Kaposiform hemangioendothelioma: five patients with cutaneous lesion and long follow-up.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi's sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of cutaneous lesions being the most commonly affected site, but also for its clinical diversity. Early diagnosis is possible even for a small skin lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are well, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.     

Surgical management of faecal incontinence in adults

Faecal incontinence has a long list of aetiology. Definitive treatment depends on the underlying condition. If a non‐surgical cause is identified, conservative treatment is often appropriate. Surgical management is more often successful when a structural damage of the anal sphincter is found. Overlapping sphincter repair has a high success rate when the anal sphincter is disrupted. Surgery has a clear role to play in the case of idiopathic faecal incontinence; and sacral nerve stimulation is the most promising treatment presently available. Dynamic muscle transposition and artificial bowel sphincter are the remaining alternatives after unsuccessful attempts at sphincter repair or in the case of end‐stage faecal incontinence. Spinal cord injury or diseases contribute significantly to faecal incontinence. Malone’s antegrade continence enema has been shown to be helpful to these patients. The greatest benefit of this treatment modality appears to occur with children. With adults, experience is sparse. For the debilitated geriatric patient whose faecal incontinence has led to perianal skin excoriation, bed sores or perianal sepsis, a well‐sited colostomy is sometimes the kindest option apart from diligent nursing care. Biofeedback and pelvic floor exercises are helpful adjuncts and sometimes the primary mode of therapy. It carries no risk in its own right and is worth trying provided the patient is motivated and a dedicated therapist is available.     

Therapeutic efficacy of hepatitis B surface antigen-antibodies-recombinant DNA composite in HBsAg transgenic mice.

Therapeutic efficacy of HBsAg-anti-HBs-recombinant DNA harboring hepatitis B virus (HBV) S gene complex was compared with three other therapeutic vaccine candidates (recombinant HBsAg, HBsAg complexed to anti-HBs antibodies and naked plasmid DNA encoding the HBV S gene). After four injections at 3-week intervals, the most pronounced decrease of serum HBsAg, the highest titer of anti-HBs response, the highest level of interferon-gamma produced by splenocytes and potent cytotoxicity T cell response were observed in the HBsAg-anti HBs-sDNA immunized group. Reduced expression of HBsAg in hepatocytes was also shown. The therapeutic mechanism of HBsAg-anti-HBs-DNA was speculated as modulation of HBsAg presentation via both endogenous and exogenous pathways.     

Results of high-density silicone oil as a tamponade agent in macular hole retinal detachment in patients with high myopia

BACKGROUND: To evaluate the use of high-density silicone oil (HDSO) as a tamponade agent for retinal detachment secondary to myopic macular hole. METHODS: 12 eyes of 12 patients with macular hole retinal detachment underwent pars plana vitrectomy, internal limiting membrane peeling and HDSO tamponade. No posturing was required postoperatively and HDSO was removed 3-4 months later. Outcome measures included macular hole closure and retinal attachment rates, best-corrected visual acuity (BCVA), and intraoperative and postoperative complications. RESULTS: The mean age of the patients was 67.8 years and the mean spherical equivalent refractive error was -13.4 diopters. After the removal of HDSO, 10 (83%) eyes had macular hole closure with retinal reattachment without any tamponade. One eye had retinal reattachment after re-operation and the other refused further surgery. At the last follow-up, the median BCVA improved from 20/800 to 20/600 (p = 0.046). A transient increase in intraocular pressure was observed in 5 (42%) eyes and one eye each developed mild oil emulsification and transient peripheral choroidal detachment. None of the eyes was found to have severe intraocular inflammation postoperatively. CONCLUSIONS: HDSO seemed to be an effective tamponade agent for myopic macular hole retinal detachment. Further prospective controlled studies seem warranted.     

Regulation of MYCN expression in human neuroblastoma cells

Background  

Amplification of the MYCN gene in neuroblastoma (NB) is associated with a poor prognosis. However, MYCN -amplification does not automatically result in higher expression of MYCN in children with NB. We hypothesized that the discrepancy between MYCN gene expression and prognosis in these children might be explained by the expression of either MYCN-opposite strand (MYCNOS) or the shortened MYCN-isoform (ΔMYCN) that was recently identified in fetal tissues. Both MYCNOS and ΔMYCN are potential inhibitors of MYCN either at the mRNA or at the protein level.     

Western Australian cigarette smokers have fewer small lung nodules than North Americans on CT screening for lung cancer

To determine the prevalence of small lung nodules on low‐dose helical computed tomography (CT) in a Western Australian cohort of asymptomatic long‐term cigarette smokers and to compare this with a large, similarly derived cohort of North Americans from the Mayo Clinic Lung Cancer Screening Trial. Forty‐nine asymptomatic long‐term cigarette smokers of minimum age 50 years underwent a low‐dose 64‐slice helical CT of the lungs. Images were viewed on a soft copy reporting station with thin section axial and coronal images, maximum intensity projection images, and advanced image manipulation tools. The prevalence of all nodules was 39%, significantly lower than the Mayo Clinic cohort prevalence of 51% (P < 0.01, Fisher's exact test), despite the use of more advanced imaging technology and image manipulation designed to increase the sensitivity for nodules. The prevalence of small nodules in asymptomatic long‐term cigarette smokers in Western Australia is high, though significantly less than that found in a large study in North America. The authors postulate this is due to the relatively low rates of mycobacterium tuberculosis and soil‐derived fungal pulmonary infections in Western Australia, as well as a lower degree of urban air pollution.     

Promoter methylation of the Wnt/β‐catenin signaling antagonist Dkk‐3 is associated with poor survival in gastric cancer

BACKGROUND:

Abnormal activation of the Wnt/β‐catenin signaling pathway is common and critical in the pathogenesis of digestive cancers. In this study, the authors investigated the promoter methylation of the dickkopf homolog 3 gene Dkk‐3 in these cancers and its prognostic significance in gastric cancer.

METHODS:

Dkk‐3 methylation was assessed in 173 patients with gastric cancers (including 104 patients who were followed for up to 4090 days) and in 128 patients with colorectal cancer. Cell growth was evaluated by using a colony‐formation assay. For survival analyses, the authors used Kaplan‐Meier plots, the log‐rank test, and Cox proportional regression.

RESULTS:

Dkk‐3 was silenced or down‐regulated in 12 of 17 gastric cancer cell lines (70.6%) and in 3 of 9 colon cancer cell lines (33.3%). The loss of gene expression was associated with promoter methylation, which could be restored by demethylating agents. Ectopic expression of Dkk‐3 suppressed colony formation. Moreover, methylation of Dkk‐3 was detected in 117 of 173 primary gastric tumors (67.6%) and in 67 of 128 colorectal tumors (52.3%). The clinical significance and the prognostic value of Dkk‐3 methylation also were examined in 104 gastric cancers and in 84 colorectal cancers. Multivariate analysis indicated that Dkk‐3 methylation was associated significantly and independently with poor disease survival (relative risk, 2.534; 95% confidence interval, 1.54–4.17; P = .002) in gastric cancer, but not in colorectal cancer. Kaplan‐Meier survival curves revealed that patients who had Dkk‐3 methylated gastric cancers had a significantly shorter survival (median, 0.76 years) compared with patients who did not have Dkk‐3 methylation (median, 2.68 years; P < .0001; log‐rank test).

CONCLUSIONS:

Epigenetic silencing of the Dkk‐3 gene by promoter methylation was a common event in gastric cancer and was associated with a poor outcome in such patients. Cancer 2009. © 2008 American Cancer Society.     

Malignant oncocytoma of the lacrimal sac as an unusual cause of epiphora

A 72-year-old man presented with a 2-year history of epiphora. CT revealed an extensive inferomedial orbital tumor connected to the lacrimal sac and duct. Incisional biopsy revealed malignant oncocytoma of the lacrimal sac. The patient was treated with exenteration and maxillectomy followed by a course of postoperative radiotherapy. Patients with malignant oncocytoma may present with simple epiphora in absence of other signs and symptoms such as blood stained tearing or purulent rhinorrhea.