Dados do Trabalho

Título

A rare case of primary cutaneous precursor T-cell lymphoblastic lymphoma in a Brazilian child.

Resumo

Objectives: To describe a rare case of cutaneous Non-Hodgkin's Lymphoblastic T lymphoma.

Methods: The case history is described of a five-year-old boy presented to our clinic with multiple cutaneous stain on the body. The history revealed that the first lesion had occurred ten months ago on the left cheek and had progressive growth. Dermatological examination revealed a 5,6x5,5 cm sized, hard, brown-red-colored, dome-shaped lesion. There were others similar smalls lesions on the right cheek, limbs, and chest. On the last four months he developed periodic fever, joint pain, night sweats, and weigh loss. Histopathological examination revealed small, round, relatively undifferentiated cells. Immunohistochemically, cells were positive for CD3 and TdT and negative for MPO, CD10, CD1a and CD79. The KI 67 proliferation index was 50%. Peripheral blood and bone marrow aspiration smear, cerebrospinal fluid analysis revealed no evidence of any neoplastic populations. Whole-Body magnetic resonance reveled multiple cutaneous lesions spread over the body, the largest on the left cheek, with signs of extension to the subcutaneous tissue. Chest computed tomography was normal. After the diagnosis of cutaneous non-Hodgkin Lymphoblastic T lymphoma, the patient received treatment according to The Brazilian Non-Hodgkin Lymphoma Protocol for Childhood and Adolescence (LNH2016). Currently he is in remission and going to the last phase of the protocol.

Discussion: Lymphoblastic lymphoma (LBL) is an uncommon non-Hodgkin lymphoma (NHL) and accounts for 20% of all NHL. It can be of T-cell or B-cell lineage.1 Cutaneous T-cell LBL comprises a heterogeneous group of lymphoproliferative disorders defined by the primary expansion of malignant T lymphocytes to the skin. T-cell LBL account for around 3.5% to 7% of all cutaneous lymphomas, and skin involvement by T-cell LBL is a rare, with incidence around 4.3% of the cases.2,3 Although LBL is a highly aggressive malignant neoplasm, its prognosis is improved with the use of aggressive multi-agent chemotherapy ALL-regimens with up to 90% of 5-year EFS rate.

Conclusion: This case serves as an important reminder to consider this entity as a differential diagnosis for head and neck tumors especially in the pediatric population.