Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children
Alison R Huppmann, Stefania Pittaluga, Mark Raffeld, Liqiang Xi, Elaine S Jaffe. National Institutes of Health/National Cancer Institute, Bethesda, MD
Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare neoplasm of αβ cytotoxic T cells that typically occurs in young adults. Cases in children have previously been described only as case reports or included in larger series also encompassing adults but no dedicated pediatric series has been reported. This study describes the clinical features and histologic, immunohistochemical (IHC) and molecular findings in a cohort of pediatric patients with SPTCL.
Design: The surgical pathology files of our institution were searched for all cases of SPTCL diagnosed between 1999 and July 2011. Only patients age 21 or younger at the time of biopsy were included. All available H&E and IHC slides were reviewed, as well as results of PCR for T-cell receptor (TCR) gene rearrangement. The provided clinical history was also recorded.
Results: A total of 19 biopsies from 16 patients were identified. Age ranged from 5 months to 21 years, with a male:female ratio of 0.6. One child was reported to have trisomy 13, another had cardiovelofacial abnormalities, and a third was reported to have mixed connective tissue disease. Specimens were most commonly taken from the extremities (9) or trunk (7), with 1 from the neck and 2 unspecified. Most cases displayed rimming of adipocytes by neoplastic cells, karyorrhexis, and fat necrosis without epidermal or dermal involvement. Plasma cells were focally prominent in 2 cases, a feature which is more common in lupus panniculitis. CD3 and CD8 were each positive in 18/18, and all tested cases were negative for CD4 (0/17) and/or CD56 (0/12). Cytotoxic markers (TIA-1, granzyme B) were positive in every stained case, as was beta F1. In-house or reported TCR PCR detected 11 clonal rearrangements, 2 indeterminate cases, and 4 cases with no significant clone. Slides from 2/4 sections without a clone demonstrated only focal involvement.
Conclusions: The clinical and pathological features of SPTCL occurring in children are similar to those described in adults. Additional follow-up information will be sought to investigate whether these pediatric patients share a relatively indolent course.
Wednesday, March 21, 2012 9:30 AM
Poster Session V # 191, Wednesday Morning