Marjolin's ulcers: theories, prognostic factors and their peculiarities in spina bifida patients

BackgroundDue to improved care, more and more children born with spina bifida in rural Kenya are surviving into adulthood. This improved survival has led to significant challenges in their lifestyles, especially the need to ensure pressure ulcer prevention and treatment. Malignant degeneration of pressure ulcers in spina bifida patients is very rare. The author describes the clinical presentation of two pressure ulcer carcinomas that are at variance from classical descriptions.Materials and methodsAn internet/Medline/PubMed search of English literature for theories on Marjolin's ulcer evolution and prognostic features of Marjolin's ulcers was performed.A chart review of two young adults with spina bifida who had presented to the author's hospital between 2004 and August 2010 with chronic pressure ulcers found to be Marjolin's ulcers on histo-pathological examination was performed, and the clinical features are reported.ResultsThe two ulcers appeared clinically benign: one was a deep ulcer, while the other was shallow; both had normal, benign-appearing edges, and a foul smelling discharge. The two ulcers were surrounded by induration and multiple communicating sinuses, with no evidence of chronic osteomyelitis. The internet search revealed a total of nine theories on Marjolin's ulcer development, as well as seven clinical and four histological prognostic features.DiscussionThe multifactorial theory, a coalescence of a number of proposed theories, best explains the evolution of Marjolin's ulcers. Poor prognostic features include pressure ulcer carcinomas, lesions and location in the lower limbs/trunks, all present in the two patients making their prognosis dim: this is despite the surgical margins being clear of tumor. Benign appearance, induration and presence of multiple communicating sinuses are features that have not been previously described as presenting features of pressure ulcers carcinomas.ConclusionThere is need for spina bifida patients and their guardians/caretakers to receive a close follow-up throughout life; health education focused on pressure ulcer prevention as well as early treatment of pressure ulcers when they occur, will avert the development of Marjolin's ulcers, and save lives.

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title: Marjolin's ulcer arising in a burn scar.
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title: THE EXPERIMENTAL DISCLOSURE OF LATENT NEOPLASTIC CHANGES IN TARRED SKIN
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title: A New Concept in the Management of Marjolin's Ulcers
abstract: Marjolin's ulcers have a grave prognosis, especially when regional nodes are involved. Recent studies suggest such cancers are in an immunologically privileged sites due to the dense scar tissue. The prognosis has been shown to be much worse for tumors not having a round cell infiltrate prior to surgery, as in Marjolin's ulcers. The use of topical 5-fluorouracil (5-FU) induces a round cell infiltrate. Three case reports of large Marjolin's ulcers are presented which were first treated with topical 5-FU. Radical ablative surgery was avoided in these patients with a successful outcome.

title: HLA DR4 is associated with the development of multiple basal cell carcinomas and malignant melanoma.
abstract: An association between HLA DR4 and the development of multiple basal cell carcinomas (BCC) and malignant melanoma (MM) was detected in southern Australia. There were highly significant differences in HLA DR frequencies between patients with multiple BCCs and MM and matched patients with multiple BCCs only. These findings suggest that hereditary factors associated with the HLA system influence what types of multiple skin cancers people develop.

title: Deletion of the p53 gene in a patient with aggressive burn scar carcinoma.
abstract: Aggressive squamous cell carcinoma (SCC) is known to occur in scars that develop after a burn injury, especially in the underdeveloped areas of the world where care is lacking. Because most SCC are associated with abnormalities in tumor suppressor genes, particularly p53, we postulated that similar mechanisms may underlie the development of burn-associated SCC.



We analyzed tissue DNA from a patient who died from an aggressive SCC in a burn scar for evidence of p53 gene abnormalities by polymerase chain reaction and immunohistochemical staining for p53 protein.



Using polymerase chain reaction, the p53 gene could not be detected in DNA from the patient's cancer. The p53 protein was also undetectable by immunohistochemical staining.



These studies indicate that there was a homozygous deletion of the p53 gene in this burn-related carcinoma. Further studies of other patients may lead to new understanding of this cancer, explain in part the usual aggressive behavior, and lead to new methods of prevention and treatment.

title: Marjolin ulcers: secondary carcinomas in chronic wounds.
abstract: Marjolin ulcers are malignant tumors arising in chronic wounds. Strictly defined, they include carcinomas that transform from the chronic open wounds of pressure sores or burn scars. They behave aggressively and have a propensity for local recurrence and lymph node metastases. A retrospective study was done at a single institution identifying six individuals who had chronic wound ulcers that underwent malignant transformation into a carcinoma. Sinus tract degeneration in osteomyelitis was not included. The average latency time between ulcer formation and documentation of a malignancy was 30 years. All wounds were about the pelvis or flank. Major oncologic surgical procedures were done in an attempt to eradicate the cancer. High-grade tumors had positive lymph node metastases, portending a grave prognosis. All four individuals with nodal metastases eventually died of systemic disease. Early recognition and proper staging offers the best chance for cure.

title: Marjolin’s Ulcers in sub-Saharan Africa
abstract: BackgroundCutaneous malignancies are considered rare among Africans. Trauma, its sequelae, and other chronic non-healing wounds are known to predispose to malignant degeneration. Not much is known of the demographics of Marjolin’s ulcers in sub-Saharan Africa.MethodsPathology records on patients suspected to have Marjolin’s ulcers submitted to the Pathology Department were extracted from a database of 75,124 specimens. A review of the English literature on Marjolin’s ulcers from Nigeria, a sub-Saharan country, was also performed.ResultsOf 210 specimens from suspected Marjolin’s ulcers, 167 records had a histological diagnosis of malignancy, with a male to female ratio of 1:1.4, and a mean age of 48 years (range: 4–97 years). There were 163 (97.6%) squamous cell carcinomas, 3 (1.8%) sarcomas, and 1 (0.6%) malignant melanoma. Burn scars, chronic ulcers, osteomyelitis, and “other” ulcers constituted 82 (49%), 70 (42%), 9 (5.4%), and 6 (3.6%), respectively. Subjects in six sub-Saharan Marjolin’s ulcer studies had a mean age between 36 and 42 years, with a mean latent period 16 years.ConclusionsMarjolin’s ulcers in sub-Saharan African have a shorter latent period, and they occur in younger patients. Provision of early stable wound cover is essential for prevention of malignant degeneration of scars, while early appropriate intervention is crucial in the treatment of chronic ulcers.

title: Squamous-cell carcinoma complicating chronic osteomyelitis.
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title: Marjolin's ulcers arising in burn scars.
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title: Prognostic and therapeutic use of microstaging of cutaneous squamous cell carcinoma of the trunk and extremities
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title: Burn scar carcinoma: Diagnosis and Management
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