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Ann Thorac Surg 2006;82:1501-1504
© 2006 The Society of Thoracic Surgeons

Case Reports

Elastofibroma Dorsi as Differential Diagnosis in Tumors of the Thoracic Wall

Clemens Schafmayer, MDa, Volker Kahlke, MD, PhDa, Ivo Leuschner, MD, PhDb, Madhava Pai, MDc, Juergen Tepel, MDa,*

a Department for General Surgery and Thoracic Surgery, Kiel, Germany
b Institute for General Pathology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany
c Department of HPB Surgery, Hammersmith Hospital, London, United Kingdom

Accepted for publication October 17, 2005.

* Address correspondence to Dr Tepel, Department for General Surgery and Thoracic Surgery, University Hospital of Schleswig-Holstein, Campus Kiel Arnold-Heller-Strasse 7, Kiel, 24105 Germany (Email: apries{at}chirurgie-sh.de).


   Abstract
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 Abstract
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We report the case of a 73-year-old woman who presented with a soft tissue tumor located between the scapula and the rib cage. Magnetic resonance imaging showed an inhomogeneous tumor on the right dorsolateral thoracic wall that measured 7 x 4 x 7 cm with contrast enhancement. The findings were suggestive of partial infiltration of intercostal muscles and were suspicious of a malignant tumor. After local excision at a district hospital had failed to render definitive diagnosis, the patient underwent complete resection of the tumor at our institution. Histology from the specimen was consistent with elastofibroma dorsi with free resection margins.


   Introduction
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Soft tissue tumors apart from lipoma are rare tumors of the thoracic wall [1]. Elastofibroma dorsi is an important differential diagnosis for these tumors. It occurs mainly in women, commonly in the subscapular region, and is characterized by slow growth [2]. The pathogenesis remains unclear; however, abnormal degeneration of elastic fibers (eg, after repetitive local trauma as well as neoplastic mechanisms are being considered) [3, 4]. We present a case that illustrates typical difficulties in diagnosing these rare tumors.

A 73-year-old woman who had retired as a window cleaner, and who was otherwise fit and well, presented with a 6-month history of swelling between the tip of the scapula and the thoracic wall on the right side, which had moderately increased in size during this time period. She complained of increasing pain and resistance to movement on lifting the arm over her head. There was no history of trauma or malignancy, and there was no significant past medical history. On physical examination, the tip of the right scapula appeared prominent, and on elevating the right arm, a fist sized, nontender tumor was palpable. All her laboratory measurements were within normal range.

The magnetic resonance image showed an inhomogeneous tumor of the thoracic wall with contrast enhancement located medially to the right scapula that measured 7 x 4 x 7 cm in size. There were features of local infiltration suggestive of malignancy (Fig 1).


Figure 1
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  		Fig 1. Magnetic resonance image, coronary section. Elastofibroma dorsi appears as inhomogeneous formation of the thoracic wall (white arrow) below the scapula (black arrow).

 
In view of the magnetic resonance image findings, local excision was performed in a district hospital. Histology showed fibrolipoma tissue with regressive changes. Thereafter the patient was referred to our hospital and subsequently underwent complete resection through a posterior incision. During the subcutaneous dissection, the latissimus dorsi muscle seemed to have shifted inferolaterally and thinned. The tumor extended 5 cm under the right scapula and was fixed to the thoracic wall through a pedicle, which seemed to have infiltrated the surrounding tissue. The tumor was resected with clear surgical margins and minimum defect in the serratus anterior and latissimus dorsi muscles (Fig 2).


Figure 2
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  		Fig 2. Magnetic resonance image, transverse section. Elastofibroma dorsi (white arrow) is located under the scapula (black arrow).

 
The specimen measured 8 x 6 x 4 cm and histopathology (hematoxylin-eosin staining) showed a lesion composed of fibrotic connective tissue of low cellularity with strongly eosinophilic, partially swollen, and plump fibers intermingled with islets of mature fat tissue consistent with elastofibroma dorsi (Fig 3). Elastic stain (elastica van Giesson stain) showed deeply stained, branched, and unbranched fibers with margins serrated and irregular. The lesion extended into surrounding muscle fibers; however, the resection margins were free of tumor (R0 resection). The postoperative course was uneventful, and the patient was discharged on postoperative day 5.


Figure 3
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  		Fig 3. Hematoxylin and eosin staining with 20-fold magnification (large image) and van Giesson staining with 80-fold magnification (inset). Both images show fibrotic tissue with few cells and eosinophilic, partly swollen, and plump collagen fibers. In addition, the large image shows insular univascular fatty tissue.

 

   Comment
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Elastofibroma dorsi is a rare, benign, soft tissue tumor almost always located at the inferior pole of the scapula, deep to the serratus anterior muscle that was described by Järvi und Saxen [5] for the first time in 1961. It is extremely rare in children and is usually seen in patients of middle or old age, with an apparent peak in the female population beyond the sixth decade [6]. Bilateral, often asynchronous involvement occurs in 10%, whereas multiple distinct tumors (as many as 17) in different locations have also been described [2]. On physical examination, with shoulder and arm in the neutral position, a projecting inferior scapula is a common finding. On elevation of the arm, the tumor is often palpable with relative ease. Most patients present only after developing symptoms of pain and restriction of movement.

Nagamine and co-workers [6] in 1982 published the largest study on elastofibroma dorsi. They showed that out of the 170 patients studied, 55 belonged to one family whereas 158 were females, and the mean age of the study group was 70 years. All tumors described in this study (99%) were between the scapula and the rib cage. However, other sites have been reported in the literature [7–10]. A postmortem study revealed that most elastofibroma dorsi remain clinically inconspicuous. In 235 autopsies, 39 previously undiagnosed subscapular elastofibroma dorsi were found with an average size of 3 x 2 cm, and 18 of these were bilateral. All patients were older than 58 years of age [11].

Pathogenesis of elastofibroma dorsi still remains unclear; abnormal degeneration of elastic fibers after repetitive local trauma or excessive scapulo-thoracic motion has been postulated [4]. This may well have been the case in our patient, because professional window cleaning includes regular work above head level. Besides such environmental influences, there is evidence of cytogenetic and molecular genetic changes in elastofibroma dorsi. In one study, aberrations of the short arm of chromosome 1 in three cases and a translocation involving chromosomes 8 and 12 have been described [3]. This is normally not only observed in nonneoplastic tissues, but also in aggressive fibromatosis, and this indicates a possible neoplastic pathogenesis rather than a reactive process [3]. Comparative genomic hybridization of 27 elastofibroma dorsi showed DNA copy number changes in 33%. Eight of 6 cases showed gains at chromosomal location Xq12-22 and 19 [12]. Such findings support the hypothesis of a multistage pathogenic mechanism. In addition to these, a recent report about elastofibroma dorsi coexisting with a high-grade sarcoma has given reason to speculate a causal link between the two pathological entities [13].

Among the tumors of the thoracic wall, lipoma is the most common benign tumor, whereas elastofibroma dorsi, sarcomas, and metastases from other malignancies form the rest of the tumors. In most soft tissue neoplasms, diagnosis can confidently be made or is at least strongly suggestive on the basis of magnetic resonance imaging. Elastofibroma dorsi typically presents as a sickle-shaped mass of low signal intensity interspersed with areas of high signal intensity on T1-weighted and T2-weighted spin–echo–sequence (SE) images. It may also show marked contrast enhancement, whereas the presence of multiple cysts is not uncommon [14]. On ultrasound, elastofibroma dorsi usually appears as a hypoechoic mass with interspersed linear echogenicity resembling muscle [15]. Color and power Doppler may be helpful, but often both a clear cleavage surface and a specific vascular pattern are absent [16]. In most cases, such as the one presented here, the morphologic appearance therefore nourishes doubts about the benign nature of this tumor requiring biopsy.

Fine-needle aspiration represents the simplest and quickest method to obtain material for cytology, but correct diagnosis may be missed due to the nature of elastofibroma dorsi resulting in hypocellular smears [17]. Typical findings are mature adipocytes, fibroblasts, aggregates of petaloid globules within a collagenous matrix, and fern-like structures, revealing degenerative elastic fibers [18]. These altered elastic fibers show a green-yellow autofluorescence with ultraviolet light [19]. Immunohistochemically, elastofibroma dorsi is stained positively with vimentin but not with {alpha}-smooth muscle actin (SMA), S-100, desmin, or p53 [5]. Fine-needle aspiration features in correlation with radiologic, clinical, and electron microscopic findings without preoperative histology are believed to be sufficient for reliable diagnosis [20].

The recommended mode of treatment for elastofibroma dorsi is complete resection. The low recurrence rate in the study by Nagamine and coworkers [6] (1 in 170 cases) has been explained by radical excision, whereas a recurrence rate of 7% in another study has been attributed to incomplete resection [21]. Recent studies have questioned the general indication for surgical excision because clinical presentation and magnetic resonance findings are typical and because diagnosis of this entirely innocuous tumor can be easily confirmed by biopsy [22]. In our view, surgery for elastofibroma dorsi should be reserved for symptomatic patients and cases with an unclear diagnosis.


   References
Top
 Abstract
 Introduction
 Comment
 References
 

  1. Friemann J, Roessner A, Müller KM. Primary and secondary chest wall tumors from the pathologist's viewpoint Langenbecks Arch Chir 1987;372:757.[Medline]
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  3. McComb EN, Feely MG, Neff JR, Johannson SL, Nelson M, Bridge JA. Cytogenetic instability, predominantly involving chromosome 1, is characteristic of elastofibroma Cancer Genet Cytogenet 2001;126:68-72.[Medline]
  4. Kayaselcuk F, Demirhan B, Kayaselyuk U, Ozerdam OR, Tuncer I. Vimentin, smooth muscle actin, desmin, S-100 protein, p53 and estrogen receptor expression in elastofibroma and nodular fasciitis Ann Diagn Pathos 2002;6:94-99.
  5. Jarvi OH, Saxen E. Elastofibroma dorsi Acta Pathol Microbiol Scand 1961;51:83-84.
  6. Nagamine N, Nohara Y, Ito E. Elastofibroma in Okinawa Cancer 1982;50:1794-1805.[Medline]
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  8. Bae SJ, Shin MJ, Kim SM, Cho KJ. Intra-articular elastofibroma of the shoulder joint Skeletal Radiol 2002;31:171-174.[Medline]
  9. Saint-Paul MC, Musso S, Cardot-Leccia N, et al. Elastofibroma of the stomach Pathos Res Pract 2003;199:637-639.
  10. Potter TJ, Summerlin DJ, Rodgers SF. Elastofibroma: initial report in the oral mucosa Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97:64-67.[Medline]
  11. Jarvi OH, Länsimies PH. Subclinical elastofibromas in the scapular region in an autopsy series Acta Pathol Microbiol Scand 1975;83:87-108.
  12. Nishio JN, Iwasaki H, Ohjimi Y, et al. Gain of Xq detected by comparative genomic hybridization in elastfibroma Int J Mol Med 2002;10:277-280.[Medline]
  13. Alberghini M, Pignatti G, Zanella L, et al. Elastofibroma associated with high grade leiomyosarcoma of the soft tissues: a light and ultrastructural study of one case J Submicrosc Cytol Pathos 2003;35:43-48.
  14. Schick S, Zembsch A, Gahleitner A, et al. Atypical appearance of elastofibroma dorsi on MRI: case reports and review of the literature J Comput Assist Tomogr 2000;24:288-292.[Medline]
  15. Dalal A, Miller TT, Kenan S. Sonographic detection of elastofibroma dorsi J Clin Ultrasound 2003;31:375-378.[Medline]
  16. Solivetti FM, Bacaro D, Di Luca Sidozzi A, Cecconi P. Elastofibroma dorsi: ultrasound pattern in three patients J Exp Clin Cancer Res 2003;22:565-569.[Medline]
  17. Modica WD, Kuntzman T. Elastofibroma dorsi: elaboration of cytologic features and review of its pathogenesis Diagn Cytopathol 2000;23:293-296.
  18. Nagira K, Yamamoto T, Akisue T, et al. Scrape and fine-needle aspiration cytology of elastofibroma Anticancer Res 2002;22:3561-3567.[Medline]
  19. Harigopal M, Seshan SV, DeLellis RA, Yankelevitz D, Vasquez M. Aspiration cytology of elastofibroma dorsi: case report with ultrastructural and immunohistochemical findings Diagn Cytopathol 2002;26:310-313.[Medline]
  20. Domanski HA, Carlen B, Sloth M, Rydholm A. Elastofibroma dorsi has distinct cytomorphologic features, making diagnostic surgical biopsy unnecessary: cytomorphologic study with clinical, radiologic and electron microscopic correlations Diagn Cytopathol 2003;29:327-333.[Medline]
  21. Kransdorf MJ, Meis JM, Montgomery E. Elastofibroma: MR and CT appearance with radiologic-pathologic correlation AJR Am J Roentgenol 1992;159:575-579.[Abstract/Free Full Text]
  22. Hayes AJ, Alexander N, Clark MA, Thomas JM. Elastofibroma: a rare soft tissue tumor with a pathognomonic anatomical location and clinical symptom Eur J Surg Oncol 2004;30:450-453.[Medline]



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