IMG-08. UNUSUAL IMAGING FINDINGS IN TWO CASES OF PAEDIATRIC LOW GRADE GLIOMA

Low grade gliomas (LGG), including pilocytic astrocytoma (PCA), are the commonest paediatric brain tumours and their behaviour is well understood, typically following a benign course. BRAF fusion is common, particularly in PCA of the cerebellum and optic pathway. Here we present two patients whose LGG behaved in an unusual fashion. The first patient who was treated 6 years previously on LGG2 with vincristine and carboplatin for a tectal plate lesion was identified on routine imaging to have local tumour progression and underwent completion staging. This showed a new enhancing soft tissue abnormality within the spinal cord at the level of L2. Due to radiological dubiety both lesions were biopsied for histological and molecular analysis, confirming LGG of the tectal plate and finding the spinal lesion to be a myxopapillary ependymoma. The second patient presented with acute hydrocephalus following a 2 year history of neurocognitive impairments. He was found to have a large, complex tumour centred in and expanding the bodies of both lateral ventricles with significant mass effect. Radiologically this was most in keeping with a central neurocytoma but histological analysis confirmed it to be a PCA with KIAA1549-BRAF fusion. The first case demonstrates the utility of molecular analysis in confirming two distinct tumour types in one patient, in a situation where metastasis would not be expected and would significantly alter treatment and prognosis. The second is an example of how imaging can be misleading in a KIAA1549-BRAF fused PCA presenting as an intraventricular mass.

Evaluation of Intra-abdominal and Retroperitoneal Soft Tissue Masses

Proper workup of intra-abdominal and retroperitoneal sarcomas cannot be overstated in the management of these rare tumors. In particular, evaluation should begin with a thorough history and physical examination, including careful consideration of genetic syndromes. Next, computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast should be performed to determine whether a tumor is completely resectable as complete resection is the mainstay of sarcoma management. Completion staging studies with CT of the chest should also be performed. Following imaging, a core-needle biopsy, usually an interventional radiology–guided biopsy, is performed to obtain a tissue diagnosis. In certain large, intermediate- to high-grade tumors involving other vital organs, neoadjuvant chemotherapy or neoadjuvant radiation is considered to try to decrease the tumor in the hope of improving the chance of obtaining an R0 resection and to limit the extent of surgery. Overall, it is important to remember that prior to the initiation of any therapy, all patients should be evaluated and managed by a multidisciplinary team with expertise and experience in sarcoma. This review contains 3 figures, 3 tables and 39 references Key words: biopsy, genetic testing, intra-abdominal and retroperitoneal sarcoma, neoadjuvant chemotherapy, neoadjuvant radiotherapy

Evaluation of Intra-abdominal and Retroperitoneal Soft Tissue Masses

Proper workup of intra-abdominal and retroperitoneal sarcomas cannot be overstated in the management of these rare tumors. In particular, evaluation should begin with a thorough history and physical examination, including careful consideration of genetic syndromes. Next, computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast should be performed to determine whether a tumor is completely resectable as complete resection is the mainstay of sarcoma management. Completion staging studies with CT of the chest should also be performed. Following imaging, a core-needle biopsy, usually an interventional radiology–guided biopsy, is performed to obtain a tissue diagnosis. In certain large, intermediate- to high-grade tumors involving other vital organs, neoadjuvant chemotherapy or neoadjuvant radiation is considered to try to decrease the tumor in the hope of improving the chance of obtaining an R0 resection and to limit the extent of surgery. Overall, it is important to remember that prior to the initiation of any therapy, all patients should be evaluated and managed by a multidisciplinary team with expertise and experience in sarcoma. This review contains 3 figures, 3 tables and 39 references Key words: biopsy, genetic testing, intra-abdominal and retroperitoneal sarcoma, neoadjuvant chemotherapy, neoadjuvant radiotherapy