Importance of high clinical suspicion in diagnosing a Marjolin's ulcer with an unusual presentation

Marjolin’s ulcer is a squamous cell carcinoma (SCC) arising in the setting of chronic skin inflammation. The inflammation is most often secondary to a burn injury, although a range of tissue injuries have been reported as inciting events. We report an interesting case of a SCC arising in a non-healing dialysis catheter site. A 59 year old female with multiple medical problems, including a history of lupus and end stage renal failure, presented with a chronic, non-healing wound at the entry site of a previously placed, tunneled hemodialysis catheter. The catheter had been removed approximately one year prior to presenting to her primary care physician (Figure 1). Given the chronic nature and appearance of the wound, a punch biopsy was performed which demonstrated well- to moderately-differentiated invasive SCC at least 5.2 mm in thickness with no clear vascular or perineural invasion. Given the size of the primary lesion, she underwent systemic imaging with a PET/CT scan with no evidence of distant metastases. An MRI of the chest was also performed due to the proximity of the lesion to the clavicle with no evidence of clavicular involvement. Figure 1 Chronic wound at previous tunneled hemodialysis catheter site She was subsequently taken to the operating room where radical resection of the right chest wall tumor was performed. Additionally, a sentinel lymph node biopsy (SLNB) was attempted but lymphoscintigraphy mapping was unsuccessful in localizing a lymph node. The entire ulcerated area was excised with a two cm margin. Full thickness excision of pectoralis major muscle was performed near the clavicle where tumor was most adherent (Figure 2). Additionally, the entire length of the catheter tract was excised up to its insertion into the internal jugular vein, given that it was quite calcified and concerning for tumor extension. Frozen section analysis of the distal-most portion of this catheter tract as well as of the tissue immediately overlying the clavicle was negative. The final size of the excision defect was 15 X 15 cm. The sternocleidomastoid muscle was approximated to cover the internal jugular vein. A wound vac dressing was placed as a temporary dressing, with planned split thickness skin grafting when final pathology was confirmed to be negative. The final pathology showed well- to moderately-differentiated invasive squamous cell carcinoma with negative margins. In addition, the remaining portion of the catheter tract was found to contain benign fibrous tissue and skeletal muscle. Figure 2 Chest wall resection margin In our case, a high clinical suspicion for a Marjolin’s ulcer was a critical factor in identifying disease and planning appropriate surgical management. A significant delay in diagnosis occurred despite our patient’s risk factors for SCC, including a personal history of SCC and chronic immunosuppression. Surveillance for neoplasms is an important component of care for any patient with a chronic, non-healing wound such as ours; not only is the time course to development of a neoplasm unpredictable, but a growing body of evidence suggests increasing rates of metastatic SCC when the primary lesion arises in the setting of a chronic wound.1 Treatment of Marjolin’s ulcer is primarily surgical, consisting of a wide local excision to negative margins. Limited data exists to support exact margin width, although margins of two to four have been suggested.2 In practice, exact margin width is influenced by a number of factors including primary tumor location, size, and relationship with surrounding structures. In advanced cases presenting with bone, joint space, or extensive tissue involvement, limb amputation may be necessary to obtain negative margins. Advanced cases should be assessed by imaging to exclude the possibility of metastatic disease, as primary treatment in that setting is likely to be radiation or chemotherapy. Regional lymph node dissection should be considered for patients with clinically or histologically positive nodes after ruling out distant metastatic disease.2 Management of the clinically negative nodal basin is more controversial. In patients with traditional SCC, SLNB has been demonstrated to be accurate and is recommended for consideration in those patients at higher than average risk of nodal metastases.3 Factors reported to be associated with an increased risk of nodal metastases include tumor diameter >2.0 cm, tumor thickness >2.0 mm, and immunosuppression.3,4 Further, as previously mentioned, Marjolin’s ulcers are considered to be more aggressive than traditional SCC.1 As accurate assessment of nodal metastases impacts adjuvant treatment planning, it would be reasonable to consider SLNB for patients with Marjolin’s ulcers. Given the accuracy of SLNB, there is no clear role for elective lymph node.1 In conclusion, it is important to consider Marjolin’s ulcer in the setting of chronically inflamed skin or a non-healing wound with a low threshold for biopsy and further evaluation. Definitive surgical management includes wide local excision with two to four cm margins and consideration of SLN biopsy should be made. Given the aggressive nature of Marjolin’s ulcer, aggressive local control and systemic staging is critical to obtaining optimal cancer outcomes.