Lymphoma is the seventh most common type of malignancy in both males and females. It may develop in any location where lymphomatous tissue exists. Although extranodal presentation in the lower limb and pelvis are uncommon, it could present with diverse manifestations. We report an unusual case of primary extranodal large B-cell lymphoma of the ankle joint initially presumed to be a chronic osteomyelitis. This case report discusses the impact of imaging studies on decision-making and highlights the need to consider malignancy in chronic infections.

The differential diagnosis of Burkitt lymphoma/leukemia (BL) and the double or triple hit lymphomas remains often problematic in terms of immunophenotyping in association with MYC gene analysis. In the past, BL-like B-cell malignancy with B-cell precursor phenotype (BCP-ALL)

In the 2017 revision of the WHO classification of lymphomas, the term high-grade B-cell lymphoma has been repurposed. Double and/or triple-hit lymphomas with MYC and BCL2 and/or BCL6 rearrangements are now designated as high-grade B-cell lymphoma. Such high grade B-cell lymphomas occur in <10% of cases of diffuse large B-cell lymphoma. On the other hand, Burkitt lymphoma/leukemia (BL; consisting of Burkitt lymphoma and/or Burkitt leukemia variant) was once thought to be a highly aggressive B-cell malignancy, but currently BL does not belong to high grade lymphoma because it is a MYC only rearranged B-cell malignancy. All types of BL are characterized by dysregulation of the MYC gene located at the 8q24 with chromosomal translocations, such as most common t (8;14) (q24;q32), or rare t (2;8) (p12;q24), or t (8;22) (q24;q11) variants.

In clinical practice, the differential diagnosis of BL and the double or triple hit lymphomas remains often problematic in terms of immunophenotyping in association with MYC gene analysis. However, it was necessary to differentiate BL from the high grade B-cell lymphomas based on atypical immunophenotying. Eventually, molecular analysis helped us successfully reaching the correct diagnosis.

Precursor B lymphoblastic neoplasm usually presented as childhood leukemia. Most precursor lymphoblastic lymphoma are T-cell lineage and precursor B lymphoblastic lymphoma constitutes only about 10% of cases according to the WHO Classification of Tumours of Haematologic and Lymphoid Tissues. The most frequent sites of involvement in precursor B lymphoblastic lymphoma are the skin, soft tissue, bone and lymph nodes. Primary appendiceal involvement is an uncommon condition. We present an unusual case of primary appendiceal precursor B lymphoblastic lymphoma in an 11-year-old boy with peculiar histological morphology mimicking diffuse large B cell lymphoma. Histologically, the tumor was composed of diffusely infiltrated large cells from mucosa and extended to the subserosal area. The tumor cells were positive to CD79a, CD20, PAX5, BCL2, CD10, TdT, p53 but not to CD3, BCL6 and CD34 by immunohistochemical studies. The response to conventional treatment regimen for lymphoblastic lymphoma was not good, with early relapse within three months. Partial remission was achieved by adding rituximab. Unfortunately, the patient died in ten months due to uncontrolled relapsed disease with generalized lymphadenopathy and massive pleural effusion. The special morphologic changes and poor response to chemotherapy may be related to the overexpression of p53.

This case underlines the importance of family history in considering the possibility of malignancy in chronic inflammation. Lymphoma should be considered in patients with bone and joint pain not responding to analgesic and antibiotics. While MRI imaging can be useful in the diagnosis and staging of lymphoma, it has its limitations. In the diagnosis and prognostication of DLBCL, histopathology, molecular and cytogenetic analysis are essential features.

For more visit our home Journal Page Biomedical Research

Media Contact

Joel James

Biomedical Research