Cases reported "Carcinoma, Squamous Cell"

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11/28. Increased risk of squamous cell carcinoma in junctional epidermolysis bullosa.

    Non-Herlitz junctional epidermolysis bullosa (JEB) is an autosomal recessive genodermatosis characterized by skin fragility and blistering. It is usually caused by mutations in the genes encoding the basement membrane proteins laminin 5 or type XVII collagen. Clinically, impaired wound healing and chronic erosions cause major morbidity in affected patients. Previously it was thought that these individuals, unlike patients with dystrophic EB, did not have an increased risk of developing skin cancer. However, we describe three patients with non-Herlitz JEB (aged 42, 56 and 75 years) who developed cutaneous squamous cell carcinomas (SCCs). The tumours were well-differentiated in two cases, but one patient had multiple primary SCCs that were either well- or moderately differentiated. Most cases of SCC in non-Herlitz JEB described have occurred in those with laminin 5 defects and on the lower limbs. These clinicopathological observations have important implications for the management of patients with this mechanobullous disorder as well as providing further insight into the biology of skin cancer associated with chronic inflammation and scarring.
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keywords = epidermolysis bullosa, epidermolysis, bullosa
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12/28. Squamous cell carcinoma of the maxillary sinus and palate in epidermolysis bullosa: CT demonstration.

    Extracutaneous squamous cell carcinoma is an unusual complication of epidermolysis bullosa (EB). We present a patient with long-standing EB who developed squamous cell carcinoma involving the palate and the maxillary sinus, and we describe the findings on CT.
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keywords = epidermolysis bullosa, epidermolysis, bullosa
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13/28. Management of squamous cell carcinoma in a patient with recessive-type epidermolysis bullosa dystrophica.

    BACKGROUND: Surgical treatment of a recessive dystrophic epidermolysis bullosa patient is very difficult for both the surgeon and the anesthetist because of the fragility of the skin and abnormal nature of the tumor bed. OBJECTIVE: We report a case of 54-year-old Japanese recessive dystrophic epidermolysis bullosa patient with squamous cell carcinoma (SCC) of the lateral malleolus. methods: A tumor measuring 5.0 x 5.5 cm was surgically excised. The defect was then reconstructed by full-thickness skin grafting. To avoid airway complications, general anesthesia was administered using a face mask. Because the regional lymph nodes were swollen before surgery, the patient underwent sentinel lymph node biopsy. RESULTS: The patient remains well with no sign of recurrence or metastasis 7 months after surgery. CONCLUSION: To preserve activities of daily living, surgery should be performed for squamous cell carcinomas arising in recessive dystrophic epidermolysis bullosa patients.
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ranking = 1.4
keywords = epidermolysis bullosa, epidermolysis, bullosa
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14/28. Artificial skin as a valuable adjunct to surgical treatment of a large squamous cell carcinoma in a patient with epidermolysis bullosa.

    BACKGROUND: Among tissue-engineered skins, two bilayered cellular constructs and one cryopreserved dermal substitute have been approved for the treatment of epidermolysis bullosa. Nevertheless, the application of artificial skin technology to surgical treatment of squamous cell carcinomas in a patient with epidermolysis bullosa has never been reported. OBJECTIVE: To reconstruct the large defect remaining after squamous cell carcinoma excision in a patient with dominantly inherited dystrophic epidermolysis bullosa. methods: To apply a 10 x 15 cm Integra sheet (Integral life-sciences Corporation, Plainsboro, NJ, USA) (an acellular collagen matrix coated with a thin polysiloxane elastomer) to the excised area and 3 weeks later to cover the Integra sheet with an ultrathin meshed skin graft. RESULTS: The graft take was complete, and the donor site totally regenerated, except for three small bullae at 7 weeks postoperatively. CONCLUSION: Integra offers the advantage of filling huge defects with its dermal layer of collagen fibers and provides an optimal graft bed. This first step makes it possible to use very thin grafts 3 weeks later.
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ranking = 1.4
keywords = epidermolysis bullosa, epidermolysis, bullosa
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15/28. Metastatic squamous cell carcinoma resembling angiosarcoma complicating dystrophic epidermolysis bullosa.

    We report a patient with generalized recessive dystrophic epidermolysis bullosa (RDEB) who developed 3 squamous cell carcinomas. The tumours appeared simultaneously at acral sites on both upper limbs and were poorly differentiated. Despite surgery and radiotherapy the patient died from metastatic disease within 6 months of presentation. This case highlights many of the typical features of this complication of RDEB, including the overall poor prognosis. Of particular interest was the histology of one of the tumours which caused diagnostic difficulties: haematoxylin and eosin staining suggested an angiosarcomatous pathology, but the use of immunocytochemistry proved that the tumour was a squamous cell carcinoma in origin.
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keywords = epidermolysis bullosa, epidermolysis, bullosa
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16/28. Squamous cell carcinoma developing in epidermolysis bullosa dystrophica.

    Two patients with epidermolysis bullosa dystrophica recessiva who had squamous cell carcinoma are presented. Case 1 is a 40-year-old woman who had ulcers on her left lower leg. Case 2 is a 42-year-old man who had a tumor on his left first toe. Wide surgical excision with skin coverage by autograft was performed in case 1. amputation of the toe in case 2 was performed. A review of the cases of epidermolysis bullosa dystrophica associated with cancer reported in japan is also presented.
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ranking = 1.2
keywords = epidermolysis bullosa, epidermolysis, bullosa
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17/28. Bazex syndrome (acrokeratosis paraneoplastica). An analytic review.

    Bazex syndrome (acrokeratosis paraneoplastica) is characterized by a psoriasiform eruption that favors acral sites and has been associated with an underlying malignancy in all reported cases. Of the 93 patients in this series, 89 were male with a mean age of 60 /- 8.5 years. Squamous cell carcinomas of the head and neck and squamous cell tumors of unknown primary with cervical lymph node metastases were the most commonly associated neoplasms, suggesting that the factor(s) responsible for the development of the syndrome are relatively specific for tumors of the upper aerodigestive tract. The cutaneous lesions were erythematous to violaceous in color and had associated scale; the most frequently observed sites of involvement were the ears, nose, hands, and feet, including the nails. In 63% of the cases, the cutaneous lesions preceded the initial symptoms or diagnosis of the tumor by an average of 11 months (range, 1-72) and, in general, the eruption was resistant to a variety of topical treatments. Occasionally, a reappearance of the papulosquamous lesions signaled the recurrence of the tumor (6 cases) or the appearance of skin lesions coincided with the development of metastatic disease (3 cases). In 91% (64/70) of the patients, the skin eruption either improved significantly following treatment of the underlying malignancy or did not improve in the setting of persistent tumor. However, even when all of the skin lesions cleared, the nail dystrophy often persisted. Fifteen of the patients developed vesicles, bullae, and crusts in addition to papulosquamous lesions. Possible explanations include the formation of an epidermal-dermal split via a bullous lichen planus-like mechanism, or the coexistence of two diseases; i.e., acrokeratosis paraneoplastica plus either porphyria cutanea tarda, bullous pemphigoid, or epidermolysis bullosa acquisita. One possible explanation for the development of the characteristic cutaneous eruption is an immune reaction, humoral or cellular, directed against a common antigen present on the tumor and the normal skin. Alternatively, tumor production of a keratinocyte growth factor such as TGF-alpha may be involved in the induction of the psoriasiform skin lesions.
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ranking = 0.2
keywords = epidermolysis bullosa, epidermolysis, bullosa
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18/28. Squamous cell carcinoma in epidermolysis bullosa. Treatment with systemic chemotherapy.

    Dystrophic epidermolysis bullosa is associated with a high incidence of cutaneous squamous cell carcinoma. Despite aggressive surgical treatment, metastases occur frequently, and survival is generally poor. Chemotherapy for advanced disease has usually been avoided because of the potential for severe cutaneous toxicity. Two patients with autosomal recessive dystrophic epidermolysis bullosa and advanced squamous cell carcinoma are described. Both received cisplatin-based systemic chemotherapy without significant toxicity.
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ranking = 1.2
keywords = epidermolysis bullosa, epidermolysis, bullosa
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19/28. death from metastatic, cutaneous, squamous cell carcinoma in autosomal recessive dystrophic epidermolysis bullosa despite permanent inpatient care.

    A 36-year-old female patient with severe autosomal recessive dystrophic epidermolysis bullosa, who had spent her entire life from age 2 as an inpatient in the dermatology unit, recently died of metastatic squamous cell carcinoma of the skin. The development of malignancy was not prevented by continuous medical and nursing supervision and, despite early detection, rapidly led to her death. Oral phenytoin and topical mupirocin ointment had not reduced blistering.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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20/28. Successful prosthetic fitting of a patient with epidermolysis bullosa dystrophica. Case report.

    A patient with recessive dystrophic epidermolysis bullosa developed a squamous cell carcinoma of the right foot resulting in a below the knee amputation. Despite the multiple real and potential skin problems of the stump, she was successfully fitted with a simply designed prosthesis and rehabilitated.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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