Single institutional experience of the treatment of angiosarcoma of the face and scalp.

OBJECTIVE: Angiosarcoma is a rare malignant neoplasm with a poor prognosis. A retrospective study was performed to accumulate radiotherapy (RT) data. METHODS: Data from 17 patients with angiosarcoma of the face and scalp (AFS) who were treated with definitive RT between January 1999 and July 2011 were retrospectively analysed. The total radiation dose was 70 Gy, and the fractional doses were 2.0-2.5 Gy. Combined with RT, chemotherapy using docetaxel alone, recombinant interleukin-2 immunotherapy alone and both of these was performed in 10, 4 and 2 patients, respectively. Three patients underwent limited surgery before RT. RESULTS: The response rate was 82%, and the median overall survival (OS) rate was 26 months. Locoregional relapse alone, distant metastasis alone and both of these were confirmed in 4, 5 and 4 patients, respectively. Patients treated with docetaxel showed a better prognosis (p=0.0477), a distant metastasis-free rate (p=0.0063) and a better in-field control rate, although the last was not statistically significant (p=0.1645). CONCLUSION: Definitive RT combined with docetaxel chemotherapy provided an effective approach for treating AFS. ADVANCES IN KNOWLEDGE: Since patients treated with chemoradiotherpy using docetaxel showed better OS and distant metastasis-free rates than those who did not receive docetaxel, it was warranted to continue use of docetaxel. In chemoradiotherapy at a dose of 70 Gy using docetaxel, 2-year in-field control rate was 67%.

Partial response of angiosarcoma of the scalp to sorafenib: association with decreased expression of vascular endothelial growth factors and their receptors.

The prognosis of angiosarcoma remains very poor, even with combined, multimodal therapy. We report a case with partial response of angiosarcoma of the scalp to sorafenib, which is a new oral, molecular, targeted, multiple-kinase inhibitor. In addition, we confirmed, using immunohistochemistry, that sorafenib suppressed the expression of vascular endothelial growth factors and their receptors on the angiosarcoma tumour cells, and decreased cell numbers by inhibiting cellular proliferation.

A child case of acute right coronary obstruction due to catheter-induced coronary dissection: successful reperfusion without stenting.

We report on a 10-year-old child who suffered from acute right coronary obstruction due to catheter-induced coronary dissection. Immediate placement of a perfusion catheter into the obstructed right coronary artery and subsequent overnight reperfusion allowed successful recovery of the right coronary artery lumen without implantation of a stent. Follow-up angiography demonstrated spontaneous regression of the dissected coronary artery and normal right and left ventricular wall motion. The indication of stent implantation should be carefully determined in a child case of iatrogenic coronary dissection because stenting may induce coronary stenosis during growth.

Zonisamide treatment of early infantile epileptic encephalopathy.

A case of early infantile epileptic encephalopathy with suppression-bursts (EIEE or Ohtahara syndrome) with an excellent response to zonisamide is reported. Tonic spasms began immediately after birth and increased in number, such that they formed a series of several dozen spasms lasting for 5 to 15 minutes. When the patient was 1 month old, a partial clonic seizure emerged predominantly on the right side of her face and extremities. It was often followed by tonic spasms and later continued after the beginning of the spasm series. Interictal electroencephalography demonstrated an atypical suppression-burst pattern and focal paroxysmal discharges. Detailed hematologic tests, magnetic resonance imaging, single-photon emission computed tomography, and auditory brainstem response did not reveal any metabolic or structural abnormalities. Oral administration of zonisamide first controlled the partial seizures and, subsequently, the tonic spasms in a dose-dependent fashion. The mode of response to zonisamide and the findings on electroencephalography indicate that EIEE may be caused by multiple independent brain lesions and that EIEE is a heterogeneous disorder. It is suggested that zonisamide may be useful in the treatment of EIEE.

[Two cases of metastasizing benign leiomyomatosis from myoma uteri].

Case 1. A 50-year-old woman was referred to our hospital because of multiple bilateral small round lesion on chest radiography. She had undergone total hysterectomy for myoma uteri at the age of 33. She underwent thoracoscopic tumor excision at left lung. The lesion was proved benign. Right side lesions were laterly excised using thoracotomy. Case 2. A 49-year-old woman was referred to us because of two ovoid lesion at left lung area of chest radiography. She also had undergone total hysterectomy for myoma uteri at the age of 37. She underwent tumor extirpation using thoracotomy. All samples of two patients revealed, pathologically, lesions were consisted of benign spindle-like calls similar to those of myoma uteri. Therefore, we consider these lesions were pulmonary metastasis of myoma uteri. Myoma uteri has certain potential of metastasizing to the lung, in spite of benign disease.

Relationship between antibody-dependent cell-mediated cytotoxicity due to anti-HTLV-1 and negative signal of major histocompatibility complex class I antigens on adult T-cell leukemia cell lines.

Natural killer (NK) cells possess two types of cytotoxic activity: natural killer cytotoxicity (NKC) and antibody-dependent cell-mediated cytotoxicity (ADCC). The NKC is regulated by the negative signal of the NK receptor, which recognizes major histocompatibility complex (MHC) class I antigens. However, it is not known whether or not the negative signal influences the ADCC. In this study, the relationship of the ADCC and negative signal was investigated. As target cells, adult T-cell leukemia (ATL) cell lines were used. When the target cells were treated with an anti-human T-lymphotropic virus type-1 (anti-HTLV-1) antibody, they were killed by the NK cells by means of the ADCC (ADCC/anti-HTLV-1). The killing levels were parallel with the cell surface HTLV-1 antigenicity. However, when these cells lines were treated with an anti-HLA, the ADCC (ADCC/anti-HLA) showed inverse correlation with the HLA antigenicity. Furthermore, when HLA polymorphic and monomorphic determinants of these target cells were blocked by F(ab')2 fragments of the anti-HLA and W6/32, the ADCC/anti-HLA was enhanced, but the ADCC/anti-HTLV-1 was not enhanced. These results suggest that the ADCC/anti-HLA may have an intimate relationship with the MHC class I antigens. The ADCC/anti-HLA may be suppressed by the negative signal. On the other hand, the ADCC/anti-HTLV-1 may have no relationship with the class I antigens and the negative signal may have no influence against the ADCC/anti-HTLV-1. The biological mechanism of this difference remains to be investigated.

Coronary artery aneurysms develop in weanling rabbits with serum sickness but not in mature rabbits. An experimental model for Kawasaki disease in humans.

The involvement of the cardiovascular system in rabbits with serum sickness is similar of that in Kawasaki disease, both with respect to the histologic changes of the myocardium, the valves, the coronary arteries, and the aorta and to the sequence with which they appear. Although coronary arteritis appears the same histologically, aneurysms did not occur in mature rabbits, even in case of necrosis of an arterial segment with deletion of muscle and elastic tissue. On the other hand, weanling rabbits with serum sickness had a tendency to develop aneurysms of coronary arteries, despite little cellular infiltration and reactive fibrocellular hyperplasia. The age factor thus appears to play an important role in the development of coronary aneurysms in Kawasaki disease, irrespective of the underlying mechanism, be it a markedly increased permeability to mediators through degenerated endothelial cells, or primary degenerated muscle cells with marked thinning of the media without much inflammatory cellular reaction. Furthermore, this study provides an insight into the long-term prognosis of transient dilatation of coronary arteries in the acute stage of Kawasaki disease.

QS pattern in the right precordial leads of a child with extensive myocardial fibrosis following myocarditis.

A QS pattern in the right precordial leads of the electrocardiogram was noted in a 9-year-old asymptomatic boy. Echocardiography revealed paradoxical movement of the interventricular septum, which showed interstitial myocardial fibrosis in the biopsy specimen and a defective uptake of thallium-201. Extensive myocardial fibrosis was probably a sequela of an attack of myocarditis in infancy.

R-wave voltage in the right precordial leads in anthracycline cardiomyopathy: a clinical study.

It was observed that the QS pattern in the right precordial leads of electrocardiograms of patients treated with anthracycline antibiotics resulted in congestive heart failure. In a review of the literature dealing with patients with anthracycline cardiomyopathy, terminal electrocardiograms, when available, showed the QS pattern in the right precordial leads in all patients. The relationship between a decreased R-wave voltage in lead V1 and an increase of the anthracycline dose was evaluated by a clinical study of ten patients. Analysis was subdivided into two categories (group A and B). In group A, there was a dose-response relationship. Electrocardiograms in group B, on the other hand, did not show low R-waves. The present study suggests that increased injury to myocardial cells in the regions of the anterior septum and anterior left ventricular wall might be important in the pathogenesis of anthracycline cardiomyopathy. Patients in group B seemed to be able to tolerate chronic anthracycline cardiac toxicity.

R-wave voltage in the right precordial leads in anthracycline cardiomyopathy: experimental animal model.

Electrocardiographic abnormality, similar to that observed in cancer patients during chemotherapy with the anthracycline antibiotics daunomycin and adriamycin, was produced in rabbits. They were chronically treated with adriamycin, and before each administration of the drug an electrocardiogram was recorded. They were subdivided into two categories with respect to the R-wave voltage in lead V1, on the same basis as our previous investigation in humans. In group A, there was a dose-response relationship, and they ultimately showed a QS pattern in the right precordial leads with or without ST-T changes in the left precordial leads. In group B, the electrocardiograms did not show a low R-wave in lead V1, but ST-T changes in the left precordial leads. The similarity of these electrocardiographic changes to those produced by myocardial infarction that we observed in humans suggests that more severe injury to the myocardium at the anterior septum and anterior left ventricular wall may be important in the pathogenesis of anthracycline cardiomyopathy.

Rheumatic fever as a cause of myocardial infarction with coronary narrowing.

A 12-year-old boy with rheumatic fever associated with myocardial infarction is presented. As sequelae, left ventricular aneurysm and diffuse narrowing of the anterior descending branch of the left coronary artery were demonstrated by angiography. Given the high incidence of coronary arteritis in rheumatic fever, it is likely that arteritis contributed to the infarction.