Portal venous tumor thrombus associated with hepatic metastasis of renal cell carcinoma: case report.

We report a case of liver metastasis of renal cell carcinoma with portal venous tumor thrombus. Abdominal computed tomographic images showed a large hepatic mass that enhanced slightly during arterial phase. Multiple hypoattenuating lesions were seen in the intrahepatic portal venous branches and were traced directly from the mass. The histologic specimen confirmed metastatic liver tumor of renal cell carcinoma with portal venous tumor thrombus.

Role of percutaneous image-guided biopsy in the evaluation of renal masses.

OBJECTIVE: The objective of the present study was to evaluate the indications, accuracy, complications and impact of image-guided percutaneous biopsy of renal masses. MATERIALS AND METHODS: Between 1994 and 1999, percutaneous biopsies under ultrasonography or computerized tomography guidance were performed in 33 patients with renal mass (22 men and 11 women, mean age 57.5 years, range 21-88). We retrospectively analyzed the relationship between clinical and histopathological findings, and discuss the appropriateness of the indications for image-guided percutaneous biopsy in the diagnosis of renal masses. RESULTS: The indications used in our institution were as follows: (1) clinical and radiological findings to suggest a diagnosis other than primary renal cell carcinoma (RCC) (n = 15); (2) suspicious lesions of RCC in multiple cystic renal masses (n = 7); (3) differentiation of transitional cell carcinoma of the renal pelvis from RCC (n = 7); (4) differentiation of angiomyolipoma from RCC (n = 4). Sufficient amounts of tissues were obtained from all patients for pathological diagnosis. Among 33 patients, 21 (63.6%) were diagnosed positive for malignancy, and 15 underwent surgical intervention. The histopathological findings between percutaneous biopsy and surgically resected tissue were identical in 13 cases (86.7%). No patient developed major complications requiring surgical treatment. CONCLUSION: If performed under appropriate selection of patients, percutaneous image-guided biopsy is a safe, reliable and accurate method of managing suspicious and/or indeterminate renal mass, and may contribute to the selection of appropriate clinical management by avoiding unnecessary procedures.

Hepatocellular carcinoma with a sarcomatous appearance: report of a case.

A 59-year-old man was admitted with general fatigue, an epigastric mass, and remittent fever. Radiological examinations disclosed a huge solid-to-cystic mass in the right lobe of the liver, and the mass severely compressed the right diaphragm, the inferior vena cava, and the right atrium. In addition, the patient suffered from chronic hepatitis; however, the serum alpha-fetoprotein, carcinoembryonic antigen, and PIVKA II levels were all within the normal ranges. The serum C-reactive protein level was 7.71mg/dl. With a clinical diagnosis of a malignant hepatic tumor invading the right diaphragm, surgery was performed. The tumor originated from segments IV and VII of the liver, was well defined, and grew extrahepatically. The tumor was resected using an ultrasonic cavitational aspirator together with the infiltrated right diaphragm. The resected tumor measured 23 x 13 x 23cm in size and weighed 3,700 g. Histologically, the tumor was found to consist of hepatocellular carcinomatous component and sarcomatous component. In the sarcomatous component, spindle-shaped cells which were positive for the immunohistochemical localization of vimentin, alpha-smooth muscle actin, and keratin were identified. The postoperative course was uneventful. The value of the serum C-reactive protein returned to within the normal range, and the patient became afebrile. The patient received a postoperative combination chemotherapy (etoposide, epirubicin, and cisplatin), and remains well with no signs of recurrence 12 months after the operation.

Pigmented neurofibroma: report of two cases and literature review.

Two cases of pigmented neurofibroma of the skin are reported. In case 1, the tumor was removed from the back of a 55-year-old man with no associated neurofibromatosis. In case 2, the tumor was removed from the abdominal wall of a 21-year-old woman with neurofibromatosis. Both tumors consisted of benign, short spindle cells and multiple foci of scattered melanin-laden cells. In case 1, the spindle cells were arranged in a storiform pattern, resembling features of dermatofibrosarcoma protuberans. Immunohistochemically, the spindle cells of both cases were demonstrated to be positive for S-100 protein and CD34. The melanin-laden cells stained positively for HMB-45. This report describes an additional two cases of pigmented neurofibroma that conform to the new diagnostic criteria for this disease.

Papillary fibroelastoma of the left atrial appendage: echocardiographic findings.

Papillary fibroelastoma is a small and rare benign intracardiac tumor that most frequently arises from the valvular endocardium. We report a patient with acute myocardial infarction in whom a papillary fibroelastoma in the left atrial appendage was detected by a transesophageal echocardiography during evaluation of the myocardial infarction. The roles of transesophageal echocardiography and surgical intervention are discussed.

Multifocal fibrosclerosis as a possible cause of panhypopituitarism with central diabetes insipidus.

Multifocal fibrosclerosis denotes a combination of similar fibrous disorders occurring at different anatomical sites. We encountered a 53-year-old male patient with orbital pseudotumor, chronic paranasal sinusitis, fibrous nodules of the lungs, intracranial pachymeningitis, and panhypopituitarism with central diabetes insipidus (DI) as a possible manifestation of multifocal fibrosclerosis. It has been reported that intracranial pachymeningitis or orbital pseudotumor associated with multifocal fibrosclerosis could invade the sella turcica causing a variety of anterior and/or posterior pituitary dysfunctions. In our case, intracranial pachymeningitis apparently involved the pituitary stalk and gland. Isolated gonadotropin deficiency, in addition to central DI, preceded panhypopituitarism. Although panhypopituitarism with central DI due to multifocal fibrosclerosis is quite rare and only one case has ever been reported, this systemic fibrotic disorder can be a possible cause of panhypopituitarism with central DI.

Hepatotoxicity of acetaminophen and N-acetyl-p-benzoquinone imine and enhancement by fructose.

1. Although oral administration of 400 mg/kg acetaminophen (APAP) or 1.8-3.4 g/kg sucrose had no effect on serum levels of alanine aminotransferase (ALT) and sorbitol dehydrogenase (SDH), their co-administration resulted in 20-fold increases in ALT/SDH activities. APAP alone (1250 mg/kg, p.o.) caused the elevation hepatotoxicity parameters, but the levels were lower than observed with co-administration of APAP (400 mg/kg) and sucrose (2.6 or 3.4 g/kg). 2. Sucrose-associated increase in serum ALT/SDH activities was selective with APAP and not detected with carbon tetrachloride (160 mg/kg, i.p.), D-galactosamine (400 mg/kg, i.p.) or alpha-naphthyl isothiocyanate (100 mg/kg, p.o.). 3. To verify the synergistic mechanism of sucrose, a major reactive intermediate of APAP, N-acetyl-p-benzoquinone imine (NAPQI), was given via the portal vein to rat pretreated with sucrose. Clear elevation of ALT/SDH activities was detected in the co-treated group. These results, together with an allopurinol-inhibition experiment, suggest the involvement of high-dose sucrose at a step(s) occurring after the metabolic activation of APAP. 4. Co-administration of glucose or fructose as well as sucrose elevated APAP-induced hepatotoxicity parameters in rat. Fructose but not glucose elevated APAP- or NAPQI-induced LDH leakage in a primary hepatocyte system. The results suggest the primary role of fructose is on the sucrose enhancement of APAP toxicity in rat.

Anaplastic osteosarcoma with abundant eosinophilic cytoplasm and minimal osteoid production.

A case of osteosarcoma with unusual microscopic features, occurring in the right proximal tibial metaphysis of a 12-year-old boy is reported. Radiographically, the tumor was ill-defined and purely osteolytic. On gross examination, the tumor was soft, fragile, spongy and red to brown in color. Microscopically, the tumor consisted of pleomorphic cells possessing abundant eosinophilic cytoplasm, including cells larger than 100 microm in diameter. The cells were arranged in a sheet, with few extracellular collagen fibers. Multiple sectioning of the specimens revealed a small amount of osteoid production. Immunohistochemical study revealed a positive reaction for vimentin and osteocalcin. Electron microscopic study suggested the fibroblastic or osteoblastic origin of the cells.

Dedifferentiated liposarcoma with chondroblastic osteosarcomatous dedifferentiation.

We describe a rare case of dedifferentiated liposarcoma with features resembling chondroblastic osteosarcoma in the dedifferentiated component. The tumor was removed from the left thigh in a 78-year-old male. It consisted of a well-differentiated liposarcoma and an anaplastic component that contained numerous osteoid and cartilaginous tissues surrounded by high-grade spindle cell sarcoma. To our knowledge, only two cases similar to the divergent chondroblastic osteosarcomatous dedifferentiation of this disease have been reported in the literature.

Cystic hamartoma of the renal pelvis: imaging features.

We report a case of a 50-year-old woman with histologically proven cystic hamartoma of the renal pelvis, focusing on the imaging findings and pathologic features. A large multicystic tumor with solid components in the left kidney was enhanced on both contrast-enhanced CT and gadolinium-enhanced MR images. Angiographic images showed irregular tumor vessels with moderate tumor stain.

Solitary infantile myofibromatosis in the femur.

We report on a case of the solitary type of infantile myofibromatosis of the right femur in an 11-month female. Radiographically, a well-defined osteolytic lesion was seen in the diaphysis of the right femur. Pathological study revealed that the spindle-shaped cells with eosinophilic cytoplasm were arranged in a fascicular and intertwining fashion. The cleft-shaped vascular spaces were observed between tumor nodules. Immunohistochemical staining caused many tumor cells to react for vimentin, alpha-smooth muscle actin and desmin. Ultrastructural study revealed numerous thin and intermediate types of filaments in the cytoplasm of the cells. To our best knowledge, this is the third reported case of solitary infantile myofibromatosis of long bones.

Orbital cholesterol granuloma with destruction of the lateral orbital roof.

PURPOSE: Orbital cholesterol granuloma in a 51-year-old man is described. METHODS: Computed tomography (CT) and magnetic resonance imaging (MRI) were done. RESULTS: Both studies showed a mass in the left orbit, with evidence of orbital roof destruction in the CT scan. On the basis of clinical and imaging findings, a diagnosis was made of malignant orbital tumor with destruction of the lateral orbital roof. Surgical exploration revealed a thickly encapsulated mass densely adherent to the left superior orbital bone and periosteum. Although the dura mater was intact, bone destruction in the lateral orbital roof was seen. The entire mass was successfully excised and histopathological evaluation was performed. Histopathology showed numerous inflammatory cells, blood degradation products, and cholesterol clefts. The absence of epithelial elements led to the diagnosis of cholesterol granuloma. CONCLUSIONS: Care must be taken to differentiate cholesterol granuloma from malignant orbital tumor. CT scan and MRI imaging seem well-suited to detecting the characteristic findings of cholesterol granuloma.

p53 adenoviral vector (Ad-CMV-p53) induced prostatic growth inhibition of primary cultures of human prostate and an experimental rat model.

BACKGROUND: Benign prostatic hyperplasia (BPH) is the most common proliferative disease affecting men. Numerous minimally invasive technologies are being developed or are currently in use to obviate the need for transurethral surgery. The goal of the present study was to develop a novel molecular based approach for the treatment of BPH using recombinant p53 adenoviral vector. The over-expression of wt-p53 can cause cell apoptosis or cell growth arrest, thus preventing the uncontrolled cell proliferation underlying BPH pathophysiology. METHODS: Ad-CMV-p53, a replication-deficient recombinant adenovirus containing cytomegalovirus promoter driving p53 gene, was used. Human prostate stromal (PS) cells were evaluated for apoptosis (TUNEL assay), mRNA levels of key cell cycle regulators influencing apoptosis (p-53, Bax and Bcl-2) using quantitative RT-PCR and cytotoxicity after Ad-CMV-p53. Ad-CMV-p53 was unilaterally injected into rat ventral prostates and growth inhibition was measured by prostate weight 3 weeks after injection. RESULTS: In vitro exposure to Ad-CMV-p53 significantly inhibited the proliferation of PS cells, induced mRNA over-expression of both wt-p53 and Bax, and increased the proportion of apoptotic cells. A 30% decrease in average prostate weight was demonstrated in rodents after Ad-CMV-p53 injection. CONCLUSIONS: The results suggest that further investigation of molecular gene therapy with recombinant wt-p53 adenovirus for the treatment of BPH is warranted.

Fibromatoses of multicentric origin: a case report.

We experienced a very rare case with fibromatoses of multicentric origin. One of the 2 intraabdominal fibromatoses showed a extremely rapid growing and another fibromatosis arising from the abdominal wall showed an invasive behavior. All lesions were diagnosed and resected simultaneously. This patient has been followed for 2 years postoperatively and no recurrent lesion has been detected so far.

Adrenal endothelial cyst with massive central calcification.

A 55-year-old woman was treated for a rare case of adrenal endothelial cyst that was detected incidentally during management of abdominal pain. Computed tomography of the tumor displayed massive central calcification and a fluid-fluid level by intracystic hemorrhage. Magnetic resonance imaging showed an intracystic papillary part with calcification. After surgery, the tumor was diagnosed as an angiomatous type of adrenal endothelial cyst.

Complete agenesis of the dorsal pancreas.

Pancreatic anomalies are occasionally reported, but complete agenesis of the dorsal pancreas is extremely rare. We report a 47-year-old woman with complete agenesis of the dorsal pancreas. This patient initially presented with jaundice. Computed tomography did not reveal the pancreatic corpus or tail. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography did not visualize the dorsal pancreatic duct. Choledochojejunostomy was performed because she had obstructive jaundice. At laparotomy, there was an enlarged pancreatic head, but no distal pancreas was seen. Histological examination of the pancreatic biopsy specimen showed scattered islets of Langerhans in diffuse fibrosis, with destruction of the glandular parenchyma. This case was diagnosed as complete agenesis of the dorsal pancreas.

[A case of true carcinosarcoma in bladder diverticulum].

We report a case of carcinosarcoma arising from a bladder diverticulum. A 71-year-old male was referred to our hospital for macroscopic hematuria. Two diverticula were identified in the left wall of the urinary bladder, one of which showed a broad-based tumor. The bladder tumor was resected using a transuretheral approach and the tumor was histologically diagnosed as leiomyosarcoma. The patient underwent partial resection of the bladder including the two diverticula and the tumor. Pathological examination revealed that the resected specimen was composed of three elements, transitional cell carcinoma (G3), squamous cell carcinoma, and leiomyosarcoma. Thus, the patient was diagnosed with carcinosarcoma. He died 5 months after surgery to remove the panperitonitis carcinomatosa. This case is the 38th reported case of bladder carcinosarcoma in Japan.

Differential expression of SHP2, a protein-tyrosine phosphatase with SRC homology-2 domains, in various types of renal tumour.

SHP2, a widely distributed protein-tyrosine phosphatase with src homology-2 (SH2) domains, is highly expressed in the brain and may play a role in synaptic communications or cellular proliferation. In this study, we examined SHP2 protein expression in 110 renal cell tumours of various histological subtypes, including clear, granular, papillary, chromophobe, collecting duct, and sarcomatoid-type renal cell carcinoma (RCC), and oncocytoma. SHP2 was expressed predominantly in normal distal tubules and collecting ducts, and positivity in various types of renal tumours was as follows: clear cell RCC, 0% (0/77 cases); granular, 7.7% (1/13); papillary, 50% (3/6); sarcomatoid, 0% (0/1); chromophobe, 85.7% (6/7); collecting duct carcinoma, 0% (0/2); oncocytoma, 100% (4/4). Clear and granular-type RCCs showed a very low but positive expression of SHP2. Chromophobe RCC and oncocytoma showed the highest rates and strongest intensities of SHP2 protein on immunostaining. SHP2 may serve as a powerful marker in detecting rare tumours. Estimates of its expression may be useful in histological diagnosis.

Expression of type IV collagen in the developing human kidney.

The distribution of alpha1-6 chains of type IV collagen (alpha1-6(IV)) in human fetal kidneys was examined by indirect immunofluorescence. By 11 weeks of gestation, alpha1, 2, 3, 4, and 6(IV) were already present, but alpha5(IV) appeared relatively late, at 21 weeks. Alpha1(IV) and alpha2(IV) were present in all basement membranes, alpha3(IV) and alpha4(IV) were restricted to the glomerular basement membrane and parts of the tubular basement membrane. Alpha5(IV) was distributed in the glomerular basement membrane, Bowman's capsule, and parts of the tubular basement membrane. Alpha6(IV) was present in the Bowman's capsule, parts of the tubular basement membrane, and occurred in parts of the glomerular basement membrane at the early capillary loop stage, but disappeared during the later capillary loop stage.