Page 42 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 2-15 Integumentary System

Hidradenoma papilliferum is most frequently
located on the external genitalia of women.

HIDRADENOMA PAPILLIFERUM

Hidradenoma papilliferum is a rare benign tumor of the
genital and perianal regions. It is most commonly
located on the vulva, although extragenital locations
have been described. It has a predilection for women in
the fourth and fifth decades of life. Typically, these are
small tumors a few millimeters in diameter, but some
large tumors have been described. There is no connec-
tion to the overlying epidermis or mucosa. Syringocystadenoma papilliferum arising within
Clinical Findings: Hidradenoma papilliferum is an a nevus sebaceous. Transformation of a nevus
extremely rare benign tumor located in the dermis. It sebaceous into various tumors, including syringo-
seen almost exclusively in middle-aged women. The cystadenoma papilliferum and basal cell carcinoma,
lesions are almost always located in the genital region. occurs most frequently after puberty.
They typically manifest as asymptomatic nodules that
are discovered incidentally. There are usually no over-
lying epidermal changes, and the tumor is well circum-
scribed, freely movable, and firm in consistency. They
do not have a connection with the overlying epithelium.
In rare instances, they can be tender or pruritic and can
bleed or ulcerate. Most of these tumors are found on
routine gynecological examination. The most common
location is the labia majora. The differential diagnosis
of a solitary, firm dermal nodule in the genital region
is very broad, and a biopsy for histopathological exami-
nation is required in all cases to make the diagnosis. It
is essential for dermatologists and gynecologists to be
aware of this tumor and the common locations in which
it is found.
Pathogenesis: Hidradenoma papilliferum is a tumor
that is believed to be derived from apocrine tissue. For
this reason, it is considered to be a type of apocrine
adenoma. Apocrine glands are found in higher density
in the anogenital region, and that may be one reason
for the unequal cutaneous distribution of this tumor.
The tumor is benign and is closely related to another
benign adnexal tumor, the syringocystadenoma papil-
liferum. The latter tumor is more common on the head
and neck, with a predilection for the scalp. Histologi- Low power. Symmetrically arranged dermal High power. Close-up of the papillary projections.
cally, these two tumors are almost identical, with the tumor, with multiple papillary projections. The projections are lined by cells with an apocrine
major differentiating factor being that the syringocyst- origin. Apocrine secretion (decapitation secretion) is
adenoma papilliferum has a connection to the overlying often noted in various sections of the tumor.
epidermis. Clinically, the syringocystadenoma papil-
liferum usually manifest as an ulcerated papule or
plaque. Both of these tumors can develop within a nevus
sebaceus.
Histology: Hidradenoma papilliferum is a well- an apocrine origin that have a columnar configuration. infiltrate and has an attachment to the overlying epi-
circumscribed dermal tumor. It almost never has any Apocrine secretion (decapitation secretion) is often dermis, which usually manifests as an invagination of
overlying epithelial abnormalities. The syringocystad- noted in various sections of the tumor. There is also a the epidermis into the tumor lobule.
enoma papilliferum, on other hand, has a connection thin layer of myoepithelial cells. Within the papillary Treatment: A complete excision is diagnostic and
with the overlying epidermis. They both commonly projections is a background stroma composed of many curative at the same time. Often, a biopsy is performed
arise in conjunction with a nevus sebaceus. On closer vascular spaces and lymphocytes. to ascertain the diagnosis, followed by the curative
inspection, the hidradenoma papilliferum is composed Syringocystadenoma papilliferum has almost identi- complete excision. These are rare and benign tumors.
of vascular papillary projections into the center of the cal central characteristics. Compared with the hidrad- There have been reports of malignant degeneration,
tumor lobule. These projections are lined by cells with enoma papilliferum, it has a more dense plasma cell but this is exceedingly rare.

28 THE NETTER COLLECTION OF MEDICAL ILLUSTRATIONS