1/7. A surgical case of solitary plasmacytoma of rib origin with biclonal gammopathy.Localized solitary plasmacytoma of the bone (SPB) is a rare disease and is characterized by only one or two isolated bone lesions with no evidence of disease dissemination. A previously healthy 44-year-old male was admitted for evaluation of an abnormal radiographic shadow in the left middle lung field with symptoms of left back pain. Radiological evaluation revealed a peripheral opacity in the left chest wall, which was highly suspected to be a chest wall tumor. CT-guided transcutaneous needle biopsy of the tumor was performed and the specimens showed a monomorphous population of mature plasma cells. The bone marrow biopsy findings revealed no evidence of myeloma and bone scanning revealed only abnormal accumulation in the left seventh rib. He had mild M-proteins in a urine sample and Bence-Jones protein was detected. immunoelectrophoresis revealed mild biclonal gammopathy of Bence-Jones protein of both the kappa and lambda light-chain types. Under a diagnosis of solitary bone plasmacytoma, preoperative radiation therapy with doses of 40 Gy for the tumor was performed. He underwent complete en bloc resection of the chest wall, including one-third of the left sixth and seventh ribs, the intercostal muscle and the parietal pleura. The protein abnormalities in the urine sample disappeared following surgical resection. Adjuvant chemotherapy using melphalan and prednisolone was performed. He is doing well without evidence of tumor recurrence 2 years following his initial diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Solitary plasmacytoma of the spine in an adolescent: a case report.Solitary plasmacytoma of bone is a rare disease in patients younger than 30 years, and only a few cases have been reported in children and adolescents. We report the findings of a destructive lesion of the spine due to solitary plasmacytoma in an adolescent patient.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Fine-needle aspiration cytology of a para-aortical solitary plasma-cell tumor.Extramedullary plasma-cell tumor (EMP) is a very rare disease and mainly arises in the head and neck area. An EMP arising in the paraaortical space was diagnosed by fine needle aspiration cytology and immunocytochemistry, in a 48-year-old male. Smears were cellular and showed dissociated monomorphic plasma cells. Immunocytochemistry demonstrated monoclonal expression of kappa-light immunoglobulin chain and CD38 positivity. Cytomorphology and immunocytochemical profile allowed a definitive diagnosis of plasmacytoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Solitary bone plasmocytoma of the spine in an adolescent.Solitary plasmocytoma (SP) represent only about 5% of plasma cell neoplasia. Most patients have generalized disease, that is, multiple myeloma (MM). Solitary bone plasmocytoma (SBP) is a localized plasma cell tumor and is a very rare disease in young patients. We reported here, a case of SPB in a 14-year-old girl with a 10-year disease-free survival after an aggressive treatment. The relationship of SBP to MM continues to be controversial. Recommendations on the diagnosis and management of SBP in adults, based on a literature search and consensus of expert opinion, were recently published on behalf of the Guidelines Working Group of the United Kingdom Myeloma Forum 1. MRI of the spine is necessary to assess local disease. radiotherapy with doses of 45-50 Gy is the recommended treatment and gives a high rate of local control (83-96%). Chemotherapy remains controversial in contrast to MM, in which intensive chemotherapy with autologous bone marrow transplantation (ABMT) is widely accepted. At the present time, considering the good prognosis of patients with a normal MRI at diagnosis and a complete disappearance of the M protein after radiotherapy, we believe that ABMT should be reserved for relapse or primary therapeutic failure.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Targeting T cells to hit B cells: successful treatment of cutaneous plasmacytosis with topical pimecrolimus.Cutaneous plasmacytosis is a rare disease predominantly found in Japanese patients. We describe the case of a 75-year-old white female with cutaneous plasmacytosis of the face and involvement of the bone marrow. In contrast to other cases of cutaneous plasmacytosis, the patient revealed hypogammaglobulinemia and elevated levels of free light chains in the urine. Treatment with topical pimecrolimus 1%, which primarily targets T cells, led to almost complete clinical and histological remission of the skin lesions. Our report indicates that the therapeutically induced disappearance of the plasma cells was due to indirect T-cell-mediated effects.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Detection of human papillomavirus type 16 in plasma cells.Chronic benign plasma cell tumor of the cervix, also called chronic plasma cell cervicitis, is a rare disease of unknown etiology, characterized by a heavy infiltration of plasma cells forming granulation tissue. To identify infectious agents associated with this disease, we extracted and analyzed dna from the 17-year-old paraffin section of the original case report and from granulation tissue surgically removed from a patient at our institution with a chronic benign plasma cell tumor. The dna from both patients was shown by a polymerase chain reaction (PCR) technique to contain a human papillomavirus 16 (HPV 16) sequence. Genomic Southern analysis of the fresh-frozen tissue confirmed the initial PCR finding. in situ hybridization further demonstrated that the HPV 16 was present in the plasma cells and not a contaminant from the surrounding epithelial tissue. The etiological role of HPV 16, an oncogenic virus associated with cervical carcinoma, in this disease is not yet clear. Our results, however, suggest that the types of cells that are infected by HPV may include cells of lymphoid origin, and that HPV may be associated with chronic benign plasma cell tumors of the cervix.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Endobronchial extramedullary plasmacytoma. Report of one case.A patient underwent a subtotal resection of the tracheobronchial carina for an obstructing endobronchial lesion. Preoperative biopsies of the lesion were not diagnostic. After resection, the histological examination of the specimen removed demonstrated an extramedullary plasmacytoma infiltrating the bronchial wall. Immunohistochemical studies showed monoclonality for kappa light chains. The postoperative course was uneventful and the screening for multiple myeloma was negative. No adjuvant treatment was given and the patient is currently alive and free of disease 63 months after the resection. Primary endobronchial plasmacytoma is a very rare disease: it is unclear which is the best treatment for endobronchial plasmacytoma. However, complete surgical resection has allowed a long-term survival, free of disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |