Primary non-Hodgkin lymphoma of tibia

Clinical Cases 19.10.2012
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 17 years, male
Authors: 1. Luz M. Moran 2. Dolores Ponce
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Details
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AI Report

Clinical History

A 17-year-old Nigerian boy attended the emergency department with a 9-month history of pain in his right leg. Physical examination revealed localized swelling and a palpable mass.

Imaging Findings

Plain radiography and magnetic resonance imaging (MRI) were performed, and a biopsy specimen was taken. The patient was diagnosed with non-Hodgkin lymphoma.
Imaging studies revealed an osseous lesion with a destructive pattern involving the medullary and cortical bone of the proximal tibial metadiaphysis and associated soft-tissue masses.
The radiograph showed an osteolytic lesion with aggressive features, such as cortical breakthrough, pathologic fracture, soft-tissue masses, and interrupted periosteal reaction (Codman triangle) (Figure 1). Cortical destruction was complete in the proximal portion of the tumour and partial with a permeative pattern in the distal portion. MRI and, more particularly, contrast-enhanced imaging revealed permeative cortical bone with a linear pattern of intracortical high signal intensity (SI) and no disturbance of cortical integrity (Figure 2).
MRI showed marked extension of the tumour to the soft tissues and into the knee joint. On the T2-WI sequence, the SI of the intraosseous tumour was lower than that of subcutaneous fat.

Discussion

Primary bone lymphoma (PBL) is one of the rarest primary malignant tumours of bone, and most cases involve non-Hodgkin lymphoma. Lymphoma in bone with no evidence of lymphomatous disease elsewhere for at least 6 months after diagnosis confirms the diagnosis of PBL (1).
The clinical presentation of PBL is insidious bone pain that can persist for months, sometimes with systemic symptoms. Other signs include local swelling and a palpable mass in long bones, particularly the femur, tibia, pelvis, and humerus. Although PBL presents in several age groups, it is more frequent in patients aged over 30 years.
Plain radiography findings of PBL are variable and non-specific, and the differential diagnosis includes a wide range of lesions (osteomyelitis and malignant tumors such as osteosarcoma and Ewing sarcoma). Despite the lack of specificity in the radiographic pattern, a diagnosis of PBL should be considered when a solitary lytic tumour near the end of a long bone has a permeative or moth-eaten destructive pattern accompanied by an aggressive periosteal reaction (2). The differences in treatment and improved survival rates with PBL compared with other primary malignant tumours of bone, stress the importance of recognizing PBL (2).
On other imaging studies (CT, MRI, and Tc 99m radionuclide imaging), PBL does not have a specific pattern. The literature on the MRI features of PBL focuses mainly on the appearance of cortical bone, soft-tissue abnormalities, and SI characteristics (3). Involvement of cortical bone is subdivided into complete, focal, and permeative cortical destruction and cortical thickening. The MRI pattern of permeative cortical destruction showing multiple linear foci of intermediate or high SI penetrating the cortex is characteristic of bone lymphoma, but not specific, and it is observed in other small round cell tumors and metabolic and inflammatory processes, including hyperparathyroidism and osteomyelitis.
Low SI on T2-WI sequences is reported as a feature of bone lymphoma owing to a high content of fibrous tissue.
The pattern of large soft tissue masses without extensive cortical destruction is common in PBL.
In summary, although radiographic and MRI findings may suggest a diagnosis of bone lymphoma, it is not possible to differentiate lymphoma from other processes based solely on imaging. Therefore, biopsy is necessary. MRI, and particularly Gd-enhanced sequences, can determine the response to chemotherapy and/or radiation therapy, which in turn has a direct impact on individual patient care.

Differential Diagnosis List

Primary non-Hodgkin lymphoma of tibia
Osteomyelitis
Osteosarcoma
Ewing sarcoma

Final Diagnosis

Primary non-Hodgkin lymphoma of tibia

Liscense

Figures

Figure 1.Anteroposterior and lateral right leg radiograph at presentation.

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Figure 1.Anteroposterior and lateral right leg radiograph at presentation.
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Figure 1.Anteroposterior and lateral right leg radiograph at presentation.

Figure 2. MRI before and after intravenous injection of gadopentetate dimeglumine.

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Figure 2. MRI before and after intravenous injection of gadopentetate dimeglumine.
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Figure 2. MRI before and after intravenous injection of gadopentetate dimeglumine.
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Figure 2. MRI before and after intravenous injection of gadopentetate dimeglumine.
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Figure 2. MRI before and after intravenous injection of gadopentetate dimeglumine.