Keywords
Issue: Volume 61 - Issue 2 - February 2015 ISSN 1943-2720
Index: Ostomy Wound Manage. 2015;61(2):48-50.
Login or Register to download PDF
Abstract
Marjolin’s ulcer is the malignant transformation of long-standing chronic pressure ulcers and requires prompt diagnosis and treatment. A 46-year-old man with an 8-year history of traumatic spinal injury with paraplegia presented with a recurrent ischial pressure ulcer.
The initial ulcer, which developed 6 years earlier, was a Stage IV sacral ulcer. The wound was debrided and pathology showed epithelial hyperplasia, acanthosis, hyperkatosis accompanied by mild inflammation, and fibrosis without any malignant transformation. The lesion was covered with a fasciocutaneous bipedicled flap. Four years later, the patient presented with a similar ulcer in the same location. Histology showed the presence of a well-differentiated squamous cell carcinoma (SCC). Following a wide excision, the lesion was covered with a gluteal maximal V-Y musculocutaneous advancement flap. At last follow-up 14 months postoperatively, there was no evidence of recurrence or metastatic disease. Clinicians must be aware of known risk factors for the development of SCC.
Introduction
Pressure ulcers are among the most common conditions encountered in patients acutely hospitalized or requiring long-term institutional care. Approximately 1% of cutaneous skin cancers arise in chronically inflamed skin, and approximately 95% of these are squamous cell carcinoma (SCC).1,2 Cutaneous SCC is the second most common type of skin cancer in the United States behind basal cell carcinoma (BCC) and accounts for approximately 20% of nonmelanoma skin cancers. Other types of cancers include melanomas, fibrosarcoma, angiosarcoma, liposarcoma, leiomyosarcoma, osteosarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, malignant schwannoma, and mesenchymal tumor.3
Chronic inflammation is among the multiple known risk factors for the development of SCC.4 The latent period between injury and the development of cancer typically averages 31 years3; acute onset occasionally has been documented. Malignant degeneration of a chronic inflammatory skin lesion, known as Marjolin’s ulcer, is a rare and often aggressive cutaneous malignancy with a metastasis rate of 20% to 30%. Marjolin’s ulcer (a type of SCC) may result from previously traumatized, degenerated, chronically inflamed skin or scar tissue.5 It is commonly associated with burn scars but may develop secondary to pressure ulcers, chronic venous ulcers, traumatic wounds, osteomyelitis, fistulas, and leprosy ulcers.
The exact mechanism for development of Marjolin’s ulcer has not yet been defined but is likely multifactorial. Most occur in cases involving osteomyelitis, burn scars, and diabetic ulcers.3,5 Chronic irritation and repeated attempts at healing provide a prolonged stimulus for cellular proliferation and may increase the rate of malignant change. The development of SCC in pressure ulcers is even more rare, with an incidence of 0.5%.6 For unknown reasons, especially in spinal cord injury patients, 2-year mortality rates of 67% to 80% have been reported with pressure ulcer-related malignancies.7
Most pressure ulcer carcinomas are situated in the sacral and ischial region, which has rich lymphatic drainage to the pelvic region. This may explain the high metastatic rate.7 The patient with spinal cord injury who develops a large sacral pressure ulcer often has underlying osteomyelitis as well. It is important to distinguish whether the bone erosion is from infection or malignancy.8
According to Tobin and Sanger,9 early detection and aggressive management with wide local excision yield optimal results when treating patients with Marjolin’s ulcer. A lower recurrence rate has been noted when 3-cm to 5-cm safe margins were obtained.10 It is also imperative to provide early and definitive wound coverage or replacement of unstable scar tissue with healthy tissue to prevent the wound degeneration into SCC. Radiation therapy has been used as palliation; the response to systemic chemotherapy is generally poor.11,12
Although Marjolin’s ulcer is rare, awareness of the potential for an ulcer to become a Marjolin’s ulcer is important for chronic wound management. The case of a recurrent pressure ulcer that eventually progressed to a well-differentiated SCC (within 6 years of the initial presentation) is presented.
Case Report
Mr. Y, a 46-year-old man without any history of systemic disease, presented with a history of traumatic spinal cord injury with paraplegia for 8 years. Initially, he developed a Stage IV pressure ulcer over the right ischial region in 2008. Multiple fungating skin lesions arose from this ulcer’s subcutaneous pocket. The wound was debrided and pathology showed epithelial hyperplasia, acanthosis, hyperkatosis accompanied by mild inflammation, and fibrosis without any malignant transformation. Following excision, a fasciocutaneous bipedicled flap was used for resurfacing (see Figure 1).
Mr. Y subsequently remained relatively healthy until 2012; the lesion recurred in the same area and was 5 cm2 x 3 cm2 with the similar subcutaneous inflammatory pocket from the right ischial tuberosity. The lesion was debrided and the histological examination revealed a well-differentiated SCC (see Figure 2). The histological report revealed loss of maturation, nuclear pleomorphism with hyperchromasia, and atypical mitosis. No palpably enlarged lymph nodes were found in the right groin area. A sentinel lymph nodes biopsy was suggested, but Mr. Y refused. Magnetic resonance imaging showed a focal defect over the right ischial region with surrounding soft tissue swelling and fatty strand; no fistula formation or residual malignancy was noted. Abdominal sonography and positron emission tomography were performed to confirm the clinical staging and that no distal metastasis had occurred. Following a wide excision (2-cm margin), a gluteal maximal V-Y musculocutaneous advancement flap was used for reconstruction. Mr. Y tolerated surgery well, and he healed uneventfully without evidence of metastatic disease or local recurrence 14 months postoperatively (see Figure 3).
Discussion
Cutaneous SCC is the second most common type of skin cancer in the United States behind BCC and accounts for approximately 20% of nonmelanoma skin cancers. The development of SCC is among multiple known risk factors related to chronic inflammation.4 According to the Tobin and Sanger,9 early detection and aggressive management with wide local excision yield optimal results when treating patients with Marjolin’s ulcer. A lower recurrence rate has been noted when 3-cm to 5-cm safe margins were obtained.10 It is also imperative to provide early and definitive wound coverage or replacement of unstable scar tissue with healthy tissue to prevent the wound degeneration into SCC. Radiation therapy has been used as palliation; the response to systemic chemotherapy is generally poor.11,12 In this case, the patient did not undergo radiation or chemotherapy. He was cancer-free during his last follow-up visit 14 months postoperatively.
Conclusion
Awareness of known risk factors for the development of SCC and a high index of suspicion, especially with recurrent pressure ulcers, is necessary for early detection and the prevention of metastatic disease. To that end, histological tissue examinations are imperative to prevent missed or delayed diagnoses.
Affiliations
Dr. Chou is a plastic surgeon, Dr. Huang is a pathologist, Dr. Chiao is a plastic surgeon, Dr. Wang is a plastic surgeon, Dr. Sun is a family physician, Dr.S Chen is a plastic surgeon, Dr. DM Chen is a plastic surgeon, and Dr. Chang is a plastic surgeon, practicing in Taipei City, Taiwan. Please address correspondence to: changyi@office 365.ndmctsgh.edu.tw..
