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KMID : 0387820060130020227
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Clinical Pediatric Hematology-Oncology
2006 Volume.13 No. 2 p.227 ~ p.232
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Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma in a Child
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Yi Hye-Ryoung
Min Kyung-Sun
Baek Hee-Jo
Han Dong-Kyun
Yun Sook-Jung
Nam Jong-Hee
Kook Hoon
Hwang Tai-Ju
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Abstract
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Primary cutaneous CD30+ lymphoproliferative disorders (LPDs) are very rare in children, which include primary cutaneous anaplastic large cell lymphoma (PCALCL), lymphomatoid papulosis (LyP), and borderline cases. It is now generally accepted that PCALCL and LyP form a spectrum of disease and they are difficult to differentiate from each other. A 8-year-old boy presented with a month-long history of a rapidly enlarging cutaneous mass in the periumbilical region. A nodular mass measuring 3×2×2 cm was completely excised at a local clinic. There was no lymphadenopathy or hepatosplenomegaly. There was no nodules or pustules, suggestive of LyP. Biopsy revealed sheets of large anaplastic cells. The cells showed positive immunoreactivity for CD30, CD45RO and CD3 but they didn’t express ALK, or EMA. No systemic involvement was noted. Bunn and Lamberg tumor staging was IIB (T3N0M0). Local radiation of 1,980 cGy was given to prevent relapse. One should aware that PCALCL, although rare, can occur in children. Close communication with clinicians and pathologists are essential, not only for making the diagnosis but also for preventing unnecessary aggressive therapy in these patients. Experiences in children and adolescents, however, are still limited, and long-term follow-up is needed.
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KEYWORD
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Primary cutaneous lymphoma, CD30, Anaplastic, Children
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