Meningeal plasma cell granuloma with relapsing polychondritis

Abstract

Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent inflammation of the cartilaginous structures and connective tissue. Central nervous system lesions in association with RP have occasionally been reported, but intracranial mass lesions have not been described. The authors report the first such case, in which a 51-year-old man presented with parasagittal meningeal plasma cell granuloma with RP. The mass was subtotally resected and adjuvant radiotherapy was administered. The patient did not experience any recurrence of the lesion during an 8-year follow-up period. In this case, the exact diagnosis of RP was made based on symptoms of respiratory tract chondritis, which was successfully treated by the placement of tracheobronchial stents.

Isolated plasma cell granuloma of the meninges

“…3 As all three conditions are rare, their radiological features can easily be confused with meningeal carcinomatosis and histological features can be easily confused with other diseases like plasmacytoma or lymphoplasmacyte-rich meningioma. 6 In any of these instances, brain and meningeal biopsy and careful histological examination is crucial in reaching the correct diagnosis. Immunohistochemistry on kappa and lambda light chains showed polyclonal plasma cell population (x20).…”

Primary intracranial plasma cell granulomas presenting as malignant neoplasms

“…Other concomitant diseases have been described with PCG. Interestingly, in many cases, a systemic inflammatory disease was present such as IRIS [9], relapsing polychondritis [22], or Sjogren's disease [23]. This may suggest that a subacutely activated systemic inflammatory state can predispose to a locally exuberant inflammatory mass.…”

“…The maleto-female ratio was 2.3:1. Thirteen additional cases have been reported since 2003 per our literature search [9,17,22,[24][25][26][27][28][29][30][31][32]. Of these, three cases involved the cerebral hemispheres [17,24,25] and one case involved the cerebellar hemisphere [9].…”

“…Four cases had both brain parenchymal and dural infiltration [26][27][28]. Two cases involved primarily the meninges [22,29], and one case each involved the pituitary gland [30] and trigeminal nerve [31]. Cerebral PCG has been reported in nearly every intracranial location including the meninges, cerebral hemispheres, ventricles, hypothalamus, sellar region, and cerebellum [5].…”

“…Similarly, we also observed a clinical response to high-dose corticosteroid treatment in our first patient. Another patient with incomplete resection of the intracranial PCG was treated with external beam radiation therapy with favorable outcome [22]. Finally, a case of recurrent intracranial PCG was successfully treated with chemotherapy (methotrexate and 6-mercaptopurine) following radiation and steroids [49].…”