Intracranial Plasmacytoma Research Articles

Intracranial Epidural Plasmacytoma Presenting as a Neurosurgical Emergency: Case Report

Intracranial plasmacytomas are infrequently encountered in neurosurgical practice, and the literature consists predominantly of isolated case reports. We present the first English medical literature case of intracranial epidural plasmacytoma with no calvarial lytic changes masquerading as an extradural hematoma in a 60 year-old-man. In addition, we discuss the pathogenesis of this unusual tumor location with brief review of the relevant literature concerning its treatment and outcome.

Conversion of Non-Secretory to Hyper-Secretory Myeloma: The Antithesis of the Common Pathway of Dedifferentiation with Myeloma Progression: A Case Study

Abstract 5052 Introduction:Non-secretory myeloma is characterized by the absence of M protein in both the serum and urine along with normal immunofixation. It occurs in approximately 2% of all patients with multiple myeloma (MM). Here we present the case of a non-secretory myeloma, which converted to secretory disease after treatment with high intensity chemotherapy. Presentation:A 50-year-old male without any significant past medical history presented in April of 2007 with diplopia and left-sided headaches. Imaging studies with CT and MRI of the brain revealed an enhancing lesion in the clivus extending into the sella turcica invovlving the cavernous sinus as well as a calvarial mass. A subsequent trans-sphenoidal biopsy of the mass was consistent with plasmacytoma and the patient underwent resection of both masses. Further bone marrow, imaging and serum studies showed no evidence of MM. Patient was subsequently treated with local radiation, which resulted in a great improvement of the patient’s vision, followed by Thalidomide 200 mg for 7 months. In May 2008 the patient presented to our institute for re-staging and follow up. MRI studies showed a recurrence of the plasmacytoma in the sphenoid sinus as a 4. 5 cm focal lesion and new 1. 5 cm focal lesion in the manubrium sterni. A CT scan revealed more than 30 osteolytic lesions; however, a PET scan remained negative. Serum IgG and kappa free light chain were within normal limits and a bone marrow aspirate revealed 14% plasma cells (PC) with a normal karyotype. Flow cytometric analysis (DNA/cIg) showed a near diploid clone with kappa light chain excess in 1% with cIg index of 3. 2. The patient was treated with Bortezomib, Dexamethasone, Thalidomide, Cisplatin, Adriamycin, Cyclophosphamide, Etoposide (VDT-PACE) followed by Melphalan 200 mg/m2 based autologous transplantation, achieving a very good partial response by IMWG criteria. The one-year follow-up in October 2009, in the absence of maintenance therapy, revealed that all focal lesions had resolved on MRI. However a slight increase in kappa free light chains (up to 10 mg/dl) was noted, prompting the start of Lenalidomide as maintenance therapy. In May 2010 the patient had a bone marrow with 30% PC’s on aspirate and 20% on biopsy with a cellularity of 30%. Gene expression profiling revealed low risk disease. MRI showed small focal lesions in the spine, shoulders, and left ilium, that were not seen on PET. For the first time, serum (free kappa 60 mg/dl) and urine markers (urine M 889 mg/l) were significantly increased. Treatment was changed to VDT with normalization of all markers within a month. The current evaluation as of May 2012 shows increased free kappa light chain levels of 1080 mg/dl, urine M protein of 6704 mg/l, with undetectable serum M protein at any point in time. Bone marrow aspirate and biopsy have 16% and < 5% PC respectively with a cellularity 40%. DNA/cIg flow cytometry remains normal and MRI and PET show no evidence for focal lesions, whereas cytogenetic analysis reveals a complex karyotype in 3/20 cells with a t[11:14] consistent with MM(43, X, -Y, add(1)(p36. 3), dup(1)(q12q44), add(11)(q25), t(11;14)(q12∼13. 1;q32), −14, +15, −16, −22, inc[3]/46, XY[17]) and transformation to high risk disease on GEP. Comparison of the first available GEP from 5/2010 with the last from 4/2012 reveals a 2. 5 –fold increase of the immunoglobulin kappa constant gene (IGKC). Discussion:Since the implementation of the free light chain assays, the diagnosis of non-secretory myeloma has become increasingly rare. Here we report an unusual case, which initially presented as a non-secretory solitary intracranial plasmacytoma. After initial response to local treatment this disease recurred systemically. Treatment with high dose chemotherapy resulted in the resolution of all active focal lesion and decreasing bone marrow plasmacytosis. However, the disease recurred within one year with the slight increase of kappa free light chains in the serum. That followed 2 years later by the detection of a clone with a t[11:14] with free light chains in the serum as well as M protein in the urine, indicating the emergence of a secretory clone, reaching hyper-secretory features on further progression. Contrary to the well-known dedifferentiation of secretory MM to “Bence Jones” escape and on to non-secretory status, the opposite development has to our knowledge not been reported. Disclosures:No relevant conflicts of interest to declare.

Extramedullary Plasmacytoma Presenting as a Solitary Mass in the Intracranial Posterior Fossa

A patient with a 3-month history of headache refractory to pain medication was admitted. The CT scan and MRI showed evidence of a posterior fossa mass. This was pathologically confirmed as an extra medullary plasmacytoma (EMP). He had a pathologic fracture of the left humerus 7 years ago while the radiologist was unaware at the time of diagnosis. A solitary bone plasmacytoma (SBP) was the cause of the pathologic fracture. This report includes the first description of MRI findings in a patient with a rare-incidence intracranial solitary extra medullary plasmacytoma (SEP) in Iran. There is a striking similarity between the features of intracranial SEP and meningiomas. Intracranial SEP, although rare, should be included in the differential diagnosis of brain tumors in areas where meningiomas commonly arise. The MRI findings and differential diagnosis of plasmacytoma are reviewed. Before this case report, only few cases have been reported in the literature. Nonetheless, this is the first report of posterior fossa EMP from Iran.

Multiple cranial nerve palsies: As first presentation of multiple myeloma with intracranial plasmacytoma

Compression of cranial nerves (CNs) by an intracranial plasmacytoma is considered to be an unusual presentation of multiple myeloma (MM). Here, we report a case of right 3 rd , 6 th , 9 th , 10 th and 12 th CN involvement, which emphasizes the fact that multiple CN palsy can be the first presenting feature of MM.

Solitary Intracranial Plasmacytoma Located in the Clivus: A Diagnostic and Theraputic Challenge

Solitary intracranial plasmacytoma (SIP) is a rare entity. To date, only 20 cases of SIP have been reported in English literature. Occasionally, SIP is comprised of atypical plasma cells which may not be plasma cell-appearing at all. As a result, pathologic diagnosis is extremely challenging, especially on frozen sections. A recent case demonstrating this profile is reported here. The patient was a 40-year-old white male who originally presented with diplopia. MRI revealed an enhancing non-infiltrating mass (5 x 4 x 3 cm) within the clivus. A transnasal endoscopic biopsy was done. Frozen showed sheets of pleomorphic cells with abundant eosinophilic cytoplasm and round/oval nuclei with stippled chromatin. On touch prep, naked nuclei with homogenous/salt and pepper chromatin, mild crush artifacts and rare plasmacytoid cells were noted. A frozen section diagnosis of unclassified neoplasm, defer to permanent sections was made. Immunohistochemical analysis on permanent showed that tumor cells were positive for CD138, CD56, kappa light chain, BCL-1 and negative for chromogranin A and lambda light chain. The Ki67 proliferation index was 5-10%. The immunostaining pattern was consistent with plasmacytoma. A subsequent complete work up for systemic disease revealed normal bone marrow biopsy, normal flow cytometry, and negative skeletal survey. The patient did have a serum M-spike of 0.75 g/dL but his quantitative immunoglobulins, kappa or lamda free light chains and beta-2 microglobulin were within normal limits. The patient was treated by radiation therapy accompanied by high-dose dexamethasone. However, four months post-radiation, repeat MRI showed a similar mass in the same area. His serum M-spike had dropped to 0.3 g/dL during his radiation therapy, but persisted at this level subsequently. The diagnosis of persistent residual plasmacytoma was reached. The patient underwent gamma knife radiosurgery and tolerated the procedure well. This case demonstrated that SIP should be considered as one of the differential diagnoses for intracranial tumor of unknown origin.

Solitary Intracranial Plasmacytoma Located in the Clivus: A Diagnostic and Theraputic Challenge

Solitary Intracranial Plasmacytoma Located in the Clivus: A Diagnostic and Theraputic Challenge

Cranial nerve palsy in multiple myeloma and solitary plasmacytoma

Cranial nerve involvement in multiple myeloma and solitary plasmacytoma is rare. We report on two patients who developed cranial nerve palsy due to skull base plasmacytoma. Patient 1, a 55-year old man with multiple myeloma, developed right sixth cranial nerve palsy during a phase of chemotherapy. He had an intracranial plasmacytoma in the clival region and was treated with 30 Gy whole brain radiotherapy. Patient 2, a 40-year old man, presented with right third cranial nerve palsy and was detected to have a solitary skull base plasmacytoma. He was treated with radiotherapy followed by thalidomide plus dexamethasone.

Solitary Intracranial Plasmacytoma Located in the Spheno-Clival Region Mimicking Chordoma: A Case Report

Solitary intracranial plasmacytoma (SIP) is very rare. This case report presents serial findings of SIP located in the spheno-clival region in a 54-year old female who presented with an inferior hemianopia in the right eye and an enlarged physiological blind spot in both eyes. Based on the initial diagnosis of a spheno-clival region chordoma, the tumour was partially resected by the nasal-sphenoidal sinus approach. Subsequently, the correct diagnosis of SIP was made based on the pathology and immunohistochemical staining of the tumour. The patient was treated using a whole skull-base radiation therapy protocol with 45 Gy and she was in good physical condition during the subsequent 22 months. The findings of a series of similar case reports documenting SIP in 20 cases published from 1976 to 2008 are also reviewed. Based on these case reports, the key features of SIP, including their clinical manifestations, clinical imaging characteristics, treatment and prognosis, are described.

Intracranial plasmacytoma as an initial presentation of multiple myeloma

Abstract Intra-cranial involvement is an uncommon complication of multiple myeloma. We report a 68-year-old woman hospitalized for acute renal failure, vertigo and headache. Magnetic Resonance Imaging (MRI) revealed two and a half and 3cm length cranial lesion, which proved to be plasmacytoma. After complete staging, we retained the diagnosis of immunoglobulin G lambda-type multiple myeloma with intracranial involvement. Cytogenetic analysis of plasma cells detected no change. Currently, she is a regular hemodialysis patient. She is planned to have both systemic therapy with 40mg dexamethasone and cranial radiotherapy. Involvement of the CNS in multiple myeloma is very rare. Diagnosis of multiple myeloma subsequent to initial intrcranial involvement is confined to exceptional cases. Despite aggressive systemic treatments, including autologous stem cell transplantation and local treatments such as cerebral radiotherapy, the prognosis for patients with CNS myeloma is extremely poor. Our unique case implies that physicians should be alert in case of vertigo or headache in cases with acute renal failure.

Impaired vision associated with a solitary intracranial plasmacytoma

Solitary intracranial plasmacytomas (SICPs) are extremely uncommon tumors in the central nervous system, and are often misdiagnosed pre-operatively. We report a patient with SICP, describe the neuroradiological and neurosurgical features and the clinical management of this patient, and review the pertinent literature.

First Manifestation of Lambda Positive Plasmacytoma in the Orbital Apex with Acute Unilateral Loss of Vision

An intracranial plasmacytoma is a rare form, which can involve the calvarium, dura or the cranial base. Only few case reports describe the manifestation of plasmacytoma of the skull base with affection of visual acuity. We describe the case of a 43-year-old woman, presenting with an acute unilateral loss of vision. The presumption diagnosis was retrobulbar neuritis as first manifestation of multiple sclerosis. MR imaging disclosed a tumour in the left orbital region and a meningioma was suspected. After complete resection with decompression of the optic nerve, the neuropathological examination revealed a lambda positive plasmacytoma. Additional work-up disclosed an involvement of multiple vertebral bodies. Due to the diagnosis of multiple myeloma, oncological therapy had been initiated. Skull base plasmacytoma is a rare disease. Solitary lesions causing neurological deficits should be treated aggressively including surgery for histological diagnosis and decompression of neural structures. Prognosis and further therapy depends on the systemic stage of disease, which has to be defined by diagnostic work-up.

A Patient with Chronic Inflammatory Demyelinating Polyneuropathy Associated with an Intracranial Plasmacytoma

A Patient with Chronic Inflammatory Demyelinating Polyneuropathy Associated with an Intracranial Plasmacytoma

Intracranial plasmacytoma with apoplectic presentation and spontaneous intracerebral hemorrhage: Case report and review of the literature

Involvement of the nervous system is not uncommon in patients with multiple myeloma, with polyneuropathy and myelopathy predominating. Intracranial involvement producing neurological symptoms, however, is distinctly uncommon. Massive intraparenchymal hemorrhage from a previously unrecognized intracranial plasmacytoma is exceedingly rare. The authors report the case of a 57-year-old male who presented with sudden onset of severe headache, rapid onset of right-sided weakness and deterioration in level of consciousness while at work. Two years earlier the patient had completed treatment for multiple myeloma and was considered to be in remission, with a recent bone marrow biopsy that was negative, and complete normalization of serum protein electrophoresis. Imaging studies revealed a massive intracerebral hemorrhage with the possibility of an underlying lesion, and the patient was taken for emergent hematoma evacuation and tumor resection. The patient made an excellent recovery and was treated with intracranial radiation. Even in patients with multiple myeloma without evidence of systemic disease following successful treatment, the possibility of unrecognized lesions lingers. The onset of new symptoms referable to potential intracranial pathology in this setting should prompt consideration of intracranial plasmacytoma in the differential diagnosis.

Multiple myeloma presenting as an intracranial plasmacytoma: a case report

Multiple myeloma presenting as an intracranial tumor (plasmacytoma) is very rare. An 81-year-old woman was admitted to our hospital because of gait disturbance. A blood laboratory test revealed a mildly increased lactate dehydrogenase (236 IU/L) and glucose (121 mg/dl). Blood protein fractions were normal. Brain computed tomography and magnetic resonance imaging revealed an intracranial mass (5 × 4 × 3 cm) in the brain base next to the clavus, and it was clinically diagnosed as chordoma. An excision of the brain tumor was performed. Imaging modalities including ultrasound, x-ray, computed tomography, magnetic resonance imaging and positron emission tomography did not reveal any tumors other than the brain tumor. The tumor was soft, fragile, and bloody. Microscopically, a monotonous proliferation of atypical plasma cells with hyperchromatic nuclei was recognized. Histochemically, the tumor cells were pyroninophilic and the congo-red stain revealed amyloidosis. Immunohistochemically, the tumor cells were positive for κ-chain and negative for cytokeratin, epithelial membrane antigen, vimentin, CD45, CD20, CD45RO, λ-chain, IgM, IgA, IgG, synaptophysin, chromogranin, S100 protein, desmin, α-smooth muscle antigen, myoglobin, p53 protein, and glial fibrillary acidic protein. The Ki-67 labeling was 11%. Intracranial plasmacytoma was pathologically diagnosed. The patient was treated by adjuvant chemoradiation, and entered into the complete remission stage. However, multiple metastases emerged in the vertebral bones and ribs six months after the remission. A diagnosis of multiple myeloma was made. The urine revealed Bence-Jones protein of monoclonal IgG κ-chain type, but blood M protein was not recognized. The patient's condition gradually deteriorated. The patient died of respiratory failure due to bronchopneumonia 18 months after the admission. The present case indicates that multiple myeloma may manifest as an intracranial brain tumor (plasmacytoma).

Intracranial plasmacytoma mimicking meningioma in a patient with Castleman's disease

Intracranial plasmacytoma mimicking meningioma in a patient with Castleman's disease

Extramedullary relapse of IgA-lambda myeloma after recent bortezomib therapy: a case report

Intracranial plasmacytomas are an uncommon presentation of extramedullary relapse of multiple myeloma. The optimal management of extramedullary plasmacytomas remains unclear, with initial reports of bortezomib showing promising clinical results. We describe a case of multiple extracellular, including intracranial, plasmacytoma, with no evidence of marrow involvement, in a patient with relapsed IgA multiple myeloma. To our knowledge, this is the first reported case of a patient with rapid extramedullary relapse of disease despite recent exposure to bortezomib and dexamethasone.

Development of Solitary Plasmacytoma in the Internal Auditory Canal and Inner Ear after Allogeneic Hematopoietic Stem Cell Transplantation for Plasma Cell Leukemia

Here we report the first case of the development of intracranial solitary plasmacytoma in the inner ear after allogeneic stem cell transplantation (allo-SCT) in a 39-year-old Japanese female with primary plasma cell leukemia (PCL). A point to note is that this is not a case on multiple myeloma but on PCL. She was successfully treated with local irradiation and survived more than 6 years from the time of diagnosis and transplantation. This case elucidates the biology of PCL and stresses the need for an individual approach to the clinical management of patients with plasma cell neoplasm undergoing allo-SCT.

Radiologic Manifestations of Primary Solitary Extramedullary and Multiple Solitary Plasmacytomas

Primary solitary extramedullary and multiple solitary plasmacytomas are rare manifestations of plasma cell tumors. This study reviews their imaging spectrum in 12 patients. Imaging features of primary solitary extramedullary and multiple solitary plasmacytomas are nonspecific but are compatible with solid tumors that are isointense on T1-weighted images and iso- to hyperintense on T2-weighted images relative to muscle and white matter with variable enhancement. Large tumors may show necrosis and destruction, infiltration, or encasement of adjacent structures. Multiplicity of lesions and regional lymphadenopathy were evident on imaging in only five cases each. Imaging alone cannot differentiate these tumors from more common malignant entities such as carcinoma, meningioma in cases of intracranial extramedullary plasmacytomas, or metastasis from other primaries. The role of imaging should be focused on early detection of additional or recurrent lesions and the presence of regional lymphadenopathy, which will influence clinical management.

Solitary plasmacytoma: experiences from Central Anatolia

Solitary plasmacytoma localised to bone or soft tissue without myeloma. Clinical features and survival was analysed in patients from Central Anatolia. Twenty-three solitary plasmacytoma (18 male, 5 female) were evaluated retrospectively. Median age was 58 years (46-72). The major localisation was vertebral column. All patients but one (larynx) had surgical resection and 21 patients received radiotherapy postoperatively. Multiple myeloma developed in eight patients (35%) and local relapse was detected in one patient. Eight patients died, causes of death were multiple myeloma progression in six patients, local relapse of intracranial plasmacytoma in one patient and cranial trauma in one patient who was in complete remission. Three and 5 years progression free survival were 45.6% and 22.8% respectively and overall survivals were 54.4% and 27.2% respectively. Solitary plasmacytoma cases should be followed carefully regarding local relapse and progression to myeloma.

Misleading imaging features of intracranial dural plasmacytoma: report of two cases

Dural plasmacytomas are very rare and often disregarded in the differential diagnosis of more common lesions of the convexity. We report two cases. Both patients exhibited headache as a main symptom. Computed tomography and magnetic resonance showed a homogeneously enhanced lesion without calcification and without bone involvement.