Syringocystadenoma papilliferum of eyelid – A deviant case report

Syringocystadenoma papilliferum of eyelid ISSN: 2394-0026 (P)Case Report ISSN: 2394-0034 (O)Syringocystadenoma papilliferum of eyelid – A deviant case reportVani D*, Giri Punja M, Geetanjali S, Bharathi M, Dayananda TRDepartment of Pathology, Mysore Medical College and Research Institute, Mysore, India *Corresponding author email: [email protected] to cite this article: Vani D, Giri Punja M, Geetanjali S, Bharathi M, Dayananda TR.Syringocystadenoma papilliferum of eyelid – A deviant case report. IAIM, 2015; 2(4): 199-202.Available online at www.iaimjournal.comReceived on: 04-04-2015 Accepted on: 08-04-2015AbstractSyringocystadenoma papilliferum, a distinct dermatologic entity, is an exceedingly rare benign skinneoplasm and seems to have arisen from apocrine or eccrine adnexal structures. The diagnosis isclinically suspected and histologically confirmed due to its non-distinct clinical findings and variedpresentations. Since it usually appears at birth or during puberty and adolescence, it is called asChildhood tumor. Complete surgical excision is the treatment of choice followed by detailedhistologic examination, to avoid the risk of malignant degeneration. Herein, we have reported a rarecase of Syringocystadenoma papilliferum of eyelid in an adolescent female.Key wordsSyringocystadenoma papilliferum, Eyelid, Childhood tumor.Introduction Scalp, face and neck are the common sites although rarely these tumours are found on theSyringocystadenoma is a benign hamartomatous trunk, chest, breast, axillae, genitalia, arm, vulva,adnexal tumor, the histogenesis of which is still vagina, thigh, ear canal and eyelid.unclear [1]. It has been theorized that Syringocystadenoma papilliferum can rarelypluripotent cells of apocrine lineage give rise to affect the eyelid skin [4] with very fewSyringocystadenoma papilliferum [1, 2]. This documented cases in the literature.rare tumor may occur de-novo or within a nevussebaceous of Jadassohn and presents commonly Case reportas a solitary lesion. Clinically, most of the lesionshave non-specific features and are first noted at 19 year old female was presented with a wellbirth in 50% of the patients, while the remainder defined growth in the left upper eyelid sinceof 15-30% present later during adolescence and four years, which was gradually progressive inadulthood [3]. size during the past one year. On local examination, it was a well defined,International Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 199Copy right © 2015, IAIM, All Rights Reserved.

Syringocystadenoma papilliferum of eyelid ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)erythematous nodular lesion measuring 0.5x0.5 Photo - 2: Microphotograph showing papillarycm and a clinical diagnosis of pyogenic projections lined by outer cuboidal cells andgranuloma was considered followed by inner columnar cells with luminal decapitationexcisional biopsy for histopathological secretions. (400X)examination. Ophthalmic examination andsystemic examination of the subject wasinsignificant.On gross examination, a solitary lesion with Discussion1.0x0.5 cm dimensions was noted which wasfirm and fleshy. Histopathological examination Skin, the largest organ of the body [5] isdemonstrated a well circumscribed lesion which commonly affected by a multitude of neoplastic,showed a papillary projection covered with congenital and developmental lesions andsquamous epithelium and ductal invaginations aberrations, some of which are so rare to beinto the deeper portions of the lesion was mentioned in aeons of medical literature.noted. (Photo – 1) These ductal structures were Syringocystadenoma papilliferum, an unusuallined by an inner columnar epithelium skin tumour was first described by Stokes [6] indemonstrating luminal apical decapitation 1917 termed as “Nevus Syringocystadenomasecretions, and an outer layer of small cuboidal papilliferum”.cells. Underlying the papillary epithelium, thefibrovascular dermal tissue exhibited prominentplasma cell rich infiltrate with occasionallymphocytes and eosinophils. (Photo – 2) Withthe above classic histologic findings, a diagnosisof Syringocystadenoma papilliferum was arrivedat.Photo - 1: Microphotograph showing a well Clinically, most of the cases ofcircumscribed lesion with cystic spaces and Syringocystadenoma papilliferum are first notedpapillary projections in the papillary dermis. at birth and other cases develop in infancy,(100X) childhood and adolescence. The lesion presents as an erythematous or brownish papule, nodule or tumour of varying sizes, usually between 1 to 4 cm [7] with flat, smooth, verrucous or papillomatous surface and is more common in females [8]. Most patients of Syringocystadenoma papilliferum present with a solitary lesion, however multiple papules arranged in linear pattern are usually associated with nevus sebaceous. Clinical differential diagnosis for syringocystadenoma papilliferum includes viralInternational Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 200Copy right © 2015, IAIM, All Rights Reserved.

Syringocystadenoma papilliferum of eyelid ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)warts, verrucous carcinoma, pyogenic Rapid enlargement or ulceration over thegranuloma, tuberculosis verrucous cutis, growth is indicative of a malignantsubcutaneous fungal infection and giant transformation and is termedlymphangioma. The differential diagnosis for Syringocystadenocarcinoma papilliferum. Up tolinear lesions includes nevus comedonicus, 10% cases of Syringocystadenoma papilliferumlinear epidermal nevus, eccrine nevus, show transformation into basal cell carcinomacylindroma and basaloid follicular hamartoma. [7]. However, no such malignant transformation or association with malignant neoplasm ofFugita, et al. [1] noted that Syringocystadenoma eyelid has been reported [9].papilliferum is most commonly associated withnevus sebaceous followed by basal cell Conclusionepithelioma, sebaceous epithelioma, apocrinehydrocystoma, trichoepithelioma, and eccrine In conclusion, we have added to the literature,spiradenoma. However, association of another case of SyringocystadenomaSyringocystadenoma papilliferum with many papilliferum of upper eyelid, which is frequentlyother lesions has also been noted [1]. misdiagnosed clinically and such a presentation may generate multitude of differentialEyelid is a very rare site for the occurance of diagnoses, hence it must be sent forSyringocystadenoma papilliferum [9]. Yap FB, et histopathological examination.al., in 2010 reviewed for cases ofSyringocystadenoma papilliferum outside the Referenceshead and neck region in the English literature,only 69 cases were with such characteristics and 1. Yap FB, Lee BR, Baba R.none was reported on the eyelid [1]. In a study Syringocystadenoma papilliferum in anby Barbarino etal in 2009, fourteen cases with unusual location beyond the head andSyringocystadenoma papilliferum of the eyelid neck region: A case report and review ofwere identified [9]. Helmi A, et al. in their study literature. Dermatol online j., 2010;in 2011, reported three cases of arising in the 16(10): 4.eyelid [10]. To date, there have been onlyseventeen cases of Syringocystadenoma 2. Lever WF, Schaumburg-Lever G.papilliferum reported on this rare location. Tumours of the epidermal appendages. In Histopathology of the skin. 9th edition;The histogenesis of Syringocystadenoma Philadelphia: JB Lippincott; 2005; p. 890-papilliferum is controversial. It was suggested by 6.Harkey and helming that, the lesion is eitherderived from the glandular ducts intermediate 3. Bruno CB, Corderio FN, Soares Fdo E,between apocrine and eccrine or an adenoma of Takano GH, Mendes LS. Dermoscopiceccrine ductal origin [1]. According to Lever, it is aspects of Syringocystadenomaa hamartoma derived from pluripotent cells [2]. papilliferum associated with nevusPinkus postulated that papillomatous area may sebaceus. An Bras Dermatol., 2011;have been derived from apocrine or eccrine 86(6): 1213-6.glands and hamartomatous proliferation of theinvolved skin would give rise to the 4. Rao VA, Kamath GG, Kumar A. Anadenomatous component of the lesion [1]. unusual case of Syringocystadenoma papilliferum of the eyelid. Indian J Ophthalmol., 1996; 44: 168-9. 5. Godkhindi VM, Meshram DP, Deshpande SA, Suvernekar SV.International Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 201Copy right © 2015, IAIM, All Rights Reserved.

Syringocystadenoma papilliferum of eyelid ISSN: 2394-0026 (P) ISSN: 2394-0034 (O) Syringocystadenoma Papilliferum-Case syringocystadenoma papilliferum. Ann Report. IOSR Journals (IOSR Journal of Clin Lab Sci., 2002; 32(4): 434-7. Dental and Medical Sciences), 2013; 9. Barbarino S, McCormick SA, Lauer SA, 5(1): 43-46. Milman T. Syringocystadenoma6. Stokes JH. A clinic-pathologic study of an papilliferum of the eyelid. Ophthal Plast unusual cutaneous neoplasm combining Reconstr Surg., 2009; 25(3): 185-8. nevus Syringadenomatosus papilliferus 10. Helmi A, Alaraj AM, Alkatan H. Report and a granuloma. J Cutan Dis., 1917; 35: of 3 histopathologically documented 411-9. cases of Syringocystadenoma7. Sangma MMB, Dasiah SD, Bhat VR. papilliferum involving the eyelid. Syringocystadenoma papilliferum of the Canadian Journal of Ophthalmology, Scalp in an Adult Male – A Case Report. J 2011; 46(3): 287-289. Clin Diagn Res., 2013; 7(4): 742-43.8. Monticciolo NL, Schmidt JD, Morgan MB. Verrucous carcinoma arising withinSource of support: Nil Conflict of interest: None declared.International Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 202Copy right © 2015, IAIM, All Rights Reserved.