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Acquired Gaucher Cells Located in Dermis near a Malignant Hidradenoma

Jules J. Berman, PhD, MD and Oscar A. Iseri, MD

Ultrastructural Pathology, 12:245-246, 1988

Gaucher cells are histiocytes containing accumulated cerebroside. These cells are typically large (20-80 microns) and engorged with a faintly fibrillar material. On electron microscopic examination the cytoplasm is filled with membrane-bounded sheaths of long tubular elements. In Gaucher's disease, a metabolic deficiency of betaglucocerebrosidase results in Gaucher cells appearing in many organs. Acquired Gaucher cells appear in the RES in conditions that generate, through rapid cell turnover, such large amounts of cerebroside that local macrophages become engorged.

For the most part, this phenomenon is limited to conditions of rapid bone marrow turnover, as blood cells, particularly neutrophils, generate large levels of ceramide lactoside. Acquired Gaucher cells are seen in chronic myelogenous leukemia, thalassemia, congenital dyserythropoietic anemia, and the so-called sea-blue histiocyte syndrome (1).

In addition, Gaucher cells have also been encountered in lobo mycosis owing to breakdown of fungal cell walls (2) and in granular cell tumors (3).

As an incidental finding in a case of recurrent malignant hidradenoma, we noticed plump histiocytes surrounding a dermal Pacinian corpuscle (Fig. 1). This structure was located several millimeters from a tumor diagnosed in our department as malignant hidradenoma. The tumor was poorly differentiated with a high mitotic rate. Many cells had abundant clear cytoplasm. No other similar hystiocytes were seen in related dermal structures or in the tumor itself. The tissue containing the Pacinian corpuscle was cut from the paraffin block and processed for electron microscopic examination.



Pacinian corpuscle surrounded by Gaucher cells Gaucher cells surrounding Pacinian corpuscle

FIG. 1. Dermal Pacinian corpuscle surrounded by histiocyte. Higher power on right. H&E.



Around the Pacinian corpuscle were large cells filled with sheaths of rod like material (Fig. 2). These rodlike structures, seen at higher power, were smooth, rather straight tubes (Fig. 3). Delimiting membranes were apparently lost in paraffin processing. The patient expired several months later with widespread metastases. No other Gaucher cells were noted at autopsy with careful examination of the spleen, bone marrow, and liver.

Gaucher cell.  Electron microscopy

FIG. 2. Histiocyte filled with sheaths of thin tubular rods, EM x 5000.



Gaucher cell with sheaths.  Electron microscopy Sheaths in Gaucher cell.  Electron microscopy

FIG. 3. Oval packet of rods showing tubelike structure, EM x 33,200.

We presume that hidradenoma-derived cell lipid drained to the space around the Pacinian corpuscle to be engorged by local phagocytic cells. With the exception of granular cell tumor, this is, to our knowledge, the only report of Gaucher cells associated with a nonhematologic neoplasm.



REFERENCES

1. Wintrobe MM: Clinical Hematology, 8th Ed., pp. 1340-1344. Philadelphia: Lea and Febiger, 1981.

2. Bhawan J, Bain RW, Purtilo DT, et al.: Lobomycosis. An electron microscopic, histochemical and immunologic study. J Cutan Pathol 3:5-16, 1976.

3. Bhawan J, Malhotca R, Neik DR: Gaucher-like cells in a granular cell tumor. Hum Pathol 14:730-733, 1983.

Author note [jb]. This work was prepared by the authors as a part of their work activites for the U.S. Government. Therefore, text and images cannot be copyrighted and belong to the public domain.

Key words. Gaucher disease, Gaucher's disease, electron microscopy, surgical pathology, dermatopathology, histopathology, photomicrograph, histology, public domain images


   Last modified: Jan 12, 2013

Books by Jules J. Berman, covers