A solitary plasmacytoma of donor origin arising 14 years after kidney allotransplantation

Summary Allotransplantation of solid organs transfers passenger leucocytes which may give rise to a state of persistent microchimaerism. In this report we describe the case of a patient who developed a solitary plasmacytoma in a transplanted kidney more than 10 years after allografting. The diagnosis was established on the basis of the presence of a monoclonal IgG kappa peak in the serum, and light chain proteinuria, the plasmacytoid features of tumour cells including cell surface expression of IgG, kappa light chains, CD20, CD38 and CD56, the absence of lytic bone lesions and a normal bone marrow biopsy, and the disappearance of the monoclonal IgG peak after graft nephrectomy. A donor origin of the tumour was established by HLA DNA typing of tumour, tumour-free kidney tissue, and peripheral blood leucocytes, respectively.     

Sonographic Appearance of Plasmacytoma of the Testis

A case of solitary extramedullary plasmacytoma of the testis is presented demonstrating the appearances on ultrasound. The clinical implications of this diagnosis are discussed.     

Solitary extramedullary plasmacytoma of the premaxilla

Solitary extramedullary plasmacytoma is a rare tumour with radiotherapy playing an important role in its management. This report describes the case history of a man with a solitary extramedullary plasmacytoma at an extremely rare site, the premaxilla, that posed certain diagnostic and therapeutic dilemmas. The patient underwent surgery followed by postoperative radiotherapy. The use of two modalities of treatment for this localized tumour has been justified by briefly reviewing the literature and defining the various prognostic factors. These prognostic factors in turn should guide the treatment of these uncommon tumours.     

Multiple myeloma presenting as stridor: A case report

Extrame dullary plasmacytoma of larynx is extremely rare. We report a case of multiple myeloma which presented as extrame dullary manifestation in larynx. The rarity of incidence and difficulty in diagnosis of the disease is discussed.     

Five cases of orbital extramedullary plasmacytoma: diagnosis and management of an aggressive malignancy

Purpose: Multiple myeloma is an insidious haematological malignancy characterised by monoclonal proliferation of plasma cells in the bone marrow. Extramedullary plasmacytoma is a rare manifestation of multiple myeloma and usually occurs in the upper respiratory tract. Orbital involvement is particularly uncommon, but may be associated with devastating visual impairment and poor clinical outcomes. Therefore, this article aims to highlight the need for multidisciplinary management of orbital extramedullary plasmacytoma.

Methods: This is a retrospective observational case series of five patients. All presented to the authors for management of orbital extramedullary plasmacytomas from 2004 to 2015 at Prince of Wales and Mater Hospitals in Sydney, Australia. Medical records were reviewed for pertinent information including demographics, disease features, management strategy, and clinical progress. The study met Medical Ethics Board standards and is in accordance with the Helsinki Agreements.

Results: This case series of five patients underscores the poor prognosis of orbital extramedullary plasmacytoma. Despite aggressive multidisciplinary management, four of these five patients succumbed to their illness during the study period. However, multidisciplinary management did manage to minimise symptoms and preserve quality of life.

Conclusions: On a case-by-case basis, patients may derive palliative benefit from orbital surgery in conjunction with radiotherapy and chemotherapy. Orbital surgeons are encouraged to work within a multidisciplinary framework of medical specialists, including haematologists and radiation oncologists, when determining the optimal management plan in cases of orbital extramedullary plasmacytoma.     

Plasmablastic lymphomas with light chain restriction – plasmablastic extramedullary plasmacytomas?

J Oral Pathol Med (2010) 39 : 435–439 Background: It is diagnostically difficult to differentiate plasmablastic lymphomas (PBLs) from plasma cell neoplasms with plasmablastic differentiation. Plasmablastic lymphomas are currently classified as ‘PBL of the oral mucosa’ and ‘PBL with plasmacytic differentiation’. Methods: Forty‐five cases of PBL were retrieved from the Departments of Oral Pathology of the Universities of Pretoria and Limpopo, South Africa. Clinical features and HIV status were recorded and each case was classified as ‘PBL of the oral mucosa type’ or as ‘PBL with plasmacytic differentiation’. Immunohistochemistry included: CD45, CD3, CD20, CD79a, CD38, CD138, MUM1, Ki‐67 and kappa and lambda light chains. Positivity was recorded based on the percentage of positive staining cells as focal (5–20%); intermediate (20–70%) or diffuse (>70%). In situ hybridization was performed for Epstein–Barr virus (EBV) and HHV‐8. Results were recorded as positive or negative. Results: All cases showed some degree of plasmacytic differentiation. All were negative for CD20 with reactive T cells detected with CD3. Diffuse and strong positive staining was found with Ki‐67 and MUM1, but variable immunoreactivity was found with CD79a, CD45, CD38 and CD138. Twenty cases (47%) showed light chain restriction. Epstein–Barr virus was detected in 44/45 cases and HHV‐8 in none. Conclusions: The morphological classification of PBLs is not valid as all cases showed some degree of plasmacytic differentiation. We propose that PBLs with light chain restriction be reclassified as ‘plasmablastic extramedullary plasmacytomas’ and managed accordingly. The rest represents true PBLs. The true nature of these neoplasms as an entity should be further investigated with molecular and genetic studies.     

Clinical significance of vascular endothelial growth factor and hepatocyte growth factor in multiple myeloma

Angiogenesis is a crucial process in the progression of multiple myeloma (MM). Vascular endothelial growth factor (VEGF) and hepatocyte growth factor (HGF) are multifunctional cytokines that potently stimulate angiogenesis including tumour neovascularization. Serum levels of VEGF and HGF were measured in 52 patients with MM by enzyme-linked immunosorbent assay (ELISA). Serum levels of VEGF and HGF were elevated in MM patients compared with healthy controls (VEGF: mean 0.31 ng/ml and 0.08 ng/ml respectively, P < 0.01; HGF: mean 2.17 ng/ml and 0.45 ng/ml, respectively, P < 0.001). In serial samples taken after chemotherapy, serum VEGF and HGF levels were correlated with M-protein levels. Serum levels of VEGF were higher in patients with extramedullary plasmacytomas than in patients without them (P < 0.05). They were also significantly higher in a group of patients who showed poor response to chemotherapy (P < 0.01). Serum levels of HGF were higher in patients with complications such as anaemia, hypercalcaemia and amyloidosis than in patients without these complications (P < 0.01, P < 0.05, P < 0.05 respectively). Both serum VEGF and HGF levels were significant predictors of mortality (P = 0.01, P = 0.02, respectively, log-rank test). The present study demonstrated that serum levels of VEGF and HGF are significantly elevated and dependent on the severity of MM, suggesting that measurement of VEGF and HGF may be useful for assessing disease progression and for predicting the response to chemotherapy in MM patients.     

Testicular plasmacytoma with bone dissemination without medullary plasmacytosis

A 34-year-old man was diagnosed as having solitary testicular plasmacytoma. He had received palliative radiotherapy, several combined chemotherapies including CHOP chemotherapy (vincristine, cyclophosphamide, Adriamycin, and prednisone), MP (melphalan and prednisone) and M-2 protocol (melphalan, prednisone, vincristine, carmustine, and cyclophosphamide), and interferon therapy as 3 million units subcutaneous injection three times a week for 1 year. Extensive bone plasmacytoma developed 7 years later without bone marrow involvement. We suggest that early use of combined chemoradiotherapy and high-dose chemotherapy with autologous stem cell support should be investigated in patients with testicular plasmacytoma with dissemination.     

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.     

Radiotherapy in the management of solitary extramedullary plasmacytoma

BACKGROUND: Solitary extramedullary plasmacytoma (SEP) is a rare tumour for which the standard treatment remains local radiotherapy (RT). We present a study of a multi-institutional experience, between 1980 and 1999, in an attempt to better understand the natural history of SEP and to identify possible prognostic factors. METHODS: The records at Radiation Oncology Victoria and Peter MacCallum Cancer Centre, Melbourne, were used for the identification of patients. The inclusion criteria were as follows: (i) histological confirmation of clonal plasma cells involving a single extramedullary site with or without lymph node involvement; (ii) no histological evidence of bone marrow involvement; (iii) no evidence of distant bone lesion on radiographic skeletal survey (bone erosions adjacent to the primary thought to be due to contiguous involvement were permitted) and (iv) no anaemia, hypercalcaemia or renal impairment due to plasma cell dyscrasia. RESULTS: A total of 16 patients was identified, with a median follow up of 66 months (range 12-211 months). The head and neck region accounted for the majority of presentations (88%). Among all patients, a serum mono-clonal paraprotein was found in three patients and bone erosion was identified in seven patients. All patients received local RT, although two patients also received elective nodal irradiation (ENI). The median RT dose was 45 Gy (range 40-50.4 Gy). Local control was achieved in all patients (100%), however, regional recurrence outside the RT fields occurred in 2/16. Multiple myeloma (MM) developed in five patients, all within 5 years. The predicted 10-year myeloma free survival is 75% and 10-year overall survival is 54%. CONCLUSION: RT can achieve excellent local control of SEP. The rate of conversion to MM is 31%. Moderate dose RT of at least 40 Gy using limited radiation fields is recommended, although ENI should be considered if toxicity is not increased.