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| CASE REPORT |
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| Year : 2023 | Volume
: 16
| Issue : 1 | Page : 136-138 |
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A rapidly growing pedunculated pilomatrixoma of the eyelid
Walaa Alturkistany1, Mohammed Alrajeh2, Osama Alsheikh3
1 Department of Ophthalmology, King Abdulaziz University, Jeddah; Department of Oculoplastic, King Khaled Eye Hospital, Riyadh, Saudi Arabia
2 Department of Ophthalmology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
3 Department of Oculoplastic, King Khaled Eye Hospital, Riyadh, Saudi Arabia
| Date of Submission | 24-Mar-2022 |
| Date of Decision | 11-May-2022 |
| Date of Acceptance | 02-Dec-2022 |
| Date of Web Publication | 21-Feb-2023 |
Correspondence Address:
Osama Alsheikh
Oculoplastic Department, King Khalid Eye Specialist Hospital, Riyadh
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ojo.ojo_79_22
 Abstract | | |
A pilomatrixoma is a benign tumor originating from a hair follicle that most frequently occurs in the head-and-neck region. It usually presents as a subcutaneous, slow-growing, nodular, painless firm mass. There are few reported cases on eyelid pilomatrixoma. We reporting an unusual presentation of a rapidly growing pedunculated eyelid pilomatrixoma in a 29-year-old female patient. Surgical excision was performed, and histological examination showed a cavity containing proliferating cords of basaloid cells differentiated into eosinophilic keratinized shadow cells confirming the pilomatrixoma diagnosis. Only a few cases with pedunculated eyelid masses have been reported in the literature; pedunculated lesions can be misdiagnosed as vascular tumors or malignancies. Therefore, pilomatrixoma should be considered in the differential diagnosis of such a presentation. A complete excisional biopsy of the mass is diagnostic and therapeutic.
Keywords: Eyelid, pedunculated lesion, pilomatrixoma
How to cite this article:
Alturkistany W, Alrajeh M, Alsheikh O. A rapidly growing pedunculated pilomatrixoma of the eyelid. Oman J Ophthalmol 2023;16:136-8 |
| Introduction | |  |
Pilomatrixoma of the eyelid is a benign neoplasm originating from the matrix of the hair root[1]. In 1880 Malherbe and Chenantias described this lesion as a as a “calcifying epithelioma” and thought it originated from the sebaceous glands[1]. It was in 1961, when Forbis and Helwig discovered that it is originated from the matrix of the hair root and named it pilomatrixoma.[1]
The classical presentation of pilomatrixoma is a subcutaneous nodular mass. Usually, it is slow in progression, firm, and nontender on palpation.[1] Pilomatrixoma is often misdiagnosed clinically, and the correct diagnosis can only be made after excision and histological examination.[2]
We report a case of an uncommon presentation of a rapidly growing pedunculated pilomatrixoma of the eyelid margin to highlight that this entity should also be considered in the differential diagnosis of lesions involving the eyelids.
| Case Report | | |
A 29-year-old female patient, medically free, came to the emergency department complaining of a mass on the right upper eyelid with a 2-month duration and progressive enlargement.
On examination, her best-corrected visual acuity was 20/30 in the right eye and 20/25 in the left eye. The ocular examination was unremarkable, except for a pedunculated mass on the right upper eyelid involving the central pretarsal area, which was bluish in color and contained chalky and darkish material. It was firm in consistency, nontender on palpation, and measured 4 mm × 5 mm [Figure 1].
Surgical excision of the right upper eyelid mass was performed, followed by simple direct closure. The lesion was sent for histopathological study.
Histopathological examination revealed a cavitary lesion surrounded by a thick fibrous capsule. The cavity contained proliferating cords of basaloid cells differentiated into eosinophilic keratinized shadow cells. Focal areas of eosinophilic epithelioid cells lining the inner wall of the cavity were also noted. The lesion was consistent with pilomatrixoma; there was no evidence of malignancy [Figure 2]. | Figure 2: Histological findings showing basaloid cells differentiated from shadow cells
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| Discussion | | |
Pilomatrixoma is known to present as a subcutaneous nodule[1] . It is also known to grow slowly over months to years[1]. In this case, the unique presentation is the pilomatrixoma's rapid progression and the pedunculated appearance at the lid margin.
These tumors have a bimodal distribution. They commonly present in the pediatric population and the sixth decade of life, but they can appear at any age.[3] It is known to affect females more than males[1] and can occur anywhere in the body. The most common sites are the head and neck regions, which usually involve the eyebrow and rarely affect the eyelids. The upper extremities follow this[1]. Although that the exact etiology is unknown, some studies linked it to mutations in the B-catenin gene(CTNNB1).[1],[4]
Clinically, these benign lesions have a blue or pink color on the underlying skin. They also have yellow or chalky patches within the tumor, and telangiectatic vessels on the skin overlying the lesion[5]. This classical clinical presentation was present in our patient. The lesion contained white chalky material, and the overlying skin was bluish, possibly due to thinning and stretching of the skin.
Pilomatrixomas are mostly described as solitary lesion, but in rare cases, may be multifocal and can recur after excision[6]. This should raise the suspicion of systemic associations such as turner syndrome, constitutional mismatch repair deficiency, Kabuki syndrome, Steiner's myotonic dystrophy and Gardener syndrome.[6],[7]
It is usually misdiagnosed preoperatively, and the definitive diagnosis is made by surgical excision and histopathology.[2] Clinical differential diagnosis includes epidermoid cysts, dermoid cyst, sebaceous adenoma or carcinoma, chalazion, vascular eyelid tumor like capillary hemangioma, juvenile xanthogranuloma, and in young children, rhabdomyosarcoma.[2]
Histopathologically, pilomatrixoma is a well-circumscribed lesion, with cells arranged in a circular configuration, and intermediate-sized, nucleated, basaloid germinative matrical cells on the periphery that transition into enucleated shadow cells in the center. The eosinophilic shadow cells represent dead cells, with a central unstained area corresponding to the lost nucleus areas of calcification. Foreign body giant cells can be observed.[1]
The definitive treatment is a complete excision with clear margins to minimize the risk of recurrence.[2] Close surveillance after local recurrence is recommended due to the risk of developing secondary malignant lesions.[2],[8],[9] Forbes and Hewig reported a relapse rate between 2% and 6% possibly due to incomplete excision.[1]
Pilomatrix carcinoma is an extremely rare. They occur more often in middle aged men and can either arise from a previous pilomatrixoma as a malignant transformation or arise de novo[8]. Initially they are locally aggressive, can recur if incompletely excised and also can metastasize to lungs, bones and lymph nodes.[9]
Although the typical appearance of pilomatrixoma is nodular, our case showed a pedunculated lesion close to the eyelid margin. It was fixed to the pretarsal part of the orbicularis muscle and progressed rapidly. It had a typical bluish-red skin discoloration, with chalky patches inside the tumor which is a characteristic of pilomatrixoma.
Pilomatrixoma is a benign neoplasm that is often misdiagnosed and should be suspected in any young patient with a firm, mobile mass of the eyelid or eyebrow area.
Only few cases of pedunculated pilomatrixoma were reported in the literature; such pedunculated lesion can be misdiagnosed as vascular malformation or malignant tumor. Therefore, pilomatrixoma should be considered in the differential diagnosis for such presentation. Complete excisional biopsy of the mass is diagnostic and therapeutic.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Jones CD, Ho W, Robertson BF, Gunn E, Morley S. Pilomatrixoma: A comprehensive review of the literature. Am J Dermatopathol 2018;40:631-41.  |
| 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;53:526-35. |
| 3. | Guinot-Moya R, Valmaseda-Castellon E, Berini-Aytes L, Gay-Escoda C. Pilomatrixoma. Review of 205 cases. Med Oral. Published online 2011:e552-e555. doi:10.4317/medoral.16.e552. |
| 4. | Hassanein AM, Glanz SM. beta-catenin expression in benign and malignant pilomatrix neoplasms. Br J Dermatol. 2004;150(3):511-516. doi:10.1046/j.1365-2133.2004.05811.x. |
| 5. | Yap EY, Hohberger GG, Bartley GB. Pilomatrixoma of the Eyelids and Eyebrows in Children and Adolescents: Ophthalmic Plastic & Reconstructive Surgery. 1999;15(3):185-189. doi:10.1097/00002341-199905000-00008. |
| 6. | Zloto O, Fabian ID, Dai VV, Ben Simon GJ, Rosner M. Periocular Pilomatrixoma: A Retrospective Analysis of 16 Cases. Ophthalmic Plastic & Reconstructive Surgery. 2015;31(1):19-22. doi:10.1097/IOP.0000000000000164. |
| 7. | Richet C, Maza A, Dreyfus I, Bourrat E, Mazereeuw-Hautier J. Childhood pilomatricomas: Associated anomalies. Pediatr Dermatol. 2018;35(5):548-551. doi:10.1111/pde.13564. |
| 8. | Hardisson D, Linares MD, Cuevas-Santos J, Contreras F. Pilomatrix Carcinoma: A Clinicopathologic Study of Six Cases and Review of the Literature: The American Journal of Dermatopathology. 2001;23(5):394-401. doi:10.1097/00000372-200110000-00002. |
| 9. | Flynn A. Malignant Pilomatricoma: A Report of Two Cases and Review of Literature. JCDR. Published online 2017. doi:10.7860/JCDR/2017/27589.10260. |
[Figure 1], [Figure 2]
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