ABSTRACT
BACKGROUND AND PURPOSE: Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) is a new, molecularly defined glioneuronal CNS tumor type. The objective of the present study was to describe MR imaging and clinical characteristics of patients with DGONC. MATERIALS AND METHODS: Preoperative MR images of 9 patients with DGONC (median age at diagnosis, 9.9 years; range, 4.2-21.8 years) were reviewed. RESULTS: All tumors were located superficially in the frontal/temporal lobes and sharply delineated, displaying little mass effect. Near the circle of Willis, the tumors encompassed the arteries. All except one demonstrated characteristics of low-to-intermediate aggressiveness with high-to-intermediate T2WI and ADC signals and bone remodeling. Most tumors (n = 7) showed a homogeneous ground-glass aspect on T2-weighted and FLAIR images. On the basis of the original histopathologic diagnosis, 6 patients received postsurgical chemo-/radiotherapy, 2 were irradiated after surgery, and 1 patient underwent tumor resection only. At a median follow-up of 61 months (range, 10-154 months), 6 patients were alive in a first complete remission and 2 with stable disease 10 and 21 months after diagnosis. The only patient with progressive disease was lost to follow-up. Five-year overall and event-free survival was 100% and 86±13%, respectively. CONCLUSIONS: This case series presents radiomorphologic characteristics highly predictive of DGONC that contrast with the typical aspects of the original histopathologic diagnoses. This presentation underlines the definition of DGONC as a separate entity, from a clinical perspective. Complete resection may be favorable for long-term disease control in patients with DGONC. The efficacy of nonsurgical treatment modalities should be evaluated in larger series.
Subject(s)
Brain Neoplasms , Central Nervous System Neoplasms , Glioma , Neoplasms, Neuroepithelial , Oligodendroglioma , Humans , Child , Oligodendroglioma/diagnostic imaging , Oligodendroglioma/surgery , Glioma/pathology , Central Nervous System Neoplasms/pathology , Magnetic Resonance Imaging , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/therapySubject(s)
Anemia, Refractory, with Excess of Blasts/therapy , Hematopoietic Stem Cell Transplantation , Hemophilia A/therapy , Myelodysplastic Syndromes/therapy , Anemia, Refractory, with Excess of Blasts/diagnosis , Anemia, Refractory, with Excess of Blasts/genetics , Child, Preschool , Exons/genetics , Factor VIII/genetics , Follow-Up Studies , Hemophilia A/blood , Hemophilia A/diagnosis , Hemophilia A/genetics , Humans , Male , Mutation, Missense/genetics , Myelodysplastic Syndromes/diagnosis , Myelodysplastic Syndromes/genetics , Partial Thromboplastin Time , Platelet Count , Platelet Transfusion , Treatment OutcomeSubject(s)
Head and Neck Neoplasms/diagnosis , Hodgkin Disease/diagnosis , Mediastinal Neoplasms/diagnosis , Nephrotic Syndrome/diagnosis , Adolescent , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Doxorubicin/administration & dosage , Etoposide/administration & dosage , Female , Fluorodeoxyglucose F18 , Head and Neck Neoplasms/drug therapy , Head and Neck Neoplasms/pathology , Hodgkin Disease/drug therapy , Hodgkin Disease/pathology , Humans , Magnetic Resonance Imaging , Mediastinal Neoplasms/drug therapy , Mediastinal Neoplasms/pathology , Multimodal Imaging , Neoplasm Staging , Nephrotic Syndrome/drug therapy , Nephrotic Syndrome/pathology , Positron-Emission Tomography , Prednisone/administration & dosage , Tomography, X-Ray Computed , Vincristine/administration & dosageABSTRACT
24 families of a child with cancer were questioned for a 3rd time, 8.4 years after initial diagnosis and 6.5 years after family-oriented rehabilitation, before and after which they had been interviewed twice. Subject to this follow-up was the current family situation and the stability of the remarkably positive effect the rehabilitation had shown in the 1st course of the study. Also we hoped to be able to identify problem-families. Generally the family situation seems to have returned to normal, the standardised tests applied showed no significant difference to the general public. Nonetheless some families could be identified as having severe coping difficulties with 2 to 4 members displaying psychosocial problems of a very high degree. Both groups of families profited in the same degree from the rehabilitation; this effect shows great stability for the families coping good, whereas those with coping difficulties worsen considerably, almost always beyond the degree displayed before the rehabilitation. To identify these families quickly and offer them support it seems necessary to establish an out-patient psychosocial follow-up.
