Strahlentherapie bei solitärem Plasmozytom oder multiplem Myelom

The treatment of choice for solitary plasmacytoma is radiation therapy. The local response rate exceeds 90%. Solitary plasmacytoma is characterized by the neoplastic proliferation of a single clone of plasma cells involving invasion of skeletal structures or extramedullary soft tissues. Progression to multiple myeloma is more common in solitary plasmacytoma of the bone than in extramedullary plasmacytoma. In the treatment of multiple myeloma radiotherapy is effective in palliating local symptoms, such as painful bone metastases, radiculopathy and spinal cord compression. Long-course radiotherapy (10×3 Gy, 20×2 Gy) results in better outcome of pain relief, recalcification rates and local control rates. Patients with a poor survival prognosis benefit from short-course radiotherapy (1×8 Gy, 4×5 Gy). Total body radiation as part of conditioning protocols prior to autologous blood stem cell transplantation is no longer a treatment of choice.     

Solitary bone plasmacytoma and extramedullary plasmacytoma

Opinion statement Solitary bone and extramedullary plasmacytomas are rare plasma cell proliferative disorders. Their diagnosis is based on histologic confirmation of monoclonal plasma cell infiltration of a single disease site and on the exclusion of systemic myeloma. For both entities, the treatment of choice is localized radiotherapy. With modern radiotherapy and with a total dose of at least 4000 cGy, the risk for local recurrence is less than 5%. There is no role for systemic chemotherapy in the management of these disorders. Approximately 30% of patients with solitary bone plasmacytoma (SBP) remain disease-free for several years; some of these patients may be cured. Patients with the best prognosis are those in whom the monoclonal protein disappears by 1 year after radiotherapy. The prognosis of patients with solitary extramedullary plasmacytoma (SEP) appears to be better than for patients with SBP because approximately 70% of patients with SEP remain disease-free at 10 years. With more sensitive staging procedures, the diagnosis of SBP and SEP may become less common, but the number of patients with prolonged stability and cure may increase.     

Solitary extramedullary plasmacytoma of the bladder: a case report and literature

Plasmacytoma is a rare B-lymphocyte neoplastic disorder that usually presents as the generalized disease multiple myeloma. Less than 5% of the cases present as a solitary mass of monoclonal plasma cells in the bone or soft tissue. Although solitary extramedullary plasmacytoma (SEP) may arise in any organ, it rarely involves the urinary bladder. A 67-year-old male without a history of multiple myeloma presented with urinary frequency and nocturia; he was later diagnosed with SEP of the bladder. The patient was initially treated with a course of radiation therapy without symptomatic improvement; therefore a chemotherapy regimen consisting of lenalidomide and dexamethasone was subsequently given for six cycles. SEP usually carries a better prognosis and higher cure rate than solitary plasmacytoma of bone, as SEP is radiation sensitive. The role of adjuvant chemotherapy in the treatment of SEP that is resistant to radiation therapy is not clear, since most of the recommendations have been derived from the experience of head and neck SEP. The literature also lacks recommendations for choice of a chemotherapy regimen and surveillance of isolated bladder plasmacytoma. Here we present the first case of a radiation-resistant solitary plasmacytoma of the bladder that was successfully treated with lenalidomide and dexamethasone with successful clinical remission.     

Plasma cell neoplasms. Review of disease progression and report of a new variant

Plasma cell neoplasms are generally categorized into four groups; multiple myeloma (MM), solitary plasmacytoma of the bone (SPB), plasma cell leukemias, and extramedullary plasmacytomas (EMP). A plasmacytoma is defined as any discrete, usually solitary mass of neoplastic plasma cells, either in the bone marrow or in various soft tissue sites. Each manifestation of a plasma cell neoplasm differs in terms of tumor recurrence and progression to MM. A gastric plasmacytoma (GP) is a rare presentation of extramedullary plasmacytoma and has not been previously reported as a site of recurrence for a SPB. This pattern of tumor recurrence is unique and the management of gastric plasmacytoma as part of this complex disease is discussed. The continuum of progression between various sites and manifestations of plasma cell manifestations is reviewed including a previously undiscovered sequence of bone disease, gastric disease, and finally multiple myeloma.     

Up-date on solitary plasmacytoma and its main differences with multiple myeloma

Solitary plasmacytoma is plasma cell neoplasm. It is a localized bone disease and for this reason it is different from multiple myeloma (systemic plasma cell neoplasm). Sometimes, solitary plasmacytoma precedes a following multiple myeloma. Clinical findings of solitary plasmacytoma are related to the univocal localization on damaged bone, while laboratory findings could be similar to multiple myeloma (i.e. M component, kidney dysfunction, blood calcium alterations, increased beta-2-microglobulin). However, during a solitary plasmacytoma, laboratory findings could not be present contemporaneously such clinical complications (i.e. kidney failure, immunological disorders with a trend toward infectious disease and/or autoimmunity, neurological disorders, haematological disorders, amyloidosis, POEMS syndrome). These raise the reason because solitary plasmacytoma has better prognosis compared to multiple myeloma.     

1例锁骨近端孤立性骨浆细胞瘤的临床报告

孤立性骨浆细胞瘤是一种以浆细胞单克隆异常增殖为特征的恶性肿瘤。临床上主要以单一骨破坏常见,约占所有浆细胞恶性肿瘤的5%。发生于锁骨近端的孤立性骨浆细胞瘤临床上少见,诊断较困难且容易发生误诊误治。     

Strahlentherapie beim Myelom

Tumoricidal radiation is the treatment of choice for a solitary plasmacytoma and the local response rate exceeds 80–90%. Progression to multiple myeloma (MM) is much less common in extramedullary plasmacytoma than in solitary plasmacytoma of the bone. MM is characterized by the neoplastic proliferation of immunoglobulin-producing plasma cells, often resulting in extensive skeletal destruction with focal lytic lesions. Radiotherapy is especially useful to palliate bone pain, fractures, radiculopathy, and spinal cord compression. High-dose chemotherapy has become a common treatment of MM. The role of total body irradiation (TBI) in the conditioning regimens before transplantation remains controversial. To overcome TBI-related toxicity, total marrow irradiation (TMI) with lung and liver shielding was developed representing a specific therapeutic approach for multiple myeloma instead of the standard TBI procedures.     

12例骨孤立性浆细胞瘤的临床特点与预后回顾性分析

背景与目的：骨孤立性浆细胞瘤（SBP）少见，目前国内有关该病的相关临床报道较少。本研究对其特点与预后作一探讨。方法：收集1998--2007年北京大学第三医院确诊的12例SBP患者的临床资料，并对其临床特点、治疗及预后作一回顾性分析。结果：12例SBP患者年龄37～71岁，平均年龄49．6岁，男女比3：1。免疫表型：11例表达CD79a，10例表达VS38C，均不表达CD20。随访12-87个月，平均随访时间（40±22）个月。4例（33％）转为多发性骨髓瘤，其中2例死于感染，中位存活时间73个月，3年和5年生存率分别为90％和75％。结论：SBP好发于中老年男性，预后较好，部分可转化为多发性骨髓瘤。     

Solitary plasmacytoma of bone and extramedullary plasmacytoma

A small proportion of patients with plasma cell myeloma have a solitary plasmacytoma of bone. Strict staging criteria, including normal MR imaging studies of the axial skeleton and the long bones and absence of monoclonal plasma cells detected by flow cytometry or PCR, are required for diagnosis. Radiotherapy at a dose of 4500 cGy is required to eradicate the local tumor. Many patients enjoy prolonged disease-free survival, but the incidence of systemic relapse is high. It is expected, however, that if strict diagnostic criteria are applied some patients may be cured. Extramedullary plasmacytoma is an even rarer plasma cell disease which usually occurs in the head and neck area. Careful microscopic and immunohistochemical studies are required for the correct diagnosis, because this disease can be confused with other malignancies, particularly lymphomas. The treatment of choice is radiotherapy which, in cases of head and neck plasmacytomas, should encompass the adjacent lymph nodes. Most patients with extramedullary plasmacytoma can be cured, and fewer than 30% develop a distant failure in the form of multiple myeloma or multiple extramedullary tumors.     

Intracellular Ig identification on paraffin-embedded solitary plasmacytoid proliferation: a routine method to define the biological nature of plasma cells.

The difficulty to recognize morphologically the biological nature of the exuberant plasma cell proliferations is known by every pathologist. Therefore, an immunohistochemical method has been applied to routine paraffin-embedded sections of biopsies from 26 patients suspected to bear a solitary plasmacytoma. The method has allowed to distinguish between monoclonal (likely to be neoplastic) and polyclonal cases (likely to be reactive).     

Clinical course of solitary extramedullary plasmacytoma.

BACKGROUND AND PURPOSE: Solitary extramedullary plasmacytoma (EMP) represents a rare category of malignant disease on which there are limited data in regard to diagnosis, staging and natural history. This study attempted to clarify the clinical course of solitary extramedullary plasmacytoma after radiation or surgical therapy given with curative intent. MATERIALS AND METHODS: The diagnosis was based on a mass of clonal plasma cells separate from bone or bone marrow without evidence of occult disease elsewhere. Between 1963 and 1996, 22 previously untreated patients with an EMP were diagnosed. Disease presented in the head or neck in 86%, usually in the nasal cavity (NC) or maxillary sinus (MS), and in these areas local bone destruction was found in 10 of 11 patients. Among all patients, serum myeloma protein was present in three patients (14%) and Bence Jones protein alone was found in two patients (9%). Radiation therapy was the sole treatment for 18 of 22 patients, and the median radiotherapy dose was 50 Gy (range, 40-60 Gy); five of seven patients with an EMP of oral cavity (OC), oropharynx (OP), nasopharynx (NP), parotid or larynx also received elective neck irradiation. Two patients underwent surgery plus postoperative irradiation of a plasmacytoma of the sigmoid colon or pleura, and two patients had resection alone of a plasmacytoma of the colon or cervical lymph node. RESULTS: Local control was achieved in 21 of 22 patients (95%), and disease never recurred in regional nodes. Disappearance of myeloma protein occurred in three of five patients with an evaluable abnormality. Multiple myeloma developed in seven patients (32%), all within 5 years. The 5-year rate of freedom from progression to multiple myeloma was 56% and the median survival was 9.5 years. CONCLUSION: Radiation therapy achieved excellent locoregional control of EMP with an approximate cure fraction of 50%.     

Solitary plasmacytoma of bone vs. extramedullary plasmacytoma and their relationship to multiple myeloma.

Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMPC) are unusual solitary tumors of plasma cell origin. The clinical differences between these tumors and multiple myeloma are the subject of continued study. This review examines the radiotherapy experience at M. D. Anderson Hospital. Between 1948 and 1977, 12 patients with SPB and 12 patients with EMPC were treated with radiotherapy. Radiotherapeutic doses were most often (22/24) greater than 4,000 rads, at 200 rads per day. In order to qualify as solitary plasmacytoma a disease-free interval of 3 years was required. In the group of SPB, 5 patients of 9 were alive with no evidence of disease (NED) at 3 years, 4 of 5 at 5 years, and none of 4 at 10 years. In the EMPC group, 8 patients of 11 were NED at 3 and 5 years, and 6 of 9 at 10 years. Six patients with SPB developed multiple myeloma compared with only two with EMPC. The results confirm the better prognosis of EMPC and support the theory that SPB and EMPC are two different entities.     

Role of radiotherapy in solitary bone plasmacytomas

Background: Solitary bone plasmacytoma (SBP) is a rare tumor for which the standard treatment remains local radiotherapy (RT). While this results in high rates of local control, the majority of patients ultimately develop multiple myeloma (MM). Here we present a 20‐year multi‐institutional review of our experience in an attempt to better understand the natural history of SBP and to identify possible prognostic factors. Methods: A total of 44 patients were identified. The inclusion criteria were the presence of a solitary bone lesion with histologic confirmation of plasma cells on biopsy, a normal bone marrow aspiration and trephine (<10% plasma cells), a negative radiographic skeletal survey, and no anemia, hypercalcemia or renal impairment secondary to plasma cell dyscrasia. Patient ages ranged from 25 to 83 years (median of 54 years) and 64% of patients had a monoclonal paraprotein in the serum and/or urine. All patients received local megavoltage RT with doses ranging from 30 to 54 Gy. Potential prognostic factors including age, gender, site of involvement, presence or absence of soft tissue extension, presence or absence of monoclonal paraprotein at diagnosis, persistence or disappearance of monoclonal paraprotein following RT, the time taken for monoclonal paraprotein to disappear following RT, and the use of magnetic resonance imaging (MRI) staging were evaluated to determine their impact on the likelihood of developing MM. Results: Median follow up was 6 years (range 0.1–15.2 years). Effective local control was achieved in 42 (95%) patients, but 30 (68%) patients subsequently progressed to MM at a median time to progression of 1.6 years. The median survival from initial diagnosis was 7.5 years. We were unable to define any prognostic factors that were associated with an increased risk of developing MM. Conclusion: Our series confirms the excellent local control achievable with RT. However, the majority of patients with SBP will ultimately progress to MM and strategies, such as adjuvant chemotherapy, should be explored in selected patients.     

Solitary plasmacytoma of the jaw occurring in an elderly woman affected by hepatitis C virus infection: a case report

Solitary plasmacytoma accounts for a small percentage of plasma cell neoplasms. The disease often affects older people and is potentially curable. Only a few cases of solitary jaw plasmacytoma have been described in the literature. Here we report the case of a 76-year-old woman affected by chronic hepatitis C infection and isolated plasmacytoma of the left jaw. Plasmacytoma was diagnosed in January 2001, but the swelling of the mandible had been present since 1993. Two different pathologists made the diagnosis on the basis of biopsy material from the mandibular swelling.     

CD56-positive small round cell tumor: osseous plasmacytoma manifested in osteolytic tumors of the iliac bone and femora

Monoclonal gammopathy of undetermined significance does not overexpress cluster of differentiation (CD) 56, but plasma cell myeloma frequently overexpressed it. However, plasma cell leukemia and extramedullary plasmacytoma usually down-regulate CD56 expression. Plasmacytoma, especially 'solitary plasmacytoma of bone', is difficult to diagnose as plasma cell neoplasm, because it occasionally appears similar to other bone tumors, both clinically and pathologically, and is rarely accompanied by monoclonal protein in the serum or urine. The present case was a patient with an osteolytic 'small round cell tumor' of the iliac bone, which also invaded the femora. An immunohistopathological finding of CD56 expression played a key role in making a diagnosis. The definitive diagnosis of plasmacytoma was made based on the electron microscopic findings. The plasma cells which infiltrated her sternum showed the same restriction to kappa light chain expression in their cytoplasms as that of the iliac bone tumor cells, but did not express CD56. Locally infiltrating osteolytic bone tumors should be examined for surface immunoglobulin light chains as well as CD56 expression when plasmacytoma is suspected.     

SOLITARY PLASMACYTOMA OF BONE AND EXTRAMEDULLARY PLASMACYTOMA

ＳＯＬＩＴＡＲＹＰＬＡＳＭＡＣＹＴＯＭＡＯＦＢＯＮＥＡＮＤＥＸＴＲＡＭＥＤＵＬＬＡＲＹＰＬＡＳＭＡＣＹＴＯＭＡＹａｎｇＤｉｓｈｅｎｇ杨迪生；ＦａｎＳｈｕｎｗｕ范顺武；ＴａｏＨｕｉｍｉｎ陶惠民；ＨｅＲｏｎｇｘｉｎ何荣新；ＹｅＺｈａｏｍｉｎｇ叶招明；Ｚｈ...     

Solitary plasmacytomas of bone and extramedullary plasmacytomas. A clinicopathologic and immunohistochemical study

Twenty-two patients with solitary plasmacytoma of bone (SPB) and 13 with extramedullary plasmacytomas (EMP) were studied. The average follow-up period for SPB was 90 months and 86 months for EMP. Thirty-six percent of patients with SPB developed multiple myeloma (MM) in an average of 39 months, and 23% of patients with EMP developed MM in an average of 23 months. No significant differences in survival, incidence of MM, or interval to the development of MM were found between the two groups. The 11 cases of EMP with evaluable tissue for immunohistochemical study were either monotypic kappa or lambda, as were 9 of 10 SPB. Presence of monoclonality did not predict the development of MM. The histologic parameters of nuclear immaturity and presence of prominent nucleoli seem to be the best indicators of which patients will develop MM. Solitary plasmacytoma of bone and EMP appear to be more closely related than has been previously recognized.     

Immunoglobulin Gene Rearrangement in Plasma Cell Dyscrasias: Detection of Small Clonal Cell Populations in Peripheral Blood and Bone Marrow

The bone marrow (BM) and peripheral blood (PB) samples of 71 patients with plasma cell dyscrasias were analysed by the Southern blot technique for the presence of clonal immunoglobulin (Ig) gene rearrangements. 53% of BM samples examined were archival material such as air dried BM slides or frozen trephine biopsies. The results were related to bone marrow plasmacytosis as determined by cytology and flow cytometry, and other clinical parameters. Clonal Ig gene rearrangements were found in BM samples of 45 (83%) of 54 MM patients and in 3 of 6 patients with monoclonal gammopathy of unknown significance (MGUS). Clonal cell populations in the PB were detected in 11 (30%) of 37 examined MM patients, but in none of the patients with MGUS or solitary plasmacytoma of bone. PB involvement was associated with progressive disease. Circulating monoclonal cells were significantly associated with higher M-protein levels (p 0.05). Thus, circulating clonal precursor cells are encountered more frequently in active MM.     

骨孤立性浆细胞瘤的影像学表现

目的分析不同部位骨孤立性浆细胞瘤的X线、CT和MRI表现,进一步认识与提高骨孤立性浆细胞瘤的诊断准确率。方法回顾性分析12例经临床病理证实的发生于椎体、肱骨、肋骨的孤立性浆细胞瘤病例,总结分析其影像学表现。结果12例孤立性浆细胞瘤为肋骨3例、胸椎6例、肱骨3例,其中1例肱骨发生病理性骨折。X线及CT表现为穿凿样溶骨性骨质破坏、膨胀性生长,边界较清,其中1例骨皮质增厚、硬化,所有均未见明显骨膜反应。与肌肉信号相比,MRI在T1WI表现为稍低信号,T2WI表现为高信号,所有均未见瘤周水肿,但可见不同程度的软组织浸润,5例可见软组织肿块形成,增强明显强化。结论X线平片对骨孤立性浆细胞瘤的诊断敏感度较低;CT能清晰显示瘤体内增厚的骨嵴或残存的骨质;MRI可明确髓腔内外病变与周围软组织的关系。平片、CT和MRI三者联合综合评价有助于提高骨孤立性浆细胞瘤的诊断准确性。     

Solitary intracranial plasmacytoma--a case report and review of the literature

A 49 year old man had had headache and some cranial nerve paralyses for about three years. A tentative diagnosis of meningioma of the skull base or chordoma was made before his death. In the postmortem examination, a large tumor lying on the skull base was found. The sella turcica, a part of the basilar bone were destroyed. The neoplasm had invaded the adjacent cerebrum, cranial nerves and extra-cranial tissues. Histopathologically, the lesion showed a typical poorly-differentiated plasmacytoma. Both clinic and pathologic characteristics of this tumor are discussed and the literature on solitary intracranial plasmacytoma is reviewed.