Syringocystadenoma Papilliferum: a Rare Tumor in an Unusual Location

Case report Paediatrics Today 2015;11(1):50-53 DOI 10.5457/p2005-114.109

SYRINGOCYSTADENOMA PAPILLIFERUM: A RARE TUMOR IN AN UNUSUAL LOCATION

Jelena ROGANOVIĆ1, Ela BRGODAC1, Nives JONJIĆ2

1Department of Pediatrics, Clinical Hospital Objective – To report a very rare case of syringocystadenoma papil- Center Rijeka, Croatia, 2Department of liferum, describing its clinical presentation, histopathological appear- Pathology and Pathological Anatomy ance and surgical management. Case report – An 11-year-old girl School of Medicine, University of Rijeka presented with a partially lobulated linear plaque on her right lower Rijeka, Croatia back present since birth, which was clinically diagnosed at first as lin- Corresponding author: ear epidermal nevus. The tumor was surgically removed. The histopa- Roganović Jelena thology confirmed the diagnosis of syringocystadenoma papilliferum. Clinical Hospital Center Rijeka Conclusion – Syringocystadenoma papilliferum is a benign adnexal Istarska 43, 51000 Rijeka tumor that occurs most often in the head and neck. The tumor has [email protected] characteristic histological features, and varied and non-distinct clinical Tel.: + 385 51 659 103/109 findings. Although extremely rare, this lesion should be considered in Fax.: + 385 51 623 126 the differential diagnosis of skin tumors in children. Received: July 27, 2014 Accepted: November 1, 2014 Key words: Syringocystadenoma papilliferum ■ Skin hamartoma ■ Child. Copyright © 2015 by University Clinical Centre Tuzla. E-mail for permission to publish: [email protected]

Introduction Case report Syringocystadenoma papilliferum (SCAP) An 11-year-old girl presented with a discrete is a very rare benign hamartomaous skin tu- pink-colored superficial, occasionally pruritic mor. Its exact histogenesis remains unclear, lesion on the right lower back. The lesion and possible apocrine, eccrine or apoeccrine had been present since birth, and no sig- origin has been broadly debated (1). Tumors nificant change in size or general appearance are usually seen at birth or appear during was noted in the following years. A physical childhood and tend to proliferate around pu- examination revealed a solitary, 1,5x1 cm in berty (2). SCAP may occur de novo or within size hairless plaque, with partially lobular a nevus sebaceous. The majority of patients structure. The clinical suspicion was a linear present with a solitary lesion in the head and epidermal nevus. There were no associated neck region (2). The localization over abdo- systemic symptoms. The routine hemato- men is rare, and only seven cases have been logical and biochemical tests were normal. published in the literature. We report an un- The patient was referred to the pediatric usual case of SCAP presenting on the back. surgeon for complete excision. Histopatho-

50 www.paediatricstoday.com J. Roganović et al. ■ Syringocystadenoma papilliferum

Fig. 1 Syringocystadenoma papilliferum: (A) invaginations from the overlying epidermis (B) fulfilled with papillae, covered with a luminar layer of tall columnar cells and the inner layer of small myoepithelial cells with hyperchromatic nuclei. logic examination (Fig. 1) demonstrated unclear, and the results of immunohisto- cystic invagination of epidermis with papil- chemistry and electron microscopy have been lary proliferation. Papillae were mostly lined conflicting (4). It was postulated that SCAP by multilayer squamous epithelium, with arises from a pluripotent cells with the po- inner row of small myoepithelial cells and tential to exhibit either apocrine or eccrine columnar cells in the outer layer. Glandular differentiation, but there is an increasing evi- formations were interspersed in epithelium. dence for an apocrine origin (1). Three clini- The fibrovascular core of the papillae showed cal types have been described. It may present plasmacellular inflammatory infiltrate. The as a solitary nodule (solitary nodular form), underlying deep dermis showed two cystic a nodular hairless plaque (plaque type), or enlarged channels. Slightly hyperplastic, hy- as a linear group of brownish or erythema- perkeratotic and parakeratotic epithelium tous papules or nodules (linear type) (3). The was located peripherally. These findings were tumor varies in size from 5 to 160 mm (5). consistent with the diagnosis of SCAP. The The surface can be smooth, flat, papilloma- postoperative period was uneventful. At one- tous, or verrucous. Most of the patients pres- year follow-up, the girl is doing well with no ent with solitary lesions. Multiple lesions are evidence of local recurrence. usually associated with naevus sebaceous (3, 5). SCAP is also associated with linear nae- Discussion vus verrucosus, apocrine poroma, apocrine hidrocystoma, tubulopapillary hidradenoma, SCAP is an uncommon benign hamartoma- hidradenoma papilliferum, papillary eccrine tous adnexal tumor. The histogenesis remains adenoma, apocrine acrosyringeal keratosis,

51 Paediatrics Today 2015;11(1):50-53

poroma folliculare, linear naevus verrucosa, Allelic loss at 9q22 is consistent with the clin- atypical fibroxanthoma, clear cell syringoma, ical observation of transition of SCAP to bas- basal cell epithelioma, sebaceous epithelio- al cell carcinoma. Squamous cell carcinoma, ma, trichoepithelioma, verruca vulgaris, and verrucous carcinoma and ductal carcinoma naevus comedonicus. The most common as- arising from SCAP have been published (3). sociated lesion is nevus sebaceous (31,7%), Syringocistadenocarcinoma papilliferum is a followed by basal cell epithelioma (10,3%), malignant counterpart of SCAP (8, 9). sebaceous epithelioma (3,2%), apocrine The treatment of choice for SCAP is hidrocystoma (3,2%), trichoepithelioma complete surgical excision. Carbon dioxide (1,6%), and eccrine spiradenoma (0,8%) (6). laser can be useful for lesions on anatomic In contrast to a widely variable clinical ap- sites not suitable for surgery (9). To date, pearance, the histopathology is uniform and there are few reported cases of SCAP of aris- confirmatory. SCAP is characterized by cystic ing on the abdomen (7, 10-17). Our patient invaginations extending downwards from the had an extremely rare location of the plaque epidermis. The upper regions of the cystic in- type. Her tumor was present since birth and, vaginations are commonly lined by keratin- unusual for this type of the tumor, did not izing squamous epithelial cells. The lower change over time. There was no association regions, which contain numerous papillary with other benign adnexal lesion. Based on projections, are lined by glandular epitheli- the appearance of the lesion and the long his- um consisting of two rows of cells: a luminar tory, SCAP was not clinically suspected in layer of high columnar cells frequently dem- our case. We performed a complete excision onstrating “decapitation” secretion (charac- with detailed histologic examination. This teristic of the secretory cells of the apocrine case emphasizes that SCAP should be consid- sweat glands), and a basal layer of small cu- ered in the differential diagnosis of childhood boidal cells with strongly basophilic nuclei. adnexal tumors. The connective tissue stroma within the papillary projections is marked by rich infil- Conclusion tration of inflammatory cells; most notably there is a large number of plasma cells. Solitary skin lesions in unusual locations in Almost half of the tumors are present at children have a wide array of differential di- birth or appear during infancy. The lesion agnoses, including SCAP. The treatment of usually increases in size at puberty, becoming choice is a local excision with confirmatory papillomatous and crusted. The great major- histopathology. ity of SCAP occur in the head and neck area. Mammino et al. reviewed 145 cases of SCAP Authors’ contributions: Conception and design: JR; Acquisition, analysis and interpretation of data: JR, EB, and found 108 cases (75%) on the head and NJ; Drafting the articleJR, EB, NJ; Revising it critically neck, 29 cases (20%) on the trunk, and 8 for important intellectual content: JR. cases (5%) on the extremities (2). However, over last two decades there are increasing re- Conflict of interest:The authors declare that they have no conflict of interest. ports of SCAP occurring in unusual locations outside the head and neck region (7). Malignant transformation may occur. References Basal cell carcinoma development has been 1. Paradiso B, Bianchini E, Cifelli P, Cavazzini reported in up to 10% of SCAP, in the major- L, Lanza G. A new case of syringocystadeno- ity of them with coexistent nevus sebaceous. carcinoma papilliferum: a rare pathology for a

52 J. Roganović et al. ■ Syringocystadenoma papilliferum

wide-ranging comprehension. Case Rep Med. 9. Hoekzema R, Leenarts MF, Nijhuis EW. Syringo- 2014;2014:453874. doi: 10.1155/2014/453874. cystadenocarcinoma papilliferum in a linear nevus Epub 2014 May 15. PubMed PMID: 24959179; verrucosus. J Cutan Pathol. 2011;38(2):246-50. PubMed Central PMCID: PMC4052556. 10. Xu D, Bi T, Lan H, Yu W, Wang W, Cao F, et al. 2. Mammino JJ, Vidmar DA. Syringocystadenoma Syringocystadenoma papilliferum in the right low- papilliferum. Int J Dermatol. 1991;30(11):763-6. er abdomen: a case report and review of literature. Onco Targets Ther. 2013;6:233-6. 3. Katoulis AC, Bozi E. Syringocystadenoma papilliferum. Orphanet Encyclopedia. 2004 April. 11. Suzuki T, Ikeda H, Hamasaki Y, Hatamochi A, [cited 2014 May 10]. Available from: https://www. Yamazaki S. Syringocystadenoma papilliferum as- orpha.net/data/patho/GB/uk-Syringocystadeno- sociated with apocrine poroma. J Dermatol. 2006; ma-papilliferum.pdf. 33(4):249-51. 4. Yamamoto O, Doi Y, Hamada T, Hisaoka M, Sa- 12. Yap FB, Lee BR, Baba R. Syringocystadenoma saguri Y. An immunohistochemical and ultrastruc- papilliferum in an unusual location beyond the tural study of syringocystadenoma papilliferum. Br head and neck region: a case report and review of J Dermatol. 2002;147(5):936-45. literature. Dermatol Online J. 2010;16(10):4. 5. Agrawal R, Kumar R, Varshney R. Syringocystade- 13. Chauhan A, Gupta L, Gautam RK, Bhardwaj M, noma papilliferum: an unusual presentation. Jour- Gopichandani K. Linear syringocystadenoma pap- nal of Clinical and Diagnostic Research [serial on- illiferum: a case report with review of literature. line] 2014 May [cited: 2014 Jul 27]; Available from: Indian J Dermatol. 2013;58(5):409. http://www.jcdr.net/back_issues.asp?issn=0973- 14. Kar M, Kar JK, Maiti S. Giant linear syringocyst- 709x&year=2014&month=May&volume=8&issu adenoma papilliferum of the back. Indian J Der- e=5&page=QD03-QD04&id=4336. matol Venereol Leprol. 2012;78(1):123. 6. Fujita M, Kobayashi M. Syringocystadenoma pap- 15. Lee HJ, Cho E, Kim MH, Cho SH, Lee JD. Syrin- illiferum associated with poroma folliculare. J Der- gocystadenoma papilliferum of the back combined matol. 1986;13(6):480-2. with a tubular apocrine adenoma. Ann Dermatol. 7. Ghosh SK, Bandyopadhyay D, Chatterjee G, Bar 2011;23(Suppl 2):151-4. C. Syringocystadenoma papilliferum: an unusual 16. Townsend TC, Bowen AR, Nobuhara KK. Syrin- presentation. Pediatr Dermatol. 2009;26(6):758-9. gocystadenoma papilliferum: an unusual cu- 8. Ishida-Yamamoto A, Sato K, Wada T, Takahashi taneous lesion in a pediatric patient. J Pediatr. H, Iizuka H. Syringocystadenocarcinoma papil- 2004;145(1):131-3. liferum: case report and immunohistochemical 17. Monticciolo NL, Schmidt JD, Morgan MB. Verru- comparison with its benign counterpart. J Am cous carcinoma arising within syringocystadenoma Acad Dermatol. 2001;45(5):755-9. papilliferum. Ann Clin Lab Sci. 2002;32(4):434-7.