|Year : 2009 | Volume
| Issue : 1 | Page : 52-53
Primary non-Hodgkin lymphoma of bone: An unusual presentation
Ronald F Pinheiro, Francisco DR Filho, Gabrielle G Lima, Francisco VA Ferreira
Instituto do Cancer do Ceará, Universidade Federal do Ceará, Brazil
|Date of Web Publication||17-Mar-2009|
Gabrielle G Lima
Eusebio de Sousa Street, 1331, 60050-110, Fortaleza-Ceará
Source of Support: None, Conflict of Interest: None
Primary lymphoma of bone (PLB) is an extremely rare condition that is usually confused with other primary injuries of the bone. It is characterized by the involvement of one or more bone locations, with or without involvement of regional lymph nodes and viscera. PLB constitutes 3-7% of all malignant bone tumors and approximately 3% of all extranodal lymphomas. It is found at all ages, being most frequently seen in adult life. Any part of the skeleton can be involved, but a trend exists in favor of bones with persistent bone marrow. We report a case of PLB with an unusual presentation: involvement of the proximal phalanx of the thumb. Treatment with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus etoposide established complete remission. Consolidation with radiotherapy of the femur and phalanx was performed. There was no evidence of recurrence at the 14 th month follow-up.
Keywords: Bone, primary non-hodgkin lymphoma, proximal phalanx
|How to cite this article:|
Pinheiro RF, Filho FD, Lima GG, Ferreira FV. Primary non-Hodgkin lymphoma of bone: An unusual presentation. J Can Res Ther 2009;5:52-3
| > Introduction|| |
Primary lymphoma of bone (PLB) is an uncommon bone tumor with a relatively homogeneous morphology and clinical behavior.  Distinguishing PLB from other bone tumors is important because the former has a better response to therapy and a better prognosis, especially when compared to patients with systemic lymphomatous disease.  At presentation PLB may be complicated by pathological fractures or spinal cord compression.  Any location in the skeleton can be involved, but a trend exiats in favor of bones with persistent bone marrow. The sites commonly affected are the long bones. The femur has been reported as the most common site to be involved when only one isolated site is affected. , Recently, Beal et al .  studied the clinical characteristics of 82 PLB patients and reported the most common sites to be involved as the femur (27%), pelvis (15%), tibia (13%), humerus (12%), spine (9%), mandible (2%), skull (1%), scapula (1%), radius (1%), and ulna (1%).
We report a patient who had a rare presentation, with a lesion in the proximal phalanx of the right thumb plus an osteolytic lesion in the right distal femur. To the best of our knowledge this is the first report showing involvement of a phalanx.
| > Case Report|| |
A 63-year-old man was admitted in December 2005 with a 4-month history of pain and swelling in the right knee and difficulty in walking. At physical examination a tumor was detected in the right knee. The superficial lymph nodes were not enlarged. The patient also complained of pain in the right thumb and weight loss. A plain radiograph showed pathologic fractures of the right distal femur and the proximal phalanx of the right thumb, with periosteal reaction [Figure 1] and [Figure 2]. Histologic examination of the femur and phalanx revealed a primary diffuse large B-cell lymphoma. Immunohistochemistry was positive for CD20 and negative for CD3, CD4, and CD56. Computed tomography of the thorax, abdomen, and pelvis did not reveal any enlarged lymph nodes. Bone marrow examination was normal. Treatment with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus etoposide (nine courses) was successful in establishing complete remission. Consolidation with radiotherapy (total dose: 40 Gy) of the femur and phalanx was performed. There was no evidence of recurrence at the 14 th month follow-up.
| > Discussion|| |
PLB constitutes less than 1% of all non-Hodgkin lymphomas and 3% of all malignant tumors of bone.  Although the radiographic appearance of PLB is variable and nonspecific, one typical picture is that of a solitary lytic lesion near the end of a long bone. In the case reported here, this (solitary lytic lesion) was the picture observed in both the femur and the phalanx. To the best of the author's knowledge, involvement of this site (a phalanx) with this particular pattern has not been reported earlier. Recently, Ramadan et al .  studied 131 cases of PLB and reported that approximately one-third (42/131) presented with disease in the spine and one-third (42/131) with disease in the long bones; they did not detect any case with involvement of a phalanx.
Malignant bone tumors of the hand are rare and few centers have extensive experience in the care of patients with such lesions. In their 65-year retrospective review, Frassica et al . found only 22 primary malignant bone tumors of the hand (in 18 patients). There were nine chondrosarcomas, four hemangioendothelial sarcomas, two cases each of osteosarcoma and fibrosarcoma, and a single case of Ewing's tumor. Ten lesions each were located in the metacarpals and phalanges and two lesions in the carpus. Also, only about 0.1% of all metastatic lesions were seen in the hand. 
Our patient achieved complete remission with combined therapy (chemotherapy and radiotherapy). Over the last two decades, combined therapy has become the standard treatment for PLB. A retrospective study at the Memorial Sloan Kettering Center,  evaluating various treatment strategies (i.e., radiation therapy alone, chemotherapy alone, or combined-modality therapy), showed a significant benefit with combined therapy. Combined modality therapy was associated with a 5-year survival of 95% vs 78% for patients treated with single-modality therapies ( P = 0.013). Three smaller series, Zinzani et al ,  Rathmell et al,  and Fidias et al ,  have also shown a significant survival advantage with the use of combined-modality therapy. More recently, Ramadan et al ,  reported the use of rituximab in 28 PLB cases. Although there was only a short period of follow-up, they were able to demonstrate that rituximab improved progression-free survival (PFS); the 3-year PFS for patients who received rituximab plus CHOP was 88% compared with 52% for those who received standard CHOP alone ( P = 0.025).
In this case report we present a rare pathologic lesion of the phalanx. The differential diagnoses for this lesion includes metastases, carcinoma, granulocytic sarcoma, and plasmacytoma and, therefore, biopsy is required for diagnosis. This case reinforces the evidence in favor of combination therapy as the standard treatment for PLB. Although we did not use rituximab, we believe that it may add benefit to the treatment of these patients.
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[Figure 1], [Figure 2]