Intravascular ALK-negative anaplastic large cell lymphoma with localized cutaneous involvement and an indolent clinical course: Toward recognition of a distinct clinicopathologic entity

Ryan A. Metcalf, Sameer Bashey, Ashley Wysong, Jinah Kim, Youn H. Kim, Dita Gratzinger

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Intravascular large T-cell or NK-cell lymphomas rarely present with cutaneous involvement. Intravascular cytotoxic T or NK lymphomas presenting in the skin (cIT/NKL) are often EBV, and reported cases follow a highly aggressive clinical course. Intravascular anaplastic large cell lymphoma (ALCL) by contrast is extraordinarily rare and, when it presents in the skin, raises the question of aggressive clinical behavior in the manner of cIT/NKL versus indolent clinical behavior in the manner of primary cutaneous ALCL. Here we describe a case of localized cutaneous intravascular anaplastic lymphoma kinase-negative ALCL (cIALCL) with a very indolent clinical course. The patient experienced a single cutaneous relapse and remains alive without disease 4 years after diagnosis. Review of the literature reveals multiple clinicopathologic differences between cIALCL and cIT/NKL: distribution (cIALCL, single skin region, P=0.021, Fisher exact test); histology (cIALCL, cohesive with necrosis, P=0.005); immunophenotype (cIALCL, strongly CD30, P=0.021; cIT/NKL, CD56 and/or EBV, P=0.003); and indolent clinical behavior with a trend toward better overall survival (P=0.067, Kaplan-Meier survival analysis). Our index case of cIALCL and 1 other tested case were immunohistochemically confirmed to be intralymphatic (contained within D2-40+vessels) as compared with the blood vessel localization of cIT/NKL. Recognition of cIALCLs as a distinct clinicopathologic entity, and in particular their distinction from aggressive, usually EBV cIT/NKLs, may be possible on the basis of a combination of clinicopathologic criteria, allowing for localized therapy in a subset of patients.

Original languageEnglish (US)
Pages (from-to)617-623
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume37
Issue number4
DOIs
StatePublished - Apr 1 2013

Fingerprint

Anaplastic Large-Cell Lymphoma
Skin
Human Herpesvirus 4
Primary Cutaneous Anaplastic Large Cell Lymphoma
Lymphoma
Kaplan-Meier Estimate
Survival Analysis
Natural Killer Cells
Blood Vessels
Histology
Necrosis
T-Lymphocytes
Recurrence
Survival

Keywords

  • anaplastic large cell lymphoma
  • cutaneous
  • intravascular
  • lymphatic

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Intravascular ALK-negative anaplastic large cell lymphoma with localized cutaneous involvement and an indolent clinical course : Toward recognition of a distinct clinicopathologic entity. / Metcalf, Ryan A.; Bashey, Sameer; Wysong, Ashley; Kim, Jinah; Kim, Youn H.; Gratzinger, Dita.

In: American Journal of Surgical Pathology, Vol. 37, No. 4, 01.04.2013, p. 617-623.

Research output: Contribution to journalArticle

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