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Journal of Clinical Oncology, Vol 22, No 7 (April 1), 2004: pp. 1331-1333 © 2004 American Society of Clinical Oncology. DOI: 10.1200/JCO.2004.05.060
Unusual Presentations of Hematologic MalignanciesCASE 2. Precursor B-Cell Lymphoblastic Lymphoma Presenting As Spinal Cord CompressionHenry Ford Hospital, Detroit, MI A 58-year-old African American woman presented to the emergency room with a 3-day history of upper back pain and numbness in her abdominal wall. The numbness was rapidly progressive and had involved her chest and legs by the time she presented to us. She also had developed weakness of her lower extremities. On examination in the emergency department, she had tenderness over her upper thoracic spine, and motor and sensory loss in her lower extremities. Laboratory evaluation on admission was unremarkable. Emergent magnetic resonance imaging was done in the emergency department showing an enhancing epidural mass from T2 to T5 (Fig 1, arrows), predominantly on the left, causing marked spinal cord compression (Fig. 1 and 2; "c" indicates the spinal cord). The patient underwent emergent surgery to decompress her spinal cord. She had gradual improvement in her motor and sensory symptoms following surgery. Histological sections of the biopsy material showed soft tissue heavily infiltrated by sheets of small- to medium-sized lymphocytes with scant amounts of cytoplasm and irregular nuclei (Fig 3). Mitoses were easily identified (Fig 3). Immunohistochemical staining was positive for CD79a and TdT. The combined morphologic and immunophenotypic profile of the epidural mass was that of precursor B-lymphoblastic lymphoma (B-ALL)/leukemia (B-LBL). Bone marrow aspirate and biopsy revealed only 4% lymphoblasts, and flow cytometric analysis was positive for involvement with precursor B-ALL/B-LBL. Flow cytometry was positive for CD10, CD19, CD34, CD45, CD56, CD79a, and TdT, and negative for CD3, CD5, and CD7. No evidence of lymphadenopathy or other mass lesion was found on physical examination or body imaging. Magnetic resonance imaging of the brain was also negative. There were no skin or bone lesions. A peripheral smear also did not reveal any abnormalities. Chemotherapy was initiated.
Precursor B-ALL/ B-LBL is a neoplasm of lymphoblasts committed to the B-cell lineage. Because of the biologic unity of B-ALL and B-LBL, the use of one or the other term in some patients is arbitrary. When the process is confined to a mass lesion with no or minimal blood and bone marrow involvement (< 25% blasts in the bone marrow), the diagnosis is LBL,1 while patients with ALL present with extensive bone marrow and blood involvement.1 This is an arbitrary distinction. Accordingly, LBL and ALL should be considered the same disease with different clinical presentations.2 It is not unusual for a patient with LBL to relapse in the CNS despite prophylaxis. Diffuse leptomeningeal infiltration after the diagnosis or during relapse is also common,3 and there is a case report of discrete spinal cord mass formation during remission.4 However, a mass causing cord compression as an initial manifestation of lymphoblastic lymphoma is rare, and to our knowledge, has not been previously reported. LBL is a high-grade subtype of non-Hodgkin's lymphoma. The majority of the LBL's are of the precursor T-cell type, and only a small proportion (< 20%)5 exhibit a precursor B-cell phenotype. The median age at presentation of the precursor B-LBL's is 20 years. The most frequent sites of involvement of the precursor B-LBL are the skin,6 especially in the head and neck region6,7; lymph nodes; and soft tissue. However, there are case reports in which the initial presenting symptoms involve ovaries, retroperitonium, tonsil,8 uterus,9 stomach and colon,10 mediastinum,10 and both lytic11 and blastic2 bone lesions. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Recine M, Castellano-Sanchez AA, Sheldon J, et al: Precursor B-cell lymphoblastic lymphoma/leukemia presenting as osteoblastic bone lesions. Ann Diagn Pathol 6:236-243, 2002[CrossRef][Medline]
2. Harris NL, Jaffe ES, Diebold J, et al: WHO classification of neoplastic diseases of the hematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee meeting—Airlie House, Virginia, Nov 1997. J Clin Oncol 17:3835-3849, 1999 3. Weizsaecker M, Koelmel HW: Meningeal involvement in leukemias and malignant lymphomas of adults: Incidence, course of disease, and treatment of prevention. Acta Neurol Scand 60:363-370, 1979[CrossRef][Medline]
4. Kudoh T, Otoi H, Oda T, et al: Simultaneous development of a pineal tumor and an intradural spinal mass during remission of acute lymphocytic leukemia. Jpn J Clin Oncol 30:105-108, 2000
5. Harris NL, Jaffe ES, Stein H, et al: A revised European-American classification of lymphoid neoplasms: A proposal from the International Lymphoma Study Group. Blood 84:1361-1392, 1994 6. Sander CA, Jaffe ES, Yano T, et al: Lymphoblastic lymphoma of B-cell lineage (B-LBL) involving skin. Lab Invest 68:99, 1993 (abstr) 7. Shibata K, Shimamoto Y, Yamada H, et al: Correlation between immunophenotypic diversity and clinical features in B-cell lymphoblastic lymphoma. Ann Hematol 71:319-323, 1995[CrossRef][Medline]
8. Maitra A, McKenna RW, Weinberg AG, et al: Precursor B-cell lymphoblastic lymphoma: A study of nine cases lacking blood and bone marrow involvement and review of the literature. Am J Clin Pathol 115:868-875, 2001 9. Koliopoulos G, Parkin D, Paraskevaidis E: A case of B-cell lymphoblastic lymphoma involving the uterus. Eur J Gynaecol Oncol 23:113-114, 2002[Medline] 10. Lin P, Jones D, Dorfman D, et al: Precursor B-cell Lymphoblastic Lymphoma: A predominantly extranodal tumor with low propensity for leukemic involvement. Am J Surg Pathol 24:1480-1490, 2000[CrossRef][Medline] 11. Iravani S, Singleton T, Ross CW, et al: Precursor B lymphoblastic lymphoma presenting as lytic bone lesions. Am J Clin Pathol 112:836-843, 1999[Medline]
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Copyright © 2004 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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