Renal primitive neuroectodermal tumor: An immunohistochemical and cytogenetic analysis

The cytogenetic and morphologic characteristics of a case with a primitive neuroectodermal tumor (PNET) arising from the left kidney in a 22 year old man are presented. The patient was detected as having a left renal mass with a tumor embolus In the inferior vena cava and multiple pulmonary metastases. A radical nephrectomy with tumor embolectomy of the Inferior vena cava, along with a resection of the pulmonary nodules were performed. Histologic examination revealed a dense proliferation of small round cells with many Homer-Wright type rosettes and perlvascular pseudo-rosettes. Immunohlstochemically, the tumor cells stained strongly positive for HBA71(p30/32^{M IC2}), a surface glycopro-teln specific to PNET and Ewlng's sarcoma. In addition, the tumor cells expressed several neural markers (neuron specific enolase, neurofilament, synaptophysin, and Leu-7) and vimentin, while the epithelial, muscular, and lymphocytlc markers were negative in the tumor cells. Cytogenetic analysis of cultured tumor cells showed a reciprocal translocation t(11; 22)(q24; q12) that is considered to be specific to PNET and Ewing's sarcoma. In conclusion, this case suggested that a karyotyping analysis is a useful diagnostic tool for renal PNET, and it may therefore be utilized to help distinguish between difficult cases of small round cell tumors and Wilms' tumor of the kidney.     

Malignant eccrine poroma with multiple visceral metastases: report of a case with autopsy findings

An autopsy case of malignant eccrine poroma (MEP) with multiple visceral metastases is reported. A flat, dark tumor of 1 cm in diameter developed on a pre-existing pigmented spot at the left side of the waist of a 58-year-old male. The histopathology suggested the tumor to be malignant melanoma. Nine years later, a painless swelling occurred in the left lower leg, resulting from the obstruction of lymphatics at the left inguinal region. The swelling continued and spread with pain, reaching the inguinal region. Two years later, several papules appeared around the left knee, from which an extensive lymphorrhea occurred. The histopathology of the resected papules suggested epidermoid carcinoma or trichilemmal carcinoma, mainly localized within the lymphatics of the upper dermis. Re-examination of the first skin tumor and electron microscopy of the tumor obtained at autopsy revealed that both tumors were MEP. Although the metastases to the local cutaneous regions and lymph nodes via the lymphatics occur in 20% of MEP, cases with multiple visceral metastases are very few.     

The behavior of99mTc-hexamethylpropyleneamineoxime (99mTc-HMPAO) in blood and brain

^99mTc-hexamethylpropyleneamineoxime (^99mTc-HMPAO) is a reagent for scanning cerebral blood flow. We investigated how^99mTc-HMPAO changed in the blood and brain. The^99mTc-HMPAO, which was prepared by adding of^99mTcO _- ⁴ to HMPAO and Sn(II), consisted of primary and secondary complexes, reduced hydrolyzed^99mTc, and^99mTc0pertechnetate. The percentage of the primary complex in^99mTc-HMPAO decreased with time after preparation. The primary complex converted to the secondary one very rapidly in the presence of plasma. When^99mTc-HMPAO was injected into patients,^99mTc activity was immediately partitioned in the plasma fraction, with approximately 60% in whole blood. In plasma,^99mTc was found to be associated with proteins such as albumin and globulin.^99mTc trapped in red cells was not washed out with either plasma or saline. Biodistribution studies showed that the less lipophilic compounds of^99mTc-HMPAO could not pass through the blood brain barrier (BBB), and therefore did not accumulate in the brain. The results of gel chromatography and equilibrium dialysis indicated that no specific^99mTc binding protein was present in the brain. Considering the instability of^99mTc-HMPAO in vivo, we proposed that the speed at which the primary complex converted to the less lipophilic compounds was important in allowing^99mTc-HMPAO to pass through the BBB and to be fixed in the brain.     

Multiple Regression Analysis of Airway Responsiveness in Adult Asthmatic Patients

The aim of this study was to determine which background variables exert the most influence on airway responsiveness in adult asthmatic patients. The relationships between airway response and background variables were investigated by multiple linear and logistic regression analysis in 97 asthmatic patients. Airway responsiveness was measured by the oscillation technique during metha-choline inhalation challenge. The regression analyses revealed that modified Aas score “one of asthma severity score,” was the major factor contributing to airway hyperreactivity. We conclude that the number of attacks during a previous year is the most important background factor related to airway hyperre-sponsiveness on a clinical basis.     

Perioperative Prostaglandin E1 Treatment for the Prevention of Postoperative Complications After Esophagectomy: A Randomized Clinical Trial

Purpose We conducted a prospective randomized clinical study to examine whether perioperative prostaglandin E1 (PGE1) could help in the prevention of postoperative complications after esophagectomy for esophageal cancer.Methods Forty patients with esophageal cancer eligible for radical esophagectomy were randomly assigned to an experimental group (n = 20), given perioperative PGE1, or to a control group (n = 20), given standard postoperative treatment. The main clinical endpoints examined were the incidence of postoperative complications, hospitalization, duration of systemic inflammatory response syndrome (SIRS), portal vein blood flow, and serum bilirubin levels.Results Severe postoperative complications developed in five patients in the control group and two in the PGE1 group. There was one surgery-related death in the control group. The duration of SIRS was significantly shorter in the PGE1 group than in the control group (5.74 days vs 7.50 days; P = 0.047). Portal vein flow was also significantly lower on postoperative day (POD) 1 in the control group than in the PGE1 group (P = 0.042). Maximum postoperative serum bilirubin levels were significantly lower in patients treated with PGE1, at 2.91 vs 4.38mg/dl in the control group (P = 0.040).Conclusions The perioperative administration of PGE1 helps maintain adequate portal blood flow, improves hyperbilirubinemia, and attenuates the duration of SIRS, thereby reducing the risk of postoperative complications after esophagectomy and lymphadenectomy for esophageal cancer.     

Accumulation of genetic alterations during esophageal carcinogenesis

Using polymerase chain reaction amplification of microsatelliteregions in DNA from 11 epithelial dysplasias of the esophagusand 21 early squamous cell carcinomas, we were able to detectfrequent loss of heterozygosity (LOH) on chromosomes 3p21.3and 9q31 even in low-grade dysplasias. In contrast, we observedfrequent LOHs on chromosomes 9p22 and 17p13 (TP53 locus) onlyin high-grade dysplasias and carcinomas, but not in any low-gradedysplasias. Analysis of LOH at the same four chromosomal regionsIn DNA of five additional minimal carcinomas and accompanyingdysplastic lesions revealed loss of alleles at the loci on 3p21.3and 9q31 throughout various degrees of dysplasia and carcinoma;again, LOHs on 9q22 and 17p13 occurred only in high-grade dysplasiaand carcinoma in situ. Our results indicated that Inactivationof putative tumor suppressor genes on 3p21.3 and 9q31 may beearly genetic events during esophageal carcinogenesis, and thatadditional genetic alterations on 9p22 and 17p13 probably playroles in progression.     

A case of a giant phyllodes tumor of the breast with hypoglycemia caused by high-molecular-weight insulin-like growth factor II

We report the case of a patient who presented with hypoglycemia associated with a giant breast mass and presence of serum high-molecular-weight insulin-like growth factor II (big IGF-II). In July 2005, a 49-year-old woman was admitted because of delirium, transient loss of consciousness, and a giant mass of about 28 cm in diameter on the right breast. She had noticed the mass for more than 2 years, but had refused medical attention at that time. A blood examination indicated hypoglycemia (21 mg/dl) and decreased levels of endogenous insulin. Furthermore, a western blot analysis revealed that big IGF-II (20 kDa) was the predominant serum IGF-II peptide (mature IGF-II is 7.5 kDa). Because we suspected that the big IGF-II was produced by the breast tumor and was likely the cause of the hypoglycemia, a mastectomy was performed. A histological examination determined that the mass was a benign phyllodes tumor. After surgery, the hypoglycemia resolved, and endogenous insulin levels improved. We suspected that the patient had non-islet cell tumor hypoglycemia (NICTH), but the behavioral symptoms of the hypoglycemia caused by NICTH were similar to some mental diseases, which made diagnosis based on the behavior alone difficult. We suggest that co-occurrence of symptoms such as recent appearance of mental disease-like behavior, hypoglycemia, and giant breast tumor may help diagnose NICTH caused by big IGF-II.     

Efficacy of prophylactic sclerotherapy in patients with hepatocellular carcinoma and varices negative for the red color sign

Background: A prospective randomized controlled study was performed to evaluate the usefulness of prophylactic endoscopic sclerotherapy in patients with hepatocellular carcinoma complicated by esophageal varices. Methods: The subjects included 58 patients with esophageal varices negative for the red color sign and hepatocellular carcinoma without tumor emboli in the portal trunk or primary portal branches. Patients were randomly assigned to prophylactic sclerotherapy (n = 29) or control (n = 29) groups, and their bleeding and survival rates were compared. Results: A mean of 3.0 sclerotherapy sessions was required for complete disappearance of varices in patients receiving prophylactic sclerotherapy. During the observation period, transcatheter arterial embolization for hepatocellular carcinoma was performed more often in patients with prophylactic sclerotherapy (mean 3.8 times) than in control patients (mean 2.0 times) (p < .05). Percutaneous ethanol injection therapy was performed more often in patients with prophylactic sclerotherapy than in controls (mean 8.1 times vs 5.0 times, respectively) (p < .05). The 3-year bleeding rates were 50% for the control group and 18% for the prophylactic sclerotherapy group (p < 0.05), and the 3-year survival rates were 16% for the control group and 37% for the therapy group (p < 0.05). Conclusions: Prophylactic sclerotherapy improves survival in patients with hepatocellular carcinoma complicated by red color sign–negative esophageal varices without tumor emboli in the portal trunk or primary portal branches. (Gastrointest Endosc 1997;45:498-502.)     

Gitelman's syndrome with exercise-induced ventricular tachycardia.

A 62-year-old female with palpitations was admitted to hospital where she recorded 12,299 monofocal ventricular premature contractions (VPCs) in 24 h and nonsustained ventricular tachycardia (VT) on exertion. She had hypokalemia with renal potassium wasting, a chloride-resistant metabolic alkalosis, elevated plasma renin, elevated plasma aldosterone (relative to the serum K concentration), hypomagnesemia with renal magnesium wasting, decreased urine calcium excretion, and normal blood pressure. The hypokalemia and hypomagnesemia were thought to have precipitated the VT. The coronary angiogram showed normal coronary arteries; however, the left ventriculogram revealed akinesis of the posterolateral wall. Because the VT could not be induced by programmed electrical stimulation either before or during intravenous administration of isoproterenol, the VPC with the same QRS morphology as the VT became the target of radiofrequency catheter ablation (RF-CA). Intracardiac mapping showed that the earliest activation site was situated in the asynergic area of the left ventricle (LV) and radiofrequency catheter ablation directed at the LV asynergy area completely eliminated the VPCs without any complications. During the follow-up period (6 months), she was free from palpitation and VT was not clinically documented.     

Embolotherapy for persistent hemoptysis: The significance of pleural thickening

Elective embolotherapy for recurrent hemoptysis was evaluated for its effectiveness. Forty embolotherapeutic procedures were performed in 30 patients. In the short term, complete hemostasis was achieved in 16 of 40 procedures (40%) and significant improvement in another 16 procedures (40%). Long-term complete hemostasis was achieved in 14 of 34 procedures (41%) and significant improvement in 12 (35%). Embolotherapy was consistently more effective in patients who had no pleural abnormalities compared with those with pleural thickening. Long-term complete hemostasis was achieved in 7 of 10 procedures (70%) for the patients without pleural thickening and only 7 of 24 (29%) in patients with pleural thickening. Thus, pleural abnormalities negatively influence long-term effectiveness of embolotherapy.