Intracranial Plasmacytoma Research Articles

Extramedullary Intracranial Plasmacytomas: A Systematic Review of Literature.

Extramedullary plasmacytoma (EMP) is an uncommon disorder characterized by the development of abnormal plasma cell tumors outside the bone marrow. These tumors are typically observed in various locations, including the upper respiratory tract, gastrointestinal tract, and other soft tissues. Among the less explored manifestations of EMP is intracranial EMP, which remains poorly understood due to the limited literature available on the subject. The objective was to comprehend the population characteristics, localization, type, treatment, and outcomes of intracranial EMP. A systematic review of the literature for EMPs was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The strategy "extramedullary plasmacytoma AND multiple myeloma" was used for the search. The search terms were queried using PubMed, Embase, Scopus, Cochrane, and Web of Science databases. We included only those studies that presented clinical studies with patients diagnosed with intracranial plasmacytomas. In this study, a total of 84 patients from 25 studies were analyzed. The average age of diagnosis was 57.25 years, with a slightly higher proportion of females (57%) compared to male patients (43%). The most common locations of intracranial plasmacytomas were the clivus (29.7%), frontal lobe (18.9%), parietal lobe (8.1%), occipital lobe (6.7%), temporal lobe (6.7%), and sphenoid (4%). Chordoma and meningioma were the most common differential diagnoses encountered during clinical investigations. Treatment modalities included radiotherapy (RT), chemotherapy (QT), surgical resection (SR), and conservative approaches. The most frequent treatment combinations were SR + RT (19%) and RT only (17.8%). Mortality was reported in 48% of the cases, with complete resolution observed in 10 cases and partial resolution in 3 cases. The average follow-up duration was 37.5 months. The clivus is the most frequently reported site of extramedullary intracranial plasmacytoma (EMIP) occurrence, representing 29.7% of cases. Chordomas were commonly observed alongside EMIPs and emerged as the primary differential diagnosis. RT was the predominant treatment modality, with SR considered when feasible. RT alone demonstrated the highest effectiveness in managing EMIPs (30%), while QT as a sole intervention showed lower efficacy. However, a combination of dexamethasone, lenalidomide, and targeted RT displayed promising results, offering improved tumor response and increased safety.

Enhanced clinical outcomes with radiotherapy in diagnostically challenging intracranial plasmacytomas: Analysis of 190 cases.

Intracranial plasmacytomas are rare tumors arising from plasma cells with approximately half of the cases progressing to multiple myeloma (MM). However, there is a lack of comprehensive clinical cohort analysis on the clinical and pathological features, progression, and outcomes of intracranial plasmacytomas. A retrospective analysis of 190 cases was conducted, combining data from 38 cases in a single institution and 152 cases from the literature. Patient demographics, clinical presentations, tumor locations, imaging features, surgical treatments, and follow-up outcomes were collected and analyzed. Survival analysis and Cox regression analysis were performed to identify prognostic factors. A total of 190 intracranial plasmacytoma patients with an average age of 55.4 years were included in the study. The preoperative misdiagnosis ratio was high at 55.3%, and 59.7% of the tumors affected the calvaria convexity, compared to 40.3% located at the skull base. Resection and biopsy were achieved in 72.4% and 27.6% patients, respectively. Among them, 34.2% (65/190) of patients were initially diagnosed with MM with intracranial plasmacytoma as their first presentation (MM-IPFP), while 63.2% (120/190) of patients were diagnosed with solitary intracranial plasmacytoma (SIP), including 61 extramedullary plasmacytomas and 59 solitary bone plasmacytomas. In the SIP group, 22.4% (24/107) of patients experienced disease progression leading to the development of MM during a median follow-up time of 42.6 months (range 1-230 months). Multivariate analysis unveiled that radiotherapy (HR, 0.05; 95% CI, 0.00-0.87; p = 0.04), not surgery, was a protective prognostic factor for overall survival in MM-IPFP patients. Comparison between the SIP progression group and non-progression group revealed a significant difference of Ki-67 index (non-progression vs. SIP progression, 8.82% ± 7.03 vs. 16.5% ± 10.5, p < 0.05). AUC analysis determined that a cutoff value of 9.0% was the best predictor of SIP progression, with an area under the curve of 0.712. This retrospective clinical analysis highlights the potential role of radiotherapy, rather than surgical resection, in improving the outcomes of intracranial plasmacytoma. Additionally, the Ki-67 index is identified as a valuable marker for predicting disease progression. This would provide some evidence for the paradigm of diagnosis and treatment modalities for intracranial plasmacytomas from the large cohort.

A case of autoimmune encephalitis in a patient with a solitary intracranial plasmacytoma.

A 65-year-old woman presented with fever and abnormal behavior. Magnetic resonance imaging showed swelling of the left medial temporal lobe and an intracranial extra-axial occipital tumor. While her neurological symptoms improved after the administration of corticosteroid therapy under the suspicion of autoimmune encephalitis, the occipital tumor unexpectedly shrank, and the diagnosis of a solitary plasmacytoma was confirmed by biopsy. Additional examinations revealed elevated anti-glutamate receptor antibodies in the cerebrospinal fluid. The patient was diagnosed with autoimmune encephalitis concurrent with an intracranial solitary plasmacytoma. Central nervous system involvement can be considered a neurological complication in patients with a solitary plasmacytoma.

Multiple Myeloma with Intracranial Distribution

Multiple myeloma (MM) is a malignant immunoproliferative disease involving plasma cells, leading to their malignant proliferation and the production of monoclonal paraproteins. Intracranial tumor spread in MM is very rare. This is a unique and infrequent localization, which makes it difficult to diagnose the disease. We report a rare case of treatment of a patient with MM intracranial spread, whose disease was presumably in remission and recurred as an intracranial plasmacytoma with a clinical picture of acute cerebrovascular accident. Surgical intervention was performed to remove the tumor, a histopathological analysis of the removed tissue was carried out. Even with early diagnosis, the prognosis remains bleak, with median survival rates of 3 to 6 months. However, aggressive multimodal treatment can prolong life by months.

PLASMOCITOMA SOLITARIO INTRACRANEAL: REPORTE DE CASO

Introduction: Solitary intracranial plasmacytoma is a rare plasma cell tumor that affects the skull, meninges, and brain. Unlike multiple myeloma, it does not have systemic manifestations. Its symptoms are characterized by a progressively growing tumor that generates local pain. It does not have a pathognomonic image and can be confused with other lesions, being its diagnosis histological. The prognosis is good and the main treatment modalities are surgery and/or radiation therapy. Clinical Case: 64-year-old female patient with a clinical picture characterized by loss of strength in lower limbs and thermal rise. On examination: Patient awake, Glasgow 15 points, paraparesis 4/5, photoreactive and isochoric pupils. Imaging examinations show an extensive contrast-enhancing lesion involving cerebral meninges at the frontoparietal level bilaterally, with a moderate mass effect. A subtotal resection of the lesion is performed, with the patient presenting a good clinical evolution. The histological result was a plasma cell tumor. In the 6-month follow-up, no residual lesion was observed, maintaining outpatient control by an outpatient clinic. This case shows a rare pathology that is sometimes confused with a meningioma. Conclusion: Solitary intracranial plasmacytoma is a rare tumor that can easily be confused with other more common lesions. Its diagnosis is only made with a histological study. It has a good prognosis and can be treated by surgical resection and/or radiotherapy. Keywords: Plasmacytoma, Brain, Meninges, Neoplasms, Plasma Cell (Source: MeSH NLM)

Multiple myeloma with intracranial plasmacytoma

We present a case of a 43-year-old male who was found to have an intracranial mass lesion on headache imaging. On further follow up, the patient developed systemic features of hypercalcemia, anemia and renal dysfunction. The biopsy of the mass was negative multiple times. A bone marrow biopsy was done due to high suspicion of multiple myeloma which showed plasma cell infiltration of the bone marrow. The patient's serum was however negative for an M band with only a narrow M band in the urine suggestive of oligosecretory multiple myeloma.

Purely Intraparenchymal Plasmacytoma: A Diagnostic Conundrum. To Operate or Radiate?

Intracranial plasmacytomas are rare entities that are thought to spread to the intraparenchymal space via pre-existing lesions from the dura or skull. Purely intraparenchymal plasmacytomas, without cranial or dural involvement, are therefore, rarer and seem to contradict the mechanism of spread that have been attributed to these lesions. Only a few cases of purely intraparenchymal lesions have been described in the literature, and they represent diagnostic conundrums when identified as they mimic the radiographic appearance of other parenchymal lesions, such as metastatic tumors or infectious collections. The authors report an unusual case of a purely intraparenchymal plasmacytoma in the right frontal lobe of a 68-year-old female with a known history of multiple myeloma. No evidence of extension from a cranial or dural-based lesion was found radiographically or intraoperatively and given the non-diagnostic radiographic features of this plasmacytoma, the diagnosis was made upon pathological examination after gross total resection. A review of the literature on purely intraparenchymal plasmacytomas is provided and the management and prognosis of these lesions are addressed.

Solitary Intracranial Plasmacytoma in an Unusual Location – A Case Report

Plasmacytoma is rare form of plasma cell dyscrasias, where there is malignant proliferation of plasma cells. Solitary plasmacytoma developsin isolation without systemic manifestations of multiple myeloma (MM). We report a case of cerebellopontine angle (CPA) plasma cytoma, which masqueraded as a schwannoma with multiple cranial nerve involvement and review a literature pertaining to the same pathological entity.

Clinical features, radiological profiles, and surgical outcomes of primary intracranial solitary plasmacytomas: a report of 17 cases and a pooled analysis of individual patient data.

We aim to delineate the clinical characteristics of patients with primary intracranial solitary plasmacytoma (PISPC) and prognostic factors for their outcomes. This study retrospectively reviewed 17 patients with PISPC from our center and an additional 70 cases of PISPC published previously to analyze outcome predictors. The entire cohort included 38 (43.7%) males and 49 (56.3%) females with a mean age of 54years. Skull base tumors were found in 49 (56.3%) patients. Gross total resection (GTR) was achieved in 31 (35.6%) patients. Postoperative adjuvant treatments, including radiotherapy (RT) alone, chemotherapy (CMT) alone, and RT + CMT were administered in 49 (56.3%) patients, 3 (3.5%) patients, and 16 (18.4%) patients, respectively. After a median follow-up of 24 (mean 42.4) months, the 5-year disease progression-free survival (PFS), recurrence-free survival (RFS), multiple myeloma (MM)-free survival (MMFS), and overall survival (OS) were 52.9%, 76.2%, 69.6%, and 76.1%, respectively. Multivariate analysis unveiled that a skull base tumor location (HR 2.395, p = 0.040) and no RT (HR 3.115, p = 0.004) were negative prognostic factors for PFS, no RT (HR 10.526, p = 0.003) for RFS, each 1-year increase in age (HR 1.039, p = 0.049) for MMFS, and increasing age (HR 1.052, p = 0.043) and CMT (HR 6.022, p = 0.005) were risk factors for OS. However, GTR did not benefit the aforementioned outcomes. For patients with presumed PISPC, a biopsy followed by RT is recommended for skull base PISPC. However, the role of CMT is still not clear. Our findings need to be verified in a larger prospective cohort in the future. Systematic review registration number CRD42018098782.

Sudden and unexpected death due to intracranial sellar extramedullary plasmacytoma

The incidence of sudden death caused by undiagnosed primary intracranial neoplasm in young adults is extremely low. The aim of the work is to present a case report of the sudden death of a 24-year-old woman. The autopsy has revealed enlargement of sella turcica with an intrasellar tumorous mass extending into the adjacent basal parts of the brain. A tumour was diagnosed as an extramedullary plasmacytoma. The cause of death was established as a failure of the central nervous system. Toxicological analyses of biological materials were negative. According to the case history, a woman reportedly had no serious health problems. Finding of an extensive intracranial tumour in the sella turcica was random and surprising. The presented case is an extremely rare case of sudden death caused by intracranial intrasellar extramedullary plasmacytoma without previous clinical manifestation.

Atypical Recurrence Presentation of Pre-Transplant Period Multiple Myeloma: Multiple Intracranial Plasmacytomas With Intratumoral Hemorrhage

Multiple Myeloma may have extraosseous manifestations in the cranial region. It may be a solitary intracranial tumor (extramedullary plasmacytomas without any other signs of multiple myeloma) or intracranial disease may be a part of generalized disease. We report herein a 65-year-old multiple myeloma patient with the involvement of different regions of brain parancyhma with spontaneous intratumoral hemorrhages. It is an unusual recurrence presentation and unusual situation that intracranial involvements are both with and without dural attachment and all of them have intratumoral hemorrhages.

Plasmocitoma intracraniano solitário

Os autores relatam caso de plasmocitoma solitário, sem manifestação sistêmica da doença, em paciente de 43 anos, com história de cefaléia parietal esquerda. São discutidos os aspectos tomográficos e a conduta, bem como a revisão de literatura, visto ser este o primeiro caso publicado na literatura nacional.

Intracranial Plasmacytoma with Unilateral Vision Loss: An Unusual Initial Presentation of Multiple Myeloma

Intracranial Plasmacytoma with Unilateral Vision Loss: An Unusual Initial Presentation of Multiple Myeloma

PC3 - 216 Presentation of Intradural Extramedullary Plasmacytoma with Intratumoral Hemorrhage: Case Report and Review of Literature

Plasmacytomas are solitary tumours characterized by neoplastic proliferation of plasma cells and can be found isolated or in associated with multiple myeloma. Plasmacytomas uncommonly occur intracranially, and dural plasmacytomas without involvement of the calvarium are exceedingly rare. Reported cases indicate durally-based plasmacytomas mimick the appearance of meningioma, lymphoma or sarcoma of the dura. The authors report a case of a 77-year-old male with known multiple myeloma who presented with a 3-week history of confusion, speech impediment, and right sided weakness. A non-contrast CT scan revealed a dense extra-axial mass in the left frontal lobe with initial concerns of an extra-axial hemorrhage. A subsequent MRI demonstrated a contrast enhancing mass with a broad-based dural tail and no underlying calvarial lesion. Differential diagnosis included meningioma or intracranial plasmacytoma. The patient underwent surgical resection and was found to have intratumoural hemorrhage, with pathology confirming plasmacytoma. In the published literature, there are only 20 prior reports of dural plasmacytomas (with and without primary calvarial infiltration), of which only five previous cases reported associated intratumoural hemorrhage. Our case, along with this literature, suggests that new onset of focal neurologic deficits in patients with a history of multiple myeloma merits careful investigation, and that intracranial plasmacytoma should be considered on the differential diagnosis even when imaging reveals masses consistent with hemorrhage or meningioma.

Multiple myeloma with intracranial extension and bilateral renal infiltration: A case report and review of the literature

Multiple myeloma (MM) is a rare hematological malignancy, characterized by uncontrolled proliferation of plasma cells in the bone marrow. MM is usually confined to the bone marrow, however, it may occasionally infiltrate other tissues, which is known as extramedullary plasmacytoma (EMP). The majority of EMPs involve the head and neck region, although different anatomical sites, including the gastrointestinal tract, central nervous system, thyroid gland and breast may also be affected. The simultaneous presentation of EMP in the kidney and head is rare, presenting diagnostic challenges due to its unusual location and non-specific or absent symptoms. To the best of our knowledge, no case of extramedullary plasmacytoma presenting with simultaneous renal and intracranial infiltration has been reported in the literature thus far. However, the present study reports a case of primary renal and intracranial extramedullary plasmacytoma in a 76-year-old male patient. The patient presented with a swelling over the right side of the forehead, which had slowly increased in size prior to hospital admission. The swelling was associated with dizziness and weakness, without bone pain. Contrast magnetic resonance imaging suggested an osteolytic skull lesion with intracranial extension. Abdominal enhanced computed tomography scanning revealed a large tumor mass extending around and into the kidneys. Immunohistochemical examination of the renal tumor biopsy, and blood and serum samples, as well as immunoelectrophoresis of serum proteins, resulted in a diagnosis of EMP being proposed. Therefore, the patient was administered with two cycles of cyclophosphamide and thalidomide in combination with dexamethasone. Follow-up imaging performed 4 months later revealed almost complete disappearance of the intracranial tumor mass and renal infiltration. The current study also presented a review of the literature. This study revealed that EMPs may co-exist with MM or present as the main symptom of MM. The diagnosis of an EMP is complex and requires radiological, hematological, biochemical and histological investigation. At present, no guidelines for EMP treatment have been established and thus, treatment options include surgery, chemotherapy and radiotherapy, either alone or in combination. We hypothesize that combined treatment may provide the best patient outcome.

A Challenging Case of IgD Kappa Multiple Myeloma Associated With Primary Amyloidosis: Importance of Serum Free Light Chains in Monitoring Treatment Response and Disease Relapse

Multiple Myeloma (MM) is a malignancy of B cells characterized by an atypical proliferation of plasma cells. IgD MM has a very low incidence (2% of total MM cases) and its characterized by an aggressive course and a worse prognosis than other subtypes. The serum free light chains (sFLC) are very important markers for monitoring patients with MM and other monoclonal gammopathies. When the sFLC are present in low concentrations, it is often difficult to detect them by conventional methods such as serum protein electrophoresis and serum immunofixation. This case is a good example of the utility of sFLC in the monitoring of treatment and relapse of patients with MM. The sFLC determinations detected when chemotherapy applied was not being completely effective and it predicted a relapse before new clinical findings were present. We report the rare case of a patient diagnosed of IgD MM with associated light chain kappa Primary (AL) Amyloidosis and characterized by clinical findings of very bad prognosis (high levels of sFLC kappa at diagnosis, high renal impairment and subsequent development of intracranial plasmacytoma), aggressive course and refractory to several lines of treatment.

Multiple cranial nerve palsies in a case of intracranial plasmacytoma and multiple myeloma: A report

Multiple myeloma is usually associated with neurological features like peripheral neuropathy and compressive myelopathy. It is unusual for multiple myeloma to present with multiple cranial nerve palsies. We report a case of multiple myeloma with an extramedullary plasmacytoma in the maxillary antrum, which resulted in multiple cranial nerve palsies. The patient was diagnosed to have IgG λ subtype in stage II according to the international staging system (ISS). The patient did not respond to a combination of radiotherapy and chemotherapy.

Initial Presentation Of Multiple Myeloma As Intraparenchymal Brain Mass

Case presentation38 year old African female presented with complaints of severe headache which had started couple of weeks prior to presentation and was progressively becoming worse. Patient underwent a CT scan of the head which revealed a 3.2 X 3.3 X 3.7 cm lobulated hyperdense periventricular mass in the left temporoparietal area with surrounding vasogenic edema with mass effect on the adjacent left lateral ventricle and 0.6 cm midline shift to the right with uncal herniation and effacement of left cerebral peduncle with dilatation of left temporal horn. MRI of the brain revealed a 3.1 X 2.6 X 3.6 cm lobulated mass in left lateral ventricle trigone. Radiologically, differential diagnoses included intraventricular meningioma, lymphoma, choroid plexus papilloma or metastasis. Patient underwent left craniotomy for tumor resection. Patient had an uneventful post-operative recovery and the resected tumor was high grade malignant neoplasm with plasmablastic features and immunohistochemical stains revealed that the tumor cells were positive for CD138, CD30, MUM-1, Bcl-2, vimentin and lambda light chains and were negative for kappa light chains, CD3, CD20, PAX-5, CD79a, GFAP, cytokeratin, AE1/AE3, synaptophysin, chromogranin, EMA, S-100, Melan-A, CD45, CD56 and EBV(EBER-ISH). Ki-67 was about 80%. Serum protein electrophoresis (SPEP) was sent that showed an M-spike of 3.3 g/dl that was IgG lambda. A bone marrow biopsy showed 100 % infiltration with plasma cells. Patient underwent a CT chest/abdomen/pelvis and a PET/CT scan which revealed multiple scattered subcutaneous masses throughout the body and an asymptomatic mass near spinal cord at C1. Patient was treated with VTD-PACE regimen (bortezomib, thalidomide, decadron, cisplatin, liposomal doxorubicin, cyclophosphamide, and etoposide). She received two cycles of VTD-PACE chemotherapy regimen with excellent response to the treatment. Her M-spike protein which prior to treatment was 3.3 g/dl disappeared after second cycle of chemotherapy and her subcutaneous lesion also dramatically improved on repeat PET/CT scan. The C1 dural lesion also had significant improvement after the chemotherapy. Her IgG also decreased from 5070 mg/dl to 791 mg/dl. She was referred for autologus stem cell transplant. She was subsequently started on weekly cyclophosphamide, bortezomib and decadron. DiscussionMalignancies of plasma cells comprise 1% of malignant neoplasms which includes multiple myeloma, solitary plasmacytomas (including solitary bone plasmacytoma and extramedullary plasmacytomas) and immunoglobulin deposition syndromes. Central nervous system (CNS) involvement of multiple myeloma itself is not a common entity. Fassas et al. published data of 18 cases over a course of 10 years. Gozzetti et al. published their data of 50 patients in 2012. Of these 50 patients, 76% had osteo-dural or primary dural multiple myeloma (OD-DMM) and 24% had central nervous system myelomatosis. They found that patients treated with novel agents had better outcome than patients treated with conventional drugs. Cases with initial presentation of intracranial plasmacytomas are even rarer. Patients with CNS myeloma have poor prognosis with median survival being around 4- 5 months. Our patient had excellent response with two cycles of VTD-PACE regimen with negative M-spike, normalization of IgG and decrease in size of subcutaneous nodules and C1 spinal lesion. She has survived for 5 months without autologus transplant and is currently on weekly cyclophosphamide, bortezomib and decadron. Disclosures:No relevant conflicts of interest to declare.

Intracranial plasmacytomas mimicking epidural hematoma and revealed by brain hernia

Intracranial plasmacytomas mimicking epidural hematoma and revealed by brain hernia

Astrocytoma development following complete multiple myeloma remission in a 49-year-old patient: A case report

Multiple myeloma (MM), one of the B-cell non-Hodgkin lymphomas, is a bone marrow-derived, antibody-producing cancer of the plasma cells. In the advanced stages, the cancer cells frequently cause widespread osteolytic bone damage; however, in rare cases, MM also manifests as an intracranial plasmacytoma. In the present study, we describe a case in which a patient, initially treated for MM and with subsequent complete remission, was admitted to hospital with a lesion in the right cerebellar hemisphere and neurological symptoms of a brain tumor. Our initial diagnosis was an MM relapse with the rare occurring intracranial manifestation. However, pathological tests confirmed the diagnosis of a high-grade astrocytoma. In this case report, we describe the characteristics, as well as the treatment issues, diagnoses and clinical developments of this patient.