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Ophthalmic Plastic and Reconstructive... Mar 2024Orbital plasmacytoma is a rare plasma cell tumor that may arise as an aggressive form of extramedullary multiple myeloma. Treatment modalities include surgical excision,...
Orbital plasmacytoma is a rare plasma cell tumor that may arise as an aggressive form of extramedullary multiple myeloma. Treatment modalities include surgical excision, radiation, and chemotherapy. Chimeric antigen receptor T cell therapy is currently reserved for refractory disease. The authors present a case of a 69-year-old woman with an extensive orbital plasmacytoma refractory to multimodal therapy who was treated with idecabtagene vicleucel chimeric antigen receptor T cell therapy. Four days after infusion, the patient exhibited grade 1 cytokine release syndrome, which resolved with tocilizumab. The orbital plasmacytoma significantly decreased in size 1 month after treatment and demonstrated complete serological response and sustained tumor burden reduction at 10-month follow-up. This case highlights the efficacy of chimeric antigen receptor T cell therapy for refractory orbital plasmacytoma and calls attention to potential inflammatory toxicities.
PubMed: 38534054
DOI: 10.1097/IOP.0000000000002651 -
Case Reports in Ophthalmology 2024Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are...
INTRODUCTION
Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are presentations of MM. EMD is usually the sign of a more aggressive form of the disease. Herein, we report a patient with refractory MM presenting with extramedullary plasmacytoma in the superior oblique (SO) muscle.
CASE PRESENTATION
A 51-year-old female presented complaining of gradual protrusion of the left eye and ocular pain from 20 days prior. She received bone marrow transplantation 1 year prior and was on a chemotherapy regimen for MM for the past 1 year. Ocular examination revealed proptosis of the left eye and mild limitations of adduction and elevation. Orbital magnetic resonance imaging demonstrated remarkable enlargement of the left SO muscle with focal contrast enhancement. The patient underwent a biopsy and mass debulking. The histopathologic exam revealed fibromuscular tissue containing a neoplasm composed of sheets of plasmacytoid cells in a varying degree of differentiation with intervening scantly vascularized stromal components. The plasmacytoid cells were diffusely positive for a cluster of differentiation 138 (CD138), leading to a diagnosis of EMD involving the EOM and soft tissue of the orbit. The patient underwent palliative radiotherapy and a systemic workup. The PET-CT scan revealed involvement of the pelvic bone and left calf. Accordingly, the chemotherapy regimen was upgraded to reflect the aggressive nature of the disease. In the last follow-up, there was no sign of tumor reactivation in the orbital soft tissues. Unfortunately, the patient succumbed to her illness 7 months following her most recent presentation.
CONCLUSION
Early recognition of disease recurrence is lifesaving in MM patients; ophthalmic manifestations should be seriously considered as a sign of MM activity.
PubMed: 38529002
DOI: 10.1159/000538120 -
Zhonghua Xue Ye Xue Za Zhi = Zhonghua... Jan 2024Extramedullary plasmacytoma (EMP) is a special type of malignant plasmacytosis, which is complex and heterogeneous. Most EMP patients have poor prognosis and lack a...
Extramedullary plasmacytoma (EMP) is a special type of malignant plasmacytosis, which is complex and heterogeneous. Most EMP patients have poor prognosis and lack a stratified prognostic system or ideal treatment strategy supported by evidence-based medical evidence, which cannot meet clinical needs. In order to improve the understanding of this disease entity, Plasma Cell Disease Group, Chinese Society of Hematology, Chinese Medical Association and Chinese Myeloma Committee-Chinese Hematology Association developed the "Chinese Expert Consensus on the diagnosis and treatment of extramedullary plasmacytoma", which aims to standardize the clinical diagnosis and treatment of EMP and ultimately improve the overall survival of patients with plasmacytoma.
Topics: Humans; Plasmacytoma; Consensus; Multiple Myeloma; Prognosis; Paraproteinemias
PubMed: 38527832
DOI: 10.3760/cma.j.cn121090-20231107-00253 -
Brain & Spine 2024Intracranial multiple myeloma (MM) is a rare manifestation of MM, a malignant plasma cell disorder that primarily affects bone marrow. Dural involvement in MM is even...
INTRODUCTION
Intracranial multiple myeloma (MM) is a rare manifestation of MM, a malignant plasma cell disorder that primarily affects bone marrow. Dural involvement in MM is even rarer and can manifest as a dural mass. We present a case of MM presenting as an intracranial dural tumor with primary hemi-dural involvement.
RESEARCH QUESTION
This case report aims to investigate the clinical presentation, diagnostic challenges, and treatment approaches for intracranial multiple myeloma, with a focus on the extensive hemi-dural thickening and enhancement seen in this case.
MATERIAL AND METHODS
A 73-year-old male presented with progressive dysphasia and weakness. MRI revealed a solid left frontal mass with significant mass-effect. Hemi-dural thickening and enhancement was present along with invasion of the skull. The patient underwent surgical resection of the tumor with dural and bone reconstruction.
RESULTS
Histopathological examination confirmed MM diagnosis. Chemotherapy was started. Follow-up MRI showed complete tumor resection, but extensive hemi-dural thickening and enhancement persisted. Postoperative radiation therapy was considered.
DISCUSSION AND CONCLUSION
MM with primary dural involvement is rare and poses diagnostic challenges. Postoperative treatment involves chemotherapy, the role of surgery and radiotherapy is not established. The extensive hemi-dural thickening and enhancement observed in this case require further investigation, and a wait-and-scan policy was recommended instead of radiotherapy.
PubMed: 38510597
DOI: 10.1016/j.bas.2023.102730 -
Handbook of Clinical Neurology 2024Peripheral neuropathy is a common referral for patients to the neurologic clinics. Paraneoplastic neuropathies account for a small but high morbidity and mortality... (Review)
Review
Peripheral neuropathy is a common referral for patients to the neurologic clinics. Paraneoplastic neuropathies account for a small but high morbidity and mortality subgroup. Symptoms include weakness, sensory loss, sweating irregularity, blood pressure instability, severe constipation, and neuropathic pain. Neuropathy is the first presenting symptom of malignancy among many patients. The molecular and cellular oncogenic immune targets reside within cell bodies, axons, cytoplasms, or surface membranes of neural tissues. A more favorable immune treatment outcome occurs in those where the targets reside on the cell surface. Patients with antibodies binding cell surface antigens commonly have neural hyperexcitability with pain, cramps, fasciculations, and hyperhidrotic attacks (CASPR2, LGI1, and others). The antigenic targets are also commonly expressed in the central nervous system, with presenting symptoms being myelopathy, encephalopathy, and seizures with neuropathy, often masked. Pain and autonomic components typically relate to small nerve fiber involvement (nociceptive, adrenergic, enteric, and sudomotor), sometimes without nerve fiber loss but rather hyperexcitability. The specific antibodies discovered help direct cancer investigations. Among the primary axonal paraneoplastic neuropathies, pathognomonic clinical features do not exist, and testing for multiple antibodies simultaneously provides the best sensitivity in testing (AGNA1-SOX1; amphiphysin; ANNA-1-HU; ANNA-3-DACH1; CASPR2; CRMP5; LGI1; PCA2-MAP1B, and others). Performing confirmatory antibody testing using adjunct methods improves specificity. Antibody-mediated demyelinating paraneoplastic neuropathies are limited to MAG-IgM (IgM-MGUS, Waldenström's, and myeloma), with the others associated with cytokine elevations (VEGF, IL6) caused by osteosclerotic myeloma, plasmacytoma (POEMS), and rarely angiofollicular lymphoma (Castleman's). Paraneoplastic disorders have clinical overlap with other idiopathic antibody disorders, including IgG4 demyelinating nodopathies (NF155 and Contactin-1). This review summarizes the paraneoplastic neuropathies, including those with peripheral nerve hyperexcitability.
Topics: Humans; Paraneoplastic Polyneuropathy; Multiple Myeloma; Peripheral Nervous System Diseases; Isaacs Syndrome; Autoantibodies; Peripheral Nerves; Immunoglobulin M; Pain
PubMed: 38494281
DOI: 10.1016/B978-0-12-823912-4.00020-7 -
Surgical Neurology International 2024Parasellar plasmacytomas are rare neurosurgical entities. Intrinsic characteristics of these tumors, such as adjacent bone erosion and symptoms resulting from invasion...
BACKGROUND
Parasellar plasmacytomas are rare neurosurgical entities. Intrinsic characteristics of these tumors, such as adjacent bone erosion and symptoms resulting from invasion and mass effect, may lead to the possibility of a solitary extramedullary plasmacytoma (SEP) as a differential diagnosis.
CASE DESCRIPTION
We present the case of a 39-year-old male with a 1-month history of bilateral decreased visual acuity, retroocular pulsating pain, and chromatic vision loss. A computed tomography scan of the head revealed a parasellar lesion causing chiasmatic compression, as well as clival, orbital, sphenoidal, and ethmoidal invasion. A combined transorbital and endonasal endoscopic approach was found suitable, and gross total resection was achieved. Histological analysis of the lesion established the diagnosis of a SEP. After radiotherapy, a new magnetic resonance imaging was performed, revealing a recurrence of the lesion with a high grade of invasion. The patient was treated with palliative radiotherapy, as surgical resection did not seem feasible.
CONCLUSION
Surgical resection and radiotherapy may achieve remission of these lesions; however, recurrence rates remain high despite any treatment modality. Patients with this condition must be followed up with a multidisciplinary team due to the high risk of multiple myeloma progression.
PubMed: 38468666
DOI: 10.25259/SNI_915_2023 -
Cureus Feb 2024Orbital plasmacytomas are exceedingly rare neoplasms characterized by the proliferation of monoclonal plasma cells in the orbital soft tissues. Their presentation and...
Orbital plasmacytomas are exceedingly rare neoplasms characterized by the proliferation of monoclonal plasma cells in the orbital soft tissues. Their presentation and clinical course can be diverse, making early diagnosis and management challenging. This case report sheds light on one such instance, emphasizing the diagnostic and therapeutic aspects of this uncommon condition. We present an 81-year-old patient with a prior diagnosis of multiple myeloma and bilateral orbital plasmacytomas, highlighting the importance of imaging in diagnosis and management.
PubMed: 38465072
DOI: 10.7759/cureus.53823 -
Cancer Medicine Feb 2024Intracranial plasmacytomas are rare tumors arising from plasma cells with approximately half of the cases progressing to multiple myeloma (MM). However, there is a lack...
BACKGROUND
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.
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.
Topics: Humans; Middle Aged; Plasmacytoma; Retrospective Studies; Ki-67 Antigen; Multiple Myeloma; Bone Neoplasms; Disease Progression
PubMed: 38457205
DOI: 10.1002/cam4.7017 -
Cureus Feb 2024Solitary plasmacytoma of the breast is an extremely rare neoplastic entity characterized by the localized proliferation of neoplastic plasma cells within the breast...
Solitary plasmacytoma of the breast is an extremely rare neoplastic entity characterized by the localized proliferation of neoplastic plasma cells within the breast tissue that requires careful consideration due to its clinical and radiological resemblance to more common breast malignancies. While plasmacytomas are typically associated with multiple myeloma (MM), primary involvement of the breast as a solitary lesion is exceptionally rare. In this report, we present a case of solitary plasmacytoma of the breast in a 55-year-old female patient who presented with a palpable breast mass and no signs of systemic multiple myeloma. Our objective is to discuss the clinical presentation, radiological features, and histopathological findings and highlight the importance of comprehensive diagnostic workup and management planning for solitary plasmacytomas of the breast.
PubMed: 38449940
DOI: 10.7759/cureus.53612 -
Case Reports in Oncology 2024Non-secretory multiple myeloma (NSMM) is a rare form of multiple myeloma (MM) that is often difficult to detect and has not yet been well characterized. This is due to...
INTRODUCTION
Non-secretory multiple myeloma (NSMM) is a rare form of multiple myeloma (MM) that is often difficult to detect and has not yet been well characterized. This is due to the lack of production or the presence of monoclonal protein (MP) levels below levels detectable by testing such as serum/urine electrophoresis and immunofixation.
CASE PRESENTATION
Two patients of ours were being treated for MM with typical courses of systemic therapy. By the third-line therapy, both developed an extramedullary mass, one in the pelvis and the other in the neck. In both cases, blood work showed no measurable MP, normal free light chain levels, and unremarkable skeletal surveys. Secondary malignancies were suspected due to the clinical presentation in each case, and biopsies confirmed the presence of non-secretory plasmacytomas. Both patients were only treated with localized radiotherapy with a total dose of 2,000 cGy in 5 fractions over 1 week. Ultimately, this resolved the original masses with no residual tumors. No changes had to be made to their systemic therapies, and both patients remained stable.
CONCLUSION
NSMM relapse is not unusual and should be suspected in patients with relapsed refractory disease. Relapse should be confirmed by a tissue biopsy, and secondary malignancies should be ruled out. Radiotherapy is an excellent option to treat localized relapse and preserve the current line of systemic anti-myeloma therapy.
PubMed: 38444889
DOI: 10.1159/000536675