Novel case of hybrid perineurioma-neurofibroma of the orbit

Orbital hybrid schwannoma/perineurioma: A case presentation

“…1 The remaining one has components of perineurioma and neurofibroma. 2 Herein, we would like to share the first case of orbital tumor showing combined histomorphology of schwannoma and perineurioma.…”

Hybrid Schwannoma-Perineurioma of the Orbit

“…This tumor is composed of a plexiform neurofibroma with areas of perineurial differentiation. 3 HPNSTs are extremely rare in the orbit, with 8 cases reported in the literature: 6 cases describe neurofibroma-schwannoma, 1 case describes a neurofibroma-perineurioma, and 1 case describes a schwannoma-perineurioma. 1,3,7,10,[14][15][16][17] The paucity of cases of HPNSTs in this region may be an underestimate, reflecting a lack of awareness of this relatively recently recognized entity.…”

“…3 HPNSTs are extremely rare in the orbit, with 8 cases reported in the literature: 6 cases describe neurofibroma-schwannoma, 1 case describes a neurofibroma-perineurioma, and 1 case describes a schwannoma-perineurioma. 1,3,7,10,[14][15][16][17] The paucity of cases of HPNSTs in this region may be an underestimate, reflecting a lack of awareness of this relatively recently recognized entity. Four previously reported patients were male and 4 were female, with an average age of 52 years (median 52.5, range 31-78).…”

“…2 Several subtypes of HPNST have been identified, including neurofibroma-schwannoma, schwannoma-perineurioma, and neurofibroma-perineurioma. [2][3][4] Orbital involvement of HPNST is extremely rare with only 7 cases of all types reported in the literature. Due to its rarity, its clinical characteristics and prognosis remain largely unknown.…”

Localized Bilateral Superior and Inferior Orbital Neurofibroma in the Absence of Neurofibromatosis

“…Ultrastructural studies showed that this tumor was composed of a mixture of three cell types: Schwann cells, perineural cells, and fibroblasts [ 7 , 16 ]. Immunohistochemistry showed immune reactivity in EMA and S100 protein (Figures 4(a) – 4(c) ) in perineurioma and neurofibroma, respectively [ 17 , 18 ]. Immunohistochemistry in our case was compatible with neurofibroma [ 15 ] ( Table 1 ).…”