Peripheral primitive neuroectodermal tumor of the cervical spine.
BACKGROUND
Primitive neuroectodermal tumor (PNET) is a malignant neoplasm that generally arises from bone and soft tissues, with predilection for young adults. This neural crest origin tumors share biologic and histologic features with Ewing's sarcoma (ES).


METHODS
We present a case of a 22-year-old woman with history of severe progressive neck pain, without radiation, associated with paresthesia in the right arm, and palpable right posterior cervical mass. Neurological examination showed increased reflexes in all four limbs, bilateral Hoffman's sign, right Babinski's sign, and right hemi-hypoesthesia. Neuroimaging revealed a right posterior cervical lesion with heterogeneous contrast enhancement extending to the neural foramina of the atlas and axis. Patient underwent microsurgical removal of the lesion, and histopathological and immunohistochemical analysis confirmed the diagnosis of peripheral primitive PNET (pPNET). The patient had adjuvant treatment with radiotherapy and chemotherapy. After twelve months, neuroimaging showed no signs of tumor regrowth and the patient had no neurological deficits. However, three months later, the patient developed hydrocephalus and cerebrospinal fluid (CSF) was positive for neoplastic cells. No other treatment was administered and the patient died.


CONCLUSIONS
pPNET is a rare malignant tumor with poor prognosis, although promising results with multimodal treatment-surgery, radiotherapy, and chemotherapy. Diagnosis requires immunohistochemical analysis, with identification of neuronal differentiation markers.
Department of Neurosurgery, Galeão Air Force Hospital, Rio de Janeiro, Brazil.
Gustavo A P S
GA
Cabral
Cristian F
CF
Nunes
José O
JO
Melo
Rodrigo D
RD
Guimarães
Mariangela B
MB
Gonçalves
Ruy S
RS
Rodrigues
Jorge Luiz A
JL
Correa
Orlando M
OM
Teixeira
João
J
Klescoski
Mario A
MA
Lapenta
José A
JA
Landeiro