Eur J Cardiothorac Surg 2005;27:353-355
© 2005 Elsevier Science NL
Calcifying fibrous pseudotumor causing thoracic outlet syndrome
Soon-Ho Chona,
Chul Burm Leea,*,
Young-Ha Ohb
a Department of Thoracic and Cardiovascular Surgery, Hanyang University Guri Hospital, Kyonggi Do, Kyomun Dong 249-1, Guri 471-701, South Korea
b Department of Pathology, Hanyang University Guri Hospital, Guri, South Korea
Received 13 September 2004;
received in revised form 4 November 2004;
accepted 8 November 2004.
* Corresponding author. Tel.: +82 31 560 2301; fax: +82 31 568 9948. (E-mail: cblee{at}hanyang.ac.kr).
 |
Abstract
|
|---|
Calcifying fibrous pseudotumor (CFP) is a relatively rare benign mass lesion that generally involves the soft tissues. We report a case of CFP occurring in an extremely rare location. The patient is a 34-year-old woman presenting symptoms of numbness of her right upper extremity caused by a CFP in her thoracic outlet and we discuss its clinical significance with a review of the literature.
Key Words: Thoracic outlet • Tumor, benign • Calcifying fibrous pseudotumor
|
1. Introduction
|
|---|
Although CFPs have recently been reported in various locations in the literature, to the best of our knowledge this is the only case of CFP causing thoracic outlet syndrome. We describe a case of CFP with impingement of her brachial plexus and subclavian artery causing symptoms of numbness in her right upper extremity. This tumor was successfully removed with a simple approach, sparing her clavicle and avoiding a more radical operation.
|
2. Case report
|
|---|
A 34-year-old woman visited our outpatient clinic with symptoms of 1-year onset of right upper extremity and shoulder numbness. Physical examination revealed a positive Adson test. The patient had a history of tuberculosis 15 years ago and had received antituberculous medication for 6 months. Simple chest X-rays revealed a calcified mass situated in the thoracic outlet between the first rib and clavicle. An osteochondroma with impingement over the subclavian artery was initially suspected of clavicle origin on computer tomographic scans (Fig. 1), but, a chest MRI confirmed no connection to the first rib or clavicle. A whole body bone scan was unremarkable. Her sedimentation rate was 17mm/h, rheumatoid factor and serum IgG were negative, and values for antinuclear antibody were weakly positive. Human leukocyte antigen typing revealed A2, B35,51, Cw3, DR4,15,51,53, DQ6,8,9, and DRB3,4,5. Unfortunately, an important ulnar nerve conduction velocity test was not performed.
View larger version (137K):
[in this window]
[in a new window]
|
Fig. 1. A three-dimensional recreated image showing impingement of the subclavian artery between the mass and the first rib. The mass (white arrow head) is situated within the thoracic outlet, between the first rib (black star) and clavicle (asterisk).
|
|
The operation commenced with the patient in a supine position with a slightly elevated right shoulder. The incision was made directly over the clavicle in its midportion to allow for a supraclavicular and infraclavicular approach. The platysma muscle was dissected off the clavicle and the attachments of the pectoralis major muscle were detached with electrocautery. The mass was movable and palpable in the supraclavicular area when the arm was adducted and palpable in the infraclavicular area when abducted. The mass was located under the subclavius muscle as confirmed by computer tomographic and MRI scans. The mass in the supraclavicular area was pushed and the same mass in the infraclavicular area was pulled until the mass could be excised. There were no osseous attachments to the rib or clavicle. The movable, non-fixed nature and its benign nature, allowed for a less invasive procedure that might have caused injury to the brachial plexus.
On gross examination, the mass measured at 4.6x2.7x1.0cm in dimension and weighed at 12.7g. The mass was well circumscribed, lobulated, and solid, but, non-encapsulated. When the mass was sectioned, a gritty texture was noted and the cut surface revealed a varied appearance with irregular calcification. Microscopically, there were areas of diffuse and extensive calcification with numerous psammomatous calcification and other areas that were densely collagenized (Fig. 2). In focal areas of the mass, there were minor foci of bone formation and patchy infiltrates of plasma cells and lymphocytes. Immunohistochemical stains using antibodies to CD34, desmin, smooth muscle actin (SMA), and anaplastic lymphoma kinase-1 were performed and all have presented negative results. The gross, microscopic, and immunohistochemical findings were compatible with CFP.
View larger version (150K):
[in this window]
[in a new window]
|
Fig. 2. Numerous psammomatous calcifications are a hallmark of this mass (hematoxylin and eosin, x200).
|
|
The patient's postoperative course was uneventful and was discharged on the 9th postoperative day. The patient is doing well at 7 month postoperative follow-up at our outpatient clinic with no limitation of motion and disappearance of her numbness.
|
3. Comment
|
|---|
Rosenthal and Abdul-Karim [1] had reported the first two cases of CFP in 1988 under the name of ‘childhood fibrous tumor with psammoma bodies’. Thereafter, an additional 10 more cases were reported 5 years later by Fetsch and associates [2] and the term calcifying fibrous tumor was first used. Since then, several cases have been reported in soft tissues of the trunk, limbs and neck, in the pleura, mediastinum, abdominal cavity, spermatic cord, and other strange areas such as in the orbit [3–6]. To the best of our knowledge, this is the first case occurring within the thoracic outlet.
There are many factors that may cause compression of the neurovascular bundle at the thoracic outlet. The most common etiology of thoracic outlet syndrome is deranged anatomy, among which bony abnormalities comprise 30%. Tumors causing thoracic outlet syndrome are rare. The clinical manifestations of thoracic outlet syndrome are generally caused by neurogenic origin, causing pain and paresthesias, motor weakness and occasionally atrophy of hypothenar and interosseous muscles (most commonly the ulnar type) [7].
The pathologic features of CFP are now well established. The lesion is characterized by a nonencapsulated, diffusely hyalinized mass composed of benign-appearing spindle cells intermixed with variably prominent chronic inflammatory infiltrate. The hallmark of the lesion is the presence of calcifications, which may be psammomatous, and/or dystrophic in type [5]. Immunohistochemically, the tumor shows only focal to absent expression of CD34 and nonspecific expression of alpha smooth muscle actin and desmin [3–5]. Anaplastic lymphoma kinase-1, a distinctive feature of inflammatory myofibroblastic tumor, is not a common feature for CFPs [6]. We had performed immunohistochemical stains using antibodies to CD34, anaplastic lymphoma kinase-1, desmin, and smooth muscle actin. In our case, the mass was composed of mostly collagenous stroma with almost no spindle cell proliferation; therefore, the results were all negative, except for CD34, which was seen only in the endothelial cells of blood vessels.
The differential diagnosis of such a benign lesion includes osteochondroma, solitary fibrous tumor, ectopic calcification of soft tissue, and inflammatory myofibroblastic tumor. The discrete nature of this case rules out the possibility of exostosis (osteochondroma). Solitary fibrous tumors are characterized by a patternless arrangement of spindle cells with alternating areas of hypercellularity and hypocellularity, and collagenous deposition. Calcification is not a hallmark of this tumor [6]. Inflammatory myofibroblastic tumors are composed of varying amounts of collagen fibers, myofibroblasts, and inflammatory cells arranged into a variety of histologic patterns. In contrast to CFPs, calcifications are again uncommon [8]. In our case, exuberant calcifications, psammomatous and dystrophic in type clearly delineate any confusion of possible diagnosis of ectopic calcification, solitary fibrous tumor, and inflammatory myofibroblastic tumor.
aVan Dorpe and associates [8] have reported a case that presents evidence of transitional stages of inflammatory myofibroblastic tumor and CFPs, suggesting that they are not separate entities, but, a continuum. Other reports stress that CFPs are a distinctive lesion and there are much further studies needed to clarify its origin. Cytogenetic studies have evidence of clonal chromosomal abnormalities seen in inflammatory myofibroblastic tumors involving the 2p23 region [9], the region containing the anaplastic lymphoma kinase-1 gene. But, cytogenetic studies in CFPs are difficult due to poor cellularity, as was in our case, and are lacking.
CFPs have recently been reported in the literature as a discrete benign lesion occurring in various locations. We report a case of CFP in an unusual location causing thoracic outlet syndrome. Simple local excision appears to be the treatment of choice due to its benign nature, good prognosis, and extremely low recurrence rate. Further studies must be done to delineate its origin.
|
References
|
|---|
- Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor with psammoma bodies. Clinicopathologic features in two cases. Arch Pathol Lab Med 1988;112:798-800.[Medline]
- Fetsch JF, Montgomery EA, Meis JM. Calcifying fibrous pseudotumor. Am J Surg Pathol 1993;17(5):502-508.[Medline]
- Ammar A, Hammami SE, Horchani H, Sellami N, Kilani T. Calcifying fibrous pseudotumor of the pleura: a rare location. Ann Thorac Surg 2003;76:2081-2082.[Abstract/Free Full Text]
- Dumont P, de Muret A, Skrobala D, Robin P, Toumieux B. Calcfying fibrous pseudotumor of the mediastinum. Ann Thorac Surg 1997;63:543-544.[Abstract/Free Full Text]
- Hill KA, Gonzalez-Crussi F, Chou PM. Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: a histological and immunohistochemical comparison. Mod Pathol 2001;14(8):784-790.[CrossRef][Medline]
- Nascimento AF, Ruiz R, Hornick JL, Fletcher CD. Calcifying fibrous ‘pseudotumor’. Int J Surg Path 2002;10(3):189-196.
- Sabiston DC, Spencer FC. Surgery of the chest. vol. 6. Philadelphia, PA: Saunders; 1995p. 613–33.
- Van Dorpe J, Ectors N, Geboes K, D'Hoore A, Scoit R. Is calcifying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor?. Am J Surg Pathol 1999;23(3):329-335.[CrossRef][Medline]
- Griffin CA, Hawkins AL, Dvorak C, Henkle C, Ellingham T, Perlman EJ. Recurent involvement of 2p23 in inflammatory myofibroblastic tumors. Cancer Res 1999;59:2776-2780.[Abstract/Free Full Text]
Top
Abstract
1. Introduction
