Cases reported "Facial Neoplasms"

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1/83. Genital angiofibromas in tuberous sclerosis: two cases.

    Cutaneous lesions are an important feature of tuberous sclerosis (TS). Facial angiofibromas usually begin to appear at the end of the first decade of life and are considered to occur in about 88% of adults with TS. They are only rarely reported on sites other than the face or front. In this paper, we report two patients with the complete syndrome of TS that had, in addition to classic facial lesions, multiple papules on the genital area. Histopathology confirmed the diagnosis of angiofibroma. One patient's lesions were misdiagnosed as genital warts and were so being treated before the correct diagnosis was made. Although we don't know the incidence of genital angiofibromas in TS patients, we believe that they might be underreported, because dermatologists generally don't look for them when they see these patients.
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2/83. The association of latent Epstein-Barr virus infection with hydroa vacciniforme.

    patients with hydroa vacciniforme (HV)-like eruptions and malignant potential have been reported from asia and mexico, and those patients frequently had an associated latent Epstein-Barr virus (EBV) infection. In order to elucidate the association of latent EBV infection with HV, we studied six children with typical manifestations of HV by detection of EBV genes and EBV-related RNAs in biopsy specimens from cutaneous lesions. Cutaneous lesions of all six children with typical HV contained EBV-encoded small nuclear rna (EBER) cells in 3-10% of the dermal infiltrates, whereas no Bam HI-H, l-fragment (BHLF) mRNA, or transcripts encoding EA-D antigen, were detected. No EBER cells were detected in other inflammatory or benign lymphoproliferative skin disorders tested. polymerase chain reaction amplification confirmed the presence of EBV dna sequences in five of six biopsy specimens from the patients. Latent EBV infection is associated with the development of cutaneous lesions of HV.
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3/83. Composite cervicofacial flap for reconstruction of complex cheek defects.

    The authors present the reconstructive technique for complex cheek defects using the composite cervicofacial flap and study the possibilities, advantages, disadvantages, and results that can be expected. The design follows the classic outline of Mustarde's flap. The skin is undermined for 2 cm anterior to the ear, then after incision of the superficial musculoaponeurotic system (SMAS), undermining is continued below the plane of the SMAS, level with the facial nerve branches. It is continued forward to the facial vessels, which give rise to branches that ensure the blood supply of this composite flap and contribute to its high reliability. In the cervical region, undermining is done beneath the platysma, which is transected transversely in the lower cervical region to allow good upward mobility and satisfactory transposition of the flap. The flap is adapted to the defect and the medial suture line is placed as near as possible to the medial limit of the cheek aesthetic unit. The authors carried out a retrospective study of 7 patients with complex facial reconstruction after excision of malignant lesions. The defects measured from 4x4 cm to 9x7 cm. In 4 patients excision included the periosteum, and in 1 patient excision involved the entire thickness and removed the entire anterior half of the cheek. In 4 patients reconstruction involved the cheek and eyelid. In spite of the advanced age of the patients (88, 69, 91, 67, 70, 82, and 59 years), there was no distal edge necrosis. The only complication was a single case of facial paresis, which resolved spontaneously. The results were considered very good in all 7 patients. The authors conclude that the composite flap increases the possibilities of the cervicofacial flap. It is more mobile, more reliable, thicker, and more adaptable. It can be used in complex cheek defects that involve the periosteum, or even in full-thickness defects. The quality of the results obtained using this flap represents a considerable advance in facial reconstruction.
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4/83. Proliferative actinic keratosis: three representative cases.

    OBJECTIVE: This article describes a new subtype of actinic keratosis that exhibits proliferative characteristics both histologically and clinically. We describe three representative cases occuring in the presence of infiltrative squamous cell carcinoma (SCC) and/or basal cell carcinoma (BCC). methods: Histories of each lesion in the three cases discussed were obtained. The lesions were removed by Mohs micrographic surgery. Permanent sections, stained with hematoxylin and eosin, were examined and studied under light microscopy. RESULTS: All three lesions had failed conventional treatment with liquid nitrogen and/or 5-fluorouracil (5-FU). Histologic examination of the lesions revealed sheets of dysplastic cells growing along the basal layer of the epidermis and migrating down hair follicles and sweat ducts. An associated infiltrative SCC and/or BCC was found in each case. CONCLUSIONS: Proliferative actinic keratosis is resistant to standard therapies because of deep migration of abnormal cells along hair follicles and sweat ducts. It has a strong propensity to develop infiltrative SCC and may occur concomitantly with BCC.
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5/83. Basal cell carcinoma in children: report of 3 cases.

    BACKGROUND: The peak incidence of basal cell carcinoma occurs in the seventh decade of life and is rare in children. When found in the pediatric age group, basal cell carcinoma is usually associated with a genetic defect, such as basal cell nevus syndrome, xeroderma pigmentosum, or nevus sebaceus. In areas of intense UV radiation exposure, such as the southwestern united states, children may be at increased risk of developing this malignancy de novo. OBSERVATIONS: Three children (2 boys, aged 8 and 16 years, and an 11-year-old girl) from Tucson, Ariz, with isolated basal cell carcinoma unassociated with any other disease or syndrome are described. CONCLUSIONS: Basal cell carcinoma in children is probably the result of a combination of UV radiation exposure and genetic background. Early recognition in children can prevent extensive tissue destruction and excess scarring after excision. A higher index of suspicion for basal cell carcinoma may also aid in prompt diagnosis of a possible genetic disorder, such as basal cell nevus syndrome.
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6/83. arteriovenous malformations of the orofacial area.

    Vascular lesions are pathological residues of the embryonic vascular system and can be divided into two main groups. The first group comprises haemangiomas, which are typical of childhood and involute spontaneously. The second group is formed by lesions without active proliferation, which include, among others, arteriovenous malformations that are congenital and grow proportionately with the subject. The authors present two cases of arteriovenous malformations of the orofacial area and discuss possibilities for modern diagnosis and treatment. Precise diagnosis and effective treatment of vascular lesions should be ensured by a diagnostic and therapeutic team of specialists from several disciplines (maxillofacial, ENT, plastic and general surgeon, paediatrician, haematologist, anaesthesiologist and possibly a neurosurgeon), headed by an intervention radiologist.
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7/83. Malignant transformation in congenital sebaceous naevi in childhood.

    Sebaceous naevi are uncommon congenital skin lesions with a well-recognised potential for neoplastic change. They should be considered premalignant lesions as malignant degeneration, most commonly basal cell carcinoma and squamous cell carcinoma, occurs with a lifetime risk of between 5% and 22%. This incidence is equal to that of actinic keratosis and exceeds that of oral leukoplakia. Such change, however, is rare before puberty. Basal cell carcinoma may develop in children with naevoid basal cell carcinoma syndrome, xeroderma pigmentosum and rarely de novo but sebaceous naevus is the only solitary lesion in childhood associated with the development of basal cell carcinoma. We present two cases of malignant transformation in a congenital sebaceous naevus occurring in childhood and review the literature and discuss the evidence upon which to base management guidelines.
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8/83. Merkel cell carcinoma: an overview and case report.

    Merkel cell carcinoma is an extremely rare neoplasm with a high rate of recurrence and metastasis. The lesions predominantly arise on sun-exposed areas of skin in whites between the sixth and seventh decades of life. Within the head and neck region, the cheeks and eyelids are the most common sites. This article outlines the etiology, pathogenesis, and treatment of this rare but highly aggressive tumor.
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9/83. Ectopic extramammary Paget's disease arising on the cheek.

    BACKGROUND: Extramammary Paget's disease (EMPD) is a rare neoplasm affecting apocrine gland-bearing skin. patients usually present with a long-standing unilateral, ill-defined, erythematous or whitish-gray, crusted patch or plaque. This tumor almost never occurs outside of the anogenital or axillary areas. OBJECTIVE: We report the first case of "ectopic" extramammary Paget's neoplasm afflicting the face. methods: The excisional specimen from a 67-year-old man was reviewed. histology and immunohistochemistry studies were performed on the specimen. RESULTS: histology revealed hyperkeratosis, parakeratosis, acanthosis, and proliferation of cytologically atypical epithelioid cells permeating the epidermis and the cutaneous adnexae. Special stains showed reactivity to cytokeratins (AE1/AE), epithelial membrane antigen, and carcinoembryonic antigen in the atypical cells. There was no evidence of apocrine glands in the area. CONCLUSION: Herein we present the first case of truly "ectopic" extramammary Paget's disease on the face.
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10/83. Intradermal nodular fasciitis of the face.

    Nodular fasciitis represents reactive myofibroblastic proliferative lesions in the subcutaneous tissue, skeletal muscles, and fascia. Uncommon clinical and pathological variants of nodular fasciitis, such as intravascular, cranial, ossifying, and proliferative fasciitis, have been described. Six cases documenting a dermal location of nodular fasciitis have been reported in the English literature. Some authors have described that the histopathological features of intradermal nodular fasciitis are virtually identical to those of postoperative/posttraumatic spindle cell nodule of the skin. We herein report a 41-year-old Korean woman presenting with a 0.5 cm facial papule in which nodular fasciitis occurred in the dermis.
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