Diagnostic Evaluation

Initial work-up typically consists of imaging of the affected anatomic site to confirm the cause. Although both CT and magnetic resonance imaging (MRI) are adequate for evaluation, MRI is preferred, particularly for extremity desmoids, as it better reveals involvement of surround­ing tissues. As neither imaging modality can distinguish a desmoid from a malignant soft-tissue tumor, histologic evaluation is needed to confirm the diagnosis. A biopsy can confirm monoclonal growth of fibroblast cells, which appear as a bundle of spindle cells in a fibrous stroma. Cellularity is typically low. Distinguishing desmoids from fibrosarcomas is particularly important and necessitates an incisional biopsy, rather than fine-needle aspiration. Pathologically, this is accomplished by observing that the cells in the desmoids lack nuclear and cytoplasmic fea­tures of malignancy (ie, there are few mitotic bodies, and necrosis is absent). Immunohistochemical staining can aid in the process of diagnosis, as can nuclear beta-catenin staining. Additionally, spindle cells often contain vimentin and smooth muscle actin but rarely stain for desmin, cytokeratins, and S-100.

Treatment

Early referral to a center that specializes in multimodality care of sarcomas is warranted. In asymptomatic patients, close observation is often the preferred strategy. Patients with symptoms are typically treated, given the inevitably progressive growth of desmoid tumors. Surgery with a wide margin of resection is the preferred treatment when­ever feasible, particularly for intra-abdominal desmoids. Limitations include tumor involvement of vital anatomic structures, where resection would lead to functional limi­tation or significant morbidity and mortality. Similarly, desmoids frequently recur at a high rate (16—39%), even when surgical margins are free of tumor, and these recur­rences are often more aggressive than the initial tumor.
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Radiation therapy has been shown to be an effec­tive alternative for definitive care in patients who refuse surgery, patients who are not good surgical candidates, or patients in whom surgery carries a high risk. Addition­ally, trials are being conducted to evaluate neoadjuvant radiation prior to surgery, which has shown promise.

For patients with extra-abdominal tumors as well as those with recurrence despite local therapy, systemic medical therapy is often prescribed. Options include tamoxifen, which is thought to suppress desmoid growth due to the presence of estrogen receptor beta on tumor cells, particularly extra-abdominal desmoids. Similarly, nonsteroidal anti-inflammatory drugs (NSAIDs) such as sulindac, alone or with tamoxifen, have shown promis­ing response rates. With either anti-estrogen or NSAID therapy, tumor shrinkage is often slow and delayed by many months.

Finally, in patients who do not respond to non- cytotoxic therapy, trials have shown good response to treatment with either doxorubicin-based regimens or methotrexate with vinca alkaloids.

Post-treatment surveillance includes clinical exami­nation and radiographic assessment every 6 months for at least 3 years and then yearly thereafter.
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Summary

This case report highlights the incidence of desmoid tumors, a rare but clinically significant cause of neoplastic disease. Although desmoids are uncommon, the asymp­tomatic nature of their progression and their locally invasive and aggressive behavior require clinicians to be aware of them, as they can cause significant functional impairment as well as morbidity and mortality if vital structures are involved. Surgical resection is the preferred therapy, though recurrence is high despite complete resection. Radiation therapy, as well as medical therapy with noncytotoxic and cytotoxic agents, has a role as an alternative to surgical therapy or experimentally in the neoadjuvant setting. Close follow-up of these patients is warranted, given the high rate of recurrence.