Current Treatment Concepts for Extra-Abdominal Desmoid-Type Fibromatosis: A Narrative Review

Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal...

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Bibliographic Details
Published in:Cancers 2024-01, Vol.16 (2), p.273
Main Authors: Lee, Yong-Suk, Joo, Min Wook, Shin, Seung-Han, Hong, Sungan, Chung, Yang-Guk
Format: Article
Language:English
Subjects:
Abdomen
Anti-inflammatory agents
Antimitotic agents
Antineoplastic agents
Cancer
Care and treatment
Chemotherapy
Clinical trials
Connective tissue diseases
Cytotoxicity
Drug approval
Drug dosages
Enzyme inhibitors
Inflammation
Literature reviews
Medical research
Medicine, Experimental
Nonsteroidal anti-inflammatory drugs
Pain
Pediatrics
Quality of life
Radiation
Radiation therapy
Secretase
Surgery
Surveillance
Tumors
Tyrosine
Tyrosine kinase inhibitors
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Summary:Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal tissues, making it difficult to completely eliminate them without adjacent healthy tissue injury. Surgical excision of these locally aggressive tumors without clear resection margins often leads to local recurrence. The aim of this thorough review was to assess the current treatment concepts for these rare tumors. A comprehensive search of articles published in the Cochrane Library, MEDLINE (PubMed), and EMBASE databases between January 2008 and February 2023 was conducted. Surgical intervention is no longer the first-line approach for most cases; instead, strategies like active surveillance or systemic therapies are used as initial treatment options. With the exception of EADTFs situated near vital structures, a minimum of 6-12 months of active surveillance is currently advocated for, during which some disease progression may be considered acceptable. Non-surgical interventions such as radiation or cryoablation may be employed in certain patients to achieve local control. The currently preferred systemic treatment options include tyrosine kinase inhibitors, low-dose chemotherapy, and gamma-secretase inhibitors, while hormone therapy is not advised. Nonsteroidal anti-inflammatory drugs are utilized primarily for pain management.
ISSN:2072-6694
2072-6694
DOI:10.3390/cancers16020273