Paraplegia in a patient with Von Hippel Lindau syndrome: surgical and reconstructive treatment of Marjolin's ulcer. A case report.
METHODS
Marjolin's ulcer is a squamous cell carcinoma that develops in posttraumatic scars and chronic wounds. Suspicion of such lesions should be raised in chronic wounds demonstrating characteristic changes. We have reported the peculiar phenomenon of malignant transformation of chronic pressure sores that occurred in a paraplegic patient.


OBJECTIVE
The aim of this study was to cover the extensive defects by a last resort reconstructive option.


METHODS
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.


RESULTS
A 40-year-old paraplegic man, with multiple hemangioblastomas of the brain and spinal cord due to Von Hippel Lindau syndrome developed pressure ulcers with unstable healing over the sacral, trochanteric, bilateral, and ischiatic areas after 15 years from neurosurgery. The biopsy result showed an invasive squamous carcinoma. Carcinomas in pressure sores are highly aggressive, and they need to be treated more radically. In our case we opted for a demolitive surgical treatment including musculocutaneous rotational flap harvested from total left thigh to cover the extensive defects. The limb was previously disarticulated.


CONCLUSIONS
In Marjolin's ulcer, multiple biopsies are the first-line modality for the early diagnosis as they are a safe method with high rate of accuracy. First-line treatment is surgery consisting of radical excision with lymph node dissection, if they are involved. Adjuvant radiation therapy may be used in selected patients. Management of massive pelvic defects can be a challenging problem. The pedicled lower limb flap offers a technique that can be considered as a last resort procedure for extensive defects where other options are insufficient or not available anymore. In our case the patient is disease-free after 2 years of follow-up.
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.
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A
Scalise
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.
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C
Tartaglione
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.
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Pierangeli
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.
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Bolletta
Department of Plastic and Reconstructive Surgery, Università di Torino (ASO, Città della Salute e della Scienza), Torino, Italy.
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Fraccalvieri
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.
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Grassetti
Spinal Unit, Azienda Ospedaliera Santa Corona, Pietra Ligure, Italy.
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Ottonello
Department of Clinical Surgical, Diagnostic and Paediatric Sciences, Università di Pavia, Italy.
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Nicoletti
Spinal Unit, Azienda Ospedaliera Santa Corona, Pietra Ligure, Italy.
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Massone
Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.
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Di Benedetto
D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000209:etiology / Q000601:surgery*; D002528:Cerebellar Neoplasms / Q000209:etiology / Q000601:surgery*; D018325:Hemangioblastoma / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008297:Male; D010264:Paraplegia / Q000209:etiology / Q000601:surgery*; D019651:Plastic Surgery Procedures; D013120:Spinal Cord Neoplasms / Q000209:etiology / Q000601:surgery*; D013848:Thigh / Q000601:surgery; D016896:Treatment Outcome; D014456:Ulcer / Q000150:complications / Q000601:surgery; D014945:Wound Healing / Q000502:physiology; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000601:surgery*