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POPPER, H., 1979. Epidemiology of Thorotrast-induced hepatic angiosarcoma in the United States. Environmental Research, 18, 65-73. GONDOS, B., 1973. Late clinical and roentgen observations following Thorotrast administration. Clinical Radiology, 24, 195-203. ISHIKAWA, Y., KATO, Y. & HATEKEYAMA, S., 1989. Late effects

of a-particles on Thorotrast patients in Japan. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 129-131. ITO,

Y., KOJIRO, M., NAKASHIMA, T. & MORI, T., 1988.

Pathomorphological characteristics of 102 cases of Thorotrast related hepatocellular carcinoma, cholangiocarcinoma and hepatic angiosarcoma. Cancer, 62, 1153-1162. JANOWER, M. L., MIETTINEN, O. S., & FLYNN, M. J., 1972.

Effects of long-term Thorotrast exposure. Radiology, 103, 13-20.

LOONEY, W. B., 1960. Investigation of late clinical findings following Thorotrast (thorium dioxide) administration. American Journal of Roentgenology, 83, 163-185. MACMAHON, H. E., MURPHY, A. S. & BATES, M. I., 1947.

Endothelial cell sarcoma of the liver following Thorotrast injections. American Journal of Pathology, 23, 585-611. MAYS, C. W., 1979. Liver cancer risk. In International Atomic Energy Agency, Comp. Biological Implications of Radionuclides Released from Nuclear Industries. Vol. 2. (International Atomic Energy Agency, Vienna), pp. 146-165. MIYAJIMA, J., OKAJIMA, S., TAKAO, H., NAKASHIMA, A. &

HOMBO, Z., 1985. Estimation of thorium deposited in Thorotrast patients by CT scanner in comparison with whole body counter. Journal of Radiation Research, 26, 196-210. MORI, T., KUMATORI, T., HATAKEYAMA, S., IRIE, H., MORI, W., FUKUTOMI, K., BABA, K., MARUYAMA, T., UEDA, A., IWATA,

S., TAMAI, T. & AKITA, Y., 1989. Current (1986) status of the Japanese follow-up study of the Thorotrast patients, and its relationships to the statistical analysis of the autopsy series. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 119-124.

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IKEZAKI, H., 1982. Thorotrast-induced hepatic angiosarcoma and combined hepatocellular and cholangiocarcinoma in a single patient. Cancer, 49, 2161-2164. KOJIRO, M. & ITO, Y., 1989. Pathomorphologic study of 106 autopsy cases of Thorotrast-related hepatic malignancies with comparison to non-Thorotrast-related cases. In BIR Report 21, Risks from Radium and Thorotrast, Ed. by D. M. Taylor, C. W. Mays, G. B. Gerber and R. G. Thomas (BIR, London), pp. 125-128.

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1989. Tumour induction in rat liver by fractionated irradiation with neutrons and a foreign body burden (Zirconotrast) in comparison to Thorotrast induced tumours. In BIR Report 21, Risks from Radium and Thorotrast, Ed by D. M. Taylor (BIR, London) 149-152. TAYLOR, D. M., MAYS, C. W., GERBER, G. B. & THOMAS,

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Radiological manifestation of elastofibroma: a case report and review of the literature By Takashi Nakano, MD, Zenta Tsutsumi, MD, Toshikazu Hada, MD and Kazuya Higashino, MD The Third Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan {Received December 1990 and in revised form March 1991) Keywords: Elastofibroma, CT, Rib fracture, Subscapular, Chest wall tumour

Elastofibroma is a very rare benign tumour of connective tissue, whose features are distinguished by the presence of abundant elastinophilic polymorphic structures and characteristic location. The lesion usually appears in elderly women (50-80 years) characteristically arising from connective tissue in the subscapular region. There

are few reports of elastofibroma in the radiological literature (Berthoty et al, 1986; Marin et al, 1987). We report a case of elastofibroma dorsi presenting with chest radiographic abnormalities and its radiological manifestation is reviewed. Case report

Correspondence should be addressed to Takashi Nakano, The Third Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663 Japan.

Vol. 64, No. 767

An 84-year-old Japanese female with no significant medical history was referred to hospital for evaluation of an abnormal chest X ray and a feeling of fullness in the upper abdomen. 6 months earlier she began to experience an occasional feeling of fullness in the upper abdomen. Initial evaluation revealed a

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Case reports

Figure 3. Weigert's elastic stain showing numerous globular and a string of bead-like elastic materials mingling with collagen. Note elastinophilic fibres with a central dark core and grey periphery (arrow).

Figure 1. Chest radiograph showing a mass with indistinct upper margins in the right mid zone and fractures of the sixth and seventh ribs. gastric polyp, 1.5 x 1.5 cm in diameter, and a mass on the chest radiograph. The polyp was biopsied and histologic examination showed no malignancy. On physical examination, the right scapula was displaced slightly, both laterally and upwardly, and a non-tender hard mass was apparent beneath it. The mass was obvious when the patient raised her arms and rotated them anteriorly but was not adherent to the overlying skin. She had no perisubscapular pain, discomfort, or restriction of movement. She had noticed a swelling at that site 5 years earlier and although the mass had increased in size, it was otherwise unremarkable. There was no lymph node enlargement and otherwise the physical examination was normal. Laboratory

Figure 2. CT scan of the chest showing an ill-defined inhomogeneous mass located in the subscapular lesion.

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studies were also normal. The chest radiograph revealed an opacity with indistinct upper margin in the right upper zone and fractures of the sixth and seventh ribs (Fig. 1). A computed tomographic (CT) scan of the chest was performed, and this confirmed an inhomogeneous ill-defined mass located in the subscapular region (Fig. 2) corresponding in position to the mass seen on the chest radiograph. It clearly displaced the right scapula. Tru-cut biopsy of the mass demonstrated elastofibroma (Fig. 3). Discussion

Elastofibroma was first described by Jarvi and Saxen (1961). Subsequently, there have been no more than 300 cases in the literature, consisting mainly of sporadic individual case reports. A review of the literature up to 1986 comprising 79 patients was reported by Wening et al (1986). Elastofibroma is a rare fibroproliferative benign lesion of uncertain cause, which usually arises in the subscapular region. Bilateral lesions are common and they may occur asynchronously (Jarvi et al, 1969; Nagamine et al, 1982). Other sites of occurrence are uncommon, including the infraolecranon area (Nagamine et al, 1982), the deltoid muscle (Mirra et al, 1974), the ischial tuberosity (Waisman & Smith, 1968), the greater trochanter (Barr, 1966), the intraspinal space (Prete et al, 1983), the axilla (Deutsh, 1974), the greater omentum (Tsutsumi et al, 1985), the orbital area (Austin et al, 1983) and the foot (Cross et al, 1984). In addition, a gastric elastofibroma in association with a subscapular lesion has been reported (Enjoji et al, 1985). A gastric polyp was found in our case, but histological examination of the biopsied specimen did not identify elastinophilic material. The typical microscopic appearance of the lesion has been well described and is composed of bundles of collagen and elastic fibres. Elastin stains, such as with VerhoefFs and Weigert's, demonstrate the elastic substances in the lesion to be a string of bead-like or necklace-like arrangement, with a central darker stained core and grey periphery. With hematoxylin and eosin The British Journal of Radiology, November 1991

Case reports Table I. Summary of the reports of radiographic findings in elastofibroma dorsi (1961 ~ ) References

Number of cases

Site

Findings of chest X-ray film and/or CT scans Chest X-ray film: peripheral shadow involving the pleura Chest X-ray film: badly-defined shadow, only slightly more opaque than the surrounding soft tissue Chest X-ray film: a soft tumour seen over the ribs in the subscapular region

Deutsch, 1974

1

right

Scapinelli et al, 1982

1

right

Nagamine et al, 1982

170

Berthoty et al, 1986

1

Marin et al, 1987

2

bilateral (112/170, 65.9%) right (35/170, 20.6%), left (22/170, 12.9%) bilateral

right right

staining, these structures stain eosinophilically. Figure 3 is a photomicrograph of the present subscapular lesion using Weigert's stain. The findings were typical of elastofibroma. Chronic microtrauma and occupational stress have been implicated in the pathogenesis of elastofibroma, but the relationship has not been definitely established (Jarvi et al, 1969). Our patient had no occupational history of heavy manual labour. For this report, we also carried out a search of the radiological literature since the first description by Jarvi and Saxen (1961). We were able to review the radiological appearances of individual cases of elastofibroma in five different publications (Table I). Marin et al (1987) reported a case of elastofibroma whose plain chest radiograph showed an ill-defined opacity projected over the lower zone. In our case, the chest radiograph showed a mass overlying the ribs with an indistinct upper margin in the right mid-zone and with associated fractures of the sixth and seventh ribs. Elastofibroma is usually found in the lower subscapular space deep to the rhomboids and latissimus dorsi muscles, and is frequently firmly fixed to the posterior thoracic cage in the region of the sixth to eighth rib (Stemmermann & Stout, 1962; Jarvi et al, 1969). Elastofibroma has no capsule and usually infiltrates surrounding tissues, thus mimicking a malignant neoplasm. On plain chest radiography, elastofibroma may be associated with rib fracture. Thoracic CT is useful in the diagnosis of elastofibroma and our search reveals only two reports of Vol. 64, No. 767

Chest X-ray film: normal CT scan: subscapular poorly defined nonhomogeneous mass with irregular linear low-density streaking Chest X-ray film: (patient 1) normal (patient 2) a vague opacity extending 8 cm inferior to the inferior angle of the scapula CT scan: (patient 1) a subscapular softtissue-density mass without clear marginal definition (Patient 2) an inhomogeneous soft-tissuedensity mass elevating the scapula from the posterior chest wall Margins of the mass varied between poorly demarcated and partially well demarcated

three patients describing the CT features of elastofibroma (Table I). CT can readily show extension of the mass into the chest wall and ribs, when the mass may be shown pushing the ribs inwards and, presumably, resulting in rib fracture due to the resultant deformity rather than direct invasion. Differential diagnosis in patients with subscapular tumour includes liposarcoma (Wilinsky et al, 1984), fibromatosis (Campbell et al, 1983) and malignant fibrous histiocytoma (Westreich & Yeshua, 1989). The radiological discrimination between elastofibroma and other mesenchymal malignancy is difficult and needle biopsy is recommended even if the radiological findings are typical of elastofibroma. Deutsh (1974) reported a case showing a good response of elastofibroma to radiotherapy treatment. However, no treatment is necessary in the asymptomatic patient because the lesion is benign. The treatment of choice for symptomatic elastofibroma is local excision and recurrence has not been reported after surgical excision. As our patient was virtually asymptomatic excision was not performed. Elastofibroma dorsi describes the findings in this patient because of its typical site, but it should be noted that this condition is not exclusively confined to the subscapular region. References AUSTIN, P., JAKOBIEC, F. A., IWAMOTO, T. & HORNBLASS, A.,

1983. Elastofibroma oculi. Archives of Ophthalmology, 101, 1575-1579.

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Elastofibroma: chest wall pseudotumor. Radiology, 160, 341-342. CAMPBELL, A. N., CHAN, H. S. L., DANEMAN, A. & MARTIN,

NAGAMINE, N., NOHARA, Y. & ITO, E., 1982. Elastofibroma in

Okinawa: a clinicopathologic study of 170 cases. Cancer, 50, 1794-1805. PRETE, P. E., HENBEST, M., MICHALSKI, J. P. & PORTER, R. W.,

1983. Intraspinal elastofibroma. A case report. Spine, 8, 800-802.

D. J., 1983. Aggressive fibromatosis in childhood: computed tomographic findings in three patients. Journal of Computed Tomography, 7, 109-113.

SCAPINELLI, R. & VALBONESI, L., 1982. Juxtascapularly-situated

CROSS, D. L., MILLS, S. E. & KULUND, D. N., 1984. Elasto-

STEMMERMANN, G. N. & STOUT, A. P., 1962. Elastofibroma

fibroma arising in the foot. Southern Medical Journal, 77, 1194-1196. DEUTSH, G. P., 1974. Elastofibroma dorsalis treated by radiotherapy. British Journal of Radiology, 47, 621-623. ENJOJI, M., SUMIYOSHI, K. & KUEYOSHI, K., 1985. Elasto-

fibromatous lesion of the stomach in a patient with elastofibroma dorsi. American Journal of Surgical Pathology, 9, 233-237. JARVI, O. H. & SAXEN, E., 1961. Elastofibroma dorsi. Acta Pathologica et Microbiologica Scandinavica, 51, 83-84. JARVI, O. H., SAXEN, A. E., HOPSU-HAVU, V. K., WARTIOVAARA, J. J. & VAISSALO, V. T., 1969. Elastofibroma:

a degenerative pseudotumor. Cancer, 23, 42-63. MARIN, M. L., AUSTIN, J. H. M. & MARKOWITZ, A. M., 1987.

Elastofibroma dorsi: CT demonstration. Computer Assisted Tomography, 11, 675-677.

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"elastofibrama dorsi". Italian Journal of Orthopaedics and Traumatology, 8, 351-356. dorsi. American Journal of Clinical Pathology, 37, 499-506. TSUTSUMI, A., KAWABATA, K., TAGUCHI, K. & Doi, K., 1985.

Elastofibroma of the greater omentum. Acta Pathologica Japonica, 35, 233-241. WAISMAN, J. & SMITH, D. W., 1968. Fine structure of an

elastofibroma. Cancer, 22, 611-611. WESTREICH, M. & YESHUA, R., 1989. Scapulectomy with

latissimus dorsi musculocutaneous flap in the treatment of sarcoma of the upper back. Annals of Plastic Surgery, 23, 337-340. WENING, J. V., THOMA, G. & MORL, F. K., 1986. Familial

fibroelastoma dorsi: first case report and review of literature. British Journal of Clinical Practice, 40, 390-394. WlLINSKY,

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Liposarcoma involving the scapula. Journal of Computed Tomography, 8, 341-343.

MIRRA, J. M., STRAUB, L. R. & JARVI, O. H., 1974. Elasto-

fibroma of the deltoid: a case report. Cancer, 33, 234-238.

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The British Journal of Radiology, November 1991

Radiological manifestation of elastofibroma: a case report and review of the literature.

1991, The British Journal of Radiology, 64, 1069-1072 Case reports epidemiological, clinical and pathological studies (experience in Portugal). Enviro...
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