Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit.
Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.
Kim
Usha R
UR
Orbit, Oculoplasty and Oncology Clinic, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology Madurai, Tamil Nadu, India. usha@aravind.org
Arora
Vipul
V
Devanand
J
J
Khazei
Hadi M
HM
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001706:Biopsy; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy*; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy*; D018714:Radiotherapy, Adjuvant; D014057:Tomography, X-Ray Computed
D000970:Antineoplastic Agents
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