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Der Internist Jan 2019Skeletal lesions caused by multiple myeloma often lead to pain, pathological fractures, spinal instability, and compression of the spinal cord and nerve roots. The... (Review)
Review
Skeletal lesions caused by multiple myeloma often lead to pain, pathological fractures, spinal instability, and compression of the spinal cord and nerve roots. The choice of therapy options is made by an interdisciplinary basis with oncologists and radiotherapists on the basis of comorbidities, prognosis, localization, and number of lesions. Surgical management has a supportive role in pain relief, the prevention and treatment of pathological fractures, and the decompression of the spinal cord and nerve roots. An adequate preoperative workup is essential for planning surgical treatment, which includes magnetic resonance imaging of the spine. In the case of unstable spinal lesions, minimally invasive and open surgical procedures, as well as their combination, are available for stabilization and decompression. In impending and pathological fractures of the extremities, surgical procedures are superior to conservative therapy for pain relief, restoring stability, walking ability, and limb function. There are multiple options available, including osteosynthesis using bone cement and plates, intramedullary nails, and bone replacement with implants and tumor endoprostheses with good functional results. Permanent reconstruction of the lesions should be the goal of any surgical intervention. The indication for curative, wide resection of the tumor should be considered for solitary plasmocytomas of the bone. Adjuvant radiotherapy leads to significantly improved local tumor control and should be considered after resection and stabilization.
Topics: Bone Neoplasms; Decompression, Surgical; Humans; Magnetic Resonance Imaging; Multiple Myeloma; Neoplasm Metastasis; Plasmacytoma; Radiotherapy, Adjuvant; Spinal Neoplasms
PubMed: 30560368
DOI: 10.1007/s00108-018-0530-2 -
European Journal of Nuclear Medicine... Jan 2018Solitary plasmacytoma (SP) is a rare plasma-cell neoplasm, which can develop both in skeletal and/or soft tissue and frequently progresses to multiple myeloma (MM). Our...
PURPOSE
Solitary plasmacytoma (SP) is a rare plasma-cell neoplasm, which can develop both in skeletal and/or soft tissue and frequently progresses to multiple myeloma (MM). Our aim was to study the metabolic behavior of SP and the role of 18F-FDG-PET/CT in predicting progression to MM.
MATERIALS AND METHODS
Sixty-two patients with SP who underwent 18F-FDG-PET/CT before any treatment were included. PET images were qualitatively and semiquantitatively analyzed by measuring the maximum standardized uptake value body weight (SUVbw), lean body mass (SUVlbm), body surface area (SUVbsa), metabolic tumor volume (MTV), total lesion glycolysis (TLG) and compared with age, sex, site of primary disease, and tumor size.
RESULTS
Fifty-one patients had positive 18F-FDG-PET/CT (average SUVbw was 8.3 ± 4.7; SUVlbm 5.8 ± 2.6; SUVbsa 2 ± 1; MTV 45.4 ± 37; TLG 227 ± 114); the remaining 11 were not 18F-FDG-avid. Tumor size was significantly higher in patients avid lesions compared to FDG not avid; no other features are associated with FDG-avidity. Progression to MM occurred in 29 patients with an average of 18.3 months; MM was more likely to develop in patients with bone plasmacytoma and in patients with 18F-FDG avid lesion. Time to transformation in MM (TTMM) was significantly shorter in patients with osseous SP, in 18F-FDG avid lesion, for SUVlbm > 5.2 and SUVbsa > 1.7.
CONCLUSIONS
18F-FDG pathological uptake in SP occurred in most cases, being independently associated with tumor size. PET/CT seemed to be correlated to a higher risk of transformation in MM, in particular for 18F-FDG avid plasmacytoma and SBP. Among semiquantitative features, SUVlbm > 5.2 and SUVbsa > 1.7 were significantly correlated with TTMM.
Topics: Adult; Aged; Aged, 80 and over; Bone Neoplasms; Female; Fluorodeoxyglucose F18; Humans; Male; Middle Aged; Plasmacytoma; Positron Emission Tomography Computed Tomography; Predictive Value of Tests; Radiopharmaceuticals; Soft Tissue Neoplasms
PubMed: 28822997
DOI: 10.1007/s00259-017-3810-5 -
Turkish Journal of Haematology :... Feb 2022
Topics: Humans; Liver; Multiple Myeloma; Plasmacytoma
PubMed: 34753278
DOI: 10.4274/tjh.galenos.2021.2021.0545 -
Expert Review of Hematology Apr 2022Extramedullary myeloma has a more aggressive clinical course and inferior prognosis. Two types of extramedullary myeloma, paraosseous (PO) and extraosseous (EO), differ... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Extramedullary myeloma has a more aggressive clinical course and inferior prognosis. Two types of extramedullary myeloma, paraosseous (PO) and extraosseous (EO), differ significantly in pathogenesis, clinical features, and outcomes, but there have been limited studies comparing them based on large patient cohorts.
METHODS
Relevant studies published before June 2021 were searched in MEDLINE, Embase, and Cochrane databases. Two reviewers independently assessed the quality of included studies, and discrepancies were evaluated by a third reviewer. Extracted data were analyzed by Stata 12.
RESULTS
Nine hundred ninety-seven MM patients with PO and 288 with EO plasmacytoma were analyzed. Patients with EO plasmacytoma tended to have larger tumor burden according to the International Staging System though it did not reach statistical significance (P = 0.1). Newly diagnosed MM (NDMM) patients with EO plasmacytoma had significantly inferior outcome than those with PO plasmacytoma (OS: OR = 2.32, 95% CI 1.80-2.98, P < 0.001, I = 40.9%; PFS: OR = 1.66, 95% CI 1.39-1.99, P < 0.001, I = 0.0%). No significant difference in OS was found in relapsed MM patients. Auto-HSCT seemed to attenuate the disadvantage in response rate of EO plasmacytoma.
CONCLUSIONS
NDMM patients with EO plasmacytoma had significantly inferior prognosis than those with PO plasmacytoma, while relapsed MM patients did not show such difference. More strategies are needed to improve its clinical outcome.
Topics: Humans; Immunotherapy; Multiple Myeloma; Plasmacytoma; Prognosis
PubMed: 35387532
DOI: 10.1080/17474086.2022.2063117 -
Journal of Postgraduate Medicine 2018Primary tracheal tumors are rare and trachea is an exceedingly rare site of extramedullary plasmacytoma (EMP). We report a case of solitary tracheal plasmacytoma causing...
Primary tracheal tumors are rare and trachea is an exceedingly rare site of extramedullary plasmacytoma (EMP). We report a case of solitary tracheal plasmacytoma causing symptoms of airway obstruction in a 59-year-old man. Flow/volume loop indicated the fixed central airway obstruction. Computerized tomography and bronchoscopy demonstrated a sessile tumor on posterior tracheal wall obstructing 80% of the lumen. Partial tracheal resection with T-T anastomosis was performed. Pathologic analysis of resected mass revealed EMP. Additional investigations excluded multiple myeloma. There are no signs of disease recurrence after 7-year follow-up.
Topics: Airway Obstruction; Anastomosis, Surgical; Bronchoscopy; Humans; Male; Middle Aged; Multidetector Computed Tomography; Plasmacytoma; Trachea; Tracheal Neoplasms; Treatment Outcome
PubMed: 29067926
DOI: 10.4103/jpgm.JPGM_739_16 -
Ophthalmic Plastic and Reconstructive...To report a rare case of crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma of the lacrimal sac and to review literature on the 2...
PURPOSE
To report a rare case of crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma of the lacrimal sac and to review literature on the 2 entities to summarize important diagnostic, management, and prognostic considerations.
METHODS
A case report of the ophthalmologic presentation, pathology workup, and oncologic management is presented. Literature search with focus on lesions occurring in ophthalmic sites and management guidelines from expert panels and working groups.
RESULTS
Crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma arose within the lacrimal sac of a previously healthy middle-aged woman and presented as a painless nodule with epiphora. The biopsy tissue showed sheets of crystal-filled histiocytes, interspersed with monoclonal plasma cells and rarely demonstrated plasma cell phagocytosis. Imaging and laboratory studies confirmed the localized nature.
CONCLUSIONS
Crystal-storing histiocytosis is an uncommon entity in which crystals, most commonly arising from altered immunoglobulins, aggregate within histiocytes and form symptomatic mass lesions. It has been reported in ophthalmic regions in patients with a concurrent lymphoproliferative or plasma cell disorder and can rarely predate a malignancy. The current case is notable because crystal-storing histiocytosis occurs with a localized process, solitary extramedullary plasmacytoma, and presents in an unusual site, the lacrimal sac. Tissue biopsy with multimodal pathological evaluation is necessary to make the diagnosis. Ophthalmologists should recognize that crystal-storing histiocytosis is commonly associated with a hematologic malignancy and, when appropriate, refer the patient for oncologic management. Surveillance may be indicated in cases with no established etiology. Solitary extramedullary plasmacytoma should also be monitored, as a proportion of cases progress to multiple myeloma.
Topics: Bone Neoplasms; Female; Histiocytes; Histiocytosis; Humans; Middle Aged; Nasolacrimal Duct; Plasma Cells; Plasmacytoma
PubMed: 34406151
DOI: 10.1097/IOP.0000000000002028 -
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke... Feb 2022To explore the clinical characteristics, treatment methods and outcomes of extramedullary plasmacytoma of the head and neck. A retrospective analysis was conducted on...
To explore the clinical characteristics, treatment methods and outcomes of extramedullary plasmacytoma of the head and neck. A retrospective analysis was conducted on 10 cases with extramedullary plasmacytoma of the head and neck who were admitted to Henan Tumor Hospital from January 2005 to January 2020. Among the 10 patients, 6 were male and 4 were female. The average age at diagnosis was 56.3 years old (34-74 years old). Among them, 3 cases were located in the nasal cavity, 2 cases in the nasopharynx, 1 case in the sinuses, 2 cases in the larynx, 1 case in the oropharynx, and 1 case in the cervical lymph nodes. Treatments were administered according to tumor size and resection extent. Complete surgical excision (negative margins) was preferred, followed by adjuvant radiotherapy or radiotherapy alone. The clinical characteristics, diagnosis, treatment and prognosis of EMP were analyzed. The patients' symptoms were not specific, frequently with local obstruction symptom and localized masses. All patients were confirmed pathologically as suffering from monoclonal plasmacytoma, with negative bone marrow biopsy and negative skeletal survey. Five patients received surgery, 3 received radiotherapy, and 2 received surgery with additional radiation. The follow-up time was 16-125 months, with a median of 92 months. Two patients developed into multiple myeloma. One patient who received radiotherapy after surgery relapsed after 7 years of follow-up and again received surgical treatment, with no evidence of second recurrence. The remaining patients had no recurrence or progression. Extramedullary plasmacytoma of the head and neck has a good prognosis. Surgical treatment can be considered for completely resectable lesions.
Topics: Adult; Aged; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Multiple Myeloma; Plasmacytoma; Prognosis; Retrospective Studies
PubMed: 35196764
DOI: 10.3760/cma.j.cn115330-20210424-00222 -
BMJ Case Reports Jun 2018Testicular plasmacytomas are rare, accounting for only 1.3% of all extramedullary plasmacytomas. The infrequency in which it is encountered, coupled with its...
Testicular plasmacytomas are rare, accounting for only 1.3% of all extramedullary plasmacytomas. The infrequency in which it is encountered, coupled with its non-specific clinical and sonographic presentation, makes its diagnosis a challenge. We present a case of a 70-year-old man with multiple myeloma, which was systemically responding to chemotherapy, who developed testicular swelling, erythema and pain. Ultrasound findings were concerning for infection, although urine and serum testing were unremarkable. The patient did not improve after several rounds of antibiotics prompting further evaluation. The patient underwent radical orchiectomy which revealed testicular plasmacytoma. Fluorescent in situ hybridisation (FISH) of the testicular tissue noted mutations which had not been present in the FISH analysis of bone marrow. Positron emission tomography scan later revealed new retroperitoneal plasmacytomas involving the new clone and his chemotherapy regimen needed to be adjusted for treatment.
Topics: Aged; Anti-Bacterial Agents; Antineoplastic Combined Chemotherapy Protocols; Diagnostic Errors; Humans; In Situ Hybridization, Fluorescence; Male; Multiple Myeloma; Neoplasms, Second Primary; Orchitis; Plasmacytoma; Positron Emission Tomography Computed Tomography; Retroperitoneal Neoplasms; Testicular Neoplasms; Ultrasonography; Ultrasonography, Doppler
PubMed: 29866662
DOI: 10.1136/bcr-2017-222046 -
Journal of Cancer Research and Clinical... Feb 2024Extramedullary plasmacytoma (EMP) is a rare plasma cell malignancy, especially when the tumor originates in skeletal muscle. Plasmablastic plasmacytoma is an anaplastic... (Review)
Review
BACKGROUND
Extramedullary plasmacytoma (EMP) is a rare plasma cell malignancy, especially when the tumor originates in skeletal muscle. Plasmablastic plasmacytoma is an anaplastic round cell tumor with highly malignancy and poor prognosis. To date, there have been no reports on the transformation of skeletal muscle EMP into plasmablastic plasmacytoma. Therefore, the diagnosis, treatment, and prognosis of cases of this pathologic transformation are unclear.
CASE PRESENTATION
This article reports a case of an elderly male patient who presented with a painless mass in the right calf and was diagnosed with EMP by puncture pathology. Complete remission was obtained after sequential chemoradiotherapy. 6 months later, another puncture was performed due to plasmablastic plasmacytoma multiple distant metastases, and the pathology showed that EMP was transformed to plasmablastic plasmacytoma. Despite aggressive antitumor therapy, the disease continued to deteriorate, and the patient ultimately died of respiratory failure.
CONCLUSION
The transformation of EMP into plasmablastic plasmacytoma is very rare, and its diagnosis and treatment require the participation of both experienced pathologists and clinicians. We report this case in order to raise clinicians' awareness of the diagnosis and treatment of EMP and its transformation to plasmablastic plasmacytoma, and to avoid misdiagnosis and underdiagnosis.
Topics: Aged; Male; Humans; Plasmacytoma; Chemoradiotherapy; Death; Muscle, Skeletal; Pathologic Complete Response
PubMed: 38300308
DOI: 10.1007/s00432-023-05604-2 -
Pituitary Jun 2017Parasellar plasmacytomas are rare tumors localized to the sellar region arising from plasma cells. Knowledge of clinical, imaging, surgical, and pathological... (Review)
Review
PURPOSE
Parasellar plasmacytomas are rare tumors localized to the sellar region arising from plasma cells. Knowledge of clinical, imaging, surgical, and pathological characteristics is limited to single case reports.
METHODS
A retrospective analysis of five primary cases was conducted, followed by systematic review of English language articles using PubMed in accordance with PRISMA guidelines.
RESULTS
Five primary case patients include four men and one woman, ages 60-77, followed up to 3 years. A systematic review identified 65 additional patients, of whom 65% presented with cranial nerve palsies and 15% with hypopituitarism. Sixteen percent had history of known multiple myeloma (MM) while 37% were diagnosed concurrently with MM on presentation of parasellar plasmacytoma. Imaging showed median tumor size of 38 mm (range, 4-70 mm), with MRI intensity similar to that of other sellar masses. Surgical biopsy with immunohistochemical studies confirmed plasmacytoma diagnosis. Eighty-one percent underwent parasellar radiotherapy, and chemotherapy initiated in 59% of the 69 patients with MM. Overall survival rate was 74% at follow-up (median 12 months), with 18% having parasellar recurrences and 38% progressing to systemic MM after presentation of a solitary plasmacytoma (median 3 months).
CONCLUSIONS
Parasellar plasmacytomas are rare tumors that should be considered in the differential diagnosis for lesions involving the sella and arising from the clivus, especially when cranial nerve paresis is apparent, even in the absence of known MM. Although recurrence rates for parasellar plasmacytoma is low, patients should be monitored for progression to MM. Treatment depends on the presence of systemic disease at diagnosis.
Topics: Aged; Female; Humans; Male; Multiple Myeloma; Plasmacytoma; Retrospective Studies
PubMed: 28251542
DOI: 10.1007/s11102-017-0799-5