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Solitary plasmacytomas of the occipital bone: a report of two cases.

The radiological appearances of two cases of solitary plasmacytoma in the occipital bone are described. One arose in the lateral part and the other in the squama. They showed characteristic radiological features on CT, MRI and angiography. Bone scintigraphy and gallium scintigraphy were also available. Solitary plasmacytoma of the skull is a rare condition and usually occurs in the calvarium. The skull base is an extremely rare site and only four cases have been reported. The literature of solitary plasmacytoma of the skull is reviewed.

Aged↗

Testicular plasmacytoma.

The case of a sixty-five-year-old man with multiple myeloma and a testicular plasmacytoma is described. This represents the thirty fourth reported case of testicular plasmacytoma and the first in which immunoperoxidase histochemistry has been used to demonstrate that the testicular plasma cells contain immunoglobulin of the same isotype as the patient's paraprotein. The clinical and morphologic features of previously reported testicular plasmacytoma are reviewed.

Aged↗

Novel myc oncogene RNA from abortive immunoglobulin-gene recombination in mouse plasmacytomas.

We have found that the myc oncogene has been modified by abortive recombination with the alpha heavy-chain immunoglobulin constant-region (C alpha) gene in five different mouse plasmacytoma lines. Recombination occurred approximately 0.8-2.0 kb to the 5' side of two distinct coding regions, defined by sequence homology between the chicken cellular and plasmacytoma myc genes. The myc and C alpha genes were always in opposite transcriptional orientation, with the recombination site within the C alpha switch region sequences. DNA recombination was found to correlate with the production of a novel 2.1 kb species of myc RNA that was 0.4 kb shorter than the normal cellular transcript. No elevated levels of myc RNA were evident, suggesting that DNA rearrangements have altered the myc oncogene product. This oncogene activation corresponds to the chromosomal translocations found in nearly all plasmacytomas.

Animals↗

Computed tomography in the diagnosis of extramedullary plasmacytoma.

Extramedullary plasmacytomas involving the retroperitoneum are rare. Review of the literature reveals only one case in which computed tomography (CT) findings were illustrated, and this case involved the pancreas. Described here is unusual case in which pancreatic and renal plasmacytomas occurred in a patient who 18 months previously had presented with a solitary plasmacytoma of bone. The clinically unsuspected renal masses was diagnosed by CT, allowing easy biopsy and radiotherapy planning.

Bone Neoplasms↗

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.

Adult↗

Extramedullary plasmacytoma confined to the choroid.

PURPOSE: To report a case of an extramedullary plasmacytoma confined to the choroid. METHODS: Interventional case report with cytopathologic correlation. RESULTS: A 76-year-old asymptomatic woman presented with 2 globular orange and amelanotic choroidal lesions in the right eye. Fine needle aspiration biopsy of one of the choroidal lesions followed by immunocytochemical studies revealed monoclonal plasma cells, diagnostic of plasmacytoma. Systemic evaluation was negative, except for increased serum gamma globulin with immunoglobulin kappa spike on electrophoresis. The patient was managed by ocular external beam radiotherapy. At 9 years follow-up, she was free of local disease and never developed multiple myeloma. CONCLUSION: Extramedullary plasmacytoma confined to the choroid is rare. It may present simulating other common amelanotic choroidal lesions. Fine needle aspiration biopsy and proper systemic evaluation are critical in establishing the diagnosis.

Aged↗

Bilateral testicular plasmacytoma.

A case is reported of a 55-year-old man with asynchronous bilateral testicular plasmacytoma. Despite chemotherapy and radiotherapy he died seven and a half years after the original diagnosis of testicular plasmacytoma, and 13 months after developing evidence of disseminated myeloma. The testicular plasmacytomas may have arisen as a result of seeding from a systemic myelomatous process.

Humans↗

Plasmacytoma of the urethra treated with transurethral resection and radiotherapy.

Plasmacytoma, which is a localized malignant plasma cell tumor, occurs rarely and is found mostly in the upper airways of older patients. Two urethral plasmacytomas have been reported, 1 of which was a primary lesion. We describe a primary plasmacytoma of the urethra in a 23-year-old woman who had been exposed to diethylstilbestrol in utero. The patient underwent transurethral resection of the tumor and radiation therapy and has been free of disease for more than 10 years.

Adult↗

Primary extramedullary plasmacytoma of the omentum associated with recurrent adenocarcinoma of the colon: first case report.

Extramedullary plasmacytomas are rare tumors, with 272 cases reported in the literature in English. Less than 10 per cent of these are located in the gastrointestinal tract. This report documents the first primary plasmacytoma of the omentum in association with a recurrent colonic adenocarcinoma. Extramedullary plasmacytoma, a B cell neoplasm, characteristically arises in areas containing lymphoid tissue. In our case the tumor most likely arose in a lymph node or nodes in the omental fat, with subsequent replacement of the entire greater omentum and involvement of the colonic serosa by direct extension. Although the extent and nature of the association between myeloma and carcinoma remain obscure, a review of the literature suggests that such association may occur more frequently than has been supposed. Further investigation would appear to be warranted.

Adenocarcinoma↗

Extramedullary plasmacytoma arising from an adrenal gland.

Extramedullary plasmacytoma is a rare malignant neoplasm typically arising outside the bone marrow of patients who show no clinical evidence of multiple myeloma. We report a rare case of extramedullary plasmacytoma that was primarily found in a right adrenal lesion. Surgical excision and radiotherapy were performed for treatment of the primary lesion. Although laboratory examinations demonstrated the presence of M-protein and elevation of monoclonal IgG-lambda, a surgically excised mass revealed monoclonality of IgG-kappa. We therefore diagnosed extramedullary plasmacytoma independent of simultaneous benign M proteinemia, because the types of M-protein were quite different. One year after radiotherapy, no local recurrence was observed.

Adrenal Gland Neoplasms↗

Plasmacytoma-refractory BALB/cAnPt mice have naive T cell and highly specific B cell responses to antigen.

Recently, a reduction in the incidence of pristane-induced plasmacytomas in BALB/cAnPt (BALB/c) mice that were kept in viral specific pathogen-free (SPF) conditions has been reported. Environmentally, these SPF-BALB/c mice differed from conventionally-housed (CON) mice only in viral exposure and diet (i.e. sterilization of mouse chow), since microbial colonization of the intestinal tract was seen to be equivalent. This report assessed the ability of SPF- and CON-BALB/c mice to respond to immunologic challenge with soluble antigen, i.e. hen egg white lyzosyme (HEL), as a means of evaluating differences in T and B cell function and, indirectly, evaluating the possible effects these differences might have on plasmacytoma development. When cultured in vitro for 5 days with HEL, HEL-primed lymph node cells (LNC) from SPF-BALB/c mice proliferated to a significantly lesser extent than HEL-primed CON-BALB/c LNC. Moreover, HEL-induced production of IFN-gamma and IL-5 was significantly lower in SPF LNC. Serum IgG1 levels were 10-fold lower in SPF-BALB/c mice with, or without prior immunization with HEL and were not reconstituted by repeated injections of HEL in adjuvant. Serum IgM levels of SPF- and CON-BALB/c mice were equivalent. This reduction in immune responses could not be attributed to a lack of colonization of secondary lymphoid organs, since flow cytometric analysis of LNC revealed no difference in the number of recoverable cells and the proportion of lymphocyte subsets (CD4+, CD8+ and CD45+ cells) obtained from SPF- and CON-BALB/c mice. However, only CON LNC were induced to increase surface expression of CD44 after antigenic or mitogenic stimulation in vitro. Antibody responsiveness to HEL, as evidenced by serum anti-HEL binding or splenic hybridoma studies, demonstrated higher levels of IgG1 antibodies in CON BALB/c mice than in SPF mice. However, a greater proportion of the SPF IgG1 antibodies present were specifically directed against HEL, so that specific activity was greater in SPF-BALB/c mice. Therefore, while SPF BALB/c mice have a more restricted response to HEL than CON-BALB/c mice, those antibodies that are produced are more specifically directed against HEL with very little apparent bystander/polyclonal activation of multireactive cells. Resistance to plasmacytomas in SPF-BALB/c mice, therefore, may stem from a reduced number of circulating memory T and B cells, which are capable of reacting and/or crossreacting with a chronic inflammatory stimulus.

Animals↗

Concomitant downregulation of IgH 3' enhancer activity and c-myc expression in a plasmacytoma x fibroblast environment: implications for dysregulation of translocated c-myc.

Regulation of immunoglobulin heavy chain (IgH) gene expression is controlled by a B cell-specific promoter, intronic enhancer and additional B cell-specific enhancer elements identified recently in the 3' end of the IgH locus. One of the latter elements, the IgH 3' enhancer, is of particular interest: (1) it is B cell-specific and active only in late B cell development; (2) in rodent plasmacytomas and in some human Burkitt's lymphomas it is part of a locus control region (LCR) that is involved in deregulation of the c-myc oncogene as a result of translocation into the IgH locus; and (3) it has been implicated in the mechanisms that control Ig gene class switch recombination. We have used a somatic cell hybridization approach to genetically analyse regulation of the activity of the IgH 3' enhancer. When mouse MPC11 plasmacytoma cells, in which the IgH 3' enhancer is active, are fused with fibroblasts, Ig expression is extinguished at the level of transcription. Here we show that in a MPC11 plasmacytoma x fibroblast environment, the IgH 3' enhancer is transcriptionally inactive. Furthermore, we demonstrate that binding of several B cell-specific transcription factors, essential for IgH 3' enhancer activity, is lacking, which may explain 3' enhancer inactivity, although the binding of repressors cannot be excluded. Moreover, the high expression level of c-myc, characteristic of the parental MPC11 cells carrying the t(12;15) translocation, is down-regulated in the hybrids to that in unfused fibroblasts. Therefore, inactivation of the IgH 3' enhancer is a multifactorial process affecting several transcription factors that control the cell-specific and developmental activity of the enhancer.

Animals↗

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.

Aged↗

Extramedullary plasmacytoma in cardiac transplant recipients.

Extramedullary plasmacytomas are an immunoproliferative, monoclonal disease of B-cell lineage and are classified as non-Hodgkin's lymphomas. Cutaneous extramedullary plasmacytomas are rare. We report 2 cases of transplantation-associated cutaneous extramedullary plasmacytomas in a setting of chronic immunosuppression.

Diagnosis, Differential↗

Primary plasmacytoma of the skin.

Primary plasmacytoma of the skin without evidence of bone marrow plasmacytosis is a rare disorder belonging to the heterogenous spectrum of plasma cell neoplasms. With immunohistochemical techniques, differentiation from benign plasma cell aggregates can be accomplished by demonstrating the monoclonality of tumor cells. We describe a patient in whom a solitary primary cutaneous plasmacytoma developed on the left thigh. Immunohistochemically, plasma cells showed restriction of immunoglobulin lambda-chain expression. Underlying multiple myeloma was excluded by serum protein and immunoglobulin electrophoresis, roentgenographic skeletal survey, and bone marrow biopsy. The tumor responded well to local electron-beam radiotherapy. Whereas multiple primary cutaneous plasmacytomas seem to carry a significant early mortality rate, the prognosis of solitary lesions is likely to be more favorable.

Female↗

Plasmacytoma of the oral cavity and jaws: a clinicopathologic study of 13 cases.

Plasmacytoma of the jaw bones and oral cavity, as in other anatomic sites, comprises three distinct entities: multiple myeloma, solitary plasmacytoma of bone, and extramedullary plasmacytoma. This article is a retrospective study of 13 cases; 9 occurred in the mandible and 4 in the maxilla. The most common radiographic finding was a radiolucency that many times was superimposed on the roots or apices of nearby teeth. The most common symptom was localized pain, and the most frequent clinical sign was a raised red lesion on the alveolar ridge. The disease affects older persons, and the posterior mandible is the most frequently reported location. The degree of dysplasia of tumor cells was evaluated as there is a reported correlation with survival rates.

Adult↗

Pulmonary plasmacytomas: a clinicopathologic and immunohistochemical study of five cases.

Five cases of primary plasmacytoma of the lung are presented. The patients were four men and one woman between the ages of 50 and 79 years (mean age, 57 years; median age, 54 years). Two patients presented with symptoms related to their tumor; these included cough, dyspnea, and hemoptysis. In two patients, the tumor presented as a hilar mass, whereas in the remaining three patients, the tumor was located intraparenchymally. Clinically, only one patient had a reported monoclonal gammopathy (IgG, kappa). Because of the proximal location of these tumors, three patients underwent pneumonectomy; one other underwent a lobectomy, and one had a segmental resection. Grossly, the tumors ranged from 2.5 to 8 cm in maximum diameter (mean, 4.4 cm); they were either peribronchial or involved a major bronchus. Histologically, they were characterized by sheets of plasma cells that were well differentiated in two cases and moderately differentiated in three. Amyloid was present in two cases. In four tumors, there was a monoclonal population of lambda light chain-bearing plasma cells, whereas in one, the plasma cells expressed a monoclonal kappa light chain. The tumor cells predominantly expressed IgG heavy chains in two cases. Peribronchial and mediastinal lymph nodes were involved in three cases. Follow-up information ranged from 4 days to 262 months (average, 115 months; median, 36 months). Two patients survived more than 20 years before dying of non-tumor-related causes. Two patients died 28 months and 4 days after surgery with concurrent tumor in liver and mediastinal and para-aortic lymph nodes. Comparing the present cases and those reported in the literature, we noted that the patients herein presented are, on average, older than those published previously. Combining our cases with 14 other verifiable, previously published cases, the overall 2- and 5-year survivals of pulmonary plasmacytomas are 66% and 40%, respectively. Patients with pulmonary plasmacytomas can have a long-term survival, as evidenced by two of our patients who survived 20 or more years.

Aged↗

Primary extramedullary plasmacytoma of the parotid gland: a case report and review of the literature.

Solitary extramedullary plasmacytomas are uncommon malignant neoplasms with a great predilection for the upper respiratory tract. Extramedullary plasmacytoma involving the parotid gland is an extremely rare condition. This report describes the case of a primary extramedullary plasmacytoma arising in the right parotid gland of a 63-year-old man. A review of the literature reveals that this disease has generally a good prognosis. provided that multiple myeloma has been excluded. The treatment of choice should be surgical excision followed by radiotherapy, although from this study there is insufficient evidence to suggest that this is more effective than surgery alone. All patients must have regular long-term follow-up to monitor for recurrence or dissemination, in which event chemotherapy may be considered in addition to the other treatment modalities.

Diagnosis, Differential↗