Observing People With Desmoid-Type Fibromatosis

Study Overview
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Brief Summary
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The purpose of this study is to closely observe people with desmoid-type fibromatosis over 1 months.

Official Title
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A Prospective Analysis of Active Observation in Patients With Desmoid-Type Fibromatosis

Conditions
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Desmoid Fibromatosis

Intervention / Treatment
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* Diagnostic Test: Cross-sectional imaging


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Clinical diagnosis of desmoid fibromatosis, either new or newly recurrent. Both patients with sporadic and FAP-associated disease will be eligible for inclusion. Patients with clinical diagnosis of desmoid (as assessed by MSK physician) but without pathologic diagnosis may be enrolled on the study to facilitate acquisition of appropriately protocoled baseline scans and allow for biospecimen collection; these patients will be replaced for purposes of analysis if pathology does not confirm clinical diagnosis. At least 18 years of age. Able to undergo cross-sectional imaging by either MRI or CT with intravenous contrast Disease detectable on cross-sectional imaging with target lesion measuring >/= 1 cm Eligible for management by active observation as assessed by primary MSK care provider Willing and able to sign an informed consent document Exclusion Criteria: Symptomatic desmoid fibromatosis requiring narcotic pain control, resulting in intestinal fistulization, or requiring inpatient admission during the 3 months prior to enrollment Patients with intraabdominal desmoids greater than 7cm in diameter or localized within 1cm of the central mesenteric vessels and those with tumors in the head and neck KPS performance status </= 70 if performance status is limited due to tumor. Patients with low KPS performance status related to co-morbidities but without symptoms related to the tumor will be eligible for enrollment. Age less than 18 years old Strict contraindication for both cross-sectional MRI and CT imaging with intravenous contrast (e.g., as per standard institutional protocols regarding renal insufficiency or MRI incompatible implanted devices; patients requiring pre-medication to prevent contrast reactions will be eligible for study) Unable to grant reliable informed consent Ongoing systemic treatment for desmoid fibromatosis

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Desmoid Fibromatosis<br>Participants will have a clinical diagnosis of desmoid fibromatosis, either new or newly recurrent | Diagnostic Test: Cross-sectional imaging<br>* MRI with gadolinium contrast will be the imaging modality of choice. In patients with contraindications to MRI who instead undergo baseline evaluation by CT scan, tumor evaluation will be by serial CT scans with IV contrast throughout the study period.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression free survival | The primary objective of this study is to estimate progression-free survival at 12 months, with the events defined by progression via radiographic criteria (RECIST 1.1). | 12 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Desmoid-Type Fibromatosis, Desmoid Fibromatosis, Desmoid, Fibromatosis, 20-026, Memorial Sloan Kettering Cancer Center