Journal of Gastroenterology and Hepatology | 2019
Gastrointestinal: Lymphoblastic lymphoma presenting as huge abdominal masses
Abstract
An 18-year-old woman presented to the emergency department with a 2-week history of diffuse abdominal pain. On physical examination, there were tender palpable abdominal masses in the right upper quadrant and periumbilical areas. Initial laboratory data showed leukocytosis (12 470/μL) with elevated C-reactive protein (17.99 mg/dL) and lactate dehydrogenase (397 U/L) levels. Abdominopelvic computed tomography and positron emission tomography showed three huge heterogeneous enhancing masses with hypermetabolism (10 × 7.8 cm, 3.1 × 9.6 cm, and 4.2 × 5.1 cm) in the abdominal cavity (Figs. 1 a,b). The patient agreed to perform further evaluation, and there was no significant lesion noted on upper and lower endoscopy. Ultrasound-guided gun biopsy using an 18-gauge needle for the right upper quadrant mass was performed without complication (Fig. 1c). Microscopic findings showed a malignant small round cell tumor. Immunohistochemical (IHC) staining showed positivity for cluster of differentiation (CD)10, CD19, CD79a, and PAX-5 and negativity for CD1a, CD3, and terminal deoxynucleotidyl transferase (Fig. 2). The final pathologic diagnosis was B-cell lymphoblastic lymphoma (LBL). The patient received intensive acute lymphocytic leukemia (ALL)-type chemotherapy. LBL is one of the immature or precursor lymphoid cell neoplasms and is classified with B-cell or T-cell lineage. LBL is relatively rare, and B-cell LBL only accounts for less than 10% of cases of LBL. LBL predominates in young adults and adolescents and shows a slight male predominance. The clinical presentation varies according to the cell type. While T-cell LBL predominantly involves the mediastinum, B-cell LBL more frequently involves the skin, subcutaneous tissue, or bone. LBL with abdominal involvement is rare. Bone marrow involvement is found in 10–40%. For precise diagnosis, core needle biopsy or excisional biopsy with IHC staining is essential. In LBL, current treatment strategies are based on intensive multidrug ALLtype chemotherapy, including central nervous system prophylaxis and/or mediastinal radiation therapy, depending on protocol design and early therapeutic response.