Cystic change in primary paediatric optic nerve sheath meningioma

Abstract

Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size.

Natural history of primary paediatric optic nerve sheath meningioma: case series and review

“…Follow-up after 89 months demonstrated no tumour growth and vision was 20/20. Patient 3 presented with 20/20 vision and was also treated conservatively 16. After 16 years, vision was 20/30 and the tumour had undergone cystic degeneration and decreased in size.…”

Superonasal Cystic Orbital Mass

“…If degenerative myxoid changes are present within the mass, however, cystic areas may be seen on radiographic imaging . Cystic schwannomas must be distinguished from other cystic lesions of the orbit, including parasitic infections, dermoid cysts, optic nerve sheath gliomas and meningiomas, solitary fibrous tumors, and orbital malignant neoplasms, such as adenoid cystic carcinoma, that may occasionally present with cysts; biopsy is typically needed for identification.…”

Cystic Lesions of the Orbit

“…[124][125][126] In addition to the more common cystic neoplasms, case studies have described cystic morphology in primary orbital solitary fibrous tumor, meningioma, pilomatrixoma, and synovial sarcoma, among others. [127][128][129][130][131] Cystic orbital metastases have been described in a diverse range of primary diseases, including melanoma, breast, neuroendocrine, bladder, renal cell, and endometrial cancers. 122,[132][133][134] Though the exact pathogenesis leading to cystic degeneration is not well understood, the process is likely provoked by necrotic destruction and consequent thrombosis of tumor vasculature, resulting in intralesional hemorrhage and cyst formation.…”

“…In addition to the more common cystic neoplasms, case studies have described cystic morphology in primary orbital solitary fibrous tumor, meningioma, pilomatrixoma, and synovial sarcoma, among others 127–131 . Cystic orbital metastases have been described in a diverse range of primary diseases, including melanoma, breast, neuroendocrine, bladder, renal cell, and endometrial cancers 122,132–134 …”