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ORIGINAL ARTICLE Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 267-270
Primary non-hodgkin's lymphoma of bone: Experience of a decade

Section of Histopathology, Aga Khan University, Karachi, Pakistan

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Date of Web Publication12-Jun-2010


Objective: To study the prevalence and characterization of primary non Hodgkin's lymphoma of bone in a major referral center of Pakistan. Methodology: All cases of primary non Hodgkin's lymphoma of bone, diagnosed at a referral center in Pakistan from January 1998 to July 2008 have been included. Patients with diagnosis of lymphoma at some other site prior to diagnosis in bone biopsy were excluded from the study. Results: Sixty cases of primary non Hodgkin's lymphoma of bone were received, with a male to female ratio of 7: 3 (42 males and 18 females). Overall, 52/60 (86.6 %) patients were diagnosed with Diffuse large B-cell non-Hodgkin's lymphoma. The largest fraction of patients was found in the pediatric group i.e. 33/60(55%) followed by elderly group i.e.15/60 (15%) and adults i.e. 12/60 (20%). The commonest sites of involvement were femur (28.3 %), hip bone (16.6%) and humerus (10%). Conclusion: Diffuse large B-cell lymphoma is the commonest type of primary non Hodgkin's lymphoma of bone in the Pakistani population. It is common in all ages. The sites most often involved are femur followed by hip bone. Primary non Hodgkin's lymphoma of bone appears to be more common in males. Our results are comparable to international data with various studies also showing that diffuse large B cell non Hodgkin lymphoma is the commonest primary bone lymphoma. However, primary Bone Lymphoma is more common in children and young adults.

Keywords: Bone Primary lymphoma, extra-nodal lymphoma, non Hodgkin lymphoma

How to cite this article:
Qureshi A, Ali A, Riaz N, Pervez S. Primary non-hodgkin's lymphoma of bone: Experience of a decade. Indian J Pathol Microbiol 2010;53:267-70

How to cite this URL:
Qureshi A, Ali A, Riaz N, Pervez S. Primary non-hodgkin's lymphoma of bone: Experience of a decade. Indian J Pathol Microbiol [serial online] 2010 [cited 2023 Sep 30];53:267-70. Available from:

   Introduction Top

Primary bone lymphoma (PBL) is a rare condition which accounts for approximately 7% of all primary bone malignancies and 5% of all extra-nodal lymphomas. [1],[2] Although bone involvement may be found in advanced-stage of lymphoma originating at other sites, PBL is defined as a lymphoma which occurs primarily in the bone without the known involvement of any other site in the body. It was described initially as a discrete clinico-pathological entity by Parker and Jackson in 1939 as reported by Roussel et al.[1],[3] Non-Hodgkin lymphoma is the commonest primary bone lymphoid malignancy and diffuse large B-Cell lymphoma (DLBCL) accounts for the greatest percentage of cases. [4],[5],[6] In most published studies, long bones appear to be the main site of PBL, with percentages varying from 44% to 71% in different studies. [7],[8],[9] Femur has been cited as the most common site in several studies. [10],[11] A major fraction of patients present in the fifth or sixth decade of life with limited-stage disease. [12],[13] Most series also show a male preponderance. [14],[15],[16]

Presenting symptoms of bone lymphoma may include bone pain that is not relieved by rest, palpable masses, pathological fractures; spinal cord compression or other systemic B-cell lymphoma related symptoms (fever weight loss etc). [9],[10]

   Materials and Methods Top

All cases of primary NHL from January 1998 to July 2008 (10.5 Years) diagnosed at our center were included. All cases had a confirmed histopathological diagnosis of lymphoma of bone with no evidence of lymphoma at any other site of the body. A comprehensive panel of Immunohistochemical markers including LCA,CD 20,CD 79 a, CD 10, CD 3, CD 30, Alk, EMA and Mib 1 were performed in all cases.

Medical records of patients were analyzed for the following variables; presenting signs and symptoms, site of disease, radiological findings and other investigations. Only those cases of bone lymphoma were included where lesion was limited to bone and no lymph node enlargement was present at the time of diagnosis (as per WHO criteria for bone lymphomas). [5]

   Results Top

A total of 60 cases of primary bone NHL were identified and included in the study. The period of study was from January 1998 to July 2008 (10.5 years), 48 patients were male, while 12 were female. Male to female ratio was 4:1. Majority of the cases were stage 1 and a few were 2.

DLBCL: Diffuse Large B cell Lymphoma; BL: Burkitt's Lymphoma; ALCL: Anaplastic Large cell Lymphoma; PTCL: Peripheral T cell Lymphoma. The median age of patients was 28 years (range 4-70 years)

The patients were divided into three main groups pediatric: (under 18 years of age, in our hospital pediatric age group is uptil 18 years), adults (18 to 60 years) and elderly (over 60 years).Majority of the cases (55%) occurred in the pediatric age group.

The commonest sites of involvement were femur (28.3 %), hip bone (16.6%) and humerus (10%). Overall, DLBCL was the most common primary bone lymphoma (52/60), followed by Burkitt's lymphoma and anaplastic large cell lymphoma [Table 1].

The cases were categorized as per their morphology and immunohistochemistry.

Burkitt's lymphoma [Figure 1] showed sheets of atypical lymphoid cells with fairly monotonous nuclei and inconspicuous nucleoli (multiple small peripheral nucleoli were seen). There were apoptotic bodies. Tingible body macrophages were present giving a starry sky appearance. The cells were diffusely positive for CD 20, CD10 and negative for Bcl2. The proliferative index was almost 100%.

Cases of diffuse large B cell lymphoma showed sheets of large cells with pleomorphic nuclei containing prominent nucleoli. These were diffusely positive for LCA, CD 20 [Figure 2] and showed a proliferative activity of 70-80%. Anaplastic large cell lymphoma histologically showed bizarre large cells which were positive for LCA and CD 30 [Figure 3]. Proliferative activity was 70-80%.Both cases also showed positivity for EMA and Alk protein.

T cell lymphoma NOS comprised a heterogeneous population of cells with a mixed inflammatory background these were positive for LCA and CD 3 and had a proliferative activity of 50-60%.

Radiological Correlation

Radiological correlation was available in 44 of 60 cases; of these, single bone involvement was seen in 41 cases whereas multiple bone involvement was seen in three cases. Site of tumor was diaphyseal (in long bones) with lytic appearance on X-Rays. The tumors in flat bones also showed lytic lesion [Figure 4]. No osteoid was seen. Extension into soft tissue was seen in eight out of 44 cases.

   Discussion Top

In 1939, Parker and Jackson for the first time clearly distinguished primary reticulum cell sarcoma of bone from Ewing's sarcoma and noted that the former had better prognosis, as reported by Roussel et al.[1] Their study was followed by a series of publications defining reticulum cell sarcoma as a distinct entity. Intraosseous lymphoma is now a well recognized entity accounting for primary malignant tumors of the bone in the Mayo clinic series of bone lymphomas. [5]

Lymphoma affects portions of bone with persistent red marrow. Lymphoma of small bones of hands and feet are extremely rare. When lymphomas present in the spine or maxillary antrum it is often difficult to determine whether the process is in the bone or soft tissues.

Majority of the patients with primary bone lymphoma present with bone pain while others present with a palpable mass. Patients with PBL rarely present with B symptoms such as fever or night sweats. Radiological findings are quite variable and non specific. In long bones, the tumor preferentially involves diaphysis. There is variable sclerosis; and rarely the tumors maybe sclerotic or entirely lytic. The cortex is frequently destroyed leading to the formation of a soft tissue mass. [11],[13] On gross inspection, the lesion has a soft fish flesh appearance similar to appearance of lymphoma elsewhere in the body. Microscopically diffuse sheets of cells typically replace the normal architecture. Cytologically the cells are pleomorphic. Characteristically the cell of PBL of DLBCL type has multilobated nuclei.

The prognosis of lymphoma is associated with the cell type; however the most important predictor is the stage of the disease. In our study, the majority of cases were DLBCL and the pediatric age group was the most commonly affected. The optimal approach to treatment of PBL is not established. In published series, differences in definition, staging and treatment modalities have rendered comparisons difficult. [16],[17],[18]

Our results are comparable to the international data. The largest reported study was carried out at department of radiation oncology, Memorial Sloan-Kettering cancer center, New York with a follow up of 82 patients. In this study, majority of the cases were diffuse large B cell lymphoma (80%) and the mean age of the patients was 48 years (range, 11-83 years) 81% presented with stage 1 or stage 2 disease and the median follow-up was 67 months (2 to 280 months). [19]

Another study carried out at Washington Hospital Center looked at 33 patients. These patients were evaluated for international prognostic index (IPI) score and overall survival. The conclusions were based on stage, sex, age and chemotherapy. Outcome by virtue of treatment was related to stage, IPI score and age at diagnosis. Patients with primary bone lymphoma particularly those with low IPI scores were shown to have good outcome and responded well to chemotherapy. [20]

Another study carried out in Pakistan at Shaukat Khanum Cancer Hospital reviewed a series of seven consecutive patients diagnosed and treated with primary non-Hodgkin lymphoma of bone between March 2002 and January 2007. All patients underwent chemotherapy with half receiving radiotherapy as their initial treatment. Six (84%) patients were male and one (16%) female with a median age of 33 (range: 23-85). All had diffuse large B-cell lymphoma (DLBCL) of bone except one (85 - M) who had chest wall cutaneous T-cell lymphoma with iliac blade involvement. The femur was the most frequently involved site (43%). Except for three patients that involved the lymph nodes, all patients had disease limited to bone. The 5-year overall survival rate was 43%. Although the number of patients was small, the data presented here revealed several characteristics of Primary non Hodgkin lymphoma of bone. [21]

A similar study was carried out at the department of medical oncology, Tata Memorial Hospital, Mumbai, India, where 25 patients with diffuse large cell lymphoma of the bone were registered. Pain at the local site and soft tissue swelling were the commonest symptoms.

Involvement of the bones in the lower half of the body was more frequent than the bones in the upper half. Osteolytic lesions and an associated soft tissue mass were the common radiological findings. The overall response to therapy was 96%. On follow-up, two patients had a nodal relapse. There were no deaths due to treatment-related toxicity. The mean progression free survival was 9.39 years and the overall survival was 11.66 years.They concluded that primary bone lymphoma is a malignancy that is highly curable with a combination of chemotherapy and radiotherapy. [22]

   Conclusion Top

Diffuse large B-cell lymphoma is the commonest type of primary bone lymphoma in our patients, common in all age groups. The sites most often involved were femur followed by hip bone. These tumors were more common in males. Our results are comparable to international data in which diffuse large B cell lymphoma is the commonest primary bone lymphoma.

   References Top

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2.Pettit CK, Zukerberg LR, Gray MH, Ferry JA, Rosenberg AE, Harmon DC, et al. Primary lymphoma of bone. A B cell neoplasm with a high frequency of multilobated cells. Am J Surg pathol 1990;14:329-34.  Back to cited text no. 2  [PUBMED]    
3.Van Baarlen J, Schuurman HJ, Van Unnik JA. Multilobated Non Hodgkin Lymphoma. A clinicpathologic entity. Cancer 1988;61:1371-6.  Back to cited text no. 3  [PUBMED]    
4.Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, et al. A revised European-American classification of lymphoid neoplasms: a proposal fromthe International Lymphoma Study Group. Blood 1994;84:1361-92.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Dahlin DC, Unni KK. Bone tumors; general aspects and data on 8542 cases. In: Thomas CC, editor. 4th ed. Springfield, 1986. p. 208-26.  Back to cited text no. 5      
6.Baar J, Burkes RL, Gospodarowicz M. Primary non-Hodgkin's lymphoma of bone. Semin Oncol 1999;26:3:270-5.  Back to cited text no. 6      
7.Limb D, Dreghorn C, Murphy JK, Mannion R. Primarylymphoma of bone. Int Orthop 1994;18:180-3.  Back to cited text no. 7  [PUBMED]    
8.Stein ME, Kuten A, Gez E, Rosenblatt KE, Drumea K, Ben-Shachar M, et al. Primary lymphoma of bone-a retrospective study. Oncology 2003;64:322-7.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Dubey P, Ha CS, Besa PC, Fuller L, Cabanillas F, Murray J, et al. Localized primary malignant lymphoma of bone. Int J Radiat Oncol Biol Phys 1997;37:1087-93.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Heyning FH, Hogendoom PC, Kramer MH. Primary non-Hodgkin's lymphoma of bone: a clinicopathological investigation of 60 cases. Leukemia1999;13:2094-98.  Back to cited text no. 10      
11.Mulligan ME, McRae G, Murphey MD. Imaging features of primary lymphoma of bone. Am J Roentgenol 1999;173:1691-7.  Back to cited text no. 11      
12.Beal K, Allen L, Yahalom J. Primary bone lymphoma:treatment results and prognostic factors with long-term follow-up of 82 patients. Cancer 2006;106;12:2652-6.  Back to cited text no. 12      
13.Mulligan ME, McRae GA, Murphy MD Imaging features of primary bone lymphoma. Am J Roentgenol 1999;173:1691-7.  Back to cited text no. 13      
14.Heyning FH, Hogendoorn PC, Kramer MH, Hermans J, Kluin-Nelemans JC, Noordijk EM, et al. Primary non-Hodgkin's lymphoma of bone: A clinicopathological investigation of 60 cases. Leukaemia 1999;13:2094-8.  Back to cited text no. 14      
15.Zinzani PL, Carrillo G, Ascani S, Barbieri E, Tani M, Paulli M, et al. Primary bone lymphoma: experience with 52 patients. Haematologica 2003;88:280-5.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]  
16.Barbieri E, Cammelli S, Mauro F, Perini F, Cazzola A, Neri S, et al. Primary non-Hodgkin's lymphoma of the bone: treatment and analysis of prognostic factors for stage I and stage II. Int J Radiat Oncol Biol Phys 2004;59:760-4.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]  
17.Rathmell AJ, Gospodarowicz MK, Sutcliffe SB, Clark RM. Localized lymphoma of bone: prognostic factors and treatment recommendations. The Princess Margaret Hospital Lymphoma Group. Br J Cancer 1992;66:603-6.  Back to cited text no. 17  [PUBMED]    
18.Pathology and genetics of tumors of the lymphoid tissues. LYON: IARC; 2001. p. 120-1.  Back to cited text no. 18      
19.Beal K, Allen L, Yahalom J. Primary bone lymphoma: treatment results and prognostic factors with long-term follow-up of 101 patients. Cancer 2006;106:2652-6.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]  
20.Catlett JP, Williams SA, O'Connor SC, Krishnan J, Malkovska V. Primary lymphoma of bone an institutional experience. Leuk Lymphoma 2008;49:2125-32.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]  
21.Ahmad I, Saeed U, Rehmani R, Ahmed ME. Primary bone lymphoma: a series from a cancer institute in Pakistan. J Pak Med Assoc 2009;59:179-81.  Back to cited text no. 21  [PUBMED]    
22.Deshmukh C, Bakshi A, Parikh P, Nair R, Pai V, Gupta S, et al. Primary non hodgkins Lymphoma of bone a single institution experience. Med Oncol 2004;21:263-7.  Back to cited text no. 22  [PUBMED]  [FULLTEXT]  

Correspondence Address:
Asim Qureshi
Pathology and Microbiology, Aga Khan University, Karachi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.64340

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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