Peliosis Hepatis in a Child with X-Linked Myotubular Myopathy Treated with Living-Donor Liver Transplant: A Case Report

BackgroundMyotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage.Case ReportWe present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing living-donor liver transplant, the patient showed no recurrence of peliosis hepatis.ConclusionsAlthough the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.     

Allelotype and replication error phenotype of small cell lung carcinoma

Allelotype and replication error (RER) phenotype analyses were performed to clarify the pathogenetic significance of inactivation of tumor suppressor genes and genomic instability in the genesis and progression of small cell lung carcinoma (SCLC). We examined 37 cases of SCLC for loss of heterozygosity (LOH) and microsatellite instability at 49 loci on all 39 nonacrocentric chromosomal arms. LOH was frequently (>70%) detected on chromosomes 3p (29/32, 90.6%), 5q (15/21, 71.4%), 13q (25/26, 96.2%), 17p (22/25, 88.0%), and 22q (24/33, 72.7%). Frequent LOH (>70%) on these loci was observed even among seven cases of stage I tumors. The incidence of LOH on all 39 nonacrocentric chromosomal arms was not significantly different between primary tumors and metastases. These results suggest that inactivation of multiple tumor suppressor genes accumulates relatively early during progression of SCLC and it may be responsible for clinically and biologically aggressive phenotype of SCLC. RER was observed in 6/37 (16.2%) of SCLC, however, RER at multiple loci was observed only in two cases. Therefore, it was indicated that genomic instability is uncommon, but might play a role in the genesis of a small subset of SCLC.      

Correlation between high-resolution computed tomographic, magnetic resonance and pathological findings in cases with non-cancerous but suspicious lung nodules

Computed tomography scans, including thin-section high-resolution computed tomography (HRCT), occasionally fail to differentiate between small non-cancerous nodules from lung cancers. We describe nine such lesions ( < 20 mm in diameter) initially identified through our screening program for lung cancer using CT scanning. Pathological diagnoses included nodular fibrosis (n = 4), granuloma (n = 1), cryptococcoma (n = 1), localised organising pneumonia (n = 1), inflammatory pseudo-tumour (n = 1) and sclerosing haemangioma (n = 1). High-resolution CT findings, together with MRI findings with contrast-enhanced dynamic studies, were retrospectively evaluated. Additional cases should be identified and radiologically characterised in order to reduce the number of non-cancerous tumours that are treated by unnecessary surgery. Received: 28 February 2000; Accepted: 29 February 2000     

An autopsy case of a stunt man who jumped into seawater riding a motorcycle: a case report

We report a rare case of the death of a stunt man who jumped into the sea driving a motorcycle on location for a TV drama. A special ramp had been constructed for the jump. The stunt man and a passenger drove up the ramp at a speed of 60-70 km/h and jumped into the sea from a height of about five metres. Both men swam up, but the driver fell unconscious at the surface. He was sent to hospital, but did not recover. The autopsy revealed a deep laceration of the right axilla with complete rupture of the axillary artery, bone fractures of the right ribs and the right pelvis, and a small laceration of the upper lobe of the right lung. Other visceral organs were not injured. We finally judged that his cause of death was exsanguination due to rupture of the right axillary artery on the basis of the presence of a small amount of blood inside the heart and ischemic organs. All injuries were probably inflicted by a collision with the motorcycle upon reaching the water surface; the axillary injury might be due to the left handlebar piercing into the part.     

Relationship Between Diffusion-Weighted Magnetic Resonance Imaging and Histological Tumor Grading of Hepatocellular Carcinoma

Background

Intrahepatic and extrahepatic recurrence remains a significant problem for hepatocellular carcinoma (HCC). The aim of this study was to determine the usefulness of diffusion-weighted magnetic resonance imaging (DWI) for histological tumor grading and preoperative prediction of early HCC recurrence within 6 months of operation.

Methods

A total of 44 patients who had undergone hepatic resection for HCC (50 nodules) were reviewed retrospectively. DWI was performed within 30 days before hepatectomy, and apparent diffusion coefficients (ADCs) were measured using 2 methods: mean ADC and minimum-spot ADC. Relationships between ADCs and histological differentiation and between ADCs and early recurrence of HCC were analyzed.

Results

Mean ADC was significantly lower in poorly differentiated HCC (n = 18, 1.07 ± 0.15 × 10^?3 mm²/s) than in moderately differentiated HCC (n = 29, 1.29 ± 0.21 × 10^?3 mm²/s; P < .05). Minimum-spot ADC was significantly lower in poorly differentiated HCC (n = 18, 0.69 ± 0.19 × 10^?3 mm²/s) than in well-differentiated HCC (n = 3, 1.15 ± 0.10 × 10^?3 mm²; P < .01) or in moderately differentiated HCC (n = 29, 0.98 ± 0.18 × 10^?3 mm²/s; P < .0001). Of 34 patients who were able to be observed for >6 months after resection, 9 showed early recurrence. Minimum-spot ADC was significantly lower in patients with early recurrence (n = 9, 0.64 ± 0.24 × 10^?3 mm²/s) than in patients without early recurrence (n = 25, 0.88 ± 0.19 × 10^?3 mm²/s; P < .05). On multivariate analysis, minimum-spot ADC was a significant risk factor for early recurrence (P < .05).

Conclusion

Quantitative measurement of ADC of HCC with magnetic resonance diffusion weighted imaging is a promising functional imaging tool in the prediction of histological grade and early recurrence before treatment.     

Successful Combination Therapy of Radical Liver Resection With 5-Fluorouracil/leucovorin,Oxaliplatin, Plus Bevacizumab for Ascending Colon Cancer With Pulmonary and 43 Liver Metastases: Report of a Case

At the time of diagnosis, 20% to 25% of patients with colorectal cancer already have liver metastases, the presence of which is a most important prognostic factor. A 64-year-old man was admitted to our hospital for investigation of anemia and multiple liver tumors. Examinations revealed ascending colon carcinoma with more than 40 liver metastases and 2 lung metastases. We performed right hemicolectomy with lymph node dissection, which was followed by 5-fluorouracil/leucovorin, oxaliplatin, plus bevacizumab (FOLFOX-BV). After 4 courses of chemotherapy, the lung metastases were in complete remission and the liver metastases had shrunk. We suggested the option of radical liver resection, but the patient declined initially as he had not suffered any severe side effects of FOLFOX-BV. After 23 courses of the chemotherapy, he agreed to undergo hepatectomy. We performed extended right lobectomy with partial left and caudal lobe resection. All of the macroscopic metastatic lesions were resected. Histopathologically, viable cancer cells were recognized in 7 of the 43 liver metastatic lesions. Postoperatively, FOLFOX-BV was restarted and continued for 10 months. At the time of writing, 15 months after the hepatectomy, the patient was well without evidence of recurrence of the cancer.     

Superselective continuous arterial infusion chemotherapy through the superficial temporal artery for oral cavity tumors

BACKGROUND AND PURPOSE: High-dose intraarterial chemotherapy with repeated one-shot infusion may be useful for treating head and neck tumors. We evaluated the efficacy of superselective continuous arterial infusion chemotherapy administered via a coaxial catheter system and compared the results with those of subselective catheterization for treatment of oral cavity tumors. METHODS: Forty-nine consecutive patients with tumors of the oral cavity (clinical stage I, 12 cases; stage II, 19 cases; stage III, six cases; stage IV, 12 cases) were treated by arterial infusion chemotherapy. After a guiding catheter was advanced into the superficial temporal artery, superselective catheterization was performed using a coaxial system microcatheter. Superselective catheterization was accomplished in 34 cases, and was unsuccessful in 15, owing to difficulties in performing catheterization or to multiple feeding arteries. In the latter cases, the tip of the catheter was placed near the origin of the feeding arteries (subselective catheterization). RESULTS: Thirty (88%) of 34 patients had a complete response to superselective arterial infusion chemotherapy and two (6%) had a partial response. Twelve (80%) of 15 patients had a complete response to subselective arterial infusion chemotherapy and three (20%) had a partial response. Local recurrence was more frequent after subselective treatment (13%) than after superselective (6%) treatment. CONCLUSION: Superselective continuous arterial infusion chemotherapy may be suitable for local control of oral cavity tumors, with a low rate of recurrence.     

Human brain microsomes: their abilities to metabolize tetrahydrocannabinols and cannabinol

In spite of the psychedelic action of Δ⁹-tetrahydrocannabinol (Δ⁹-THC) in the brain, no report on its metabolism by human brain microsomes has been published. In this study, the metabolism of Δ⁸-THC, Δ⁹-THC and cannabinol (CBN) was studied using human brain microsomes. The metabolites formed were analyzed by gas chromatography–mass spectrometry after trimethylsilylation. The three cannabinoids were biotransformed to two main metabolites by human brain microsomes. Δ⁸- and Δ⁹-THCs were mainly oxidized at the allylic positions. The main metabolites of Δ⁸-THC were 7α-hydroxy- and 11-hydroxy-Δ⁸-THCs, whereas those of Δ⁹-THC were 8α-hydroxy- and 11-hydroxy-Δ⁹-THCs. CBN was metabolized to 8-hydroxy- and 11-hydroxy-CBNs. Although the primary metabolic pathways of the THCs and CBN in brain microsomes are different from those in liver microsomes for other mammalian species, those in human brain microsomes were similar to those in human liver microsomes.     

A gastric duplication cyst with an aberrant pancreatic ductal system: report of a case

We report an extremely rare case of a gastric duplication cyst together with an aberrant pancreatic ductal system, which communicated with the stomach rather than the pancreatic ductal system with no evidence of pancreatitis. A 46-year-old woman developed severe abdominal pain after a 10-year history of occasional mild abdominal pain. Upper gastrointestinal barium radiography showed a rigidity of the stomach wall, and gastroscopy revealed a fistula orifice at a greater curvature of the gastric body. Subsequent endoscopic suction of mucous secretion from within the fistula provided immediate pain relief. Abdominal computed tomography and ultrasonography showed a cystic mass contiguous with the stomach wall. Surgical exploration revealed an uncommon anomaly of a gastric duplication cyst with the aberrant pancreatic lobe. The patient made an uneventful recovery and remains well 4 years after surgery. We also herein review ten other similar cases of this uncommon congenital anomaly reported in the literature. Received: August 20, 2001 / Accepted: January 8, 2002