Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

What is Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans?

Dermatofibrosarcoma protuberans (DFSP) is an uncommon skin tumor that can become locally invasive. DFSP starts from the dermal layer of the skin, which is the layer beneath the surface. It generally develops as a slow-growing hardened or elevated area of skin. Interestingly, this tumor frequently shows up on the trunk and upper limbs due to its origin in a specific part of the dermal layer.

DFSP does not commonly spread to other areas of the body (metastasize). However, some can change into a more aggressive form, which can affect how it is diagnosed and treated. When DFSP does spread, it usually follows the pattern of tissue layers, infiltrating nearby structures but staying within tissue boundaries. Though rare, spread to regional lymph nodes or distant sites typically happens when the tumor undergoes an aggressive transformation.

Micrographic dermatologic surgery (MDS), a procedure including methods like Mohs micrographic surgery (MMS), becomes critical in attaining complete tumor removal while preserving normal function and appearance of the skin. This technique has become a crucial tool in managing DFSP. It allows for precise removal of the tumor while making sure healthy tissue is left untouched.

What Causes Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans?

The exact causes of DFSP, a type of skin cancer, are still not completely known. However, both our genes and the environment we live in might have a part in causing it. Scientists have discovered that more than 90% of DFSP cases share a specific genetic abnormality, an unusual swapping of pieces between chromosomes 17 and 22. This results in the rearranging of two genes, named COL1A1 and PDGF-β. PDGF-β, when over-activated, fuels cell growth and the formation of new blood vessels, which together contribute to the development of DFSP.

Besides these genetic changes, environmental factors like sun damage or injuries could also play a role in developing DFSP. Ultraviolet (UV) radiation from the sun can harm our DNA, which may lead to the accumulation of harmful mutations, increasing the risk of DFSP. Furthermore, recurring injuries or scarring have been suggested to initiate DFSP growth in individuals who are genetically predisposed. Yet, how exactly this works is still not fully known.

Risk Factors and Frequency for Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

DFSP, or Dermatofibrosarcoma Protuberans, makes up about 1% of all soft tissue sarcomas in the United States. This equates to an annual rate of about 4.1 cases per million people. Generally, it is diagnosed in adults between the ages of 30 and 50, although it can occur at any age, including in children and elderly adults. The condition does not seem to affect one gender more than the other. However, it has been observed that Black individuals are almost twice as likely to be diagnosed with DFSP than White people. Globally, the rate of DFSP varies in different regions and among various populations. Unfortunately, the worldwide incidence of DFSP is difficult to establish comprehensively due to limited studies and registries.

• DFSP accounts for about 1% of all soft tissue sarcomas in the United States, or approximately 4.1 cases per million people per year.
• It usually affects adults between the ages of 30 and 50 but can occur in individuals of any age.
• No predominant sex has been observed in DFSP cases.
• Black individuals have almost double the incidence of DFSP compared to White individuals.
• The incidence of DFSP varies across different regions and populations worldwide.
• Comprehensive assessment of global DFSP occurrence is difficult due to limited availability of studies and registries.

Signs and Symptoms of Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma Protuberans (DFSP) is a condition that often grows slowly and subtly, making it difficult to diagnose. Patients typically report a painless, hard, thickened patch of skin or a small bump, which can look like other, less serious skin conditions such as dermatofibroma or keloid scars. These bumps or patches are most commonly found on the torso, upper limbs, head, and neck.

The affected area often arises from the middle layer of the skin and can be various colors, including reddish-brown or purple, though the color can differ. The characteristic DFSP bump is firm and tends to stick out above the skin surface, giving it a raised appearance. The bump or patch can have clear or vague borders and may feel rubbery or fibrous when touched. In more advanced cases, DFSP might also show visible blood vessels on the surface or develop into an ulcer (a break in the skin). The size of the DFSP can range anywhere between 0.5 cm to more than 10 cm, but it is typically between 2 to 3.5 cm.

Testing for Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

A definitive diagnosis and appropriate treatment plan for DFSP, a type of skin cancer, typically involves several steps. These include a clinical assessment by a doctor, a thorough examination of tissue under a microscope (histopathological examination), and medical imaging studies such as ultrasound, MRI, or CT scans. It’s recommended that these evaluations follow the National Comprehensive Cancer Network guidelines for DFSP. Where possible, patients should consider consultation at treatment centers that have specific experience with DFSP, especially for cases that are large or have come back after initial treatment.

In a clinical evaluation, the doctor will inspect and feel the area of concern to check its size, location, and texture, and whether it’s causing any symptoms. Complete health records, including family and personal medical history, and a close examination of the patient’s skin help in evaluating anyone with a skin lesion that might be DFSP. A tool known as a dermoscope can help the doctor make a diagnosis by highlighting typical features of DFSP. However, to confirm the diagnosis, a piece of the affected tissue needs to be removed for further examination, which includes a punch, incisional, or core biopsy.

Histopathologic evaluation, referring to examining the tissue sample under a microscope, is an essential step for diagnosing DFSP and distinguishing it from other skin tumors. Experienced pathologists must evaluate tissues that could potentially be DFSP. Typical features of DFSP when seen under a microscope include cells that look like fibroblast cells arranged in a storiform or cartwheel pattern. Additional laboratory tests are used to look for specific proteins (such as CD34) in the cells.

Sometimes, the diagnosis isn’t clear after the initial biopsy, in such cases, re-biopsying the tissue may be needed. Likewise, the tissue may need to be examined for platelet-derived growth factor receptor (PDGFR) changes, typically seen in DFSP. Molecular tests may be required to look for specific genetic changes.

Radiographic imaging, like ultrasonography, MRI, or CT, helps to determine the tumor’s depth, size, and whether it has spread locally. These are especially useful if it’s unclear from previous tests whether it’s DFSP or when planning surgery. MRI is the preferred imaging method to evaluate DFSP because it provides a clear picture of the tumor’s size and its relationship with neighboring tissues and bone. Some patients might also need chest X-rays or CT scans to look for any disease spread, especially those who are at high risk or have symptoms that suggest the cancer may have spread. However, routine imaging to look for disease spread is typically not needed for localized DFSP unless there are symptoms or signs of advanced disease.

Treatment Options for Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

The goal of treating a DSFP (a type of malignant skin tumor) is to completely remove the tumor while minimizing potential side effects and reducing the chances of it coming back. Treatment plans can vary based on the patient’s unique disease characteristics and symptoms, and they may include surgery, medication, or other therapies.

Surgery with a particular technique known as MDS (a method of examining the tissue as it is removed during surgery) is usually the first step. If the tumor can’t be easily removed or is on the border of another structure, doctors may recommend tumor mutation analysis and a medicine called imatinib before surgery. Regular check-ups are important for tumors with negative margins (tumors that had enough surrounding tissue removed), while those with positive margins should be re-operated where possible. When surgery isn’t possible, other interventions such as radiation therapy may be used.

The primary goal of surgical removal of a DSFP tumor is to ensure all cancer cells are removed while maintaining function and appearance as much as possible. Typically, MDS is used, which allows for precise removal of the tumor and preserves healthy tissue.

Interestingly, DSFP can sometimes grow unexpectedly, with cancer cells extending beyond the usual tumor margins. This may result in the tumor not being entirely excised despite using MDS. Moreover, wide undermining (i.e., cutting a large area of skin tissue loosely from the underlying structures) after the surgical extraction is not a good idea as it could potentially spread malignant cells if the tumor is not entirely removed.

Reconstruction of the operated area should be postponed until a comprehensive examination confirms that all the peripheral and deep margins are free of the tumor. However, there is currently no agreement among experts on the best width of margins. Importantly, results have shown a high variability in local recurrence rates depending on the width of the surgical margin. Therefore, it is advisable to delay reconstruction until the margins are confirmed to be negative.

Sometimes a technique called Slow Mohs, which involves embedding the tissue in paraffin, is used for evaluating tumors like DFSP that have high-risk characteristics. It allows for more thorough tumor staging (determination of cancer’s extent), but it requires more time.

As a rule, MMS is suitable for DFSP regardless of its location in the body. In cases where MMS is not practical, the tumor may be removed with a wider margin, ensuring all cancer cells are removed. Despite its high success rate, MMS has some drawbacks. Sometimes, it can be difficult to identify tumor cells, and the technique can be complex and time-consuming. However, if the tumor has invaded deep structures, such as bone, nerves, or blood vessels, MMS may be used along with input from an interdisciplinary team.

If the disease recurs, further surgery is usually needed. When surgery isn’t possible, other therapies like radiation or imatinib therapy may be considered. Patients with metastatic (spread to other parts of the body) lesions should have a discussion with an interdisciplinary team to determine the best personalized treatment plan.

DSFP with fibrosarcomatous differentiation (DFSP-FS) is a more aggressive subtype with higher local recurrence and metastasis rates. These patients may require multiple therapies to achieve tumor clearance. If the disease is not removable or would result in unacceptable functional outcomes, other methods such as radiation, systemic therapy, or even amputation may be required.

Patients with cancer that has spread to multiple parts of the body (stage IV disease) may undergo treatments similar to those for earlier-stage disease. However, they may also consider treatments aimed at the metastases itself, such as removal of metastases, stereotactic body radiation therapy, ablation, embolization, observation, or palliative care.

Auxiliary therapies like radiation and systemic therapy may be considered for DFSP cases with larger tumor size, positive margins, or tumor unresectability. However, more research is needed for an optimized treatment plan and long-term outcomes.

What else can Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans be?

When testing for DFSP, which is a type of skin cancer, doctors need to consider if it could be other conditions. These may include:

• Dermatofibroma (a common skin growth)
• Schwannoma (a tumor that forms on nerves)
• Cutaneous neurofibroma (a skin tumor associated with nerve tissue)
• Solitary fibrous tumor (a rare tumor of the soft tissues)
• Intradermal spindle cell lipoma (a rare fat tissue tumor)
• Spindle cell/desmoplastic melanoma (a type of skin cancer)

Even though these conditions share similarities with DFSP, there are features that can help distinguish between them. For instance, dermatofibroma does not present with CD34 protein, spindle cell lipomas are S100 positive and spindle cell melanoma expresses various proteins.

Accurate diagnosis is critical for proper treatment and to improve patient’s outcome. To achieve this, collaboration among dermatologists, surgical oncologists, and experts in analyzing disease tissues is necessary.

What to expect with Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

The outlook for DFSP, a type of skin cancer, is generally good. It has a low chance of spreading to other areas of the body and a high probability of survival, especially if caught and treated early on. Things such as the tumor size, how deeply it has invaded the skin, whether or not it was entirely removed during surgery, and if it has spread, can all affect the person’s outlook.

When DFSP is removed by surgery and the margins (edges of the tissue removed) are checked, the chances of it not coming back within 5 years and 10 years are 86% and 76%, respectively. However, being male, being of Black race, having the tumor on the head or limbs, larger tumor size, a specific change in the tumor called fibrosarcomatous transformation, cancer in the edges of the removed tissue, and being older each lower these chances.

The disease comes back in 20% to 50% of people, typically between 32 and 38 months after the surgery. Because of this, it’s recommended that these individuals have regular check-ups every 6 to 12 months for an extended period.

Possible Complications When Diagnosed with Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

MMS is a very effective treatment for DFSP, but like every other surgical procedure, it does come with certain risks. These risks can include spreading the tumor cells, delayed healing of the wound, wound separation, infection, and changes in appearance such as visible scars or changes in skin texture. Depending on the size and location of the tumor, MMS might even lead to physical impairment or loss of movement, particularly in areas involving important body structures.

Although the treatment method has successful cure rates, there’s still a chance of the disease coming back locally after MMS surgery. This is more likely in cases where the surgical borders are too close or positive. DFSP may sometimes grow in disconnected areas, with abnormal cells extending beyond the visible tumor borders. This leads to small, hard-to-detect disease spots that can be missed initially. These spots increase the difficulty for MMS, as these missed groups of tumor cells may escape detection and not be completely removed, which could lead to the disease coming back. Uncommon complications include things like nerve damage, bleeding, formation of a blood-filled swelling, and tightening of the scar. Reducing these risks requires careful preoperative examination, precise surgical method, and attentive aftercare. Good communication between the surgical team and the patient is essential to get optimal outcomes and properly address any patient concerns after MMS for DFSP.

Common Side Effects:

• Spreading of tumor cells
• Delayed wound healing
• Wound separation
• Infection
• Visible scars or skin texture changes
• Physical impairment or limited movement
• Reoccurrence of disease
• Nerve damage
• Bleeding
• Formation of a blood filled swelling
• Tightening of scar tissue

Recovery from Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

After going through MMS (a type of skin cancer surgery) for DFSP (a rare type of skin cancer), it’s crucial to take good care of the healed area to ensure it recuperates well, avoid problems, and achieve the best possible functional and aesthetic results. It’s important to keep the surgical area clean, dry and safe from excessive touching or injury. Clear instructions on how to care for the wound, like changing the dressing and observing the wound, are given, and frequent follow-up appointments should be made to check how well the wound is healing and to look out for any signs of infection or other complications.

The size and position of the tumor may require patients to undertake physical therapy or do some rehabilitation exercises to recover movement, strength, and normal function in affected areas. Different exercises like early movement and gentle stretches may reduce stiffness and help tissue heal faster. This is particularly helpful when DFSP involves critical body structures or results in a large amount of tissue being removed.

After having MMS for DFSP, any worries about the appearance of the wound should be managed through suitable wound handling and scar treatment techniques. Patients are given advice on different techniques like scar massages, applying silicone gels, and adopting sun protection measures to decrease scar enlargement, changes in skin color, and other aesthetic concerns. Long-term follow-up care is crucial to check for signs of the cancer coming back, assess functional and aesthetic results, and manage any problems or complications. Patients are advised to undergo regular skin checks every 6 to 12 months to catch any recurrence or new skin issues early.

Preventing Mohs Micrographic Surgery Evaluation and Treatment of Dermatofibrosarcoma Protuberans

Teaching patients about their medical conditions is essential, especially when dealing with DFSP, a kind of skin cancer. In this case, patients need to be well-informed about the early warning signs and typical symptoms. This will help with recognizing the disease early on and will underline the importance of seeking medical help when they spot a suspicious skin growth or lesion.

Patients also need to be familiar with what puts them at risk for DFSP; for example, having had an injury or surgical scars. It becomes quite important for patients to regularly check their skin themselves to watch if any skin changes or new growths are happening.

Seeking help from professionals who know a lot about skin conditions, such as dermatologists, is essential too. Patients need to understand what options are available for diagnosing and treating DFSP. This might include procedures called MMS and WLE. It’s important that patients are part of deciding what kind of treatment to go with. Also, practicing good habits like wearing sunscreen, covering up in the sun, and staying in the shade when the sun is brightest is important in preventing DFSP and other skin cancers.

Lastly, teaching patients about the need for regular skin check-ups and long-term care after treatment for DFSP is important for spotting any returning cancer and for the best results. Patients, in this case, need to take an active role in managing and monitoring their skin health.