Disseminated Cerebrospinal Embryonal Tumor in the Adult.
Introduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient.Illustrative Case. A 49-year-old male presenting with low back pain, dysuria, and hypoesthesia of the lower extremities referred to our emergency department. Brain and whole spine contrast-enhanced MRI documented a diffusively disseminated heterogeneous neoplasm with intradural extra- and intramedullary involvement of the cervicothoracic tract and cauda equina. A primary biopsy of the lumbosacral localization was performed through L5 bilateral laminectomy. Histologic diagnosis was Embryonal Tumor Not Otherwise Specified. The patient underwent chemotherapy with postoperative adjuvant alternating Vincristine-Doxorubicin-Ifosfamide (VAI) and Ifosfamide-Etoposide (IE).Discussion. Spinal ETs are exceedingly rare especially when presenting in the adult patient. Neurosurgical and oncologic management is still unclear. When feasible, surgical removal should always be performed to obtain a histologic diagnosis. Postoperative adjuvant therapy might entail both chemo- and radiotherapy; however a consensus on this matter is still lacking.
Department of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, Italy.
Alessandro
0000-0003-2776-5399
A
Caporlingua
Department of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, Italy.
Daniele
D
Armocida
Department of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, Italy.
Federico
F
Caporlingua
Department of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, Italy.
Gennaro
G
Lapadula
Department of Radiological, Oncological and Anatomo-Pathological Sciences, Sapienza University of Rome, Rome, Italy.
Grazia Maria
GM
Elefante
Department of Radiological, Oncological and Anatomo-Pathological Sciences, Sapienza University of Rome, Rome, Italy.
Manila
M
Antonelli
Department of Science and Medical Surgical Biotechnology, Sapienza University of Rome, Rome, Italy.
Maurizio
M
Salvati