Papillary Hidradenoma: Causes, Symptoms, and Treatment

Introduction

Adenoma is a non-cancerous or benign tumor that can affect any organ. Adenomas begin in the epithelial tissue that covers the organs and glands. Papillary hidradenoma is a rare, benign apocrine gland tumor that typically manifests in females between the ages of 30 and 49. Papillary hidradenoma is a benign papulonodular dermal tumor that usually affects women in the vulva or perianal region. Apocrine glands are a type of exocrine secretory gland. They can be found in various locations, but the axillae, areolae, and anogenital regions are the most common. The typical presentation is a solitary dermal or subcutaneous nodule that is unrelated to the epidermis. These lesions are commonly seen on the vulva.

What Is Hidradenoma?

Hiradenoma is a benign adnexal apical sweat gland tumor. These are translucent blue cystic nodules measuring 1 to 3 cm in diameter. It typically manifests as a single small skin-colored lesion and is thought to be related to or a variant of poromas.

Hiradenoma is classified based on histologic differences, such as:

• Cystic.

• Tubular.

• Papillary.

• Syringocystadenoma papilliferum-like.

What Is Papillary Hidradenaoma?

A papillary hidradenoma, also known as a mammary-like gland adenoma of the vulva or hidradenoma papilliferum is a benign tumor that is most commonly found in females between the anal and genital regions or the anogenital area. It is a rare condition. These hidradenomas are nodules with sharp borders that typically form in the vulva or anogenital region of women. At the same time, they can also rarely occur in other body parts of both sexes.

Ectopic papillary hidradenomas, also known as ectopic hidradenoma papilliferums, are papillary hidradenomas that form outside of the anogenital area. Hidradenoma papilliferum appears as a flesh-colored nodule in the anogenital region (between the anal and genital areas) of women. Ectopic hidradenoma or ectopic papillary hidradenomas are paillary hidradenomas that form outside of the anogenital region.

What Causes Papillary Hidradenoma?

The cause of papillary hidradenoma is unknown. Papillary hidradenoma is almost exclusively found in the female anogenital area; however, there have been a few isolated reports of perianal hidradenoma papilliferum in male patients. Hidradenoma papilliferum tumors were previously thought to originate from apocrine sweat glands; however, some authors believe that rudimentary glandular structures, eccrine sweat glands, or ectopic vulvar breast tissue could also be a possible source of the tumor. In addition, mammary-like glands have been found in non-anogenital areas such as the ears, face, chest, and scalp, and hidradenoma papilliferum tumors are rare in nonanogenital areas. Therefore, they are known as ectopic hidradenoma papilliferum.

What Are the Symptoms of Papillary Hidradenoma?

Papillary hidradenoma is almost exclusively found in middle-aged women and manifests as a solitary, circumscribed nodule with a diameter of less than 1 cm that may resemble hemorrhoids. Papillary hidradenomas are small, slow-growing tumors that may have been present for months or years before diagnosis. The tumors are usually asymptomatic but can cause intermittent or constant pain, ulceration, and minor bleeding. The pain caused by these tumors can range from simple tenderness to more or less constant, mild pain. In females with papillary hidradenoma, pain during and immediately following sexual intercourse (dyspareunia) is also seen.

What Are the Histological Features of Papillary Hidradenoma?

A papillary hidradenoma is a well-circumscribed dermal nodule that is usually not connected to the epidermis. A double-layered epithelium lines tubules and broad, elongated fronds, forming an arborizing pattern. The inner layer comprises cuboidal myoepithelial cells, while the outer layer comprises tall columnar apocrine cells with pale eosinophilic cytoplasm and decapitation secretion. The fibrous core of larger fronds is possible. A pseudo capsule is formed when the fibrous tissue surrounding the tumor is compressed.

How Is Papillary Hidradenoma Diagnosed?

• Anogenital papillary hidradenoma lesions are frequently diagnosed based on clinical and histologic features. Histology and surgical excision are required for diagnosis. Hidradenoma papilliferum is a well-circumscribed dermal nodule that is usually unconnected to the epidermis.

• The lesions are cystic papillary tumors of papillary fronds and loose fibrous connective tissue lined with a double-layered epithelium. The inner layer is made up of cuboidal myoepithelial cells. In contrast, the outer layer comprises tall columnar apocrine cells with pale eosinophilic cytoplasm that may show decapitation secretion. A fibrous core may be present in larger fronds. A pseudo capsule can form when fibrous tissue surrounding the tumor is compressed. These histologic features can be used for the diagnosis of papillary hidradenoma.

• Benign papillary hidradenoma tumors can be clinically confused with cancerous tumors, and recent studies have shown that other types of tumors are frequently misdiagnosed as papillary hidradenomas. More research is needed to better define the criteria for diagnosing papillary hidradenomas, particularly ectopic papillary hidradenomas.

How Is Papillary Hidradenoma Treated?

Surgical excision of the tumor while preserving surrounding structures is the preferred treatment, regardless of location or size. The lesion is curative, and recurrence is only reported when the primary tumor is not completely removed.

What Is the Prognosis of Papillary Hidradenoma?

The prognosis is favorable for both anogenital and ectopic papillary hidradenomas. Hidradenoma papilliferum rarely becomes malignant, but a few cases have been documented.

What Are the Complications of Papillary Hidradenoma?

• Papillary hidradenoma is a non cancerous lesion. However, in rare cases, it may progress into malignancies that did not spread or invade beyond the layer of tissue where they first appeared.

• It may sometimes progress to a locally invasive malignancy (very rare).

• Papillary hidradenoma can progress into complex tumors mixed with other cancers such as melanoma, extramammary Paget disease, and squamous cell carcinoma.

What Is the Differential Diagnosis of Papillary Hidradenoma?

• Adenocarcinoma - Papillary hidradenoma may be misdiagnosed as adenocarcinoma. However, adenocarcinoma shows an infiltrative growth pattern, marked nuclear atypia, and lacks a myoepithelial layer.

• Malignant Intraductal Carcinoma - These lesions can be differentiated based on their extensive growth pattern, nuclear polymorphism, and prominent mitotic activities.

• Syringocystadenoma Papilliferum - It is an apocrine or eccrine benign adnexal skin tumor with distinct histological features. This crusted lesion is distinguished by continuity with the surface epithelium, and a prominent plasma cell infiltrates the fibrous cores.

Conclusion

Papillary hidradenoma is a non-cancerous, slow-growing tumor that affects the anogenital areas of women. Lesions are usually asymptomatic. However, if the lesion is ulcerated, it may cause pain, itching, bleeding, or discharge. Local excision is the treatment of choice, regardless of the site. Recurrence of anogenital and ectopic papillary hidradenoma is unusual and is usually due to incomplete excision of the primary tumor.