Eur J Plast Surg (1997) 20:98-100
© Springer-Verlag 1997
Leiomyosarcoma in a smallpox vaccination scar:
case report and review of literature
P. Sendi, S. Krupp
Service de Chirurgie Plastique et Reconstructive CHUV Lausanne, Switzerland
Abstract.
T h e d e v e l o p m e n t o f s a r c o m a s in s m a l l p o x v a c c i n a t i o n scars is v e r y rare. I n c l u d i n g this case, o n l y five h a v e b e e n r e p o r t e d . T h i s is the first r e p o r t o f a l e i o m y o s a r c o m a a r i s i n g in a s m a l l p o x v a c c i n a t i o n scar. K e y w o r d s : S m a l l p o x v a c c i n a t i o n s c a r - L e i o m y o s a r c o - m a I n 1882, M a r j o l i n d e s c r i b e d a s q u a m o u s c e l l c a r c i n o m a d e v e l o p i n g in a c h r o n i c l e g u l c e r [1]. " M a r j o l i n ' s u l c e r " d e s c r i b e s m a l i g n a n c y in scars [2], v e n o u s stasis u l c e r s , c h r o n i c o s t e o m y e l i t i s a n d c h r o n i c f i s t u l a a n d s i n u s e s [3], i r r a d i a t i o n d e r m a t i t i s [4], c h r o n i c t r a u m a t i c w o u n d s [5], a n d p r e s s u r e s o r e s [6]. C a n c e r s a r i s i n g in scars o f s m a l l p o x v a c c i n a t i o n s are u n c o m m o n , the first b e i n g r e p o r t e d b y A u g e r in 1943 [7]. T h e s e are u s u a l l y o f e p i t h e l i a l origin. I n 1993, a p a t i e n t p r e s e n t e d w i t h a l e i o m y o s a r c o m a in a r e c u r r e n t h y p e r t r o p h i c s m a l l p o x v a c c i n a t i o n scar; it i n f i l t r a t e d the u n d e r l y i n g s u b c u t i s a n d m u s c u l a t u r e [8]. T h i s is the first r e p o r t o f a l e i o m y o s a r c o m a a r i s i n g in a s m a l l p o x v a c c i n a t i o n scar.Case report
A 33-year-old female was vaccinated against smallpox on her left deltoid area. Five years later in 1976, a painful nodule 1.5 cm in diameter developed in the scar. The clinical diagnosis was a hy- pertrophic scar. It was excised but six years later it recurred and was again excised (1982). A second recurrence was excised in 1987. Histology confirmed the clinical diagnosis of scar hyper- trophy.
Four months after this a painful nodule appeared. Six years la- ter 1993, because of patient delay, a biopsy revealed a leiomyosar- coma. MRI and CT scans (Figs. 5, 6) determined the extent of the tumor and the patient was referred to us.
Correspondence to: S. Krupp, Avenue des Bergi~res 2, CH-1004 Lausanne, Switzerland
There was a 6 cm in diameter painful mass (Fig. 1) with a 1 cm nodule in the oblique scar of her left shoulder. The tumor was widely excised with the superficial portion of the underlying del- toid, the defect being 10.5 cm in diameter (Fig. 2). A split thick- ness skin graft was applied.
A biopsy of a scar-like structure in the skin transplant revealed another recurrence in October 1995. Resection of the deltoid muscle and musculocutaneous reconstruction, supplemented by pre- and postoperative radiotherapy was proposed but rejected by the patient.
Investigations
• Preoparative CT scans showed a lobular mass on the left deltoid area measuring 5 cm in the anteroposterior and 4 cm in the latero- lateral scan (Fig. 5). It was located in the subcutaneous tissue, in- filtrating the cutis with elevation of the overlying scar and adja- cent skin. No local and regional adenopathy was present.
• Preoperative MRI confirmed the CT scan findings (Fig. 6). The superficial muscle fibers of the deltoid were infiltrated.
• Histology showed proliferated spindle-shaped cells with abun- dant eosinophilic, fibrillar cytoplasm. Polymorphism was rare. Mitotic rate was 15-20 per 10 HPF (x400) (Fig. 7). The leiomyo- sarcoma was mainly located in the subcutis. It infiltrated the der- mis superficially and deeply spot-like in the deltoid muscle (Fig. 8). Histology of the overlying dermis showed characteristics of scar hypertrophy (preceding excisions and biopsies). Diagnosis - grade II sarcoma.
• Immunohistochemistry confirmed the presence of muscle specif- ic actin and desmin, but absence of cytokeratin and vimentin.
Discussion
M a l i g n a n t t u m o r s a r i s i n g in s m a l l p o x v a c c i n a t i o n scars are rare. T h e first was r e p o r t e d in 1943 [7], a n d 70 cases, i n c l u d i n g our one, h a v e b e e n d e s c r i b e d [8]. O f these, 48 w e r e m a l e a n d 22 f e m a l e , 5 8 % after p r i m a r y v a c c i n a - tion, 2 5 % after r e v a c c i n a t i o n , a n d 17% u n k n o w n . T h e m e a n a g e o f p a t i e n t s was 53 y e a r s , the y o u n g e s t p a t i e n t b e i n g 15, the o l d e s t 81. T h e m e a n l a t e n t p e r i o d ( i n t e r v a l b e t w e e n v a c c i n a t i o n and o n s e t o f the l e s i o n or c o n s u l t - ing a p h y s i c i a n ) was 25 year. T h r e e p a t i e n t s w e r e B l a c k , the m a j o r i t y w e r e C a u s a c i a n s . R i s k factors w e r e l o n g -
99
Fig. 1. Preoperative condition shows the leiomyosarcoma in a re- current hypertrophic smallpox vaccination scar. A nodule is visi- ble at one end of a 3 cm long scar
Fig. 2. Intraoperative view after wide local excision of the tumour. The cutis, subcutis and superficial part of the deltoid muscle have been removed
Fig. 3. The undersurface of the excised specimen containing the tumoral mass is shown in this figure
Fig. 4. Postoperative condition after 4 months. The wound had been covered with thin split thickness skin graft. The tissue defect has still not been reconstructed for oncologic reasons
Fig. 5. CT-scan at the level of the upper chest shows the tumour infiltrating the subcutaneous fat and the superficial part of the del-
toid muscle. The tumoral mass presents the same density as the adjacent muscles
Fig. 6. This MRI shows the location of the turnout in the subcuta- neous fat overlying the deltoid muscle
Fig. 7. Mitoses of the superficial leiomyosarcoma are shown in this figure. The tumour consists of fascicles intersecting at various angles (HE, x30)
Fig. 8. This figure shows the leiomyosarcoma invading the dermis (HE, xl0)
Fig. 9. This figure shows the leiomyosarcoma infiltrating the sub- cutaneous fat (HE, xl0)
t e r m p h y s i c a l i r r i t a t i o n such as sun e x p o s u r e or c h r o n i c scarring, the t r e a t m e n t o f c h o i c e w a s l o c a l e x c i s i o n [8].
F i f t y - s e v e n p e r c e n t o f m a l i g n a n c i e s in s m a l l p o x v a c - c i n a t i o n scars w e r e b a s a l c e l l c a r c i n o m a s . T h e m a l e to f e m a l e ratio was 3:1. T h e m e a n a g e o f p a t i e n t s was 54 years. T h e m e a n l a t e n t p e r i o d was 24 y e a r s [8]. T w e n t y - t w o p e r c e n t o f all r e p o r t e d m a l i g n a n c i e s in s m a l l p o x v a c c i n a t i o n scars w e r e m a l i g n a n t m e l a n o m a s . T h e m a l e to f e m a l e r a t i o was 3:2. T h e m e a n age o f p a - tients w a s 49 y e a r s , 5 0 % a r i s i n g b e t w e e n 39 a n d 58 y e a r s . T h e m e a n l a t e n t p e r i o d was 22 y e a r s [8]. F o u r t e e n p e r c e n t o f all r e p o r t e d m a l i g n a n c i e s in s m a l l p o x v a c c i n a t i o n scars w e r e s q u a m o u s c e l l c a r c i n o -
100
mas. The male to female ratio was 2; 1. The mean age of patients was 55 years. The mean latent period was 19 years [81.
Seven percent of all reported malignancies in small- pox vaccination scars were sarcomas. This represented five cases, one fibrosarcoma, two dermatofibrosarcomas protuberans, one malignant fibrohistiocytoma, and this leiomyosarcoma.
Acknowledgement.
We thank Drs. L. Guillon, G. Zimmer and E. Saraga of the Institute of Pathology of the University of Lausanne for the histologic examination of the specimen and Dr. H. Hauser, Department of Radiology of the Permanence de l'H6pital de la Tour, Meyrin, Geneva, for the radiologic imaging.References
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