Syringocystadenoma Papilliferum of Sweat Gland of Caruncle: A Unique Case Report.

Syringocystadenoma papilliferum (SP) is a rare benign hamartomatous malformation of skin which arises from apocrine or eccrine sweat glands. Skin of the head and neck is the usual site for this but rarely involves the eyelids. It is also called as a childhood tumor since it usually appears at birth or during puberty. The diagnosis is confirmed on histopathological examination after surgical excision. We are first to report a case of SP involving the caruncle in an elderly female.

Introduction

Syringocystadenoma papilliferum (SP) is an uncommon benign adnexal tumor of sweat gland.[1] SP is the hamartoma arising from pluripotent cells, and it usually occurs as a solitary lesion.[2] It typically occurs at a young age, and most of the cases present later in the third or fourth decade in extraocular locations.[3] It rarely involves eyelid skin and is not usually associated with nevus sebaceous.[4] To the best of our knowledge, SP involving the caruncle has not been reported yet in the available literature.

Case Report

A 63-year-old female presented to the Outpatient Department of Ophthalmology, Himalayan Institute of Medical Sciences, with a complaint of growth on the inner aspect of the left eye. The growth was painless and progressive in nature and was associated with watering and mucoid discharge. It started as a small wheat grain sized growth and progressively increased over a period of 2 years and became firmer in consistency. The patient came to us as she was uncomfortable due to watering and incomplete closure of the eye due to the protrusion of the mass from the medial angle of the interpalpebral fissure. No history of diplopia was there. Systemic history was unremarkable.

On examination, best-corrected visual acuity was 20/20 and 20/120 for oculus dexter and oculus sinister, respectively. Intraocular pressure was 16 and 14 for the right and left eye, respectively. Clinical examination revealed an irregular oval mass located in the caruncle with multiple yellow-white dots and engorged blood vessels on its surface.

The mass was firm in consistency with irregular papillomatous surface and had a hyperpigmented patch on it [Figure 1]. Mass was moderately tense, mobile, and nontender.

Figure 1

Oval mass in caruncle with hyperpigmented patch and yellow dots on its surface

Yellowish white discharge was extruding out of various points on the surface of growth. Eyelid closure was compromised because of protrusion of the mass through the medial angle of the interpalpebral fissure. Lid margins were intact without any ectropion or punctual involvement. Mild restriction on adduction of the left eye was noted because of the growth.

On slit-lamp examination, the left eye showed cataractous crystalline lens. Rest of anterior and posterior segment examination was unremarkable. Lacrimal passages were freely patent on syringing.

Surgical excision of the mass was done with excision of normal healthy conjunctival frill of 3 mm along with subconjunctival tissue till the sclera plane on all sides of the mass. The mass was dissected in one piece and dissection was completed at medial canthus. The raw area created by excision was covered by the conjunctival graft obtained from the superotemporal aspect of the same eye with a different set of instruments after local infiltration. The mass excised was sent for histopathological examination.

Histopathology

Gross examination showed greyish white to greyish brown soft-tissue piece which measured 20 mm × 20 mm × 10 mm. Outer surface showed firm greyish brown area of exudates.

On microscopic examination, section showed tissue covered with stratified squamous epithelium which was flattened and thinned out. Subepithelial tissue showed partially circumscribed benign lesion composed of glandular structures of variable shapes and sizes which were lined mostly by tall columnar cells having oncocytic appearance. At places, the cells were cuboidal or flattened. The cells were having uniform round nuclei. A few glands were without any lumen. Some glands showed cystic dilatation with mucin in their lumen. The intervening tissue showed inflammatory infiltrates which consisted mainly of lymphocytes. No lymphoid follicle was seen. Many dilated congested blood vessels were seen [Figure 2].

Figure 2

Glands of variable sizes and shapes with some cystically dilated. All are lined by oncocytic cells with surrounding tissue abundant in lymphocytes. Lumen of cystic spaces contains pinkish material. Numerous dilated and congested vessels seen (H and E, ×40)

Diagnosis was established as SP of sweat gland with apocrine change on basis of histopathology.

Discussion

SP is a skin tumor and most of these cases are clinically first noted at birth and other cases develop in infancy, childhood, and adolescence. The lesion presents as an erythematous or brownish papule, nodule, or tumor of varying sizes, usually between 1 cm and 4 cm[5] with flat, smooth, verrucous, or papillomatous surface and is more common in females.[6] Some tumors may show central umbilicated small fistulae which may show discharge. Mostly SP presents with a solitary lesion; however, multiple linear patterns are usually associated with nevus sebaceous. These lesions mostly show gradual enlargement although a few can attain considerable size within a few months. These lesions can cause marked itching which can bleed on scratching, crust formation and breakdown.[4]

Clinical differential diagnosis for SP includes viral warts, verrucous carcinoma, pyogenic granuloma, tuberculosis verrucous cutis, subcutaneous fungal infection, and giant lymphangioma. The differential diagnosis for linear lesions includes nevus comedonicus, linear epidermal nevus, eccrine nevus, cylindroma, and basaloid follicular hamartoma. Fugita et al. noted that SP is most commonly associated with nevus sebaceous followed by basal cell epithelioma, sebaceous epithelioma, apocrine hidrocystoma, trichoepithelioma, and eccrine spiradenoma. However, association of SP with many other lesions has also been noted.[7] Eyelids are the only ocular site for SP reported in the literature till date.[18]

Christakopoulos et al.[9] reported the first case of nodular hidradenoma which is an adnexal tumor of the dermis in mucosal site (caruncle).

On account of the histological composition of the caruncle, which includes conjunctiva, hair follicles, sweat glands, sebaceous glands, and accessory lacrimal tissue, it may develop lesions similar to those of the skin, conjunctiva, or lacrimal gland. The vast majority of lesions of the caruncle are benign, mainly nevi. The most common tumors of the caruncle are nevi, papilloma, oncocytoma, sebaceous tumors, epithelial inclusion cysts, and inflammatory lesions. The diagnosis of caruncular lesions is difficult and depends mainly on histologic examination.[10]

Till date, 18 cases of SP have been reported in eyelids according to Vani et al.[11] To the best of our knowledge, caruncle is still unreported site for SP. The present case has added to the newer ocular sites for SP.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.