title: Primary cutaneous CD30-positive anaplastic large cell lymphoma in childhood: report of 4 cases and review of the literature.
abstract: We present the clinicopathologic findings in 4 children with primary cutaneous anaplastic large cell lymphoma (C-ALCL). The patients ranged in age from 13 months to 8 years, with 3 females and 1 male. All presented with a rapidly enlarging mass involving the skin and subcutaneous tissue. Histologic evaluation showed sheets of large pleomorphic lymphoid cells that were diffusely and strongly CD30(+). Tumor cells were CD45(+) in 1 of 4 cases. Cells were of T-cell phenotype, with variable positivity for CD3 (3 of 4 cases) and CD5 (2 of 4 cases). All 4 cases were positive for CD4 and clusterin. Staining for anaplastic lymphoma kinase was negative in all cases. No evidence of systemic involvement was noted at initial presentation or over a follow-up of 5 to 78 months, although 3 patients had cutaneous recurrences. Primary C-ALCL has only rarely been described in the pediatric population. The high-grade histologic appearance of this lymphoma belies its generally favorable clinical course and prognosis. Recognition of this entity and its differentiation from other T-cell lymphomas that secondarily involve the skin is important to avoid unnecessarily aggressive therapy in these children.
authors.affiliation: Department of Anatomic Pathology, Children's National Medical Center, 111 Michigan Avenue NW, Washington, DC 20010, USA. shimareet.kumar@med.va.gov
authors.forename: Shimareet
authors.initials: S
authors.lastname: Kumar
authors.forename: Stefania
authors.initials: S
authors.lastname: Pittaluga
authors.forename: Mark
authors.initials: M
authors.lastname: Raffeld
authors.forename: Michael
authors.initials: M
authors.lastname: Guerrera
authors.forename: Nita L
authors.initials: NL
authors.lastname: Seibel
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D017730:Ki-1 Antigen / Q000378:metabolism*; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D008297:Male; D012878:Skin Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology
chemical_list: D017730:Ki-1 Antigen