Presentation
Tongue weakness.
Patient Data
Nodular dural thickening centered over the right clivus, and foramen magnum extends over the hypoglossal canal and right jugular foramen. Transosseous extension through the right clivus into the right prevertebral tissues. There is also distortion of the upper cervical cord.
Nodular focus of enhancement along the right tentorium, along the falx, and elsewhere over the convexity.
Prior right frontal craniotomy at the site of biopsy.
Dural thickening seen at the level of the foramen magnum descends down to the level of the C2-3 resulting in canal stenosis without cord signal abnormality within the cord. A with a further focus of a smooth dural thickening also demonstrated posterior to T3 -T5 without significant canal stenosis.
The sections show dense, fibrous tissue with a central hypercellular region containing a dense lymphoplasmacytic infiltrate admixed with larger cells with irregular pale ovoid nuclei and abundant clear cytoplasm. Occasional binucleate forms and intranuclear inclusions can be seen. Russel bodies are noted. Mitotic count is <1 per 10 HPF. There is no sheetlike growth, nucleoli prominence, or necrosis evidence.
Immunoperoxidase demonstrates strong membranous staining with SSTR2a *. EMA is negative. CD3 and CD20 highlight background lymphocytes.
Final diagnosis: lymphoplasmacyte-rich meningioma (CNS WHO grade 1).
Pathology courtesy of Dr Samuel Roberts-Thomson.
Note: * Somatostatin receptors (SSTR), especially SSTR2, are reliable markers for meningiomas 1.
Case Discussion
Lymphoplasmacyte-rich meningiomas are rare and somewhat controversial, in many ways presenting more like an inflammatory pachymeningeal process than a localized tumor. This case is a good example of this, with extensive areas involved.
The main differential is that of other causes of pachymeningeal thickening.
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