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Radiologia 2014Extramedullary plasmacytomas are uncommon malignant plasma cell tumors that present outside the bone marrow; 80% of extramedullary plasmacytomas are located in the upper...
Extramedullary plasmacytomas are uncommon malignant plasma cell tumors that present outside the bone marrow; 80% of extramedullary plasmacytomas are located in the upper respiratory tract, and gastrointestinal plasmacytomas are rare. We present the case of an asymptomatic 65-year-old man in whom a pancreatic mass was found incidentally. The lesion was determined to be a pancreatic plasmacytoma after fine-needle aspiration cytology and surgical resection. No clinical, laboratory, or imaging findings indicative of multiple myeloma or association with other plasmacytomas were found, so the tumor was considered to be a primary pancreatic plasmacytoma.
Topics: Aged; Humans; Male; Pancreatic Neoplasms; Plasmacytoma
PubMed: 22738942
DOI: 10.1016/j.rx.2011.10.010 -
Ophthalmic Plastic and Reconstructive... 2018To evaluate the clinical and anatomical location of orbital plasmacytomas and assess local control following therapy.
PURPOSE
To evaluate the clinical and anatomical location of orbital plasmacytomas and assess local control following therapy.
METHODS
The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried to identify patients diagnosed with orbital plasmacytoma. These patients' records were reviewed for demographic characteristics, clinical and radiologic findings, treatments, and outcomes.
RESULTS
Thirty patients from 4 institutions (24 from MD Anderson Cancer Center, 3 from SUNY Downstate Medical Center, 2 from University of California, Los Angeles, and 1 from Oregon Health and Science University) were identified. Eighteen patients (60%) were diagnosed with multiple myeloma (MM) before and 11 (37%) were diagnosed with MM immediately after orbital plasmacytoma. Based on imaging, 4 distinct anatomical patterns were identified: 1) bony plasmacytoma affecting the superotemporal orbit, epidural space, and temporal fossa (15 patients; 50%); 2) discrete orbital plasmacytoma (7 patients; 23%); 3) infiltrative plasmacytoma either originating from a sinus (4 patients; 13%); or 4) originating from the orbital floor and infiltrating facial soft tissue (4 patients; 13%). Of the 29 patients with available treatment data, 2 had radiation only, 3 had chemotherapy only, 6 had chemoradiation, and 18 had stem cell transplant following chemoradiation (n = 17) or only chemotherapy (n = 1). Following treatment, 10 patients achieved complete and 11 achieved partial responses.
CONCLUSION
Orbital plasmacytomas were found exclusively in patients with MM diagnosed before or immediately after orbital plasmacytoma. Plasmacytomas can have 4 distinct anatomical patterns of origin. Following treatment, all patients had good to excellent local control of their orbital lesions.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Multiple Myeloma; Orbital Neoplasms; Plasmacytoma; Retrospective Studies
PubMed: 28582371
DOI: 10.1097/IOP.0000000000000939 -
European Archives of... Feb 2018Head and neck extramedullary plasmacytoma is a rare localized plasma cell neoplasm. We intended to perform this review of the published literature to assess the... (Review)
Review
Pattern of care and impact of prognostic factors on the outcome of head and neck extramedullary plasmacytoma: a systematic review and individual patient data analysis of 315 cases.
INTRODUCTION
Head and neck extramedullary plasmacytoma is a rare localized plasma cell neoplasm. We intended to perform this review of the published literature to assess the demographic profile, pattern of care and survival outcomes.
METHODS
Two authors independently searched PubMed, Google search and Cochrane library for eligible studies from 1950 till July 1, 2016, published in English language.
RESULTS
Median age of the cohort was 57 years (range 11-85). Site-wise distributions were paranasal sinuses 22.3% (70), nasal cavity 17.5% (55), nasopharynx 10.8% (34). Median size of SEMP was 3 cm (range 0.3-12 cm). Treatment distribution was radiotherapy (RT) in 52% (164), surgery (S) 19% (60), chemotherapy (C) 5% (16), S + RT 23.49% (74),CRT 1.9% (6), S + C 0.6% (2), S + RT + C 0.95% (3).Radiation was used as a modality in 78.4%(247), surgery in 44.1%(139), chemotherapy in 4.8%(15). Median radiation dose used was 45 Gy with range 20-61 Gy. Median overall survival (OS) was 40 months (range 0.5-298). Median local progression-free survival was 36 months (range 0-298). Median myeloma relapse-free survival was 36 months (range 0.5-298). Five- and 10-year OS was 78.33 and 68.61%. Five-year cause-specific survival (CSS) and 10-year CSS was 90.15 and 83.31%. Five-year LPFS was 94.78%, and 10-year LPFS was 88.43%. Five-year myeloma progression-free survival was 84.46%, and 10-year myeloma PFS was 80.44%. The factors associated with risk of local relapse were site of disease (sinonasal), secretory EMP, type of treatment received (surgery + RT > RT alone > surgery on univariate analysis). Risk factors for myeloma relapse were coexisting diseases, site of disease (sinonasal), bony erosion, size of lesion > 5 cm and type of treatment received on univariate analysis.
CONCLUSION
Our study shows that combined modality S + RT is superior compared to uni-modality in preventing local recurrence. Radiation dose of 45 Gy is optimal. Nodal irradiation has no impact on local recurrence.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Combined Modality Therapy; Disease-Free Survival; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Multiple Myeloma; Neoplasm Recurrence, Local; Plasmacytoma; Prognosis; Radiation Dosage; Survival Analysis; Treatment Outcome; Young Adult
PubMed: 29224044
DOI: 10.1007/s00405-017-4817-z -
British Journal of Haematology Feb 2016This study aimed to review the clinical features and outcome of 53 patients with solitary plasmacytoma managed at our Institution between 1976 and 2012. Thirty-five...
This study aimed to review the clinical features and outcome of 53 patients with solitary plasmacytoma managed at our Institution between 1976 and 2012. Thirty-five patients had bone solitary plasmacytoma and 18 extramedullary solitary plasmacytoma. Tumour sizes were larger in patients with bone involvement (P = 0·003). Treatment consisted of local radiotherapy (n = 26), radiotherapy + chemotherapy (n = 15), surgery (n = 4) and chemotherapy (n = 8); the local control rate was 94·3%. Progression to multiple myeloma was recorded in 20/35 (57·1%) patients with bone involvement and in 1/18 (5·5%) patients with extramedullary disease (P = 0·0003). The 5-year overall survival (OS) rate was 78·4%; bone solitary plasmacytoma patients had a significantly worse OS (71·9% vs. 88·2%, respectively; P = 0·029) and 5-year progression-free survival (PFS; 53·0% vs. 88·5%; P = 0·0003) compared to extramedullary solitary plasmacytoma patients. On univariate analysis, bone disease and size (≥5 cm) impacted negatively on PFS (P = 0·0027 and P = 0·04, respectively). Bone disease also affected OS (P = 0·04). In multivariate analysis bone location was the only independent prognostic factor for PFS (P = 0·0041) and OS (P = 0·021). Patients with bone solitary plasmacytoma have a significantly worse prognosis than extramedullary solitary plasmacytoma cases.
Topics: Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Bone Neoplasms; Combined Modality Therapy; Disease Progression; Disease-Free Survival; Female; Humans; Male; Middle Aged; Multiple Myeloma; Plasmacytoma; Prognosis
PubMed: 26684545
DOI: 10.1111/bjh.13870 -
Frontiers in Endocrinology 2022Extramedullary plasmacytoma (EMP) can occur in various parts of the body. It is generally accepted that the highest site of occurrence is the head and neck region (80%...
RATIONALE
Extramedullary plasmacytoma (EMP) can occur in various parts of the body. It is generally accepted that the highest site of occurrence is the head and neck region (80% to 90%), followed by the gastrointestinal tract and the skin. It is worth mentioning that the site of disease, in this case, was the urethral meatus, which is extremely rare in clinical practice.
PATIENT CONCERNS
A 50-year-old female complained of an episode of painless gross hematuria without symptoms of frequent urination, urgency, abdominal pain, abdominal distension, fever, or oliguria. The patient has no history of smoking or drinking and denied any family history of solid malignancy or hematological disease.
DIAGNOSIS AND INTERVENTIONS
Urethrocystoscopy revealed urethral polypoid hyperplasia, which we initially thought could be a urethral caruncle. The patient was asked to undergo caruncle resection after 1 week of potassium permanganate sitz bath, and postoperative pathology revealed plasmacytoma. After that, a whole-body MRI showed no other lesions. She received postoperative radiotherapy.
OUTCOMES
During radiotherapy, the patient's condition and appetite were fair, and the skin mucositis was I°. Pelvic contrast-enhanced MRI and whole-body PET-CT 6 months after urethral meatus lumpectomy and radiotherapy showed changes without obvious abnormal signs. No recurrence or metastasis was detected after one year of follow-up.
LESSONS
Urinary EMP is a rare disease. It is not easy to diagnose due to its rare site of occurrence and non-specific symptoms. The diagnosis of EMP requires a combination of imaging studies and pathological findings. Currently, surgery combined with radiotherapy or radiotherapy alone is the mainstay treatment and usually results in an acceptable local control rate. At the same time, chemotherapy cannot be ignored.
Topics: Female; Humans; Magnetic Resonance Imaging; Middle Aged; Plasmacytoma; Positron Emission Tomography Computed Tomography
PubMed: 35250855
DOI: 10.3389/fendo.2022.783855 -
Medicine Nov 2018The incidence of extramedullary plasmacytoma (EMP) accounts for 3% to 4% of all cases of generalized plasmacytoma. The most common pathogenic sites are the head and the... (Review)
Review
RATIONALE
The incidence of extramedullary plasmacytoma (EMP) accounts for 3% to 4% of all cases of generalized plasmacytoma. The most common pathogenic sites are the head and the neck. It is noteworthy that the pathogenic site in this case is extraperitoneal, which is uncommon in clinical practice. In this case report, we aim to discuss the clinical features and diagnosis as well as the treatment methods of EMP.
PATIENT CONCERNS
A 30-year-old female was admitted to our hospital due to a palpable right upper abdominal mass without symptoms of abdominal pain, diarrhea, constipation, fever, or oliguria.
DIAGNOSIS AND INTERVENTIONS
Enhanced CT scan showed a right retroperitoneal mass, which we considered to be isolated fibroma. Multiple myeloma (MM) was excluded through whole-body diffusion weighted imaging (DWI) and bone marrow biopsy. The patient underwent retroperitoneal tumor resection, and the postoperative pathology revealed plasmacytoma. Thereafter, she received only postoperative radiotherapy.
OUTCOMES
During the radiotherapy, the patient's condition and appetite were acceptable with I° gastrointestinal reaction. The CT examination of the chest and upper abdomen performed 4, 8, and 12 months after the radiotherapy still showed postoperative and postradiotherapy changes after retroperitoneal plasmacytoma resection without obvious abnormal signs. No recurrence and metastasis were detected after a one-year follow-up.
LESSONS
Retroperitoneal extramedullary plasmacytoma (EMP) is a rare condition that is frequently a diagnostic challenge, mainly due to its unusual location and nonspecific symptoms, especially in the early stages. The diagnosis of EMP is made through a combination of imaging and pathological examination. Presently, the combinations of radiotherapy and surgery or radiotherapy are the primary treatments, usually leading to an acceptable local control rate. The application of chemotherapy, however, should be carefully considered.
Topics: Abdominal Pain; Adult; Diagnosis, Differential; Female; Humans; Plasmacytoma; Retroperitoneal Neoplasms
PubMed: 30431616
DOI: 10.1097/MD.0000000000013281 -
World Neurosurgery Feb 2019Parasellar plasmacytomas are rare tumors arising from the sellar region that should be considered in the differential diagnosis of lesions involving the sella and... (Review)
Review
BACKGROUND
Parasellar plasmacytomas are rare tumors arising from the sellar region that should be considered in the differential diagnosis of lesions involving the sella and clivus. Before surgical pathologic examination, parasellar plasmacytomas have often been misdiagnosed as invasive pituitary adenomas or chordomas owing to the similarity of the clinical presentation and imaging findings.
METHODS
We retrospectively reviewed the data from 5 patients with parasellar plasmacytoma who underwent endonasal endoscopic tumor resection in Beijing Tiantan Hospital from January 2008 to January 2018. Their clinical symptoms, radiological features, and treatment modalities and outcomes were summarized.
RESULTS
We enrolled a total of 5 patients (3 men and 2 women; median age at diagnosis, 54 years; range, 47-61) with parasellar plasmacytoma in the present clinical series. The presenting symptoms mainly included diplopia, headache, and blurred vision. All the patients had undergone endonasal endoscopic surgery combined with adjuvant therapy. After postoperative radiotherapy, complete remission was achieved within a median follow-up period of 41 months (range, 15-120).
CONCLUSIONS
The differential diagnosis of parasellar plasmacytoma with pituitary adenoma and chordoma should include a thorough endocrine workup, neurological examination, and radiological assessment. Our findings support radiotherapy as the main treatment of choice for parasellar plasmacytoma, given its typical feature of relatively high radiosensitivity. Tumor resection through the endonasal endoscopic approach combined with adjuvant radiotherapy could be the optimal initial treatment strategy in long-term control of the lesion and alleviation of neurological symptoms.
Topics: Adenoma; Chordoma; Combined Modality Therapy; Diagnosis, Differential; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neuroendoscopy; Pituitary Neoplasms; Plasmacytoma; Radiotherapy; Retrospective Studies; Sphenoid Sinus
PubMed: 30414521
DOI: 10.1016/j.wneu.2018.10.183 -
The Journal of Small Animal Practice Dec 2021To further characterise solitary osseous plasmacytoma in dogs, an extremely rare disease. To describe diagnosis, disease progression and treatment outcomes in dogs with...
OBJECTIVES
To further characterise solitary osseous plasmacytoma in dogs, an extremely rare disease. To describe diagnosis, disease progression and treatment outcomes in dogs with solitary osseous plasmacytoma.
MATERIALS AND METHODS
Retrospective review of dogs with solitary osseous plasmacytomas that were diagnosed and treated at a single institution from 2005 to 2019. Kaplan-Meier single group survival analysis was used to estimate median survival time and progression-free interval.
RESULTS
Thirteen dogs met the inclusion criteria for the study, and of those, 11 were treated. The median age at diagnosis was 8 years (range 4 to 11). Most solitary osseous plasmacytomas occurred in the vertebrae (n=8). Other sites included the maxilla (n=2), the mandible (n=1), the tibia (n=1) and the carpus (n=1). The median survival time for all dogs with solitary osseous plasmacytoma was 912 days (range 5 to 2179), and the progression-free interval for treated dogs was 310 days (range 22 to 2179). Most dogs were treated with radiation therapy (n=10) with nine of 10 receiving a definitive, daily fractionated protocol and with five of ten having had neoadjuvant surgery. Seven dogs received chemotherapy, which was initiated after progressive disease in five dogs. The median survival time for dogs that completed radiation therapy (n=9) was 1166 days (range 545 to 2179). While five dogs developed lesions at other sites, no dogs progressed to multiple myeloma.
CLINICAL SIGNIFICANCE
Canine solitary osseous plasmacytomas can be managed long term with appropriate local therapy. This observation reflects the biologic behaviour observed in humans.
Topics: Animals; Bone Neoplasms; Dog Diseases; Dogs; Multiple Myeloma; Plasmacytoma; Retrospective Studies; Treatment Outcome
PubMed: 34467522
DOI: 10.1111/jsap.13411 -
Der Radiologe Jan 2022Treatment options for patients with solitary plasmacytoma (SP) or multiple myeloma (MM) should be discussed in an interdisciplinary context. This systematic review... (Review)
Review
BACKGROUND
Treatment options for patients with solitary plasmacytoma (SP) or multiple myeloma (MM) should be discussed in an interdisciplinary context. This systematic review focuses on the importance of radiotherapy in MM and SP.
OBJECTIVE
Summary of local radio-oncological treatment options for patients with SP and MM.
MATERIALS AND METHODS
Based on a systematic literature search, the current evidence on the topic was analyzed and summarized.
RESULTS
Patients with SP should be primarily treated with radiotherapy with or without surgery. Irradiation concepts may vary depending on risk factors and manifestation (solitary bone plasmacytoma vs. solitary extramedullary plasmacytoma). Although local control rates are high after radiotherapy, progression to multiple myeloma frequently occurs. In patients with MM, radiation is mainly used in palliative settings for pain relief, prevention of fractures or in patients who suffer from neurological symptoms due to spinal cord compression. Irradiation dose and fractionation should be selected based on treatment indication and general condition of the patient.
CONCLUSION
Although most patients receive systemic treatment at initial diagnosis, approximately 40% of patients with MM will require radiation during the course of their disease. While radiation is mainly used for palliation in patients with MM, it represents the primary and curative treatment option in patients with SP.
Topics: Bone Neoplasms; Humans; Multiple Myeloma; Plasmacytoma; Radiation Oncology; Risk Factors
PubMed: 34762165
DOI: 10.1007/s00117-021-00935-y -
Current Medical Imaging 2023The study aimed to analyze computed tomography and magnetic resonance imaging features of patients with solitary extramedullary plasmacytoma. (Review)
Review
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.
Topics: Male; Female; Humans; Young Adult; Adult; Middle Aged; Aged; Plasmacytoma; Retrospective Studies; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Bone Neoplasms
PubMed: 36788682
DOI: 10.2174/1573405619666230213114231