A Case of Multiple Eyelid Trichilemmoma Associated with Cowden Syndrome

Ho Cho Chan; Hyuk Son Jun

doi:10.3341/jkos.2013.54.5.803

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Chan and Jun: A Case of Multiple Eyelid Trichilemmoma Associated with Cowden Syndrome

Original Article

J Korean Ophthalmol Soc 2013;54(5):803-807.

Published online: 7 September 2013

DOI: https://doi.org/10.3341/jkos.2013.54.5.803

A Case of Multiple Eyelid Trichilemmoma Associated with Cowden Syndrome

Ho Cho Chan, MD, Hyuk Son Jun, MD, PhD

¹Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Korea

Address reprint requests to Jun Hyuk Son, MD, PhD Department of Ophthalmology, Yeungnam University Medical Center #170 Hyeonchung-ro, Nam-gu, Daegu 705-703, Korea Tel: 82-53-620-3444, Fax: 82-53-626-5936, E-mail: sjh@med.yu.ac.kr

Received 10 August 201216 December 2012 Accepted 25 March 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To report a case of multiple eyelid trichilemmomas associated with Cowden syndrome.

Case summary

A 27-year-old woman presented with multiple upper and lower eyelid skin masses that developed over several years. The masses were as large as whitish millet, and were around the upper and lower eyelid margin and the face. The patient had previously undergone subtotal thyroidectomy for a thyroid mass and a mass excision for extremity hemangioma. Excisional biopsy was performed for the diagnosis, and trichilemmoma was diagnosed based on histopatho-logic examination. Consequently, multiple trichilemmoma associated with Cowden syndrome was diagnosed, and breast evaluations for existence of further masses were recommended. On breast examinations, intraductal papilloma and fi-broadenoma were detected.

Conclusions

The trichilemmoma was a hair-follicle benign tumor that also appeared on the skin around the eyelid. If multi-ple trichilemmoma is diagnosed, an association with Cowden syndrome should be considered as well as presence of masses in other organs.

Keywords: Cowden syndrome, Eyelid mass, Trichilemmoma

References

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Figure 1.

External appearance of the patient. (A) She pre-sented with multiple whitish masses on her upper and lower eyelids and face. (B) Magnified image of the right upper medial canthus. A milium-sized whit-ish mass is seen.

Figure 2.

Surgical biopsy of the eyelid mass. (A) The tumor showed lobular, follicle-centered proliferation with overlying keratosis (arrow head; hematoxylin and eosin stain, ×40) (B) The tumor was composed of bland, amitotic, uniform polyhedral cells with clear glycogenated cytoplasm (small arrow), surrounded by cuffs of basement membrane material. (hematoxylin and eosin stain, ×100) (C) All tumor cells demonstrate basal cell peripheral nuclear palisading pattern in the cellular lobules (big arrow; hematoxylin and eosin stain, ×100) The tumor was diagnosed as trichilemmoma.

Figure 3.

Mammogram (A) and specimen (B) of the right breast. (A) Small oval isoechogenic nodules in cystic echoes and ductal dilatation were seen at the subareolar area (suspected intraductal papilloma). (B) Specimen obtained from the right breast mass after surgical excision. The cut surface shows a dilated duct with solid mural nodule. The tumor was finally di-agnosed as fibroadenoma with a complex sclerosing lesion and intraductal papilloma with florid ductal hyperplasia.

Table 1.

International Cowden Consortium operational criteria for the diagnosis of cowden syndrome (CS), Version 2000

Pathognomonic criteria	Major criteria	Minor criteria
Mucocutaneous lesions	Breast carcinoma	Other thyroid mass (eg, adenoma or multinodular goiter)
Trichilemmomas, facial	Thyroid carcinoma (non-medullary), esp. follicular thyroid carcinoma	Mental retardation (Say, IQ ≤ 75)
Acral keratoses	Macrocephaly (megalencephaly)(Say, ≥95 percentile)	GI harmatomas
Papillomatous papules	Lhermitte-Duclos disease (LDD)	Fibrocystic disease of the breast
Mucosal lesions	Endometrial carcinoma	Lipomas
		Fibromas

GU tumors (eg, renal cell carcinoma, uterine fibroids) or malformation 　　

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