinal metaplasia, in which endocrine cell micronests were frequently observed, and was consistent with type A gastritis. The mass was composed of both adenocarcinoma and carcinoid tumor. The adenocarcinoma was restricted to the lamina mucosa and submucosal area, and constituted a minor component of the tumor mass. The carcinoid tumor was the dominant constituent of the tumor, that invaded continuously the subserosa and muscularis propria. Based on this examination together with the detailed immunohistochemical and ultrastructural studies, the adenocarcinoma was presumed to have developed from the pre-existent carcinoid tumor. Ultrastructurally there were no amphicrine cells in the tumor, containing both endocrine granules and mucin droplets.

Lipid-rich clear-cell hepatocellular carcinoma arising in non-alcoholic steatohepatitis in a patient with diabetes mellitus.

A case of lipid-rich clear-cell hepatocellular carcinoma arising in non-alcoholic steatohepatitis is described in a patient with diabetes mellitus. The patient was a 67 year-old Japanese female with a history of tuberculosis, appendicitis, ischaemic heart disease, and non-insulin-dependent diabetes mellitus. The patient denied alcohol consumption. A liver mass was diagnosed as hepatocellular carcinoma of clear-cell type with early cirrhosis of the peri-tumoral liver tissue. Tumour cells had clear cytoplasm containing lipid droplets, and Mallory bodies. Surrounding non-tumoral liver tissue also showed lipid, and fibrosis in peri-portal areas with moderate bridging fibrosis. The features were consistent with clear-cell hepatocellular carcinoma arising in the fibrosis of non-alcoholic steatohepatitis. By electron microscopy, tumour cells had lipid droplets, glycogen, swollen mitochondria, rough endoplasmic reticulum, Mallory bodies, small bile canaliculi, desmosomes and gap junctions. Surrounding non-tumoral hepatocytes had a largely normal ultrastructure with prominent glycogen and lipid droplets. Clear-cell hepatocellular carcinoma within non-alcoholic steatohepatitis associated with diabetes mellitus is an extremely rare condition, and this report provides a detailed histopathological description with both immunohistochemical and ultrastructural data.

Cardiac sarcoma showing bone formation and neurogenic markers: report of a case.

A 54 year-old Japanese female with cardiac insufficiency was found to have a left atrial mass and smaller masses on the mitral valve. Excisional surgery of the masses and mitral valve replacement were carried out. In spite of intensive post-operative radiation therapy, the patient died of intra-atrial recurrence and brain metastases after 8 months. Tumour cells were spindled to oval, were positive for vimentin, S100 protein and neurone specific enolase. Laminin and fibronectin were also demonstrated. Bone formation and myxoid areas were present. An ultrastructurally identifiable stromal component, possibly responsible for laminin and fibronectin staining, was also present. The merits of the two main diagnostic possibilities - a mesenchymal/fibroblastic sarcoma showing bone and aberrant S100 protein, and a malignant peripheral nerve sheath tumour with bone - were discussed. In practical terms, the tumour was given the diagnosis of unclassifiable sarcoma of the left atrium. Atrial sarcomas showing neural markers and bone formation are exceedingly rare, and this report adds a further exceptionally uncommon case to the literature.

Rhabdomyolysis and aggravation of arthritis in a rheumatoid arthritis patient as a result of sepsis due to Staphylococcus aureus infection of a rheumatoid nodule; a catastrophic outcome.

A 63-year-old man with rheumatoid arthritis presented with rhabdomyolysis and intractable arthritis of acute onset. He was diagnosed to have sepsis due to Staphylococcus aureus infection through of an ulcerated rheumatoid nodule. Staphylococcus aureus isolated from pus in the ulcerated rheumatoid nodule and a blood sample obtained from the heart post-mortem produced the toxic shock syndrome toxin-1 (TSST-1). The TSST-1 and/or unmethylated CpG motifs in the oligonucleotides present in a bacterium, Staphylococcus aureus in this case, might be implicated in the induction of rhabdomyolysis and intractable arthritis.

[Subrenal capsule assay for chemosensitivity testing].

The subrenal capsule (SRC) assay for cancer chemotherapy was tested according to Bogden's methodology. Of 37 patients providing tumor tissue for assay, 29 cases were considered suitable for evaluable assays. Fourteen patients had clinically evaluable diseases and 10 cases were evaluable for SRC assays. Correspondence between sensitive assay and clinical sensitivity was seen in 2 cases, and that between resistant assay and clinical resistance was seen in 4 cases. Discordance between sensitive assay and clinical resistance was seen in 4 cases. In histological studies, cancer tissues implanted in the subrenal space in immunocompetent mice did not show marked proliferation and were replaced by prominent leukocyte infiltration and fibrosis on day 6 after inoculation. The degree of leukocyte infiltration in the xenografts in the mice administered some anti-cancer drugs was slight in comparison with that in untreated control mice, which showed a remarkable trend in xenografts treated with 5-fluorouracil and cyclophosphamide, respectively. Our study suggests that there are many problems involved in the SRC assay methodology of Bogden, and that careful examination of this aspect will be required.

[Induction of mucosal immunity to mycobacterial heat shock protein (hsp) 65 by colonic inoculation of plasmid DNA encoding hsp65].

Mycobacterial heat shock protein (hsp) 65 has more than 50% sequence homology with human hsp60 and immune responses against mycobacterial hsp65 may cross-react with human hsp60 and could cause autoimmune diseases including inflammatory bowel diseases (IBD). Since the colonic mucosa is a main inflammatory site in IBD, mucosal immunity to hsp65 may be more important for the mucosal inflammation than systemic immunity to hsp65. We inoculated plasmid DNA (pDNA) encoding mycobacterial hsp65 (pACB-hsp 65) into the colon of Wistar rats and evaluated the mucosal humoral immune response and the effect of these immune responses on the colonic mucosa. Four weeks after pDNA inoculation, significantly elevated titers of hsp65-specific IgA antibody were seen in fecal extracts of rats immunized intra-colonic mucosa with pACB-hsp65 (40 +/- 9 U/ml), whereas the fecal IgA antibody titers of rats inoculated intradermal with pACB-hsp65 did not arise (8 +/- 5 U/ml). Colonic inoculation of pACB-hsp65 induced systemic and mucosal immune responses to hsp65. However, macroscopic and histological examinations of the colonic mucosa inoculated with pACB-hsp65 showed no evidence of mucosal damage. These results suggested that the mucosal immunity to hsp65 on the colonic mucosa may not play a crucial role in the induction of colonic mucosal inflammation as was seen in IBD.

Adherence of cyanoacrylate which leaked from gastric varices to the left renal vein during endoscopic injection sclerotherapy: a histopathologic study.

We report a case involving leakage of cyanoacrylate (CA) to the inferior vena cava (IVC) through a gastrorenal shunt and left renal vein. A 72-year-old man with liver cirrhosis was admitted to our hospital to undergo emergency treatment for massive hemorrhage of gastric varices. Endoscopic injection sclerotherapy (EIS) using CA was performed on the varices. Radiographic fluoroscopy revealed that most of the injected CA had adhered firmly to the gastric varices, but a certain portion of the CA had flowed to the IVC through the gastrorenal shunt and left renal vein. At that point, the patient did not complain of any symptoms. However, 6 months later, he died of hepatic failure and an autopsy was performed. Histopathologic examination of the wall of the IVC and renal vein, to which CA had adhered, revealed that the CA was covered with endothelial cells of the vessel and no nearby thrombus was present. Long-term anticoagulant therapy may not be indicated in cases of leakage of CA from the gastric varices to other veins, since the leaked CA may be readily covered with endothelium without thrombus formation as in our patient. It is possible for CA to flow to the IVC and have a fatal impact. Our patient was fortunate, and for safe EIS it is important that these complications are prevented.