A rare case of peripheral primitive neuroectodermal tumor in palate

Abstract

Primitive Neuroectodermal Tumor (PNET) is an aggressive round cell malignant tumor which belongs to Ewing's Sarcoma family. Peripheral PNET is rare in head and neck region and even rare in palate with handful of cases reported till date. A case of Peripheral PNET of palate in a 32-year-old female pa tient along with its clinico-pathologic, radiologic and immunohistologic features as well as its management is reported here. We emphasize the need of histopathology and immunohistochemistry (IHC) for early diagnosis of such aggressive tumors to improve the prognosis of patient by providing timely management.

Oral primitive neuroectodermal tumor associated with mandibular lymphadenopathy in three-year-old female Indonesian children: A rare case report

“…There hasn't been a documented case report among the Indonesian race and ethnicity developing PNETs or pPNETs. This case is considered to be within the youngest group of pPNETs onset, previously documented from India recorded the age of 3 months to 7 years old individuals developing pPNETs.12 Also, the progression of tumors originating from the oral cavity itself is not a common finding in pPNET cases documented [4,9] . In this particular case, a tissue sample was obtained from the second surgery following the debridement procedure.…”

“…PNETs usually tested positive with CD99, synaptophysin, Chromogranin A, and cytokeratin. 4 The integration of these diagnostic modalities enables accurate identification and classification of PNETs, leading to appropriate treatment planning and management strategies.…”

“…A primitive neuroectodermal tumor (PNET) is a rare and aggressive malignancy that arises from primitive neural crest cells in the central nervous system (CNS) [1] . However, the identification of these tumors in the soft tissues of the trunk and axial skeleton demonstrated the existence of variants of peripheral primitive neuroectodermal neoplasms (pPNETs) that were probably derived from undifferentiated mesenchyme [2][3][4] . pPNET is considered a well-differentiated version of the small cell tumor family, while Ewing's sarcoma and Askin's thoracic tumor are considered poorly differentiated variants of small round cell tumors (tumors in the Ewing's sarcoma family) [4] .…”

“…However, the identification of these tumors in the soft tissues of the trunk and axial skeleton demonstrated the existence of variants of peripheral primitive neuroectodermal neoplasms (pPNETs) that were probably derived from undifferentiated mesenchyme [2][3][4] . pPNET is considered a well-differentiated version of the small cell tumor family, while Ewing's sarcoma and Askin's thoracic tumor are considered poorly differentiated variants of small round cell tumors (tumors in the Ewing's sarcoma family) [4] . Identifying and accurately diagnosing PNETs can be challenging due to their rarity and overlapping features with other neoplastic conditions.…”