ABSTRACT
Hemangioblastoma (HB) is a Central Nervous System (CNS) tumor with a generally favorable behavior and prognosis, classified as WHO grade 1. Sporadic HB is not related to any inherited disease, and it usually appears in a single location. Sporadic or VHL-related HBs show variable patterns of growth velocity. Cases of growing HB can cause mild symptoms such as headache, but some cases develop serious complications such as accumulation of cerebrospinal fluid in the brain with secondary neurological damage sometimes being irreversible when early treatment is not started. Our case showed some clinical characteristics more frequently observed in VHL-related HB rather than sporadic HB, and the presence of alterations in MDM2 and EGFR that could be related to the oncogenesis of these tumors. Even when the treatment of choice for HB is surgery, the presence of these genetic alterations could open a new window for research aimed at assessing the possibility of new therapies with TKIs-EGFR and anti-MDM2 inhibitors in those HB cases with multifocal recurrences or cases with an adverse clinical behavior.
ABSTRACT
Paragangliomas are rare tumors and most are benign: less than 10% show criteria for malignancy. In the head and neck, the main locations are carotid, jugulo-tympanic and vagal. Surgery continues to be the mainstay of treatment but both external beam radiotherapy (EBRT) and stereotactic radiosurgery (SRS) have been shown to be effective therapeutic alternatives. EBRT requires large volumes to cover the lesion, while radiosurgery allows administration of a high dose in a smaller volume, with high precision, which should reduce the number of long-term complications. The results in terms of local control with both treatment modalities are good, as reflected in published series. The role of chemotherapy is restricted to malignant paragangliomas. The treatment used includes the combination of chemotherapy, which provides short-lasting responses, and the role of new molecular targets is currently being investigated. The present article reviews the indications and results of the non-surgical treatment of paragangliomas.
Subject(s)
Head and Neck Neoplasms/drug therapy , Head and Neck Neoplasms/radiotherapy , Paraganglioma/drug therapy , Paraganglioma/radiotherapy , HumansABSTRACT
Los paragangliomas (Pg) son tumores poco frecuentes, la mayoría benignos; menos del 10% presenta criterios de malignidad. En la cabeza y el cuello las principales localizaciones son carotídeos, yúgulo-timpánicos y vagales. La cirugía sigue siendo la principal modalidad de tratamiento, pero tanto la radioterapia externa (EBRT) como la radiocirugía(SRS) han demostrado su eficacia como alternativa terapéutica. La EBRT precisa volúmenes amplios para cubrir la lesión, en tanto que la radiocirugía permite la administración de una dosis elevada en un volumen más restringido, con alta precisión, lo que en principio produciría menos tasa de complicaciones a largo plazo. Los resultados en cuanto a control local con ambas modalidades de tratamiento son elevados, como se refleja en las series publicadas. El papel de la quimioterapia está limitado a los Pg malignos. El tratamiento utilizado incluye la combinación de quimioterapia, que ofrece respuestas de corta duración, y en la actualidad se está investigando el papel de nuevas moléculas dianas. En este capítulo se revisarán las indicaciones y los resultados del tratamiento no quirúrgico de los Pg (AU)
Paragangliomas are rare tumors and most are benign: less than 10% show criteria for malignancy. In the head and neck, the main locations are carotid, jugulo-tympanic and vagal. Surgery continues to be the mainstay of treatment but both external beam radiotherapy (EBRT) and stereotactic radiosurgery (SRS) have been shown to be effective therapeutic alternatives. EBRT requires large volumes to cover the lesion, while radiosurgery allows administration of a high dose in a smaller volume, with high precision,which should reduce the number of long-term complications. The results in terms of local control with both treatment modalities are good, as reflected in published series. The role of chemotherapy is restricted to malignant paragangliomas. The treatment used includes the combination of chemotherapy, which provides short-lasting responses, and the role of new molecular targets is currently being investigated. The present article reviews the indications and results of the non-surgical treatment of paragangliomas (AU)
