Extramedullary Plasmacytoma of the Colon

Multiple myeloma with extramedullary plasmacytoma is unusual, but associated gastrointestinal involvement is even rarer. A case with initial presentation as solitary plasmacytoma of the clavicle and subsequent lesions in the maxillary sinus, skin, and ascending colon is reviewed. The variable radiographic features of plasmacytoma of the colon are discussed, whether occurring as a solitary finding or in a patient with multiple myeloma.     

Response of the extramedullary lung plasmacytoma with pleural effusion to chemotherapy

 An elderly patient with an extramedullary lung plasmacytoma and subsequent pleural effusion is described. The presence of abnormal plasma cells in the pleural fluid led to diagnosis. Histologically similar conditions such as multiple myeloma and solitary myeloma of bone were ruled out by clinical evaluation. These neoplasms usually occur in the head and neck area and are not characterized by paraprotein accumulation. Few cases in the lung have been reported. We describe a case of extramedullary plasmacytoma of the lung with plasmacytoma-induced pleural effusion and the presence of monoclonal paraprotein in both the serum and urine. Chemotherapy with melphalan was effective in reducing the size of the plasmacytoma, and pleurodesis was used to manage the pleural effusion. Received: 16 December 1996 / Accepted: 16 April 1997     

Solitary pleural plasmacytomas manifested as a massive pleural effusion without evidence of monoclonal gammopathy

Abstract:   The majority of extramedullary plasmacytomas involve the upper respiratory tract but only a few are associated with serum monoclonal gammopathy. Most extramedullary plasmacytomas respond to local radiotherapy and have a better prognosis than multiple myeloma. Solitary plasmacytomas, involving the pleura, are very rare. This case report describes a patient with a solitary extramedullary plasmacytoma involving the pleura, and no evidence of serum, urine or pleural fluid paraproteins. Diagnosis was made by strongly positive immunohistochemical stains with CD38, CD138 and MUM-1 of the pleural tumour. The response to both radiotherapy and chemotherapy was poor and the patient died shortly after diagnosis.     

Gastrointestinal bleeding as initial presentation of extramedullary plasma cell neoplasms: A case report and review of the literature

BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.CASE SUMMARY Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search(Pub Med database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma(EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males(67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach(41%), followed by the duodenum(15%). The most common presenting complaint was melena(44%). In the majority of cases, the EMPs were a secondary manifestation(63%) at the background of multiple myeloma(26%), plasmablastic myeloma(7%) or high-grade plasma cell myeloma(4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.CONCLUSION Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.     

Rare pancreas tumor mimicking adenocarcinoma: Extramedullary plasmacytoma

Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma, is a rare manifestation of multiple myeloma. Although computerized tomography is useful for the diagnosis of extramedullary plasmacytoma, there are no specific radiologic markers that distinguish it from adenocarcinoma. Histological confirmation by biopsy is necessary for accurate diagnosis and management of the tumor. Endosonography is the most sensitive method for the diagnosis of pancreatic tumors, and the use of fine needle aspiration by endosonography is associated with a lower risk for malignant seeding and complications. Here, we report a case of pancreatic plasmacytoma in newly identified multiple myeloma as diagnosed by endosonography. Endosonography is a reliable and rapid method for the diagnosis of extramedullary plasmacytoma. Therefore, endosonographic fine needle aspiration should be the first choice for histological evaluation when pancreatic plasmacytoma is suspected. Ideally, the pathology would be performed at the same site as endosonographic biopsy.     

Rarepancreas tumormimickingadenocarcinoma:Extramedullary plasmacytoma简

Neoplastic proliferation of plasma cells is called plasma cell dyscrasias, and these neoplasms can present as a solitary neoplasm or multiple myeloma. Extramedullary plasmacytoma, in particular pancreatic plasmacytoma, is a rare manifestation of multiple myeloma. Although computerized tomography is useful for the diagnosis of extramedullary plasmacytoma, there are no specific radiologic markers that distinguish it from adenocarcinoma. Histological confirmation by biopsy is necessary for accurate diagnosis and management of the tumor. Endosonography is the most sensitive method for the diagnosis of pancreatic tumors, and the use of fine needle aspiration by endosonography is associated with a lower risk for malignant seeding and complications. Here, we report a case of pancreatic plasmacytoma in newly identified multiple myeloma as diagnosed by endosonography. Endosonography is a reliable and rapid method for the diagnosis of extramedullary plasmacytoma. Therefore, endosonographic fine needle aspiration should be the first choice for histological evaluation when pancreatic plasmacytoma is suspected. Ideally, the pathology would be performed at the same site as endosonographic biopsy.     

Primary extramedullary plasmacytoma: similarities with and differences from multiple myeloma revealed by interphase cytogenetics

Primary extramedullary plasmacytoma is an indolent neoplasm that infrequently converts to multiple myeloma. Since cytogenetic data on extramedullary plasmacytoma are lacking, we studied 38 cases of this type of neoplasm by fluorescence in situ hybridization. Fourteen cases (37%) contained IGH breaks, including six with a t(4;14) translocation. No translocations t(11;14), t(14;16), t(8;14), nor breaks involving MALT1, BCL6 or FOXP1 were found. Loss of 13q (40%), as well as chromosomal gains (82%) were common. There was no correlation between chromosomal alterations and clinical features or local relapse. Cytogenetically, extramedullary plasmacytoma and multiple myeloma are closely related. However, the distribution of IGH translocation partners, with the notable absence of t(11;14), is different. Key words: extramedullary plasmacytoma, multiple myeloma, cytogenetics, IGH translocation, fluorescence in situ hybridization.     

Clinical course and survival in 16 patients with localized plasmacytoma

The clinical course of 16 patients with localized plasmacytoma, 9 with solitary plasmacytoma of bone (SPB), and 7 with extramedullary plasmacytoma (EMP) are presented. Median follow up of SPB was 77 months and of EMP 74 months. The EMPs were localized in the upper respiratory tract (4 cases), the gastro-intestinal tract (2 cases) and the skin (1 case). The SPBs were localized in the ribs (3 cases), the spine (3 cases), the humoral bone (1 case), the skull (1 case) and the mandibular bone (1 case). In 3 patients with EMP and in 5 patients with SPB, the monoclonal protein could be determined at presentation. Local recurrences following surgical removal or irradiation occurred in 2 patients with EMP and in 1 patient with SPB after 12-60 months. Classical multiple myeloma developed in 3 patients with EMP and in 4 patients with SPB, 9-130 months after diagnosis. The monoclonal protein level proved to be a useful disease marker which (re)appeared at local recurrence or at dissemination. The median survival of the 16 patients with solitary plasmacytoma was 138 months, which is considerably longer than the median survival of 42 months in 14 stage-I patients with classical multiple myeloma who were observed during the same follow-up period.     

Radiotherapy of solitary plasmacytoma

Solitary plasmacytoma of the bone (SBP) or extramedullary plasmacytoma (EP) are rare neoplasms amenable to local radiotherapy. In this retrospective analysis, we report the University Heidelberg experience in the treatment of solitary plasmacytoma. From 1995 to 2008, 18 patients were treated with local radiotherapy. Ten patients suffered from SBP, eight patients showed a single extramedullary lesion. Local radiotherapy with a median dose of 45 Gy yielded excellent local control with only one patient suffering from local relapse. SBP and EP had significantly different 5-year multiple myeloma-free survival rates of 36.8% and 86.7%, respectively. However, no significant difference in overall survival could be detected. Radiotherapy can achieve excellent local control of solitary plasmacytoma. Progression to multiple myeloma, especially in the case of SBP, remains to be addressed by further studies.     

Duodenal plasmacytoma. A rare primary extramedullary localization simulating a carcinoma.

A patient with duodenal plasmacytoma is described. The extramedullary localization preceded the complete humoural picture of multiple myeloma by 15 months. The previously reported cases of myeloma of primary gastrointestinal localization are reviewed.     

Primary Small Bowel Plasmacytoma with Intracellular Fibrils

This is a report of a rare plasma cell tumor, an extramedullary plasmacytoma arising in the small bowel. The tumor first occurred in the jejunum without evidence of multiple myeloma. One year later, classical signs of multiple myeloma appeared. The rarity and unusual course of this tumor plus the finding of intracellular fibrils make the case worthy of note.     

Solitary tracheal plasmacytoma

Primary tracheal tumours are rare and trachea is an exceedingly rare site of extramedullary plasmacytoma. We report a case of extramedullary plasmacytoma involving trachea and causing symptoms of airway obstruction in a 44-year-old man. Chest radiograph was normal. Flow-volume loop showed characteristic pattern localizing the lesion at thoracic inlet. Computerised tomography and bronchoscopy demonstrated a broad based pedunculated intratracheal mass obstructing 85% of the lumen. Tracheal tumour was excised via low tracheostomy. Histopathology of resected mass revealed extramedullary plasmacytoma. Relevant investigations excluded multiple myeloma. He was managed with radiotherapy with good response and there has been no recurrence after one-year follow-up.     

Extramedullary plasmacytoma: report of two cases with uncommon presentation

Lymph node infiltration by monoclonal plasma cells can occur either in aggressive forms of myeloma or may represent regional extension of extramedullary plasmacytomas, whereas lymph node plasmacytoma presenting as a solitary extramedullary plasmacytoma is very unusual. We report two cases of lymph node plasmacytomas without systemic disease diagnosed after surgical excision. Clinical remission was achieved after local radiotherapy although one patient relapsed with multifocal extramedullary plasmacytomas 20 months after radiotherapy.     

以胸腔积液为首发症状的多发性骨髓瘤1例并文献复习

目的 提高对多发性骨髓瘤(MM)诊断的认识.方法 报道1例以胸腔积液就诊的MM病例,并对相关文献进行复习.结果 45岁女性,胸闷、憋气3个月.近1个月有少尿及头痛、头晕症状.胸部CT示右侧液气胸,左侧大量胸腔积液,胸壁见扁丘状软组织影突出.胸腔镜下见胸腔内大量淡黄色液体,脏层胸膜和膈胸膜光滑,壁层胸膜大量大小不一的结节,质硬,活检不易出血的.病理示浆细胞瘤.胸骨穿刺骨髓象检查示大量的浆细胞瘤细胞骨髓浸润.结论 MM患者以髓外病变就诊时,应结合临床表现考虑到MM的可能,及时行骨髓检查明确诊断.并发髓外病变时预后不良.     

Refractory multiple myeloma preceded by extramedullary plasmacytoma of lymph node--a case report and review of the literature]

It has been reported that extramedullary plasmacytoma (EMP) tends to be characterized by an indolent clinical course and lower incidence of progression to multiple myeloma. Primary plasmacytoma of lymph nodes is extremely rare and details of its clinical picture remain unclarified. We recently encountered an unusual case of EMP of lymph nodes that progressed to refractory multiple myeloma only 18 months later. A 74-year-old woman was admitted to our hospital because of a painless swelling in the right inguinal region. A tumor was removed surgically, and a histological diagnosis of EMP of the lymph nodes was made. Bence Jones protein (BJP) was detected in the urine, but there was no other evidence of systemic myelomatosis. The patient received local irradiation, which resulted in the elimination of BJP. Eighteen months later, however, a tumor developed in her right stemo-clavicular joint. A bone survey revealed multiple osteolytic lesions, and many atypical plasma cells were observed in the bone marrow, indicating multiple myeloma. The patient deteriorated despite several regimens of combination chemotherapy, and died four and a half years after the initial diagnosis of EMP.     

Duodenal localization of plasmablastic myeloma

Gastrointestinal involvement in plasma cell neoplasms,either as primary localizations(extramedullary plasma-cytomas) or as secondary involvement in systemic multiple myeloma, is a well-known event. Accurate histological examination is crucial in defining the diag-nosis. In this report, an uncommon case of duodenal localization of myeloma with plasmablastic features is described, with emphasis on the role of clinical data and findings from ancillary immunostaining techniques to avoid misdiagnosis.     

Late and localized extramedullary relapse of a light chain kappa myeloma after syngeneic bone marrow transplantation

We report the case of a 54-year-old female patient with stage IIIA kappa light chain myeloma (MM) who relapsed 7 years after syngeneic bone marrow transplantation (BMT). The relapse occurred as a voluminous soft tissue plasmacytoma in the leg, developing after local trauma. The patient was successfully treated with local radiotherapy and has remained progression-free for more than 2 years. This case represents one of the longest survivors, in complete remission, after syngeneic transplantation for MM. The presentation of recurrent disease as localized plasmacytoma with extramedullary growth is unusual in the post-transplant setting. Bone Marrow Transplantation (2000) 25, 115-117.     

P53 deletion may drive the clinical evolution and treatment response in multiple myeloma

We report a patient with multiple myeloma presenting with a paraspinal plasmacytoma with a marked dissociation between the response obtained in bone marrow (BM) infiltration and that achieved in soft tissue masses. While a complete remission was reached and maintained in BM, extramedullary plasmacytomas were refractory to every line of treatment. Genetic analysis identified the presence of t(4;14) and RB deletion in myeloma cells of both origins. However, a P53 deletion was only detected in plasma cells from extramedullary plasmacytomas. This finding suggests that P53 deletion has a role in the lack of treatment response of extramedullary plasmacytomas.     

Obstructing Plasmacytoma of the Duodenum: First Manifestation of Relapsed Multiple Myeloma

A patient with an obstructing, annular duodenal neoplasm is described. Percutaneous fine needle aspiration biopsy, and biopsy performed through an endoscope, confirmed that the lesion was an extramedullary plasmacytoma. The patient had been in clinical remission from multiple myeloma for the preceeding 13 months. Radiation therapy resulted in cessation of symptoms including gastrointestinal blood loss. After 5 months, recurrent obstructive symptoms and melena led to surgical extirpation of the tumor, with relief of all symptoms.     

Extramedullary plasmacytoma of the breast simulating breast cancer

A patient with a palpable mass in the breast suggestive of carcinoma underwent radical modified mastectomy. Surprisingly, histology of the tumor revealed an extramedullary plasmacytoma. Further diagnostic work up showed no evidence of underlying multiple myeloma. Among neoplastic lesions of the breast, although rare, malignancy of mesenchymal or lymphoproliferative origin should always be considered.