No abstract

Calcified cerebral necrosis was an unusual finding at the autopsy of a 13-year-old girl who died after prolonged therapy for ALL. The patient had shown symptoms of progressive cerebral damage subsequent to a second cycle of prophylactic high-dose cytostatic therapy combined with cranial irradiation. Pathoanatomic examination revealed extensive florid recurrency of meningosis and leukemic encephalosis with scalloped calcified necroses measuring up to 5 cm, in the medullar layer of brain and cerebellum. Located predominantly near the ventricular area, encapsulated necroses showed many fibrous vessels with thickened walls and stenosed or obstructed lumina. The cerebral cortex remained largely unaffected by tissue destruction. Besides methotrexate toxicity and the enhancing effect of irradiation the vascular involvement was interpreted as a particularly important factor. Formal pathogenesis is attributed to combined chemo- and radiotherapy in parallel to leukemic infiltration of vascular walls and partial obstruction of lumina by tumor emboli. Wall damage, severe fibrosis, and consecutive nutritional defects result in the destruction of cerebral tissue. The preferential occurrence of necroses in cortex-adjacent medullar layers is explained by the relatively poor blood supply of this border zone between meningeal and intracerebral tissue, no safe conclusion can be drawn pathoanatomically with regard to the actually fatal factor, whether it is the leukemic infiltration of vascular walls, the effect of cytostatic agents, or that of irradiation. The proposed multifactorial pathogenesis of cerebral calcification may be supported by computed tomography (CT) intra vitam.

A patient is reported who developed the first symptoms of spinal motor neuron affection 20 years prior to his death at the age of 79. In the course of the disease dementia and spasticity of the legs occurred. The patient died of metastasizing carcinoma of the colon. The autopsy revealed amyloid angiopathy of the brain and cervical spinal cord, corresponding to the clinical symptomatology. So far, 11 cases of amyloid angiopathy have been reported in which dementia was preceded by dysarthric speech, ataxia and/or spasticity of the legs. We assume that these cases represent a distinct nosological entity, different from a variant course of Alzheimer's disease. The atypical symptomatology may cause problems in the diagnosis of amyloid angiopathy of the CNS.

Two cases of fatal pneumopathy during cytostatic therapy for acute lymphatic leukemia of childhood, are reported with pathoanatomical lung findings and general clinical features. Histology revealed massed atypical epithelial proliferation in the bronchiolar terminal pathways (tumourlets) with multinucleated polymorphic giant cells beside pulmonary fibrosis. As causative factors for pulmonary chages hypersensitivity reactions, direct toxicity, or pharmacologic effects are discussed. Formal pathogenesis is explained by an impairment of endothelial cells in alveolar capillaries followed by permeability disorders and interstitial edema with disturbed perfusion. Disseminated intravasal microthrombi are frequent. Restitution to integrity appears possible only under favorable conditions. If the exsudative turns into the proliferative phase, intraalveolar and interstitial pulmonary fibrosis may develop with atypical epithelial proliferations. The prognosis of cytostatics-induced pneumopathies depends essentially on the time when it is diagnosied.