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Pilomatricoma of the pinna.

Abstract

A 4-year-old boypresented with a painless swelling over the right pinna of 4 months' duration. Histopathologic examination of the excised mass revealed features of pilomatricoma--a rare, benign skin neoplasm arising from hair follicle matrix cells. The unusual location of the lesion over the pinna, despite its frequent occurrence in the head and neck, prompted this article. We discuss the etiology, clinical presentation, and management of pilomatricoma of the auricular region

Introduction

Pilomatricomas are superficial, rare, benign skin tumors typically located in the head and neck region. Excluding lymph nodes and epidermoid cysts, they are the next most commonly excised superficial masses in children. (1) However, the pinna is an uncommon location for this condition, and hence it is frequently misdiagnosed by otolaryngologists. Ear lesions accounted for only 4.5% of the cases in a review of 179 cases of head and neck pilomatricoma; this review did not describe pinna lesions and their management in detail, however. (1)

We report a case of pilomatricoma of the pinna occurring in a 4-year-old child. This article meets all the requirements of our Institutional Review Board and Ethical Committee.

Case report

A 4-year-old boy presented with a painless, slowly growing swelling over the right pinna of 4 months' duration. Examination revealed a 1 x 2-cm, firm to hard, nontender swelling on the outer rim of the helix, not fixed to the cartilage but attached to the skin, with a smooth surface (figure 1). Findings from the rest of the ENT examination were normal.

A provisional diagnosis of a calcified dermoid was made. However, fine-needle aspiration cytology (FNAC) was inconclusive. Under general anesthesia, the swelling was excised along with the overlying skin, as it was attached to the mass. Postoperative histopathology showed irregularly shaped masses of ghost, or shadow, cells as well as scattered basaloid cells and numerous giant cells with areas of calcification (figure 2). The postoperative period was uneventful, and no recurrence of the lesion was seen after 6 months of follow-up (figure 3).

Discussion

Pilomatricoma, previously known as pilomatrixoma, (1) is a rare, benign neoplasm of hair follicle origin. It was initially thought to arise from sebaceous glands and was called calcifying epithelioma of Malherbe by Malherbe and Chenantais in 1880. (2) In 1942, Turhan and Krainer proposed that this neoplasm is derived from the hair cortex cells. (3) It was Forbis and Helwig who renamed this neoplasm as pilomatrixoma in 1961 and described its origin as the hair follicle matrix. (4) The name was further changed to pilomatricoma in 1977 by Arnold. (5)

According to Moehlenbeck, the incidence of pilomatricoma is 0.12% of all the cutaneous neoplasms. (6) The most common location for these tumors is the head and neck region, accounting for 40 to 77% of all cases. (1) The next most common site is the upper extremities, followed by the trunk and the lower extremities? In the head and neck, the neck is most commonly involved, followed by the cheek, scalp, and preauricular and periorbital areas. (1)

Pilomatricomas are not known to occur on nonhair-bearing areas such as the palms, soles, and genital region. (1,7) Although lesions are not uncommon in the preauricular area, their occurrence over the pinna is not well documented in the literature. In one of the largest reviews of head and neck pilomatricoma, out of 179 cases, only 8 cases (4.5%) occurred over the pinna. (1)

Because of the lack of literature regarding the involvement of the pinna, the presentation and management of pilomatricomas at this site are not well understood.

[FIGURE 1 OMITTED]

Pilomatricomas are more common in females, and approximately 60% occur in patients younger than 20 years, with a smaller peak seen in the elderly. (7) Multiple lesions are rare, seen in 2 to 10% of patients, and have been associated with Gardner syndrome, Steinert disease (also known as myotonic dystrophy), and sarcoidosis. (8)

Pilomatricoma typically presents as a superficial, solitary, slowly growing, rock-hard, painless nodular mass. Overlying skin may exhibit a bluish discoloration or ulceration. (1) A "tent sign," elicited by stretching the skin over the lesion to feel the irregular surface, has been described. (8)

Pilomatricomas are not usually considered in the differential diagnosis of head and neck masses because otolaryngologists are not well aware of this entity. In one of the series, a correct preoperative diagnosis was made in only 1.1% of the cases. (1,7) In the preauricular region, tumors of the parotid must be ruled out.

Imaging studies are of limited help. (1,7) and are often Unnecessary (8) in identifying superficial, benign skin lesions. However, they may be of some help in the preauricular region to differentiate these lesions from parotid lesions. The pilomatricoma lesion appears as a sharply demarcated, calcified, subcutaneous nodule on computed tomography and as high-intensity signal bands in T2-weighted magnetic resonance imaging. (7)

It is difficult to make a diagnosis by FNAC. (7) FNAC may reveal the presence of ghost cells, basaloid cells, and calcium deposits in the mass, but without the presence of ghost cells in the aspirate, the diagnosis may be misleading. (8)

[FIGURE 2 OMITTED]

Histopathologic examination shows sharply demarcated dermal nodules surrounded by a capsule of compressed fibrous tissue located in the lower dermis and extending into the subcutaneous fat. (1) Nucleated basaloid cells are located in the periphery, and enucleated ghost cells are present in the center. The basaloid cells have small, uniform nuclei, scant cytoplasm, and indistinct cell borders. The ghost cells evolve from the basaloid cells and represent dead cells that retain their cellular shape. Calcification is mostly seen in the ghost cell regions.

The treatment of choice for pilomatricoma is complete surgical excision. Occasionally, overlying skin needs to be excised because of tumor adherence to dermis. (9) Recurrences after surgery are rare, with an incidence of 0 to 3%. (1) Malignant transformation should be suspected in patients with repeated local recurrences.

In conclusion, otolaryngologists should consider pilomatricoma in the differential diagnosis for a superficial, firm to hard mass in the head and neck region so that an accurate preoperative diagnosis can be established. Complete surgical excision is the treatment of choice. Recurrence is rare.

References

(1.) Lan MY, Lan MC, Ho CY, et al. Pilomatricoma of the head and neck. A retrospective review of 179 cases. Arch Otolaryngol Head Neck Surg 2003;129(12):1327-30.

(2.) Malherbe A, Chenantais J. Note sur l'epithelioma calcifie des glandes sebacees. Progres Medical 1880;8:826-37.

(3.) Turhan B, Krainer L. Bemerkungen uber die sogenannten verkalkenden Epitheliome der Haut und ihre Genese. Dermatologica 1942;85: 73-90.

(4.) Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermato| 1961;83:606-8.

(5.) Arnold HL Jr. Pilomatricoma [Letter]. ArchDermato11977;113(9):

(6.) Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma). A statistical study. Arch Dermatol 1973;108(4):532-4.

(7.) Reddy SS, Gadre SA, Adegboyega P, Gadre AK. Multiple pilomatrixomas: Case report and literature review. Ear Nose Throat J 2008; 87(4):230-3.

(8.) Danielson-Cohen A, Lin SJ, Hughes CA, et al. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg 2001; 127(12):1481-3.

(9.) Yencha MW. Head and neck pilomatricoma in the pediatric age group: A retrospective study and literature review. Int J Pediatr Otorhinolaryngol 2001;57(2):123-8.

Aroor Rajeshwary, MS (ENT); Gangadhara Somayaji, MS (ENT); Sai Manohar, MS (ENT)

From the Department of ENT, KS Hegde Medical Academy (Dr. Rajeshwary), and the Department of ENT, Yenepoya Medical College, Deralakatte, Mangalore, India (Dr. Somayaji and Dr. Manohar). The case described in this article occurred at Yepenoya Medical College.

Corresponding author: Dr. Gangadhara Somayaji, Department of ENT, Yenepoya Medical College Hospital, Deralakatte, Nithyananda nagara, Mangalore, Karnataka India 575018.Email: ksgsomayaji@gmail.com.
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Article Details
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Title Annotation:ORIGINAL ARTICLE
Author:Rajeshwary, Aroor; Somayaji, Gangadhara; Manohar, Sai
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Jan 1, 2013
Words:1278
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