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Non-Hodgkin’s Lymphoma Arising in Bone in Children and Adolescents Is Associated With an Excellent Outcome: A Children’s Cancer Group Report

From the Pathology Department, Children’s Hospital of Orange County/St Joseph Hospital, Orange, CA; Department of Pathology, University of Utah Health Sciences Center, Salt Lake City, UT; Operations Center, Children’s Oncology Group, Arcadia, CA; Keck School of Medicine, University of Southern California, Los Angeles, CA; Department of Pathology, Beth Israel Deaconess Hospital and Harvard Medical School, Boston, MA; Pathology Department, Children’s Mercy Hospital, Kansas City, MO; and Division of Pediatric Oncology, Children’s Hospital of New York, Columbia University, New York, NY.

Address reprint requests to Mark A. Lones, MD, Children’s Oncology Group, PO Box 60012, Arcadia, CA 91066-6012; email: smason@ childrensoncologygroup.org.

PURPOSE: Non-Hodgkin’s lymphoma (NHL) arising in bone is a heterogeneous histologic type of NHL that includes large-cell lymphoma, lymphoblastic lymphoma, and small noncleaved-cell lymphoma. NHL arising in bone is well recognized in adults but is less well characterized and infrequent in children and adolescents.

PATIENTS AND METHODS: We performed a retrospective review of Children’s Cancer Group (CCG) studies treating children and adolescents with NHL over a 20-year period (CCG-551, CCG-501, CCG-502, CCG-503, CCG-552, CCG-5911, and CCG-5941) and determined the response and event-free survival (EFS) rates in 31 patients with NHL arising in bone.

RESULTS: The patients ranged in age from 3 to 17 years (median, 11 years; mean, 11 years), and 64.5% were male. All 31 (100%) patients achieved complete response. For 31 patients with NHL arising in bone, the product-limit estimated 5-year EFS was 83.8% ± 6.7%. EFS in 17 patients with localized disease (Murphy stages I and II) was 94.1% ± 5.7%, and EFS in 14 patients with disseminated disease (Murphy stage III) was 70.7% ± 12.4% (log-rank P = .10). EFS in 17 patients treated with chemotherapy and radiation was 70.1% ± 11.2%, and EFS in 14 patients treated with chemotherapy without radiation was 100% (P = .03). EFS in 26 patients with histology-directed treatment (LSA2-L2 or ADCOMP for lymphoblastic, other therapy for nonlymphoblastic) was 92.2% ± 5.3%, and in five patients with nonhistology-directed treatment it was 40.0% ± 21.9% (P < .001).

CONCLUSION: NHL arising in bone is a heterogeneous type of NHL that makes up approximately 2.0% of NHL in children and adolescents on CCG studies. Response and survival in this young age group seem superb, with histology-directed treatment protocols without radiation in both localized and disseminated disease.

Presented in part at the Forty-Second Annual Meeting of the American Society of Hematology, December 1-5, 2000, San Francisco, CA.

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