Download Slide 1

The clinical and
phatologycal features of the
naevus
syringocystadenomatosus
Floarea Sărac, Corina Bud,
Simona Frăţilă
University of Oradea, Faculty of Medicine and
Pharmacy, Dermatology Department, Romania
Case report
• We describe a 38 - year-old man with syringocystadenoma
papilliferum developing within a nevus sebaceus with sebaceous
differentiation in an intradermal tubular apocrine component of the
syringocystadenoma papilliferumThe patient reported a tumoral
lesion with a history of 1 month developing in a preexisting scalp
lesion that had been present from birth. The patient had no previous
personal and family history of malignancy.
• Local examination revealed a well delimited pink-brownish alopecic
plaque measuring 2cm, in the occipital area, with a papilomatous
surface. For a month a firm, hemispheric ulcerated nodular tumor of
0.5 cm diameter. General clinical examination was negative and
biologic investigations were within normal values. The clinical
examination suggests a neoplastic tumor (basal or squamous cell
carcinoma) arising on a sebaceous nevus Jadassohn (Fig.1)
• Histopathological examination confirms the diagnosis of sebaceous
nevus, showing papillomatosis and important dermal sebaceous
hyperplasia. One or several cystic invaginations extend downward
from the epidermis. Cystic changes of the sweat glands can be
observed, i.e. dilated glands and ducts with numerous villus
papillary projections are lined by a glandular epithelium consisting of
two rows of cells: an outer layer of small cuboidal cells with strongly
basophilic nuclei
Discussion
•
•
•
•
•
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 linear type is usually seen on the neck or face. Lesions consist of
multiple pink to red to brown, firm papules or nodules, 1-10 mm of diameter,
that occur in groups.
The solitary nodular form shows predilection for the trunk, especially for the
shoulders, axillae and the genital area
The plaque type most often presents as a hairless area in the scalp. At
puberty it may become larger, nodular, verrucous or crusted, resembling
nevus sebaceous. Such a lesion often develops in association with a
sebaceous nevus of Jadassohn or may appear de novo without preexisting
lesion.
Association with hamartomas of follicular or sebaceous gland is common.
In about one-third of 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.
Conclusions
•
•
•
•
Syringocystadenoma papilliferum is a rare benign adnexal skin tumor of
apocrine or eccrine glands.
Syringocystadenoma papilliferum is commonly located on the head (scalp
or face ) and neck region.
It is frequently seen in association with other benign adnexal lesions.
Transition of SP to basal cell carcinoma and, rarely, to metastatic
adenocarcinoma may occur..
Our observation of syringocystadenoma arising within sebaceous nevus is
important because its rarity