Background: The effectiveness of autologous stem cell transplantation (ASCT) in preventing the development of central nervous system (CNS) plasmacytomas in multiple myeloma (MM) patients is not well understood. An ASCT patient who developed CNS extramedullary (EM) lesions is presented. The literature was reviewed for similar cases in which the transplant did not prevent the development of CNS lesions.
Case description: A 42-year-old female was evaluated after complaining of a sudden severe headache and complete vision loss. Two years before, she was diagnosed with MM and treated with systemic chemotherapy and an ASCT. The patient was in remission; however, a new brain magnetic resonance imaging showed a sellar and suprasellar mass. Additional smaller lesions were identified at the parietal convexity and the splenium. Due to the history of MM and evidence of multiple intracranial lesions, it was suspected that the lesions were secondary to EM disseminated disease. Due to the sudden loss of vision, the patient underwent a right frontotemporal craniotomy with subtotal sellar/suprasellar tumor resection to decompress the optic nerves. Histopathological examination of the lesion confirmed an immunoglobulin A (IgA) EM sellar and suprasellar plasmacytoma.
Conclusion: In the majority of MM patients with CNS involvement, ASCT did not prevent the development of EM sellar plasmacytomas. The IgA subtype is associated with more aggressive disease biology for CNS relapses.
Keywords: Extramedullary; Multiple myeloma; Plasmacytoma; Relapse; Sella; Stem cell; Suprasellar; Transplantation.
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