Pediatric inflammatory myofibroblastic tumor of the bladder with ALK–FN1 fusion successfully treated by alectinib

An inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm characterized by the proliferation of myofibroblasts and inflammatory cell infiltration. Although radical resection is the only established treatment strategy for IMT, it can cause functional disorders when vital organs are affected. We describe a case of pediatric IMT of the bladder with FN1–ALK (fibronectin 1–anaplastic lymphoma kinase) fusion. Radical resection might lead to urinary disturbance due to the large tumor size at diagnosis. However, the tumor was successfully treated with alectinib, a second-generation ALK inhibitor, followed by transurethral resection of the bladder tumor without any complications.     

Medial temporal lobe epilepsy associated with misplacement of a ventricular shunting catheter

We report the case of a 35-year-old woman who developed right medial temporal lobe epilepsy associated with misplacement of a ventricular shunting catheter in the apex of the right temporal lobe. At 8 years of age, the patient had undergone total removal of a cerebellar astrocytoma and placement of a Torkildsen's ventriculo-cisternal shunt for obstructive hydrocephalus. Although the postoperative course was uneventful, she developed medically intractable psychomotor seizures with secondary generalization at 24 years of age. CT revealed that the tip of the shunting catheter was misplaced in the apex of the right temporal lobe, through the posterior and inferior horn of the right lateral ventricle. Intraoperative electrocorticography revealed frequent paroxysmal activity in the hippocampus, so hippocampectomy as well as removal of the shunting catheter was performed. Postoperatively, the patient became seizure-free, and pathological examination revealed hippocampal sclerosis.     

Vasoconstricting effect of angiotensin II in human hand veins: influence of aging, diabetes mellitus and hypertension.

We examined human hand veins to determine whether venoconstricting response to angiotensin II (Ang II) and noradrenaline (NA) was influenced by aging or such diseases as diabetes mellitus (DM) and hypertension (HT). Twenty healthy male subjects (20-73 years), and 8 male patients with non-insulin-dependent DM and 8 male patients with essential HT were included in this study. A constant dose (50 ng/min) of Ang II or increasing dose (2-256 ng/min) of NA was infused into the dorsal hand vein and its diameter was measured using a linear variable differential transformer. The constant infusion of Ang II caused rapid desensitization or tachyphylaxis. The venoconstriction by Ang II in the 8 elderly subjects (58 to 73 years) was significantly (p<0.05) larger than that in the 8 young subjects (20 to 36 years) from 6 to 18 min after the start of the infusion (after 6 min: 63.6+/-11.6 (mean+/-SD)% vs. 39.9+/-20.8%, 12 min: 34.0+/-11.9% vs. 12.0+/-12.0%). However, the venoconstriction by Ang II in the patients with DM or HT was not significantly different from that in the 9 age-matched control subjects. No significant difference in venoconstrictor response to NA was observed between the young and elderly subjects, nor between the control subjects and the patients with DM or HT. These findings indicated that venoconstrictor response to Ang II might be greater in the elderly but might not be influenced by DM nor HT.     

Clinical, histological, and immunohistological studies of postoperative erythroderma

We report 7 cases of acute fatal illness characterized by fever, diffuse erythematous rash, and progressive leukopenia occurring 10 days after surgical operation. The outcome was uniformly fatal. The biopsy findings consisted of eosinophilic individual necrosis of epidermal cells, satellite cell necrosis, basal liquefaction degeneration, and scanty cell infiltration into the dermis. T lymphocytes were found in the epidermis but Langerhans cells disappeared. These findings are compatible with acute graft-vs-host disease following blood transfusion. Explanations based upon drug allergy, infection, toxic shock syndrome, or toxic epidermal necrolysis seem less reasonable.