Localised plasmacytomas in Taiwan: comparison between extramedullary plasmacytoma and solitary plasmacytoma of bone.

The clinical features and response to therapy of 32 Chinese patients with localised plasmacytoma are presented, and a comparison between extramedullary plasmacytoma (EMP) and solitary plasmacytoma of bone (SPB) is made. Twenty-two patients had SPB and ten had EMP, accounting for 9% of all of our plasma cell neoplasms. Both groups had a male predominance with a median age of 54 years for SPB and 63 years for EMP. The common sites of SPB included vertebral bodies (15) and the skull (4). Most EMPs occurred in the oronasopharynx (6) and paranasal sinuses (2). An M-protein was detected in eight patients with SPB and in six with EMP. Seventeen patients with SPB and seven with EMP received radiation therapy, and all achieved initial local control. The pattern of failure in 22 patients with SPB manifested as local recurrence in two, multiple bone metastases without bone marrow plasmacytosis in two, multiple EMP progression in two, and development of multiple myeloma (MM) in one. There were two local recurrences, one further solitary bone involvement and one MM conversion in the EMP group. Local recurrence or dissemination was associated with the appearance of M-protein or an increase in the M-protein level in both groups. There was no significant difference in M-protein status or incidence and patterns of failure between the two groups. Patients with EMP had a more favourable overall survival than those with SPB (P = 0.03). The 5 year disease-free survival rate was 79% for EMP and 58% for SPB (P = 0.53). Patients aged less than 60 years had a better overall survival in the SPB group, but location of tumour, presence of M-protein, radiation dose and chemotherapy did not influence prognosis in either group. Our results indicate that adequate local therapy can result in long-term survival with a low frequency of MM progression for patients with localised plasmacytomas, and both EMP and SPB appear to be similar in terms of frequency and patterns of failure.     

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.     

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.     

The treatment of solitary plasmacytoma of bone and extramedullary plasmacytoma

Eleven patients with solitary plasmacytoma of bone (SPB) and six patients with extramedullary plasmacytoma (EMP) were treated at the UCLA Center for the Health Sciences. Primary treatment in 14 of 17 patients was with radiation, while three patients were irradiated for recurrent disease. Eleven patients with SPB were irradiated with dose of 32-55 Gy, with 10 of 11 patients receiving doses of 40-55 Gy. Local control was achieved in 10 of 11 patients with SPB. One patient died with metastatic disease with unknown local status. Six patients with EMP were irradiated with doses of 38-56 Gy. Of these patients, two were locally controlled; one patient failed locally; one patient died during treatment; one patient died with local disease at 85 months after multiple resections, chemotherapy, and two courses of irradiation; and one patient was lost to follow-up. Progression to multiple myeloma was seen in 5 of 11 patients with SPB and in none of six patients with EMP. For patients with SPB, we recommend treating the entire bone to 40 Gy, with a boost when feasible. Patients with EMP receive the same dose, including the lymph nodes in tumors at high risk for spread. Radical surgical resections appear to be unwarranted.     

Solitary plasmacytoma. II: Solitary plasmacytoma of bone.

The clinical features and the results of treatment of twelve patients with solitary plasmacytoma of bone (SPB) are reported. The median age at presentation was 53 years and there was a predominance of males. Five patients have developed multiple myeloma (MM), for within three years and one after twelve years. Seven patients have not shown evidence of dissemination, and five are alive 1--15 years (median 9 years) after diagnosis. Two patients had metastases to local lymph nodes. There are no reliable criteria by which patients with truly SPB can be prospectively distinguished from those with occult MM, and it is recommended that all patients with apparent SPB should receive "curative" local therapy.     

50例孤立性浆细胞瘤临床分析

目的对孤立性浆细胞瘤(SP)的特点和预后因素进行回顾性分析,以帮助临床诊断、治疗及对预后的判断。方法入组50例SP患者,并对其进行随访,应用Kaplan-Meier法对其临床特点、治疗及预后因素进行回顾性分析。结果髓外浆细胞瘤(EMP)组与骨孤立性浆细胞瘤(SPB)组、放疗组与未放疗组、年龄﹤60岁与年龄≥60岁组的log rank检验结果提示,生存曲线差异均无统计学意义(P均>0.05)。50例SP患者与168例多发性骨髓瘤(MM)患者的生存曲线差异有统计学意义(P<0.05)。结论 SP患者中,EMP与SPB的生存状况无明显差异,SP好发于中老年人,预后较好,部分可转化为MM。     

Plasmacytoma. Treatment results and conversion to myeloma.

Forty-six cases of solitary plasmacytoma were reviewed for response to radiation and progression to multiple myeloma. Cases were classified as solitary plasmacytomas of bone (SPB) (32 cases) or extramedullary plasmacytomas (EP) (14 cases). There was an overall 93% response rate of the tumor to radiation therapy: 62% had a complete response after radiation therapy, whereas 31% had a partial response. Conversion to multiple myeloma was influenced by the type of plasmacytoma; 53% of the patients with SPB converting to myeloma versus 36% of the patients with EP. Time from diagnosis to conversion for patients with SPB showed no evidence of plateau, with conversion continuing to occur even after 17 years. The median survival time for patients after conversion to myeloma was 14.5 months and was not affected by time to conversion. Serum protein level, presence of monoclonal gammopathy, and size of primary lesion were of some prognostic significance in predicting conversion to myeloma. Adjuvant chemotherapy did not affect the incidence of conversion but did appear to delay conversion to myeloma. Seven patients in whom multiple sequential solitary plasmacytomas developed formed a distinct subset, with a median time to a second plasmacytoma of 63 months. In three of these patients, conversion to myeloma occurred subsequently. This study supports the idea of EP having a lower incidence of conversion to myeloma and a different natural history from SPB, with SPB likely to be multiple myeloma in evolution.     

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.     

The role of radiation therapy in the treatment of solitary plasmacytomas

Between 1960 and 1985, 30 patients with solitary plasmacytomas were treated with radiotherapy at the University of Iowa: 13 patients with extramedullary plasmacytomas (EMP) and 17 with solitary plasmacytomas of bone (SPB). The local control rates were 92% for patients with EMP and 88% for those with SPB. Two of nine patients (22%) with EMP treated to the primary tumor only developed regional lymph node metastasis, indicating the need for elective irradiation of this area. The most common pattern of failure in both groups was progression to multiple myeloma. This occurred in 23% of the patients with EMP and 53% of those with SPB. The time course of progression to multiple myeloma differed for the two groups. All of those who progressed to multiple myeloma in the EMP group did so within 2 years, whereas a significant number of those in the SPB group progressed more than 5 years after initial therapy. None of five patients who received adjuvant chemotherapy in the SPB group progressed to multiple myeloma, compared to 75% (9/12) of the patients who did not receive chemotherapy.     

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.     

Primary isolated extramedullary plasmacytoma of mesentry: A rare case report

Extramedullary plasmacytoma (EMP) is an uncommon entity that most commonly involves nasopharynx and upper repository tract. Involvement of GIT occurs in approximate 10% of cases. According to WHO plasma cell tumors have been classified into two main groups: Multiple myeloma and plasmacytoma. Plasmacytoma includes solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. EMP can be either primary without evidence of bone marrow involvement or may occur simultaneously with multiple myeloma representing extramedullary spread of the disease. It may occur in association with multiple myeloma and it may precede, accompany or follow the onset of multiple myeloma. Diagnosis of primary EMP requires the exclusion of associated multiple myeloma as shown by negative Bence Jones Proteins in urine, normal serum electrophoresis, normal bone marrow biopsy, normal skeletal survey and normal calcium levels. Here we present a case of 55-year male who came to Nephrology Department for urinary tract infection and pain abdomen. Patient was referred to Radiology for ultrasonography which revealed bilateral renal parenchymal disease with a well-defined mass in the mesentry which was further confirmed on computed tomography. Patient was surgically operated and diagnosis of primary EMP of mesentry was made on histopathological examination. Only three cases have been reported so far in the literature. Keywords: Extramedullary plasmacytoma, mesentry, plasma cell dyscrasias.     

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.     

Solitary plasmacytoma of bone and soft tissue

Between 1962 and 1978, 15 patients presenting with a solitary plasmacytoma were treated with curative intent by radiotherapy alone at the University of Florida. Criteria for admission to this study were: 1) a biopsy-proven, apparently solitary focus of plasmacytoma; 2) bone marrow biopsy showing less than 10% plasma cells; and 3) no evidence of disseminated disease. In 9 patients the primary site was osseous and in 6 patients extramedullary; 5 of the 6 extramedullary lesions were located in the upper respiratory passages. Two of the 6 extramedullary plasmacytomas progressed to multiple myeloma at 2 and 7 months. The remaining 4 patients have been disease free for periods ranging from $\text{2}\text{2}\text{3}$ years to over 16 years. Of the 9 patients with osseous lesions, 3 developed multiple myeloma in 3–23 months and one developed a solitary second bone lesion at 9 years. One patient with a large sacral lesion developed a local recurrence following an initial radiation dose of 3000 rad. The recurrence was re-treated with radiation, and local control was obtained. No other local failures occurred. This study presents a detailed analysis of the time-dose relationship required for local control, based on a study of our own patients and a review of the literature.     

髓外浆细胞瘤的诊断和治疗

目的 探讨髓外浆细胞瘤（EMP）的诊断和治疗。方法 4例髓外浆细胞瘤的临床资料。结果 1例疗后失访。2例分别随访74月和56月，无局部复发、远处转移，无进展证据。1例治疗后6个月复发，18个月时死于多发性骨髓瘤（MM）。结论 EMP的诊断确立必须有组织学结果，并经系统检查除外MM；放疗和手术是EMP的主要治疗手段。     

Clinical analysis of 36 multiple myeloma patients with extramedullary plasmacytoma invasion of the spinal canal

Few physicians are familiar with extramedullary plasmacytoma (EMP) invasion of the spinal canal in multiple myeloma (MM) patients, and little information about this rare disease is available. The purpose of the present study was to investigate the clinical features, prognosis and treatment of MM patients with EMP invasion of the spinal canal. We evaluated 36 MM patients with EMP invasion of the spinal canal. EMP invasion was confirmed by magnetic resonance imaging, computed tomography and/or histopathological analysis of bone marrow biopsy samples. Patients underwent surgery followed by chemotherapy or received chemotherapy alone. Chemotherapy consisted of bortezomib‐containing regimens and other combination therapies. The patients' median age was 58.6 years (range, 31–78 years). Eight patients had negative immunofixation electrophoresis results, and nine patients had a bone marrow plasma cell infiltration rate of less than 5%. Of the 36 MM patients with EMP invasion of the spinal canal that we identified, 19 had thoracic spinal cord involvement, 10 had lumbar spinal cord involvement, 2 had sacral spinal cord involvement and 5 had both lumbar and thoracic spinal cord involvement. The findings of our study, which is the largest study in MM patients with EMP spinal canal invasion conducted to date, suggest the importance of the early detection of spinal invasion in MM patients. Extramedullary disease was resistant to conventional treatments but responded well to regimens containing novel drugs such as bortezomib. In patients with symptoms of nerve root involvement, the tumour should be resected as soon as possible to relieve spinal cord compression. Copyright © 2014 John Wiley & Sons, Ltd.     

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 bone plasmacytoma: management of isolated local relapse following radiotherapy

Radiotherapy is the prime treatment modality for solitary plasmacytomas of bone (SPB). Although local control rates are excellent, progression to multiple myeloma is frequent, albeit with varying latency. Local failure in the absence of dissemination is rare and thus management is poorly documented. We discuss such a patient who presented 3 years after local radiation for a pelvic SPB and review the relevant literature. Radiation doses, portals employed and prognostic factors that may predict progression to myeloma are discussed. This report shows that an isolated recurrence of SPB in a previously irradiated field was successfully treated with orthopaedic surgery. This resulted in good pain relief and mobility for the patient, who remains free of disease 6 months after operation.     

Metastatic extramedullary plasmacytoma of the lung

Extramedullary plasmacytomas (EMP) comprise 4% of all plasma cell neoplasms and commonly in the upper airway or digestive tract but rarely develop in the lungs. We present a case of primary pulmonary plasmacytoma in an 89 year old man, presented as a hilar mass with associated intrathoracic and extrathoracic lymph node metastases, but without evidence of myeloma. Treatment options for EMP include surgery, surgery and radiotherapy, surgery and chemotherapy or chemotherapy alone. Local recurrence rate is reported as 10-30%, with 17-48% progressing to multiple myeloma and median survival being 63-101 months. In view of the advanced age of this patient, who was initially treated unsuccessfully with intravenous cyclophosphamide and subsequently with two cycles of VAMP chemotherapy with good resolution of his disease, he is undergoing regular follow-up only.     

A case report of tracheal extramedullary plasmacytoma

Introduction Extramedullary plasmacytoma (EMP) is an uncommon tumor that often develops outside the bone and arises from clonal proliferation of atypical plasma cells before EMP is diagnosed. Multiple myeloma(MM) must be excluded by performing laboratory tests such as serum protein electrophoresis, bone marrow biopsy and skeletal imaging ex-aminations. A bone marrow biopsy should show no evidence of mul-tiple myeloma, and less than 3% of plasma cells. Monoclonal bands of serum protein and Bence-Jones protein in the urine can sometimes be detected. EMP can involve any extraosseous organs, but it pre-dominantly affects the head and neck areas. Any extra-osseous organ may also be involved[1,2]. Tracheal involvement is a rare finding. Only a few cases of primary tracheal extramedullary plasmcytoma have been reported[2-4], Here we present a rare case of truly localized tra-cheal extram edullary plasmacytoma without evidence of myelomaelsewhere.     

14例髓外浆细胞瘤患者的临床特征分析及治疗

  目的   探讨伴髓外浆细胞瘤（extramedullary plasmacytoma,EMP）的多发性骨髓瘤（multiple myeloma,MM）患者的临床特点,治疗选择及预后。   方法   对14例确诊的伴髓外浆细胞瘤的多发性骨髓瘤进行回顾性分析。   结果   14例患者男女比为1:l,中位年龄56.9岁。9例患者初诊时即出现EMP,5例在治疗过程中发生。治疗选择包括化疗,手术,放疗或自体移植。6例患者单发EMP,8例多发EMP（multiextramedullary plasmacytomas,MEP）;单发患者中5例部分或接近完全缓解,1例病情进展;多发患者中5例死亡,1例病情进展,2例部分缓解。   结论   MM伴有MEP者对常规化疗反应差,预后不良,需要探讨新的治疗策略。