J Korean Pediatr Soc.  1994 Sep;37(9):1296-1304.

A Case of Congenital T Cell Lymphoblastic Lymphoma

• Yoo ES
• Hong YM
• Kim KH
• Gyu HS
• Chung EC

Abstract

T cell lymphoblastic lymphoma is characterized by immature lymphoid cells that are indistinguishable from the lymphoblasts and prolymphocytes of acute lymphoblastic leukemia. Several characteristic clinical features of lymphoblastic lymphoma, with include a high male-to-female ratio, a relatively high incidence in older children and young adults, the frequent presence of mediastinal involvement at the time of diagnosis. Also, this disease is rapidly progressive, and early dissemination to the bone marrow, blood, and central nervous system leads to the evolution of a picture resembling a acute lymphoblastic leukemia. We have experienced a rare case of congenital T-cell lymphoblastic lymphoma in which 1/365 year old female newborn had generalized multiple irregular protruding mass on her body. On bone marrow biopsy, CSF analysis, ultrasonogram and whole body MRI studies. We found metastasized tumor mass to, orbit, abdominal cavity, bulva, skin, and lower extremities. Histopathologically, specimen from mass on the right thigh showed diffuse infiltration of poorly differentiated and immature lymphoid cells in the skelectal muscle and subcutaneous soft tissue. By immunophenotyping studies using anti T cell and B cell monoclonal antibody, these cells were reactive with UCHL-1, MB2, but unreactive with MB1, L26. Based on these findings, the lesion was diagnosed as lymphoblastic lymphoma of the T-cell type, which occurred congenitally. Most T cell lymphoblastic lymphoma are noted, but congenital case was not reported. So we report it with a brief review of literature.

MeSH Terms

Abdominal Cavity
Biopsy
Bone Marrow
Central Nervous System
Child
Diagnosis
Female
Humans
Immunophenotyping
Incidence
Infant, Newborn
Lower Extremity
Lymphocytes
Magnetic Resonance Imaging
Orbit
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
Skin
T-Lymphocytes
Thigh
Ultrasonography
Young Adult