Atypical primary meningioma in the nasal septum with malignant transformation and distant metastasis

Abstract

BackgroundPrimary extracranial meningiomas (PEMs) originating from the nasal septum are extremely rare, as are extracranial metastases of meningiomas.Case presentationA 44-year-old male presented with a 2-month history of left-side nasal obstruction and frequent episodes of epistaxis. A friable mass originating from the nasal septum was resected completely via an endoscopic endonasal approach. According to WHO criteria, the tumor was diagnosed as an atypical meningioma radiologically and histopathologically. Two years later, a tumor recurred at the primary site with the same histopathological findings, and the patient was given local external radiotherapy (6840 cGy in 38 fractions). Two months after this local recurrence, a left anterior chest wall mass and a left parietal area scalp mass were observed. The subcutaneous mass was resected and showed histological evidence of malignant transformation. Several months after the last operation, the patient died.ConclusionsWe describe the clinical, radiological, and bio-pathological features of this unique case and review the literature on atypical PEMs originating in the nasal septum. To our knowledge, this is the first reported case of an atypical PEM originating from the nasal septum that recurred with malignant transformation and extracranial metastasis.

Metastatic meningioma presenting as a malignant soft tissue tumour

“…Doxtader et al [14] described an aggressive meningioma in an 8 year old patient with metastasis to cervical soft tissue and lymph nodes. There is also a report of an isolated “chest wall” metastasis and of a bone/soft tissue metastasis from an atypical primary meningioma of the nasal septum that clinically simulated a soft tissue sarcoma [15, 16]. In a review of the literature between 1886 and 1958, Karasick et al [5] reported 56 patients who developed extracranial meningioma metastasis, none of which were in soft tissue.…”

“…There are very few reports of malignant meningioma metastasising to deep soft tissues [4, 14–16]. We report a case of a metastatic anaplastic meningioma which presented as a growing soft tissue tumour in the trapezius muscle and review the literature regarding soft tissue involvement by metastatic meningioma.…”

Imaging features of benign mass lesions in the nasal cavity and paranasal sinuses according to the 2017 WHO classification

“…In most cases, direct extension by permeative growth from the intracranial primary lesion can be documented. MR imaging shows various signal intensities on T2-weighted image, and hypointensity on T1-weighted image with homogenous contrast enhancement [44].…”

Paraneoplastic symptoms caused by extracranial meningioma metastases?

“…[1345678101114151617] The incidence of extracranially metastasizing meningiomas seems to be less than 1:1000. [7] If distant metastases occur, they usually affect the lungs, bones, spinal canal, liver, or kidneys.…”