1/20. Nevoid basal cell carcinoma syndrome.A case report of a young girl with nevoid basal cell carcinoma syndrome is presented. The patient showed cutaneous and skeletal findings characteristic of the syndrome. Multiple basal cell carcinomas, rib abnormalities, along with clinical evidence of frontal bossing and ocular hypertelorism were the primary features of the syndrome in this patient. It is suggested that other characteristics of the syndrome, such as jaw cysts, palmar and plantar pitting and calcification of the falx cerebri will develop as the patient grows older. Careful observation, particularly for medulloblastoma and malignant degeneration and invasiveness of basal cell carcinomas, will be an integral part of this young patient's care.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
2/20. Multiple hereditary infundibulocystic basal cell carcinomas: a genodermatosis different from nevoid basal cell carcinoma syndrome.BACKGROUND: Infundibulocystic basal cell carcinoma is a recently described distinctive clinicopathologic variant of basal cell carcinoma. Histopathologic differential diagnosis among infundibulocystic basal cell carcinoma, trichoepithelioma, and basaloid follicular hamartoma has generated controversy in the literature. OBSERVATIONS: Members of 2 families with multiple infundibulocystic basal cell carcinomas are described. Each patient showed multiple papular lesions, mostly located on the face. No patient showed palmar pits or jaw cysts. Forty-two cutaneous lesions from 5 patients were studied histopathologically. Thirty-nine lesions were infundibulocystic basal cell carcinomas. This clinicopathologic variant of basal cell carcinoma consists of a relatively well-circumscribed basaloid neoplasm composed of buds and cords of neoplastic cells arranged in anastomosing fashion and with scant stroma. Some of the neoplastic cords contain tiny infundibular cysts filled by cornified cells with abundant melanin. Linkage analysis in family 2 was performed using polymorphic markers (D9S196, D9S280, D9S287, and D9S180), and the affected members shared the same haplotype. loss of heterozygosity analysis was performed in 2 affected members of this family from whom tumoral dna was available, and although these individuals were constitutively heterozygous for D9S196, they did not show loss of heterozygosity for this marker in their neoplasms. CONCLUSIONS: Multiple hereditary infundibulocystic basal cell carcinomas represent a distinctive genodermatosis different from multiple hereditary trichoepitheliomas and nevoid basal cell carcinoma syndrome. We propose clinical and histopathologic criteria to distinguish infundibulocystic basal cell carcinoma from trichoepithelioma, basaloid follicular hamartoma, and folliculocentric basaloid proliferation.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
3/20. Palmar basal cell carcinoma: case report and literature review.BACKGROUND: Although basal cell carcinoma (BCC) is the most common tumor of the skin, its occurrence on the palm is very rare. Only eight cases not associated with the basal cell nevus syndrome having been reported in the world literature so far. Among these eight cases, one was associated with epidermolysis bullosa dystrophica and another with previous trauma to the site of the appearance of the lesion. OBJECTIVE: The authors report a case of palmar BCC due to its rare occurrence on that site. The case is not associated with any predisposing factors such as basal cell nevus syndrome, trauma, or preexistent dermatosis. methods: An incisional biopsy was performed into the border of the palmar ulcer. The histopathologic findings of the fragment, stained by hematoxylin and eosin, were characteristic of BCC. RESULTS: Following surgical removal of the lesion, there was complete healing and the patient has presented no signs of relapse after 3 years of clinical follow-up. CONCLUSION: Palmar BCC not associated with the basal cell nevus syndrome or any other predisposing condition is very rare. Nevertheless, when facing an ulcerated lesion on the palm, BCC should be considered as a diagnostic possibility.- - - - - - - - - - ranking = 4keywords = palm (Clic here for more details about this article) |
4/20. Basal cell carcinoma of the eyelid associated with Gorlin-Goltz syndrome.PURPOSE: To describe the ophthalmic and systemic features in a series of patients initially seen with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Of 105 consecutive patients with eyelid basal cell carcinoma managed at an Ocular Oncology Center between January 1973 and December 1999, four patients with Gorlin-Goltz syndrome were identified. methods: The ophthalmic and systemic features, management, and outcome of patients with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome were analyzed. The published literature on Gorlin-Goltz syndrome, specifically related to genetics, systemic features, ophthalmic associations, and prophylactic management strategies, was reviewed. MAIN OUTCOME MEASURES: Response of the eyelid basal cell carcinoma to treatment and the final systemic condition were the main outcome measures. RESULTS: All four patients had a family history of Gorlin-Goltz syndrome. The systemic manifestations included multiple basal cell carcinomas in all the patients, frontal bossing or increased occipitofrontal circumference in three patients, palmar pits in two patients, odontogenic keratocyst in one patient, ectopic calcification in one patient, and bifid rib in one patient. The mean age at the detection of the first basal cell carcinoma was 30 years (range, 16-38 years). All four patients had multiple basal cell carcinomas on the face and elsewhere. The eyelid basal cell carcinoma was advanced with orbital infiltration in three patients, one of whom opted for palliative radiotherapy, whereas the other two underwent orbital exenteration. The fourth patient, who had localized recurrent basal cell carcinoma in the upper eyelid, was treated with excision and eyelid reconstruction. At the final follow-up (mean, 41 months), eyelid basal cell carcinoma was cured in three patients and stable in one patient. No patient had life-threatening sequelae of Gorlin-Goltz syndrome. CONCLUSIONS: Gorlin-Goltz syndrome is a rare autosomal dominant cancer predisposition syndrome that may be associated with eyelid basal cell carcinoma. The associated systemic findings may be a clue to the diagnosis of this condition. It is important to recognize Gorlin-Goltz syndrome when a patient has multiple basal cell carcinomas or when a young patient with eyelid basal cell carcinoma is seen by an ophthalmologist, because lifelong monitoring is essential for patient management.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
5/20. Trichoblastoma of the skin occurring in the breast. A case report.BACKGROUND: Trichoblastoma is a rare benign skin appendage tumor constituted mostly of follicular germinative cells. It can arise on any part of the body except the palms, soles, nail units and mucosal membranes. No case of it in breast skin has been reported before. Furthermore, fine needle aspiration cytology findings on this lesion have not been described before. CASE: A 76-year-old female presented with a firm nodule in her left breast. The tumor was well demarcated, about 1.5 cm in diameter. Fine needle aspiration cytology revealed clusters composed of relatively uniform cells with a high nuclear/cytoplasmic ratio. In the midst of some clusters, the tumor cells had a more abundant cytoplasm. Fibrocellular interstitium or dense cyanophilic acellular material occasionally was attached to them. The tumor cells had oval or fusiform nuclei that had fine, evenly dispersed chromatin. To exclude a diagnosis of breast cancer, it is important to recognize that the clusters are composed of basaloid cells with focal squamous eddies and that there is at least focally peripheral palisading. The histopathologic diagnosis was trichoblastoma. CONCLUSION: Fine needle aspiration cytology can distinguish trichoblastoma from malignant diseases of the breast and may be used to diagnose the lesion in conjunction with clinical findings.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
6/20. Nevoid basal cell epithelioma syndrome in japan.A 49-year-old Japanese man with typical features of nevoid basal cell epithelioma syndrome is reported. The patient had cystic lesions of the jaw bone, multiple basal cell epitheliomas of the scalp and face, pits on the palms and soles, bifid ribs, scoliosis and calcification of the falx cerebri. In japan five certain cases have been reported previously. We added one new case and reviewed the other five cases briefly.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
7/20. Coverage of the cranium with latissimus dorsi in the recurrent basal cell carcinoma of Gorlin syndrome: for protection against clinical invasion.Gorlin's syndrome is a genetic disorder of autosomal dominant inheritance with characterized primarily by five major findings: multiple basal cell carsinoma (BCC), jaw cysts, pits on the palms and soles, ectopic calcification of the falx cerebri and skeletal anomalies. BCC is the most frequently accompanied tumor with this syndrome. The risk of recurrent BCC with Gorlin's syndrome is higher than non-syndromic BCC. The authors present a 25-year-old man affected by recurrent basal cell carcinoma on the scalp. The patient was treated by excising the tumor and reconstructing latissimus dorsi musculocutaneous flap. The fascial component of the scalp forms an additional layer between the skin and the cranium. This structure creates an extra distance before the invasion into the cranium which needs to be penetrated by the skin tumor. Muscle tissue transformed in scalp reconstruction imitates the fascia layer in forming an additional layer against the invasion of skin tumors such as recurrent BCC into the cranium. Free flap reconstruction for recurrent scalp BCC can be best therapy model at Gorlin's syndrome.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
8/20. Nevoid basal cell carcinoma syndrome (Gorlin's syndrome): a case report.A case of nevoid basal cell carcinoma syndrome is presented and its varied clinical manifestations and multi-system involvement are emphasised. Our case presented with an early onset of symptoms but sought medical help later on for progressively increasing jaw swelling and pain. On further evaluation, multiple pigmented skin papules, palmar pits, multiple jaw cysts, skull bone osteoporosis, bifid ribs and kyphosis were present. Systemic involvement was minimal. There was no significant family history.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
9/20. Nevoid basal-cell carcinoma syndrome.The nevoid basal-cell carcinoma syndrome is characterized by major manifestations, such as multiple basal-cell carcinomata, cysts of the jaws, and skeletal--specifically, rib--abnormalities. Findings in 53 patients and a review of the literature document both major and lesser-known manifestations of the disorder. The odontogenic keratocyst, which usually appears during adolescence, has a marked tendency to recur. In addition to the skin tumors, milia, epidermoid cysts, chalazia, comedones, and palmar and plantar pits are frequent. The skin tumors, originally thought to be independent of sun exposure, are more common in sun-exposed areas and are far less frequent and occur at a much later age in blacks than in whites. There is some evidence that radiation of the skin promotes the appearance of skin cancers in this disorder. Unilateral linear nevoid basal-cell carcinomas with comedones may represent postzygotic somatic mutation. A proclivity to other forms of neoplasia exists. patients with this syndrome have had medulloblastoma, meningioma, ovarian fibroma, ovarian fibrosarcoma, fibrosarcoma of the jaws, cardiac fibroma, fetal rhabdomyoma, and lymphatic or chylous cysts of the mesentery. In addition to the usual modalities of treatment, topical immunotherapy and topical 5-fluorouracil have been used with success. Oral synthetic retinoids, such as 13-cis-retinoic acid, have been used to prevent new lesions from appearing and to arrest the growth of older lesions by inducing differentiation. The independent observations of increased prostaglandin levels associated both with odontogenic keratocyst expansion and aggression of basal cell cancers merit further investigation both as a fundamental cellular mechanism and as a possible basis for treatment (e.g., with antiprostaglandins). The key to pre- and early postnatal diagnosis of the disorder--as well as to an understanding of the basic abnormality--lies in molecular genetics. Prime attention should be given to finding the site of the gene through the use of dna and other markers.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
10/20. The ophthalmological significance of the basal cell naevus syndrome.Ten cases of the basal cell naevus syndrome (BCNS) are reported. The study is based on clinical findings with no investigations beyond two skull radiographs. The diagnosis is made on some or all of the following findings: family history, jaw cysts, progressive development of basal cell carcinomas (BCCs), pitting of the palms and soles, frontal bossing, abnormality of the skeletal system and ectopic calcification. Eight of the cases are in one family covering three generations. With the exception of a one-year-old child in the third generation (not included) all of this group have the syndrome. The dominant trait is demonstrated, as is the high degree of expressivity and penetrance. Genetic counselling is essential where applicable. Sympathetic regular clinical examination over a lifetime is recommended to avoid disastrous complications.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
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