Primitive Neuroectodermal Tumor of the Orbit in Adults: A Case Series

Abstract

To the authors' knowledge, this is the largest series of orbital PNET in adults. This tumor has an age demographic wider than previously believed and should be considered in the differential diagnosis of a hypercellular small round cell orbital tumor in both children and adults. Current treatment regimens are not standardized but typically use a similar approach to the treatment of Ewing sarcoma. Orbital PNET appears to have less propensity for metastasis compared with PNET in other locations. However, long-term aggressiveness remains to be proven.

Primary orbital Ewing sarcoma family of tumors: a study of 12 cases

“…35 The poor prognostic factors for ESFTs are metastatic disease at presentation, large tumor 45 cm, older age, male gender, central tumor location including skull, clavicle, ribs, pelvis, vertebrae, and the upper extremities. 35,[36][37][38] Orbital ESFT is considered to be less aggressive than ESFT at other locations in the body, [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]39 as the survival has been more than 6 months in majority of the reported cases. Although extraorbital extension is frequently reported with primary orbital ESFTs, systemic metastases at presentation or during follow-up duration is rare as per the available literature.…”

“…3 Primary orbital ESFT is extremely rare with isolated case reports and small case series reported in literature. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Herein we discuss the demographic profile, clinical features, radiological features, treatment modalities, and outcomes of primary orbital ESFTs.…”

Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma

“…3,8–11 Primary extraskeletal or extraosseous (soft tissue) orbital ES is rare with only 3 cases reported in the literature. 12–14 Recurrent ES carries a poor prognosis 15 with no past reports of successful treatment of recurrent primary ES of the orbit, which often progresses rapidly and has a propensity to metastasize.…”

Congenital orbital primitive neuroectodermal tumour

“…These include combinations of surgical removal/debulking and chemotherapy with or without radiotherapy. 2 One case had the tumour excised and chemoradiotherapy, but the tumour rapidly re-grew at the resection margin, and she passed away months later. The other case only had chemotherapy but died during the fourth cycle.…”

“…It appears that congenital orbital PNET appears more aggressive with rapid growth and fatality compared with other age groups. 2 The diagnosis of pPNET can be made based on histology, immunohistochemical and cytogenetic testing. Primary orbital PNET is rare, and even more rarely presents at birth.…”