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Related Experiment Video

Updated: Dec 15, 2025

Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors

Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors

Published on: May 17, 2024

Desmoid-Type Fibromatosis.

Dorian Yarih Garcia-Ortega¹, Karla Susana Martín-Tellez², Mario Cuellar-Hubbe¹

¹National Cancer Institute (Instituto Nacional de Cancerologia), Mexico City 14080, Mexico.

|July 15, 2020

Summary

Desmoid tumors are rare, locally aggressive growths. While often monitored, progressive cases may require systemic treatment, radiotherapy, or surgery for management.

Keywords:

aggressive fibromatosis desmoid desmoid tumor desmoid-type fibromatosis β-catenin

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Published on: December 22, 2020

Area of Science:

Oncology
Genetics
Pathology

Background:

Desmoid tumors are monoclonal, locally aggressive neoplasms without metastatic potential.
They arise sporadically (CTNNB1 mutation) or hereditarily (APC mutation), both leading to abnormal beta-catenin accumulation.
Common sites include extremities and thoracic wall; familial adenomatous polyposis-associated cases present intra-abdominally or in the abdominal wall.

Purpose of the Study:

To provide a narrative review of desmoid tumors.
To summarize current knowledge on molecular pathogenesis, diagnosis, and treatment strategies.

Main Methods:

Literature review of desmoid tumors.
Synthesis of information on molecular basis, diagnostic modalities, and therapeutic options.

Main Results:

Desmoid tumors exhibit variable behavior, with potential for spontaneous stabilization or regression.
Diagnosis relies on histopathology and imaging (ultrasound, CT, MRI).
Management includes active surveillance, systemic therapy, radiotherapy, or surgery based on clinical presentation.

Conclusions:

Desmoid tumors require careful evaluation and tailored management strategies.
Active surveillance is often the initial approach for non-progressive disease.
Multidisciplinary treatment is essential for symptomatic or disabling cases.