Elastofibroma dorsi is a rare, slow growing, soft tissue lesion, which occurs mainly in elderly women, tipically located in the infrascapular region. This lesion is characterized by a poorly defined solid mass of

fibroelastic and fatty tissue, enlarging into intermuscular spaces. Elastofibroma may simulate an aggressive behaviour, and differential diagnosis with malignant tumors is sometimes difficult. Surgical resection is generally recommended in symptomatic lesions or in the suspicion of

malignancy. We report our experience of three cases of elastofibroma, two of which typically located in the infrascapular region and one located in the supraclavear fossa, atypical site never reported so far

Bettarini, Francesca

Caruso, Stefano

Di Martino, Marianna

Falzarano, Sara Moscovita

Marrelli, F.

Neri, Annasilvia

Pedrazzani, Corrado

Roviello, Franco

Vuolo, Giuseppe

Elastofibroma dorsi is a rare, slow growing, soft tissue lesion, which occurs mainly in elderly women, tipically located in the infrascapular region. This lesion is characterized by a poorly defined solid mass of fibroelastic and fatty tissue, enlarging into intermuscular spaces. Elastofibroma may simulate an aggressive behaviour, and differential diagnosis with malignant tumors is sometimes difficult. Surgical resection is generally recommended in symptomatic lesions or in the suspicion of malignancy.
We report our experience of three cases of elastofibroma, two of which typically located in the infrascapular region and one located in the supraclavear fossa, atypical site never reported so far

NERI A.

CARUSO S.

BETTARINI F.

FALZARANO S. M.

DI MARTINO M.

VUOLO G.

PEDRAZZANI C.

MARRELLI D.

ROVIELLO F.

Archivio della Ricerca - Università degli Studi di Siena

Elastofibroma dorsi: three cases of personal experience

Elastofibroma dorsi is a rare, slow growing, soft tissue lesion, which occurs mainly in elderly women, typical located in the infrascapular region. This lesion is characterized by a poorly defined solid mass of fibro-elastic and fatty tissue, enlarging into intermuscular spaces. Elastofibroma may simulate an aggressive behaviour, and differential diagnosis with malignant tumors is sometimes difficult. Surgical resection is generally recommended in symptomatic lesions or in the suspicion of malignancy. We report our experience of three cases of elastofibroma, two of which typically located in the infrascapular region and one located in the supra-clavicle fossa, atypical site never reported so far

Caruso, S

Bettarini, F

Falzarano, S M

Di Martino, M

Vuolo, G

Pedrazzani, C

Marrelli, D

Roviello, F

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G Chir Vol. 30 - n. 3 - pp. 96-99Marzo 200996IntroductionElastofibroma dorsi is a rare non-encapsulated beni-gn soft tissue lesion (1). It is characteristically locatedin the distal infrascapular region, between the scapulaand the thoracic wall. Even if rarely observed, the cor-rect identification of this lesion is important since it maymimic other tumors of the thoracic wall that could re-quire more aggressive treatment (2). Although some authors report that ultrasound scan(US) and magnetic resonance imaging (MRI) can dif-ferentiate elastofibroma from the other soft tissue lesions(3, 4), others emphasize the importance of surgical re-section and histological examination for a definite dia-gnosis (3, 5-8).We report our experience on three cases of elastofi-broma, two of which located in the typical infrascapu-lar region and one located in the supraclavear fossa, aty-pical site never reported so far.Clinical casesCase 1A 59 years-old woman was referred to our Division for a left-painful subscapular palpable mass dating several months. On phy-sical examination, the lesion had a hard-elastic consistence, an irre-SUMMARY: Elastofibroma dorsi: three cases of personal experien-ce.A. NERI, S. CARUSO, F. BETTARINI, S.M. FALZARANO, M. DI MARTINO, G. VUOLO, C. PEDRAZZANI, D. MARRELLI, F. ROVIELLOElastofibroma dorsi is a rare, slow growing, soft tissue lesion, whi-ch occurs mainly in elderly women, tipically located in the infrascapu-lar region. This lesion is characterized by a poorly defined solid mass offibroelastic and fatty tissue, enlarging into intermuscular spaces. Ela-stofibroma may simulate an aggressive behaviour, and differential dia-gnosis with malignant tumors is sometimes difficult. Surgical resectionis generally recommended in symptomatic lesions or in the suspicion ofmalignancy.We report our experience of three cases of elastofibroma, two of whi-ch typically located in the infrascapular region and one located in thesupraclavear fossa, atypical site never reported so far.RIASSUNTO: Elastofibroma dorsi: tre casi dall’esperienza persona-le.A. NERI, S. CARUSO, F. BETTARINI, S.M. FALZARANO, M. DI MARTINO, G. VUOLO, C. PEDRAZZANI, D. MARRELLI, F. ROVIELLOL’elastofibroma dorsi è un raro tumore dei tessuti molli, a lenta cre-scita, che si manifesta prevalentemente nel sesso femminile in età avan-zata e tipicamente nella regione sottoscapolare. Tale lesione si presentacome una massa a margini non ben definiti, costituita da tessuto fibroe-lastico e adiposo, che si accresce negli spazi intermuscolari. L’elastofibromapuò simulare una patologia tumorale maligna e spesso la diagnosi dif-ferenziale risulta difficoltosa. L’asportazione chirurgica è raccomanda-ta nelle forme sintomatiche o nei casi in cui permanga il sospetto di  neo-plasia maligna. Riportiamo la nostra esperienza di tre casi di elastofibroma dorsi,due dei quali tipicamente localizzati nella regione sottoscapolare ed unonella fossa sovraclaveare, sete atipica mai riportata prima in letteratura.Elastofibroma dorsi: three cases of personal experienceA. NERI, S. CARUSO, F. BETTARINI, S.M. FALZARANO1, M. DI MARTINO, G. VUOLO, C. PEDRAZZANI, D. MARRELLI, F. ROVIELLOUniversity of Siena, Policlinico “Le Scotte”, Siena, ItalyDepartment of Human Pathology and OncologySection of General Surgery and Surgical Oncology1 Section of Human Pathology© Copyright 2009, CIC  Edizioni Internazionali, RomaKEY WORDS: Elastofibroma dorsi - Soft tissue tumor - Surgery.Elastofibroma dorsi - Tumore dei tessuti molli - Chirurgia.97Elastofibroma dorsi: three cases of personal experience97gular shape and it was not adherent to the overlying skin. The massappeared more clearly when the shoulder was abducted forward. Theclinical appearance resembled a lipoma. The US was in agreement with the clinical evaluation descri-bing an oval mass, partially covered by the scapula, of 2.5 cm in mi-nor diameter compatible with a lipoma (Fig. 1). A MRI identifieda non-capsular, circumscribed, heterogeneous soft tissue mass of 3cm  in diameter in the subscapular region, resembling the featuresof a fibrolipoma. Surgical resection was hence recommended; thelesion resulted as a fibroelastic mass, located beneath the latissimusdorsi muscle and firmly adherent to the chest wall. Macroscopic exa-mination of the resected specimen showed a nodule of fibroadipo-se tissue of 8x7x2 cm (Fig. 2a). The final histopathological reportdiagnosed an elastofibroma dorsi (Fig. 2b). The post-operative course was uneventful and no recurrence ofdisease has occurred at 12 months of follow-up.Case 2A 56 years-old man was referred to our Division for a painful,rapidly growing mass located in the right subscapular region. Cli-nical examination was indicative of a large lipoma. An US demonstrated a well-defined, hyperechoic, oval-shapedmass of 5.5x4x2.5 cm, partially lying under the scapula. The patient underwent surgery in view of a symptomatic lipo-ma. The lesion appeared as a bulky mass, extending under latissi-mus dorsi and tenaciously adherent to the surrounding tissue, mi-micking a malignant soft tissue tumor. Macroscopic examinationshowed an egg-shaped lesion of 9x3x5 cm with fibrotic connectivetissue and fatty cells at histopathology (Fig. 2c). Diagnosis of elasto-fibroma dorsi was made. The post-operative course was uneventful and no recurrence hasoccurred at 18 months of follow-up.Case 3A 67 years-old woman was admitted at our Division presentinga palpable mass located in the right supraclavear region. She com-plained a 6-months history of a slowly growing mass associated torising pain. Patient’s clinical history included a lipomatosis of thedorsal region. On physical examination, a solid-elastic mass not adherent tothe overlying skin, clinically consistent with a lipoma, was appre-ciable. Surgical excision was hence performed and the definitive histo-logy described a 3x3x1 cm mass compatible with an elastofibroma(Fig. 2d). The post-operative course was uneventful and no relapse has oc-curred after 28 months.DiscussionElastofibroma dorsi, firstly described by Jarvi andSaxen in 1961 (1), is a benign, slow growing soft tissuelesion occurring most often in the infrascapular region(9). Although 99% of the cases are located between thedistal part of the scapula and the thoracic wall (3), thelesion has been occasionally reported in other sites su-ch as hand, foot, neck, mediastinum, stomach, greateromentum, cornea, orbit, ischial tuberosity, inguinal re-gion, intraspinal and intra-articular spaces (5). Charac-teristically, it affects elderly women, usually aged morethan 60 years, with a female to male ratio of 5-13 to 1(3, 9-11). Observation of elastofibroma in young peo-ple is rare, although its occurrence has been sporadical-ly described in children (4,11). Albeit not observed inour cases, bilateral location is a frequent event (10-66%)(3, 4, 12, 13).The pathogenesis of this rare lesion remains unde-monstrated, nevertheless it has been postulated the de-generation of connective tissue secondary to frictionof the scapula against the rib cage caused by repetiti-ve use of the shoulder girdle (3-5, 10, 11, 14). Howe-ver, cases reported in literature frequently deny a hi-story of intense physical activity or thoracic wallmalformation. Moreover, the predominance of fema-le gender and the report of uncommon localizationsin a regions not submitted to mechanical stress con-trast with this theory (14). Consistently, our three pa-tients did not present a history of sport activity or in-tense physical work.On gross appearance, elastofibroma dorsi is a non ca-psulated, solid mass, varying in diameter (1-13 cm). Itis characteristically located at the angle of the scapula,deeply  the rhomboid and latissimus dorsi muscles, adhe-ring tenaciously to deeper planes but cleavable from theoverlying muscle. In our third case the lesion was loca-ted in the right supraclavear fossa. It was not adherentto overlying skin or to other surrounding structures andit was easily enucleated under local anaesthesia. To ourknowledge, this represents the first case located in thissite and reported in literature.Histological diagnosis is based on the finding of aconnective stroma, constituted by poorly defined fibro-lipomatous tissue, enclosing an abnormal elastic com-ponent represented by altered elastic fibers embeddedin a collagenous matrix, riddled with various amountsof fat cells (2). Himmunohistochemically, it stains forvimentin but not for α-smooth muscle actin (SMA), S-100, desmin, or p53 (1).Fig. 1 - US: ovalar mass partially covered by the scapula.98A. Neri et al.Clinical examination usually reveals a slow growing,well circumscribed mass, non adherent to overlying skin(2, 6, 7, 10, 14, 15). The scapula may completely over-lie the lesion thereby masking it until the patient abductforward his arm. This allows the scapula to move up andlet the mass to emerge. The differential diagnosis of su-ch a soft tissue lesion includes lipoma, liposarcoma, sar-coma, fibromatosis, desmoid, neurofibroma, histiocy-toma, hemangioma and metastasis. In elastofibroma dorsi, imaging may give useful indi-cations but it is often not conclusive. US shows charac-teristic findings evidencing a solid mass with regularlyalternating fasciculated structures appearing as hyper-and hypoechoic striae (elastic and fat tissue, respectively).Overall, due to its muscle-like appearance, the ultra-sound findings are generally insufficient to make dia-gnosis (2, 5, 14). CT confirms the similarity of the massto the morphology of the muscular tissue and usuallyshows a heterogeneous soft tissue mass with poorly de-fined margins (7). MRI is the best non-invasive techni-que for diagnosis (3, 7, 14). At MRI, elastofibroma ap-pears as a poorly circumscribed soft tissue mass with si-gnal intensity similar to skeletal muscle, but with lowsignal intensity on T1-and T2-weighted images corre-sponding to the predominantly fibrous tissue of the le-sion. Areas of increased signal intensity, when present,can correspond to adipose tissue (3, 4, 14). In our ex-perience, US (cases 1 and 2) as well as MRI (case 1) mis-sed both the diagnosis and the extension of the lesions.The error in defining the nature of the lesions is proba-bly due to the rarity of elastofibroma, while the inaccu-racy in defining their dimension is certainly related tothe difficulty in delineating the margins from the sur-rounding muscle planes.Fig. 2 - a) Specimen 8x7x2 cm. b), c), d) At low magnification, elastofibroma appears as an irregularly shaped lesion made of a paucicellular collagenoustissue containing numerous eosinophilic elastin bands admixed with mature fat. Elastic stains reveal the large fibers with a dense core and serrated or bea-ded margins [b: Hematoxylin-Eosin, original magnification (OM) 50x – Inset: Pinkus' Acid Orcein-Giemsa, OM 400x; c: Hematoxylin-Eosin, OM 50x – Inset:Weigert OM 200x; d: Hematoxylin-Eosin, OM 100x – Inset 400x].99Elastofibroma dorsi: three cases of personal experienceRegarding surgical resection, it is not mandatory in ca-se of incidental diagnosis of asymptomatic lesions as ma-lignant transformation has never been reported so far (3,7, 8, 14, 16). Conversely, resection is recommended insymptomatic lesion and in the suspicion of malignant tu-mor. Practically, a definite diagnosis is often difficult priorto surgical excision both with imaging and fine needle aspi-ration cytology due to the scarceness of cellular elements(8, 11, 14). Regarding fine needle aspiration cytology, itmust be emphasized that its use is no more recommen-ded in the suspicion of soft tissue tumors (11, 14).In conclusion, elastofibroma dorsi is a rare lesion ofthe chest which should be kept in mind in the differen-tial diagnosis with benign or malignant lesions of thethoracic wall. The typical localization may suggest itsdiagnosis, but clinical and instrumental findings shouldbe confirmed by histological examination to exclude amalignancy. Our cases confirm the difficulty in the dia-gnosis when a misleading instrumental diagnosis con-trasted with an intraoperative aspect suspect for mali-gnancy, which may result in an inappropriate aggressi-ve surgical approach.1. Jarvi OH, Saxen E. Elastofibroma dorsi. Acta Pathol MicrobiolScand 1961;51: 83-84.2. Cota C, Solvetti F, Kovacs D, Cristiani R, Amantea A. Elasto-fibroma dorsi: histologic and echographic considerations. Int JlDermatol 2006;45:1100-03.3. Oueslati S, Douria-Khomsi W, Bouaziz MC, Zaouia K. Elastofi-broma dorsi: a report on 6 cases. Acta Orthop Belg 2006;72:237-42.4. Devaney D, Livesley P, Shaw D. Elastofibroma dorsi: MRI dia-gnosis in a young girl. Pediatr Radiol 1995;25:282-83.5. Marcos J, Russo PR, Lima S, Rodriguez EL. Elastofibroma dor-si. An unusual  case of a subscapular nodular mass. J Clin Rheu-matol 2004;10:21-24.6. Parodi PC, Nadalig B, Rampino Cordaro E, Mariuzzi L, RossiD. Non-traumatic elastofibroma dorsi. EJD 2007;17:169-70.7. Pyne D, Mootoo R, Bhanji A, Amin S: Elastofibroma dorsi [Let-ters]. Ann Rheum Dis 2002;61:278-79.8. Schafmayer C, Kahlke V, Leuschner I, Pai M, Tepel J. Elastofi-broma dorsi as differential diagnosis in tumor of the thoracicwall. Ann Thorac Surg 2006; 82:1501-04.9. Guha AR, Raja RCS, Devadoss VG. Elastofibroma dorsi. A casereport and review of literature. Int J Clin Pract 2004;58:218-20.10. Erdas E, Licheri S, Pinna G, Gerosa C, Ragazzo G. Elastofibro-ma dorsi: case report and review of the literature. G Chir2006;27:372-76.11. Marin ML, Perzin KH, Markowitz AM. Elastofibroma dorsi: be-nign chest wall tumor. J Thorac Cardiovasc Surg 1989;98:234-38.12. Jarvi OH, Lansimies PH. Subclinical elastofibromas in the scapu-lar region in an autopsy series. Acta Pathol Microbiol Scand1975;83:87-108.13. Naylor MF, Nascimento AG, Sherrick AD, McLeod RA.Elastofibroma dorsi: radiologic findings in 12 patients. Am JRoentgenol 1996;167:683-87.14. Daigeler A, Vogt PM, Busch K, Pennekamp W, Weyhe D, Lehn-hardt M, Steinstraesser L, Steinau HU, Kuhnen C. Elastofibro-ma dorsi - differential diagnosis in chest wall tumours. World JSurg Oncol 2007;5:15.15. Erkiliç S, Koçer E, Sivrikoz C. Subscapular elastofibroma inter-mingled with adipose tissue variant type of elastofibroma or lipo-ma? Ann Diagn Pathol 2005;9: 327-29.16. Fibla J, Molins L, Marco V, Perez J, Vidal G. Bilateral elastofi-broma dorsi. Joint Bone Spine 2007;20:1-3.References

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Elastofibroma dorsi: three cases of personal experience

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