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.     

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.     

Renal extramedullary plasmacytoma—One case report

Extramedullary plasmacytoma (EMP) is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.     

Renal Extramedullary Plasmacytoma——One Case Report

T Extramedullary plasmacytoma (EMP) is rare in multiple myeloma, especially kidneys are involved. We report one case, including diagnosis and treatment. The purpose of this paper is to provide an attention on the extramedullary plasmacytoma disease, reducing the misdiagnose with this disease.     

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.     

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 AND EXTRAMEDULLARY PLASMACYTOMA

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Solitary extramedullary plasmacytoma of tonsil — a rare location

Extramedullary plasmacytoma is a rare form of plasma cell dyscrasia, in which plasmacell tumours arise outside the bone marrow. The tumor may arise in any part of the body but the vast majority occur in head and neck, primary in the nasal cavity, paranasal sinuses or upper airway. We present here a rare case of extramedullary plasmacytoma arising from the tonsil The clinical, histolopathology, immunohistochemical findings are reviewed and the management discussed.     

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.     

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.     

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.     

Plasmacytoma of the Nasolacrimal Duct Simulating Dacryocystitis: An Uncommon Presentation for Extramedullary Relapse of Multiple Myeloma

The most common site for localized forms of plasma cell neoplasms (extramedullary plasmacytoma; EMP) is the upper respiratory tract, including the oropharynx, nasal cavities, sinuses and larynx. A 50-year-old woman with a history of myeloma in complete remission after autologous stem cell transplantation complained of two weeks of epiphora of the left eye with subsequent diplopia, bloody nasal discharge and progressive swelling around the nasolacrimal sac. A solitary mass in the left sinonasal area, extending to the nasolacrimal duct (NLD) was detected on MRI, whose histopathological examination was consistent with plasmacytoma. Further clinical investigation ruled out multiple myeloma (MM). The patient underwent debulking surgery and adjuvant chemotherapy followed by local radiotherapy in an attempt to achieve complete response. Despite being a rare entity, EMP of the NLD should be considered in the differential diagnosis of epiphora and dacryocystitis. To our knowledge, this is the first case of a plasmacytoma of the NLD presenting as isolated extramedullary relapse of MM. The follow-up in EMPs should include appropriate imaging studies, a systemic workup to rule out MM.Key words: Dacryocystitis, Multiple myeloma, Nasolacrimal duct, Plasmacytoma     

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.     

Retro-peritoneal plasmacytoma: a case report and review of literature

Solitary Extramedullary Plasmacytoma (EMP) is an uncommon neoplasm. When diagnosed, head and neck region is its most likely location. Rarely, it may occur in the retro-peritoneum. We report a 44 year old man with solitary extramedullary plasmacytoma in the retro peritoneum (RPEMP). The patient did not show response to three cycles of VAD chemotherapy. Thereafter Surgical excision of the mass was performed successfully. This is probably the first case report from Indian subcontinent.     

Extramedullary plasmacytoma of small intestine--a case report

Extramedullary plasmacytoma (EMP), being different from multiple myeloma, is very rare. One case of primary EMP of small intestine and review of literature are presented. The patient, 69-year-old male, was admitted because of left lower abdominal pain and anemia. A mass, table-tennis in size, was palpated. Intestinal obstruction was found by plain X-ray film. It was diagnosed as EMP of small intestine by pathology after exploratory celiotomy. Immunohistochemical stain showed that intracytoplasmic immunoglobulin was monoclonal IgG. Lambda. The blood Bence-Jones protein and bone marrow puncture were negative. The patient received chemotherapy after operation and was followed for two years giving satisfactory result. The pathogenesis, clinical feature, diagnostic criteria and treatment are discussed.     

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.     

p53 nuclear accumulation is associated with extramedullary progression of multiple myeloma

Progression of multiple myeloma (MM) from intramedullary to extramedullary sites heralds an aggressive phase of the disease but to the best of our knowledge, biologic factors have not been studied in paired biopsies from medullary and extramedullary sites. In this study, we immunostained paired bone marrow and extramedullary biopsies from 12 cases of MM for p53, CD56 and MIB-1 (proliferative index). In addition, 22 cases of extramedullary plasmacytoma (EMP) without bone marrow involvement were included as a control group. p53 nuclear accumulations were detected in myeloma cells derived from the extramedullary sites in 9 (75%) of the 12 cases, whereas only 1 of (8%) 12 bone marrow myeloma specimens expressed p53 (P = 0.003). p53 expression was also more prevalent in extramedullary sites of MM than EMP (75% vs. 18%, P = 0.003). There was no significant difference in CD56 expression between intramedullary and extramedullary MM (17% vs. 33%, P = 0.64), or between intramedullary MM and EMP (17% vs. 38%, P = 0.25). The MIB-1 proliferation index increased significantly as plasma cells migrated from the bone marrow microenvironment to extramedullary sites (P = 0.0001). Our data indicates that p53 nuclear expression but not CD56 expression, along with increased proliferation index is associated with disease progression from intramedullary to extramedullary sites in MM.     

An unusual presentation of extramedullary plasmacytoma in testis and review of the literature

Extramedullary plasmacytoma is a rare plasma cell neoplasm, and it is extremely uncommon in the testicles. We report a 73-year-old man with multiple myeloma presented with testicular plasmacytoma. He complained of left leg pain and scrotal swelling. Ultrasonography revealed testicular masses. Pathologic examination of the orchiectomy specimen showed plasmocytoma with kappa expression. Multiple lytic bone lesions were seen in bone survey scans, serum immunoelectrophoresis and bone marrow aspiration aided to the diagnosis of multiple myeloma. He received chemotherapy, melphalan and prednisolone, and palliative radiotherapy. He succumbed to disease after 8 months.     

Extramedullary plasmacytoma of the gallbladder diagnosed by endoscopic ultrasound fine needle aspiration (EUS-FNA)

Extramedullary plasmacytoma (EMP) is a rare entity that can exist independently or in conjunction with underlying plasma cell myeloma (PCM). When there is underlying multiple myeloma, the presence of EMP portends a poor prognosis. The most common locations for an EMP include the gastrointestinal tract, pleura, testis, skin, peritoneum, liver, endocrine glands and lymph nodes; involvement of the gallbladder is exceedingly rare with only five other cases reported and only one of which was associated with PCM. EMP of the gallbladder can manifest as acute cholecystitis, biliary obstruction, or may be asymptomatic. Treatment is traditionally surgical resection plus adjuvant chemotherapy or autologous stem cell transplant. We present a case of a 53-year-old man with PCM who was found to have a gallbladder mass on imaging and underwent endoscopic ultrasound (EUS) guided fine needle aspiration (FNA) of the mass, which was diagnostic of a plasma cell neoplasm.     

髓外浆细胞瘤临床分析

目的探讨髓外浆细胞瘤的生物学行为和临床特点,以帮助临床诊断、治疗及对预后的判断。方法对40多年来笔者所在科室收治的28例髓外浆细胞瘤进行回顾性分析,其中单纯放射治疗16例(57.1%),综合治疗11例(39.3%),单纯手术1例(3.6%)。结果中位随访90个月(4～280个月),2例(7.1%)局部复发,2例(7.1%)转化为多发性骨髓瘤。3、5、10年总生存率分别为92.6%、75.6%和75.6%;单纯放射治疗组与综合治疗组3、5、10年总生存率分别为93.8%、77.3%、77.3%和90.9%、72.7%、72.7%(P>0.05)。结论单纯放射治疗是治疗髓外浆细胞瘤最有效的方法之一,局部放射治疗DT40Gy～50Gy后,可获得良好的局部控制率和长期生存率。部分髓外浆细胞瘤可向多发性骨髓瘤转化,故需密切观察。