This report describes the clinical and biochemical features of 3 0 cases of ectopic ACTH-producing tumors diagnosed by the detection of ACTH .in the tumor tissues. Several uncommon tumors, such as tumors of the esophagus, stomach, and larynx, were included in this series. None of the patients with bronchogenic carcinoma showed signs of classical Cushing's syndrome, whereas 7 of the remaining 13 patients with other tumors were Cushingoid in appearance. Adrenocortical hyperfunction was present in 61% at the first examination and developed during the course of the disease in 18% more. In the remaining patients (21?6), adrenocortical function remained within normal limits. These results indicate that there exist ectopic ACTH-producing tumors without clinical and biochemical sequelae of excess hormone. In some of the tumor extracts studied, MSH and CRF-like activities and serotonin were detected. This suggests that multiple hormone production is not uncommon in ectopic ACTH-producing tumors.
bone marrow transplantation (BMT) is an appropriate treatment. ATRA administration leads to hyperleukocytosis and ATRA-syndrome, which is characterized by fever, respiratory distress, pulmonary infiltrates, pleuropericardial effusions, and edema [l]. Vitamin A toxicity is known to cause intracranial hypertension, characterized by increased intracranial pressure in the absence of any evidence of an intracranial space-occupying lesion, obstruction of the ventricular or subarachnoid pathways, infection, or hypertensive encephalopathy. Symptoms of pseudotumor cerebri consist of headache, diplopia, and other visual disturbances due to papilledema and abducens nerve dysfunction [2]. These symptoms are usually reversible upon cessation of drug administration.We describe a patient with pretransplant pseudotumor cerebri due to ATRA treatment who underwent uneventful BMT following total body irradiation (TBI) without neurological complications.A 17-year-old-girl with APL was induced into 1st CR by treatment with 5-day daunorubicin and it was followed by two courses of consolidation chemotherapy. Six months later she relapsed, oral ATRA treatment (45 mg/ mZ) was introduced, which caused hyperleukocytosis. The treatment was temporarily discontinued and she received two additional courses of Ara-C and daunorubicin. Post-chemotherapy, she had morphological remission but the cytogenetic examination revealed 3% abnormal chromosomal translocation t(15;17) in the BM. Oral ATRA therapy was reintroduced and she consequently relapsed with 20% blasts in the bone marrow. The patient was admitted for immediate allogeneic BMT from her HLA-identical brother. On admission, there were signs of mucosal dryness and the patient complained of headaches, blurred vision, and horizontal diplopia. On examination, the left optic disc was swollen with engorged veins and right abducens nerve palsy was observed. In the peripheral blood there was no sign of relapse.Cerebrospinal fluid examination disclosed an elevated pressure of 340 mmHg but no other abnormality was found. Daily, p.0. 10 mg Dexamethasone and 250 mg X 2 Diamox were introduced. CT-scan and MRI of the head did not show any anomaly. The pretransplant conditioning consisted of 1,200 cGy fractionated TBI, 600 cGy fractionated total lymphoid irradiation followed by i.v. VP-16 (1,500 mg/m'), i.v. cyclophosphamide (60 mgkg), and i.v. melphalan (60 mg/m2). She was transplanted with BM containing 5.1 X 10* nucleatedcellskg which was treated in vitro with CAMPATH-1G monoclonal antibody (kindly provided by Dr. G Hale and Dr. H Waldmann, Cambridge, UK).The post-transplant period was complicated by veno-occlusive liver disease, acute GVHD, ARDS due to severe staphylococcus, and enterococcus septicaemia. There were no CNS complications post-transplant, and the fundus and the CSF examinations were normal. Currently, the patient is in full remission 27 months after BMT, with no neurological complication.CNS complications associated with BMT are well known. Posttranplant, 11% of our patients suffer from ...
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