Treatment of lymphangioma in children: our experience of 128 cases

Purpose

The aim of this study is to investigate the optimal treatment for lymphangioma in children by analyzing the effectiveness and complications of treatment modalities.

Methods

We reviewed 128 patients with lymphangioma treated at our institution between 1979 and 2005. Periods of treatment were divided arbitrarily into 2 groups: period I, from 1979 to 1988 (n = 53); and period II, from 1989 to 2005 (n = 75). According to radiological appearance, patients were grouped into 4 types: single cystic (SI; n = 23), macrocystic (MA; n = 11), microcystic (MI; n = 69), and cavernous (CA; n = 25).

Results

Sclerotherapy as primary treatment was performed in only 2 patients (3.8%) in period I using bleomycin but increased significantly in period II to 48 patients (64.0%) using OK-432 (P < .01). Nevertheless, primary surgical excision (69/78 patients, 88.5%) was significantly more successful than sclerotherapy (32/50, 64.0%) (P < .01). The following are the locations and types of lesions: head/neck (n = 69; SI, 11; MA, 5; MI, 42; CA, 11), trunk (n = 34; SI, 6; MA, 6; MI, 15; CA, 7), and extremities/other (n = 25; SI, 6; MI, 12; CA, 7). The effectiveness of sclerotherapy in SI, MA, MI, and CA types was 90.9%, 100%, 68.0%, and 10.0%, respectively. Seventeen patients (SI, 1; MI, 8; CA, 8) who received primary sclerotherapy required surgical excision with good outcome. Complications after primary surgical excision were more serious compared with sclerotherapy.

Conclusion

Sclerotherapy with OK-432 was not as effective as reported in the literature. We recommend OK-432 injection therapy alone for SI and MA types and surgical excision after pretreatment with OK-432 for MI and CA types.     

Successful CPT-11 treatment in a patient with pancreatic cancer associated with multiple liver metastases and chronic renal failure

A 56-year-old woman, who had been receiving treatment for chronic renal failure, was admitted to our Department because of a tumor of the pancreas head and multiple liver masses diagnosed by abdominal CT scans. Gastroduodenoscopy revealed a tumor which had invaded the Vater's papilla; the lesion was histopathologically pancreatic adenocarcinoma. Due to the presence of multiple metastases to the liver, we therefore performed general chemotherapy after obtaining the patient's informed consent (IC). CPT-11 was selected as the carcinostatic agent because the patient suffered from renal failure. The initial dose of CPT-11 was 80 mg, and we thereafter made minor adjustments in the dosage depending on the occurrence of side effects. After four courses of the treatment, a CT scan revealed both the tumor of the pancreas head and the multiple liver masses to have almost completely disappeared. Our clinical results indicate that CPT-11 may therefore be a strong candidate for first-line chemotherapy for the treatment of pancreatic cancer, especially in patients with renal failure.     

Mechanisms underlying enhanced vasodilator responses to various vasodilator agents following endothelium removal in rat mesenteric resistance arteries

We reported that vasodilator responses to various vasodilator agents were augmented by endothelium removal. To explain this mechanism, we hypothesized that endothelium removal eliminates the release of endothelium-derived contracting factor EDCF, which counteracts the vasodilation. However, the underlying mechanism is unknown. Therefore the present study investigated the second messenger system further to investigate the mechanisms underlying enhanced vasodilator response after endothelium removal in rat mesenteric resistance arteries. Mesenteric vascular beds isolated from Wistar rats were perfused and perfusion pressure was measured. The vascular endothelium was removed by 30-s perfusion of sodium deoxycholate. Vasodilator responses to sodium nitroprusside (SNP) perfusion were markedly augmented and prolonged by endothelium removal. In preparations with intact endothelium and active tone, 5-min perfusion of sodium azide (non-specific guanylate cyclase (GC) activator), ANP (membrane-linked GC activator), and 8-Br-cGMP (cGMP analogue) caused a concentration-dependent vasodilation that was markedly augmented by endothelium removal. However, vasodilation induced by YC-1 and BAY41-2272 (selective soluble GC activator) was not augmented by endothelium removal. When methylene blue (soluble GC inhibitor) was present in the medium, SNP caused a concentration-dependent vasodilation in the preparation with intact endothelium, which was less augmented by endothelium removal compared with control (preparation without methylene blue). These findings suggest that endothelium removal affects intracellular cGMP-mediated signal transduction system in vascular smooth muscle cells.     

Rapamycin, but not cyclosporine or FK506, alters natural killer cell function

Infiltration of natural killer (NK) cells into solid organ allografts is observed in clinical and experimental transplantation. Studies suggest a role for NK cells in acute and chronic rejection of solid organ allografts; however, the effects of immunosuppressive agents on NK cells are not clearly established. Rat NK cell lines were analyzed for proliferation and cytotoxicity in the presence of cyclosporine, FK506, or rapamycin. Lewis recipients of DA liver allografts received immunosuppressive agents after transplantation. NK cells demonstrated robust function both in the absence and presence of cyclosporine and FK506. In contrast, rapamycin significantly inhibited proliferation and cytotoxicity of NK cells. NK cell numbers remained stable in graft recipients treated with cyclosporine and FK506, whereas there was a significant decrease in NK cells in rapamycin-treated recipients. These data indicate that immunosuppressive drugs have differential effects on NK cell function that may impact the immune response of transplant recipients.     

Bioactive lignans from Peperomia duclouxii

Six new lignans (1-6), along with 14 known compounds, were obtained from Peperomia duclouxii. The new structures were elucidated mainly by the analysis of their NMR and MS data. The absolute configurations of 1-6 were determined by comparing their optical rotations or CD spectra with those of known compounds. In cytotoxic and MDR reversal cell activity assays, compound 3 showed cancer cell growth inhibitory activity against VA-13 and HepG2 cells, with IC50 values of 5.3 and 13.2 microg/mL, and more potent effects on calcein accumulation in MDR 2780AD cells than verapamil, a positive control. Compound 6 showed anti-inflammatory activity using an ICAM-1 assay (induction of the intercellular adhesion molecule-1), stimulated by IL-1alpha and TNF-alpha.     

Churg‐Strauss syndrome with coexistence of eosinophilic vasculitis,granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid‐like blisters

The main histopathological features in the cutaneous lesions of Churg‐Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non‐vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Ishibashi M, Kudo S, Yamamoto K, Shimai N, Chen K‐R. Churg‐Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid‐like blisters.