Brooke-Spiegler syndrome

Brooke-Spiegler syndrome (BRSS or BSS), also known as CYLD cutaneous syndrome, Ancell-Spiegler cylindromas or Spiegler-Brooke syndrome, is a rare genetic skin condition that causes tumors derived from skin appendages (hair follicle tumors and sweat gland tumors) often on your face, neck and scalp ¹, ², ³, ⁴, ⁵, ⁶, ⁷, ⁸, ⁹, ¹⁰, ¹¹, ¹². The hair follicle tumors and sweat gland tumors may also grow on other parts of your body. Brooke–Spiegler syndrome (BSS) is characterized by the growth of cylindromas, spiradenomas, trichoepitheliomas, or their combination. Brooke-Spiegler syndrome (BSS) includes familial cylindromatosis and multiple familial trichoepitheliomas (MFT). The tumors often appear in your teens and early 20s. Brooke-Spiegler syndrome tumors are usually noncancerous (benign), though they sometimes become cancerous (malignant) over time ¹³. Although malignant transformation is rare, it should be considered in cases of rapid enlargement and bleeding ⁴. Large, noncancerous tumors may become open wounds and at risk of infection.

Brooke-Spiegler syndrome is an autosomal dominant disorder. This means that one of your parents is a carrier of Brooke-Spiegler syndrome and will have symptoms of the condition. You usually inherit Brooke-Spiegler syndrome from your biological parents (a genetic condition). As their child, there’s a 50% chance that you’ll also have Brooke-Spiegler syndrome. But sometimes, people with Brooke-Spiegler syndrome have no family history of the condition.

In order to diagnose Brooke-Spiegler syndrome, your doctor will first ask about your symptoms, medical history and family medical history. He/she will also do a physical exam. Brooke-Spiegler syndrome may be suspected if you have a family history of multiple benign skin tumors. Other skin tumor such as cylindromas, trichoepitheliomas and spiradenomas are difficult to distinguish clinically. Therefore skin biopsy is required to confirm the diagnosis. During a skin biopsy your doctor will remove a sample of cells within a tumor. Then, they send the sample to a laboratory for examination under a microscope. Your doctor may also suggest genetic testing. This test can find changes in your genes that cause Brooke-Spiegler syndrome.

Unfortunately, there’s no cure for Brooke-Spiegler syndrome. Although Brooke-Spiegler syndrome doesn’t go away, surgery and other treatments can help. With proper treatment, most people with Brooke-Spiegler syndrome can continue to lead full and active lives.

Figure 1. Brooke-Spiegler syndrome

Footnotes: A 52-year-old woman presented to the skin clinic with multiple firm, rubbery, flesh-colored papules localized to the face. Biopsy confirmed trichoepitheliomas. Because of clinical and histological manifestations, the patient was suspected to have Brooke-Spiegler syndrome. Patient stated that she had had these lesions since she was a teenager and had had them biopsied in the past, with some shown to be basal cell carcinomas (BCCs).

[Source ¹ ]

Figure 2. Brooke-Spiegler syndrome scalp

Footnotes: Multiple lesions on the scalp in the patient with Brooke-Spiegler syndrome. Histologically, the lesions were spiradenomas, cylindromas, and spiradenocylindromas

[Source ⁵ ]

Figure 3. Brooke-Spiegler syndrome malignant transformation

Footnotes: Large ulcerated tumor in a patient with Brooke-Spiegler syndrome. The patient died of multiple metastases.

[Source ⁵ ]

How common is Brooke-Spiegler syndrome?

Brooke-Spiegler syndrome is a rare condition. Researchers estimate that Brooke-Spiegler syndrome affects roughly 1 out of every 100,000 people. The estimated incidence of Brooke–Spiegler syndrome (BSS) is approximately 1 in 100,000 in the UK ¹⁴.

Who might have Brooke-Spiegler syndrome?

People of every age, race and sex can have Brooke-Spiegler syndrome. But you’re more at risk of Brooke-Spiegler syndrome if one of your parents carries the genetic mutation for Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome is an autosomal dominant disorder. This means that one of your parents is a carrier of Brooke-Spiegler syndrome and will have symptoms of the condition. You usually inherit Brooke-Spiegler syndrome from your biological parents (a genetic condition). As their child, there’s a 50% chance that you’ll also have Brooke-Spiegler syndrome. But sometimes, people with Brooke-Spiegler syndrome have no family history of the condition.

Most people first show Brooke-Spiegler syndrome symptoms as teenagers or young adults. The number of tumors often starts to increase when you’re in your 30s or 40s ⁴. Females often have more tumors than males.

How can I reduce my risk of Brooke-Spiegler syndrome?

Unfortunately, you can’t reduce your risk of Brooke-Spiegler syndrome. But treatment can help reduce the number and size of tumors you have.

How does Brooke-Spiegler syndrome differ from neurofibromatosis?

Both Brooke-Spiegler syndrome and neurofibromatosis are genetic conditions that cause primarily benign tumors. But Brooke-Spiegler syndrome causes tumors mainly on your skin, while neurofibromatosis causes tumors both on and under your skin and along your nerves.

There are 3 types of neurofibromatosis:

• Neurofibromatosis type 1 (NF1), where tumors (neurofibromas) form on or under your skin.
• Neurofibromatosis type 2 (NF2), where tumors (schwannomas) form on the nerves of your brain and spinal cord (peripheral nervous system). These tumors often affect the nerves that control hearing (auditory nerves).
• Schwannomatosis, where schwannomas form on nerves in your peripheral nervous system. These tumors usually don’t affect your auditory nerves.

Brooke-Spiegler syndrome cause

Genetic mutations in the CYLD gene on chromosome 16q12 cause Brooke-Spiegler syndrome ¹⁵, ⁶. The CYLD gene provides instructions for making CYLD enzyme called CYLD lysine 63 deubiquitinase that helps regulate numerous signaling pathways, many of which are involved in cell growth ¹⁵. These pathways include nuclear factor-kappa-B (NF-KB), Wnt, c-Jun N-terminal kinase (JNK), transforming growth factor beta-1 (TGFB1), and Notch signaling pathways ¹⁵. By regulating these signaling pathways, the CYLD lysine 63 deubiquitinase enzyme helps cells respond properly to signals that promote cell growth and division (proliferation) or self-destruction (apoptosis), as necessary. By regulating signals that control cell growth, the CYLD lysine 63 deubiquitinase (CYLD) enzyme acts as a tumor suppressor, which means that it helps prevent cells from growing and dividing too fast or in an uncontrolled way.

Brooke-Spiegler syndrome has an autosomal dominant inheritance pattern. This means that one of your parents is a carrier of Brooke-Spiegler syndrome and will have symptoms of the condition. As their child, there’s a 50% chance that you’ll also have Brooke-Spiegler syndrome.

In rare cases, CYLD gene mutation occurs spontaneously after conception known as de novo mutation without any family history of Brooke-Spiegler syndrome. The CYLD gene mutation may only be present on certain parts of your body, causing segmental skin tumors.

To date, a total of 51 germline CYLD mutations have been found. A wide range of ethnic and racial backgrounds have been reported in affected patients and families ⁶.

Figure 4. Brooke-Spiegler syndrome autosomal dominant inheritance pattern

People with specific questions about genetic risks or genetic testing for themselves or family members should speak with a genetics professional.

Resources for locating a genetics professional in your community are available online:

• The National Society of Genetic Counselors (https://findageneticcounselor.nsgc.org) offers a searchable directory of genetic counselors in the United States and Canada. You can search by location, name, area of practice/specialization, and/or ZIP Code.
• The American Board of Genetic Counseling (https://abgc.learningbuilder.com/Search/Public/MemberRole/Verification) provides a searchable directory of certified genetic counselors worldwide. You can search by practice area, name, organization, or location.
• The Canadian Association of Genetic Counselors (https://www.cagc-accg.ca/index.php?page=225) has a searchable directory of genetic counselors in Canada. You can search by name, distance from an address, province, or services.
• The American College of Medical Genetics and Genomics (https://www.acmg.net/ACMG/Directories.aspx) has a searchable database of medical genetics clinic services in the United States.

Brooke-Spiegler syndrome signs and symptoms

The main symptoms of Brooke-Spiegler syndrome are tumors (hair follicle tumors and sweat gland tumors) that grow on your skin. The hair follicle tumors and sweat gland tumors usually first appear on your face, neck and scalp. The tumors are round and most often between 0.5 and 3 centimeters (cm), but there are also larger lesions that can achieve up to 7 cm in diameter, resulting in major aesthetic concerns for the individual ¹². The tumors often appear in your teens and early 20s. You usually get more tumors over time. The number of lesions can vary from individual to individual ; some may develop only one to a few, while others may exhibit the emergence of hundreds of nodules all over their body.

Clinically, Brooke-Spiegler syndrome is characterized by the development of cylindromas, trichoepitheliomas, spiradenomas, and spiradenocylindromas on the skin ¹². Trichoepitheliomas (Multiple Familial Trichoepitheliomas, or MTFs) are the only manifestation for some patients, while others have cylindromas (familial cylindromatosis) ¹². Despite their historical distinction as different conditions, these syndromes are now recognized as variations of Brooke-Spiegler syndrome because of potential clinical and histological overlap under the definition of CYLD cutaneous syndrome ¹⁴, ⁵.

Brooke-Spiegler syndrome tumors may also affect other parts of your body, such as your:

• Airways, which can affect breathing.
• Ears, which can impact hearing.
• Eyes, which can affect vision.
• Genitals, which can impact sexual function.
• Mouth and salivary glands, which can impact your ability to chew and swallow.
• Nose, which can affect your sense of smell.

The skin tumors may also grow larger and can be disfiguring. And having skin tumors, especially on your face and head, can also be extremely distressing. The tumors are often painful and if they’re on your skin of your genitals, may cause sexual dysfunction. Sometimes, the skin tumors cover your entire scalp and can cause hair loss. These symptoms can lead to depression.

You may also have small, white bumps on your skin (milia). Milia are tiny cysts filled with keratin that appear on or just under your skin. Milia are often found on your face, particularly around the eyes and cheeks, but can also appear on other parts of your body. Milia are harmless but can be upsetting to have.

Brooke-Spiegler syndrome is also infrequently associated with salivary and parotid gland tumors ⁶.

Brooke-Spiegler syndrome individuals typically present in late childhood to early adulthood with multiple papules and nodules on the scalp, face and neck. They develop increasing numbers of lesions over time. However, there is a wide variability of presentation within and between families with Brooke-Spiegler syndrome ⁶.

Brooke-Spiegler syndrome skin tumors

Brooke-Spiegler syndrome includes 3 main types of skin tumors ⁷. Some Brooke-Spiegler syndrome tumors may be combinations of two or more of these types ⁷.

Although the Brooke-Spiegler syndrome tumors are usually considered harmless, there are reports of malignant transformation.

• Spiradenoma may transform into spiradenocarcinoma.
• Cylindroma may transform into cylindrocarcinoma.
• Trichoepithelioma may transform into basal cell carcinoma.

Cylindromas

Cylindroma is a rare, slow-growing, benign (non-cancerous) appendage skin tumor that typically present as a skin colored, reddish or bluish smooth-surfaced dome-shaped nodule(s) that may range in size from a few millimeters to several centimeters ¹⁶. Cylindromas are smooth, pink nodules usually affecting hair follicles on your scalp, head, face and neck. Cylindromas may also be on your face and ears and, rarely, in your lungs. Cylindromas range from several millimeters (mm) to several centimeters. Cylindromas often form a jigsaw puzzle pattern on your scalp. Although cylindromas are typically slow-growing, they are generally benign (non-cancerous). However, exceedingly rare instances of malignant transformation and potential metastases have been reported, especially in conjunction with multiple lesions associated with Brooke-Spiegler syndrome ¹⁶.

Cylindromas are benign but incomplete biopsy/removal may result in local recurrence.

Malignant cylindromas are very rare. Malignant transformation may develop within solitary cylindromas, or they may complicate the multiple variant (more common).

Multiple lesions are disfiguring and require extensive surgical management to restore anatomic appearance. An association with familial autosomal dominant cylindromatosis and basal cell carcinoma has been reported.

For solitary lesions, the treatment of choice is surgical excision ¹⁷. Large lesions should be imaged before planning treatment to determine vascularity and involvement of surrounding tissues, including underlying osseous structures. Pretreatment embolization is vital in patients with large and multiple cylindromas to minimize intraoperative blood loss, as they can be quite vascular lesions.

Other treatments include electrodesiccation/curettage and cryotherapy ¹⁸.

For small cylindromas, the carbon dioxide laser may be used ¹⁹. Retamar et al ²⁰ used carbon dioxide laser to treat facial trichoepitheliomas in 2 patients, with good results.

Multiple cylindromas usually require extensive plastic surgery that may be obviated by progressively excising a group of nodules in multiple procedures.

Figure 5. Cylindroma

Footnotes: The neoplastic nodules are arranged in a jigsaw puzzle and are composed of monomorphic basaloid cells surrounded by eosinophilic basement membrane material. The cells at the periphery show palisading and are darker than the cells located in the central parts of the nodules.

[Source ⁵ ]

Spiradenomas

Spiradenoma is a rare, benign sweat glands tumor that originates in the skin. Spiradenomas are usually small, solitary nodules that can grow to several centimeters in size. Spiradenomas form firm nodules that show up on your scalp, neck and upper body. They are often blue or flesh-colored, but may also be grey, pink, purple, red or yellow, and can be very painful. Spiradenoma skin tumors range from under 1 cm to several centimeters. Spiradenomas are sometimes painful.

Spiradenomas most commonly appear on the head, neck, and trunk, but can also occur in other areas like the breast. While spiradenomas are usually benign, long-standing lesions can sometimes develop into malignant spiradenocarcinomas, especially in people over 50. Spiradenocarcinomas (malignant spiradenocarcinomas) are aggressive and have high metastasis rates and low survival rates.

The standard treatment for spiradenoma is surgical excision. Imaging techniques like CT scans, MRIs, and ultrasounds can also be used to help with treatment.

Figure 6. Spiradenoma

Footnotes: Bland basaloid cells admixed with lymphocytes and droplets of basal membrane material. Focal ductal differentiation can be recognized.

[Source ⁵ ]

Trichoepitheliomas

Trichoepitheliomas are rare, noncancerous tumors that grow from hair follicles on your face or scalp, but can also appear on the trunk, extremities, or vulva. Trichoepitheliomas are usually skin-colored, translucent, and appear as smooth, round bumps or nodules measuring less than 1 cm. Trichoepitheliomas may also be blue, brown, flesh-colored, pink or yellow. Trichoepitheliomas are generally benign, but can rarely develop into basal cell carcinoma

Trichoepitheliomas are usually asymptomatic and don’t itch or ulcerate. They can appear as a single lesion or multiple nodules. Inherited forms of trichoepithelioma appear as multiple lesions. Trichoepitheliomas can be inherited due to mutations in the CYLD gene. And the exact cause of sporadic forms of trichoepithelioma is unknown.

Trichoepitheliomas can be safely removed surgically. Other treatments include dermal abrasion, laser surgery, and curettage. Topical 5% imiquimod cream or retinoic acid may also be used.

Figure 7. Trichoepithelioma

Footnotes: The neoplasm has two components, namely the follicular germinative epithelium and the specific follicular stroma. The epithelial component is composed of bland basaloid cells arranged in a cribriform pattern, whereas the stroma resembles follicular papillae and the perifollicular sheath.

[Source ⁵ ]

Brooke-Spiegler syndrome diagnosis

Your doctor will first ask about your symptoms, medical history and family medical history ²¹. He/she will also do a physical exam. Brooke-Spiegler syndrome may be suspected if you have a family history of multiple benign skin tumors.

Cylindromas, trichoepitheliomas and spiradenomas are difficult to distinguish clinically. Therefore skin biopsy is required to confirm the diagnosis. During a skin biopsy your doctor will remove a sample of cells within a tumor. Then, they send the sample to a laboratory for examination under a microscope.

Your doctor may also suggest genetic testing. This test can find changes in your genes that cause Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome treatment

Brooke-Spiegler syndrome is not curable. Possible treatment options for individual tumors include:

• Excision
• Electrodessication, where an electric current dries out tissue in the tumors.
• Dermabrasion, where a rotating instrument removes the top layer of your skin.
• Erbium:yttrium-aluminum-garnet (Er:YAG) laser
• Carbon dioxide laser resurfacing, where lasers remove thin layers of skin without damaging the surrounding tissue.
• Cryotherapy, where extreme cold freezes tumor tissue.
• Fulguration, where an electric current destroys tumors.
• Hyfrecation, where a small needle with an electrical pulse burns away the tumor.
• Mohs surgery, where your surgeon cuts away the tumor along with a margin of healthy tissue around it for testing.
• Photodynamic therapy, where light activates drugs (photosensitizers) to destroy tumors.
• Radiotherapy.

Your doctor may recommend surgery to remove the tumors. They’ll use a sharp knife (scalpel) to cut out the tumors (surgical excision). Your doctor will try to keep as much of your surrounding skin or scalp as possible.

If you need large areas of scalp removed, your doctor may recommend split-thickness skin grafting. Your surgeon will remove one layer of skin from another part of your body, often your buttocks or upper thigh. They use this skin to cover the wounds and help them heal.

Your doctor may also suggest medications that can help to stop tumors from growing.

Though Brooke-Spiegler syndrome tumors are usually benign, it can be even more upsetting to see them spread and grow as time goes on. Your doctor can suggest surgery to remove these tumors, although they may grow back. Frequent skin exams will help your doctor catch any malignant tumors at an early stage. With treatment, you can return to your day-to-day activities with less worry.

Monitoring

You should have a skin exam at least once a year. Some people with frequently recurring tumors see their doctor every three or four months. You can also do a skin self-exam once a month. Also, make sure to wear sunscreen to help prevent sun-damaged skin and skin cancer.

You should see your doctor immediately if you notice any tumors that:

• Are painful.
• Bleed.
• Change color.
• Grow quickly.
• Look like a wound (ulcerated).

Rapid enlargement associated with ulceration and bleeding should raise suspicion of malignant transformation, an event seen in about 5–10 % of cases ²².

If you’re planning on becoming pregnant, you may want to talk to your doctor about genetic counseling.

People with specific questions about genetic risks or genetic testing for themselves or family members should speak with a genetics professional.

Resources for locating a genetics professional in your community are available online:

• The National Society of Genetic Counselors (https://findageneticcounselor.nsgc.org) offers a searchable directory of genetic counselors in the United States and Canada. You can search by location, name, area of practice/specialization, and/or ZIP Code.
• The American Board of Genetic Counseling (https://abgc.learningbuilder.com/Search/Public/MemberRole/Verification) provides a searchable directory of certified genetic counselors worldwide. You can search by practice area, name, organization, or location.
• The Canadian Association of Genetic Counselors (https://www.cagc-accg.ca/index.php?page=225) has a searchable directory of genetic counselors in Canada. You can search by name, distance from an address, province, or services.
• The American College of Medical Genetics and Genomics (https://www.acmg.net/ACMG/Directories.aspx) has a searchable database of medical genetics clinic services in the United States.

Brooke-Spiegler syndrome prognosis

Brooke-Spiegler syndrome is a lifelong condition. It’s common for the tumors to reoccur. You may need more surgeries or other treatments to remove new tumors. But people with Brooke-Spiegler syndrome can lead full lives.

About 5% to 10% of people with Brooke-Spiegler syndrome develop malignant (cancerous) tumors. People with Brooke-Spiegler syndrome have an increased risk of:

• Adenocarcinoma cancers.
• Basal cell carcinoma (BCC).
• Salivary gland cancer, such as adenoid cystic carcinoma.
• Sarcomatoid carcinoma.
• Squamous cell carcinoma (SCC).

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