|Year : 2008 | Volume
| Issue : 6 | Page : 509-511
A giant upper eyelid ossifying pilomatrixoma
Syed AR Rizvi1, Mohammad Naim2, Mohammad S Alam1
1 Institute of Ophthalmology, Jawaharlal Nehru Medical College, AMU, Aligarh, UP, India
2 Department of Pathology, Jawaharlal Nehru Medical College, AMU, Aligarh, UP, India
|Date of Submission||01-Jan-2008|
|Date of Acceptance||27-Feb-2008|
|Date of Web Publication||14-Oct-2008|
Syed AR Rizvi
52, Alig Apartments, Shamshad Market, Aligarh-202 002, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Pilomatrixoma of the eyelid is extremely rare in middle age and rarely develops into a large tumor. A 45-year-old female developed a painless, progressive swelling (3.0 cm x 2.0 cm) of the left upper eyelid over a period of two years. Overlying skin was normal in color and texture. A differential diagnosis of dermoid, epidermoid cyst, chalazion and basal cell carcinoma was made. An excisional biopsy was performed. A diagnosis of pilomatrixoma was made on histopathological features (dystrophic calcification of matrix with keratin and foreign body granulomatous reaction, basaloid cells and shadow cells/ghost cells). It also comprises ossification apart from the usual calcification. This is a report of an unusually large ossifying pilomatrixoma in left upper eyelid of a middle-aged woman. The patient should be followed up at regular intervals to rule out any recurrence or malignant transformation.
Keywords: Eyelid, giant, ossification, pilomatrixoma
|How to cite this article:|
Rizvi SA, Naim M, Alam MS. A giant upper eyelid ossifying pilomatrixoma. Indian J Ophthalmol 2008;56:509-11
Pilomatrixoma is a benign dermal and /or subcutaneous tumor most commonly affecting children and adolescents with a slight female preponderance.  A tumor of the head and neck region, pilomatrixoma is extremely rare in middle age and rarely attains a large size, especially involving the eyelid. , Clinically, it is almost always deceptive and often diagnosed on histopathology after excision.  Complications are extremely rare in pilomatrixoma . Pilomatrixoma occurs more frequently in patients with myotonic dystrophy. Active mutations of betacatenin gene (CTNBB1) are found to contribute to the tumorigenesis of pilomatrixoma.  The distribution of pilomatrixoma depends on the density of pilosebaceous glands in a given area. Periorbital pilomatrixoma usually involves the upper eyelid and eyebrow. 
We report a case of an unusually large pilomatrixoma in the left upper eyelid of a middle-aged woman which showed ossification along with calcification.
| Case Report|| |
A 45-year-old female presented to us with history of two years of gradually increasing, painless swelling of the left upper eyelid. On examination, there was a 3.0 cm x 2.0 cm, single, large, stony hard, non-tender, mobile swelling with well-defined margins involving the left upper eyelid [Figure 1]. The overlying skin was slightly adherent to the nodule but was normal in color and texture. Severe mechanical ptosis caused by the swelling was noted in left upper lid but no other ocular abnormality was noted. Systemic examination did not reveal any lymphadenopathy, organomegaly or nodular lesion elsewhere. Differential diagnoses of dermoid cyst, epidermoid cyst, chalazion or basal cell carcinoma were considered.
Surgical excision of the lesion was done under infiltrative anesthesia. A skin incision was undertaken in the upper eyelid crease. The lesion was meticulously dissected from the surrounding tissues and its complete removal was accomplished.
On gross examination, a single, globular yellow-orange encapsulated mass with smooth outer surface measuring 2.5 cm x 1.5 cm x 0.5 cm was seen [Figure 2]. Histopathology showed confluent foci of dystrophic calcification of the matrix with keratin and foreign body granulomatous reaction, basaloid cells and shadow cells/ghost cells. Amid the pilomatrical matrix, a well-defined area of bone formation with osteocytes was also seen [Figure 3a] and [Figure 3b]. Careful serial sections did not show malignant change in any field of the lesion. Based on these features, a histopathological diagnosis of a pilomatrixoma was made. The patient did not show any sign of recurrence or malignant transformation after nine months of follow-up.
| Discussion|| |
Pilomatrixoma is a rare benign skin appendegeal epithelioma with differentiation towards hair root cells. It usually presents with a solitary nodule that slowly grows over several months or years. Lesions are usually the color of the normal skin, but reddish-blue lesions have been observed probably resulting from hemorrhage. ,
The size of the pilomatrixoma varies from 0.5-3.0 cm but giant periorbital lesions up to 3.0 cm have been rarely reported. In their review of 11 cases of eyelid and eyebrow pilomatrixoma Yap et al. , found tumor diameter of 1.0 cm or less.  Another study reported 17 patients of eyelid pilomatrixoma but none had attained the size of 3.0 cm or more.  However, the most recent one reported a rapidly growing and bleeding upper eyelid pilomatrixoma which measured 3.0 cm x 1.0 cm.  The present case reported by us differs from the above mentioned case in size (3.0 cm x 2.0 cm), onset (slow), the nature of overlying skin (normal) and the presence of ossification.
Most of the eyelid/eyebrow pilomatrixomas reported so far have been seen in young adults and rarely in older patients. , A giant tumor in general and in middle age in particular as in the present case has rarely been reported.
Histopathologically, pilomatrixoma is categorized into various chronological stages.  Early pilomatrixoma lesions are usually cystic and consist of uniform basaloid cells lining the cystic cavity and shadow cells/ghost cells admixed with keratin.  The shadow cells, also termed ghost cells, are pathognomonic of pilomatrixoma which evolve from basaloid cells, represent dead cells that retain their cellular shape and show a central unstained area that corresponds to the lost nucleus. 
The older lesions become solid and are characterized by shadow/ghost cells, keratin debris, secondary multinucleated giant cells, dystrophic calcification and sometimes by bone formation. , Ossification as well as all the above mentioned features including chronicity were seen in the present case too.
Tumor necrosis, calcification and ossification also take place in rare cases of pilomatrix carcinoma.  Though no recurrence or malignant change is seen in the present case, there is need for vigilance and such patients should be followed up at regular intervals.
| References|| |
Alsad KO, Obaidat NA, Ghazarian D. Skin adnexial neoplasms- Part 1: An approach to tumours of the pilosebaceous unit. J Clin Pathol 2007;60:129-44.
Mencia GE, Gutierrez DE, Garcia SE, Ricky JR. Eyelid pilomatricomas in young adults: A report of 8 cases. Cutis 2002;69:23-6.
Izquierdo-Rodrνguez C, Mencνa-Gutiιrrez E, Gutiιrrez-Dνaz E, Suαrez-Gauthier A. An unusual presentation of pilomatrixoma in the eyelid. Arch Soc Esp Ofthalmol 2006;81:483-6.
Macleod AJ, Scobie WG. Pilomatrixoma as a diagnostic problem: the Edinburgh experience. J Roy Coll Surg Edinb 1991;36:261-3.
Huerva V, Sanchez MC, Asenjo J. Large, rapidly growing pilomatrixoma of the upper eyelid. Ophthal Plast Reconstr Surg 2006;22:401-3.
Yap EY, Hohberger GG, Bartley GB. Pilomatrixoma of the eyelids and eyebrows in children and adolescents. Ophthal Plast Reconstr Surg 1999;15:185-9.
Seitz B, Holbach LM, Naumann GO. Pilomatrixoma of the eyelids - clinical differential diagnosis and follow up: Report of 17 patients. Ophthalmologe 1993;90:746-9.
Kaddu S, Sover HP, Hodl S, Kerl H. Morphological stages of pilomatricoma. Am J Dermatopathol 1996;18:333-8.
Lan MY, Lan MC, Ho CY, Li WY, Lin CZ. Pilomatricoma of the Head and Neck. Arch Otolaryngol Head Neck Surg 2003;129:1327-30.
[Figure 1], [Figure 2], [Figure 3a], [Figure 3b]