The development of sarcomas in smallpox vaccination cars is very rare. Including this case, only five have been reported. This is the first report of a leiomyosarcoma arising in a smallpox vaccination sca

Krupp, S.

Sendi, P.

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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 t Reconstructive CHUV Lausanne, Switzerland Abstract. The development of  sarcomas in smal lpox vaccination scars is very rare. Including this case, only five have been reported. This is the first report of  a le iomyosarcoma arising in a smal lpox vaccination scar. Key  words :  Smal lpox vaccination scar - Le iomyosarco-  ma In 1882, Mar jo l in  descr ibed a squamous cell carc inoma developing in a chronic leg ulcer [1]. "Mar jo l in 's  ulcer" describes mal ignancy in scars [2], venous stasis ulcers, chronic osteomyel i t is  and chronic f istula and sinuses [3], i rradiat ion dermatit is [4], chronic traumatic wounds [5], and pressure sores [6]. Cancers arising in scars of  smal lpox vaccinations are uncommon,  the first being reported by Auger  in 1943 [7]. These are usual ly of  epithel ial  origin. In 1993, a patient presented with a le iomyosarcoma in a recurrent hypertrophic smal lpox vaccination scar; it inf i ltrated the underly ing subcutis and musculature [8]. This is the first report of  a le iomyosarcoma arising in a smal lpox vaccinat ion 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 ti sue, 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 Mal ignant tumors arising in smal lpox vaccination scars are rare. The first was reported in 1943 [7], and 70 cases, including our one, have been descr ibed [8]. Of  these, 48 were male and 22 female, 58% after pr imary vaccina- tion, 25% after revaccination, and 17% unknown. The mean age of  patients was 53 years, the youngest patient being 15, the oldest 81. The mean latent per iod (interval between vaccination and onset of  the lesion or consult- ing a physician) was 25 year. Three patients were Black, the major i ty were Causacians. Risk factors were long- 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 iew 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 kin 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 atvarious 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) term physical  irritation such as sun exposure or chronic scarring, the treatment of  choice was local excision [8]. F i f ty-seven percent of mal ignancies in smal lpox vac- cination scars were basal  cell carcinomas. The male to female ratio was 3:1. The mean age of  patients was 54 years. The mean latent per iod was 24 years [8]. Twenty-two percent of all reported mal ignancies in smal lpox vaccination scars were mal ignant melanomas.  The male to female ratio was 3:2. The mean age of  pa- tients was 49 years, 50% arising between 39 and 58 years. The mean latent per iod was 22 years [8]. Fourteen percent of  all reported mal ignancies in smal lpox vaccination scars were squamous cell carcino- 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 f 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 1. Marjolin JN (1828) Dictionnaire de m6decine. Paris, vol 21:46 2. Treves T, Pack GT (1930) The development of cancer in burn scars. Surg Gynecol Obstet 51:749-782 3. Cruickshank AH, McConnell EM, Miller DG (1963) Malig- nancy in scars, chronic ulcers and sinuses. J Clin Pathol 16:573-580 4. Scharnagl E, Smola MG, Hellbom BA, Pierer G, Hoflehner H (1991) Narbenkarzinom: Beobachtungen u d Ergebnisse in23 FNlen. Langenbecks Arch Chir 376:341-345 5. Look P, Kleinan W, Henze E. Das Fistelkarzinom auf dem Boden der chronischen Osteomyelitis. Zentralbl Chir 102: 998-1005 6. Mustoe T, Upton J, Marcellino V, Tun CJ, Rossier AB, Hach- end JH (1986) Carcinoma in chronic pressure sores. A fulmi- nant disease process. Plast Reconstr Surg 77:116-121 7. Auger C (1943) Cancer sur tatouage t sur cicatrice de vacci- nation antivariolique. Laval Med 18:300-307 8. Sendi P (1995) Case report of a cutaneous and subcutaneous leiomyosarcoma developing ina recurrent hypertrophic small- pox vaccination scar. Thesis, Facult6 M6d, University of Lau- sanne 

Leiomyosarcoma in a smallpox vaccination scar: case report and review of literature

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Leiomyosarcoma in a smallpox vaccination scar: case report and review of literature

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