Desmoid tumor

PBL#1 RESOLUTION

DESMOID TUMOR & AFFECTED ANATOMICAL

STRUCTURES

Luiza Niyazmetova

INTRODUCTION

Desmoid tumors (aggressive fibromatosis) - rare tumors of mesenchymal origin that vary widely in presentation & arises from deep muscle fascia, aponeurosis, and tendons

Locally aggressive tumors but have no capacity to metastasize Can be multifocal, and locally infiltrate surrounding structures

Can occur in association with familial adenomatous polyposis, trauma, prior surgery, or pregnancy

Wnt/β-catenin pathway drives the pathogenesis of both sporadic and FAP-associated desmoid tumors

Hematology/Oncology Clinics of North America, 2013-10-01, Volume 27, Issue 5, Pages 989-1005, Copyright © 2013 Elsevier Inc.

INTRODUCTION Many treatment modalities ranging from conservative,

nonsurgical approaches to aggressive cytotoxic chemotherapy

Frequently recur w/in 2 years after the initial excision Recurrence rates have higher rates reported in those

arising in extra-abdominal locations & in patients with FAP

Despite their aggressive behavior and propensity to recur, they rarely lead to death with overall survival rates at 10 years exceeding 90%

CLASSIFICATION Extra-abdominal Abdominal Intra-abdominal (mesenteric fibromatosis and pelvic

fibromatosis)

a Head and neck b Lower extremity c Intra-abdominal (mesenteric) desmoid

Litchman, Ch. (2011). Desmoid tumors. Springer Science & Business Media

MAJOR ANATOMIC SITES


a. Locations of all desmoid tumors b. FAP-associated desmoid tumors

CLINICAL PRESENTATION Symptoms: Painless swelling or lump Pain or soreness caused by compressed nerves or

muscles. Pain and obstruction of the bowels Cachexia, renal failure, small bowel compression,

peritonitis Limping or other difficulty using the legs, feet, arms or

hands or other affected part of the body

EXTRA-ABDOMINAL DESMOID TUMOR

In upper extremity & shoulder girdle, the tumors are often present in the deltoid, scapular region, supraclavicular fossa, posterior cervical triangle, axilla, & upper arm

Vital structures affected: brachial plexus (resulting in ulnar nerve entrapment, numbness in 4th & 5th fingers), subclavian, axillary, & brachial vessels

Tumors of this region may grow large in size & cause significant symptoms such as pain, swelling, & sensory-motor deficits


DIFFERENTIAL DIAGNOSIS

Reactive conditions: scar and nodular fasciitis Mesenchymal neoplasms: low-grade fibromyxoid sarcoma,

malignant peripheral nerve sheath tumor, nonlipogenic well-differentiated liposarcoma, inflammatory myofibroblastic tumor, schwannoma, and gastrointestinal stromal tumor

TREATMENT MODALITIES


Desmoid tumor

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