Solitary Intracranial Plasmacytoma Research Articles

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.

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)

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.

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.

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.

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

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.

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.

Solitary plasmacytoma with intracranial intraorbital and, paranasal sinus extension

Intracranial solitary plasmacytomas (ICSPs) are extremely rare tumours in neurosurgical practice, and are often misdiagnosed preoperatively. Here we present a solitary intracranial plasmacytoma with orbital, nasal and paranasal sinus extension. A subtotal excision of the tumour was performed and the complete response was seen after postoperative radio-chemotherapy. The neuroradiological and neurosurgical features of the case are discussed with the pertinent literature.

Las características clínico­imaginológicas clásicas de los meningiomas son imitadas por otras lesiones expansivas

To analyze the surgical neurology cases in which both the clinical and neuroimaging features suggested supratentorial meningioma, and resulted in different entities from the neuropathological point of view. The clinical histories of patients harboring intracranial, supratentorial mass lesions diagnosed as meningioma operated in different hospitals of Maracaibo, Venezuela, during the period 1993-1997 were reviewed. Our analysis revealed 15 cases with different neuropathological diagnosis. They were distributed as follows: three cases of hemangiopericytoma, two cases each of anaplastic ependymoma, metastatic carcinoma, and solitary intracranial plasmacytoma, and one case each of plasma cell granuloma, pleomorphic xanthoastrocytoma, chondroma, actinomycetoma, meningeal fibroma and chronic inflammation not otherwise specified. A heterogeneous group of intracranial, supratentorial expansive mass lesions can masquerade as meningiomas both from the clinical and neuroimaging points of view.

Solitary intracranial plasmacytoma

Solitary intracranial plasmacytomas (SIPs) are rare tumours that may be mistaken radiologically for meningiomas. According to the literature, the predominant feature on polytomography and computed tomography (CT) is a bone-eroding, contrast-enhancing soft tissue mass, part of which might have extended extracranially before initial presentation. The lesions can be solitary and are therefore amenable to surgical excision. Although no recurrence is seen for up to 10 years after treatment, some authors believe progression to multiple myeloma is inevitable. Lesions that are extramedullary in origin are thought to have the best prognosis. We describe three cases treated surgically at Westmead Hospital in the past 6 years. All had CT features similar to those reported previously. On magnetic resonance imaging, which in this condition has not been fully documented, the lesions were slightly hyperintense with the brain on T1-weighted sequences, and were inhomogeneous and hyperintense with T2-weighted images. The two patients who received intravenous contrast showed dense and uniform enhancement. Occasional intralesional signal voids suggested increased vascularity, which was confirmed surgically. The presence of intracellular immunoglobulins may account for the hyperintensity on T2 sequence. Although no specific imaging features were identified to distinguish meningiomas from SIP, the latter should be included in the differential diagnosis of solitary bone eroding intracranial tumours.

Management of intracranial plasmacytoma.

The authors report on a study of eight cases of intracranial plasmacytoma to identify the risk of progression to multiple myeloma and suggest the treatment required for cure of solitary lesions. The diagnosis of multiple myeloma or myelomatous changes was made in the immediate postoperative period in four patients (50%), two of whom had skull base lesions. Of the four remaining patients, three were treated with complete surgical resection and radiation therapy and had no recurrence of plasmacytoma or progression to multiple myeloma during mean follow up of 12 years (range 2-25 years); one patient underwent subtotal surgical resection and had recurrence of the tumor despite radiation therapy. It is concluded that multiple myeloma is unlikely to develop during the long term in patients with intracranial plasmacytoma who do not develop multiple myeloma or myelomatous changes in the early postoperative period. However, lesions that infiltrate the skull base are not likely to be solitary, and patients who harbor these neoplasms should undergo complete evaluation and close follow-up review to exclude multiple myeloma. A recurrence of solitary intracranial plasmacytoma is possible with subtotal surgical resection despite radiation therapy. Definitive treatment should consist of complete surgical resection with adjuvant radiation therapy.

Solitary intracranial plasmacytoma presenting as lateral rectus palsy: Report of a case and review of neuro-ophthalmic manifestations of plasmacytoma

A 65-year-old man presented with an isolated lateral rectus palsy. Neuroradiological studies were negative initially. The abduction deficit subsequently became bilateral and reinvestigation showed a mass centred on the pituitary fossa. Trans-sphenoidal biopsy revealed a plasmacytoma. This presentation of plasmacytoma has not previously been reported. The case also emphasizes the importance of re-investigation of a persistent abduction deficit.

Multiple Myeloma presenting as a Mass Lesion in the Frontal Bone

The patient, a 51-year-old male, was hospitalized for evaluation of a frontal subcutaneous mass. There were no signs of intracranial hypertension and no neurological deficit. His general medical status was normal, but he had been diagnosed as having immunoglobulin G (IgG) lambda type multiple myeloma by bone marrow aspiration and immunoelectrophoresis. A plain skull X-ray showed a large bony defect and multiple punched-out lesions in the frontal bone. Computed tomography showed an epidural high-density mass with marked homogeneous enhancement. Carotid angiography showed a hypervascular tumor fed by the bilateral external carotid arteries. At surgery, a reddish tumor was detached from the dura mater and totally resected. The pathological diagnosis was IgG lambda type plasmacytoma. After 40-Gy radiation and chemotherapy, the postoperative course over the following year was uneventful. Although the prognosis of multiple myeloma is poor, that of solitary intracranial plasmacytoma is reportedly good. However, since there is a high incidence of transition from solitary plasmacytoma to multiple myeloma, patients with intracranial plasmacytoma should be followed for a long period.

Foster-Kennedy syndrome caused by solitary intracranial plasmacytoma

The first case of typical Foster-Kennedy syndrome, including decreased olfaction of the side of the optic atrophy due to a primary solitary intracranial extraskeletal plasmacytoma, is reported. Its appearance on a computed tomography scan was compatible with a meningioma or subdural hematoma. The patient demonstrated a chronic, corticosteroid-sensitive, relapsing optic neuropathy associated with an elevated sedimentation rate and a monoclonal gammopathy. The diagnosis, treatment, and prognosis of such tumors are reviewed.

A steroid-sensitive solitary intracranial plasmacytoma.

A patient with a solitary intracranial plasmacytoma had involvement of the meninges, but not bone. To our knowledge, it represents the tenth reported case of this type. Two of the previously reported cases involved primarily brain substance, while the rest, including this case, involved primarily the meninges. This lesion closely simulated optic neuritis in this initial clinical presentation and then became a steroid-responsive lesion. A discrete band in the gamma region of the agar gel electrophoresis of the cerebrospinal fluid (CSF) was found in the face of a normal, standard CSF and serum electrophoresis, which disappeared after surgery and therapy with radiation. To our knowledge, this has not been reported previously, although an abnormal standard spinal fluid electrophoresis with an increase in the γ-globulin was reported in several of the previous cases. The changing pathological findings in our case are interesting in light of the possible origin of the plasmacytoma.

Solitary plasmacytoma in the cranial cavity. Case report.

S OLITARY plasmacytoma in the cranial cavity is a rare lesion. As far as we could ascertain, only five cases of solitary intracranial plasmacytoma have been reported in the literature. Several characteristics distinguish this solitary tumor from other plasmacytomas in the cranial cavity. It is not associated with adjacent lesions in the skull or with tumors of other sites. After complete removal of the tumor by surgery or irradiation, or a combination of both, patients remain free of symptoms. This case report concerns a patient with an unusually large tumor, which was treated by surgery and irradiation therapy. The patient still has no evidence of recurrent disease approximately 3 years after treatment.

Solitary intracranial plasmacytoma.

SOLITARY intracranial plasmacytoma is uncommon. For this reason and because it may respond favorably to surgery and radiation therapy, the following case was considered of special interest. Report of Case First Admission (Nov 17, 1961).— A 44-year-old woman was admitted to the Psychiatry Service at the Charity Hospital of New Orleans. The admitting psychiatric diagnosis was reactive depression, but after consultation she was transferred to the neurosurgical service. Her sister said that the patient had had headaches, memory loss, and progressive mental depression since February 1961. The patient attributed the headaches to a blow delivered by a falling board which had struck her forehead at the hairline. She was stunned but did not lose consciousness. The blow produced a lump at the site of injury which had decreased in size, but was still present at the time of admission. The first of five seizures occurred one month before admission.