Isolated Abducens Nerve Palsy: A Rare Manifestation of Recurrent Extramedullary Myeloma.

Multiple myeloma is a plasma cell neoplasm, which may present as a solitary plasmacytoma and, uncommonly, as an extramedullary plasmacytoma. Intracranial plasmacytomas may manifest in central nervous system involvement as cranial nerve palsies. Cranial nerve six palsy is the most common in cases of malignancy. However, isolated abducens palsy presenting as multiple myeloma recurrence is very uncommon. Here, we detail two cases in which intracranial plasmacytoma lesions were present within the region of the Dorello canal, resulting in acute isolated unilateral diplopia from disease recurrence in the absence of systemic marrow involvement.

IgA multiple myeloma-associated mediastinal spinal plasmacytoma presenting as spinal cord compression.

Multiple myeloma associated with extramedullary plasmacytoma at initial presentation is rare. We describe a case of a man in his 30s who initially presented with symptoms of spinal cord compression. Further imaging revealed a mediastinal tumour, with a biopsy confirming plasmacytoma. Immunofixation revealed IgA lambda paraprotein. Bone marrow biopsy demonstrated atypical T-cell cytotoxic proliferation and trilineage hypoplasia. The patient was diagnosed with extramedullary plasmacytoma with active IgA multiple myeloma. The patient received mediastinal radiation to the tumour, followed by anti-myeloma therapy. This diagnosis is critical as managing a solitary plasmacytoma drastically differs from an extramedullary plasmacytoma with active multiple myeloma.

Plasmacytoma and Amyloid Lymphadenopathy, FDG, and Fluorocholine.

ABSTRACT: A 56-year-old man with thoracal mass suspected of solitary plasmacytoma was referred for 18 F-FDG PET-CT scan. His PET-CT revealed FDG-avid rib mass and cervical lesion at level 2. He also underwent 18 F-fluorocholine (FCH) PET-CT to evaluate possible metastatic spread of the disease. FCH PET-CT showed increased uptake at the rib mass, while the cervical lesion was not FCH-avid. Biopsies confirmed rib lesion was a solitary plasmacytoma; however, the cervical lesion was an amyloid deposited lymph node. This case showed FCH PET-CT is a valuable companion of FDG scan for the evaluation of plasma cell dyscrasias with a better specificity.

18 F-FDG PET/CT in Solitary Extramedullary Plasmacytoma of the Penis.

ABSTRACT: A 72-year-old man presented with a painless penile mass for 3 months. Contrast-enhanced CT revealed heterogeneous enhancement, whereas 18 F-FDG PET/CT displayed inhomogeneous 18 F-FDG accumulation in the lesion without other abnormal activity. The histopathological examination from biopsied specimen confirmed the diagnosis of a plasmacytoma. However, the subsequent tests, including serum/urine immunofixation electrophoresis, serum/urine free light chain assay, and bone marrow smear/biopsy, did not show any abnormalities. The conclusive diagnosis was a solitary extramedullary plasmacytoma of the penis.

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

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.

Extramedullary plasmacytoma of the larynx - Case report.

Extramedullary plasmacytoma of the larynx is an extremely rare entity accounting for 0.04-0.45% of malignant tumours of the larynx. The objective of this clinical case report is to highlight the diagnosis and management of a unique case such as this. A 77-year-old gentleman presented with complaints of hoarseness for 1 year. Computed tomography image revealed a soft tissue mass lesion involving the right true vocal cord. Direct laryngoscopic biopsy was performed and subjected to histopathological examination, which showed collection of plasma cells. Immunohistochemistry confirmed the presence of Kappa and Lambda cells. Multiple myeloma (MM) was ruled out. The patient received radical intent radiation therapy using 3DCRT technique with a dose of 50Gy in 25# over 5 weeks. He experienced improvement in hoarseness on subsequent follow-up visits. At 1-year follow up, positron emission tomography computed tomography showed near total resolution of disease with no progression to MM. Radiation therapy alone is known to achieve good local control, recurrence free survival, and organ preservation in such cases.

Recurrence of solitary plasmacytoma in the liver 10 years after the onset of multiple bone lesions.

A 79-year-old man presented with a history of solitary plasmacytoma in the bone 10 years ago. Chemoradiotherapy was effective, and remission was maintained with intermittent treatment at relapse of the bone lesions. One year after the last treatment, a follow-up computed tomography (CT) scan revealed multiple liver masses, and a liver biopsy revealed plasmacytoma. There was no clonal plasma cell infiltration in the bone marrow, and the final diagnosis was solitary plasmacytomas of the liver. Although liver involvement is known in relapsed refractory multiple myeloma, solitary plasmacytoma in the relapsed stage confined to the liver is rare, and all previous reports have been from the initial presentation. To the best of our knowledge, this is the first recurrent case of solitary plasmacytoma of the liver.

Case with translating to multiple myeloma at 8 years after radiation therapy for extramedullary plasmacytoma of the larynx.

We report a case of extramedullary plasmacytoma of the larynx that was eradicated by radiotherapy; however, 8 years later, the disease had progressed to multiple myeloma. A mid -60s Japanese woman presented with a right-sided arytenoid mass in the larynx who underwent biopsy at another hospital. Based on the biopsy results, the patient was diagnosed with extramedullary plasma cell tumour and was referred to the Department of Otorhinolaryngology at our hospital. She received radiotherapy (50.4 Gy) and the laryngeal tumour was eradicated. Positron emission tomography/CT (PET-CT) revealed no abnormal accumulation in the larynx or whole body. After radiotherapy, the department of otorhinolaryngology, in consultation with Ddepartment of haematology performed follow-ups using laryngoscope, blood examination and PET-CT. Five years after the end of radiotherapy, the patient had no local recurrence or transition to multiple myeloma. However, 8 years later, blood examination and PET-CT revealed multiple myeloma. Laryngoscopy did not reveal any recurrent laryngeal tumour. Therefore, chemotherapy for multiple myeloma was administered at the department of haematology. Three months after the initiation of chemotherapy, the accumulation had disappeared in PET-CT. Three years have passed since chemotherapy initiation. At present, no recurrence or metastasis was observed in the larynx or whole body.

Solitary bone plasmacytoma of spine with involvement of intervertebral disk: a case report and literature review.

Primary malignant bone tumors of the spine are exceedingly rare, with solitary bone plasmacytoma (SBP) representing approximately 30% of all cases. Radiological assessments are crucial for localizing SBP and for ruling out a diagnosis of multiple myeloma (MM). Imaging features resembling a "mini-brain" appear to be distinctive for SBP. Vertebral lesions accompanied by adjacent disc space involvement typically suggest spinal infections, while the potential for SBP involvement is often overlooked. We present a case of a 61-year-old female with SBP who exhibited thoraco-lumbar spine destruction and adjacent disc space involvement. The patient sought treatment at our medical center due to lumbodorsal pain radiating bilaterally to the inguinal regions. Radiological findings revealed an osteolytic lesion involving the intervertebral disc, making it challenging to distinguish between tumor and inflammation. A biopsy of the vertebral lesion confirmed the diagnosis of SBP, which was further supported by laboratory results. Post-diagnosis, the patient underwent radiotherapy, receiving a total dose of 4000 Gy, which alleviated her symptoms. We also provide a comprehensive literature review on SBP with disc involvement to aid both clinical and radiological diagnoses.

18 F-FAPI-04 PET/CT in a Case of Primary Gastric Plasmacytoma.

ABSTRACT: Primary plasmacytoma of the stomach is very rare. We describe contrast-enhanced CT and 18 F-FAPI-04 PET/CT findings in a patient with primary gastric plasmacytoma and Helicobacter pylori infection. The gastric tumor appeared as a large hypervascular polypoid mass on contrast-enhanced CT and showed intense 18 F-FAPI-04 uptake with SUV max of 11.8 on 18 F-FAPI-04 PET/CT.

Extramedullary Plasmacytoma of the Retroperitoneum Detected on 18 F-FDG PET/CT.

ABSTRACT: Solitary retroperitoneal extramedullary plasmacytoma is a rare neoplasm. A 67-year-old man presented with abdominal pain and left leg weakness. Abdominal CT revealed a large mass in the left retroperitoneum, which demonstrated increased metabolic activity on subsequent 18 F-FDG PET/CT imaging. Furthermore, the patient's serum protein electrophoresis showed positive M-protein results. Pathological examination of the biopsied specimen confirmed the diagnosis of extramedullary plasmacytoma.

Extramedullary plasmacytoma of the orbit with intracranial invasion: A case report.

RATIONALE: Plasmacytoma is a disease caused by the proliferation of monoclonal immunoglobulin-producing plasma cells and divided into multiple myeloma, solitary bone plasmacytoma, and extramedullary plasmacytoma. We report a case of orbital extramedullary plasmacytoma invading the dura mater in a patient with exophthalmos and diplopia. PATIENT CONCERNS: A 35-year-old female patient with exophthalmos in the right eye and diplopia visited the clinic. DIAGNOSES: The thyroid function tests showed nonspecific results. Orbital computed tomography and magnetic resonance imaging revealed a homogeneously enhancing orbital mass infiltrating the right maxillary sinus and adjacent brain tissue in the middle cranial fossa through the superior orbital fissure. INTERVENTIONS: To diagnose and alleviate the symptoms, an excisional biopsy was performed, which revealed a plasmacytoma. OUTCOMES: One month after the surgery, the protruding symptoms and eye movement restriction in the right eye improved, and the visual acuity in the right eye was restored. LESSONS: In this case report, we present a case of an extramedullary plasmacytoma that originated in the inferior wall of the orbit and invaded the cranial cavity. To our knowledge, no previous reports have described a solitary plasmacytoma that originated in the orbit, causing exophthalmos and invading the cranial cavity at the same time.

CT and MRI Findings of Solitary Extramedullary Plasmacytoma: A Series of Ten Case Reports and Review of Literature.

OBJECTIVE: The study aimed to analyze computed tomography and magnetic resonance imaging features of patients with solitary extramedullary plasmacytoma. METHODS: Ten cases with solitary extramedullary plasmacytoma were included in this study. CT and MRI features of solitary extramedullary plasmacytoma were retrospectively analyzed. RESULTS: This study included both males (n = 8) and females (n = 2), with a median age of 48 (range 21-72 years old). The organs or anatomical structures involved were nasopharynx (n = 3), orbit (n = 1), left tentorium (n = 1), nasal cavity and meatus (n = 2), small intestine and mesentery (n = 1), as well as posterior thoracic wall (n = 2). The median maximum diameter of the tumors was 3.2 cm (range 0.8- 15.2 cm). The tumor shapes were round (n = 7), stripped (n = 1), irregular (n = 1), and nodular (n = 1). The margin of the tumors was well-defined (n = 7) and partially well-defined (n = 3). There were 3 cases with bone destruction and 1 with a tissue invasion adjacent to the tumor, calcification (n = 1), and cystic degeneration (n = 2). Enlarged tumoral vessels (n = 4) could also be observed. The CT attenuation and MR signal intensity of tumors were heterogeneous (n = 4) and homogenous (n = 6). After the injection of the contrast agent, marked (n = 5), mild (n = 1), mild to moderate (n = 1), and delayed enhancement (n = 1) could be observed. CONCLUSION: A well-defined homogeneous solitary mass occurring at the head and neck with a marked enhancement, an adjacent tissue invasion, enlarged tumoral vessels, and bone destruction can indicate the diagnosis of solitary extramedullary plasmacytoma.