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Case report

For citation purposes: Lee JY, Jeong YJ, Kwon H, Jung SN. Extra-abdominal desmoid tumour presented as neck swelling in the trapezius muscle. Head Neck Oncol. 2012 Sep 9;4(2):53.

Copyright © 2012 OA Publishing London

AbstractA desmoid tumour is a type of benign fibromatosis arising from musculo-aponeurotic structures. It is clinico-pathologically classified into two subtypes, intra- and extra-abdominal, and it is mainly developed in the ab-domen. This report aims to describe a rare case of a desmoid tumour de-veloped in the trapezius muscle ac-companying neck swelling.

IntroductionA desmoid tumour is a type of benign fibromatosis and can be classified into intra- and extra-abdominal tu-mours1,2. Extra-abdominal tumours commonly develop in the shoulder, pelvis and chest wall. This tumour can be found in all age groups, but it is more prevalent in young female adults3.

There are no specific symptoms re-lated to the tumour and it is mostly painless and shows swelling at the early stage. As the tumour progresses, variable symptoms such as pain, bowel obstruction, urinary obstruction and limitation of range of joint movement develop depending on the location of the tumour4. In this case report, along with a literature review, we have de-scribed a rare case of a desmoid tu-mour developed in the trapezius muscle accompanying neck swelling.

Case reportA 23-year-old female patient visited the outpatient department with a

Extra-abdominal desmoid tumour presented as neck swelling in the trapezius muscle

JY Lee, YJ Jeong, H Kwon, SN Jung*

chief complaint of left posterior neck swelling that started to develop about seven months prior to visit (Figure 1).

Computed tomography (CT) im-ages showed a round soft tissue tu-mour in the left trapezius muscle, with a comparatively homogeneous density of the mass and isodensity of the trapezius muscle (Figure 2).

The mass including the trapezius muscle was removed with a 2 cm safety margin and it was 4 × 3 × 3 cm in size. The resection surface was light grey or yellow in colour, and it was a

relatively homogeneous solid mass containing a densely trabeculated area (Figure 3). Histopathological findings showed bundles of fibro-blasts and large amounts of collagen indicating an extra-abdominal des-moid tumour (Figure 4).

The patient is on an 18-month follow-up study after the operation, and there are no signs of recurrence or metastasis.

DiscussionA desmoid tumour was first described in 1832 by John MacFarlane as arising from the anterior abdominal wall and it is a rare disease, with a reported prevalence of 2–4 in 1,000,000 per year3. The tumour may be located in the abdominal wall, extremities and abdominal mesenteric. Fifty percent of the tumours are found in the ab-dominal wall and trunk muscle lay-ers, 40% develop in the extremities and 10% are located in the abdominal mesenteric. The most common loca-tions apart from the abdomen are the shoulder and pelvis3.

* Corresponding author Email: jsn7190@catholic.ac.kr

Department of Plastic Surgery, Uijeongbu St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Uijeongbu City, Republic of Korea

Figure 1: Clinical presentation. Mild soft tissue swelling 5 × 5 cm in size on the left posterior neck area.

Figure 2: Neck CT findings. Neck CT showed a relatively homogenous solid mass 4 × 3 × 3 cm in size.

Figure 3: Gross photo of the excised mass. The excised mass had a grey-whitish or a pale yellowish section and a densely trabeculated area.

For citation purposes: Lee JY, Jeong YJ, Kwon H, Jung SN. Extra-abdominal desmoid tumour presented as neck swelling in the trapezius muscle. Head Neck Oncol. 2012 Sep 9;4(2):53.

Copyright © 2012 OA Publishing London

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Case report

A desmoid tumour is a mass with a hard rubbery light pink surface and unclear borderlines. It infiltrates as it progresses and there are no charac-teristic symptoms that are directly related to the tumour. It is usually painless and swelling can be ob-served at the early stage of the disease, and other symptoms can de-velop depending on the location and the extension of invasion into sur-rounding areas of the tumour. As mentioned, the diagnosis can be chal-lenging due to the lack of symptoms in the early stages4.

The genesis of a desmoid tumour is as yet unknown, but endocrinologic hormonal aetiology, previous trauma and gene mutation are believed to be the causative factors4,5. Supporting these facts, there are published re-search results indicating that the prevalence is high in women of child-bearing age the growth of the tumour is accelerated during pregnancy and dystrophic changes of the tumour have been observed at menopause. It also often develops in the areas of previous surgery or injury sites 30%–80% of the tumour reportedly develops in the areas of previously operated sites4. Recently, it has also been reported that the onset of the tumour is related to adenomatous polyposis coli and β-catenin gene mutations5.

The diagnosis can be challenging because the tumour itself does not present any characteristic symptoms but early swelling is the main charac-teristic of the disease and a desmoid tumour should be clinically sus-pected if there are any related caus-ative factors4.

Histologically, this tumour con-tains identical shapes of well- differentiated fibroblasts, collagen, fusiform nucleus and light chromatin structures4,6. The micronucleus is not clear and mitosis can be observed, but indeterminate mitosis cannot be seen. When these histological findings are observed, it must be differentiated from low-grade fibro-sarcoma. It is very important to dif-ferentiate the disease from low-grade fibrosarcoma as the borderline of a desmoid tumour is unclear and infil-trates into the surrounding tissues with high recurrence rates. The uni-form growth pattern, maturity of tu-mour cell and low-grade of mitosis are the histological differentiating points. Low-grade fibrosarcoma, inflam matory pseudotumor, other fi-bromatosis and scar hypertrophy should be considered as a differential diagnosis6.

A desmoid tumour can easily infil-trate into the surrounding tissues and shows a high recurrence rate of 25%–65%, but metastasis is rare4.

Radical total resection with a >2 cm safety margin is recommended to treat the disease, and CT and mag-netic resonance image (MRI) before the surgery provides vital informa-tion to understand the extension of the tumour and its relationship with the surrounding anatomical struc-tures7,8. Reoperation is recommended in the case of recurrence or incom-plete resection and drug therapy or radiotherapy is further recom-mended if radical excision or reoper-ation is not possible7,9.

Tamoxifen, antiprostaglandin (su-lindac), goserelin acetate, interferon, doxorubicin, dacarbazine, actinomy-cin D, vincristine and c-adenosine 3′5′ cyclic monophosphate (c-AMP) modulators (ascorbic acid, theophyl-line, testolactone) are used in drug therapy10–12. Radiotherapy can be se-lectively used in the case of recur-rence or tumour invasion to nerves such as the brachioplexus.

ConclusionIn this case report, along with a liter-ature review a rare case of a desmoid tumour developed in the trapezius muscle accompanying neck swelling-was described.

Abbreviations listc-AMP, c-adenosine 3′5′ cyclic mono-phosphate; CT, computed tomogra-phy; MRI, magnetic resonance image.

ConsentWritten informed consent was ob-tained from the patient for publica-tion of this Case report and any accompanying images.

References1. Khorsand J, Karakousis CP. Desmoid tu-mors and their management. Am J Surg. 1985 Feb;149(2):215–8.2. Kiel KD, Suit HD. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer. 1984 Nov; 54(10):2051–5.3. Reitamo JJ, Häyry P, Nykyri E, Saxén E. The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the

Figure 4: Histological findings of the mass. (a) Histologic analysis of haematoxylin and eosin stain (×100) demonstrated high cellularity of a well-differentiated spindle-shaped fibroblast with many collagen fibres. (b) Haematoxylin and eosin stain (×400) showed several typical mitoses.

(a) (b)

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For citation purposes: Lee JY, Jeong YJ, Kwon H, Jung SN. Extra-abdominal desmoid tumour presented as neck swelling in the trapezius muscle. Head Neck Oncol. 2012 Sep 9;4(2):53.

Copyright © 2012 OA Publishing London

Case report

10. Duffaud F, Le Cesne A. Imatinib in the treatment of solid tumours. Target Oncol. 2009 Jan;4(1):45–56.11. Tonelli F, Ficari F, Valanzano R, Brandi ML. Treatment of desmoids and mesen-teric fibromatosis in familial adenoma-tous polyposis with raloxifene. Tumori. 2003 Jul-Aug;89(4):391–6.12. Leithner A, Schnack B, Katterschafka T, Wiltschke C, Amann G, Windhager R, et al. Treatment of extra-abdominal desmoid tumors with interferon-alpha with or without tretinoin. J Surg Oncol. 2000 Jan; 73(1):21–5.

Geburtshilfe Frauenheilkd. 1995 Mar; 55(3):173–5.7. Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF. The enigma of desmoid tumors. Ann Surg. 1999 Jun; 229(6):866–72.8. Pairolero PC, Arnold PG. Chest wall tu-mors. Experience with 100 consecutive patients. J Thorac Cardiovasc Surg. 1985 Sep;90(3):367–72.9. Sherman NE, Romsdahl M, Evans H, Zagars G, Oswald MJ. Desmoid tumors: a 20-year radiotherapy experience. Int J Radiat Oncol Biol Phys. 1990 Jul;19(1):37–40.

Finnish population. Am J Clin Pathol. 1982 Jun;77(6):665–73.4. Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol. 2001 Dec;27(8):701–6.5. Tajima S, Hironaka M, Oshikawa K, Bando M, Ohno S, Saito K, et al. Intrathoracic sporadic desmoid tumor with the beta-catenin gene mutation in exon 3 and activated cyclin D1. Respiration. 2006;73(4):558–61.6. Schwickerath J, Künzig HJ. Spontaneous malignant transformation of extra- abdominal fibromatosis to fibrosarcoma.