J Korean Neurosurg Soc.  1993 Sep;22(9):1022-1025.

Histiocytosis-X with Giant Occipital Scalp Mass: A Case Report

• Lee YS
• Song SH
• Kim SH
• Kim KT
• Kim Y

Abstract

Histiocytosis is a relatively rare disorder of the reticuloendothelial system involving the proliferation of histicoytes, granulation tissue, and inflammatory cells in many different organ systems1). Thus, the three manifestations of the same basic pathologic process:Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease have been classified as localized, chronic disseminated and acute disseminated histiocytosis-X. They were therefore included under the term histiocytosis-X and this concept has been generally accepted. The authors have experienced one case of histiocytosis-X, a rare disease. A 11 month-old femal patient presented with gradually enlarged palpable mass on the occipital area. The occipital skull was defected in a punched out fashion. The mass was completely removed. The pathologic findings revealed Histiocytosis-X and the patient was given chemotherapy.

Keyword

Histiocytosis-X; Eosinophilic granuloma; Hand-Schuller-Christian disease; Letterer-Siwe disease

MeSH Terms

Drug Therapy
Eosinophilic Granuloma
Granulation Tissue
Granuloma
Histiocytosis
Histiocytosis, Langerhans-Cell*
Humans
Infant
Mononuclear Phagocyte System
Rare Diseases
Scalp*
Skull