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Desmoid tumor of the chest wall
European Journal of Radiology Extra 46 (2003) 119 /121 http://intl.elsevierhealth.com/journals/ejrx
Desmoid tumor of the chest wall I˙smail Yurtsever a, Ugur Topal a,*, Ramazan Yalc¸in a, S¸aduman Balaban Adım b, Sami Bayram c a
Department of Radiology, Uludag University Medical School, Gorukle Campus, 16059 Bursa, Turkey Department of Pathology, Uludag University Medical School, Gorukle Campus, 16059 Bursa, Turkey c Department of Thoracic Surgery, Uludag University Medical School, Gorukle Campus, 16059 Bursa, Turkey b
Received 7 May 2002; received in revised form 12 July 2002; accepted 16 July 2002
Abstract Desmoid tumors are low-grade fibrosarcomas that do not metastasize but are frequently associated with one or more recurrences and subsequent associated morbidity. Desmoid tumors of the chest wall are rare and have been described mainly in single case reports or as isolate cases in large series of extra-abdominal desmoid tumors. We report a case of desmoid tumor in the chest wall mimicking superior sulcus tumor. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Desmoid tumor; Chest wall; CT
1. Introduction A 20-year-old man was referred to our department for transthoracic biopsy of the right apical mass from another hospital. He had a 1-year history of a slowly enlarging, painless mass in the right supraclavicular region. He had had difficulty on bending the neck to the right and abducting the right arm over 708. For his arm and shoulder problems he had physiotherapy but his complaints were continued. He did not have history of trauma or surgery. Chest X-rays revealed a well-defined right apical mass (Fig. 1). On noncontrast CT scans (Somatom Plus 4, Siemens, Erlangen, Germany), a sharply marginated mass extending from supraclavicular region into the right hemithorax was seen. There was destruction of the adjacent ribs. CT guided transthoracic biopsy was performed with 18 gauge half automated trucut needle. Pathologic result was reported as nondiagnostic fibrotic tissues. Before the operation in order to see extension of the mass and the relation with the subclavian artery MRI (Magnetom Vision, Siemens, Erlangen, Germany) was done. MRI demonstrated the
* Corresponding author. Tel.: /90-224-442-8845; fax: /90-224442-8142. E-mail address: [email protected] (U. Topal).
right subclavian artery was compressed by the tumor (Fig. 2). On operation, mass was adherent to the first, second and third ribs and resected with the ribs. However, the extrathoracic component was not resected totally. Pathologic diagnosis was desmoid tumor. Patient received radiation therapy for the residual tumor but local recurrence was detected 6 months after the operation.
2. Discussion Desmoid tumors, also known as fibromatosis, are true neoplasms that arise from fascial and musculoaponeurotic coverings, sometimes at the site of a traumatic or postsurgical scar [1]. They may occur in a variety of sites but most commonly arise in extremity locations [1]. Chest wall is the origin in the 10 /28% of the patients [2]. Although rare, desmoid tumors have been reported to be one of the most common chest wall sarcomas [3]. Desmoid tumors at all sites are more frequent in women then in men [4,5]. The cause is unknown, but an association with trauma, pregnancy, estrogenic hormones, and Gardner’s syndrome has been reported [1,2,4].
1571-4675/03/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. doi:10.1016/S1571-4675(03)00061-0
120
I˙. Yurtsever et al. / European Journal of Radiology Extra 46 (2003) 119 /121
Fig. 2. T2-weighted Turbo spin echo image shows compression of the right subclavian artery by the tumor.
Fig. 1. (a) Chest x-ray showing sharply defined right apical mass. (b) Noncontrast CT scan, demonstrated sclerotic changes and destruction of the upper ribs.
The desmoid tumors are nonencapsulated, poorly circumscribed and infiltrative, aggressive fibromatosis grows insidiously and invades locally [1]. Histologically, the tumor exhibits a very uniform appearance, with well-differentiated fibroblasts and associated production of mature collagen [4]. Cytologic features of malignancy are lacking. The tumors generally do not metastasize, and although they are locally invasive into surrounding skeletal muscle and fat, they usually spare vital anatomic structures. Notable exceptions were reported in apical tumors, all of which became entangled in the brachial plexus [4].
Chest wall desmoids usually present with a palpable mass and pain. Nerve involvement can give rise to sensory and motor symptoms in the distribution of the nerve. Most are detectable on chest roentgenogram and may show a soft-tissue mass associated with ribs, scapula or paravertebral tissues possibly with periosteal reactive changes or bony destruction [1,2,5,6]. On CT scans, tumors have a variable attenuation similar to that of muscle and they may enhance after contrast administration [5,6]. CT scan usually reveals the size and location more precisely however, MR imaging is probably more sensitive in detecting soft tissue infiltration and evidence of local recurrence [5,6]. Signal intensity similar to that of muscle is seen on T1-weighted MR images, and variable signal intensity is seen on T2weighted MR images [6]. Definitive diagnosis requires histopathology. Transthoracic needle biopsy may not be sufficient because of the relative hypocellularity of the tumor. In our case, apical location of the tumor mimicked the Pancoast tumor. But the radiological features like sharp contour lead us to consider a kind of benign tumor. MRI demonstrated the lesion extension and relation with the adjacent structures more precisely. Additionally compression of the subclavian artery was demonstrated better by MRI. In our case, CT guided transthoracic biopsy was insufficient for the diagnosis as reported in many cases in the literature. The treatment of the desmoid tumors is wide resection. Mortality is low, but local recurrences are frequent
I˙. Yurtsever et al. / European Journal of Radiology Extra 46 (2003) 119 /121
(18 /54%) [3,7]. Local recurrence rate is common in patients with age of 30 years or younger [3]. The role of radiotherapy for desmoid tumors is not established due to the small number of the patients.
References [1] Ackman JB, Whitman GJ, Chew FS. Aggressive fibromatosis. Am J Roentgenol 1994;163:544.
121
[2] Mobeen I, Rossoff LJ, Kahn L, Lackner RP. Intrathoracic desmoid tumor mimicking primary lung neoplasm. Ann Thorac Surg 2001;71:1698 /700. [3] Brodsky JT, Gordon MS, Hajdu SI, Burt M. Desmoid tumors of the chest wall. J Thorac Cardiovasc Surg 1992;104:900 /3. [4] Dashiell TG, Payne WS, Hepper NGG, Soule EH. Desmoid tumors of the chest wall. Chest 1978;74:157 /62. [5] Klein DL, Gamsu G, Gant TD. Intrathoracic desmoid tumor of the chest wall. Am J Roentgenol 1977;129:524 /5. [6] Munden RF, Kemp BL. Desmoid tumor of the chest wall. Am J Roentgenol 1999;172:1149. [7] Kabiri EH, Aziz SA, El Maslout A, Benosman A. Desmoid tumors of the chest wall. Eur J Cardio-thorac Surg 2001;19:580 /3.
Desmoid tumor of the chest wall I˙smail Yurtsever a, Ugur Topal a,*, Ramazan Yalc¸in a, S¸aduman Balaban Adım b, Sami Bayram c a
Department of Radiology, Uludag University Medical School, Gorukle Campus, 16059 Bursa, Turkey Department of Pathology, Uludag University Medical School, Gorukle Campus, 16059 Bursa, Turkey c Department of Thoracic Surgery, Uludag University Medical School, Gorukle Campus, 16059 Bursa, Turkey b
Received 7 May 2002; received in revised form 12 July 2002; accepted 16 July 2002
Abstract Desmoid tumors are low-grade fibrosarcomas that do not metastasize but are frequently associated with one or more recurrences and subsequent associated morbidity. Desmoid tumors of the chest wall are rare and have been described mainly in single case reports or as isolate cases in large series of extra-abdominal desmoid tumors. We report a case of desmoid tumor in the chest wall mimicking superior sulcus tumor. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Desmoid tumor; Chest wall; CT
1. Introduction A 20-year-old man was referred to our department for transthoracic biopsy of the right apical mass from another hospital. He had a 1-year history of a slowly enlarging, painless mass in the right supraclavicular region. He had had difficulty on bending the neck to the right and abducting the right arm over 708. For his arm and shoulder problems he had physiotherapy but his complaints were continued. He did not have history of trauma or surgery. Chest X-rays revealed a well-defined right apical mass (Fig. 1). On noncontrast CT scans (Somatom Plus 4, Siemens, Erlangen, Germany), a sharply marginated mass extending from supraclavicular region into the right hemithorax was seen. There was destruction of the adjacent ribs. CT guided transthoracic biopsy was performed with 18 gauge half automated trucut needle. Pathologic result was reported as nondiagnostic fibrotic tissues. Before the operation in order to see extension of the mass and the relation with the subclavian artery MRI (Magnetom Vision, Siemens, Erlangen, Germany) was done. MRI demonstrated the
* Corresponding author. Tel.: /90-224-442-8845; fax: /90-224442-8142. E-mail address: [email protected] (U. Topal).
right subclavian artery was compressed by the tumor (Fig. 2). On operation, mass was adherent to the first, second and third ribs and resected with the ribs. However, the extrathoracic component was not resected totally. Pathologic diagnosis was desmoid tumor. Patient received radiation therapy for the residual tumor but local recurrence was detected 6 months after the operation.
2. Discussion Desmoid tumors, also known as fibromatosis, are true neoplasms that arise from fascial and musculoaponeurotic coverings, sometimes at the site of a traumatic or postsurgical scar [1]. They may occur in a variety of sites but most commonly arise in extremity locations [1]. Chest wall is the origin in the 10 /28% of the patients [2]. Although rare, desmoid tumors have been reported to be one of the most common chest wall sarcomas [3]. Desmoid tumors at all sites are more frequent in women then in men [4,5]. The cause is unknown, but an association with trauma, pregnancy, estrogenic hormones, and Gardner’s syndrome has been reported [1,2,4].
1571-4675/03/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. doi:10.1016/S1571-4675(03)00061-0
120
I˙. Yurtsever et al. / European Journal of Radiology Extra 46 (2003) 119 /121
Fig. 2. T2-weighted Turbo spin echo image shows compression of the right subclavian artery by the tumor.
Fig. 1. (a) Chest x-ray showing sharply defined right apical mass. (b) Noncontrast CT scan, demonstrated sclerotic changes and destruction of the upper ribs.
The desmoid tumors are nonencapsulated, poorly circumscribed and infiltrative, aggressive fibromatosis grows insidiously and invades locally [1]. Histologically, the tumor exhibits a very uniform appearance, with well-differentiated fibroblasts and associated production of mature collagen [4]. Cytologic features of malignancy are lacking. The tumors generally do not metastasize, and although they are locally invasive into surrounding skeletal muscle and fat, they usually spare vital anatomic structures. Notable exceptions were reported in apical tumors, all of which became entangled in the brachial plexus [4].
Chest wall desmoids usually present with a palpable mass and pain. Nerve involvement can give rise to sensory and motor symptoms in the distribution of the nerve. Most are detectable on chest roentgenogram and may show a soft-tissue mass associated with ribs, scapula or paravertebral tissues possibly with periosteal reactive changes or bony destruction [1,2,5,6]. On CT scans, tumors have a variable attenuation similar to that of muscle and they may enhance after contrast administration [5,6]. CT scan usually reveals the size and location more precisely however, MR imaging is probably more sensitive in detecting soft tissue infiltration and evidence of local recurrence [5,6]. Signal intensity similar to that of muscle is seen on T1-weighted MR images, and variable signal intensity is seen on T2weighted MR images [6]. Definitive diagnosis requires histopathology. Transthoracic needle biopsy may not be sufficient because of the relative hypocellularity of the tumor. In our case, apical location of the tumor mimicked the Pancoast tumor. But the radiological features like sharp contour lead us to consider a kind of benign tumor. MRI demonstrated the lesion extension and relation with the adjacent structures more precisely. Additionally compression of the subclavian artery was demonstrated better by MRI. In our case, CT guided transthoracic biopsy was insufficient for the diagnosis as reported in many cases in the literature. The treatment of the desmoid tumors is wide resection. Mortality is low, but local recurrences are frequent
I˙. Yurtsever et al. / European Journal of Radiology Extra 46 (2003) 119 /121
(18 /54%) [3,7]. Local recurrence rate is common in patients with age of 30 years or younger [3]. The role of radiotherapy for desmoid tumors is not established due to the small number of the patients.
References [1] Ackman JB, Whitman GJ, Chew FS. Aggressive fibromatosis. Am J Roentgenol 1994;163:544.
121
[2] Mobeen I, Rossoff LJ, Kahn L, Lackner RP. Intrathoracic desmoid tumor mimicking primary lung neoplasm. Ann Thorac Surg 2001;71:1698 /700. [3] Brodsky JT, Gordon MS, Hajdu SI, Burt M. Desmoid tumors of the chest wall. J Thorac Cardiovasc Surg 1992;104:900 /3. [4] Dashiell TG, Payne WS, Hepper NGG, Soule EH. Desmoid tumors of the chest wall. Chest 1978;74:157 /62. [5] Klein DL, Gamsu G, Gant TD. Intrathoracic desmoid tumor of the chest wall. Am J Roentgenol 1977;129:524 /5. [6] Munden RF, Kemp BL. Desmoid tumor of the chest wall. Am J Roentgenol 1999;172:1149. [7] Kabiri EH, Aziz SA, El Maslout A, Benosman A. Desmoid tumors of the chest wall. Eur J Cardio-thorac Surg 2001;19:580 /3.