View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2013) 17, 33–35 King Saud University Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com CASE REPORT Giant pilomatricoma of the arm: An unusual presentation (A case report) Amine Marzouki *,1, Badr Chbani, Anas Bennani, Kamal Lahrach, Faouzi Boutayeb Orthopedic Surgery and Traumatology A, University Hospital Hassan II FEZ, Morocco Received 5 November 2012; revised 3 December 2012; accepted 6 December 2012 Available online 18 January 2013 KEYWORDS Abstract Pilomatricoma is a benign tumor of the hair matrix. It is most often seen in children and Pilomatrixoma; young adults as a slowly growing nodule in the skin of the head and neck. The frequency of pre- Pilomatricoma; sentation of this lesion in the upper extremities is relatively rare. Pilomatricoma is a frequently mis- Epithelioma of Malherbe; diagnosed entity in clinical practice. They are usually asymptomatic, solitary, firm or hard, freely Giant; mobile, dermal or subcutaneous nodules. Most tumors are smaller than 3 cm in diameter. We report Arm; a 32-year-old woman with a giant pilomatricoma of the arm. Surgical excision was curative without Benign recurrence. In this paper, we discuss the clinical, histopathologic characteristics of pilomatricoma and review the literature regarding pilomatricomas in the upper extremity. ª 2013 King Saud University. Production and hosting by Elsevier B.V. All rights reserved. 1. Introduction diameter. Pilomatricoma prognosis is generally good. Cure without recurrence is the rule after total surgical resection. Pilomatricomas are of ectodermal origin and arise from the We present a case of an unusual presentation of giant piloma- outer root sheath cell of the hair follicle (Rook et al., 1998). tricoma in the arm of a 32 year old woman. It is most often seen in children and the most common ana- tomical location is the head and neck region (Pirouzmanesh 2. Case report et al., 2003). Involvement of the upper extremities is relatively uncommon (Pirouzmanesh et al., 2003; Daoudi et al., 2006; A 32-year-old woman with an unremarkable medical history, Ioannidis et al., 2010). Pilomatricomas usually are asymptom- presented with a nodular mass on the left arm. The patient atic, deeply seated, firm, nontender subcutaneous masses had noted the mass in her left arm, one year before initial pre- adherent to the skin but not fixed to the underlying tissue sentation. The lesion had begun growing over the last few (Rook et al., 1998). Most tumors are smaller than 3 cm in months. There was no history of pain, infection or trauma. Clinical examination revealed a giant solitary, firm, well-cir- * Corresponding author. Tel.: +212 6 61 26 10 22. cumscribed, and freely mobile mass located below the lateral E-mail address: [email protected] (A. Marzouki). face of the middle part of the arm (Fig. 1). The overlying skin 1 Home address: Appt 1, Imm 5, Re´sidence Rawd Alazhar, Avenue Beyrout, Route d’Imouzzer, Fe` s, Maroc, BP 30000. was normal in appearance. The tumor which measured Peer review under responsibility of King Saud University. 6 · 6 cm was fixed to the underlying tissue, and bimanual pal- pation failed to localize the origin of the tumor. A preoperative radiograph revealed a sharply outlined subcutaneous mass, which was calcified in its central portion. MRI reveals a rim- Production and hosting by Elsevier enhancing lesion with small areas of signal dropout which 2210-836X ª 2013 King Saud University. Production and hosting by Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.jssdds.2012.12.004 34 A. Marzouki et al. Figure 1 A nodular mass on the left arm: a giant solitary, firm, well-circumscribed, located below the lateral face of the middle Figure 3 The surgical specimen revealed a hard irregular mass part of the arm. which was calcified in its central portion. our patient have been reported in the literature (Pirouzmanesh et al., 2003; Daoudi et al., 2006; Ioannidis et al., 2010). Despite the frequency of presentation of this lesion in the upper extremities, discussion of this lesion is essentially limited to the literature of otolaryngology, pathology, and dermatology (Silva et al., 2003; Khammash et al., 2001). A search of the lit- erature revealed few well-documented cases of pilomatricomas in the arm (Daoudi et al., 2006; Ioannidis et al., 2010). Since the first description of pilomatricoma, there has been a gradual increase in understanding of the morphologic features and clinical presentation of this tumor (Kaddu et al., 1994). How- ever, difficulties still persist in making clinical and cytologic diagnosis (Kaddu et al., 1994). In its typical form, the piloma- tricoma results in a small subcutaneous nodule asmptomatique and sometimes painful (Rook et al., 1998; Pirouzmanesh et al., Figure 2 MRI reveals a rim-enhancing lesion with small areas of 2003; Kaddu et al., 1994). Calcification of the tumor was ob- signal dropout which may be consistent with calcifications. served in 80% of cases, sometimes achieving a true osteoma subcutaneous (Daoudi et al., 2006; Ioannidis et al., 2010). The usual size is less than 3 cm, however, few cases of giant pilomaricoma than 5 cm in diameter were observed as in our may be consistent with calcifications (Fig. 2).The tumor was case (Percin et al., 1996; Khammash et al., 2001). Pilomatrico- removed with a fusiform surgical excision. The surgical speci- ma presents most commonly in children and young adults, and men revealed a hard irregular mass, calcified in its central por- they are noted more commonly in females (Pirouzmanesh tion, measuring 6 · 6 · 4 cm, and was yellowish-white in color et al., 2003). It occurs mainly during the second decade of life (Fig. 3). Macroscopic examination revealed a nodular tumor and rarely beyond (Pirouzmanesh et al., 2003). Our patient is located in the deeper layers of the dermis adjacent to the sub- 32 years old which is an unusual age of onset. Diagnostic cutaneous tissue. The overlying skin was healthy. Microscopi- imaging is generally not obtained in the evaluation of cally it consisted of solid islands of shadow cells and extensive pilomatrixomas as they are usually superficial, small, and ossification. Even the usual nests of small basaloid cells were well-circumscribed. Conventional radiography in the piloma- absent. The diagnosis of giant pilomatricoma was established. trixomas may demonstrate foci of calcification (Daoudi Follow-up at 3 years revealed no evidence of recurrence. et al., 2006; Ioannidis et al., 2010; Haller et al., 1977). Ultra- sound demonstrates a well-defined mass with inner echogenic 3. Literature review foci and a peripheral hypoechoic rim or a completely echo- genic mass with strong posterior or acoustic shadowing in Pilomatrixoma, pilomatricoma or calcifying epithelioma of the subcutaneous layer (Hughes et al., 1999; Hwang et al., Malherbe is a benign neoplasm derived from hair follicle ma- 2005). Computed tomography (CT) demonstrates a sharply trix cells (Rook et al., 1998). These lesions are typically found demarcated, subcutaneous lesion of soft tissue density, with in the head and neck region (Pirouzmanesh et al., 2003). Only or without calcification (Agarwal et al., 2001). MRI may reveal a few isolated locations at exceptional members as the case of a rim-enhancing lesion with small areas of signal dropout Giant pilomatricoma of the arm: An unusual presentation (A case report) 35 which may be consistent with calcifications as is the case in our Haller, J.O., Kassner, E.G., Ostrowitz, A., Kottmeier, P.K., Rerts- patient (Agarwal et al., 2001). In all cases histology confirmed chuk, L.P., 1977. Pilomatrixoma (calcifying epithe´lioma of Mal- the diagnosis (Heenan et al., 1996). It is important to note that herbe): radiographic features. Radiology 123, 151–153. the pilomatrixoma was incorrectly diagnosed by fine needle Heenan, P.J., Elder, D.E., Sobin, L.H., 1996. WHO Histological Typing of Skin Tumours, 2th ed. Springer, Berlin. aspiration cytology (Wang et al., 2002). The microscopic Hughes, J., Lam, A., Rogers, M., 1999. Use of ultrasonographie in appearance of pilomatricomas is characterized by irregularly diagnosis of childhood pilomatricoma Pediatr. Dermatology 16 (5), shaped well-circumscribed masses of epithelial cells. Basophilic 341–344. cells and shadow cells may be observed in the cellular stroma Hwang, J.Y., Lee, S.W., Lee, S.M., 2005. The common ultrasono- (Rook et al., 1998; Pirouzmanesh et al., 2003; Heenan et al., graphic features of pilomatricoma. J. Ultrasound Med. 24 (10), 1996). The differential diagnosis of these lesions should include 1397–1402. sebaceous, dermoid and epidermoid cysts, metaplastic bone Ioannidis, O., Stavrakis, T., Cheva, A., Papadimitriou, N., Kotronis, formation, foreign body reaction, parotid gland tumor, hema- A., Kakoutis, E., Makrantonakis, N., 2010. Pilomatricoma of the toma, osteochondroma, trichoepithelioma and basal cell epi- arm with extensive ossification. Adv. Med. Sci. 55 (2), 340–342. thelioma (Pirouzmanesh et al., 2003; Kaddu et al., 1996). Kaddu, S., Soyer, H.P., Cerroni, L., Salmtofer, W., Ho¨dl, S., 1994. Clinical and histopathologic spectrum of pilomatricomas in adults. Recommended management is surgical excision (Pirouzm- Int. J. Dermatol. 33, 705–708. anesh et al., 2003). Recurrence is uncommon after adequate Kaddu, S., Soyer, H.P., Ho¨dl, S., Kerl, H., 1996. Morphological stages excision (Pirouzmanesh et al., 2003; Khammash et al., 2001). of pilomatricoma. Am. J. Dermatopathol. 18 (4), 333–338. All patients need dermatological evaluation with close long Khammash, M.R., Todd, D.J., Abakhail, A., 2001. Concurrent term follow-up. The clinical course is generally benign pilomatrix carcinoma and giant pilomatrixoma. J. Dermatol. 42, although, malignant transformations have been reported (Pir- 120–123.