An Unusual Differential Diagnosis of Orbital Cavernous Hemangioma: Ancient Schwannoma

Abstract

Schwannomas are rare lesions of the orbit that can be confused with cavernous hemangioma on imaging studies. We report the case of an 84-year-old woman with a 9-year history of a tumoral lesion in the inferolateral left orbit. The imaging studies did not reveal specific characteristics, only bone remodeling due to the long evolution of the tumor. The patient underwent complete excision of the tumor by anterior orbitotomy via the inferior conjunctival fornix. The histopathological examination revealed an ancient schwannoma, a variant of schwannoma with uncommon histological features. The follow-up was uneventful. The present case emphasizes the importance of considering neural tumors in the differential diagnosis of orbital masses with bone changes and degenerative alterations such as hemorrhagic areas, cysts, and/or calcifications.

Gegenüberstellung zweier Schwann-Zell-haltiger orbitaler Tumoren: Schwannom und Ganglioneurom

“…Orbital schwannomas quite often originate from the sensory nerve branches, primarily the supraorbital nerve and the supratrochlear nerve. However, in 50 % of cases, the origin remains unclear [1,7]. Ganglioneuromas, on the other hand, originate from sympathogonia of the neural crest along the sympathetic axis, and occur primarily in the abdomen and pelvis, including the adrenal cortex, followed by localization in the throat and neck area as well as the thorax [8].…”

“…Schwannomas belong to the family of peripheral nerve sheath tumors, and are mainly found in the head and neck region, followed by the flexor side of the extremities and the torso 6 . In the periocular area, the orbit is the most common location, with schwannomas accounting for about 1 – 2% of orbital tumors 1 , 7 . Orbital schwannomas quite often originate from the sensory nerve branches, primarily the supraorbital nerve and the supratrochlear nerve.…”

“…Im periokulären Bereich stellt die Orbita die häufigste Lokalisation dar, wobei Schwannome ca. 1 – 2% der orbitalen Tumoren ausmachen 1 , 7 . Ursprung orbitaler Schwannome sind häufiger sensorische Nervenäste, meist die Nn.…”

“…supraorbitalis und supratrochlearis. In 50% der Fälle bleibt der Ursprung unklar 1 , 7 . Das Ganglioneurom hingegen geht von Sympathogonien der Neuralleiste entlang der sympathischen Achse aus und tritt vor allem im Abdomen und Becken inkl.…”

Orbital schwannoma with calcification treated by intracapsular excision

“…The common manifestations include exophthalmos and eyeball dislocation, and some patients may have the symptom of vision decline, diplopia, periorbital spontaneous pain, or tenderness. [ 4 ]…”