Squamous cell carcinoma arising in a venous ulcer as a complication of the Klippel-Trenaunay syndrome

Simone, Clara De; Giampetruzzi, Anna Rita; Guerriero, Cristina; Masi, Mariangela De; Amerio, Paolo; Cina, G

doi:10.1046/j.1365-2230.2002.01009.x

Cited by 24 publications

(12 citation statements)

References 15 publications

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“…Cellulitis, hemorrhage, parasthesias, pulmonary emboli, stasis dermatitis, stasis ulcerations, and thrombophlebitis may also occur with varicosities 7,9,23,27 . In one report, a 48‐year‐old woman with KTS developed a large ulcer and squamous cell carcinoma on the involved leg 28 …”

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confidence: 99%

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Klippel–Trenaunay syndrome: A multisystem disorder possibly resulting from a pathogenic gene for vascular and tissue overgrowth

et al. 2006

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Section: Descriptionmentioning

confidence: 99%

“…7,9,23,27 In one report, a 48-year-old woman with KTS developed a large ulcer and squamous cell carcinoma on the involved leg. 28 Hypertrophy of bone and soft tissue is present in the majority of patients with KTS. 5,19 Bone and soft tissue hypertrophy may increase the length as well as the girth of an affected extremity.…”

Section: Descriptionmentioning

confidence: 99%

Klippel–Trenaunay syndrome: A multisystem disorder possibly resulting from a pathogenic gene for vascular and tissue overgrowth

et al. 2006

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“…Although malignant lesions associated with PWSs have been reported previously, 3–10 the etiologic factors seem to be highly individual. In the present case, no residual vascular lesion was present at the site of ulceration, whereas thick fibrous tissue was noted surrounding the SCC lesion.…”

Section: Discussionmentioning

confidence: 93%

“…Although more than 20 cases of basal cell carcinoma associated with PWSs have been previously reported, 3–6 association of squamous cell carcinoma (SCC) is extremely rare, with only 3 cases 7–9 reported to date in the English literature. We present herein a case of SCC developing within a PWS with a remote history of cryosurgery and discuss the possible etiology.…”

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Squamous Cell Carcinoma Arising in a Port-Wine Stain with a Remote History of Cryosurgery

et al. 2007

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The authors have indicated no significant interest with commercial supporters. P ort-wine stain (PWS) is a benign vascular malformation consisting of ectatic blood vessels situated predominantly in the superficial dermis, with a reported incidence of 0.3% of the population. 1 Currently developed laser treatment achieves observable lightening of PWS by reducing the number and size of the abnormal vessels without notable scar formation. 2 Previously applied methods, however, including surgical excision and grafting, radiation, dermabrasion, and cryotherapy, had produced unfavorable outcomes.The development of malignancy within a PWS lesion is a rare condition, and the mechanism for malignant changes in PWSs is not fully understood. MaganaGarcia and Magana-Lozano 3 proposed that tumor development might involve an oncogenic factor produced by the abnormal vessels that makes the overlying epidermis more susceptible to ultraviolet light. In contrast, some authors have suggested that prior treatments such as radiation are involved in the development of malignant skin lesions. 4Although more than 20 cases of basal cell carcinoma associated with PWSs have been previously reported, 3-6 association of squamous cell carcinoma (SCC) is extremely rare, with only 3 cases 7-9 reported to date in the English literature. We present herein a case of SCC developing within a PWS with a remote history of cryosurgery and discuss the possible etiology. Case ReportA 69-year-old male patient visited our clinic in November 2004 with a chronic ulceration within the boundary of a PWS involving the V1 to V2 area of the right trigeminal nerve. The history of the case was carefully taken from not only himself but also his older sister. His history revealed that he had undergone cryosurgery several times when he was 5 to 7 years old. He did not receive any other therapy including radiotherapy, laser therapy, and surgical excision. The ulcer (3 cm in diameter), of 7 months duration, was associated with a surrounding atrophic scar (Figure 1). A biopsy specimen taken from the margin of the ulcer showed histologic features of well-differentiated SCC. No distinct metastases were detected by systemic evaluation.With these findings, we performed local wide excision involving all scar tissue and the surrounding PWS in his right cheek on December 20, 2004. After affirmation of tumor-free margins on the basis of the pathologic report of frozen sections, a free deltopectoral flap sized 15 Â 10 cm was raised based on the second and third anterior perforating branches of the internal mammary vessel. The second costal cartilage was removed at a point 1.5 to 2 cm from the sternal border to facilitate isolation of a 2-cm segment of the internal mammary vessels, including the perforating branches. The internal mammary artery and vein were anastomosed to the superior thyroid artery and external jugular vein, respectively.

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“…In the literature there are casuistic reports on tumours associated with KTW syndrome, e.g., Hodgkin's lymphoma [1], rhabdomyosarcoma [2], squamous cell carcinoma of the skin [3], astrocytoma [4], meningioma [5], haemangiopericytoma [6] and angiosarcoma [7]. One patient has been described with Klippel-Trénaunay-Weber syndrome, who received previous radiation therapy to treat a benign haemangioma 1 year before the diagnosis of angiosarcoma [8].…”

Section: Discussionmentioning

confidence: 99%

Angiosarcoma With Malignant Peripheral Nerve Sheath TumourDeveloping in a Patient With Klippel–Trénaunay–Weber Syndrome

Ploegmakers

Pruszczyński

Rooy

et al. 2000

Sarcoma

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Purpose: We discuss the coexistence of Klippel-Trénaunay-Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications. Patient: We report on a 46-year-old man presenting with increasing pain and swelling of his right lower leg after fracturing his fibula. Since birth he was known as having the uncommon syndrome of Klippel-Trénaunay-Weber of his right lower leg. Methods: Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first thought to be a composite hemangio-endothelioma and/or angiosarcoma with lung metastases. Results: In the amputated extremity, a vascular malformation was found with tumour showing various components with foci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheath tumour. Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases 2 months postoperatively. Discussion: This case describes an angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel-Trénaunay-Weber syndrome. A case never described before in literature and probably, as in our case, difficult to diagnose at first biopsy.

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Squamous cell carcinoma arising in a venous ulcer as a complication of the Klippel-Trenaunay syndrome

Cited by 24 publications

References 15 publications

Klippel–Trenaunay syndrome: A multisystem disorder possibly resulting from a pathogenic gene for vascular and tissue overgrowth

Klippel–Trenaunay syndrome: A multisystem disorder possibly resulting from a pathogenic gene for vascular and tissue overgrowth

Squamous Cell Carcinoma Arising in a Port-Wine Stain with a Remote History of Cryosurgery

Angiosarcoma With Malignant Peripheral Nerve Sheath TumourDeveloping in a Patient With Klippel–Trénaunay–Weber Syndrome

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