Desmoid Tumor
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Aggressive fibromatosis or desmoid tumor is a rare condition. Desmoid tumors are a type of
fibromatosis The term fibromatosis refers to a group of soft tissue tumors which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblast ...
and related to
sarcoma A sarcoma is a rare type of cancer that arises from cells of mesenchymal origin. Originating from mesenchymal cells means that sarcomas are cancers of connective tissues such as bone, cartilage, muscle, fat, or vascular tissues. Sarcom ...
, though without the ability to spread throughout the body (
metastasize Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
). The tumors arise from cells called
fibroblasts A fibroblast is a type of biological cell typically with a spindle shape that synthesizes the extracellular matrix and collagen, produces the structural framework ( stroma) for animal tissues, and plays a critical role in wound healing. Fibrobla ...
, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. They can be either relatively slow-growing or malignant. However, aggressive fibromatosis is locally aggressive and invasive, with spindle-like growths. The tumors can lead to pain, life-threatening problems, or, rarely, death when they invade other soft tissue or compress vital organs such as intestines, kidneys, lungs, blood vessels, or nerves. Most cases are sporadic, but some are associated with
familial adenomatous polyposis Familial adenomatous polyposis (FAP) is an autosomal dominant inherited condition in which numerous adenomatous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon ...
(FAP). Approximately 10% of individuals with
Gardner's syndrome Gardner's syndrome (also known as Gardner syndrome, familial polyposis of the colon, or familial colorectal polyposis) is a subtype of familial adenomatous polyposis (FAP). Gardner syndrome is an autosomal dominant form of polyposis characterize ...
, a type of FAP with extracolonic features, have desmoid tumors. In 2020, the World Health Organization reclassified desmoid tumors (termed desmoid-type fibromatosis) as a specific type of tumor in the category of intermediate (locally aggressive)
fibroblastic and myofibroblastic tumors Fibroblastic and myofibroblastic tumors (FMTs) are tumors which develop from the mesenchymal stem cells which differentiate into fibroblasts (the most common cell type in connective tissue) and/or the myocytes/ myoblasts that differentiate into ...
. Histologically they resemble very low-grade
fibrosarcoma Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform ...
s, but they are very locally aggressive and tend to recur even after complete resection. The condition is "characterized by a variable and often unpredictable clinical course." There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery. The condition can be chronic and may be debilitating.


History and etymology

The condition was first described in 1832 by John MacFarlane. ''Desmoid'', used by
Johannes Peter Müller Johannes Peter Müller (14 July 1801 – 28 April 1858) was a German physiologist, comparative anatomist, ichthyologist, and herpetologist, known not only for his discoveries but also for his ability to synthesize knowledge. The paramesonephri ...
in 1838, comes from the Greek 'band or tendon-like', describing the tumors' consistency. The term found broad acceptance in the 1880s. Over the next several decades,
Georg Ledderhose Georg Otto Ledderhose (15 December 1855 – 1 February 1925) was a German surgeon, professor and pioneering traumatologist. Born in Bockenheim, Frankfurt am Main, he studied medicine at the University of Strasbourg and the University of Göttingen ...
and C. Pfeiffer compiled and reported a number of cases, reaching 400 by the early 1900s. In 1923, Ralph W. Nichols first described the correlation between
familial adenomatous polyposis Familial adenomatous polyposis (FAP) is an autosomal dominant inherited condition in which numerous adenomatous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon ...
(FAP) and desmoid tumors.
Arthur Purdy Stout Arthur Purdy Stout (1885–1967) was an American surgeon and pathologist. Early years and education Arthur Purdy Stout was the fourth son of Joseph and Julia Frances (née Purdy) Stout. He attended the Pomfret School and Yale University, where he ...
coined the term ''fibromatosis'' (in the name ''
congenital generalized fibromatosis A birth defect is an abnormal condition that is present at birth, regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can range from mild to severe. Birth def ...
'', describing myofibromatosis) in 1954.


Causes and risk factors

Wnt signaling pathway In cellular biology, the Wnt signaling pathways are a group of signal transduction pathways which begin with proteins that pass signals into a cell through cell surface receptors. The name Wnt, pronounced "wint", is a portmanteau created from the ...
alterations are the likely cause of desmoid tumor formation. Mutations have been discovered in both the beta-catenin encoding ''
CTNNB1 Catenin beta-1, also known as β-catenin (''beta''-catenin), is a protein that in humans is encoded by the ''CTNNB1'' gene. β-Catenin is a dual function protein, involved in regulation and coordination of cell–cell adhesion and gene transcr ...
'' gene and the tumor-suppressing '' APC'' gene, which affect the Wnt pathway. A 2015 study on desmoid tumors lacking these mutations found that almost all, 95%, "may have mutations that affect the Wnt/β-catenin pathway, suggesting a near universal relationship between desmoid tumors and Wnt signaling." The majority of cases are sporadic, most of which – 85% – involve a ''CTNNB1'' mutation. Of these, "the three distinct mutations identified are 41A, 45F, and 45. Mutation 45F is associated with a high risk of recurrence." ''APC'' mutations affect FAP patients and make up a smaller percentage, 10–15%, of sporadic cases. The disease has a tendency to occur during and after pregnancy and in exposure to higher
estrogen Estrogen (also spelled oestrogen in British English; see spelling differences) is a category of sex hormone responsible for the development and regulation of the female reproductive system and secondary sex characteristics. There are three ...
levels, suggesting a hormonal link. One study noted the formation of desmoid tumors in guinea pigs after prolonged estrogen exposure. Other factors include
trauma Trauma most often refers to: *Psychological trauma, in psychology and psychiatric medicine, refers to severe mental and emotional injury caused by distressing events *Traumatic injury, sudden physical injury caused by an external force, which doe ...
and surgery. Risk factors for desmoid disease amongst FAP patients include female sex, a 3' ''APC'' mutation, a positive family history, and a history of previous abdominal surgery.


Occurrence

The incidence of desmoid tumors is 5–6 per million per year; they constitute 0.03% of tumors and less than 3% of soft-tissue tumors. The primary age range is 15–60, with a peak between 30 and 40 years old; it is 2–3 times more common in females than males. A 2012 retrospective multi-institutional analysis of 211 patients found a median age of 36 and a 68% female prevalence. Children do not have the same sex disparity and are most commonly affected around 15 or 16 years old.


Diagnosis


Diagnosis

A biopsy is always indicated as the definitive method to determine the nature of the tumor. Diagnosis may be difficult in part due to the use of core needle biopsy over open biopsy. Similarities among bland spindle-cell lesions lead to a large number of possibilities in diagnosis, including fibroblastic sarcomas,
Gardner fibroma Gardner fibroma (GF) (also termed Gardner-associated fibroma) is a benign fibroblastic tumor (i.e. a tumor containing fibroblasts, the most common cell type in connective tissue). GF tumors typically develop in the dermis (i.e. layer of skin under ...
, scar tissue or
keloid Keloid, also known as keloid disorder and keloidal scar, is the formation of a type of scar which, depending on its maturity, is composed mainly of either type III (early) or type I (late) collagen. It is a result of an overgrowth of granulation t ...
s, superficial
fibromatosis The term fibromatosis refers to a group of soft tissue tumors which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblast ...
,
nodular fasciitis Nodular fasciitis (NF) is a benign, soft tissue tumor composed of myofibroblasts that typically occurs in subcutaneous tissue, fascia, and/or muscles. The literature sometimes titles rare NF variants according to their tissue locations. The most f ...
, myofibroma,
collagenous fibroma Collagenous fibroma, also known as desmoplastic fibroblastoma, is a slow-growing, deep-set, benign fibrous tumor, usually located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck reg ...
,
gastrointestinal stromal tumor Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs arise in the smooth muscle pacemaker interstitial cell of Cajal, or similar cells. They are defined as tumors whose behavior i ...
,
solitary fibrous tumor Solitary fibrous tumor (SFT), also known as fibrous tumor of the pleura, is a rare mesenchymal tumor originating in the pleuraTravis WD, Brambilla E, Muller-Hermelink HK, Harris CC (Eds.): World Health Organization Classification of Tumours. Pathol ...
,
phyllodes tumor Phyllodes tumors (from Greek language, Greek: ), are a rare type of Biphasic tumor, biphasic Fibroepithelial neoplasm, fibroepithelial mass that form from the periductal stromal and epithelial cells of the breast. They account for less than 1% of ...
, and other conditions. Such conditions may therefore also be incorrectly diagnosed as desmoid tumors (29% of cases in one review). Some 30–40% of desmoid tumors may be misdiagnosed.


Classification

Desmoid tumors can occur almost anywhere in the body. They are classified as extra-
abdominal The abdomen (colloquially called the gut, belly, tummy, midriff, tucky, or stomach) is the front part of the torso between the thorax (chest) and pelvis in humans and in other vertebrates. The area occupied by the abdomen is called the abdominal ...
,
abdominal wall In anatomy, the abdominal wall represents the boundaries of the abdominal cavity. The abdominal wall is split into the anterolateral and posterior walls. There is a common set of layers covering and forming all the walls: the deepest being the vi ...
, or intra-abdominal; the last is more common in patients with FAP. Most cases occur in the
mesentery In human anatomy, the mesentery is an Organ (anatomy), organ that attaches the intestines to the posterior abdominal wall, consisting of a double fold of the peritoneum. It helps (among other functions) in storing Adipose tissue, fat and allowi ...
, abdominal wall, and extremities. One study has shown extra-abdominal tumors making up 43% of cases, abdominal tumors 49%, and mesenteric 8%, though statistics vary. Pregnancy-related tumors typically arise in the abdominal wall. Tumors located intra-abdominally or in the head and neck have the highest risk of mortality due to the proximity to vital structures. One analysis has shown a median tumor size of . Though
metastasis Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
cannot occur, the tumors may in some cases be multifocal, with several located in the same body part. A 3' ''APC'' mutation is the most significant risk factor for intra-abdominal desmoid development amongst FAP patients. FAP patients presenting with an abdominal wall desmoid pre-operatively are at an increased risk of developing an intra-abdominal desmoid post-operatively. Desmoid tumors of the breast are rare, constituting 4% of extra-abdominal cases and 0.2–0.3% of breast tumors. Although benign, they can mimic breast cancer on physical examination, mammography and breast ultrasound and can also be locally invasive. Even though they occur sporadically, they can also be seen as a part of Gardner's syndrome. Some cases – up to 44% – occur in patients who have previously had breast surgery. A high index of suspicion and a thorough triple examination protocol is necessary to detect rare lesions like a desmoid tumor which can masquerade as breast carcinoma. Desmoid tumor of the breast may present a difficulty in the diagnosis especially where imaging studies are not conclusive and suggest a more ominous diagnosis. They may arise in the
chest wall The thoracic wall or chest wall is the boundary of the thoracic cavity. Structure The bony skeletal part of the thoracic wall is the rib cage, and the rest is made up of muscle, skin, and fasciae. The chest wall has 10 layers, namely (from ...
or the breast itself. Desmoid tumors may occur in the head and neck, more commonly among children, and tend to be more aggressive than in other extra-abdominal locations. These tumors constitute up to 23% of extra-abdominal cases. Treatment is typically more aggressive due to the increased dangers of a tumor in the area.


Staging

There is no standard staging system; desmoid tumors do not fall under cancer staging systems as they do not metastasize.


Disease course, treatment, and impacts


Disease course

The condition is "characterized by a variable and often unpredictable clinical course", often considered chronic, and with the potential to be debilitating. Death, however, is uncommon. Tumors may grow, regress, or remain stable: * Resolution without treatment (10–28%) * Progression and resolution (30%) * Stable (50%) * Rapid progression (10%) Management of these lesions is complex, the main problem being the high rates of recurrence particularly in FAP-associated disease. Recurrence rates in general vary from 19 to 77 percent. Conversely, for intra-abdominal fibromatosis without evidence of FAP, although extensive surgery may still be required for local symptoms, the risk of recurrence appears to be lower.


Treatment, research, and management

Nirogacestat Nirogacestat, sold under the brand name Ogsiveo, is an anti-cancer medication used for the treatment of desmoid tumors. It is a selective gamma secretase enzyme inhibitor, inhibitor that is taken by mouth. Nirogacestat was approved for medi ...
, a selective
gamma secretase Gamma secretase is a multi-subunit protease complex, an integral membrane protein, that cleaves single-pass transmembrane proteins at residues within the transmembrane domain. Proteases of this type are known as intramembrane proteases. The most ...
inhibitor Inhibitor or inhibition may refer to: Biology * Enzyme inhibitor, a substance that binds to an enzyme and decreases the enzyme's activity * Reuptake inhibitor, a substance that increases neurotransmission by blocking the reuptake of a neurotransmi ...
, was approved for medical use in the United States in November 2023. It is the first medication approved by the US
Food and Drug Administration The United States Food and Drug Administration (FDA or US FDA) is a List of United States federal agencies, federal agency of the United States Department of Health and Human Services, Department of Health and Human Services. The FDA is respo ...
(FDA) for the treatment of desmoid tumors. A Phase 2/3 trial on AL102, another selective gamma secretase inhibitor, is also ongoing , having begun in 2021. The drug was granted
orphan drug An orphan drug is a medication, pharmaceutical agent that is developed to treat certain rare medical conditions. An orphan drug would not be profitable to produce without government assistance, due to the small population of patients affected by th ...
status in 2023. Wnt pathway inhibitors are also being developed and studied . These include E7386, tegavivint and ipafricept. Additionally, the
tumor microenvironment The tumor microenvironment is a complex ecosystem surrounding a tumor, composed of cancer cells, stromal tissue (including blood vessels, immune cells, fibroblasts and signaling molecules) and the extracellular matrix. Mutual interaction between ...
in desmoid tumors is being investigated to find new targets for treatment. Surgery was the standard treatment for desmoid tumors up to the early 2000s. Due to the condition's unpredictability, more conservative management such as
watchful waiting Watchful waiting (also watch and wait or WAW) is an approach to a medical problem in which time is allowed to pass before medical intervention or therapy is used. During this time, repeated testing may be performed. Related terms include ''expe ...
has since become common due to the potential impacts of surgical interventions. As of the 2010s, there is a "clear consensus" from medical groups, including the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group and the
European Society for Medical Oncology The European Society for Medical Oncology (ESMO) is a professional organisation for medical oncology. With more than 40,000 members representing oncology professionals from over 177 countries worldwide, ESMO was founded in 1975. ''Annals of Onco ...
: immediate surgical resection is no longer the first-line treatment, particularly in asymptomatic patients. Complete removal is not always possible due to the tumors' infiltrative nature and tendril-like growth. In more advanced, recurring, or rapidly progressing cases, treatment may consist of complete
surgical Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery ...
removal,
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
,
antiestrogen Antiestrogens, also known as estrogen antagonists or estrogen blockers, are a class of drugs which prevent estrogens like estradiol from mediating their biological effects in the body. They act by blocking the estrogen receptor (ER) and/or inh ...
s (e.g.
tamoxifen Tamoxifen, sold under the brand name Nolvadex among others, is a selective estrogen receptor modulator used to prevent breast cancer in women and men. It is also being studied for other types of cancer. It has been used for Albright syndrome ...
),
nonsteroidal anti-inflammatory drug Non-steroidal anti-inflammatory drugs (NSAID) are members of a Indication (medicine), therapeutic drug class which Analgesic, reduces pain, Anti-inflammatory, decreases inflammation, Antipyretic, decreases fever, and Antithrombotic, prevents bl ...
s (NSAIDs),
chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
(e.g.
methotrexate Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immunosuppressive drug, immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancy, ectopic pregnancies. Types of cancers it is u ...
and
vinblastine Vinblastine, sold under the brand name Velban among others, is a chemotherapy medication, typically used with other medications, to treat a number of types of cancer. This includes Hodgkin's lymphoma, non-small-cell lung cancer, bladder canc ...
or
vinorelbine Vinorelbine, sold under the brand name Navelbine among others, is a chemotherapy medication used to treat a number of types of cancer. This includes breast cancer and non-small cell lung cancer. It is given by injection into a vein or by mout ...
,
doxorubicin Doxorubicin, sold under the brand name Adriamycin among others, is a chemotherapy medication used to treat cancer. This includes breast cancer, bladder cancer, Kaposi's sarcoma, lymphoma, and acute lymphocytic leukemia. It is often used toge ...
), or
ablation Ablation ( – removal) is the removal or destruction of something from an object by vaporization, chipping, erosion, erosive processes, or by other means. Examples of ablative materials are described below, including spacecraft material for as ...
(
cold Cold is the presence of low temperature, especially in the atmosphere. In common usage, cold is often a subjectivity, subjective perception. A lower bound to temperature is absolute zero, defined as 0.00K on the Kelvin scale, an absolute t ...
, heat,
ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...
). Treatment with oral
tyrosine kinase inhibitor A tyrosine kinase inhibitor (TKI) is a pharmaceutical drug that inhibits tyrosine kinases. Tyrosine kinases are enzymes responsible for the activation of many proteins by signal transduction cascades. The proteins are activated by adding a phosph ...
drugs (e.g.
imatinib Imatinib, sold under the brand names Gleevec and Glivec (both marketed worldwide by Novartis) among others, is an oral targeted therapy medication used to treat cancer. Imatinib is a small molecule inhibitor targeting multiple tyrosine kinases ...
,
sorafenib Sorafenib, sold under the brand name Nexavar, is a kinase inhibitor drug approved for the treatment of primary kidney cancer (advanced renal cell carcinoma), advanced primary liver cancer (hepatocellular carcinoma), FLT3-ITD positive AML and ra ...
,
pazopanib Pazopanib, sold under the brand name Votrient, is an anti-cancer medication marketed worldwide by Novartis. It is a potent and selective multi-targeted receptor tyrosine kinase inhibitor that blocks tumour growth and inhibits angiogenesis. It h ...
,
sunitinib Sunitinib, sold under the brand name Sutent, is an anti-cancer medication. It is a small-molecule, multi-targeted receptor tyrosine kinase (RTK) inhibitor that was approved by the FDA for the treatment of renal cell carcinoma (RCC) and imat ...
) shows promising success rates. Radiation therapy after surgery may improve outcomes. Despite the condition's hormonal link, anti-hormonal therapies only appear to work in a small subset of patients.
Intestinal transplant Intestine transplantation (intestinal transplantation, or small bowel transplantation) is the surgical replacement of the small intestine for chronic and acute cases of intestinal failure. While intestinal failure can oftentimes be treated wi ...
is a treatment option for those patients with complicated desmoid tumor, such as those involving the mesenteric root, or those with intestinal failure resulting from the tumor or prior interventions.
MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
or CT imaging scans are commonly used for monitoring. In contrast with cancer, management of desmoid tumors considers additional outcomes beyond
progression-free survival Progression-free survival (PFS) is "the length of time during and after the treatment of a disease, such as cancer, that a patient lives with the disease but it does not get worse". In oncology, PFS usually refers to situations in which a tumor is ...
and
overall survival Survival rate is a part of survival analysis. It is the proportion of people in a study or treatment group still alive at a given period of time after diagnosis. It is a method of describing prognosis in certain disease conditions, and can be use ...
as desmoid tumor patients' "survival is longer and... age of onset is generally younger compared with cancer patient populations".


Impacts

One review summarizes the disease's impact on patients stating, "the burden of esmoid tumorsis disproportionately borne by women of childbearing and working age, and because it is associated with low mortality and a relatively young patient population, it typically continues for decades." Symptoms vary significantly as they are dependent on the tumor's location and effects on the surrounding structures. Though desmoid tumors do not metastasize, their invasiveness may lead to pain and loss of function or restricted movement. Chronic pain is an issue for as many as 63% of patients and may be debilitating and lead to reliance on pain medication. Pressure on vital organs or deformity may occur. Rarely, amputation may be necessary due to injury caused by the tumor or its treatments. Tumors may be misdiagnosed (30–40%) due to their rarity and a lack of knowledge; patients may initially be given inappropriate treatment or poor prognoses due to misdiagnosis with conditions such as malignant sarcoma. Patients may need to visit multiple healthcare providers to receive a diagnosis, causing delay in care. Patients may experience issues including anxiety, fatigue, or trouble sleeping; despite the increased survival rate, their level of emotional distress has been compared to that of cancer patients, including "patients with
sarcoma A sarcoma is a rare type of cancer that arises from cells of mesenchymal origin. Originating from mesenchymal cells means that sarcomas are cancers of connective tissues such as bone, cartilage, muscle, fat, or vascular tissues. Sarcom ...
, also a malignant connective tissue disorder". A lack of knowledge by healthcare providers and of information available to patients and others have also been cited as issues. The economic burden of treatment may be significant, with surgery costs estimated at $50,000 in 2022 US dollars. Specific instruments to determine health-related
quality of life Quality of life (QOL) is defined by the World Health Organization as "an individual's perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards ...
impacts for desmoid patients, the Gounder/Desmoid Tumor Research Foundation (DTRF) Desmoid Symptom/Impact Scale (GODDESS) and the Desmoid-type fibromatosis Quality of Life Questionnaire (DTF-QOL) have been developed and validated.


ICD-10-CM diagnosis codes

Few rare diseases have a specific code in the
International Classification of Diseases The International Classification of Diseases (ICD) is a globally used medical classification that is used in epidemiology, health management and clinical diagnosis. The ICD is maintained by the World Health Organization (WHO), which is the dir ...
. As of October 2023, specific codes for desmoid tumors will be included in the
ICD-10-CM The ICD-10 Clinical Modification (ICD-10-CM) is a set of diagnosis codes used in the United States of America. It was developed by a component of the U.S. Department of Health and Human services, as an adaption of the ICD-10 with authorizatio ...
, the United States'
diagnosis code In health care, diagnosis codes are used as a tool to group and identify diseases, disorders, symptoms, poisonings, adverse effects of drugs and chemicals, injuries and other reasons for patient encounters. Diagnostic coding is the translation of ...
system, after a request from the Desmoid Tumor Research Foundation. A subcategory of D48.1, Neoplasm of uncertain behavior of connective and other soft tissue, has been created with more specific codes: * D48.11: Desmoid tumor ** D48.110: Desmoid tumor of head and neck ** D48.111: Desmoid tumor of chest wall ** D48.112: Desmoid tumor, intrathoracic ** D48.113: Desmoid tumor of abdominal wall ** D48.114: Desmoid tumor, intraabdominal *** Desmoid tumor of pelvic cavity *** Desmoid tumor, peritoneal, retroperitoneal ** D48.115: Desmoid tumor of upper extremity and shoulder girdle ** D48.116: Desmoid tumor of lower extremity and pelvic girdle *** Desmoid tumor of buttock ** D48.117: Desmoid tumor of back ** D48.118: Desmoid tumor of other site ** D48.119: Desmoid tumor of unspecified site


Notable patients

*
Dave Dravecky David Francis Dravecky (born February 14, 1956) is an American former professional baseball player, motivational speaker, and author. A left-handed pitcher, Dravecky played Major League Baseball (MLB) for the San Diego Padres (1982–87) and the ...
, American baseball pitcher and motivational speaker * Kevin Reilly, American football player


In animals

Desmoid tumors occur in dogs, primarily on the head, and more infrequently in horses and cats. A case has also been observed in a goat.


References

{{Authority control Dermal and subcutaneous growths Connective and soft tissue neoplasms Rare diseases