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Desmoid Tumors: Advancements and Emerging Therapies
During a recent AJMC Peer Exchange, a panel of experts delved into the clinical characteristics and heterogeneity of desmoid tumors, the importance of a multidisciplinary approach in both community-based and academic settings, and the profound impact of desmoid tumors on patients’ quality of life, addressing physical, functional, and psychosocial consequences. The session was moderated by Ryan Haumschild, PharmD, MS, MBA, director of pharmacy services at Emory Healthcare and Winship Cancer Institute in Atlanta, Georgia.
Clinical Characteristics & Heterogeneity
Desmoid tumors (DTs), also known as aggressive fibromatoses, deep fibromatoses, and desmoid-type fibromatoses, are rare soft-tissue tumors characterized by infiltrative growth.1,2 These are typically heterogeneous and can occur anywhere in the body.1,2 DTs do not metastasize, but they typically recur and can infiltrate nerves, blood vessels, and other critical organs.1,2 They are most commonly localized in the extremities; intra-abdominally or in the abdominal wall; or in the spine, chest wall, head, and neck.1,2 DTs rarely occur in the brain or lungs.3,4
The most common form of DT is sporadic, occurring in 85% to 90% of cases, whereas 10% to 15% of tumors occur in patients with familial adenomatous polyposis (FAP) or Gardner syndrome, a tumor predisposition syndrome.2 DTs were reported to occur in 7.5% to 16% of patients with FAP, up to 1000-fold more than in the general population.5 Molecularly, DTs are associated with alterations in the Wnt/β-catenin pathway.1 Sporadic DT is characterized by somatic point mutations in the CTNNB1 gene, T41A (55%), S45F (35%), and S45P (10%), which lead to nuclear accumulation of β-catenin.1,6 In patients with FAP, DTs harbor germline mutations in APC, which encodes for a protein that regulates β-catenin levels.1
Approximately 70% of individuals with DTs are women, and most are aged 30 to 40 years.1,6 Risk of DT development or progression appears to increase during and after pregnancy; high estrogen, trauma, and prior surgery are other known risk factors.1 Clinical presentation can vary and depends on tumor location.1 Although some patients are completely asymptomatic, in others quality of life, functioning, and movement are severely affected.1,2 Age, sex, and sociodemographic characteristics also affect clinical presentation, patient burden, and disability.1,2 Worse prognosis is associated with being younger than 37 years, having a tumor larger than 7 cm, or having extra-abdominal tumors.1 FAP also may be associated with higher mortality.7
Diagnosis
DTs are diagnosed using MRI, rather than CT, for most patients, although imaging alone is not sufficient for distinguishing DTs from other soft tissue tumors.1 CT is useful for imaging intra-abdominal DTs and associated complications, such as small bowel obstruction. Ultrasound can be used for the initial evaluation of tumors in extremities or the abdominal wall in pregnant patients.1 Additional analysis of a biopsy sample by an expert soft tissue pathologist is required to distinguish DT from other neoplasms such as lymphoma or sarcoma.1 DTs can be misdiagnosed as low-grade sarcomas because of their high tendency to recur locally after excision.8 In addition, DTs of the breast resemble carcinomas both clinically and radiologically.8
Genetic testing can be used to identify the presence of mutated CTNNB1 or APC, considered the hallmarks of DT.1,2 Because mutations in these 2 genes are mutually exclusive, mutational analysis of β-catenin has been proposed as a specific DT diagnostic tool, with wild-type CTNNB1 suggesting FAP.1 Guidelines recommend evaluation for family history of FAP or Gardner syndrome for patients with DTs.8
The morphologic heterogeneity, variable clinical presentation, and low incidence (estimated 3 to 5 cases per million person-years) of DT make diagnosis challenging.2 Misdiagnosis is common and occurs in 30% to 40% of cases, leading to delayed or inappropriate care.1 Initial evaluation by a multidisciplinary team with expertise in the management of DT, including medical oncologists, radiation oncologists, radiologists, pathologists, surgeons, and geneticists, is recommended.1 Since October 2023, location-specific International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) diagnosis codes have been available for DTs (D48.11. Desmoid tumor).9 These codes may help support increased patient identification, improve visibility of the patient journey, and enable health care providers to appropriately document a DT diagnosis.9
Stakeholder Insights
Ravin Ratan, MD, MEd, associate professor in the Department of Sarcoma Medical Oncology at The University of Texas MD Anderson Cancer Center in Houston, said that delayed diagnosis of DTs is common for a variety of reasons, including that the location is less of a guide to this tumor type because they can occur anywhere in the body and because of the sporadic nature of most DTs. In addition, patients with symptoms commonly see community physicians who encounter these tumors very rarely throughout their careers. Patients report nonspecific symptoms such as pain, stiffness, and a palpable mass. “Getting the diagnosis right is extremely important…and knowing what we are treating before we treat it [is] critically important in avoiding exposing our patients to unnecessary and potentially futile and morbid treatment,” Ratan said. He noted that educating surgical oncologists and medical oncologists is key “because very often when a mass is diagnosed, that is where the patients are sent.”
Tony Philip, MD, sarcoma oncologist and director of quality and patient safety for medical oncology at the R.J. Zuckerberg Cancer Center at Northwell Health Cancer Institute in New Hyde Park, New York, mentioned the importance of having skilled surgeons because a larger resection can sometimes increase morbidity.
The development of location-specific ICD-10-CM codes for DTs is a very important advancement in this field, said Mrinal Gounder, MD, a sarcoma oncologist and early drug development specialist at Memorial Sloan Kettering Cancer Center in New York, New York. This development was driven in large part by the Desmoid Tumor Research Foundation (DTRF), a patient advocacy group. Several outstanding questions may be elucidated now that these codes are available: What are the incidence and prevalence of DTs? In what geographical location are they most prevalent? Gounder said the new codes play a big role in understanding the extent of this disease in the US and will help further research. A better sense of where these patients are and finding the right patients at the right time could be helped by these codes. ICD codes are also very helpful for linking to treatment and the potential outcomes of treatments.
“[Previously] we’ve been coding DTs just under sarcomas…[and] losing an enormous amount of information about the patient journey and outcomes of this disease. This really gives us an opportunity to learn more about this disease and to communicate better with payers as well as among...each other,” Gounder said.
“There was a lot of criticism when ICD-10 was on the horizon that we were going to go from the 30,000 codes of ICD-9 to over 100,000 for ICD-10,” said Derek van Amerongen, MD, MS, former vice president and medical director, Humana Health Plans of Ohio & Kentucky. However, he said, “the experience we’re talking about right now with DTs is a great example of why that additional granularity is really important…. Having the correct code is going to make life easier for everyone, including the health care professional, the health care organization, and the payer.” He added that proper codes should be used as early in the process as possible and that the guidance of a professional coder is very important, especially for smaller institutions.
Physicians should avoid treating patients who may have DTs without a tissue diagnosis, Ratan said. He thinks that MRI is the most appropriate way to image DTs outside of intra-abdominal locations, although CT still has a role in diagnosing tumors in the extremities and the trunk wall. Firstly, he said, MRI helps to elucidate to a fuller extent just how infiltrated these tumors are and can help determine whether surgical intervention will be beneficial. Secondly, he said, MRI illustrates some of the heterogeneity in these tumors. “These are often characterized by sort of quiescent scarlike collagenized areas as well as more active cellular areas that may be at higher risk for progression,” Ratan said.
Considering the risk factors associated with the development of DTs, Philip said that unless patients already know their family history, most will not be aware of risk factors. “In some patients, we may see an increased incidence with pregnancy or recent trauma or surgery, and then that’s when they develop these kind of scar tissues that end up being [diagnosed] a desmoid [tumor] when they get fully evaluated. But I think for most patients, there really aren’t risk factors,” he said.
Gounder added that although it may be tempting to make connections between trauma, estrogen, use of oral contraceptives, or pregnancy with the development of DT, it is too simplistic to think that the tumor is caused by a change in hormones alone. “[There may be] a huge change in cytokines and change in a woman’s immunity and immunology that we sometimes forget, and we think it all comes down to estrogen or hormonal changes,” he said.
Ratan said that in patients with FAP and APC mutations, especially in those with a strong family history of DTs or patients who previously had a DT, additional trauma from additional surgeries may contribute to the development of DTs.
Regarding genetic testing and personalized treatment strategies, Gounder said that he thinks the data are confusing and contradictory. Although some studies found that certain mutations in CTNNB1 are associated with a worse prognosis, others did not come to similar conclusions. “So even though we have looked at technology to do very sophisticated genetic testing and to identify the type of mutation that each patient has, to my best knowledge that has not helped with prognosis [or] with the treatment decisions in any of these situations,” Gounder said.
Van Amerongen agreed and emphasized that before genetic testing is undertaken, it is important to consult with a geneticist from a research institution. This is especially relevant for community clinicians, he said. Genetic testing management experts and organizations can help physicians understand which genetic tests out of thousands are going to be impactful in decision-making and which align with National Comprehensive Cancer Network (NCCN) recommendations, he said, and “which [ones] are a fundamental part of how plans approach tumor management, and especially in an area like [DTs]. We want to make sure that genetic testing is done when it’s going to benefit the patient, when it’s going to be a part of the decision-making process, and when it’s going to directly lead to an impact on the treatment algorithm.”
Gounder added that current NCCN guidelines state that if a patient has a new diagnosis of DT, physicians should refer them to get a colonoscopy to rule out FAP. However, if genetic testing can show that this DT was caused by a CTNNB1 mutation, those patients potentially could avoid getting an invasive colonoscopy.
Abdominal wall DTs can be associated with pregnancy, and Philip said that he suspects most obstetricians and gynecologists, as well as general surgeons, may occasionally see a patient with DT but that it is not something on their radar, indicating an educational need. He believes diagnosis and treatment should involve a multidisciplinary team with input from pathology, radiology, and surgical specialties. It is important to make sure that patients are not getting inappropriately treated with unnecessary surgeries, he said. In addition, patients should discuss a watch-and-wait approach with a medical oncologist. “I can imagine in settings where they don’t have a multidisciplinary review that these patients are getting treated in ways that probably don’t go along with current guidelines,” Philip said.
Current Standards of Care and Treatment Landscape
Updated NCCN Clinical Practice Guidelines in Oncology recommend that all patients should be evaluated and treated by a multidisciplinary team with expertise and experience in DTs before the initiation of a treatment plan.8 Initial workup includes histology and pathology, as well as consideration of an evaluation for FAP.8 Appropriate imaging of the primary site with MRI or CT is also recommended before treatment.8 Biopsy should be performed for suspicious masses to confirm the diagnosis but may not be necessary if complete resection is planned.8 Following biopsy, tumors are categorized into those that are at risk of causing imminent complications if they progress, and those that are not at risk.8 For those that are not at risk of complications, active surveillance with imaging using CT or MRI as indicated is recommended.8
Active therapy for progressive, morbid, or symptomatic disease is based on anatomic location. In the case of intra-abdominal/retroperitoneal DTs, the recommended treatment is either surgery (if resectable) or systemic therapy.8 For all other sites (abdominal wall, pelvic, trunk/extremity, head/neck/intrathoracic locations), recommended treatment options are surgery, systemic therapy, ablation procedures, or definitive radiotherapy.8
Preferred systemic therapy options include the recently FDA-approved γ-secretase inhibitor nirogacestat (Ogsiveo; category 1), tyrosine kinase inhibitor sorafenib (Nexavar; category 1), methotrexate and vinorelbine, methotrexate and vinblastine, imatinib (Gleevec), liposomal doxorubicin, doxorubicin with or without dacarbazine, and pazopanib (Votrient).8 Treatment with sulindac or another nonsteroidal anti-inflammatory drug, including celecoxib (Celebrex; for pain), can be useful in certain circumstances.8 NCCN guidelines state that the best treatment of any patient with cancer is in a clinical trial and encourage participation in clinical trials.8
Guidelines for Patient Treatment
Ratan underlined the guidelines’ recommendation to divide DTs into 2 groups: those at risk of causing imminent complications if they progress and those that are not, emphasizing active surveillance as an initial step for the vast majority of patients with DTs. Ratan said, “There [are] increasing amounts of prospective data at this point from multiple studies that demonstrate that many of these patients will not only not progress but may actually regress on their own…if they are continued to be monitored for some time. This minimizes the risk of overtreating patients who wouldn’t otherwise need it.”
He noted that guidelines are not trying to be overprescriptive but are aimed at giving a general recommendation. “We have systemic therapies that are often quite efficacious. We have surgery, which is an option that is becoming less frequently used in most centers but is still on the list in select circumstances. We have local ablative procedures like cryoablation for which there’s an increasing amount of data and those are integrated into this sort of list of options for treatment as well,” Ratan said. He agreed that ensuring patients are seen in a multidisciplinary setting is important.
Van Amerongen talked about the important role of NCCN guidelines, particularly for rare diseases such as DTs, in guiding providers, community and academic physicians, and payers. “The goal of health plans is to support the standard-of-care treatment and to make sure that we are in line with the up-to-date thinking of NCCN and the most current treatment algorithms,” he said.
Gounder said that he uses several criteria when deciding treatment course: “Is the patient [experiencing] the disease symptomatically or in any other way? Is the disease rapidly progressing or is the DT in a tough location that can harm other organs or could lead to more complications in the future?” If at least 1 of these criteria are met, he would offer therapy up front. He agreed that the choice of treatment must be the result of a multidisciplinary conversation and the right choice for the right patient.
In terms of therapy options, Gounder listed local approaches, high-intensity focused ultrasound (HIFU) and transarterial chemoembolization, and systemic therapies, which can be categorized into 4 groups. “Group 1 is the traditional cytotoxic chemotherapies. Group 2 is relatively recent in the past 7 or 8 years, which are tyrosine kinase inhibitors, for example, sorafenib. Group 3 is γ-secretase inhibitors, which have just been approved, and group 4 is agents currently in clinical trials,” he said. He emphasized that “especially for rare diseases, such as [DTs], we always have to consider [whether there are] even better options than what is already available for our patients.”
In terms of similarities and differences between community-based treatment and treatment at academic institutions for DTs, Philip said he believes access to clinical trials was the biggest difference. Larger tertiary centers will likely have multiple trials for DTs, as opposed to a community or academic setting, where there may be 1 trial for DTs, if any. In addition, there may be differences in access to local modalities, including HIFU or ablation, because of the limited number of specialists. Another difference is that although both settings can decide between chemotherapy or γ-secretase inhibitors, the question is whether the community medical oncologist feels comfortable deciding between options. Do they have a personal algorithm, or would they go by NCCN guidelines? Philip said it varies depending on whether the clinician sees and treats only patients with sarcomas or with a variety of diseases.
These tumors are called benign because they don’t metastasize, but there’s nothing benign about most of them, Gounder said. He said he spends a lot of time educating patients on when to scale back and when it’s important to push forward with treatment.
Added Ratan, “One of the satisfying things as a provider is trying to understand what a patient’s priorities are, what their pain points are in terms of [adverse] effects that they are willing to tolerate or not willing to tolerate, and being able to have the tools, which I think we actually do at this point for [DTs], to tailor a plan.” He noted that he tries to understand what is important to the patient in their daily lives when choosing the right therapy.
Van Amerongen said that creating realistic expectations is key. “Work with patients to help them understand the options, help them understand what is reasonable to expect in terms of outcome, and then ideally deliver that,” he said. He believes that with shared decision-making “we will end up getting more successful outcomes because people will be more satisfied and understanding of the outcomes, which may not be the ideal or the perfect outcome.”
Educating patients and providers about DTs is done on a case-by-case basis at his institution, Philip said. “I’ve been fortunate that one of our pathologists, if she gets a desmoid tissue, reaches out to the surgeon and says, ‘Don’t take out more; send it to the medical oncologist,'” he said. There is no need to immediately remove the tumor because there are better therapy options available. He believes that partnering with patient advocacy groups or pharmaceutical companies to educate patients on social media or remote webinars is important.
When it comes to strategies to enhance access to novel therapies for DTs, Ratan said that increasing general awareness is important. He works at a large cancer center that has patients from multiple states. They collaborate with community doctors and oncologists who live and work hundreds of miles away, developing relationships, and educating them about their patients’ disease. He said that recent high-quality treatment data have greatly helped with education. Ratan works closely with a large group of physicians, surgeons, radiation oncologists, pathologists, and medical oncologists who care for patients with connective tissue tumors, and they discuss these patients a couple of times a week. He can refer patients who are eligible for a study that he is involved with at his institution.
Gounder agreed that many novel therapies are part of clinical trials and that educating patients about going to centers of excellence to get access to these drugs is important. He emphasized that the work of patient advocacy groups is critical in many rare diseases. Gounder’s philosophy is that access to a clinical trial should be offered whenever possible. He works with patient advocacy groups to increase awareness of trials.
In terms of recently approved drugs, Gounder said that γ-secretase inhibitors, particularly nirogacestat, are “the talk of the town.” He added that educating other physicians and providers about nirogacestat using Peer Exchange programs is critical to increasing awareness and education.
In terms of value proposition, van Amerongen said that plan sponsors prioritize efficacy when evaluating treatment on a routine basis.He also said that it’s very important to understand any offsets: “If we’re using treatment A, do we not need treatment B or drug C?”
He said he sees value as the clinical benefit combined with the cost. “What plans really want to do is make sure we’re supporting standard of care, make sure we’re offering the options that are medically appropriate, but also being careful to look for opportunities, as the other panelists have said, to avoid unnecessary care, to be conservative when it’s appropriate, and to provide as much insight and transparency around the options as possible,” van Amerongen said.
Patient Impact and Symptom Management
Although risk of mortality is small, DTs are associated with a significant burden of illness.2 Patients may experience difficulties in receiving a timely and accurate diagnosis, a high symptom burden, and decreased quality of life.2 Up to 63% of patients with DTs experience chronic pain, which leads to sleep disturbance in 73%, irritability in 46%, and anxiety or depression in 15%.2 In addition to pain, patients may experience limited function and mobility, fatigue, muscle weakness, and swelling around the tumor.2 There is a high unmet need for treatments that specifically target DT and improve quality of life.2
DTs typically affect patients in their 20s to 40s and therefore affect relationships, social roles, functioning, work and employment, and finances.2,3 Loss of work productivity, treatment costs, and travel for medical visits may result in financial hardship.2 Persistent depression and anxiety are common among patients with DTs, and rates are comparable to patients with malignant sarcoma and twice as high among patients with abdominal wall DTs.10
Symptoms That Affect Quality of Life
“I think only in the very recent years [have we started] to address [quality of life] with patients,” Gounder said. “This is a topic that…should be front and center and should be a recurrent conversation with patients.” Philip added that he thinks that quality of life is also a big indicator of how well treatment is working, regardless of tumor reduction.
Starting with the assumption being diagnosed with DTs is highly significant and psychosocially distressing for patients is key, Ratan said. These are very often healthy patients who don’t have other medical issues, but they are seen by an oncologist, and they are in the waiting room with other patients, some of whom have “true cancers.” This can cause a lot of distress. He said it is important to acknowledge the existence of the tumor itself, which, independent of physical symptoms, can be challenging for a patient. Physicians should then discuss the problems the tumor can cause, including mentioning that these often get better on their own and asking whether the patient is comfortable with watching and waiting. Next should be a discussion of what this would look like, explaining what treatment would look like and how it might make them feel. In addition, a discussion of how to manage financial worries and missed work is important. Given the age group, the ability to work will be of prime importance, as well as access to therapies and out-of-pocket costs, which can be complicated.
Philip agreed, adding that support groups and patient advocacy forums can help connect them with other patients. “I think within our system we try to offer psychology referrals or psychiatrists needed, but for the amount that’s needed for most large cancer centers and cancer programs, it still isn’t enough and probably needs to be augmented in some way,” he said.
In terms of pain management for patients with DTs, Ratan said, “These are mostly active young adults who may be playing with their children, going to yoga class, working in very physical jobs, and will be affected by pain in these activities.” He believes pain to be the primary issue and that it may not be treated well by various therapies.
“For patients with abdominal desmoids who are having stiffness in the abdominal wall, resection of that tumor doesn’t always make the pain go away. [With radiotherapy,] pain does often get better, but it takes a long time. One of the things that we’ve seen with some of our newer systemic agents, more than 1 of them, is demonstrable improvements in pain, which is more rapid with some than with others. That is a key part of the conversation that I have with patients, that 1 reason to treat [patients is for] symptom relief independent of the shrinkage of the tumor or making the tumor go away,” Ratan said.
Gounder added that pain has many downstream issues, such as leading to poor sleep and fatigue; it also can lead to dependency on pain medication and can result in depression. He said he would refer a patient who has a significant amount of pain to a pain clinic.
Regarding the active surveillance recommendation, Gounder and Philip emphasized this requires a lot of education, because for most patients the moment they receive a diagnosis, the first thought that comes to them is to get the tumor removed. It’s also about managing the inherent anxiety experienced by patients, families, and caretakers. Although there will be a subset of patients who are happy with this approach, for others wait and watch also becomes “wait and worry.”
“Sometimes it requires people to come back multiple times to hear the same information again and again and to reassure them,” Gounder said. Ratan added that it “often requires a discussion of goal setting, of saying, ‘My goal is for you to live your life as normally as possible.’… I’ve found over time that that approach allows patients to feel like they’re being cared for, even as we’re sort of emphasizing surveillance as the upfront approach.”
Balancing conservative vs aggressive treatment “requires an understanding of the patient’s priorities, engaging in informed consent and shared decision-making, [and] transparency around outcomes so we can set realistic outcomes with the patient. In terms of therapeutic interventions, I think that that is ultimately a customizable situation,” van Amerongen said.
All stakeholders agreed on the benefits of a second opinion. “There’s tremendous value in second opinions; [they go] a long way to helping create confidence, not necessarily in the referring physician, but in the original health care professional to understand, first of all, that professional’s willingness to have another clinician weigh in and give his or her opinion, and secondly to provide additional information and support for whatever treatment plan that is ultimately devised between the individual and the health care team,” van Amerongen said.
Ratan added, “Ultimately, the journey belongs to the patient, and ensuring that they have confidence in the information that they’re getting is probably the single most important thing that we can do to ensure that they’re comfortable with their plans.”
The best source of public information on DTs is through a patient advocacy group, Gounder said. “The DTRF and other advocacy groups in Europe have very high-quality evidence-based information that is readily available for most patients across the globe,” he said.
This information is curated by the DTRF, “key opinion leaders, surgeons, radiation oncologists, medical oncologists, and pathologists,” Gounder said, adding that consensus guidelines also can be a good source of information for patients. Ratan agreed and noted the global consensus paper from the Desmoid Tumor Working Group11 as a good source, and one in which an effort has been made to limit bias.
Final Thoughts
All stakeholders agreed that a multidisciplinary discussion is critical to good care as well as encouraging patients and providers to seek opinions at centers of excellence and other trusted sources of information.
References
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2. Bektas M, Bell T, Khan S, et al. Desmoid tumors: a comprehensive review. Adv Ther. 2023;40(9):3697-3722. doi:10.1007/s12325-023-02592-0
3. Xie Y, Xie K, Gou Q, He J, Zhong L, Wang Y. Recurrent desmoid tumor of the mediastinum: a case report. Oncol Lett. 2014;8(5):2276-2278. doi:10.3892/ol.2014.2431
4. Yazici N, Yalçin B, Soylemezoglu F, et al. Intracranial desmoid tumor with familial adenomatous polyposis coli. Pediatr Neurosurg. 2008;44(2):140-143. doi:10.1159/000113117
5. Magid D, Fishman EK, Jones B, Hoover HC, Feinstein R, Siegelman SS. Desmoid tumors in Gardner syndrome: use of computed tomography. AJR Am J Roentgenol. 1984;142(6):1141-1145. doi:10.2214/ajr.142.6.1141
6. Kasper B, Baumgarten C, Garcia J, et al; Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399-2408. doi:10.1093/annonc/mdx323
7. Anneberg M, Svane HML, Fryzek J, et al. The epidemiology of desmoid tumors in Denmark. Cancer Epidemiol. 2022;77:102114. doi:10.1016/j.canep.2022.102114
8. NCCN. Clinical Practice Guidelines in Oncology. Soft tissue sarcoma, version 3.2023. Accessed January 7, 2024. https://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf
9. Edmiston K. Effective October 1, 2023, ICD-10-CM diagnosis codes are available for desmoid tumors. NCODA. Accessed January 9, 2024. https://www.ncoda.org/effective-october-1-2023-icd-10-cm-diagnosis-codes-are-available-for-desmoid-tumors/
10. Ingley KM, Klein R, Theobalds N, et al. High prevalence of persistent emotional distress in desmoid tumor. Psychooncology. 2020;29(2):311-320. doi:10.1002/pon.5250
11. Desmoid Tumor Working Group. The management of desmoid tumors: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96-107. doi:10.1016/j.ejca.2019.11.013
