Fibrosarcoma arising from a burn scar

ARTICLE

ejd.2011.1505

Auteur(s) : Ilkin Zindanci¹ ilkin.dr@gmail.com, Ebru Zemheri², Mukaddes Kavala¹, Emek Kocaturk¹, Burce Can¹, Zafer Turkoglu¹, Vasfiye Ulucay¹, Ozge Ozbulak¹

¹ Department of Dermatology

² Department of Pathology, Goztepe Training and Research Hospital, Altaycesme mah. zuhal sk F/22, Maltepe, 34765 Istanbul, Turkey

A 72-year-old female patient was referred for a mass arising from a burn scar located on her left gluteal area, which had been increasing in size for 4 months. Her medical and familial history revealed no major disease except a burn history that occurred when she was 2 years of age. Dermatological examination showed a 4 × 4 cm burn scar, a firm, infiltrated, cone-shaped nodular lesion with an ulcerated area (figure 1A). The tumor was surgically resected with a wide margin. In pathologic examination, a tumor infiltrating all the dermis and subcutaneous fat tissue was seen. Tumoral cells were spindle-shaped with pale eosinophilic cytoplasm and spindled nuclei with tapered ends. Cells flowed in interweaving fascicles or bundles, producing a herring-bone pattern in almost all areas (figure 1B). There was no storiform pattern, pleomorphism, multinucleated giant cells, necrosis or chronic inflammatory cells. Although there was expression of CD34 in the blood and lymphatic vessels, tumor cells were negative for CD34. The diagnosis of low grade fibrosarcoma was made based on these findings. Laboratory and radiological findings were all in normal ranges. No visceral metastasis was observed. The patient was scheduled for a 2-year follow-up to monitor any local recurrence. No recurrence was observed.

Fibrosarcomas are soft-tissue tumors that are mostly seen in young or middle-aged adults and are located on lower extremities, rarely in the head and neck region. It is a slow growing tumor, not normally causing any symptoms, so that the diagnosis is only made when the tumor has already reached massive sizes. It may arise from scar tissue formed after radiotherapy or burns [1]. In 1941, Fleming and Rezek were the first to report a case of fibrosarcoma on a 4-year-old burn scar [2]. Squamous cell carcinoma, basal cell carcinoma and, more uncommonly, malignant melanoma may also emerge on burn scars, respectively [3, 4]. Fibrosarcoma arising from burn scar is much rarer. Kowal-Vern and Criswell reported a diagnosis of fibrosarcoma only in three of 412 cases with a malignancy on their burn scar [4].

The reason for malignant transformation in a burn scar, resulting in development of malignant tumors, is not yet fully understood. Giblin et al. reported that repeated trauma and decreased elasticity of the tissues may provoke ulcer formation, resulting in malignant transformation [3]. Bostwick et al. proposed that limitation of immunity due to the absence or obstruction of lymphatic channels in scar tissues laid the grounds for the development of primary tumors [5]. Fleming and Rezek showed that the mesenchymal tissue tumors arising from burn scars are rare since the deep tissues are less prone to trauma and the regeneration capability of deep tissues is poor compared to superficial layers [2].

After a burn, there is a long latent period before the development of neoplasia. In one study, there was reported to be, on average, 35.5 years (range 3-71 years) [6]. The most common site of metastasis for fibrosarcomas is the lung, via hematogenous spread, followed by the bones. In histopathological examination, observation of bundles of collagen fibers in atypical spindle cells with a herringbone appearance is typical [1]. In differential diagnosis, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, fibromatosis and other sarcomas should be considered. The conventional treatment method is surgical resection. Adjuvant chemotherapy and radiotherapy can be applied in high grade fibrosarcomas. During follow-up, the 5-year survival was reported to be 40% [1].

In conclusion, burn scars should be followed up closely because of neoplasia development in the long term, and fibrosarcoma should always be kept in mind among the possible causes of neoplasias.

Disclosure

Financial support: none. Conflicts of interest: none.

References

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2. Fleming RM, Rezek P.R. Sarcoma developing in an old burn scar. Am J Surg 1941 ; 54 : 457.

3. Giblin T, Pickrell K, Armstrong D. Malignant degeneration in burn scar: Marjolin's ulcer. Ann Surg 1965 ; 162 : 291-297.

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