Alessia Imperato1, Pietro Spennato2, Federica Mazio3, Esperanza Arcas1,4, Onur Ozgural1,5, Lucia Quaglietta6, Maria Elena Errico7, Giuseppe Cinalli1. 1. Division of Neurosurgery, Santobono-Pausilipon Children's Hospital, Via Mario Fiore 6, 80121, Naples, Italy. 2. Division of Neurosurgery, Santobono-Pausilipon Children's Hospital, Via Mario Fiore 6, 80121, Naples, Italy. pierospen@gmail.com. 3. Division of Pediatric Neuroradiology, Santobono-Pausilipon Children's Hospital, Via Mario Fiore 6, 80121, Naples, Italy. 4. Department of Neurosurgery, University Hospital Virgen de las Nieves, Granada, Spain. 5. Department of Neurosurgery, University of Ankara, Ankara, Turkey. 6. Division of Pediatric Oncology, Santobono-Pausilipon Children's Hospital, Via Mario Fiore 6, 80121, Naples, Italy. 7. Division of Pathology, Santobono-Pausilipon Children's Hospital, Via Mario Fiore 6, 80121, Naples, Italy.
Abstract
BACKGROUND: Desmoplastic infantile astrocytomas and gangliogliomas (DIA/DIG) usually present with a large size, large cystic component, large dural implant, encasement of big vessels, clinical presentation within 18 months of life, high incidence of seizures and overall good prognosis, even if tumour surgery can be very challenging at first procedure. METHODS: We retrospectively reviewed clinical and radiological data of patients diagnosed with desmoplastic infantile tumours who were surgically treated between 2008 and 2019. RESULTS: The series included 12 patients. The median age at surgery was 91 days. The average tumour volume was 212 cm3. Cystic components were predominant ranging from 0 to 295 cm3. Active hydrocephalus was pre-operatively evident in 5 cases. Eight patients (66.6%) received total or subtotal removal, three of them (25%) underwent partial removal, and one patient (8.3%) received a biopsy. One patient died within 24 h after surgery due to severe hypotension, as a consequence of significant intraoperative blood loss. Overall, seven (58.3%) patients were reoperated on the tumour after the first procedure: 4 patients were operated twice; 3 patients were operated 3 times. Two patients presented remote localizations and underwent chemotherapy. At last follow-up, 7 patients were tumour-free, 2 are alive with stable disease, and 2 are alive with progressive disease (leptomeningeal seeding). CONCLUSION: Desmoplastic infantile tumours are rare giant neonatal tumours. Total removal is the goal of treatment, but prognosis remains good even if total removal is not achieved. In case of tumour progression or epilepsy from residual tumour, reoperation is the first option, with chemotherapy reserved to unresectable or disseminated cases with mixed results, while, to date, radiotherapy still plays no role.
BACKGROUND:Desmoplastic infantile astrocytomas and gangliogliomas (DIA/DIG) usually present with a large size, large cystic component, large dural implant, encasement of big vessels, clinical presentation within 18 months of life, high incidence of seizures and overall good prognosis, even if tumour surgery can be very challenging at first procedure. METHODS: We retrospectively reviewed clinical and radiological data of patients diagnosed with desmoplastic infantile tumours who were surgically treated between 2008 and 2019. RESULTS: The series included 12 patients. The median age at surgery was 91 days. The average tumour volume was 212 cm3. Cystic components were predominant ranging from 0 to 295 cm3. Active hydrocephalus was pre-operatively evident in 5 cases. Eight patients (66.6%) received total or subtotal removal, three of them (25%) underwent partial removal, and one patient (8.3%) received a biopsy. One patientdied within 24 h after surgery due to severe hypotension, as a consequence of significant intraoperative blood loss. Overall, seven (58.3%) patients were reoperated on the tumour after the first procedure: 4 patients were operated twice; 3 patients were operated 3 times. Two patients presented remote localizations and underwent chemotherapy. At last follow-up, 7 patients were tumour-free, 2 are alive with stable disease, and 2 are alive with progressive disease (leptomeningeal seeding). CONCLUSION:Desmoplastic infantile tumours are rare giant neonatal tumours. Total removal is the goal of treatment, but prognosis remains good even if total removal is not achieved. In case of tumour progression or epilepsy from residual tumour, reoperation is the first option, with chemotherapy reserved to unresectable or disseminated cases with mixed results, while, to date, radiotherapy still plays no role.
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Authors: Melissa M Blessing; Patrick R Blackburn; Chandra Krishnan; Virginia L Harrod; Emily G Barr Fritcher; Christopher D Zysk; Rory A Jackson; Dragana Milosevic; Asha A Nair; Jaime I Davila; Jessica R Balcom; Robert B Jenkins; Kevin C Halling; Benjamin R Kipp; Amulya A Nageswara Rao; Nadia N Laack; David J Daniels; William R Macon; Cristiane M Ida Journal: J Neuropathol Exp Neurol Date: 2019-11-01 Impact factor: 3.685
Authors: Melissa M Blessing; Patrick R Blackburn; Jessica R Balcom; Chandra Krishnan; Virginia L Harrod; Michael T Zimmermann; Emily G Barr Fritcher; Christopher D Zysk; Rory A Jackson; Asha A Nair; Robert B Jenkins; Kevin C Halling; Benjamin R Kipp; Cristiane M Ida Journal: Acta Neuropathol Commun Date: 2018-11-05 Impact factor: 7.801