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Aggressive desmoid fibromatosis of the neck after total thyroidectomy.

A 21-year-old woman underwent total thyroidectomy. Three years later, she noticed a neck mass and increasing discomfort in her neck. A computed tomography (CT) scan showed a mass near the site of the previous thyroidectomy, on the right. Fine-needle-aspiration cytology tests were nondiagnostic.

The patient underwent surgery, during which a paralaryngeal mass was found firmly attached to the surrounding structures and impossible to dissect. Frozen section biopsy was negative for malignancy; a biopsy was taken for permanent section, and the wound was closed. The histologic diagnosis was desmoid tumor.

During the following weeks, the patient's symptoms rapidly worsened and she was referred to our department, complaining of severe swallowing difficulty. She was not hoarse and had no pain.

Physical examination revealed a firm, palpable neck mass fixated on the right (figure 1, A). Laryngeal endoscopy showed normal vocal fold movement and a projection of the posterior pharyngeal wall. Magnetic resonance imaging (MRI) revealed an 11-cm mass extending from the right to the left sternocleidomastoid (SCM) muscles (figure 1, B). Surgery was scheduled, with the goal to resect as much tumor as possible and to refer the patient for postoperative radiotherapy.

During surgery, a nasogastric tube was placed as a palpable guide to the esophagus. A right hockey-stick incision, including the previous thyroidectomy incision, was made. After elevation of the subplatysmal flaps, the tumor was found completely occupying the space between the right SCM and the larynx. It was dissected away from the SCM, the larynx, the cervical spine, and the esophagus. The right common carotid was surrounded by the tumor but was palpated and used as a landmark (figure 2). The internal jugular vein was saved, but the right inferior laryngeal nerve was sacrificed. An attachment to the right cricothyroid muscle was noticed as a possible point of tumor origin.

Postoperatively, the patient had bilateral vocal fold palsy, but movement of the left vocal fold returned to normal 3 days later. She also had Horner syndrome on the right side, which improved after 3 months. Her swallowing difficulty immediately resolved, and she was discharged on the fifth day. One month later, a follow-up MRI showed no sign of tumor presence, and it was decided to wait before any additional treatment was undertaken.

Two years later, the patient remains disease-free (figure 3), so she is considered cured. Histology showed the lesion to have the morphologic aspects of extra-abdominal fibromatosis.

Desmoid tumors are benign musculoaponeurotic tumors with the potential to strangle surrounding structures. They are caused by mutations of fibroblast cells (1) and do not metastasize. Their recurrence rates range between 20 and 77%. (2)

Treatment is surgical resection. (3) External beam radiation or systemic drug treatment may be used when total resection is impossible. A history of prior trauma or surgery at the site of the tumor may be elicited. However, these tumors are considered a neoplastic rather than an inflammatory reactive process. (4)

This is the third case of a desmoid tumor after thyroidectomy, (5,6) the second after total thyroidectomy, (5) and, to the best of our knowledge, the first with such a large tumor to be completely cured by surgery.

Alexander Delides, MD, PhD; Ioannis Plioutas, MD; Stephanos Konstantoudakis, MD; Pavios Maragoudakis, MD, PhD

From the 2nd Otolaryngology Department (Dr. Delides, Dr. Plioutas, and Dr. Maragoudakis) and the 2nd Department of Pathology (Dr. Konstantoudakis), Attikon University Hospital, Athens, Greece.


(1.) Ali R, Parthiban N, O'Dwyer T. Desmoid fibromatosis of submandibular region. J Surg Tech Case Rep 2014;6(1):21-5.

(2.) Hoos A, Lewis JJ, Urist MJ, et al. Desmoid tumors of the head and neck--a clinical study of a rare entity. Head Neck 2000;22(8):814-21.

(3.) Merchant NB, Lewis JJ, Woodruff JM, et al. Extremity and trunk desmoid tumors: A multifactorial analysis of outcome. Cancer 1999;86(10):2045-52.

(4.) de Bree E, Zoras O, Hunt JL, et al. Desmoid tumors of the head and neck: A therapeutic challenge. Head Neck 2014;36(10):1517-26.

(5.) Arena S, Salamone S, Cianci R, et al. Aggressive fibromatosis of the neck initiated after thyroidectomy. J Endocrinol Invest 2006;29(1):78-81.

(6.) Wang CP, Chang YL, Ko JY, et al. Desmoid tumor of the head and neck. Head Neck 2006;28(11):1008-13.

Caption: Figure 1. A: Photo shows the appearance of the neck, with enlargement of the right side and obliteration of the space between the sternocleidomastoid muscle and the larynx. The scar of the previous thyroidectomy is visible. B: T2-weighted MRI of the neck reveals the tumor occupying the space between the larynx and the spine and extending between the large vessels on both sides. The right carotid is enclosed within the tumor.

Caption: Figure 2. Intraoperative view shows the tumor being opened to reveal the common carotid. The sternocleidomastoid muscle is retracted posteriorly and the larynx anteriorly.

Caption: Figure 3. T1-weighted MRI of the neck 2 years after surgery reveals no presence of tumor.
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Author:Delides, Alexander; Plioutas, Ioannis; Konstantoudakis, Stephanos; Maragoudakis, Pavlos
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Apr 1, 2018
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