Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder in a pediatric patient successfully treated with low-dose radiation

Esther J. Kim, Alexander B. Aria, Kelly Wilmas, Daniel J. Lewis, Carlos A. Torres-Cabala, Cesar Nunez, Bouthaina S. Dabaja, Madeleine Duvic

Research output: Contribution to journalArticle

Abstract

Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.

Original languageEnglish (US)
Pages (from-to)e23-e26
JournalPediatric Dermatology
Volume36
Issue number1
DOIs
StatePublished - Jan 1 2019

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Lymphoproliferative Disorders
Radiation
Pediatrics
T-Lymphocytes
Skin
Standard of Care
Neoplasms
Histology
Radiotherapy
Cell Proliferation
Therapeutics

Keywords

  • lumps/bumps
  • neoplasms—benign
  • neoplasms—malignant
  • therapy—topical

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Dermatology

Cite this

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title = "Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder in a pediatric patient successfully treated with low-dose radiation",
abstract = "Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.",
keywords = "lumps/bumps, neoplasms—benign, neoplasms—malignant, therapy—topical",
author = "Kim, {Esther J.} and Aria, {Alexander B.} and Kelly Wilmas and Lewis, {Daniel J.} and Torres-Cabala, {Carlos A.} and Cesar Nunez and Dabaja, {Bouthaina S.} and Madeleine Duvic",
year = "2019",
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language = "English (US)",
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T1 - Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder in a pediatric patient successfully treated with low-dose radiation

AU - Kim, Esther J.

AU - Aria, Alexander B.

AU - Wilmas, Kelly

AU - Lewis, Daniel J.

AU - Torres-Cabala, Carlos A.

AU - Nunez, Cesar

AU - Dabaja, Bouthaina S.

AU - Duvic, Madeleine

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.

AB - Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.

KW - lumps/bumps

KW - neoplasms—benign

KW - neoplasms—malignant

KW - therapy—topical

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