> Trialight public data. Index of pages: https://trialight.com/llms.txt
> Web page: https://trialight.com/data/condition/desmoid-tumor-ba8c4931-d9d2-5438-88c9-5cc562a19815
> Record changed: 2026-10-13

# Desmoid tumor

Therapeutic area

Rheumatology

Rare disease

Yes

## Definition and classification

**Definition**

A desmoid tumor (DT) is a benign, locally invasive soft tissue tumor associated with a high recurrence rate but with no metastatic potential.

**Genetic basis**

Yes

**Non-human condition**

No

**Rare disease**

Yes

**Listing at source**

Listed

**Condition name**

Desmoid tumor

**Therapeutic area**

Rheumatology

### Alternative names (13)

- desmoid tumor
- aggressive fibromatosis
- deep fibromatosis
- deep fibromatosis/desmoid tumor
- deep fibromatosis/desmoid tumour
- desmoid fibromatosis
- desmoid tumor
- desmoid type fibromatosis
- desmoid-type fibromatosis
- FIF
- desmoid disorder, hereditary
- desmoid/aggressive fibromatosis
- familial infiltrative fibromatosis

## Clinical profile

**Severity**

Mild

### Disease mechanisms (2)

- Genetic
- Hereditary

### All therapeutic areas (2)

- Oncology
- Rheumatology

## Identifiers

**MONDO ID**

MONDO:0007608

Source: [monarchinitiative.org](https://monarchinitiative.org/MONDO:0007608)

**Parent condition**

MONDO:0005031

## Related records

### Broader conditions (17)

- Cancer or benign tumor
- Connective and soft tissue neoplasm
- [Connective tissue disorder](https://trialight.com/data/condition/connective-tissue-disorder-3df54ecd-c080-5b4c-b60d-fab4846ea17b.md)
- Connective tissue neoplasm
- Disease
- Disease by body system or component
- Disease by etiologic mechanism
- Disease of genetic or genomic mechanism
- Fibroblastic neoplasm
- Fibromatosis
- Hereditary disease
- Hereditary disorder of connective tissue
- Human disease
- Mesenchymal cell neoplasm
- Neoplasm
- Neoplastic disease or syndrome
- Soft tissue neoplasm

### Narrower conditions (1)

- Desmoid tumor caused by somatic mutation

### Trials (68)

- 18F-FES PET/CT in Imaging Patients With Desmoid Tumors
- A Cohort Study of Patients Treated With Brachytherapy for Selected Desmoid Patients in Gardner Syndrome
- A Pilot Study Evaluating the Use of mTor Inhibitor Sirolimus in Children and Young Adults With Desmoid-Type Fibromatosis
- A Prospective Clinical Study on the Safety and Efficacy of Radiofrequency Ablation for the Treatment of Patients With Desmoid Tumors
- A Randomized, Double-blind,Phase Ⅲ Study of Liposome Doxorubicin in Desmoid Tumor
- A Safety Study of ST316 in Participants With Familial Adenomatous Polyposis (FAP).
- A Study in Adults With Desmoid Tumors
- A Study of a New Drug, Nirogacestat, for Treating Desmoid Tumors That Cannot be Removed by Surgery
- A Study of AL102 in Patients With Progressing Desmoid Tumors
- A Study of E7386 in Participants With Advanced Solid Tumor Including Colorectal Cancer (CRC)
- A Study of Nirogacestat in Japanese Adults With Desmoid Tumors/Aggressive Fibromatosis (DT/AF)
- A Study to Evaluate the Safety and Efficacy of Vactosertib and Imatinib in Patients With Advanced Desmoid Tumor
- Arterial Chemoembolization for the Treatment of Desmoid Fibromatosis
- Burden of Disease and Living Situation in Desmoid Patients
- Clinical Study of Anlotinib Combined With Chemotherapy in the Treatment of Unresectable Advanced Desmoid Tumor
- Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma
- Compassionate Use Protocol for PF-03084014 in Patients With Advanced Solid Tumor Malignancies
- Cryoablation for Advanced and Refractory Desmoid Tumors
- Cryoablation Versus Medical Therapy in Desmoid Tumors Progressing After Watchful Waiting
- Cryotherapy in the Treatment of Desmoid Tumors

### Approved drugs (1)

- Nirogacestat hydrobromide

### Drugs in development (16)

- Aminolevulinic acid Phase 2
- Doxorubicin Phase 3
- [Hydroxyurea](https://trialight.com/data/asset/hydroxyurea-a56eda3c-3ef0-4ca7-b972-656091c627a5.md) Phase 2
- Imatinib Phase 2
- [Imatinib mesylate](https://trialight.com/data/asset/imatinib-mesylate-228c51e4-aef9-4351-8581-568722f6cb78.md) Phase 2
- [Methotrexate](https://trialight.com/data/asset/methotrexate-aa7ef9da-5b6e-44a1-942c-610e3de14a1d.md) Phase 2
- [Nirogacestat](https://trialight.com/data/asset/nirogacestat-3fcb771a-72a0-450b-adde-a88a42aee6e0.md) Phase 3
- [Sirolimus](https://trialight.com/data/asset/sirolimus-08da04e4-ea95-4587-95c4-83d583e56570.md) Phase 1
- [Sodium chloride](https://trialight.com/data/asset/sodium-chloride-a67c33bc-45ef-42e4-8022-d121f0a86585.md) Phase 3
- Sorafenib tosylate Phase 3
- Sulindac Phase 2
- [Tamoxifen citrate](https://trialight.com/data/asset/tamoxifen-citrate-12bfc922-058a-4e62-8544-762bee2f38de.md) Phase 2
- Tegavivint Phase 1
- Toremifene Phase 2
- Vactosertib Phase 1
- Vinblastine sulfate Phase 2

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