Primary carcinoid tumor of the liver: report of two cases with an emphasis on US findings.

Primary carcinoid tumor of the liver is rare, and its sonographic (US) findings have rarely been reported. We present two histologically proven cases. Both patients were asymptomatic and huge masses, found incidentally by US, were echogenic with many cystic areas. Lesions were markedly hypervascular on color Doppler US and angiography. Thus, primary carcinoid tumor of the liver should be considered when US detects this type of mass in asymptomatic patients.

Tc-99m PMT hepatobiliary scintigraphy in the differential diagnosis of extrahepatic metastases and hepatocellular carcinoma.

PURPOSE: The authors evaluated the utility of hepatobiliary scintigraphy for tissue characterization of extrahepatic metastases from hepatocellular carcinoma (HCC) using Tc-99m N-pyrydoxyl-5-methyltriptophane (Tc-99m PMT). METHODS: We examined 13 patients with HCC (29 extrahepatic metastases and 3 benign bone lesions) and 5 patients with other cancers (15 extrahepatic metastases). Thirty minutes to 6 hours after intravenous administration of Tc-99m PMT, planar (all 47 lesions) and SPECT (42 lesions) images were obtained. Accumulation of Tc-99m PMT in the lesion was evaluated visually by comparing bone scintigraphy, computed tomography, magnetic resonance imaging, or all of these. RESULTS: Findings were positive in 12 of 13 patients with HCC and extrahepatic metastases (16 of 29 on planar imaging and 21 of 26 on SPECT). Findings in all three benign bone lesions and 15 metastatic lesions from non-HCC primary lesions were negative (0 of 18 on planar imaging, 0 of 16 on SPECT). There were no false-positive findings in these lesions. Lesion-by-lesion sensitivity, specificity, accuracy, and positive and negative predictive values were 55%, 100%, 72%, 100%, and 58% by planar imaging and 81%, 100%, 88%, 100%, and 76% by SPECT, respectively. CONCLUSIONS: Because of the high specificity and reasonable sensitivity, Tc-99m PMT appears to be useful for the differential diagnosis of extrahepatic metastases from HCC. SPECT improves the detectability of small or faint accumulation in metastases from HCC.

[A case of bilateral testicular tumors with congenital adrenal hyperplasia].

We report a case of congenital adrenal hyperplasia (CAH) occurring in a 21-year-old man. He was found to have 21-hydroxylase deficiency shortly after birth in search for the cause of vomiting and adrenal insufficiency, and placed on steroid therapy. He had an uneventful childhood with normal onset of puberty. At the age of 21 years he was hospitalized with bilateral testicular masses. They were non-tender, firm and nodular on palpation. The levels of adrenocorticotrophic hormone (ACTH), 17 alpha-hydroxyprogesterone (17 alpha-OHP) were found to be elevated. Testicular biopsy revealed that the nodule comprised mainly eosinophilic sheets and nests and polygonal cells with abundant, granular cytoplasm, but no crystals of Reinke were seen. Testicular tumor with congenital adrenal hyperplasia is typically bilateral and develops in untreated or inadequately treated males with CAH.

[Renomedullary interstitial cell tumor: a case report].

Renomedullary interstitial tumor is a common tumor in the renal medulla, present in 26-41% of consecutive autopsy specimens. However clinically evident case is infrequent because this lesion is usually small (less than 3 mm). We report a case of renomedullary interstitial tumor in a 76-year-old woman. Ultrasonogram incidentally revealed a mass in the left kidney while she visited to the hospital for hypertension and unstable angina. A CT scan showed a 2 cm mass that was not clearly enhanced. MR images showed low signal intensity in both T1 and T2 images. Arteriography demonstrated no neo-vascularity. Those findings showed that this lesion was benign one or hypovascular carcinoma. So Left nephrectomy was performed and histological examination revealed a renomedullary interstitial tumor.

Absence of CD83-positive mature and activated dendritic cells at cancer nodules from patients with hepatocellular carcinoma: relevance to hepatocarcinogenesis.

Mature and activated dendritic cells (CD83-positive DCs) are essential for the recruitment and survival of activated tumor-specific lymphocytes during carcinogenesis. The frequencies of CD83 positive DCs were almost same in peripheral blood from patients with hepatocellular carcinoma (HCC) and cirrhosis of liver (LC). However, the numbers of CD83 positive DCs in liver tissues were significant lower in HCC compared with LC (6.6+/-10.9 vs. 33.3+/-24 DCs/specimen, P<0.05). Most importantly, there were no CD83-positive DCs at cancer nodules in HCC. A role of infiltration of activated DCs in liver during hepatocarcinogenesis is shown.

The effectiveness of interferon therapy on occurrence of hepatocellular carcinoma in chronic hepatitis C.

To clarify the effect of interferon (IFN) therapy for chronic hepatitis C (CHC) on the occurrence of hepatocellular carcinoma (HCC), 149 patients who were observed over a period of five years (mean: 7.6 years) were studied. The C-1 group, 33 patients with complete response to IFN; the C-2 group, 55 patients with no response to IFN; and the C-3 group, 61 patients who did not receive IFN. The occurrence rate of HCC was 0.9%/year/person. In the C-1, C-2 and C-3 groups, the rates were 0%, 0.3%, and 1.6%, respectively. The rate in C-1 + C-2 groups was significantly lower than that of the C-3 group (P<0.05). These data suggest IFN may suppress the occurrence of HCC in CHC.

Immunohistochemical and histochemical evidence for the presence of noradrenaline, serotonin and gamma-aminobutyric acid in chief cells of the mouse carotid body.

The immunohistochemical study revealed tyrosine hydroxylase (TH), dopamine beta-hydroxylase (DBH), phenylethanolamine N-methyltransferase (PNMT), serotonin, glutamate decarboxylase (GAD) and gamma-aminobutyric acid (GABA) immunoreactivities in the mouse carotid body. TH and DBH immunoreactivities were found in almost all chief cells and a few ganglion cells, and in relatively numerous varicose nerve fibers of the carotid body. The histofluorescence microscopy showed catecholamine fluorescence in almost all chief cells. However, no PNMT immunoreactivity was observed in the carotid body. Serotonin, GAD and GABA immunoreactivities were also seen in almost all chief cells of the carotid body. From combined immunohistochemistry and fluorescence histochemistry, catecholamine and serotonin or catecholamine and GABA were colocalized in almost all chief cells. Thus, these findings suggest that noradrenaline, serotonin and GABA may be synthesized and co-exist in almost all chief cells of the mouse carotid body and may play roles in chemoreceptive functions.

Immunocytochemical study of tyrosine hydroxylase and dopamine beta-hydroxylase immunoreactivities in the rat pancreas.

An immunohistochemical and immunoelectron microscopic study was used to demonstrate tyrosine hydroxylase (TH) and dopamine beta-hydroxylase (DBH) immunoreactivities in the rat pancreas. Small TH immunoreactive cells were found in close contact with large TH immunonegative ganglion cells among the exocrine glands and were occasionally found in some islets. Some of these TH immunoreactive cells were also DBH immunopositive. The immunoreaction product was seen diffusely in the cytoplasm and in the granule cores of TH immunoreactive cells. All intra-pancreatic ganglion cells were immunoreactive for DBH, but not for TH. The TH immunoreactive cells were identified as small intensely fluorescent (SIF) cells due to their localization and morphological characteristics and showed no insulin, glucagon, somatostatin or pancreatic polypeptide immunoreactivities. These results indicate that SIF cells may release dopamine or noradrenaline to adequate stimuli while the intra-pancreatic ganglion cells with only DBH may not synthesize catecholamines in a normal biosynthetic pathway. TH immunoreactive nerve bundles without varicosities and fibers with varicosities, associated or unassociated with blood vessels, were found in both the exocrine and endocrine pancreas. Close apposition of TH immunoreactive nerve fibers to the smooth muscle and endothelial cells of the blood vessels was observed. A close apposition between TH immunoreactive nerve fibers and exocrine acinar cells and islet endocrine cells was sometimes found in the pancreas. The immunoreaction product was seen diffusely in the axoplasm and in the granular vesicles of the immunoreactive nerve fibers. Since no TH immunoreactive ganglion cells were present in the rat pancreas, the present study suggests that noradrenergic nerve fibers in the pancreas may be extrinsic in origin, and may exert an effect on the regulation of blood flow and on the secretory activity of the acinar cells, duct cells and endocrine cells.

Postnatal development of neuropeptide Y- and calcitonin gene-related peptide-immunoreactive nerves in the rat urinary bladder.

The postnatal development of neuropeptide Y- and calcitonin gene-related peptide-immunoreactive (NPY-IR and CGRP-IR) nerve fibers in the rat urinary bladder was investigated using whole-mount preparations and cryostat sections. In newborn and 3-day-old rats, many NPY-IR nerve fibers were observed in the subserous and muscle layers. Many NPY-IR nerve cell bodies clustered at branching points of the subserous nerve bundles. Within 4 weeks after birth, these cell bodies drastically decreased in number and spread along the bundles, although the number of NPY-IR nerve fibers increased moderately. In contrast, CGRP-IR nerve fibers in newborn and 3-day-old rats were less developed, and no CGRP-IR nerve cell body was observed in any rat. However, CGRP-IR nerve fiber distribution in the urinary tissues conspicuously increased within 4 weeks after birth. Especially, an increase of the infraepithelial fibers showing a meshwork appearance was prominent in the fundus and corpus of the bladder. The infra- and intraepithelial CGRP-IR nerve meshwork of the ventral wall was more dense than that of the trigone. At 4 weeks, NPY-IR and CGRP-IR nerves were similar to those of the adult rat (8-12 weeks old). The present study suggests a correlation between the development of the peripheral nervous system in the urinary bladder and maturation of micturition behavior in the rat.

Ganglion cells immunoreactive for catecholamine-synthesizing enzymes, neuropeptide Y and vasoactive intestinal polypeptide in the rat adrenal gland.

Immunohistochemistry has been used to demonstrate tyrosine hydroxylase (TH), dopamine-beta-hydroxylase (DBH), phenylethanolamine N-methyltransferase (PNMT), neuropeptide Y (NPY) and vasoactive intestinal polypeptide (VIP) immunoreactivities, and acetylcholinesterase (AChE) activity was demonstrated in rat adrenal glands. The TH, DBH, NPY and VIP immunoreactivities and AChE activity were observed in both the large ganglion cells and the small chromaffin cells whereas PNMT immunoreactivity was found only in chromaffin cells, and not in ganglion cells. Most intra-adrenal ganglion cells showed NPY immunoreactivity and a few were VIP immunoreactive. Numerous NPY-immunoreactive ganglion cells were also immunoreactive for TH and DBH; these cells were localized as single cells or groups of several cells in the adrenal cortex and medulla. Use of serial sections, or double and triple staining techniques, showed that all TH- and DBH-immunoreactive ganglion cells also showed NPY immunoreactivity, whereas some NPY-immunoreactive ganglion cells were TH and DBH immunonegative. NPY-immunoreactive ganglion cells showed no VIP immunoreactivity. AChE activity was seen in VIP-immunopositive and VIP-immunonegative ganglion cells. These results suggest that ganglion cells containing noradrenaline and NPY, or NPY only, or VIP and acetylcholine occur in the rat adrenal gland; they may project within the adrenal gland or to other target organs. TH, DBH, NPY, and VIP were colocalized in numerous immunoreactive nerve fibres, which were distributed in the superficial adrenal cortex, while TH-, DBH- and NPY-immunoreactive ganglion cells and nerve fibres were different from VIP-immunoreactive ganglion cells and nerve fibres in the medulla. This suggests that the immunoreactive nerve fibres in the superficial cortex may be mainly extrinsic in origin and may be different from those in the medulla.

Gamma-aminobutyric acid (GABA) immunoreactivity in the mouse adrenal gland.

Gamma-aminobutyric acid (GABA) immunoreactivity was revealed by immunocytochemistry in the mouse adrenal gland at the light and electron microscopic levels. Groups of weakly or faintly GABA immunoreactive chromaffin cells were often seen in the adrenal medulla. By means of immunohistochemistry combined with fluorescent microscopy, these GABA immunoreactive chromaffin cells showed noradrenaline fluorescence. The immunoreaction product was seen mainly in the granular cores of these noradrenaline cells. These results suggest the co-existence of GABA and noradrenaline within the chromaffin granules. Sometimes thick or thin bundles of GABA immunoreactive nerve fibers with or without varicosities were found running through the cortex directly into the medulla. In the medulla, GABA immunoreactive varicose nerve fibers were numerous and were often in close contact with small adrenaline cells and large ganglion cells; a few, however, surrounded clusters of the noradrenaline cells, where membrane specializations were formed. Single GABA immunoreactive nerve fibers, and thin or thick bundles of the immunoreactive varicose nerve fibers ran along the blood vessels in the medulla. The immunoreaction deposits were observed diffusely in the axoplasm and in small agranular vesicles of the GABA immunoreactive nerve fibers. Since no ganglion cells with GABA immunoreactivity were found in the adrenal gland, the GABA immunoreactive nerve fibers are regarded as extrinsic in origin.

[A case of malignant paraganglioma].

Herein we report a case of paraganglioma. The metastatic lesion was incidentally found in a 64-year-old man by chest roentgenography. The primary tumor was extra-adrenal and was revealed to be malignant. The patient was normotensive without clinical symptoms despite extreme high serum and urinary dopamine levels. A venous injection of 5 mg metoclopramide elicited a significant increase of the blood pressure. This phenomenon disappeared after surgical excisions of the primary and metastatic lesions. The metoclopramide test is considered to be useful to diagnose asymptomatic paraganglioma.

[Two cases of male infertility with pathological dilatation of ejaculatory duct].

Two cases of male infertility with pathological dilatation of ejaculatory duct are reported. In both cases, the dilatated wall of the ejaculatory duct was incised at the vermontanum with a cold knife endoscopically. After the incision, the findings of semen analysis of one case improved markedly and his wife became pregnant. In another case, semen analysis was not improved. Transurethral incision seemed to be a useful modality for the treatment of pathological dilatation of ejaculatory duct.

[Pelvic fibromatosis simulating urachal tumor--a case report].

We report a case of pelvic fibromatosis, which has been rarely found. A 36-year-old man referred to our hospital complaining of right abdominal pain and a swelling mass. Abdominal CT and transabdominal echogram revealed a tumor contacted to the bladder. A nonpapillary tumor (little finger sized) at the bladder dome was observed by cystoscopy, and the tumor was clinically considered as urachal tumor. Wide radical excision including the bladder and prostate was performed. Pathological diagnosis was pelvic fibromatosis arising from musculoaponeurotic structures in the pelvis: The fibromatosis is benign nonmetastatic tumor but the recurrence rate is considerably high. However, in the present case, 14 months after operation there is no recurrence yet.

Effect of fixation conditions on the granule morphology of rat antral gastrin cells: an ultrastructural and immunocytochemical study.

After fixation at pH = 7 or pH = 5 the gastrin (G) cells in the rat pyloric antrum were investigated by conventional and immunoelectron microscopy. After fixation at pH = 7 G cells contained numerous electron-lucent granules, a few electron-dense and intermediate granules while G cells had numerous electron-dense and intermediate granules, and a few electron-lucent granules after fixation at pH = 5. Preembedding immunoelectron microscopy revealed that in G cells after fixation at pH = 7, gastrin immunoreactivity was mainly seen in the cytoplasm, cores of a few electron-dense and intermediate granules and in the periphery but not within the numerous electron-lucent granules; in G cells after fixation at pH = 5, gastrin immunoreactivity was mainly visible in the cores of numerous intermediate and electron-dense granules, in the periphery of a few electron-lucent granules, but weakly in the cytoplasm. Therefore, the variable electron density of G cells may reflect differences in the degree of the leakage of contents from the gastrum-containing granules into the cytoplasm during fixation. This phenomenon may be related to the acidity of the granule substances during intragranular maturation.

[A case report of inverted papilloma of the posterior urethra].

A case of inverted papilloma of the posterior urethra is reported. A 59-year-old male was admitted with the chief complaint of hematuria. Urethrogram revealed a small defect in the neck of the bladder. Endoscopic examination revealed a polypoid tumor on the stalk arising from prostatic urethra, and transurethral resection was performed. The patient has been subsequently followed up and there has been no evidence of recurrence. Although 141 cases of inverted papilloma have been reported in many anatomical sites of the urinary tract, only 19 cases involving the posterior urethra have been described in Japan. This is the 20th case of a posterior urethra.

Gamma-aminobutyric acid immunoreactivity in the enterochromaffin cells of the rat stomach.

The present immunocytochemical study revealed gamma-aminobutyric acid (GABA) immunoreactivity in the oxyntic and pyloric mucosa of the rat stomach at light- and electron-microscopic levels. GABA-immunoreactive endocrine cells were numerously seen in the lower half portion of the pyloric mucosa but rarely in the oxyntic mucosa. These cells were round or oval in shape and sometimes had a short cytoplasmic process. Serotonin-immunoreactive enterochromaffin (EC) cells were also observed in the oxyntic and pyloric mucosa of the stomach. The distribution and shapes of the immunoreactive cells were similar to those of the GABA-immunoreactive cells. With a double immunolabeling technique using anti-GABA and antiserotonin serum, GABA-immunoreactive endocrine cells showed serotonin immunoreactivity and were identified as EC cells. At the electron-microscopic level the GABA-immunoreactive cells contained round or oval, spindle-like, pear-shaped granules in EC cells. The immunoreaction product in the EC cells was generally confined to the granular cores. These findings suggest that GABA may be synthesized in the EC cells and be released from the granules of the cells after adequate stimuli.

[A case of Menkes' kinky hair disease with a renal calculus and diverticula of the bladder].

Menkes' kinky hair disease is a poor prognostic congenital disease with X-linked recessive inheritance. This disease is clinically characterized by seizures, friable hair, growth failure, mental retardation and others. Recently it has been known that this disease is also characterized by multiple diverticula of the bladder and other urological abnormalities. A 6-year-old boy was diagnosed as having Menkes' kinky hair disease at another hospital several weeks after birth because of seizures, friable hair and low serum copper level. He voided by Credé's maneuver by the nurse because he could not void for himself. He was referred to our hospital for macrohematuria and micturition pain on October 8, 1989. Right renal staghorn calculus and multiple diverticula of the bladder were found by roentgenographic examination. He has been treated conservatively after discharge because of his poor condition.