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Adnexal Carcinomas

Cutaneous adnexal carcinomas are a rare group of skin cancers that develop from the skin’s specialized structures—such as hair follicles, sweat glands, and oil (sebaceous) glands. This type of skin cancer is especially rare - affecting fewer than 6 out of every 1 million people in the U.S. each year. In this guide, we’ll explore the types, signs, risk factors, and treatment options for cutaneous adnexal tumors, along with the genetic conditions that can increase risk.

What are the types of Cutaneous Adnexal Carcinomas?

  • Sebaceous carcinoma

  • Eccrine and apocrine carcinomas: Arise from sweat glands, and include porocarcinoma, mucinous carcinoma, and digital papillary adenocarcinoma.

  • Hair follicle–related tumors: Such as trichilemmal carcinoma, trichoblastic carcinoma, and pilomatrix carcinoma.

  • Microcystic adnexal carcinoma (MAC) 

  • Rare or emerging types: Including NUT carcinoma, cutaneous secretory carcinoma, and endocrine mucin-producing sweat gland carcinoma.

  • Extramammary Paget disease (EMPD) 

What Do Cutaneous Adnexal Tumors Look Like?

Cutaneous adnexal tumors usually appear as pink, skin-colored, or slightly bluish bumps (papules or nodules). These tumors can range in size from small spots to larger growths. Some adnexal tumors may also be dark in color and can resemble moles or other types of skin cancer. The location of these tumors often depends on the type:

  • Sebaceous (oil) and hair follicle tumors are most common on the head and neck.

  • Sweat gland tumors can appear anywhere, but are often found on the palms and soles.

  • Apocrine tumors tend to develop in areas with more sweat glands, like the armpits, genitals, or eyelids.

If you see something new, changing or unusual on your skin, schedule an appointment with your dermatologist.

How Are Cutaneous Adnexal Tumors Diagnosed?

Cutaneous adnexal tumors are often diagnosed through a combination of physical examination and biopsy. During the physical exam, a healthcare provider will check the size, shape, and location of the growth. If the tumor appears suspicious, a skin biopsy may be performed. This involves removing a small sample of tissue to be examined under a microscope to confirm whether it's cancerous. In some cases, additional tests like imaging scans may be used to see if the tumor has spread to other areas.  

Treatment Options for Cutaneous Adnexal Carcinomas

Treatment for cutaneous adnexal tumors often involves surgical removal of the tumor. Depending on the tumor’s size, location, and whether it has spread, other treatments may be needed:

  • Mohs surgery 

  • Radiation therapy 

  • Chemotherapy or targeted therapy 

The choice of treatment will depend on the type of tumor, its stage, and your overall health. Early diagnosis and treatment are important for the best outcome.

Genetic Conditions Linked to Cutaneous Adnexal Tumors

In some cases, cutaneous adnexal tumors are linked to genetic syndromes that can run in families. If you have a family history of certain conditions, it may increase your risk of developing these tumors. Some of the most common genetic conditions associated with adnexal tumors include:

  • Muir-Torre Syndrome 

  • Brooke-Spiegler Syndrome 

  • Cowden Syndrome

  • Birt-Hogg-Dubé Syndrome 

Key Takeaways

  • Cutaneous adnexal tumors develop from structures in the skin, like hair follicles, sebaceous glands, sweat glands, or apocrine glands. 

  • These tumors often appear as pink, skin-colored, or slightly bluish bumps, and they can range in size. They are typically slow-growing, but cancerous tumors may grow more quickly and even ulcerate. They often appear on the head and neck but can develop in other areas depending on the type.

  • Biopsy is necessary to diagnose adnexal tumors, as they can look similar to other skin conditions. Additional tests may be required for more accurate identification.

  • Mohs micrographic surgery or wide local excision 

  • Certain genetic conditions like Muir-Torre syndrome, Brooke-Spiegler syndrome, Cowden syndrome, and Birt-Hogg-Dubé syndrome are linked to a higher risk of developing these tumors. If you have a personal or family history of any of these conditions, be sure to discuss it with your healthcare provider.

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