Solitary extramedullary plasmacytoma (EMP) is a plasma cell neoplasm without bone marrow involvement. EMPs are rare in the gastrointestinal (GI) tract. We report two cases of primary EMP, one in the transverse colon and the other in the stomach. In the first case, a mass on the transverse colon was found on colonoscopy. The patient underwent left hemicolectomy and has been followed-up for 3 years without recurrence postoperatively. The latter case had several masses in the stomach. The patient underwent bypass surgery and has received supportive care for 1 month. Histopathologic specimens of both the cases showed a monoclonal lambda chain EMP. Subsequent investigations included a bone marrow biopsy, serum IgA, IgG, IgM and serum protein electrophoresis, and the results were negative for multiple myeloma in both the cases. Solitary EMP in the GI tract can be mistaken for colon cancer or stomach cancer on endoscopy; therefore, a sufficient number of biopsy specimens can help diagnose solitary EMPs. Surgical resection alone or with radiation therapy in cases with positive surgical margin is currently the only treatment for solitary EMP in the GI tract. Further study is necessary to determine disease prognosis and to investigate other treatment methods.
Colonic Neoplasms/*diagnosis/pathology/therapy ; Endoscopy, Gastrointestinal ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Plasmacytoma/*diagnosis/pathology/therapy ; Positron-Emission Tomography ; Stomach Neoplasms/*diagnosis/pathology/therapy ; Tomography, X-Ray Computed

OBJECTIVE: To investigate the clinical manifestation, treatment and prognosis of extramedullary plasmacytoma(EMP) in the upper airway, and to improve the diagnosis and outcome of EMP treatment. METHOD: Clinical data of 26 EMP cases were reviewed retrospectively, and then compared with multiple myeloma(MM) patients presenting with lesions in upper airway. RESULT: Of 26 cases, 9 cases with the tumors occurred in nasal cavities, 7 in nasal sinuses, 6 in pharyngeal, 4 in throat, mainly manifesting with local masses and relevant symptoms. The manifestations of clinical, endoscopy findings and pathologic results in EMP patients were not distinguishable from the lesions of MM patients, while MM patients often accompanied by other findings, such as anemia and bone damage. Involvement of neck lymph nodes was more common in MM patients than in EMP patients. Ten patients were treated with surgery, and 16 patients with surgery and radiotherapy. Of the seven EMP patients with involvement of neck lymph nodes, four patients received additional chemotherapy besides surgery and radiotherapy, and no local relapse and MM happened in them, while of the three patients only received surgery and radiotherapy, one local relapse were found and one progressed to MM. CONCLUSION The diagnosis of EMPs mainly depends on pathological results. The judgment of pathologists and application of molecular biology technology are vital for the diagnosis of EMP in upper airway, and MM must be excluded very carefully in the diagnosis of EMP. Surgery combined with radiotherapy is the main treatment for EMP in the upper airway, and the prognosis is good but the follow-up should be taken. Besides surgery and radiotherapy, chemotherapy is beneficial for the EMP patients accompanied with lesions in neck lymph nodes.
Adult ; Aged ; Female ; Humans ; Lymph Nodes ; pathology ; Male ; Middle Aged ; Multiple Myeloma ; diagnosis ; pathology ; therapy ; Nasal Cavity ; pathology ; Plasmacytoma ; diagnosis ; pathology ; therapy ; Prognosis ; Respiratory Tract Neoplasms ; diagnosis ; pathology ; therapy ; Retrospective Studies ; Young Adult

Aged ; Diagnosis, Differential ; Dura Mater ; pathology ; ultrastructure ; Female ; Humans ; Lymphoma, B-Cell, Marginal Zone ; diagnosis ; pathology ; therapy ; Magnetic Resonance Imaging ; Male ; Meningioma ; pathology ; Middle Aged ; Plasma Cells ; ultrastructure ; Plasmacytoma ; pathology ; Tomography, X-Ray Computed

BACKGROUND: Kaposi's sarcoma-associated herpesvirus (KSHV) been shown to be associated with human diseases including Kaposi's sarcoma, pleural effusion lymphoma, multicentric Castleman's disease. The IL-6 may both stimulate myeloma growth and prevent apoptosis of malignant plasma cells. Interestingly, viral IL-6(vIL-6), homolog to human interleukin-6(IL-6) in KSHV genome retains biologic activity. Thus, oncogenic role of the KSHV has been proposed as a pathogenesis of the multiple myeloma. We used ISH to determine the frequency of patients with multiple myeloma and plasmacytosis associated with KSHV-infected BM cells in fresh core biopsies and to determine the correlation between KSHV infection and clinical characteristics. METHODS: Bone marrow(BM) biopsy samples from 16 cases of multiple myeloma, 2 cases of monoclonal gammopathy of undetermined significance(MGUS) were obtained from the pathology division of Soon Chun Hyang University Hospital, Seoul, Korea. Biopsy sample of Kaposi's sarcoma for positive control and BM biopsy samples of myelodysplastic syndrome(MDS) and malignant lymphoma for negative control were obtained. Bitinylated probe to KSHV were prepared with the following sequences: 5' to 3' TGCAGCAGCTGTTGGTGTACCACATATCT. and in situ hybridization (ISH) was performed. RESULTS: Among the 18 patients. Two patients were MGUS and among 16 patients with multiple myeloma, 1 in stage IB disease, 1 stage IIB disease, 8 stage IIIA disease, 4 stage IIIB diseases and 2 in variant of multiple myeloma, extramedullary plasmacytoma. Strong positive signal was detected in nuclei and cytoplasm of the malignant cells of biopsy sample from 1 cases of Kaposi's sarcoma by ISH(positive control). Signal was not detected in BM biopsy samples of 7 cases from MDS and malignant lymphoma(negative control). Among 16 patients with multiple myeloma, 15 demonstrated viral positive cells and 2 cases with MGUS also showed viral positive cells by ISH. Signal was detected in nuclei and cytoplasm of stromal cells. Signal was strongly detected in MGUS than multiple myeloma. Positivity of the KSHV was not related with stage of the patients with multiple myeloma. One patients with multiple myeloma was studied at diagnosis and after chemotherapy. After chemotherapy KSHV was not detected. CONCLUSION: In MGUS and multiple myeloma, KSHV infects the stromal cells of BM rather than malignant plasma cells. On the basis of these data, we have supposed KSHV to play a role in transformation from MGUS to multiple myeloma. Particularly, due to the fact that signal of ISH was strongly detected in MGUS and was not detected in one case with multiple myeloma, it was presumed that KSHV was not major role in already advanced multiple myeloma but statistic significance was not demonstrated because of small numbers of cases. Further studies to reveal the correlation of KSHV and pathogenesis of multiple myeloma are needed.
Apoptosis ; Biopsy ; Cytoplasm ; Diagnosis ; Drug Therapy ; Genome ; Giant Lymph Node Hyperplasia ; Herpesvirus 8, Human ; Humans ; In Situ Hybridization ; Interleukin-6 ; Korea ; Lymphoma ; Multiple Myeloma* ; Paraproteinemias ; Pathology ; Plasma Cells ; Plasmacytoma ; Pleural Effusion ; Sarcoma, Kaposi ; Seoul ; Stromal Cells

Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Humans ; Leukemia, Myeloid ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Plasmacytoma ; drug therapy ; metabolism ; pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; Vincristine ; therapeutic use