Clivus myeloma
A case of a 68-year-old male patient with multiple myeloma, clivus bone plasmacytoma, and cavernous sinus syndrome 1)
A case of extramedullary plasmacytoma of the clivus leading to progressive visual deficits with undiagnosed multiple myeloma requiring pulse steroids, intracranial irradiation, and high-dose chemotherapy with improvement in symptoms 2).
A 50-year-old woman presented with a right-sided isolated third cranial nerve palsy. MRI brain showed a mass lesion arising from the right clivus with extension into the cavernous sinus. Blood investigations and bone marrow biopsy were suggestive of multiple myeloma with hypercalcaemia and renal dysfunction. It was unclear at first if the intracranial lesion was due to myelomatous involvement or a separate disease entirely. The patient declined consent for a biopsy and cerebrospinal fluid analysis was inconclusive. She was treated with bortezomib based chemotherapy and the palsy resolved by day 6, which helped clinch the rare diagnosis of central nervous system (CNS) involvement by multiple myeloma. Most patients with CNS myeloma have a dismal survival of under 6 months but she is on therapy for relapse 26 months after diagnosis. While placed under the umbrella of CNS myeloma, patients with osteodural myeloma have better outcomes, perhaps due to their distinct aetiopathogenesis 3).
Multiple myeloma presents as a massive osteolytic lesion at the clivus 4)
A 69-year-old man presented with a primary complaint of diplopia and an examination consistent with bilateral abducens nerve palsy. No other deficits were noted. Magnetic resonance imaging of the skull base demonstrated a large T1 isointense moderately enhancing lesion centered within the clivus. Endoscopic biopsy of the mass revealed sheets and aggregates of mature monoclonal plasma cells. The patient's initial systemic work-up revealed that this was a solitary lesion, and he was treated with radiation therapy to the skull base with a durable local effect at an 18-month follow-up. Unfortunately, he progressed to multiple myeloma with peripheral osteolytic lesions but has been stabilized on chemotherapeutics. Conclusion The clivus is an unusual site for intracranial plasmacytomas, and enhancing lesions must be differentiated from chordoma. Characteristic findings on histopathology include an immunoglobulin light-chain restricted clonal proliferation of plasma cells. Treatment is most commonly radiotherapy with surgery reserved for biopsy and palliation. Clinicians should be aware of the increased risk of progression to multiple myeloma in skull base plasmacytomas 5).
Case report from the HGUA
A 66-year-old woman with a clivus lesion in the context of multiple myeloma is scheduled for a biopsy and extensive excision. The tumor appears to respect the saddle and gland. The medical history includes secondary diabetes mellitus due to long-term corticosteroid treatment, suspected cranial plasmacytoma with multiple lesions suggestive of multiple myeloma, and a history of multiple myeloma treated with VCD regimen and autologous hematopoietic stem cell transplantation. The patient has experienced complications, including meningitis, urinary tract infection, febrile neutropenia, and visceral leishmaniasis. The current admission is for the excision of a plasmacytoma located in the sella turcica, secondary to multiple myeloma. Preoperative hormonal studies are not available, and the patient will undergo a hormonal assessment before and after surgery. Insulin therapy will be adjusted, and contact with the hematology department will be made for further management. The patient's progress will be monitored.