Isolated Lymphadenopathy as the First Presentation of Systemic Mastocytosis – Description of Two Cases

Czech version

Authors: Z. Kinkor ¹; R. Šíma ²; A. Skálová ¹; L. Boudová ²; S. Peková ³; D. Dvořáková ⁴; K. Dědič ⁵; M. Kracík ⁶; M. Janoušek ⁷; M. Michal ²
Authors‘ workplace: Bioptická laboratoř s. r. o., Plzeň ¹; Šiklův ústav patologie, LF UK, Plzeň ²; Oddělení klinické biochemie, hematologie a imunologie, Nemocnice Na Homolce, Praha ³; Interní hemato-onkologická klinika, Dětská nemocnice, FN Brno ⁴; Fingerlandův ústav patologie, LF UK a FN, Hradec Králové ⁵; Oddělení ušní, nosní, krční, Nemocnice Jičín ⁶; Oddělení patologie, Nemocnice Kladno ⁷
Published in: Čes.-slov. Patol., 42, 2006, No. 1, p. 34-38
Category:

Overview

Presented are two cases of systemic mastocytosis in 46- and 63-year-old women, where the correct diagnosis was established in randomly disclosed cervical respectively intraabdominal lymphadenopathy. Both cases lacked characteristic skin and systemic mast-cell mediator symptoms at the time of histologic diagnosis. The first case was classified as a indolent systemic mastocytosis without any proven genetic alteration, the second one met the criteria of aggressive systemic mastocytosis with eosinophilia, where the point mutation asp816val in c-kit gene was confirmed and the patient responded unexpectedly well to Gleevec. Discussed are both conventional morphological differential diagnosis of mastocytosis in lymph nodes and recent advances in genetics of these systemic clonal mast cell proliferations. The latter not only outlines the oncopathogenesis but, in particular, also provides important prognostic and biological implications of this peculiar disease.

Key words:
lymph node – systemic mastocytosis – molecular genetic – c-kit – PDGFRa

Labels

Anatomical pathology Forensic medical examiner Toxicology

Pyloric Gland Adenoma – How to Diagnose?

Human Embryonal Tissues of all Three Germ Layers can Express the CD30 Antigen. An Immunohistochemical Study of 30 Fetuses Coming after Therapeutic Abortions from Week 8th to week 16th of Gestation

Expression of Matrix Metalloproteinases 3, 10 and 11 (Stromelysins 1, 2 and 3) and Matrix Metalloproteinase 7 (Matrilysin) by Cancer Cells in Non-Small Cell Lung Neoplasms. Clinicopathologic Studies

Cervical Cancer Precursors: Cytohistologic Correlation with the Results of HPV Testing

Known Pitfalls of the Thyroid Neoplasm Diagnostics in the View of the New (2004) WHO Classification

Histologic Findings in Subantral Augmentation (Sinus Lift)

Malignant Fibrous Histiocytoma of the Breast: Report of Two Cases

Article was published in

Czecho-Slovak Pathology

2006 Issue 1