Hybrid Neurofibroma/Schwannoma of the Supraorbital Nerve: Clinicopathologic Correlation of a Rare Tumor

Abstract

Benign peripheral nerve sheath tumors such as schwannoma and neurofibroma have long been considered distinct entities. Recently, hybrid tumors demonstrating combined morphological features of neurofibroma and schwannoma have been described, primarily in dermal locations. Only 1 case of hybrid peripheral nerve sheath tumor of the orbit has been reported in the literature. Hybrid morphology is important to recognize because of its association with the neurofibromatoses, including schwannomatosis; however, the paucity of literature on orbital hybrid peripheral nerve sheath tumor poses a diagnostic challenge. This article describes a case of hybrid neruofibroma/schwannoma of the orbit arising from the supraorbital nerve with clinicopathologic correlation.

Novel case of hybrid perineurioma-neurofibroma of the orbit

“…Malignant transformation and recurrence were reported in cases of extra-orbital neurofibroma-perineurioma hybrid. 1,2 Our index patient remained symptom-free without recurrence at 6 months. Long-term follow-up and study into pathogenesis of HPNST are required for this rare but important tumour.…”

“…5,6 Literature review identified only 2 cases of orbital HPNST, both of neurofibroma-schwannoma subtype. 2,3 We report the third case of orbital HPNST in the distribution of supratrochlear nerve that extended toward the orbital apex, evidenced by loss of sensory innervation after excision. It is also the first reported neurofibroma-perineurioma subtype to occur within the orbit.…”

“…1 Orbital involvement of HPNST is rare and has only been documented twice in the literature. 2,3 Clinical manifestation and prognosis remained largely unknown due to its scarcity. We report the third case of HPNST to occur within the orbit, which is also distinctive in its form due to the presence of perineurioma and neurofibroma components, the first subtype reported to occur within the orbit.…”

“…1 Patients often have accompanying musculoskeletal and neurological manifestations, suggested by some authors to resemble the symptoms of Lyme disease. 2,3 Although several authors have proposed various diagnostic criteria, there is currently no widely accepted consensus. Middelveen et al…”

“…proposed that Morgellons disease represents a somatic Lyme diseaseÀlike illness associated with spontaneously appearing skin lesions, the key diagnostic criterion being multicoloured filaments protruding from or embedded in the skin, often requiring 50 £ magnification to visualize. 2 The Centers for Disease Control and Prevention (CDC) suggested that "Morgellons" is a lay term used to describe an unexplained constellation of symptoms, with manifestations primarily involving the skin. 1 Their proposed definition is: reported fibers or other solid materials protruding from the skin, and skin lesions or disturbing skin symptoms.…”