Marjolin Ulcer


Article Author:
Muneeb Shah


Article Editor:
Jonathan Crane


Editors In Chief:
Sebastiano Cassaro
Joseph Lee
Tanya Egodage


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
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Steve Bhimji
John Shell
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Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
3/3/2019 9:28:29 AM

Introduction

A Marjolin ulcer is a cutaneous malignancy that arises in the setting of previously injured skin, longstanding scars, and chronic wounds. Historically, Marjolin ulcers are named for French surgeon Jean Nicolas Marjolin and first described as ulcerations with dense villi arising within a burn cicatrix.[1] Squamous cell carcinoma is the most frequent malignancy identified, although other cell types have been described (i.e., basal cell carcinoma). Lesions are aggressive and carry a poor prognosis with a high rate of recurrence. Prevention with proper burn wound management and early recognition of malignant conversion followed by surgical resection, if possible, are of the utmost importance.

Etiology

Burn scars are the most common inciting condition that leads to the development of Marjolin ulcers.[2] Malignant degeneration occurs in 0.7% to 2.0% of burn scars that have been allowed to heal by secondary intention.[3][4] Other chronic inflammatory etiologies that lead to Marjolin ulcers include traumatic wounds, venous stasis ulcers, osteomyelitis, pressure ulcers, radiation dermatitis, stings, bites, and hidradenitis suppurativa/[1][3][4][5] Individuals that are immunocompromised, either due to disease state or medication, are at increased risk for malignant conversion.[5]

Epidemiology

Marjolin ulcers occur in all age groups, sexes, and races.[2] The average latency period from the time of the initial inciting wound to the discovery of malignant degeneration is between 30 and 35 years, with the average age at presentation being 59 years.[6][7] Men are more commonly affected than women (3:1), perhaps due to the increased frequency of burns in this population in general.[2] Unlike other forms of skin cancer, the risk is equal in all races/ethnicities.[8]

Pathophysiology

The pathophysiology has not been completely elucidated, but several mechanisms have been proposed, including chronic irritation, repeated re-epithelization, local damage to immune mechanisms of the skin, genetic predisposition, and toxins from local cell damage.[1] Burn scars tend to be raised above the natural skin, leading to chronic irritation. Obliteration of local lymphatic vessels, poor vascularization, and reduced Langerhans cell activity have been observed, allowing developing lesions to avoid immune detection.[9] Overall, the pathogenesis is likely multifactorial, with chronic irritation and local toxins leading to neoplastic changes that are allowed to proliferate in the setting of altered immune mechanisms.

Histopathology

Squamous cell carcinoma is the most frequent malignancy identified on histopathologic examination (80% to 90%).[2] Most lesions are well-differentiated, but poorly differentiated subtypes have also been identified. The second most common cell type is basal cell carcinoma (9.6%) followed by melanoma (2.4%). Rare cases of sarcoma, dermatofibrosarcoma, mucoepidermoid carcinoma, and leiomyosarcoma have been described.[10]

History and Physical

A nonhealing, ulcerative, or indurated lesion appearing in a chronic wound or scar should raise suspicion for malignant degeneration to a Marjolin ulcer.[2][7] Visually, the lesions have been described as ulcerative and foul-smelling with rapid growth and rolled, elevated margins.[7] Other clinical signs that suggest Marjolin ulcer formation include exophytic granulation tissue, bleeding, and regional lymphadenopathy.[4] The ulcerative form is more common and more aggressive than the exophytic form. Superinfection of the wound may be the first presenting symptom. Lesions can occur anywhere but most frequently affect the lower extremities, followed by the scalp, upper extremities, torso, and face.[3][10] Clinicians should pay close attention to all old scars on routine physical examinations and carefully note changes from prior visits.

Evaluation

Any suspicious nonhealing or ulcerative lesion that appears in a chronic scar should be biopsied to confirm the diagnosis. Physical examination of local lymph nodes should be performed. Some centers perform an ultrasound of regional nodes due to the high rate of nodal involvement.[11] Lymphatic mapping and sentinel node biopsy have also been suggested.[3] Other imaging (i.e., chest radiograph, brain computed tomography) can be considered on a case-by-case basis to assess for metastases.[3][11]

Treatment / Management

Management should focus on prevention as unresected burn wounds that heal by secondary intention are at increased risk for malignant degeneration.[2] Once discovered, no definitive treatment protocol exists for the management of Marjolin ulcers. The most widely accepted treatment options include Mohs surgery, wide local excision with 1 to 2 cm margins, and amputation proximal to the lesion.

Mohs surgery can be considered in lesions on the face, scalp, hands, feet, areolae, and other areas where improved cosmesis is desired.[12][13] Amputation is reserved for advanced-stage disease when wide local excision and Mohs surgery are not possible.[14] Defect coverage with local flap, free flap, or avascular graft is often performed but remains controversial as some studies have shown an increased risk of recurrence with reconstruction.[3][7][15] After resection, close follow-up is necessary due to the high risk of recurrence.

Lymph node dissection is controversial but can be considered with positive lymph nodes on physical or ultrasound examination.[3][11] Adjuvant radiation and chemotherapy are indicated if surgical resection is not possible. Agents that have been suggested include topical 5-fluorouracil, methotrexate, l-phenylalanine, and platinum-based therapy.[2][11] Radiotherapy is recommended with regional lymph node metastases, high-grade lesions (grade III), and lesions greater than 10 cm in diameter.[3]

Staging

No specific TNM staging criteria exist for Marjolin ulcers. After a biopsy has been performed, the histopathologic type of tumor can be used to stage the disease. For example, if the biopsy reveals squamous cell carcinoma, existing criteria established by the American Joint Committee on Cancer (AJCC) can be used.[2]

Prognosis

Although most Marjolin ulcers are well-differentiated, lesions are aggressive and carry a poor prognosis. Metastases are found in up to 27% of patients compared to 3% for other causes of squamous cell carcinoma.[3][7][10] The recurrence rate after surgical resection is up to 50%/[7][10] High grade (grade II or III), the presence of nodal metastases, and lower extremity location indicate a worse prognosis.[1][3][10] The 5-year survival rate is 43% to 58%.[12]

Pearls and Other Issues

  • Marjolin ulcers most commonly arise from burn scars.
  • The most common cell type identified is squamous cell carcinoma.
  • All suspicious lesions should be biopsied.
  • Confirmed cases of Marjolin ulcer should be excised with clear margins.
  • Marjolin ulcers can be prevented with early excision and grafting of burn wounds.
  • Prognosis is poor with a high rate of recurrence.

Enhancing Healthcare Team Outcomes

Prevention and management of Marjolin ulcers require a multidisciplinary team approach, including providers from surgery, primary care, oncology, and dermatology. Burn scars should be excised, and the surrounding skin should be reconstructed by a surgeon trained in the reconstruction (i.e., plastic surgeon) to prevent malignant conversion. For burn scars that have been allowed to heal by secondary intention, primary care providers (physicians, nurse practitioners, and physician assistants) must be aware that burn scars are at risk for malignant conversion. All burn scars should be monitored on routine physical examination, and patients should be educated on symptoms to prompt earlier presentation.[12] [Level V]

Wound changes that are suspicious for Marjolin ulcer should be immediately biopsied. Once a malignancy has been identified, a team approach to management should be initiated. Staging should involve an examination of the regional lymph nodes, either by physical or ultrasound examination. If the malignancy is thought to be confined to the scar, treatment should be discussed with a surgical oncologist, plastic surgeon, and Mohs surgeon. Resection with wide margins or Mohs technique should be performed depending on the location and desired cosmesis.[12] For those with positive lymph nodes, radiation oncology and medical oncology should be consulted for adjuvant radiotherapy/chemotherapy.

After treatment, follow-up surveillance should occur at regular intervals with a primary care provider or dermatologist to note any evidence of recurrence.


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Marjolin Ulcer - Questions

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A 50-year-old male presents complaining of a wound on his left lower leg. He has noticed a slow growing wound on his left leg over the past year. He denies recent injury or new medications. His vital signs are within normal limits. Physical examination reveals a 5-cm ulcer on the medial aspect of his lower leg within the borders of an old scar. A biopsy reveals squamous cell carcinoma. Which of the following is the most likely predisposing lesion?



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A 65-year-old male presents with complaints of bleeding and pain from a wound on his right lower extremity. He reports that over the past few weeks he has developed an "ulcer on my shin that refuses to heal" despite proper wound care. He denies recent trauma but has a remote history of third-degree burns to his right lower extremity that healed without intervention. His past medical history consists of hypertension. He has smoked one pack of cigarettes daily for the past 20 years. Physical examination reveals a 3-centimeter ulcer with rolled borders overlying the area of his burn scar. A biopsy reveals malignant cells. Which of the following cell types is most likely identified on histopathologic examination?



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A 58-year-old female comes to the physician complaining of pain in her left, lower leg. She reports noticing an open wound that has continued to grow over the past 3 months. Initially, the wound was painless but over the past week, she has developed increasing discomfort. Her temperature is 37 C, blood pressure 128/78 mmHg, and heart rate 78/min. Physical examination reveals a 2-cm ulcer on the medial aspect of her lower leg. It is cephalad to the medial malleolus and within the borders of an old scar. She remembers the scar from childhood but cannot recall how she was injured. What is the most likely diagnosis?



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A 68-year-old male presents complaining of a wound on his left cheek. He reports that the wound initially started out as a small bump but has now progressed to a large bleeding wound. He has a history of diabetes mellitus. Vital signs are within normal limits. Physical examination reveals extensive burn scars involving the left side of his face and a 1-cm ulceration with rolled borders inferior to his left zygomatic arch. There is no palpable cervical lymphadenopathy. A biopsy reveals squamous cell carcinoma, and he is diagnosed with a Marjolin ulcer. Which of the following is the best next step in management?



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A 59-year-old male presents with complaints of bleeding from a wound on his left lower extremity. He has a remote history of third-degree burns to his left lower extremity that healed without intervention. His past medical history consists of rheumatoid arthritis, for which he takes methotrexate. Physical examination reveals a 6-centimeter ulcer with rolled borders overlying the area of his burn scar. There is palpable left inguinal lymphadenopathy. A biopsy reveals well-differentiated squamous cell carcinoma. Which of the following has been shown to prevent malignant transformation of old burn scars?



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Marjolin Ulcer - References

References

Bazaliński D,Przybek-Mita J,Barańska B,Więch P, Marjolin's ulcer in chronic wounds - review of available literature. Contemporary oncology (Poznan, Poland). 2017     [PubMed]
Elkins-Williams ST,Marston WA,Hultman CS, Management of the Chronic Burn Wound. Clinics in plastic surgery. 2017 Jul     [PubMed]
Copcu E, Marjolin's ulcer: a preventable complication of burns? Plastic and reconstructive surgery. 2009 Jul     [PubMed]
Saaiq M,Ashraf B, Marjolin's ulcers in the post-burned lesions and scars. World journal of clinical cases. 2014 Oct 16     [PubMed]
Fleming MD,Hunt JL,Purdue GF,Sandstad J, Marjolin's ulcer: a review and reevaluation of a difficult problem. The Journal of burn care     [PubMed]
Pekarek B,Buck S,Osher L, A Comprehensive Review on Marjolin's Ulcers: Diagnosis and Treatment. The journal of the American College of Certified Wound Specialists. 2011 Sep     [PubMed]
CASTANARES S, Malignant degeneration in burn scars. California medicine. 1961 Mar     [PubMed]
Sadegh Fazeli M,Lebaschi AH,Hajirostam M,Keramati MR, Marjolin's ulcer: clinical and pathologic features of 83 cases and review of literature. Medical journal of the Islamic Republic of Iran. 2013 Nov     [PubMed]
Oruç M,Kankaya Y,Sungur N,Özer K,Işık VM,Ulusoy MG,Uysal A,Koçer U, Clinicopathological evaluation of Marjolin ulcers over two decades. The Kaohsiung journal of medical sciences. 2017 Jul     [PubMed]
Giesey R,Delost GR,Honaker J,Korman NJ, Metastatic squamous cell carcinoma in a patient treated with adalimumab for hidradenitis suppurativa. JAAD case reports. 2017 Nov     [PubMed]
Metwally IH,Roshdy A,Saleh SS,Ezzat M, Epidemiology and predictors of recurrence of Marjolin's ulcer: experience from Mansoura Universityxs. Annals of the Royal College of Surgeons of England. 2017 Mar     [PubMed]
Bradford PT, Skin cancer in skin of color. Dermatology nursing. 2009 Jul-Aug     [PubMed]
Iqbal FM,Sinha Y,Jaffe W, Marjolin's ulcer: a rare entity with a call for early diagnosis. BMJ case reports. 2015 Jul 15     [PubMed]
Connolly SM,Baker DR,Coldiron BM,Fazio MJ,Storrs PA,Vidimos AT,Zalla MJ,Brewer JD,Smith Begolka W,Berger TG,Bigby M,Bolognia JL,Brodland DG,Collins S,Cronin TA Jr,Dahl MV,Grant-Kels JM,Hanke CW,Hruza GJ,James WD,Lober CW,McBurney EI,Norton SA,Roenigk RK,Wheeland RG,Wisco OJ, AAD/ACMS/ASDSA/ASMS 2012 appropriate use criteria for Mohs micrographic surgery: a report of the American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery Association, and the American Society for Mohs Surgery. Journal of the American Academy of Dermatology. 2012 Oct     [PubMed]
Challa VR,Deshmane V,Ashwatha Reddy MB, A Retrospective Study of Marjolin's Ulcer Over an Eleven Year Period. Journal of cutaneous and aesthetic surgery. 2014 Jul     [PubMed]

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