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  Table of Contents  
Year : 2014  |  Volume : 51  |  Issue : 4  |  Page : 569-570

Pilomatrix carcinoma of the eyelid

1 Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Sankara Nethralaya, Medical Research Foundation, Chennai. Tamil Nadu, India
2 Department of Pathology, Sankara Nethralaya, Medical Research Foundation, Chennai. Tamil Nadu, India

Date of Web Publication1-Feb-2016

Correspondence Address:
Dr. B Mukherjee
Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Sankara Nethralaya, Medical Research Foundation, Chennai. Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.175357

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How to cite this article:
Mukherjee B, Roy P, Adulkar N, Krishnakumar S, Biswas J. Pilomatrix carcinoma of the eyelid. Indian J Cancer 2014;51:569-70

How to cite this URL:
Mukherjee B, Roy P, Adulkar N, Krishnakumar S, Biswas J. Pilomatrix carcinoma of the eyelid. Indian J Cancer [serial online] 2014 [cited 2021 Oct 22];51:569-70. Available from: https://www.indianjcancer.com/text.asp?2014/51/4/569/175357


Pilomatrixoma, or calcifying epithelioma of Malherbe, as described by Malherbe and Cheantais in 1880, are benign hair follicle tumors that frequently present in the first two decades of life.[1] Lopansri and Mihm later reported 6 cases of pilomatrix carcinoma, which is a rare malignant neoplasm, arising from the hair matrix, believed to result from disruption of the normal hair anagen progression.[2],[3] Since then, very few cases of pilomatrix carcinoma of the eyelids have been reported in the literature.[4],[5]

We report a case of 65yearold female who presented with complaints of a swelling on the upper lid of 3 months duration. She had no complaints of pain or discharge from the mass. On examination, she was found to have a best corrected visual acuity of 20/30; J1 (Jaeger) both eyes with early cataracts. She had a superficial mass measuring 12 × 10 × 9 mm on the left upper lid with pus pointing, which was clinically diagnosed as a sebaceous cyst with secondary infection. [Figure 1] She was started on systemic and topical antibiotics, but even after a week, the patient reported no symptomatic improvement. She also complained of one episode of bleeding from the mass. Hence, the mass was removed and sent for histopathological examination.
Figure 1: Clinical photograph of the patient showing the left upper lid mass

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It showed a capsulated tumor composed of irregularly arranged proliferating lobules of squamous epithelium and amorphous keratin. Extensive areas of calcification were seen. In the squamous lobules, pleomorphism, nuclear atypia, and mitotic activity of the cells were seen along with areas of necrosis and hemorrhage. [Figure 2] This was diagnostic of a pilomatrix carcinoma.
Figure 2: Proliferations of large hyperchromatic basophilic cells with mitosis. In the center, there is transformation of the basophilic cells to eosinophilic shadow cells. (H and E, x20)

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Pilomatrix carcinoma is characterized by a locally aggressive tumor composed of hair matrix cells. It is a neoplasm of lowgrade malignancy. It should be distinguished from the benign pilomatricoma and its proliferating variant matricoma and basal cell carcinoma with matrical differentiation. It may be misdiagnosed as a benign lesion like chalazion, epidermoid cyst, calcifying hematoma, sebaceous cyst, and foreign body granuloma. The major discriminators of malignancy are presence of hyperchromatic, vesicular basaloid cells with numerous mitoses and infiltration into adjacent tissue or blood vessels.[2]

The benign pilomatricoma presents as a firm to hard, usually solitary but at times multiple, welldefined mass adherent to skin but not fixed to underlying tissue with the characteristic ‘tent sign’ caused by the stretching and reddishblue hue of overlying skin.[3] It may present at eyebrows, lids, and medial canthi. Clinical features are similar in both benign and malignant pilomatricoma, except for the course of disease and histopathological characteristics. Malignant transformation of a benign pilomatricoma has also been documented.[6]

Malignant pilomatrixoma carries a high risk of metastases to the bones, lungs, and lymph nodes.[7] These tumors may have an invasive growth pattern, with spread into the underlying bone. Local recurrence occurs in 60% of cases, and metastatic disease occurs in 10% of cases. Death has also been reported.[8]

Recurrences have been documented after simple excision of these lesions, and the preferred treatment is wide excision with histological confirmation of tumor clearance. Reports suggest excellent results with Mohs micrographic surgery.[9]

A lesion, which appears benign on clinical examination, may throw up histopathological surprises. Though rare, it is important for clinicians and pathologists to be aware of this condition because of its potential for distant metastases and the penchant to simulate innocuous lesions like epidermal cyst or chalazia.[10]

  References Top

Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol 1961;83:606618.  Back to cited text no. 1
Lopransi S, Mihm MC Jr. Pilomatrix carcinoma or calcifying epithelioma of Malherbe: A case report and review of literature. Cancer 1980;45:236873.  Back to cited text no. 2
Levy J, Ilsar M, Deckel Y, Maly A, Anteby I, Pe'er J. Eyelid Pilomatrixoma: A Description of 16 cases and a review of the Literature. Surv Ophthalmol 2008;53:52635.  Back to cited text no. 3
Wang X, Ma JM, Wang NL. Malignant pilomatricoma in the upper eyelid. BMJ Case Rep 2009;2009:bcr07.2008.0410.  Back to cited text no. 4
Cahill MT, Moriarty PM, Mooney DJ, Kennedy SM. Pilomatrix carcinoma of the eyelid. Am J Ophthalmol 1999;127:4634.  Back to cited text no. 5
Nishioka M, Tanemura A, Yamanaka T, Tani M, Miura H, Asakura M, et al. Pilomatrix carcinoma arising from pilomatricoma after 10year senescent period: Immunohistochemical analysis. J Dermatol 2010;37:7359.  Back to cited text no. 6
Niedermeyer HP, Peris K, Hofler H. Pilomatrix carcinoma with multiple visceral metastases. Cancer 1996;77:13114.  Back to cited text no. 7
Sau P, Lupton GP, Graham JH. Pilomatrix carcinoma. Cancer 1993;71:24918.  Back to cited text no. 8
Sable D, Snow SN. Pilomatrix carcinoma of the back treated by mohs micrographic surgery. Dermatol Surg 2004;30:11746.  Back to cited text no. 9
Julian CG, Bowers PW. A clinical review of 209 pilomatricomas. J Am Acad Dermatol 1998;39:1915.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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