Giant exophytic Marjolin's ulcer of the lower leg after the gunshot wound: Case report

Introduction. Marjolin's ulcer is a skin malignancy that occurs on a scar or
 chronic wound. It most commonly occurs on a burn scar. Squamous cell
 carcinoma is the most common type of tumor, in more than 90% of cases. The
 rate of this rare malignant transformation is 1-2%. Marjolin's ulcer is more
 aggressive than other skin cancers. Wide excision is the treatment of
 choice. Recurrences are common. The aim of this article is to present a
 large exophytic carcinoma of the lower leg as a rare form of this tumor
 according to the size and type. The result of the radical surgery with
 reconstruction is also presented. Case report. Male patient, aged 52 years
 was presented by large exophitic tumor on the left lower leg. Tumor was
 located at the site of the previous gunshot injury. Latent period was 22
 years. Tumor size was 14x12cm. Wide excision was performed (2-cm surgical
 margin) including the deep fascia and the defect was closed by split
 thickness skin graft from the opposite thigh. Histology showed well
 differentiated squamous cell carcinoma. There were no regional or distant
 metastases. One year after surgery there was no recurrence of the tumor.
 Conclusion. Early diagnosis of Marjolin's ulcer and wide excision are
 mandatory. Surgical margins for excision should be 2 cm and excision should
 include deep fascia. Multiple and repeated biopsies of the chronic wounds
 are advised. There is no consensus on staging of the Marjolin's ulcer and
 lymph node dissection.


Introduction
Marjolin's ulcer (MU) is a term used for skin malignancy arising from a scar. This malignant transformation, which is rarely seen, is named after French surgeon Jean-Nicolas Marjolin. Initially, the term referred to squamous cell carcinoma that occurs on a burn scar, many years after the injury. Later, it was noticed that other types of the skin malignancy can occur, and that the tumor can occur not only on the scars from the injury, but also on the chronic wounds and the sites of chronic inflammation. 1 Marjolin`s ulcer is more aggressive than other skin cancers, with a metastasis rate of 27.5%. 2 Latent period or transition time, and is about 26 years. 3 Burns are the most common cause of malignant degeneration (68%). Squamous cell carcinoma occurs in 94% of cases. 4 Marjolin`s ulcer is more common in less developed countries. The most common localization is the lower extremity, in half of the patients. Men are more commonly affected than women (2:1).
Marjolin's ulcer is most commonly seen at the age of 55 years. 5 The diagnosis of Marjolin's ulcer is based on anamnesis, clinical picture and histological findings after biopsy. Venous ulcers, pressure sores and chronic hydroadenitis are most often mentioned in the differential diagnosis. Different sizes of Marjolin`s ulcers have been reported. Treatment is primarily surgical, wide excision and skin grafting. Radiotherapy and chemotherapy are usually adjuvant or palliative treatment for advanced cases of Marjolin's ulcer. Recurrence occurs in about 16.7% of patients. 6 The aim of this paper was to present a large Marjolin's ulcer of the lower leg at the site of previous gunshot injury, with giant proportions for the exophytic form of Marjolin's ulcer in this region.

Case report
A 52-year-old male patient presented with large fungoid soft tissue mass on the posteromedial part of the left lower leg. The tumor was located at the middle and distal 1/3 of the lower leg, measuring 14 x 12 cm. (Figure 1). The patient had a gunshot injury at the same site 22 years earlier, and the wound was left for healing by secondary intention. The growth of the tumor was slow, accompanied by bleeding and infection. The pain was of medium intensity. There was no distal neurovascular deficit. Inguinal nodes were not enlarged. Radiography showed great number of metal foreign bodies with no bone damage. (Figure 2) Pseudomonas aeruginosa was isolated and the patient was treated with amikacin. In general anesthesia, a wide tumor excision was performed, using margins of 3 cm. Underlying deep fascia was included. The defect was closed by split thickness skin graft from the opposite thigh. (Figure 3) Histological analysis showed well differentiated invasive squamous cell carcinoma, with histological grade I and nuclear grade I. (Figure 4).
There were no tumor elements at the edges of the resection. Tumor cells were not found in excised muscle samples No distant metastases were found on additional examinations. The skin graft was well consolidated and the donor site healed well without complication. One year after the surgery, there were no recurrence of the tumor and there were no regional and systemic metastasis.

Discussion
Marjolin's ulcer was named after the French doctor Jean-Nicolas Marjolin, who described an ulcer on a burn scar in 1828. In addition to the burn scar, which is the most common cause of Marjolin's ulcer (in 68% of cases), MU can also occur at scars from other injuries, surgeries, chronic ulcers (vascular, pressure sore), chronic osteomyelitis, site of previous frostbite, scars from skin grafts, donor sites of skin grafts, vaccine scars, fistulas (including AV fistulas), sinuses, chronic suppurative hidroadenitis, radiodermatitis, discoid lupus erythematosus, pemphigus, herpes zoster, leprosy ulcer, etc. 4 In general, Marjolin's ulcer occurs on scars from wounds that have healed by secondary intention, on chronic wounds and on the skin with chronic inflammation with frequent disruption of the skin.
There is the case of Marjolin's ulcer developed on the surface of the pleura several years after the treatment of empyema with the Eloesser flap. 7 The incidence of Marjolin's ulcers is estimated to be 2% in post-burn scars and 1.7% in chronic wounds. It occurs more common in older age and two times more common in men. In a study of Xiang et al among 140 patients with Marjolin's ulcer, the initial injury or disease was at the age of 1-75 years, and Marjolin's ulcer developed at the age of 15-85 years, averaging 53.3 years. 8 The latency period is 11-41 years, with an average of 28.8 years. The age of the patient at the time of injury is in negative correlation with the length of the latency period. Marjolin`s ulcer most often occurs on the lower leg (62%). Other sites are head (16%), upper extremity (12%), and trunk (10%). 9  The histological finding of well-differentiated type is characterized by islands of squamous cells with minimal cellular and nuclear atypia and rare pathological mitoses. Infiltrative type is formed easy, the degree of metastasis is high and prognosis is poor.
Exophytic form grows slower and the frequency of metastases is lower. 16  The largest infiltrative Marjolin's ulcer on the lower leg was described by Sakellarion et al., which was 19x11 cm in diameter. 18 The largest Marjolin's ulcer in general was described by Saraiya et al., as an ulcerative infiltrative form, measuring 43 x 23 cm. 19 The Marjolin's ulcer we described in the paper was 14x12cm in size, which ranks it as one of the largest exophytic MU, compared to those described in the available literature.
Treatment of Marjolin's ulcer is primarily surgical, wide excision or amputation in advanced cases of MU in extremities. There is no agreement about the safety margins.
Because of the tumor size and the margins of excision, direct closure is seldom possible.
Skin graft covering is preferred. Local skin flaps are not advised, except in defects with exposed uderlying tendon or bone. Split thickness skin graft offers good assessment of tumor recurrence. Lymph node dissection is controversial, but can be considered with positive lymph nodes on physical or ultrasound examination. According to Metwally  Online First February, 2021.