Peripheral primitive neuroectodermal tumor of head-neck region: our experience

Abstract

To present four rare cases of peripheral primitive neuroectodermal tumors of different sites of head and neck region. Four cases of different age (range 8-40 years) and sex (three female, one male) with rare primitive neuroectodermal tumor of sinonasal region and neck are presented. Treatment options, biological behavior and prognostic outcome are discussed herewith. Two patients succumbed to the disease within four to six months of treatment; other two patients are still under follow-up depicting the aggressive nature of the tumor. Primitive neuroectodermal tumor belongs to the class of malignant round cell tumor. Immunohistochemistry plays a pivotal role in differentiating this tumor entity. Chemoradiation was tried, but local and systemic spread occurs early and holds poor prognosis. This case series is an attempt to describe the aggressive behavior of this rare tumor with high mortality.

Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89�cases and literature review (Review)

“…The majority of associated studies are case reports and clear diagnostic criteria have only recently been established (9). The estimated incidence of PNET is 2.9 per million per year (10). Due to its low incidence, there are presently no evidence-based guidelines for treatment.…”

Imaging Features of Primitive Neuroectodermal Tumor in the Maxilla: A Case Report and Literature Review

“…Conversely, spiral CT seems to be superior to FDG-PET in the detection of pulmonary metastases. 2,25 The imaging features of these tumors are not specific for differentiation of PNETs from other types of bone and soft tissue tumors. In terms of their radiographic appearance, they resemble large, ill-defined, non-calcified soft tissue masses with cystic or necrotic areas and aggressive infiltration into adjacent tissues and bones.…”

“…1 Because this tumor presents aggressive biological behavior and tends toward loco-regional and systemic metastases, it is a tumor with an extremely poor survival rate. 2 The incidence of head and neck PNET Imaging Features of Primitive Neuroectodermal Tumor in the Maxilla: A Case Report and Literature Review A AB BS S T TR RA AC CT T Primitive neuroectodermal tumor (PNET) is a term used to describe a group of highly malignant neoplasms composed of undifferentiated round cell tumors of neuroectodermal origin with variable cell differentiation. PNET generally occurs in the central nervous system (CNS), but peripheral PNET occurring outside the CNS has also been reported.…”

Primary Primitive Neuroectodermal Tumor of the Parotid: An Unsuspected Diagnosis

“…But, this surgery can be difficult in the head and neck region in cases of involvement of vital structures. Radiation therapy and chemotherapy can be considered in these cases [8]. The most effective chemotherapeutic regimen for PNETs are Vincristine, Cyclophosphamide, Doxorubicin ,Iphosphamide and Etoposide.…”