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 Table of Contents  
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 67-69

Pilomatrixoma of the nasal vestibule: A report of a case at an unusual site

1 Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Maharashtra, India
2 Department of Pathology and ENT, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Maharashtra, India

Date of Submission01-May-2021
Date of Acceptance29-May-2021
Date of Web Publication26-Dec-2021

Correspondence Address:
Department of Pathology, Mahatma Gandhi, Institute of Medical Sciences, Sevagram - 442 102, Maharashtra.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/dypj.dypj_24_21

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Orbital foreign bodies are still serious diagnostic problem even after the development of diagnostic imaging techniques. We present an interesting case report of a 42-year-old man who presented with a history of sudden loss of vision after sustaining injury to the left eye due to metallic piece while working. Grayscale ultrasound and computed tomography findings are discussed.

Keywords: Computed tomography orbit, ocular foreign body, ocular trauma, ultrasound

How to cite this article:
Singh S, Anshu &, Nagpure PS, Gangane NM. Pilomatrixoma of the nasal vestibule: A report of a case at an unusual site. D Y Patil J Health Sci 2021;9:67-9

How to cite this URL:
Singh S, Anshu &, Nagpure PS, Gangane NM. Pilomatrixoma of the nasal vestibule: A report of a case at an unusual site. D Y Patil J Health Sci [serial online] 2021 [cited 2022 Aug 8];9:67-9. Available from: http://www.dypatiljhs.com/text.asp?2021/9/2/67/333768

  Introduction Top

Pilomatrixomas are rare, slow-growing, benign appendageal tumors which originate from primitive germ cells which are on their way to becoming matrix cells of hair follicles. They account for 0.001%–1% of skin lumps.[1],[2],[3] This lesion was first described in 1880 by Malherbe and Chenantais, and since 1977, the terms “pilomatrixoma” or “pilomatricoma” have been universally used to name the lesion.[4] Although pilomatrixomas are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. Around 60% of these tumors occur in the first two decades of life.[2]

Typically, pilomatrixomas present as asymptomatic, firm, slow-growing, mobile, superficial skin nodules of 0.5–3 cm in diameter.[5] The most frequent anatomical location of this tumor is the head-and-neck region, followed by the upper extremities, trunk, and the lower extremities in decreasing order of frequency.[6] Pilomatrixomas of the nasal cavity and sinuses have hardly been reported in English literature, except for one case originating from the maxillary sinus. Only one case of a pilomatrixoma in the nasal vestibule could be found in a Spanish journal.

We report a case of pilomatrixoma in a 34-year-old man that occurred at a very unusual site, i.e., in the nasal vestibule.

  Case Report Top

A 34-year-old male patient presented to the otorhinolaryngology outpatient department with complaints of a mass in the right nasal cavity. The mass had first been noticed 6 months before and was slowly growing in size. The patient sought medical help only when the mass started causing difficulty in breathing. It was not associated with pain or nasal discharge. On anterior rhinoscopy, the nasal mass was found to be a solitary, pale pinkish, smooth, globular swelling of size 2.5 cm × 2.5 cm × 2.5 cm. It was attached to the lateral surface of the vestibule and free from the septum. On palpation, it was non-tender, firm, and did not bleed on touch. The nasal septum was deviated and S-shaped. No sinus tenderness was found. A clinical diagnosis of nasal polyp was made.

Contrast-enhanced computed tomography (CT) scan of the paranasal sinuses was done. The mass was reported as a non-enhancing soft-tissue lesion in the vestibule arising from the lateral wall of the right nostril [Figure 1]. The radiologists opined that it was likely to be of benign etiology. The left nostril was normal. Bilateral nasolacrimal ducts were opacified. The bilateral maxillary, frontal, ethmoid, and sphenoid sinuses appeared normal.
Figure 1: Contrast-enhanced computed tomography of the paranasal sinuses showing non-enhancing soft-tissue lesion in the right nasal vestibule

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A decision to operate the patient was made. Under the influence of local anesthesia, a lateral rhinotomy incision was given on the right side of the right nostril. The lateral wall of the nose was freed, and the mass was exposed. An elliptical incision was taken at the base of mass, and then, the mass was excised.

The sample was sent to the histopathology section of the Department of Pathology. On gross examination, the mass was solitary, globular, and measured 2.5 cm × 1.2 cm × 1 cm. It was yellowish white in color. The surface of the mass showed attached skin and hair.

On light microscopy, the sections showed a well-circumscribed nodule composed of islands of cells with basaloid cells and shadow cells [Figure 2]. Basaloid cells with very little cytoplasm and hyperchromatic nuclei were present at the periphery of the cell islands. These basaloid cells abruptly transitioned toward shadow cells toward the center of the lesion. These shadow cells had distinct cell borders, more cytoplasm, and only an outline of the nucleus [Figure 3]. Some areas showed foreign body granulomatous reaction [Figure 4]. Calcification was not seen. A diagnosis of pilomatrixoma was made on histopathology.
Figure 2: Section showing a well-circumscribed nodule composed of islands of cells with basaloid cells in the periphery and shadow cells toward the center (Hematoxylin and eosin, ×40)

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Figure 3: Section showing abrupt transition of basaloid cells to shadow cells. (Hematoxylin and eosin, ×100)

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Figure 4: Section showing the presence of a granulomatous reaction with foreign body giant cells (Hematoxylin and eosin, ×100)

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At the time of writing this case, the patient had recovered well without any complications and was discharged event free.

  Discussion Top

Pilomatrixomas are uncommon tumors, which arise due to uncontrolled proliferation of hair matrix cells. They are said to represent 0.001%–0.003% of all dermatopathologic samples.[2] The head and neck is the most common site of these tumors. Pilomatrixomas have hardly been seen in the nasal cavity and paranasal sinuses. [Table 1] summarizes the three cases which have been previously reported.[7],[8],[9] Of them, two were found in children below 1 year. One of them appeared in an adult and was found to arise from the maxillary sinus.
Table 1: Summary of previously published cases of pilomatrixoma in the nasal cavity and paranasal sinuses

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Pilomatrixomas are also known as calcified epithelioma of Malherbe as their hallmark is internal calcification. Pilomatrixomas are often misdiagnosed on preoperative evaluation. In contrast to most other nodules, diagnostic imaging is of uncertain value for pilomatrixoma. CT identified internal calcification in 81% of cases[10] whereas ultrasonography visualized the internal echogenic foci in 93%.[11] Histopathology is the gold standard in diagnosing this lesion. In one case series, histopathological examination identified either calcification or ossification in 81% of the tumors.[12],[13] We, however, did not find any areas of calcification in this lesion, although there were foreign body giant cells. Having said this, diagnosis of pilomatrixoma is not always straightforward and may even be challenging.

In a recent series of 346 pilomatrixomas, the preoperative diagnosis was accurate and consistent with the pathological diagnosis of pilomatrixoma in only 28.9% of cases.[14] Kumaran etal. reported a correct preoperative clinical diagnosis in 46% following retrospective review of 78 excised pilomatrixomas.[15]

The exact etiology of pilomatrixoma is still unknown. Some reports implicate mutation of the β-catenin gene and viral etiology. A recent study suggests that trisomy 18 is a consistent feature in pilomatrixoma, suggesting that genes carried on this chromosome such as that for the antiapoptotic oncoprotein Bcl-2 may have a role in the growth and differentiation of this benign self-limited tumor.[16]

Spontaneous regression of this lesion is never observed, and malignant degeneration is extremely rare.[14] Complete surgical excision is the treatment of choice. Recurrence after surgery is rare, with an incidence of 0%–3%. Malignant transformation to a pilomatrix carcinoma should be suspected in cases with repeated local recurrences.[17]

  Conclusion Top

The nasal vestibule is a rare site for the occurrence of a pilomatrixoma, more so in adults. However, it would be worthwhile to include this lesion in the differential diagnosis of atypical nasal masses. Histopathology will be essential for arriving at a correct diagnosis.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Forbis R Jr., Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol 1961;83:606-18.  Back to cited text no. 1
Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma). A statistical study. Arch Dermatol 1973;108:532-4.  Back to cited text no. 2
Kwon D, Grekov K, Krishnan M, Dyleski R. Characteristics of pilomatrixoma in children: A review of 137 patients. Int J Pediatr Otorhinolaryngol 2014;78:1337-41.  Back to cited text no. 3
Aherne NJ, Fitzpatrick DA, Gibbons D, Armstrong JG. Pilomatrix carcinoma presenting as an extra axial mass: Clinicopathological features. Diagn Pathol 2008;3:47.  Back to cited text no. 4
Guinot-Moya R, Valmaseda-Castellon E, Berini-Aytes L, Gay-Escoda C. Pilomatrixoma. Review of 205 cases. Med Oral Patol Oral Cir Bucal 2011;16:e552-5.  Back to cited text no. 5
Pant I, Joshi SC, Kaur G, Kumar G. Pilomatricoma as a diagnostic pitfall in clinical practice: Report of two cases and review of literature. Indian J Dermatol 2010;55:390-2.  Back to cited text no. 6
[PUBMED]  [Full text]  
Nigro LC, Fuller CE, Rhodes JL. Pilomatrixoma presenting as a rapidly expanding mass of the infant nasion. Eplasty 2015;15:e54.  Back to cited text no. 7
Inzunza PF, Hormazábal RW, Valenzuela GA, Schalper PJ, Pérez GT. Pilomatrixoma nasal: Nasal pilomatricoma. J Otolaryngol Head Neck Surg 2009;69:153-6.  Back to cited text no. 8
Sruthi TR, Behera SK, Swain S, Patsani M. Pilomatrixoma arising from maxillary sinus - First of its kind. South Asian J Cancer 2019;8:197.  Back to cited text no. 9
[PUBMED]  [Full text]  
Lim HW, Im SA, Lim GY, Park HJ, Lee H, Sung MS, et al. Pilomatricomas in children: Imaging characteristics with pathologic correlation. Pediatr Radiol 2007;37:549-55.  Back to cited text no. 10
Choo HJ, Lee SJ, Lee YH, Lee JH, Oh M, Kim MH, et al. Pilomatricomas: The diagnostic value of ultrasound. Skeletal Radiol 2010;39:243-50.  Back to cited text no. 11
Jones CD, Ho W, Robertson BF, Gunn E, Morley S. Pilomatrixoma: A comprehensive review of the literature. Am J Dermatopathol 2018;40:631-41.  Back to cited text no. 12
Yoshimura Y, Obara S, Mikami T, Matsuda S. Calcifying epithelioma (pilomatrixoma) of the head and neck: Analysis of 37 cases. Br J Oral Maxillofac Surg 1997;35:429-32.  Back to cited text no. 13
Pirouzmanesh A, Reinisch JF, Gonzalez-Gomez I, Smith EM, Meara JG. Pilomatrixoma: A review of 346 cases. Plast Reconstr Surg 2003;112:1784-9.  Back to cited text no. 14
Kumaran N, Azmy A, Carachi R, Raine PA, Macfarlane JH, Howatson AG. Pilomatrixoma – Accuracy of clinical diagnosis. J Pediatr Surg 2006;41:1755-8.  Back to cited text no. 15
Agoston AT, Liang CW, Richkind KE, Fletcher JA, Vargas SO. Trisomy 18 is a consistent cytogenetic feature in pilomatricoma. Mod Pathol 2010;23:1147-50.  Back to cited text no. 16
Goufman DB, Murrell GL, Watkins DV. Pathology forum. Quiz case 2. Pilomatricoma (calcifying epithelioma of Malherbe). Arch Otolaryngol Head Neck Surg 2001;127:218-20.  Back to cited text no. 17


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]


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