Dermatology MCQ - Viral Infections - Burkitt lymphoma

A 35-year-old man from East Africa presents with a painless, rapidly enlarging neck mass. Imaging reveals a large nodal mass in the cervical chain, and biopsy shows a "starry sky" appearance under microscopy with large, atypical lymphoid cells positive for CD20 and CD10. In situ hybridization is positive for Epstein-Barr virus-encoded small RNA (EBER). Which of the following is the most likely diagnosis?

A) Hodgkin lymphoma
B) Burkitt lymphoma
C) Nasopharyngeal carcinoma
D) Diffuse large B-cell lymphoma
E) Post-transplant lymphoproliferative disorder

Correct Answer: B) Burkitt lymphoma

Explanation

This presentation is classic for endemic Burkitt lymphoma, an aggressive B-cell neoplasm strongly associated with Epstein-Barr virus (EBV) infection.

Key Features of Burkitt Lymphoma:

• Epidemiology: Endemic form is most common in equatorial Africa and is strongly linked to EBV infection (95-100% of cases) and malaria co-infection, which may impair immune surveillance.

• Clinical Presentation: Often presents with a rapidly growing nodal or extranodal mass. Common sites include the jaw (in children) and abdomen or cervical nodes (in adults).

• Histopathology:

  • "Starry sky" appearance: Due to scattered tingible-body macrophages (containing apoptotic debris) amidst sheets of monomorphic, medium-sized lymphoid cells with basophilic cytoplasm.

  • Immunophenotype: Tumor cells are positive for CD20 (B-cell marker), CD10 (germinal center marker), and BCL6, but negative for BCL2 and TdT.

• EBV Association: EBER positivity by in situ hybridization is a hallmark of the endemic form, indicating latent EBV infection within tumor cells.

Why Not the Other Options?

• (A) Hodgkin lymphoma: Characterized by Reed-Sternberg cells (CD15+, CD30+), not the monomorphic cells of Burkitt lymphoma. EBV is associated with mixed-cellularity subtype but not as strongly as in Burkitt.

• (C) Nasopharyngeal carcinoma: An EBV-associated epithelial tumor originating in the nasopharynx, not lymph nodes. It presents with nasal obstruction, epistaxis, or cervical nodes but lacks the "starry sky" pattern.

• (D) Diffuse large B-cell lymphoma (DLBCL): May be EBV-positive but typically occurs in older adults or immunocompromised hosts. It lacks the classic "starry sky" pattern and has a more heterogeneous immunophenotype (e.g., CD10 may be negative).

• (E) Post-transplant lymphoproliferative disorder (PTLD): Occurs in immunocompromised patients (e.g., post-transplant) and is often EBV-driven. It can resemble Burkitt morphologically but is not endemic and occurs in a specific clinical context.

Management:

• High-intensity chemotherapy: Regimens like CODOX-M/IVAC or R-EPOCH are used due to the tumor's high proliferative rate.

• Rituximab: Anti-CD20 monoclonal antibody improves outcomes.

• Prognosis: Curable with aggressive therapy, but rapid diagnosis and treatment are critical.

Prevention:
Malaria control in endemic areas may reduce Burkitt lymphoma incidence.