Leukemic Phase of Histiocytic Sarcoma of the Digestive System: A Rare Manifestation of a Rare Disease

Liron Hofstetter, David Aranovich, Hanna Bernstine, Lucille Hayman, Yael Shahal-Zimra, Esther Rabizadeh, Amos Cohen, Meir Lahav, Pia Raanani, Ofir Wolach

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Histiocytic sarcoma (HS) is a rare, malignant, and aggressive subtype of histiocytosis. We present an unusual case of aggressive HS presenting in the gastrointestinal tract and gallbladder that progressed after several lines of chemotherapy with a leukemic phase. We review the clinical, pathological, and molecular characteristics of HS in this case and review the literature on HS involving the digestive system as well as on overt leukemic phase of this disease. HS is often diagnosed at an advanced stage, and mortality is high. We discuss the therapeutic approach to patients with HS. We highlight the role of overexpression and somatic alterations in the RAF-MEK-ERK pathway in the pathogenesis of HS and discuss potential targeted approaches to treat these rare tumors.

Original languageEnglish
Pages (from-to)229-235
Number of pages7
JournalActa Haematologica
Volume144
Issue number2
DOIs
StatePublished - Mar 2021
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2021 S. Karger AG. All rights reserved.

Keywords

  • Digestive system
  • Histiocytic sarcoma
  • Leukemic phase

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