Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case

Citation
H. Itoh et al., Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case, J CUT PATH, 28(7), 2001, pp. 371-378
Citations number
26
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Dermatology
Journal title
JOURNAL OF CUTANEOUS PATHOLOGY
ISSN journal
0303-6987 → ACNP
Volume
28
Issue
7
Year of publication
2001
Pages
371 - 378
Database
ISI
SICI code
0303-6987(200108)28:7<371:PCLCHS>2.0.ZU;2-S
Abstract
Background: Langerhans cell histiocytosis (LCH) is a proliferating disorder of Langerhans cells (LC) that are characterized by the presence of Birbeck granules. LCH has been considered to be a disease of childhood and there h ave been limited cases of adult LCH. We report here a fatal case of histioc ytic tumor showing Langerhans cell phenotype, arising in the skin of a 74-y ear-old woman. Method: In addition to routine histological and immunohistological sections , electron microscopic examination and human androgen receptor gene (HUMARA ) assays were performed. Results: Histological examination revealed a dense dermal infiltrative prol iferation of fairly large tumor cells with abundant ill-defined cytoplasms and oval or indented nuclei, in which numerous eosinophils were associated with the tumor nests. Tumor cells were positive with and-S-100 and CD1a ant ibodies but negative with HMB-45 antibody or other epithelial or lymphocyti c markers. Ultrastructural analysis showed typical Birbeck granules in the cytoplasm of the tumor cells. HUMARA assay of the tumor tissue revealed the nonrandom X inactivation pattern, indicating the clonal proliferation. Conclusions: We diagnosed this tumor as Langerhans cell histiocytosis with a clonal neoplastic phenotype originated in the skin. Although she demonstr ated no recurrence nor metastases for 6 months after surgical resection of primary skin lesion and subsequent radiation therapy, the tumor recurred an d extended multisystemically, and she died of multiple organ failure 14 mon ths after initial diagnosis. Therefore, we would like to emphasize this cas e as LC "sarcoma" or "malignant" LCH.