Syringocystadenoma Papilliferum

Syringocystadenoma papilliferum

Authors: Doctor Alexander C. Katoulis1, Doctor Evangelia Bozi Creation date: April 2004

Scientific Editor: Professor Nikolaos G. Stavrianeas

1Department of Dermatology and Venereology, A. Sygros Hospital, National and Kapodistrian University of Athens, Athens, Greece.

Abstract Keywords Synonyms Definition and Histogenesis Epidemiology Clinical description Diagnostic Methods Diagnosis and Differential diagnosis Treatment References

Abstract Syringocystadenoma papilliferum (SP) is a skin hamartoma, which is believed to derived from apocrine sweat glands or, less often, from eccrine sweat glands. It is an exceedingly rare neoplasm, appearing at birth or arising during infancy, around the time of puberty, or later in life. Three clinical types have been described. The plaque type presents mostly as a hairless area in the scalp, often in association with a sebaceous nevus of Jadassohn; it may also appear de novo. The linear type, which is usually seen on the neck or face and the Solitary nodular type, which shows predilection for the trunk. Association with hamartomas of follicular or sebaceous gland origin is common. In about one-third of the cases, SP is associated with a nevus sebaceous. Basal cell carcinoma development has been reported in up to 10% of the cases. Squamous cell, verrucous or ductal carcinoma may also develop, but much less frequently. The diagnosis is clinically suspected and histologically confirmed. Due to the risk of malignant degeneration, prophylactic surgical excision is advisable, wherever possible. Complete surgical excision followed by detailed histological examination is the treatment of choice.

Keywords skin hamartoma, apocrine sweat gland, sebaceous nevus, basal cell carcinoma

Synonyms Epidemiology Syringadenoma papilliferum, naevus SP is an exceedingly rare neoplasm. Almost half syringocystadenomatosus papilliferus are present at birth or appear during infancy (plaque and linear variety). It commonly arises Definition and Histogenesis around the time of puberty, or less commonly, Syringocystadenoma papilliferum (SP) is a during adolescence or adult life (solitary nodule hamartomatous malformation of the skin, which variety) (Pinkus, 1954). is believed to derive from apocrine sweat glands. However, a few may derive from eccrine sweat Clinical description glands. In the past, it was thought to represent Three clinical types have been described. It may an apocrine gland adenoma. Nevertheless, the occur as a linear group of nodules, a nodular histogenesis remains controversial (Yamamoto plaque or as a solitary nodule (Pinkus, 1954). et al, 2002) . According to the classical concept, The plaque type most often presents as a SP is a hamartoma arising from pluripotent cells. hairless area in the scalp. At puberty it may become larger, nodular, verrucous or crusted, resembling nevus sebaceous. Such a lesion

Katoulis AC, Bozi E. Syringocystadenoma papilliferum. Orphanet Encyclopedia. April 2004. http://www.orpha.net/data/patho/GB/uk-Syringocystadenoma-papilliferum.pdf 1 often develops in association with a sebaceous consisting of two rows of cells: an outer layer of nevus of Jadassohn or may appear de novo small cuboidal cells with strongly basophilic without preexisting lesion (Koga, 1999). nuclei; and an inner layer of high columnar cells The linear type is usually seen on the neck or demonstrating "decapitation" secretion face. Lesions consist of multiple pink to red to (characteristic for the secretory cells of the brown, firm papules or nodules, 1-10 mm of apocrine sweat glands). Apocrine glands are diameter, that occur in groups. The papules may prominent deep in the dermis below the be umbilicated, mimicking molluscum invaginations to which they are connected. An contagiosum. Pinpoint vesicle-like inclusions inflammatory infiltrate composed almost entirely filled with clear fluid are usually seen. of plasmacytes is present, especially in the The solitary nodular form shows predilection stroma within the papillary projections (Niizuma for the trunk, especially for the shoulders, axillae K, 1976). and the genital area (Patterson, 2001). The Ultrastructurally, the constituent epithelial cells solitary nodule is up to 1 cm in diameter, it is are divided into three types: luminar tumour cells domed, umbilicated or pedunculated, often with bearing features of the secretory or ductal a friable or crusted surface. Several such luminal cells of sweat glands, but being nodules may be scattered in the same area. somewhat immature in appearance; basal Occasionally, extensive verrucous or papillary tumour cells that were basaloid in nature; and plaques are present. undifferentiated clear cells suggesting stem or Association with hamartomas of follicular or progenitor cell properties (Yamamoto et al, sebaceous gland origin is common (Pinkus, 2002). 1954). In about one-third of the cases, SP is Immunohistochemistry associated with a nevus sebaceus. Multiple The tumor stains positively for carcinoembryonic tumours of adnexal origin (such as antigen. Histochemical studies of individual trichoblastoma, apocrine adenoma, cases showed evidence (large amount of hidradenoma papilliferum, poroma folliculare, glycogen, absence of large secretory granules, trichilemmoma etc.) have been reported to arise presence of amylophosphorylase) of eccrine on a sebaceus nevus, among which a SP may rather than apocrine secretory cells presence. be included (Stavrianeas et al, 1997). Basal cell carcinoma (BCC) development has been Diagnosis and Differential diagnosis reported in up to 10% of the cases (Helwig et al, The diagnosis is clinically suspected and 1955). In the majority of such cases, there is a histologically confirmed. Syringoma (non coexistent nevus sebaceous. It has been grouped lesions) and basal cell carcinoma suggested that allelic loss at 9q22 is consistent (especially around the eye) must be excluded. with the clinical observation of transition of SP to The plaque and linear forms on the head and BCC (Boni et al, 2001). Squamous cell neck may be difficult to distinguish from the carcinoma (SCC) may be also developed, but sebaceous nevus to which they may be related. much less frequently. Since now, only two cases Umbilicated nodules may be confused with of verrucous carcinoma in conjunction with SP Molluscum contagiosum. have been published (Monticciolo et al, 2002). Ductal carcinoma arising from SP has been Treatment reported as well (Hugel et al, 2003). Ulceration Due to the risk of malignant degeneration, or rapid enlargement is indicative of malignant prophylactic surgical excision is advisable, transformation. Association with extra-cutaneous wherever possible. Complete surgical excision abnormalities may be seen in the contact of followed by detailed histological examination is epidermal nevus syndrome. the treatment of choice. Radiotherapy is Syringocystadenocarcinoma papilliferum is the ineffective. Destructive methods are wise to be malignant counterpart of SP (Ishida-Yamamoto avoided. et al, 2001).

References Diagnostic Methods Boni R, Xin H, Hohl D, et al. Histology Syringocystadenoma papilliferum: a study of The epidermis shows papillomatosis. One or potential suppressor genes. Am J Dermatopathol several cystic invaginations extend downward 2001; 23:87-9. from the epidermis. Cystic changes of the sweat Goshima J, Hara H, Okada T, Suzuki H. glands can be observed, i.e. dilated glands and Syringocystadenoma papilliferum arising on the ducts with numerous villus papillary projections scrotum. Eur J Dermatol 2003; 13:271. extending into the lumen of cystic invaginations. Helwig EB, Hackney VC. Syringadenoma Both the invaginations and the papillary papilliferum: lesions with and without naevus projections are lined by a glandular epithelium

Katoulis AC, Bozi E. Syringocystadenoma papilliferum. Orphanet Encyclopedia. April 2004. http://www.orpha.net/data/patho/GB/uk-Syringocystadenoma-papilliferum.pdf 2 sebaceus and basal cell carcinoma. Arch Niizuma K. Syringocystadenoma papilliferum: Dermatol 1955; 71:361-72. light and electron microscopic studies. Acta Hugel H, Requena L. Ductal carcinoma arising Derm Venereol 1976; 56:327-36. from a syringocystadenoma papilliferum in a Patterson JW, Straka BF, Wick MR. Linear nevus sebaceous of Jadassohn. Am J syringocystadenoma papilliferum of the thigh. J Dermatopathol 2003; 25:490-3. Am Acad Dermatol 2001; 45:139-41. Ishida-Yamamoto A, Sato K, Wada T, et al. Pinkus H. Life history of naevus Syringocystadenocarcinoma papilliferum: case syringocystadenomatous papilliferus. Arch report and immunohistochemical comparison Dermatol Syphil 1954; 69:305-22. with its benign counterpart. J Am Acad Dermatol Stavrianeas NG, Katoulis AC, Stratigeas NP, et 2001; 45:755-9. al. Development of multiple tumors in a Koga T, Kubota Y, Nakayama J. sebaceous nevus of Jadassohn. Dermatology syringocystadenoma papilliferum without an 1997; 195:155-8. antecedent naevus sebaceous. Acta Derm Yamamoto O, Doi Y, Hamada T, et al. An Venereol 1999; 79:237. immunohistochemical and ultrastructural study of Monticciolo NL, Schmidt JD, Morgan MB. syringocystadenoma papilliferum. Br J Dermatol Verrucous carcinoma arising within 2002; 147:936-45. syringocystadenoma papilliferum. Ann Clin Lab Sci 2002; 32:434-7.

Katoulis AC, Bozi E. Syringocystadenoma papilliferum. Orphanet Encyclopedia. April 2004. http://www.orpha.net/data/patho/GB/uk-Syringocystadenoma-papilliferum.pdf 3