Abstract
Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. To date, there are no standard guidelines for management of peripheral PNETs due to the paucity of cases arising in various body sites. Therapeutic approach is derived from Ewing sarcoma family, which currently remains multimodal. Our study retrospectively analyzed 86 PNET patients from February 1, 1998 to February 1, 2018 at Peking Union Medical College Hospital with an additional 75 patients from review of literature. The clinicopathologic and treatment plans associated with survival was investigated. Surgery, chemotherapy, female sex, small tumor size, no lymph node metastasis, R0 surgical resection, (vincristine + doxorubicin + cyclophosphamide)/(isophosphamide + etoposide) regimen, and more than 10 cycles of chemotherapy were associated with improved overall survival in univariate analysis. Surgery, more than 10 cycles of chemotherapy, and small tumor size were independent prognostic factors for higher overall survival. Our data indicates that multimodal therapy is the mainstay therapeutic approach for peripheral PNET.
Highlights
Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies
Based on the tissue of origin of the tumors, they are divided into peripheral PNET and central PNET. pPNET mainly arises in the skeletal system and soft tissues, while the occurrence of cPNET is primarily intracranial and intraspinal. pPNET may be present in visceral body sites such as heart, lung, genital organs, kidney, pancreas and palate[6,7]
In terms of the location of primary tumor occurrence, 40 cases occurred in trunk, extremities and bones, 46 in abdominal and pelvic region
Summary
Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. Primitive neuroectodermal tumor (PNET) is a rare and highly malignant small round cell tumor and its concept was first introduced by Hart et al.in 19731. Annual incidence of this tumor is approximately 0.2–0.4 per 100,000 and it most commonly occurs in children and young adults with a slight male predominance[2,3,4]. To elucidate the prognosis of pPNET and improve the therapeutic approaches, the current study collectively investigated 161 PNET patients both from our own institution and from literature review, and analyzed the survival impacts of the clinical and treatment features of these patients
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