© Borgis - New Medicine 3/2008, s. 55-56
*Lechosław P. Chmielik, Magdalena Frąckiewicz, Mieczysław Chmielik
MALHERBE´S TUMOURS TREATED IN THE DEPARTMENT OF PAEDIATRIC ENT
Department of Paediatric Otorhinolaryngology, Medical University of Warsaw, Poland
Head of Department: Prof. Mieczysław Chmielik, MD, PhD
Introduction: Malherbe´s tumour is rarely recognised amongst tumours of the head and neck. However, in some papers, several tumours of this type have been described. Calcifying epithelioma is a benign neoplasm, localised within the skin. It is found more often in females than in males. The literature suggests that it is genetic in origin.
Aim: To analyse the cases of Malherbe´s tumour treated in the Paediatric ENT Clinic in
Warsaw. Between 1992 and 2007 two cases of Malherbe´s tumour were treated in the Paediatric ENT Clinic in Warsaw.
Material and method: In view of the rare incidence and difficulty in describing this tumour, the authors have decided to present two cases treated in their clinic between 1992 and 2007. The first case is that of a three-year-old girl with a Malherbe´s tumour in the pre-auricular region, and the second is that of a three-year-old girl with a Malherbe´s tumour in the retro-auricular region. Both cases were treated surgically. Diagnosis was based on histopathological examination. In both cases the postoperative period was without complications, and there have been no recurrences.
Results: Both cases were histopathologically diagnosed as Malherbe´s tumour. Surgical treatment in both cases gave a good therapeutic effect.
Conclusions: In cases of the calcifying epithelioma known as Malherbe´s tumour, the treatment is surgical. Recognition of the tumour requires histopathological examination.
Nowadays, among tumours of the head and neck, calcifying epithelioma of Malherbe (pilomatricoma) is rarely seen. Pilomatricoma was first described by Malherbe and Chenantais in 1880 (1, 3, 8). It is a benign, restricted lesion, located mainly in the skin of the head, neck, or upper limbs. It is thought to derive from the hair germinal matrix (1, 2, 4, 9, 10, 11). Pilomatricoma formation disturbs the hair follicle cycle, which stops the development into mature hair. Recent genetic research suggests that calcifying epithelioma of Malherbe may be caused by activating mutations in β-catenin, a cytoplasmic protein involved in gene transcription (6). The tumour appears mostly in Caucasians, more frequently in females than in males, and occurs in the first three decades of life. Histologically, calcifying epithelioma of Malherbe is mainly composed of two types of cells, basaloid cells and shadow (ghost) cells, as well as giant cells, foreign body type. Calcification and ossification may occur. The diagnosis is based on histopathological examination. Some authors have described family occurrence of this tumour (1, 5, 6).
To analyse the cases of Malherbe´s tumour treated in the Paediatric ENT Clinic in Warsaw. Between 1992 and 2007 two cases of Malherbe´s tumour were treated in the Paediatric ENT Clinic in Warsaw.
MATERIAL and methods
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