Hamdan A. Al-Habsi, Mustafa Al-Hinai, Ahmed Al-Waily, Salim Al-Sudairy and Vipula de Silva Online Case Report | e575 Departments of 1Family Medicine & Public Health and 2Oral Health, Sultan Qaboos University Hospital, Muscat, Oman; 3Department of Pathology, Faculty of Medicine, University of Colombo, Sri Lanka Corresponding Author e-mail: habsi@squ.edu.om SYRINGOCYSTADENOMA PAPILLIFERUM (SCAP) is a rare benign skin tumour believed to originate from the pluripotent cells and its histology exhibits either apocrine or eccrine sweat gland differentiation.1 The tumour appears predominantly in childhood; it appears at birth in approximately 50% of those affected and before puberty in a further 15–30%.2–4 However, in some cases it may appear at a later age.5 The tumour is more common in women than men.6 The clinical presentation of SCAP is not distinctive and may often be misleading—it can present as a solitary patch, papule, plaque, nodule or as several papules often arranged linearly.7–9 The plaque type usually presents as a hairless area on the scalp, while the linear type appears on the face and neck region and the solitary nodular type shows a predilection for the trunk.8,9 The colour of the tumour varies from flesh- coloured to pink, red, brown or grey.6,10 Its surface may be smooth, hyperkeratotic, verrucous, papillary or moist and fleshy. The tumour tends to grow slowly and is usually less than 4 cm in size.4 While the clinical features of SCAP vary widely, its histology is invariably uniform and confirmatory.7 Although the lesion is benign, transformation to basal cell carcinoma, metastatic adenocarcinoma or ductal carcinoma may occur.5,8,11 An unusual case of this entity occurring on the upper lip is documented below. To date, only three cases involving the upper lip have been reported in the literature.5 Case Report A 10-year-old girl presented to the Sultan Qaboos University Hospital in Muscat, Oman, in February 2012 with a non-tender, non-pruritic, solitary verrucous papule on the left side of the upper lip. The papule had been present since birth and was associated with recurrent ulceration and bleeding. It had been slowly increasing in size over time. She had consulted many different physicians but no definite diagnosis had been made. Numerous treatments had been attempted without success, including scraping the lesion on a number of occasions and the application of various topical medications, including traditional medicinal treatments. There was no other significant past medical history. A physical examination revealed a solitary painless raised papule of 4 x 5 mm on the left side of the upper lip, with an area of surrounding blood clots [Figure 1]. Sultan Qaboos University Med J, November 2014, Vol. 14, Iss. 4, pp. e575−577, Epub. 14TH Oct 14 Submitted 21ST Feb 14 Revision Req. 13TH Apr 14; Revision Recd. 11TH May 14 Accepted 9TH Jun 14 ورم الغدد العرقية احلليمي يف الشفة العليا حمدان احلب�سي، م�سطفى الهنائي، ِاأحمد الوائلي، �سامل ال�سديري، فيبوال دي �سيلفا abstract: Syringocystadenoma papilliferum (SCAP) is a rare skin tumour believed to arise from the apocrine or eccrine sweat glands. It appears predominantly in childhood, usually at birth. It is exceedingly rare for it to appear on the upper lip. We report a case of SCAP in a 10-year-old Omani girl who presented to the Sultan Qaboos University Hospital in Muscat, Oman, in February 2012 with a non-tender, non-pruritic, solitary verrucous papule of 4 x 5 mm on the left side of the upper lip. It had been present since birth and had slowly been increasing in size over the years. It was occasionally associated with recurrent ulceration and bleeding and had previously been misdiagnosed and mismanaged. An excisional biopsy was performed and the whole lesion was removed. The surgical site was then sutured and the patient was discharged on the same day. Keywords: Skin Neoplasms; Sweat Glands; Lip; Misdiagnoses; Basal Cell Carcinoma; Case Report; Oman. امللخ�ص: ورم الغدد العرقية احلليمي ورم جلدي نادر ي�سيب الغدد العرقية املفرتزة والفارزة يف اجللد. غالبا ما يظهر يف الطفولة عادة عند الوالدة. نادرا جدا ما يظهر هذا الورم على ال�سفاه العليا. يف هذا املقال نقدم تقريرا عن طفلة عمانية تبلغ من العمر 10 �سنوات زارت م�ست�سفى جامعة ال�سلطان قابو�س يف م�سقط، عمان يف فرباير 2012 وعليها ورم ثوؤلويل منفرد )5 × 4 مم( ، غري موؤمل وبغري حكة على اجلانب االأي�رس من ال�سفة العليا كان قد ظهر منذ الوالدة. ازداد حجم الورم ببطء على مدى ال�سنني مع تقرح ونزيف متكرر. مل تكن حماوالت ت�سخي�سه وعالجه ال�سابقة ناجحة. مت اإجراء خزعة ا�ستئ�سالية ومتت اإزالة التقرح كله. مت تخييط املوقع اجلراحي وخرجت املري�سة يف اليوم نف�سه. مفتاح الكلمات: االأورام اجللدية؛ غدد العرق؛ ال�سفة العليا؛ الت�سخي�س اخلاطئ؛ �رسطان اخلاليا القاعدية؛ تقرير حالة؛ عمان. Syringocystadenoma Papilliferum of the Upper Lip *Hamdan A. Al-Habsi,1 Mustafa Al-Hinai,1 Ahmed Al-Waily,1 Salim Al-Sudairy,2 Vipula de Silva3 ONLINE CASE REPORT Syringocystadenoma Papilliferum of the Upper Lip e576 | SQU Medical Journal, November 2014, Volume 14, Issue 4 No other abnormalities were detected around the lesion or on any other parts of the body. An initial diagnosis of a granuloma was made. An excisional biopsy was performed and the whole lesion was removed and sent for a histopathology examination. The defect was then closed with a suture and the patient was discharged on the same day. The pathological findings revealed a gross specimen measuring 15 x 5 x 3 mm, consisting of multiple fragments of soft tissue. The specimen was processed using a Haematoxylin and Eosin stain. Microscopically, a biopsy determined that the specimen was comprised of epidermal, dermal and skeletal muscle cells. The epidermis was hyperplastic, showing cystic invagination into the dermis, with infiltration of the lymphocytes and neutrophils [Figure 2]. In the lower part of the epidermis, a lesion was observed. The lesion was composed of a few papillary projections and ducts/gland-like structures lined by luminal and cuboidal cells. The papillary projections were lined by double epithelial cells with an abundance of plasma cell infiltrates in their cores [Figures 3 and 4]. No atypia or mitosis was seen. The overall architecture of the lesion revealed an invagination from the epidermis with overlying squamous hyperplasia, papillary projections lined by double epithelial cells and infiltration of the plasma cells. This morphology was consistent with a diagnosis of SCAP. Discussion SCAP is a rare benign tumour of the skin, usually seen in patients at birth or during infancy or early childhood. Most lesions are not distinctive and require a biopsy for the diagnosis; however, the tumour is usually described as ranging from skin-coloured to pink, and taking the form of either a hairless firm plaque, grouped nodules or a solitary nodule.12 Figure 1: Photograph of a non-tender, non-pruritic solitary verrucous papule on the left side of the upper lip of a 10-year-old girl. This papule had been present since birth. Figure 2: Histopathology of the skin lesion showing the cystic invagination of the epidermis with papillary projections (black arrows) and duct-like structures (white arrows). The specimen was processed using Hematoxylin and Eosin stain at x5 magnification. Figure 3: Duct-like structures (white arrows) and papillary projections (black arrows) lined by double epithelial cells can be observed. Plasma cells are visible in the cores (arrow heads). The specimen was processed using hematoxylin and eosin stain at x20 magnification. Figure 4: Histopathology indicated an abundance of plasma cells (white arrows). The specimen was processed using hematoxylin and eosin stain at x20 magnification. Hamdan A. Al-Habsi, Mustafa Al-Hinai, Ahmed Al-Waily, Salim Al-Sudairy and Vipula de Silva Online Case Report | e577 The differential diagnosis of SCAP is broad. It can include nevus sebaceous, hidradenoma papilliferum, molluscum contagiosum, verruca vulgaris, squa- mous cell carcinoma, keratoacanthoma and basal cell carcinoma.4,13 The clinical presentation of the tumour is non- specific and can resemble any of the aforementioned conditions;7,8 however, the histopathological features are specific and diagnostic. The tumour histology shows characteristic epidermal cystic invaginations into the dermis, papillary projections and abundant plasma cell infiltration.2,8,10 The invaginations are lined by a double layer of epithelial cells, whereas the papillary projections are lined by glandular epithelium which often exhibits decapitation secretion.4,8,10 Immunohistochemical studies using epithelial membrane antigen and cytokeratin markers show that the epithelium is composed of several cell types at different stages of development, with a tendency to differentiate toward the basal cells in the apocrine lineage or luminal cells in the secretory epithelium.1,11,14 The results of these studies support the theory that SCAP is a tumour originating from the pluripotent cells. Cases of SCAP are sporadic and most commonly seen in the scalp region. They are only rarely described in other parts of the body.4,10,13 It is hoped that this report will add to the available information on SCAP involving the upper lip. An extensive search of the literature has shown that only three similar cases have been documented so far. Although this lesion is benign, a transformation to basal cell carcinoma, metastatic adenocarcinoma or ductal carcinoma may still occur.15,16 SCAP has been associated with basal cell carcinoma in about 10% of cases.7,10 Squamous cell carcinoma may also develop, but much less frequently. Other malignant tumours associated with SCAP include verrucous and ductal carcinomas.16 Rapid growth, pain, ulceration or bleeding in a long-standing lesion may indicate a malignant transformation to syringocystadenocarci noma papilliferum.6,16 Conclusion SCAP is an uncommon tumour and its appearance on the upper lip is very rare. The clinical presentation of this tumour resembles many other skin lesions. It should be considered in the differential diagnosis of any long-standing plaque or nodular lesion, particularly if there is an increase in size or the occurrence of ulceration and/or bleeding. The tumour has characteristic histological features which can be used as the basis for diagnosis. Malignant transformation can occur; a complete excision of the lesion is therefore the treatment of choice. References 1. Yamamoto O, Doi Y, Hamada T, Hisaoka M, Sasaguri Y. An immunohistochemical and ultrastructural study of syringocystadenoma papilliferum. Br J Dermatol 2002; 147:936–45. doi: 10.1046/j.1365-2133.2002.05027.x. 2. Karg E, Korom I, Varga E, Ban G, Turi S. Congenital syringocystadenoma papilliferum. Pediatr Dermatol 2008; 25:132–3. doi: 10.1111/j.1525-1470.2007.00607.x. 3. Böni R, Xin H, Hohl D, Panizzon R, Burg G. Syringocystadenoma papilliferum: A study of potential tumor suppressor genes. Am J Dermatopathol 2001; 23:87–9. 4. Sangma MM, Dasiah SD, Bhat V R. Syringocystadenoma papilliferum of the scalp in an adult male: A case report. J Clin Diagn Res 2013; 7:742–3. doi: 10.7860/JCDR/2013/5452.2900. 5. Helwig EB, Hackney VC. Syringadenoma papilliferum: Lesions with and without naevus sebaceous and basal cell carcinoma. AMA Arch Derm 1955; 71:361–72. doi: 10.1001/ archderm.1955.01540270073011. 6. Monticciolo NL, Schmidt JD, Morgan MB. Verrucous carcinoma arising within syringocystadenoma papilliferum. Ann Clin Lab Sci 2002; 32:434–7. 7. Chauhan A, Gupta L, Gautam RK, Bhardwaj M, Gopichandani K. Linear syringocystadenoma papilliferum: A case report with review of literature. Indian J Dermatol 2013; 58:409. doi: 10.4103/0019-5154.117353. 8. Katoulis A, Bozi E, Stavrianeas NG. Syringocystadenoma papilliferum. From: www.orpha.net/data/patho/GB/uk-Syring ocystadenoma-papilliferum.pdf Accessed: Sep 2014. 9. Pinkus H. Life history of naevus syringadenomatosus papilliferus. AMA Arch Derm Syphilol 1954; 69:305–22. doi: 10.1001/archderm.1954.01540150051004. 10. Xu D, Bi T, Lan H, Yu W, Wang W, Cao F, et al. Syringocystadenoma papilliferum in the right lower abdomen: A case report and review of literature. Onco Targets Ther 2013; 6:233–6. doi: 10.2147/OTT.S42732. 11. Choccalingam C, Samuel P, Subramaniam D, Hammed F, Purushothaman V, Joshi R. Syringocystadenocarcinoma papilliferum: A case report of a rare skin adnexal tumour. Our Dermatol Online 2013; 4:221–3. doi: 10.7241/ourd.20132.54 12. Sood A, Khaitan BK, Khanna N, Kumar R, Singh MK. Syringocystadenoma papilliferum at unusual sites. Indian J Dermatol Venereol Leprol 2000; 66:328–9. 13. Yap FB, Lee BR, Baba R. Syringocystadenoma papilliferum in an unusual location beyond the head and neck region: A case report and review of literature. Dermatol Online J 2010; 16:4. 14. Kurokawa I, Mizutani H, Nishijima S, Kusumoto K, Tsubura A. 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