Solitary Plasmacytoma Of Bone Research Articles

Pathological bone fractures in a 20‐year old athletic male with multifocal solitary plasmacytoma of bone

The patient is a 20-year male who presented for neck pain 2 weeks after he fell while playing football. Commuted tomography (CT) scans revealed a lytic and expansile lesion of the right lateral C1 vertebrae with a linear fracture (Image 1A). The magnetic resonance imaging (MRI) demonstrated a diffuse signal abnormality consistent with a pathologic fracture (Image 1B). After an inconclusive fine needle aspirate examination, the radiological findings were reviewed, and the lesion was thought to be likely an aneurysmal bone cyst. The vertebral fracture was thereafter repaired with a C3 to occiput fusion (Image 1B, inset) without a hematology referral. Approximately 15 months after surgery, the patient developed intermittent deep groin pain exacerbated by exercise, and the region was further traumatized after a fall in the shower. Radiological evaluation (CT) revealed a large lytic acetabular lesion of right hip (Image 1C) and an additional lytic lesion of the left first rib. The radiological differential diagnosis remained the same, including aneurysmal bone cyst, giant cell tumor, and eosinophilic granuloma. A biopsy of the acetabular lesion unexpectedly showed sheets of abnormal plasma cells (Image 1D1), which were positive for CD38, CD138 (Image 1D2), CD56 and kappa light chain (Image 1D3), but negative for lambda light chain (Image 1D4). The plasma cells were negative for CD19, CD20, and cyclin D1. Fluorescence in situ hybridization demonstrated monosomy 13 in 55% and loss of IGH gene locus in 77% of interphase cells. Subsequent laboratory tests demonstrated serum free kappa light chain (160 mg/dL; reference range, 0.33–1.94) and urine free kappa light chain (Bence Jones protein, 643 mg/dL/24 hr) (Image 1E). Serum β-microglobulin was 2.0 mg/L (reference: <2), and complete blood cell count, renal function and metabolic profile including serum calcium were within normal limit. Bone marrow biopsy revealed no evidence of plasma cell neoplasm. A diagnosis of solitary plasmacytoma of bone was thus established. This patient was treated with local radiotherapy and systemic chemotherapy. He was started on four cycles of RVD (Lenalidomide, Bortezomib, and dexamethasone) and then high dose chemotherapy followed by autologous stem cell transplant, and responded well. Pathological bone fractures caused by multifocal solitary plasmacytoma of bone. A. Horizontal section of CT scan of the neck showing a lytic lesion with a linear fracture of C1 vertebrae (arrow). B. Coronal section of MRI scan showing a diffuse signal abnormality consistent with pathological fracture (arrow) and surgical repair (Inset). C. CT scan showing a large lytic acetabular lesion of right hip (arrow). D. Histological and immunohistochemical studies of right acetabular lesion biopsy showing sheets of plasma cells (D1, H&E stain) which are positive for CD138 (D2) and kappa light chain (D3), but are negative for lambda light chain (D4). E. Urine protein electrophoresis showing Bence Jones protein in gamma region (shaded peak area). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] Plasma cell neoplasm tends to occur in elderly [1], and patients younger than 30 years are extremely rare. Although radiographic scans demonstrated evidence of bone lytic lesions in our case, a diagnostic possibility of solitary plasmacytoma was initially overlooked, primarily due to the extremely young age of the patient. It has been reported that patients of 40 years or younger with plasma cell neoplasms frequently present with macrofocal plasma cell myeloma, often preceded by solitary plasmacytoma of bone. Studies have demonstrated a favorable clinical outcome in patients of this age group [2]. This case emphasizes a hematology referral and diagnostic differential of plasma cell neoplasms regardless of patient's age if bone lesions are present.

Cranial Plasmacytoma: A Case Series and Review of the Literature

Plasmacytomas are malignant proliferations of plasma cells which can occur with different plasma cell dyscrasias. Solitary plasmacytomas of bone or extraosseous plasmacytomas, depending on the tissue of origin, develop in isolation without systemic manifestations of multiple myeloma. Three cases of cranial plasmacytomas are described, two in patients with multiple myeloma and one extraosseous plasmacytoma. Management options are discussed.

The role of helical tomotherapy in the treatment of bone plasmacytoma

We evaluated the early clinical outcome of patients with solitary bone plasmacytoma (SP) or a solitary lesion of multiple myeloma (MM) treated with helical tomotherapy (HT) compared with 3D conformal radiotherapy (3D-CRT), in terms of target coverage and exposure of critical organs. Ten patients with SP and 3 patients with a solitary lesion of MM underwent radiation therapy (RT) delivered by HT, to a dose of 40 Gy in 20 fractions. Treatment planning was then performed with 3D-CRT and the dosimetric parameters of both techniques were compared. Patients were also assessed for response to treatment and acute toxicities. With a median follow-up of 13 months, 78% of patients with pain before RT had resolution of their symptoms. Coverage of target lesion was adequate with both techniques in 12 of 13 patients. Target coverage was significantly lower for HT (V95% = 98.55% vs. 97.15%; p = 0.04, for 3D-CRT and HT, respectively). Target overdoses were also lower with HT (V105% = 2.01% vs. 0.19%; p= 0.16), although nonsignificant. Finally, there were no significant differences in organs-at-risk irradiation between both techniques. The early treatment tolerance was excellent, with no toxicity higher than grade I. RT of SP and MM with a solitary lesion can be safely delivered with HT, with no major acute side effects and good symptomatic control. Finally, HT provides a dosimetry similar to that of 3D-CRT in terms of organs-at-risk sparing and target volume coverage.

Solitary Plasmacytoma: A Review Of Clinical, Ocular, Neurological And Other Systemic Manifestations

Solitary plasmacytomas are defined as proliferation of monoclonal plasma cells without evidence of significant bone-marrow plasma-cell infiltration. They are classified according to location into solitary plasmacytoma of bone if they occur in bone, and extramedullary plasmacytoma if they arise in soft tissues. They are more frequent in males than females and have a peak incidence in the fifth and sixth decade of life. Solitary plasmacytoma accounts for about 5% of plasma cell tumours. More than 80% of extramedullary plasmacytoma occur in the upper aero-digestive tract or the head and neck region. Diagnosis is based on histologic confirmation of monoclonal plasma cell infiltration of a single disease site and on the exclusion of systemic myeloma. The development of multiple myeloma from solitary plasmacytoma occurs in approximately 60% of patients within 10 years of diagnosis. Prognosis is good and cure can be attained in extramedullary plasmacytoma. Therapeutic options include radiotherapy, chemotherapy and surgical excision. The aetiology of plasmacytoma is still unknown. Extramedullary plasmacytoma can affect practically all the systems in the body including the eyes, nervous system, head and neck, respiratory system, breast, gastrointestinal system, urogenital system and lymph nodes. These systemic manifestations are reviewed. Key words: Plasmacytoma, ocular, outcome, neurological, prognostic factors

Advances in Imaging and the Management of Myeloma Bone Disease

Osteolytic disease is a major complication of multiple myeloma that may lead to devastating skeletal-related events (SREs). Conventional radiography remains the gold standard for the evaluation of bone disease in patients with myeloma. However, whole-body magnetic resonance imaging (MRI) is recommended in patients with normal conventional radiography and should be performed as part of staging in all patients with a solitary plasmacytoma of bone. Urgent MRI is also the diagnostic procedure of choice to assess suspected cord compression, whereas computed tomography can guide tissue biopsy. Positron emission tomography with computed tomography can provide complementary information to MRI, but its use in multiple myeloma must be better defined by further studies. The incorporation of abnormal MRI findings into the definition of symptomatic myeloma also needs to be clarified. Bisphosphonates remain the cornerstone for the management of myeloma bone disease. Intravenous pamidronate and zoledronic acid are equally effective in reducing SREs, whereas zoledronic acid seems to offer survival benefits in symptomatic patients. Caution is needed to avoid adverse events, such as renal impairment and osteonecrosis of the jaw. Novel antiresorptive agents, such as denosumab, have given encouraging results, but further studies are needed before their approval for managing myeloma bone disease. Combination approaches with novel antimyeloma agents, such as bortezomib (which has anabolic effects on bone) with bisphosphonates or with drugs that enhance osteoblast function, such as antidickkopf-1 agents, antisclerostin drugs, or sotatercept, may favorably alter our way of managing myeloma bone disease in the near future.

Differential diagnosis of plasma cell dyscrasias in rheumatological practice

Plasma cell dyscrasias (PCD) present certain difficulties in differentially diagnosing with autoimmune rheumatic diseases (RD) as they have some clinical and laboratory manifestations that are characteristic of the latter. Objective: to generalize the experience in diagnosing PCD at the Research Institute of Rheumatology (RIR), Russian Academy of Medical Sciences (RAMS). Subjects and methods. Nineteen patients admitted to the RIR, RAMS, for rheumatological referral diagnoses were diagnosed as having different types of PCD, both PCD in the presence of RD (n = 10) and primary PCD without RD (n = 9). Immunochemical studies of serum and urinary proteins and bone marrow trepanobiopsy were performed in all the patients. Histological studies were made of the minor salivary glands (n = 12), lymph nodes (n = 3), parotid salivary glands - PSG (n = 5), spleen (n = 1), cranial bones (n = 2) and humerus (n = 1). The immunophenotype of tumor lymphocytes in the biopsy and trepanobiopsy specimens were determined by an immunoflurenscence method with standard monoclonal antibody panels and the paraffin sections of biopsy specimens were examined by an immunoperoxidase technique. Biopsy and trepanobiopsy speciments and myelograms were assessed by the researchers of the N.N. Blokhin Russian Cancer Research Center, RAMS. Results. Over 10 years of follow-up, 19 patients (13 females and 6 males) were diagnosed as having different types of PCD: multiple myeloma (MM) (n = 9), extramedullary plasmacytoma of lymph nodes (n = 2), solitary plasmacytoma (SP) of bone (n = 2), Bence-Jones myeloma (BJM) (n = 2), primary amyloidosis (PA) (n = '), lymphoplasmacytic lymphoma (n = '), Waldenström's macroglobulinemia (WM) (n = 2). In the presence of RA, '0 patients with PCD developed Sjogren's disease (SD) (n = 7), rheumatoid arthritis (RA) + SD (n = 2), RA (n = '); and 9 patients had primary PCD (MM (n = 5), BJM (n = '), WM (n = '), SP (n = '), and PA (n = ')). These 9 patients with different rheumatological diagnoses were long followed up and referred to the RIR, RAMS, to specify these. One third of the patients had recurrent conjunctivitis, enlarged PSG and xerostomia (55%). Arthralgias, arthritis of minor and large joints, flexion contractures of hands, ossalgias, and pains in the lumbar spine and ribs were present in '0-45% of the patients. Vascular lesions, such as Reynaud's phenomenon, recurrent purpuras, lower extremity ulcerative lesions, and toe gangrenes, were observed in '0-35% of the patients with PCD. Lesions in the reticuloendothelial system, such as lymphadenopathy, splenomegaly ('5%), hepatomegaly (45%), and fever (25%), were also detected. Conclusion. Many clinical and laboratory manifestations of primary PCD and RD are similar and only the absence of classical laboratory markers of autoimmune disease, as well as the presence of serum monoclonal immunoglobulins and urinary Bence Jones protein suggest the presence of PCD, both primary PCDs and those with RD.

Polyclonal Serum FLC Levels: a Simple Risk Stratification Model for Identifying Aggressive Stage A CLL.

AbstractAbstract 4608 IntroductionSerum free light chain (sFLC) ratios have prognostic value in many haematological malignancies, including multiple myeloma (Kyrtsonis 2007), Waldenstrom's macroglobulinaemia (Leleu, 2007), solitary plasmacytoma of bone (Dingli et al., 2006) and chronic lymphocytic leukaemia (CLL) (Pratt et al., 2008). Polyclonal sFLC levels predict outcome in chronic kidney disease (Hutchison et al., 2009) and more recently show potential prognostic significance in identifying the risk of lymphoma transformation in HIV patients (Landgren et al., 2010). Using a previously characterised CLL population here we report the prognostic significance of polyclonal sFLC levels in this disease. Materials and Patient Description167 untreated Stage A (Binet) CLL patients (male: female ratio 1.6:1; mean age 74: range 44–98) were analysed for FLC and β-2M using nephelometric immunoassays (The Binding Site, Birmingham, UK) on the Siemens BN™II analyser. Previously recorded measurements for other biological and clinical markers (age, sex, CD38 (43 pos, 116 neg, 8 unreported), Zap70 (38 pos, 111 neg, 18 unreported), and IGVH mutational status (122 Mutated versus 17 Unmutated, 28 unreported) were used in the statistical analysis which was performed using SPSS v 18. Results35 of 167 patients had sFLC>50mg/L and had the following characteristics: Median age 79 (range 62–98), IVGH Mutated v Unmutated (22 v 7; 6 unknown), Zap 70 pos v neg (9 vs 23, 3 unknown), CD38 pos v neg (11 vs 23, 1 unknown), and b-2M >3.5mg/L (23 v 12).Analysis of this population identified summated polyclonal sFLC >50mg/L as being significantly associated with progressive disease and worse outcome. 18/35 patients have progressed to treatment and 10/35 patients have died. Median time to first treatment was 83 months in the sFLC >50mg/L group compared to 170 months for patients with sFLC <50mg/L, (p=0.031). Median survival points were not reached but 75%ile survival was significantly shortened in patients with sFLC >50mg/L compared to those with a lower sFLC concentration (98 vs 201 months p=0.006). A 3 tiered risk stratification model (n=93, n=46, n=23) based upon FLC>50mg/L and b-2M >3.5mg/L identified a group of patients exhibiting significantly shorter time to first treatment (Fig. 1 p=5×10-5). DiscussionDespite the heterogeneous clinical course of CLL the universal factor characterising the disease course is abnormal B-cell proliferation. Here we have demonstrated that subtle elevations in polyclonal FLC (>50mg/L) are associated with poorer clinical outcomes and may identify an aggressive sub-population in Stage A CLL patients. Furthermore, a risk stratification model utilising elevated polyclonal FLC and elevated b-2M may help long term clinical management decisions through prompt indication of worse prognosis and shorter survival time. [Display omitted] Disclosures:Levoguer:Binding Site Group Ltd: Employment. Faint:Binding Site Group Ltd: Employment. Harding:Binding Site Group Ltd: Employment.

Solitary plasmacytoma of the proximal tibia in an adolescent

This is the case report of a 14-year young female who was diagnosed with solitary bone plasmacytoma (SBP) of proximal tibia and was treated by local involved field radiotherapy. We present the clinical, radiological and pathological findings of the case and review of the available treatment options and prognosis of this rare site presentation of plasmacytoma in an adolescent female.

Imaging diagnosis of spinal solitary plasmacytoma of bone

浆细胞瘤源于骨髓,并以浆细胞异常增生为特征.临床上以多发性浆细胞骨髓瘤多见,而发生于脊柱的孤立性骨浆细胞瘤(solitary plasmaeytoma of bone,SPB)较为少见,文献对该病的影像表现报道较少.笔者搜集11例经病理证实的发生在脊柱的孤立性浆细胞瘤,总结其CT和MRI表现。

Protocol for the Examination of Specimens From Patients With Non-Hodgkin Lymphoma/Lymphoid Neoplasms

Jerry W. Hussong, MD,DDS; DanielA.Arber, MD;Kyle T. Bradley,MD,MS;Michael S. Brown,MD;Chung-Che (Jeff) Chang,MD,PhD; Monica E. de Baca, MD; David W. Ellis, MBBS, FRCPA; Kathryn Foucar, MD; Eric D. Hsi, MD; Elaine S. Jaffe, MD; Michael Lill, MB, BS, FRACP, FRCPA; Stephen P. McClure, MD; L. Jeffrey Medeiros, MD; Sherrie L. Perkins, MD, PhD; for the Members of the Cancer Committee, College of American Pathologists

Plasma cell tumours: cytomorphological features in a series of 12 cases diagnosed on fine needle aspiration cytology

Plasma cell tumours represent autonomous proliferation of plasma cells and can manifest as multiple myeloma, monoclonal gammopathy of undetermined significance, variants of plasma cell myeloma or plasmacytoma. We report 12 cases of plasma cell tumours, which were initially diagnosed as plasmacytoma on fine needle aspiration cytology (FNAC). The patients were further subjected to bone marrow examination, serum electrophoresis, urine examination for Bence-Jones proteins, and x-ray examination of the skeleton. The cytological smears from all cases were cellular and showed numerous plasma cells in varying degrees of maturity. Subsequent to investigations, five cases were labelled as multiple myeloma with secondary extramedullary plasmacytoma, three as solitary bone plasmacytoma and two as primary extramedullary plasmacytoma. In the remaining two cases, bone marrow and urine examination findings were not available, so a conclusive diagnosis of multiple myeloma or solitary plasmacytoma could not be made. The study highlights the role of FNAC in the diagnosis of plasma cell tumours. Subsequent work-up and follow-up of these patients is important to rule out the presence of multiple myeloma.

Solitary Plasmacytoma of Bone

From 1978 to 1992, 19 patients were diagnosed with solitary plasmacytoma of bone (SPB) accounting for 5.5% of plasma cell neoplasms in Chang Gung Memorial Hospital. Fourteen were male and five were female, with ages ranging from 21 to 71 years (median, 53 years). M-protein was detected at diagnosis in five patients, and during follow-up in three. The most common sites of SPB were the vertebral bodies, with seven involving the thoracic, four involving the lumbar and two involving the cervical spine. The remaining sites included the skull in four, and femur and pelvis in one each. Fifteen of the patients received local radiotherapy, local control was achieved in all; nine patients were alive with no evidence of disease for seven to 77 months, two patients remained in apparent remission at 14 and 92 months, two patients progressed to multiple myeloma at 12 and 84 months, one patient developed extramedullary plasmacytoma at three months, and one patient died of intercurrent disease at four months. The four patients who were not treated with radiation therapy ultimately developed local recurrence or dissemination within four years. In our series, local recurrence or dissemination was always associated with the appearance of, or an increase in, the M-component; thus, measurement of M-protein is helpful in assessing tumor control or progression. Based on our experience and a review of the literature, it is recommended that irradiation of the primary sites is the treatment of choice. Chemotherapy is indicated in those patients with disease progression, recurrence, or dissemination.

Solitary plasmacytoma of bone: a clinicopathologic, immunohistochemical and immunoglobulin gene rearrangement study

To investigate clinicopathologic features of solitary plasmacytoma of bone (SPB) and the role of immuno-phenotype and immunoglobulin gene rearrangement detection in the diagnosis and differential diagnosis of SPB. A total of 21 cases of SPB were selected during a period from 1990 to 2008. A retrospective clinicopathologic study and immunohistochemistry (EnVision or EliVision methods) of 17 antigens were performed. In addition, universal IgH (FR3A/LJH/VLJH) primers and BIOMED-2 PCR multiplex tubes were used for IgK and IgL rearrangement analysis. The age of patients ranged from 36 to 72 years with a media of 50 years. Axial skeleton was the most common site of involvement, accounting for 66.7% of the cases (14 of 21), followed by the extremities of 33.3% (7 cases). Low serum level of M-components was found in 5 cases, including two of IgG type (21.4 g/L) and three of IgA type. Clinical manifestations were closely related to the anatomic sites involved, such as pain due to bone destruction, symptoms and signs caused by compression of spinal cord or nerve root, and pathological fracture. All cases presented as a solitary osteolytic lesion. According to the histological grading criteria, grade I tumor was seen in 12 of 21 cases (57.1%). The remaining were grade II (5 cases, 23.8%) and grade III (4 cases, 19.0%). Immunohistochemically, the neoplastic cells expressed two or more plasma cell antigens, including CD138, CD38 and PC, but no CD19 and CD20. CD79a expression detected in 23.8%(5/21) of the cases. Expression of CD56, CD27 and CD44v6 were 57.1% (12/21), 15.0% (3/20) and 23.8% (5/21), respectively. Follow-up data were available in 12 of the 21 patients (57.1%). Five patients were alive and 7 died. Three patients developed multiple myeloma (MM) and died of the tumor. SPB is a rare tumor with bone pain as the most common presenting symptom due to bone destruction. The diagnosis of EMP can only be established after exclusion of an extramedullay invasion by MM. Immunophenotype and IgH gene rearrangement analysis play important roles in the diagnosis of SPB.

Differential diagnosis of dumbbell lesions associated with spinal neural foraminal widening: imaging features

Computed tomography (CT) and magnetic resonance imaging (MRI) reliably demonstrate typical features of schwannomas or neurofibromas in the vast majority of dumbbell lesions responsible for neural foraminal widening. However, a large variety of unusual lesions which are causes of neural foraminal widening can also be encountered in the spinal neural foramen. Radiologic findings can be helpful in differential diagnosis of lesions of spinal neural foramen including neoplastic lesions such as benign/malign peripheral nerve sheath tumors (PNSTs), solitary bone plasmacytoma (SBP), chondroid chordoma, superior sulcus tumor, metastasis, and nonneoplastic lesions such as infectious process (tuberculosis, hydatid cyst), aneurysmal bone cyst (ABC), synovial cyst, traumatic pseudomeningocele, arachnoid cyst, vertebral artery tortuosity. In this article, we discuss CT and MRI findings of dumbbell lesions which are causes of neural foraminal widening.

Solitary Plasmacytoma

To analyze the outcomes of patients treated for solitary plasmacytoma with definitive radiotherapy (RT). Thirty-two patients with solitary plasmacytoma of bone (SPB; 22 patients) and extramedullary plasmacytoma (EMP; 10 patients) were treated between 1963 and 2006. The median RT dose was around 42.7 Gy (range, 15-54 Gy) over a median of 25 fractions (range, 1-32 fractions). No patient received adjuvant chemotherapy. Median follow-up was 10.1 years (range, 1-33 years). Median follow-up on living patients was 7.3 years (range, 2.1-33 years). The 10-year local-control rate after RT was 87%. All 4 patients who developed a local recurrence had SPBs > or = 5 cm. The 10-year multiple myeloma-free survival rates were: SPB, 30%; EMP, 90%; and overall, 50%. Progression to multiple myeloma occurred at a median of 25.1 months after RT. The 10-year overall and cause-specific survival rates were 65% and 77%, respectively. The 10-year cause-specific survival rate was 65% for patients with SPB versus 100% for those with EMP (P = 0.006). Moderate dose RT results in a high rate of local control. Patients with SPB are more likely to progress to multiple myeloma, which adversely affects their survival compared with those with EMP.

Solitary Plasmacytoma of the Spine: An Unusual Presentation: Fig 1.

Solitary plasmacytoma of bone (SBP) is a rare hematologic malignancy, which occurs with radiologic evidence of a solitary lesion in the absence of significant bone marrow plasma cell infiltration (<5%–10%), leading to absent (or low) serum and urine levels of monoclonal protein.[1][1] This

A case of POEMS syndrome with mixed hyaline vascular and plasma

We report a 43-year-old man who developed progressive ascending sensorimotor polyneuropathy, a solitary bone plasmacytoma originating from a rib and an axillary lymph node with features of Castleman's disease, mixed hyaline vascular and plasma cell type. He was diagnosed of having non-Hodgkin's lymphoma seven years back and was treated. Examination revealed a smooth goitre, thickened skin, white nails, small testes and ankle oedema. Investigations revealed a monoclonal para-proteinaemia of 7.83 g/l and elevated levels of serum TSH and prolactin. In this case the Castleman's disease is localised and the lymphoma preceded the Castleman's disease.

International myeloma working group consensus statement and guidelines regarding the current role of imaging techniques in the diagnosis and monitoring of multiple Myeloma

Several imaging technologies are used for the diagnosis and management of patients with multiple myeloma (MM). Conventional radiography, computed tomography (CT), magnetic resonance imaging (MRI) and nuclear medicine imaging are all used in an attempt to better clarify the extent of bone disease and soft tissue disease in MM. This review summarizes all available data in the literature and provides recommendations for the use of each of the technologies. Conventional radiography still remains the 'gold standard' of the staging procedure of newly diagnosed and relapsed myeloma patients. MRI gives information complementary to skeletal survey and is recommended in MM patients with normal conventional radiography and in all patients with an apparently solitary plasmacytoma of bone. Urgent MRI or CT (if MRI is not available) is the diagnostic procedure of choice to assess suspected cord compression. Bone scintigraphy has no place in the routine staging of myeloma, whereas sequential dual-energy X-ray absorptiometry scans are not recommended. Positron emission tomography/CT or MIBI imaging are also not recommended for routine use in the management of myeloma patients, although both techniques may be useful in selected cases that warrant clarification of previous imaging findings, but such an approach should ideally be made within the context of a clinical trial.

Prognostic factors influencing survival in solitary plasmacytoma

In response to the recent article published in this journal (Dores et al, 2009), we would like to point out a few differences and additional observations we observed in our similar study using the surveillance epidemiology and end results (SEER) database for multiple myeloma. A total of 1472 patients with solitary plasmacytoma were identified between 1988 and 2004. The median survival of the entire cohort was 6·83 years. Multivariate analysis for factors affecting overall survival (OS) and disease-specific survival (DSS) indicated that younger age was associated with a significantly improved OS. Age ≤40 years was associated with the best OS when compared with the 40–60 year group [hazard ratio (HR) 1·74; 95% confidence interval (CI) 1·04–2·91] and the >60 year group (HR 5·55; 95% CI 3·40–9·06). Multivariate analysis also identified other prognostic factors for OS. Caucasians (HR 0·72; 95% CI 0·56–0·92) and those of races other than Caucasian/African American (HR 0·48; 95% CI 0·29–0·79) had improved OS when compared to African Americans. Solitary extramedullary plasmacytoma (SEP) had better OS than solitary bone plasmacytoma (SBP) (HR 1·37; 95% CI 1·13–1·65). The 5-year OS was 63·9% for SEP compared to 54·6% for SBP (P < 0·01). Disease-specific survival seemed to plateau around 80% for SEP compared with 50% for SBP, suggesting markedly different cure rates. For the entire cohort, the Kaplan–Meier estimate of DSS at 1 year was 95·4%, at 5 years 76·1% and at 10 years 63·2%. The median DSS was not reached for the cohort. Multivariate analysis demonstrated that age, gender, race and plasmacytoma location all significantly influenced DSS. Individuals ≤40 years old had improved DSS compared to the 40–60 year old group (HR 2·68; 95% CI 1·15–6·2) and the >60 year old group (HR 6·94; 95% CI 3·06–15·73). Males had better DSS (HR 0·74; 95% CI 0·58–0·94) than females. Races other than Caucasian/African American had significantly improved DSS (HR 0·31; 95% CI 0·13–0·75) compared to African Americans. SEP had improved DSS (HR 2·35; 95% CI 1·74–3·18) when compared to SBP (Table I, Fig 1). Kaplan–Meier survival curve analysis for overall survival comparing medullary (bone) and extramedullary plasmacytoma, disease-specific survival comparing extramedullary and medullary plasmacytoma, overall survival comparing different age groups for plasmacytoma and disease-specific survival comparing medullary and extramedullary plasmacytoma. Dores et al (2009) provided no information regarding the effect of treatment modalities on outcomes. We found that those treated with XRT + surgery (68·0%) had a better 5-year OS than those treated with surgery alone (57·5%) or XRT alone (54·4%) (P < 0·01). Those treated with XRT + surgery (81·9%) had a better 5-year DSS than those treated with surgery alone (79·6%) or XRT alone (71·9%) (P < 0·01). However, multivariate analysis did not identify treatment modality as a prognostic factor. Dores et al (2009) did not comment on the cause of death in their analysis of the SEER database. We found that the major cause of death was multiple myeloma in 277 patients (49·64%). However, analysis of cause of death between groups revealed statistically significant differences in cause of death between age group, gender and location. Patients between 40 and 60 years old were most likely to die of myeloma (57·9%) followed by 48·2% of patients >60 years (48·2%) and patients ≤40 (35·3%) (P < 0·01). Females died more frequently of myeloma (57·6%) than males (45·0%) (P < 0·01). 32·8% of SEP patients died of myeloma as compared to 57·7% of SBP patients (P < 0·01). The SEER database unfortunately does not provide data on details of any systemic therapy delivered. However, it may be possible to improve survival by offering adjuvant systemic therapy especially in patients with poor prognostic factors. Prospective, randomized trials comparing adjuvant chemotherapy versus observation alone after local therapy are needed for solitary plasmacytoma.

Plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma: case report

The authors present a rare case of plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma in a 42-year-old woman. The lesion was surgically removed and analyzed. Histopathological examination with immunostaining revealed it to be positive for immunoglobulin G and Kappa chains, demonstrating monoclonality. However, no abnormality was observed on serum electrophoresis, skeletal survey and bone marrow aspiration. Therefore, the tumor was diagnosed solitary plasmacytoma of bone. The patient was treated with external beam radiotherapy and has remained disease free for 5 years and 6 months until 2007, when she presented a pathological fracture due to multiple myeloma. Extensive medical work-up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving orbit or ocular adnexa is needed when the diagnosis of solitary plasmacytoma of bone is suspected because treatment and prognosis are very different.