Primitive neuroectodermal tumor of the posterior mandible: A case report

Abstract

ÖZETPrimitif nöroektodermal tümör, nöroektodermal kaynaklı değişik hücre farklılaşması ile birlikte olan bir neoplazm kategorisini tanımlamak için kullanılan bir terimdir. Bu tü-mörler vücutta birçok yerden kaynak alır. Göğüs duvarı, larinks, karın ve pelvis en sık tutulan bölgeler olup, nadiren baş ve boyun bölgesinde oluşur. Olguların yalnızca %3'ü kafatası ve çeneden köken alır. Bu raporda genç bir erişkinde mandibula sol arka kısmında gelişmiş nadir bir nöroektodermal tümör olgusu sunulmaktadır.Anahtar kelimeler: Nöroektodermal tümörler, primitif, periferik, ağız içi, mandibula ABSTRACTPrimitive neuroectodermal tumor is a term used to describe a category of neoplasms of neuroectodermal origin with variable cell differentiation. These tumors arise in many places throughout the body. The chest wall, larynx, abdomen, and pelvis being the most common primary sites; in rare cases, they occur in the head and neck area. Only 3% of cases have been reported within the skull and jaws. In this report, a rare case of primitive neuroectodermal tumor in the left side posterior mandible in a young adult is described.

Peripheral facial palsy, the only presentation of a primitive neuroectodermal tumor of the skull base

“…Ultra-structural transmission electron micrographic evidence of neural differentiation and neurosecretory granules is also helpful for the diagnosis of PNET. The CD99 and the vimentin positive staining in tumor cell is important in the differentiation of PNET from other small round cell tumors [6] .…”

A rare case of peripheral primitive neuroectodermal tumor in palate

“…Tumors exhibiting neuroectodermal differentiation occur throughout the body, and the diverse tissues of the head and neck give rise to a wide assortment of these neoplasms [7]. PNET is a term used to describe a category of neoplasm of neuroectodermal origin with variable cell differentia tion [1]. The PNET are grouped as central and peripheral tumors.…”

“…Metastasis has been observed at the time of diagnosis in up to 31% of patients, reflecting the aggressiveness of these tumors. Metastasis to oral cavity from the primary lesion located at other sites is uncommon [1]. The prognosis of PNET is poor; with two-year survival rate in only 65% cases, after diagnosis, regardless of treatment modalities applied [11].…”

“…Group (I) tumors, such as the pituitary adenomas and carcinoid tumors, represent tumors that show predominantly epithelial differenti ation. Group (II) tumors, which incorporate malignant melanoma, olfactory neuroblastoma, Ewing's sar coma (EWS) and primitive neuroectodermal tumor that are predominantly neural and non-epithelial in origin [1].…”

“…Peripheral PNETs are commonly seen in chest wall, trunks, abdomen and extremities of children and are relatively uncommon in head and neck region [5]. These tumors are aggressive and have a propen sity for local recurrence and metastasis especially in bone marrow, brain, lungs and lymph nodes, but rarely to jaws and skull [1]. Primary peripheral PNET of maxilla is an extremely rare occurrence.…”