Syringocystadenoma Papilliferum

Path Quiz Case- 31

Dr Sampurna Roy MD

Path Quiz Online: Case history and images:

http://www.histopathology-india.net/SAClass.htm

January 2008

Benign sweat gland tumours

Cysts:

Hydrocystoma -Eccrine/Apocrine

Hamartomas:

Eccrine/Apocrine naevus

Eccrine Angiomatous Hamartoma

Porokeratotic eccrine ostial naevus

Benign Neoplasms:

Syringocystadenoma Papilliferum

Hidradenoma Papilliferum

Chondroid Syringoma (benign mixed tumour)

Poroma

Hidradenoma

Apocrine adenoma

Papillary eccrine adenoma

Syringofibroadenoma

Malignant sweat gland tumour

Low grade:

Microcystic adnexal carcinoma

Eccrine epithelioma

Adenoid cystic carcinoma

Mucinous carcinoma

Primary extramammary Paget’s disease

Intermediate grade:

Porocarcinoma

Digital papillary adenocarcinoma

High grade:

Hidradenocarcinoma

Apocrine adenocarcinoma

Eccrine ductal carcinoma

Spiradenocarcinoma

Malignant cylindroma

Sebaceous tumours
Fordyce's Spots

Steatocystoma

Nevus Sebaceous

Folliculosebaceous Cystic Hamartoma

Sebaceous Hyperplasia

Sebaceoma

Sebaceous Adenoma

Sebaceous Carcinoma

Epidermal tumours:

Epidermal Naevus ; Inflammatory linear verrucous epidermal nevus

Squamous Cell Carcinoma

Verruciform Xanthoma

Drug related cutaneous lesions

Cutaneous lesion in graft-
versus host disease

Cutaneous lesions after exposure to Radiation

Cutaneous infection and infestations

Histopathological patterns in cutaneous infections

Cutaneous Deposits

Calcinosis Cutis

This adnexal tumour commonly occurs on the scalp and neck. Rarely these tumours are found on the trunk, the lower extremities, ear canal and eyelid.

Visit: Syringocystadenoma Papilliferum of the External Ear

The origin of this tumour is still being debated. The results of light , electron microscopic, immunohistochemical and anatomic studies are conflicting.

Many authors believe that syringocystadenoma papilliferum is mainly apocrine derived tumour because of the occasional presence of decapitation secretion in some of the luminal cells of the tumour and the frequent presence of tubular glands with large lumina and decapitation secretion beneath the tumour. In some lesions , where there are no apocrine glands in the dermis, the papilliferous structures represent eccrine proliferation. Most authors agree that this hamartoma develops from undifferentiated pluripotential appendageal cells.

Clinical presentation: Clinically, most of the cases are first noted at birth. Other cases develop in infancy , childhood and adolescence . The tumour presents either as one papule or several papules in a linear arrangement, or as a solitary plaque. Some papular lesion may be umbilicated and resemble molluscum contagiosum. During evolution of lesion verrucous changes can develop at puberty. The plaque type lesion is similar to nevus sebaceous.

Microscopic features:

Image Link1 ; Image Link2 ; Image Link3 ;Image Link4.

Epidermis is acanthotic and shows papillomatosis.
A cystic invagination extends downward from the epidermis. Numerous villous projections are seen within these invaginations. These papillary projection are lined by two layers of epithelial cells, a columnar luminal cell layer and an outer layer of small cuboidal cells. Occasionally, the luminal row of cells show decapitation secretion.
In the deep dermis, groups of apocrine glands may be present. Close step sections should be carried out, to demonstrate connection of the apocrine glands with cystic invagination in the upper dermis.
The stroma is usually infiltrated by a dense mononuclear infiltrate composed entirely of plasma cells.
Malformed sebaceous glands and hair structures may be present.
Syringocystadenoma papilliferum may be associated with nevus sebaceous and basal cell carcinoma and rarely with sebaceous epithelioma, trichoepithelioma, eccrine spiradenoma and apocrine hydrocystoma.

Syringocystadenocarcinoma papilliferum:

Syringocystadenocarcinoma papilliferum is a distinct dermatologic entity. This exceedingly rare neoplasm, most examples of which seem to have arisen in its benign counterpart, syringocystadenoma papilliferum.

Histologically, this malignant tumour shows in-situ adenocarcinoma with cytological atypia and pagetoid spread into surrounding epithelia. The tumour lacks typical double layered pattern. Decapitation secretion is present.

Immunohistochemistry shows immunoreactivity to EMA and human milk fat globules. Syringocystadenocarcinoma papilliferum: case report and immunohistochemical comparison with its benign counterpart.J Am Acad Dermatol. 2001 Nov;45(5):755-9.

CLINICAL IMAGE: CLICK 1 2 (DermAtlas)

IMAGE LINK: CLICK HERE (W.Weems)