Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity.

We present a series of a distinct tumorous entity named renal angiomyoadenomatous tumor (RAT). Five cases were retrieved from the consultation files of the authors. Histologic and immunohistochemical features were evaluated. Sequencing analysis of coding region of the VHL gene was carried out in all cases. The tumors were composed of admixture of an epithelial clear cell component and prominent leiomyomatous stroma. Epithelial cells formed adenomatous tubular formations endowed with blister-like apical snouts. All tubular/glandular structures were lined by a fine capillary network. The epithelial component was positive for epithelial membrane antigen, CK7, CK20, AE1-AE3, CAM5.2, and vimentin in all cases. In all analyzed samples, no mutation of the VHL gene was found. RAT is a distinct morphologic entity, being different morphologically, immunohistochemically, and genetically from all renal tumors including conventional clear cell carcinoma and mixed epithelial and stromal tumor of kidney.

Multiple myeloma: short T2 on MR imaging.

We describe a rare case of multiple myeloma with marked hypointense areas on both T1- and T2-weighted images. Amyloid deposition and calcification were not found histologically. Hypointensity on T2-weighted images may be caused by distributed free radicals produced by significant macrophages.

Statistical analysis of diagnostic failure of fine needle aspiration cytology (FNAC) in breast cancer.

Fine-needle aspiration cytology (FNAC) was performed on 300 patients. Among those, 57 cases failed in accurate diagnosis of malignancy and 243 were successful. Fourteen clinicopathological factors altogether were analyzed to elucidate any correlation with FNAC failure using uni- and multivariate analysis. The univariate analysis in each clinicopathlogical factor showed that these error cases were vaguely palpable cancers, estrogen receptor (ER) positive cancers, small-sized of tumors, scattered type of cancer cell distribution in tumor tissues, with low tumor grade, with low Nottingham prognostic index (NPI), with benign-like ultrasound findings and with low TNM stage. The multivariate analysis revealed tumor grade was the strongest factor for all, followed by cellular distribution type of cancer cells and benign-like ultrasound findings. From these results, we speculated that diagnostic failure of FNAC at first clinic visit seemed to be caused by mainly two histocytological factors: extrinsic factor (structural factors of tissue-like tumor cells' distribution pattern, etc.); and intrinsic one (cellular factors of low atypism such as benign-like ultrasound finding, low tumor grade, and so on).

Isolated heterotopic pancreas causing intussusception.

Pediatric intussusception is usually idiopathic in origin, and a pathological lesion at the leadpoint is identified in only a small proportion of cases. Meckel's diverticulum is the most common location when such a lesion is found. Heterotopic pancreas has been seen most frequently in the stomach or duodenum; however, ileal lesions are rare. In the ileum, heterotopic pancreas is usually associated with Meckel's diverticulum and may cause intestinal obstruction by intussusception. However, isolated heterotopic pancreas in the ileum is distinctly rare and usually discovered incidentally during surgery for other conditions. In a review of English language literature, there were only a few pediatric cases of isolated ileal heterotopic pancreas causing intussusception. We report three additional pediatric cases and discuss the clinical significance of this unexpected rare entity.

Congenital hemangiopericytoma: report of a case.

We report a case of congenital hemangiopericytoma arising in the lower right leg of a 4-day-old male neonate. Despite the generally good prognosis associated with this neoplasm, a complete surgical excision has so far been recommended to avoid recurrences, because no definite criteria for determining whether or not the tumor will regress spontaneously have been established to date.

[Adenocarcinoma of the ileal segment with transitional cell carcinoma of the bladder following ileocytoplasty: a case report].

A 67 year-old woman visited our hospital complaining of pollakisuria. She had undergone left nephrectomy and augmentation ileocystoplasty for tuberculous bladder atrophy 40 years previously. She underwent a total cystectomy and tubeless ureterocutaneostomy with a preoperative diagnosis of muscle-invading transitional cell carcinoma of the bladder. The pathological diagnosis was adenocarcinoma of the ileal segment and transitional cell carcinoma of the original bladder. This is the first case report of adenocarcinoma of the ileal segment and transitional cell carcinoma of the original bladder among 22 patients suffering from bladder cancer after ileocystoplasty.

A solitary fibrous tumor in the perianal region with a 13-year follow-up: report of a case.

A case of a solitary fibrous tumor (SFT) of the perianal region in a 62-year-old man is reported herein. The patient had undergone an abdominoperineal excision of the rectum for an anorectal tumor 13 years previously, and had been referred to our hospital for a perineal mass. Computerized tomography and angiography revealed a markedly hypervascular tumor measuring 11 x 8 cm in size in the pelvic cavity. After preoperative radiotherapy (total 58 Gy) and the embolization of the feeding arteries, he underwent an en bloc excision of the tumor. Microscopically, the tumor was composed of spindle shaped cells with a "patternless" arrangement in a collagenous background. There was immunohistochemical evidence that these cells were strongly positive for CD34, thus suggesting the tumor to be SFT. The previously resected anorectal tumor showed similar histological and immunohistochemical findings. The patient's recovery was uneventful.

Malignant hemangioendothelioma of the small intestine: report of a case.

A case of malignant hemangioendothelioma (MH) of the small intestine in a 27-year-old woman is reported herein. The patient developed acute abdominal symptoms during investigations for anemia, weight loss, anorexia, and recurrent gastrointestinal bleeding. An emergency laparotomy revealed perforation of a jejunal tumor. The results of a histopathological study of the resected small bowel segment were interpreted as MH with lymph node involvement. Following this case report, a review of the relevant literature on small intestinal MH is presented.

Clinicopathologic significance of protein induced vitamin K absence or antagonist II and alpha-fetoprotein in hepatocellular carcinoma.

Protein induced vitamin K absence or antagonist II (PIVKA-II) and alpha-fetoprotein (AFP) have been considered useful serum markers of hepatocellular carcinoma (HCC). In this study, we examined the clinicopathologic significance of these tumour markers in patients with HCCs by measuring their serum levels and performing immunohistochemistry. We studied 349 Japanese patients with HCCs. Their serum PIVKA-II and AFP levels were determined by enzyme immunoassay before treatment. We examined the correlations between serum PIVKA-II and AFP levels and tumour size, presence of satellite nodules, histologic HCC grade, and concomitant liver diseases and subjected tumour tissues to immunohistochemical staining to detect PIVKA-II and AFP expression. The serum PIVKA-II levels of patients with poorly differentiated HCCs were significantly higher than those of patients with well and moderately differentiated HCCs (p<0.05) and they were higher in HCC patients with than without satellite nodules. The serum AFP levels were influenced significantly by concomitant liver diseases, but not by the other factors. Immunohistochemical staining revealed the PIVKA-II expression levels of poorly differentiated HCCs were higher than those of well and moderately differentiated HCCs (p<0.05). Some HCC cells were PIVKA-II-positive, others were AFP-positive, and some expressed both. The serum PIVKA-II concentration was a better indicator of HCC than AFP, as it was not influenced by concomitant liver diseases. The presence of PIVKA-II in serum correlated with the presence of satellite nodules and the histologic HCC grade, a result concordant with the immunohistochemical findings.

Occult papillary thyroid carcinoma in Hashimoto's thyroiditis presenting as a metastatic bone tumor.

Some occult thyroid carcinomas are hypothesized to regress and be eventually obliterated. We report here a patient whose condition supports this hypothesis. A 51-year-old male with primary hypothyroidism due to Hashimoto's thyroiditis suffered from a rib bone tumor. He had a diffuse goiter with no nodular lesion. Serum FT4 and TSH concentrations were 0.8 ng/dl and 36.4 microU/ml on taking 100 microg/day of T4. Anti-Tg- and -TPO-Ab were strongly positive (99 and 1380 U/ml). The iodine 123 scintigraphy demonstrated clear accumulation in the rib tumor, whereas the thyroid was scarcely visible. Biopsy of the rib tumor showed papillary proliferation of large atypical cells, which were immunohistochemically positive for thyroglobulin. Metastatic bone tumor of papillary thyroid carcinoma was therefore strongly suspected. He underwent a total thyroidectomy and the thyroid was stepwise sectioned completely at 3 mm intervals. The thyroid condition was diagnosed as Hashimoto's thyroiditis demonstrating diffuse and dense fibrosis, lymphocyte infiltration with lymphoid follicles and flattened atrophied follicles, but no carcinomatous foci were found. He was treated with I-131 and scintigraphy after the ingestion showed distinct accumulation in the rib tumors similar to that before thyroidectomy. No other abnormal uptake was observed. It is suggested that the primary occult thyroid papillary carcinoma regressed and was obliterated possibly by some immunologic or other host-resistance factors after it metastasized to the distant bone.

Ovarian teratoma with gliomatosis peritonei: report of two cases.

Gliomatosis peritonei, a rare condition related to ovarian teratomas, involves the peritoneal implantation of numerous nodules of predominantly mature glial tissues. We report herein the cases of two patients with immature ovarian teratoma associated with gliomatosis peritonei, in one of whom a rapid progression of teratomatous implants occurred 14 weeks after her initial surgery. Gliomatosis peritonei is considered benign in most cases; however, some reports have documented the rapid recurrence of immature peritoneal implants, as implantation is associated with teratomas of all grades. Thus, in the face of peritoneal implants suspected to be of a teratomatous nature, thorough and extensive sampling is essential to exclude the presence of immature elements which may imply a poor prognosis and require aggressive therapy.

Extravesical tumor implantation caused by perforation during transurethral resection of a bladder tumor: a case report.

We report a case of invasive bladder cancer in which cancer dissemination occurred through a perforation of the vesical wall during transurethral resection of the tumor. A radical cystectomy was performed 1 month later and several clusters of viable cancer cells were histologically identified in a fibrous foreign body granuloma in the paravesicular adipose tissue of the lymphadenectomy specimen. The patient received adjuvant chemotherapy, but developed right inguinal lymph node metastasis 21 months after cystectomy.

Multiple hepatoblastomas associated with trisomy 18 in a 3-year-old girl.

A very rare case of full trisomy 18 associated with multiple hepatoblastomas is reported. The patient also had ventricular septal defect and patent ductus arteriosus, which were repaired at 6 months of age. After the cardiac surgery, she was noted to have an abdominal mass and an elevated serum alpha-fetoprotein level. A partial hepatic lobectomy was performed at 7 months of age, and the resected tumor was diagnosed as a fetal-type hepatoblastoma. At 2 years and 4 months of age, a chest radiography disclosed an elevated left diaphragm, and abdominal ultrasonography demonstrated a tumor in the left hepatic lobe. The resected tumor was also diagnosed as a fetal-type hepatoblastoma. Chromosomal analysis demonstrated that the karyotypes of peripheral blood and hepatic tumor cell obtained on two occasions were both 47,XX, +18. She has no evidence of recurrence at 3 years of age without specific therapy.

Cyclin-D1-gene amplification is a more potent prognostic factor than its protein over-expression in human head-and-neck squamous-cell carcinoma.

To evaluate the prognostic significance of cyclin D1 protein/gene expressions in human head-and-neck squamous-cell carcinoma (HNSCC), we examined amplification of the cyclin-D1 gene (CCND1) by the differential PCR method and over-expression of cyclin-D1 protein by immunohistochemistry in 45 paraffin-embedded sections from HNSCC. Amplification of CCND1 was found in 10 (22%) cases and over-expression of cyclin D1 was found in 24 (53%) cases. CCND1 amplification was also found in 3 (25%) of 12 cases of dysplastic lesions adjacent to HNSCC. The overall 5-year survival of patients with CCND1 amplification or with protein over-production was significantly lower than that of patients without (p < 0.0001 and p < 0.05, respectively). However, with multivariate analysis, only amplification of CCND1 retained an independent prognostic value (p = 0.0018). These suggest that CCND1 amplification occurs at early stages of HNSCC tumorigenesis and is a more useful prognostic factor than over-expression of cyclin D1 in HNSCC.

[A case of renal capsular liposarcoma].

A 53-year-old woman was admitted to the Department of Internal Medicine at our hospital with the primary complaint of pyrexia. Abdominal echography and computed tomography (CT) detected a right renal tumor, and the patient was transferred to our department. Angiography revealed a hypovascular tumor. The main nutrient vessels supplying the tumor were the superior and inferior capsular arteries, which arose from the renal artery. A right renal capsular tumor was suspected from these findings, and radical nephrectomy was performed. Histopathological examination revealed a pleomorphic liposarcoma. Therefore, the patient was given 50 Gy of radiation postoperatively. This is the 14th case of a primary liposarcoma of the renal capsule reported in Japan.

[Intrascrotal leiomyoma: report of a case].

A case of intrascrotal leiomyoma is reported. The patient was a 56-year-old male who complained of painless mass in the scrotum. He noticed the mass about 10 years ago and it had gradually enlarged. The mass was removed surgically. Pathohistological diagnosis of the specimen was leiomyoma. The literature revealed 13 cases of intrascrotal leiomyoma in Japan.

Microvesicular fatty liver in rats with resembling Reye's syndrome induced by 4-pentenoic acid.

To produce an animal model of Reye's syndrome (RS), 20 adult male Wistar rats were given 10 repeated i.p. injections of 50 mg/kg 4-pentenoic acid (PA) each separated by an 8-h interval. Then, 90 min after the tenth dose, they were given a final i.p. injection of 150 mg/kg PA. Thirteen control animals were injected with vehicle only using the same time schedule. More than half the animals in each group were fed a common diet, but the others were fasted during the terminal 10-h stage. All rats were sacrificed 30 min after the last injection. At the terminal stage, in comparison with the vehicle-injected controls, hypolipemia, hypoglycemia and high titers of serum ammonia and urea N were estimated significantly in the PA-treated rats fed throughout the whole period. Hypolipemia and hypoglycemia were more prominent in the terminally fasted group than the group fed continuously. Only in the PA-treated rats fed throughout the whole period moderate morphological signs of microvesicular fatty liver were exhibited. Ultracytochemical findings and biochemical determinations showed that the major lipids in the microvesicular fatty livers were triglycerides. Morphometric analysis revealed distinct hepatic mitochondrial swelling in the PA-treated rats. Therefore, the above treatment with PA was able to induce microvesicular fatty liver in rats with resembling RS, which were fed throughout the treatment procedure, but not in the terminally fasted rats.