Ann Thorac Surg 2007;84:1752-1754. doi:10.1016/j.athoracsur.2007.06.008
© 2007 The Society of Thoracic Surgeons
Case Reports
Desmoid Tumor Presenting as a Superior Sulcus Tumor: A Unique Bone Change in the Vertebral Body
Ryuhei Hara, MDa,
Haruhisa Matsuguma, MDa,*,
Haruko Suzuki, MDa,
Yoshinori Ishikawa, MDa,
Rie Nakahara, MDa,
Takehiko Yamaguchi, MDc,
Kaoru Hirabayashi, MDb
a Division of Thoracic Surgery, Tochigi Cancer Center, Utsunomiya, Tochigi, Japan
c Division of Pathology, Tochigi Cancer Center, Utsunomiya, Tochigi, Japan
b Department of Surgical Pathology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
Accepted for publication June 5, 2007.
* Address correspondence to Dr Matsuguma, Division of Thoracic Surgery, Tochigi Cancer Center, 4-9-13 Yohnan, Utsunomiya, Tochigi, 320-0834, Japan (Email: hmatsugu{at}tcc.pref.tochigi.jp).
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Abstract
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Desmoid tumor, also referred to as aggressive fibromatosis, is a relatively rare, locally infiltrative, histologically benign tumor. This report details a case of desmoid tumor presented as a superior sulcus tumor, which showed a unique manifestation in the vertebral bodies on computed tomography.
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Introduction
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Desmoid tumor is a rare, benign neoplasm that frequently behaves in an aggressive manner. Several radiographic findings have been reported that are helpful in the diagnosis and assessment of tumor extent prior to surgery. We experienced a case of desmoid tumor presenting a unique bone change on computed tomography that has never been reported before.
A 50-year-old Japanese man without any history of traumatic injuries or surgical operations was referred to the thoracic surgery department at our hospital with pain in his left shoulder. The pain had been present for the past 2 years and was gradually getting worse. A roentgenogram of the chest showed a mass at the level of the apex of the left lung. Review of the chest x-ray film taken 2 years earlier revealed a slight enlargement of soft tissue density area in the same location.
Examination of the blood showed no abnormality, and no neurologic disturbance was detected on physical examination at the time of presentation.
Contrast-enhanced computed tomography through the chest showed a large, well-defined, homogenously enhanced mass (measuring 6.0 x 5.5 cm) in the left thoracic inlet. The mass had involved the left subclavicular artery. It extended very close to the neural foramina. However, invasion beyond the foramina was not detected. Spiculation of the ribs adjacent to the mass was detected, which is considered to be a characteristic manifestation of the desmoid tumor known as "frond-like spicules." In addition to this, we observed unique findings in the vertebral bodies that looked like small caves rather than erosion which can be occasionally observed in desmoid tumors (Fig 1). These two caves showed marginal sclerosis and were located at the side of the third and fourth vertebral bodies that were extensively attached by the tumor. The mass showed a signal intensity similar to that of muscle on T1-weighted magnetic resonance imaging and a slightly high-signal intensity on T2-weighted image. The intensity of the area in the small cave was similar to that of the main tumor near the cavities. Consequently, we believed that these caves were caused by the tumor.
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Fig 1. Computed tomographic scan of the chest on the bone specific window shows unique small caves in the (A) third and (B) fourth vertebral body.
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Computed tomographic-guided percutaneous needle biopsy and histopathologic examination showed a proliferation of benign-appearing spindle cells separated by collagen, which was compatible with a desmoid tumor.
Considering information based on all examinations, the patient selected conservative treatment instead of aggressive surgical treatment. The patient was first treated with tamoxifen and a nonsteroidal anti-inflammatory drug, and then with imatinib mesylate; however, these treatments were unsuccessful. Then he received radiation therapy with a total dose of 60 Gy. The radiation therapy relieved the patient’s pain, and the tumor showed gradual signs of shrinkage 1 year after the completion of therapy.
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Comment
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Desmoid tumor, also known as aggressive fibromatosis, is a relatively rare, nonmetastasizing but locally infiltrative benign neoplasm. It arises principally from the connective tissue of muscle and the overlying fascia or aponeurosis, and may be located in a variety of anatomic locations. It most commonly arises in the extremities (42% to 51%), with involvement of the chest wall in 10% to 28%, abdominal wall (12% to 22%), and so on. The lesion presents as a soft tissue mass that interrupts the surrounding tissue planes and occasionally encroaches on adjacent bones and causes pressure erosion or superficial defects of the cortex. There have been a few reports that include detailed description of bone involvement of desmoid tumor such as lysis or erosion demonstrated on computed tomography [1–3]. One characteristic bone change is "frond-like spicules" of bone radiating into a tumor that was reflected by periosteal reaction, as can be seen in the present case [3]. In addition to this finding, computed tomography showed two small caves on the side plain of the consecutive vertebral body that were extensively attached by the tumor. We believe that this unique finding has never been reported before now. Evidence indicated that these caves may have resulted from the tumor. However, the caves do not look like contact erosion, as can usually be observed in desmoid tumor or bone destruction caused by usual malignant tumor [3, 4]. We attribute this manifestation to the desmoid tumor’s unique characteristics, which can behave as both benign and malignant.
Although there are controversies regarding the treatments of desmoid tumor, wide local excision of the tumor is believed to be a better therapeutic option as long as it is feasible [5]. However, superior sulcus tumor is often difficult to carry out wide local excision because of its anatomical situation. Few cases of desmoid tumor presented as superior sulcus tumor were reported [5, 6]. In the Dashiell’s series, all three superior sulcus desmoid tumor involved brachial plexus and resulted in incomplete resection [6]. A high incidence of local recurrence in cases with positive surgical margin has been reported [5]. Therefore, it is of great importance to define the extent of tissue involvement to achieve complete excision and local control of the tumor. Radiation therapy is an effective alternative for patients who are not good surgical candidates, for those who decline surgery, and for those whom surgical morbidity would be excessive [7]. It has also been reported that conservative treatments with hormonal agents, nonsteroidal anti-inflammatory drugs, cytotoxics, interferons, and imatinib mesylate have demonstrated activity against desmoid tumor either alone or in some combination [8]. However, our patient did not show any effect with some of these drugs in regard to both symptoms and tumor size, and has been successfully treated with irradiation.
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References
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- Abramowitz D, Zornoza J, Ayala AG, et al. Soft-tissue desmoid tumors: radiographic bone changes Radiology 1983;146:11-13.[Abstract/Free Full Text]
- Hudson TM, Vandergriend RA, Springfield DS, et al. Aggressive fibromatosis: evaluation by computed tomography and angiography Radiology 1984;150:495-501.[Abstract/Free Full Text]
- Abbas AE, Deschamps C, Cassivi SD, et al. Chest-wall desmoid tumors: results of surgical intervention Ann Thorac Surg 2004;78:1219-1223.[Abstract/Free Full Text]
- Dashiell TG, Payne WS, Hepper NG, et al. Desmoid tumors of the chest wall Chest 1978;74:157-162.[Medline]
- Nuyttens JJ, Rust PF, Thomas Jr CR, et al. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles Cancer 2000;88:1517-1523.[Medline]
- Janinis J, Patriki M, Vini L, et al. The pharmacological treatment of aggressive fibromatosis: a systematic review Ann Oncol 2003;14:181-190.[Abstract/Free Full Text]
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Abstract
Introduction
