Elastofibroma and pre-elastofibroma—a biopsy and autopsy study

European Journal of Surgical Oncology 1996; 22:93-96

Elastofibroma and pre-elastofibroma

a biopsy and autopsy study

G. D. Giebel, E. Bierhoff* and J. Vogel* Second Department of Surgery, University of Cologne, Ostmerheimer Street 200, 51109 Cologne, Germany and *Department of Patholog); University of Bonn, Sigmund-Freud-Street25, 53127 Bonn, Germany

Two biopsy cases of elastofibroma--one unilateral and one bilateral--are described. A study of 100 autopsies revealed 13 elderly patients with elastofihroma. Males (n=10; 16.9%) were more affected than females (n=3; 7.3%). Preelastofihroma-like morphological changes (e.g. few or many degenerated elastic fibres) were observed in 81% of the autopsies. Minor pre-elastofibroma-like changes were seen in males and major changes predominantly in females. In addition to physiological ageing as yet unknown factors, rather than abnormal elastogenesis or degeneration, seem to be involved in this pseudotumour.

Key words: elastofibroma; pre-elastofibroma; biopsy; autopsy.

Introduction J/irvi and Saxen (1961) first reported four cases of a peculiar tumour-like process arising from the connective tissue between the lower portion of the scapula and the chest wall. Because of its typical location it was named 'Elastofibroma dorsi', t Rare examples have also been found in other locations2-7 and therefore the term 'Elastofibroma' is preferred now. The pathogenesis is still unclear. The need for causal explanation by patients and doctors lead to microtrauma as an attempt to an explanation. That, however, would prefer a right-sided location, which cannot be proved.

Material and methods

Biopsies Case 1. A 50-year-old office-worker, who felt a pain in the right lower scapular angle, in combination with a grating noise during movement of the arm 6 weeks prior to admission to the hospital. Clinical examination revealed an immotile tumour mass close to the right lower scapular angle, precisely localized--intraoperatively--between the chest wall and the inner lower scapular angle, below the trapezoid muscle and at the base of the major rhomboid muscle.

Case2. A 47-year-old hard-working waiter, who suffered from backache around both scapules, with no movement restriction, 4 weeks before admission to the hospital. Solid Correspondence to: G. D. Giebel MD, MA, Second Department of Surgery, University of Cologne, Ostmerheimer Street 200, 51109 Cologne, Germany. 0748-7983/96/010093 +04 $12.00/0

tumours the size of a child's fist were detectable at the inner surface of the right and left scapula close to the lower angle with distension of the medial parts of both scapules away from the chest wall.

Autopsies One hundred autopsies (59 males and 41 females, age range: 14-92 years, average age 62.5 years, median age: 66 years) were studied. Cause of death was cardio-pulmonary insufficiency (n = 53), final stage of malignant tumour (n = 35), preceding accident (n=10) and inadequate liver function due to cirrhotic liver disease (n=2). Autopsy specimens were taken from the lower inner portion of both scapular angles and the corresponding area of the chest wall. A cut was made along the margo medialis and along the inferior angulus of the scapula. The trapezoid and latissimus dorsi muscles were cut. A piece of soft tissue of approximately 1 cm in diameter was excised from the intermuscular thoracic space, including fatty tissue and the starting point of the rhomboid major, infraspinalic, latissimus dorsi (scapular part) and serratus anterior muscles. The specimens of the biopsy and autopsy cases were fixed in 4% formaldehyde and embedded in paraffin wax. Sections of 5 pm in thickness were taken from each paraffin block. Routine staining included Haematoxylin and Eosin, Verhoeff-van-Gieson, Masson-Golder trichrome and Gomori's silver stain. The frequency of elastic fibres was graded semi-quantitatively into four categories: (1) no degenerated elastic fibres detectable; (2) few degenerated elastic fibres; (3) many degenerated elastic fibres; and (4) very many degenerated elastic fibres according to elastofibroma (Fig. l(a--c)). Statistical analysis was performed by Student's t-test. © 1996W.B. SaundersCompanyLimited

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Fig. 2. Elastofibroma with snake-like bands and a dense core (Verhoeff-van-Gieson stain; x 400). (Reproduced here at 60%.)

30 20 10 none

few many Elastic fibres

very many

Fig. 3. Pre-elastofibroma and elastofibroma in autopsy findings (n = 100}. Distribution and frequency (%) of elastic fibres.

Fig. 1. Pre-elastofibroma-like and elastofibroma-like changes with: (a) few elastic fibres, fatty tissue and collagen fibres (Verhoeff-vanGieson stain: × 400): (b) increase of elastic fibres in fibrous tissue (Verhoeff-van-Gieson stain; × 400); (c) very many elastic fibres with an irregular pattern similar to the morphological feature of elastofibroma (Verhoeff-van-Gieson stain; x 400). (All reproduced here at 60%.)

Results

swollen, eosinophilic collagen and elastic fibres in about equal proportions. Occasional fibroblasts, small amounts of interstitial mucoid material and aggregates of mature fat cells were observed. Elastic fibres had a degenerated appearance or were fragmented into small flower-like serrated discs or globules with a distinct linear arrangement. Elastin stains revealed deeply staining, branched and unbranched fibres with a central dense core and an irregular moth-eaten or serrated margin (Fig. 2). There was no recurrence during 5 years of observation.

Case 2. The size of the tumour mass was 7 ×5 × 4 c m on the right and 1 0 × 6 × 4 c m on the left with similar macroscopic and microscopic findings on both sides as described above. After removing the drainage an extensive haematoma in the right wound made two punctures necessary. There was no recurrence within 2 years of observation.

Biopsies

Autopsies

Case 1. The size of the firm and spherical elastic tumour mass was 7 × 7 × 4 cm with ill-defined margins. On section it presented a gray-white, glistening surface with interspersed fatty islands and streaks. On microscopic examination the tumour-like mass consisted of a mixture of intertwining

In the 100 autopsy specimens 13 cases with typical histological features of elastofibroma, including very many degenerated elastic fibres, were detected. Ten males (16.9%) and three females (7.3%) presented with elastofibroma (Figs 3 and 4).

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Elastofibroma and pre-elastofibroma

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Discussion

40 30 20 10 none

few many Elastic fibres

very many

Fig. 4. Pre-elastofibroma and elastofibroma in autopsy findings (n = 100). Distribution and frequency C/,,) of elastic fibres in males ( i ) and females (m). 50 40 30 20 10 none

few many Elastic fibres

very many

Fig. 5. Pre-elastofibroma and elastofibroma in autopsy findings (n = 100). Distribution and frequency (%) of elastic fibres <67 (Ill and >66 ([]) years of age. In six cases no degenerated elastic fibres and no preelastofibroma-like morphological changes were demonstrable. Pre-elastofibroma-like changes were observed in 81 cases, including a different degree of degenerated elastic fibres together with only weakly elastinophilic fibrillary material, with no progression to elastic tissue. Few degenerated elastic fibres (n=41) and many degenerated elastic fibres (n ---40) could be observed at similar frequencies (Fig. 3). With reference to sex distribution, no degenerated elastic fbres were seen in males (n=3; 5.1%) and females (n = 3; 7.3%) at an almost equal frequency. Few degenerated elastic fibres were observed predominantly in males (n= 25: 42.4%) compared with in females (n= 16; 39%). Many degenerated elastic fibres, however, were seen in a smaller percentage (not significant) in males (n = 21; 35.6%) than in females (n= 19; 46.3%) (Fig. 4). According to the median age of 66 years of the autopsy cases no degenerated elastic fibres were observed predominantly at ages under 67 years (7.5%), and less frequently over the age of 66 years (4.3%). Few degenerated elastic fibres occurred predominantly in the age group below 67 years (47.2%) compared with the group older than 66 years (34%). The frequency of many degenerated elastic fibres was similar in both groups (<67 years, 40% and >66 years, 40.4%). A correlation between increasing degenerated elastic fibres and age was obvious. Elastofibroma-like morphological changes could be observed predominantly in the group of patients aged more than 66 years old (21.3%) and to a lesser extent in the younger (<67 years) patients (5.7%) (Fig. 5).

Elastofibroma can be easily overlooked because the symptoms may be very subtle) -~4 It usually manifests merely as a deep-seated mass that occasionally causes tenderness, pain, or restriction of movement. 4'j2 Unilateral involvement can be observed in the majority of cases, 4 bilateral involvement is less commont'4'~---as seen in one biopsy case. Patients with elastofibroma are generally past the age of 55 years4 and it is regarded as a disease of the elderly. ~6This is supported by the data of the study; the majority of elastofibroma patients even being past the age of 66 years (Fig. 5). According to the literature patients with elastofibroma are more often female than male. 4'~-' This differs from data of the study, which revealed a majority of males presenting with elastofibroma. The origin and pathogenesis of elastofibroma is not completely uncovered. The major opinion nowadays is that elastofibroma has to be regarded as a degenerative pseudotumour and is the result of excessive formation of collagen--especially abnormal elastic fibres--secondary to repeated injury, more specifically injury caused by friction between the inferior edge of the scapula and the underlying chest wall. 4"jT'ts Hard and repetitive manual work with microtrauma of the subscapular soft tissue is regarded as a predisposing factor. 1'5'6'~°'tLt~'j9However, elastofibroma only occurs in a small number of people. An increased familial incidence, an underlying genetic disposition or inherent enzymatic defect are therefore discussed as additional causative factors, j-"Opinions differ as to the exact nature of the abnormal elastic fibres. As first suggested by J~rvi et a/. LIT they seem to be formed by abnormal elastogenesis rather than by degeneration, preformed elastic fibres or collagenf1-27 The basic cells producing abnormal elastic fibres seem to be myofibroblasts, whereas the disturbance of elastic fibrillogenesis by periostal derived cells is also debated. 4.is Elastofibroma-like changes have also been demonstrated by J~i.rvi et al. 2s in the fascia beneath the lower portion of the scapule in 39 (16.6%) patients in a series of 235 autopsies. Benisch el al. -~ introduced the term pre-elastofibroma describing a fibrous tumour that produced only weakly elastinophilic fibrillary material with no progression to elastic tissue. According to the data of the present study minor (41%) and major (40%) pre-elastofibroma-like changes have to be taken into account in a much higher frequency than reported so far in the literature (Fig. 4). For the full picture of elastofibroma, however, other as yet unknown factors--e.g, sex, familial incidence, underlying genetic disposition or inherent enzymatic defect--rather than abnormal elastogenesis or degeneration seem to be involved.

Conclusion

Elastofibroma has to be regarded as a degenerative pseudotumour and seems to be the result of excessive formation of collagen and abnormal elastic fbres. Repeated injury is thought to be an important causative factor. Elastofibroma is a tumour-like process of old age, as

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supported by the data of the study. Clinical symptoms are generally subtle. According to the data, but in contrast to the literature, males show a higher incidence of elastofibroma than females. Pre-elastofibroma-like degenerative changes can be observed in a much higher frequency than reported in the literature so far. F o r the full picture of elastofibroma, however, other as yet unknown factors rather than abnormal elastogenesis or degeneration seem to be necessary. Elastofibroma is a benign condition with no tendency to recur after excision, which is the therapy of choice; radiotherapy with its known side-effects, however, is thought to give equally good results. 3°

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