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Primary peripheral primitive neuroectodermal tumor of the mandible.
Arvind Krishnamurthy1, Vijayalakshmi Ramshankar, Urmila Majhi
1Department of Surgical Oncology, Cancer Institute (Women's India Association), Adyar, Chennai, Tamil Nadu, India.
Annals of Maxillofacial Surgery
|November 9, 2013
Summary
Peripheral primitive neuroectodermal tumors (pPNET) are rare, especially in the mandible. This study discusses a challenging case, highlighting diagnostic and management complexities of this rare small round cell tumor.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Primitive neuroectodermal tumors (PNET) encompass diverse lesions with varying differentiation potential in the central and peripheral nervous systems.
- Peripheral PNET (pPNET) can occur in various body sites, but mandibular involvement is exceptionally rare, with only seven prior reports in scientific literature.
Observation:
- Accurate diagnosis of pPNET is challenging due to insidious symptoms, varied locations, and rarity.
- PNETs possess distinct immunophenotypical and genetic markers differentiating them from other small round cell tumors.
Findings:
- This case report details the clinical course, histopathology, and immunohistochemistry of a rare mandibular pPNET.
- The study emphasizes the need for a multidisciplinary approach in managing patients with PNET.
Implications:
- Early and accurate diagnosis of rare pPNETs is crucial for effective management.
- Further research is needed to establish consensus on optimal therapeutic strategies for PNETs, particularly rare presentations like mandibular involvement.
