Clinical presentation and long-term outcome of primary spinal peripheral primitive neuroectodermal tumors

Abstract

Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare entities that predominantly occur in children and young adults. Few studies have reported more than three cases. There are no current optimum treatment strategies due to the paucity of data. Here, we present 13 patients (nine females and four males) with primary intraspinal pPNETs who were surgically treated from April 2008 to February 2014. Histopathologic findings revealed the expression of CD99 in all cases. Limb weakness was the most common initial symptom (11/13, 85 %). The tumors were located mainly at the cervical level (6/13, 46 %) and in the epidural space (10/13, 77 %). The radiological diagnosis was neurinoma or meningioma in most cases (10/13, 77 %). Gross total resection was achieved in 77 % (10/13) of patients. During a mean follow-up of 25.5 months, local relapse occurred in 8 (61.5 %) patients and distant metastases occurred in 8 (61.5 %) patients. The overall 1-year survival rate was 77 % (10/13), and the overall 2-year survival rate was 54 % (7/13). The 2-year survival rate was 57.1 % in patients with adjuvant chemotherapy and 50 % in those without chemotherapy. Gross total resection and adjuvant radiotherapy with or without chemotherapy demonstrated a longer survival period (1-year survival rate: 100 %; 2-year survival rate: 86 %). Our data showed that primary spinal pPNETs are extremely rare and aggressive tumors with a poor prognosis. Radical resection is advocated. Gross total resection combined with adjuvant radiation may help to significantly improve patient survival period. Chemotherapy may also help to slightly prolong patient life.

Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases

“…The study concluded gross total resection and adjuvant RT with or without chemotherapy demonstrated a longer survival period (2-year survival rate 86%), and chemotherapy demonstrated a trend for survival benefit in patient with PNET. [ 11 ] In a case report and literature review presented by Kiatsoontorn et al, 13 cases were reviewed, where 1 patient received operation followed by RT and 1 patient received operation alone, whereas others received operation with adjuvant chemoradiation. Seven of the 12 previous patients received local adjuvant RT, and 4 patients received craniospinal irradiation.…”

Spinal peripheral primitive neuroectodermal tumors: a radiological study of ten cases

“…The first-line treatment for spPNET is a multimodal treatment strategy that consists of surgery, neo-adjuvant or adjuvant chemotherapy or radiotherapy. Although GTR of the tumor is suggested as a cornerstone of therapy (19), most patients spPNET using these non-specific symptoms, diagnosis is largely dependent on imaging results.…”

Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature

“…As a result of these special clinical symptoms, the patient received timely intervention and the tumor was surgically removed. pPNET is a rare tumor, which can be found in a wide variety of tissues including the pancreas[ 8 ], neck[ 9 ], spine[ 10 ], prostate[ 11 ] and parotid[ 12 ]. It is categorized in the Ewing family of tumors and is composed of malignant small round cells[ 13 - 16 ].…”