© 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
Summary
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.
INTRODUCTION
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).
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 methods
Considering the rarity and diagnostic difficulties the authors present two cases of calcifying epithelioma of Malherbe treated in the Children´s Otolaryngology Clinic in Warsaw between 1992 and 2007.
CASE I. A three-year-old girl was admitted to the Clinic with a single, painless tumour in the right preauricular region. On physical examination and in laboratory tests no abnormalities were found.
CASE II. A three-and-a-half-year-old girl was admitted to the Clinic with a single, painless tumour in the region of the right retro-auricle.
In both cases the lesion was removed completely, the material being sent for histopathological examination in Department of Paediatric Pathologic Anatomy Medical University of Warsaw. The results showed calcifying epithelioma of Malherbe. In both girls no complications or recurrences were observed.
RESULTS
Both cases were histopathology diagnosed as Malherbe´s tumour. Surgical treatment in both cases gave a good therapeutic effect.
CONCLUSIONS
1. In cases of the calcifying epithelioma known as Malherbe´s tumour, the treatment is surgical.
2. Because of the small size the tumour should be wholly removed.
3. Recognition of the tumour must be confirmed by a histopathological examination.
4. In the literature there are no reports about pharmacological treatment of Malherbe´s tumour.
5. In Poland occurrence of Malherbe´s tumour is rare, whereas in the Mediterranean countries the tumour occurs more often.
Piśmiennictwo
1. Bień S, Kamiński B, Okła S.: Ocena kliniczna nabłoniaka wapniejącego Malherba Otolaryngologia. 2005; 4 (1), 43-46. 2. Borsuk J.: Nabłoniak Malherbe okolicy kąta żuchwy. Otolaryngologia. 1961; XV,373-376. 3. Boyd AS, Martin III RB.: Pathologic Quiz Case 1 Arch Otolaryngol Head & Neck Surg/Vol 118, Feb 1992; 212-214. 4. Chmielik M, Stelęgowska-Piórkowska D.: Nabłoniak wapniejący Malherbe´a okolicy przedusznej prawej u 3-letniego dziecka. Otolaryng. Pol. 1993; XLVII, 6, 550-552. 5. Cigliano B, Baltogiannis N, De Marco M, et al.: Papilomatricoma in childhood: a retrospective study from three European pediatric centres. Eur J Pediatr (2005); 164: 673-677. 6. Goufman DB.: Quiz Case 2 Arch Otolaryngol Head & Neck Surg/Vol 127, Feb 2001; 218-220. 7. Kobos J.: Epithelioma Calcificans Malherbre w Świetle Danych z Piśmiennictwa i Badań Własnych. Pat. Pol, 1980; XXXI, 3, 429-437. 8. Lopansri S, Mihm MC.: Pilomatrix Carcinoma or Calcifying Epitheliocarcinoma of Malherbe. Cancer 1980; 45: 2368-2373. 9. Moehlenbeck FW.: Pilomatrixoma (Calcifying Epithelioma) Arch Dermatol/Vol 108, Oct 1973; 532-534. 10. Pietniczka M, Dubicki J.: Nabłoniak wapniejący Malherbe´a okolicy przdusznej prawej u 10-letniego dziecka. Otolaryng. Pol 1996; L, 2, 200-202. 11. Unger P, Watson C, Phelps RG.: Fine Needle Aspiration Cytology of Pilomatrixoma (Calcifying Epithelioma of Malherbe). Acta Cytologica vol 34, 6, Nov-Dec 1990; 847-850.
