title: Bexarotene is active against subcutaneous panniculitis-like T-cell lymphoma in adult and pediatric populations.
abstract: BACKGROUND
  Subcutaneous panniculitis-like T-cell lymphoma (SPTL-AB) and cutaneous gamma/delta T-cell lymphoma (CGD-TCL) are rare T-cell lymphomas with varying clinical courses. There is no standard treatment, although chemotherapy and hematopoietic stem cell transplantation are commonly used. We describe results using bexarotene for children and adults with these disorders.
  
  
  METHODS
  We identified 15 patients (12 adults, 3 children) who were treated with bexarotene between 2000 and 2010 from the Memorial Sloan-Kettering Cancer Center lymphoma database, the Stanford Cancer Center Registry, and the National Cancer Institute (NCI) pediatric lymphoma database. There were 8 females and 7 males, with a median age of 45 years (range, 3 years to 85 years). All patients had stage IV disease. Two of 15 and 4 of 15 patients had documented CGD-TCL and SPTL-AB, respectively; others were presumed to have SPTL-AB. Bexarotene was administered at flat doses corresponding to 91 to 339 mg/m(2)/d. Two of 15 patients received concurrent denileukin diftitox. Two children received bexarotene as maintenance therapy and were not evaluable for response.
  
  
  RESULTS
  Among those treated with bexarotene alone, the overall response rate (ORR) was 82% (6/11 complete response [CR], 3/11 partial response [PR]). One of the 2 patients treated with concomitant denileukin diftitox responded for an ORR of 10/13 (77%), including 54% CR and 23% PR. Median progression-free survival was 38.4 months; median duration of response was 26.3 months. Six patients developed hypothyroidism and 9 developed hyperlipidemia; one patient developed dose-limiting hypertriglyceridemia. One pediatric patient developed insulin-dependent diabetes mellitus.
  
  
  CONCLUSIONS
  In this retrospective series, bexarotene showed a high response rate in SPTL-AB and CGD-TCL. It was generally well-tolerated with durable responses; therefore, bexarotene represents a promising therapy for children and adults with these disorders.
authors: Department of Medicine, New York Presbyterian-Weil Cornell Medical Center, 1275 York Avenue, New York, NY 10021, USA.
authors: Neha
authors: N
authors: Mehta
authors: Alan S
authors: AS
authors: Wayne
authors: Youn H
authors: YH
authors: Kim
authors: Gregory A
authors: GA
authors: Hale
authors: Carlos S
authors: CS
authors: Alvarado
authors: Patricia
authors: P
authors: Myskowski
authors: Elaine S
authors: ES
authors: Jaffe
authors: Klaus J
authors: KJ
authors: Busam
authors: Melissa
authors: M
authors: Pulitzer
authors: Jeffrey
authors: J
authors: Zwerner
authors: Steven
authors: S
authors: Horwitz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016588:Anticarcinogenic Agents / Q000009:adverse effects / Q000627:therapeutic use; D000077610:Bexarotene; D002648:Child; D002675:Child, Preschool; D003922:Diabetes Mellitus, Type 1 / Q000139:chemically induced; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D006949:Hyperlipidemias / Q000139:chemically induced; D015228:Hypertriglyceridemia / Q000139:chemically induced; D007037:Hypothyroidism / Q000139:chemically induced; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D015434:Panniculitis / Q000188:drug therapy* / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000473:pathology; D013764:Tetrahydronaphthalenes / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016588:Anticarcinogenic Agents; D013764:Tetrahydronaphthalenes; D000077610:Bexarotene