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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report a diagnostic dilemma involving a child who, 8 years previously, had total excision of a medulloblastoma. On x-ray studies, lytic lesions of the skull were seen. The differential diagnosis and some of the clinical and pathological aspects of the nevoid basal-cell carcinoma syndrome versus metastases are discussed.
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PMID:Multiple nevoid basal-cell carcinoma syndrome (Gorlin's syndrome): possible confusion with metastatic medulloblastoma. Case report. 75 69

Nevoid basal cell carcinoma syndrome is a relatively uncommon clinical entity. It is essentially characterized by the early onset of multiple basal cell carcinomas of the skin generally above wrist-line level, by the presence of jaw cysts, musculoskeletal and other anomalies. Metastatic disease secondary to ordinary basal cell carcinoma is indeed rare. What is even more rare is pulmonary metastases from basal cell carcinoma. Very few cases have been reported in which pulmonary resection was performed for metastatic basal cell carcinoma. We were unable to find a reported case in which pulmonary resection was performed for a pulmonary nodule metastatic from the nevoid basal cell carcinoma syndrome. Herein is reported what we believe to be the first case of pulmonary resection performed for a metastatic nodule in this syndrome.
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PMID:Pulmonary metastasis in nevoid basal cell carcinoma syndrome. 204 56

The nevoid basal cell carcinoma syndrome is an autosomal dominant condition that consists of multiple basal cell carcinomas of the skin, odontogenic keratocysts, pits of the hands and feet (especially the palms and soles), various ectopic calcifications, notably lamellar calcification of the falx cerebri and skeletal anomalies. As well as these major findings, there are many other associated abnormalities. This paper is a 15-year follow-up study on twelve cases from the Ottawa Valley with details on nine new cases discovered during that time. Sixteen were males; 5, females. The number of basal cell cancers requiring removal varied greatly from patient to patient. There was one patient with a horror lesion; none had metastatic disease.
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PMID:Nevoid basal cell carcinoma syndrome. A 15-year follow-up of cases in Ottawa and the Ottawa Valley. 358 81

A patient with basal cell naevus syndrome (Gorlin-Goltz Syndrome) is presented. Of note in the case was the extensive symmetrical tumour invasion of both external auditory canals requiring bilateral radical resection. The patient expired 14 months later, at which time the autopsy revealed widespread metastases to the pleura, diaphragm, pericardium, epicardium and myocardium. Although lung metastases have been reported in this syndrome, no cases have been reported of metastases to these sites.
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PMID:Multiple metastases from basal cell naevus syndrome. 367 86

Multifocal Merkel's cell tumors developed in a 24-year-old patient with an unusual cutaneous dysplasia syndrome. The neoplasms behaved in an aggressive fashion, with the development of multiple lymph node metastases. In this case, the dermatologic syndrome had features of both dyshidrotic ectodermal dysplasia and the basal cell nevus syndrome. In addition to Merkel's cell tumors, basal cell carcinomas and actinic elastosis were seen in sun-exposed skin sites. These findings, in conjunction with a known tendency for Merkel's cell tumors to arise in solar-damaged areas, suggest a pathogenetic relationship between these neoplasms and the underlying cutaneous syndrome in this patient.
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PMID:Multifocal Merkel's cell tumors associated with a cutaneous dysplasia syndrome. 684 21

A 57-year-old man was admitted with complaints of progressive anorexia, weight loss and right flank pain. He had been treated for basal-cell carcinoma of the skin 19 years before. On physical examination, eight moles in the face, back and left thigh were found along with palmar pits. In addition, a painful induration in his right thigh was evident. Biopsy proved that six moles were basal-cell carcinomas and the thigh mass a high-grade leiomyosarcoma. Computed tomographs revealed multiple metastases in the lungs and the liver. The patient was treated with epirubicin, with partial response, and subsequently with ifosfamide. He died 17 months after diagnosis. Whereas the world literature records several cases of soft tissue tumors in patients with nevoid basal-cell carcinoma syndrome, this is the first report of a simultaneous occurrence of leiomyosarcoma and nevoid basal-cell carcinoma syndrome.
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PMID:Leiomyosarcoma of the soft tissues in a patient with nevoid basal-cell carcinoma syndrome. 783 73

Many genes originally identified because of their role in embryonic development are also important in postnatal control of cell growth and differentiation. Mutations in some of these genes have been shown to cause cancer. Basal cell carcinoma (BCC) of the skin is the most common cancer in humans. More than 750000 new cases are diagnosed annually, and the incidence is rising. BCCs are slow-growing, locally invasive tumors that rarely metastasize but can result in extensive morbidity through local recurrence and tissue destruction. Epidemiologic studies suggest that sunlight (particularly UVB radiation) is a strong risk factor for BCC formation, although other factors are also involved. The nevoid basal cell carcinoma syndrome (NBCCS), a rare genetic disorder, is characterized by predisposition to BCCs and other tumors as well as to a wide range of developmental defects. NBCCS maps to chromosome 9q22.3, and loss of heterozygosity at this site in both sporadic and hereditary BCCs suggests that it functions as a tumor suppressor. The gene for NBCCS was recently cloned and is the human homologue of the Drosophila gene "patched." Genetic studies in Drosophila show that patched is part of the hedgehog signaling pathway, which is important in determining embryonic patterning and cell fate in multiple structures of the developing embryo. Human patched is mutated in both hereditary and sporadic BCCs, and inactivation of this gene is probably a necessary, if not sufficient, step for BCC formation. Delineation of the biochemical pathway in which patched functions may lead to rational medical therapy for BCCs and possibly for other tumors associated with NBCCS.
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PMID:Developmental genes and cancer: role of patched in basal cell carcinoma of the skin. 926 47

Basal cell carcinoma is the most frequent of all cancers. Its incidence has risen those last decades because of the increase in sun exposure habits. People with light skin complexion are particularly at risk. Ionizing radiations, arsenicism, various genodermatoses (Gorlin syndrome, xeroderma pigmentosum, nevus sebaceous) are other pre-disposing factors. Basal cell carcinoma usually present as small lesion with a pearly border and telangiectasias. Other clinical types must also be recognized such as the nodular, the infiltrative, the superficial and the pigmented forms. They are usually located on the head and trunk. The prognosis of basal cell carcinoma is usually good since they can be cured by surgical excision and because they usually do not metastasize. However, large, multiple or recurrent basal cell carcinomas can be difficult to treat. In these cases, cryosurgery, radiotherapy or intralesional interferon-alfa may be needed.
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PMID:[Basal cell carcinoma]. 1033 93

A 48 year-old white male with basal cell nevus syndrome presented with a metastatic basal cell carcinoma with neuroendocrine features. The tumor manifested aggressive behavior, having deep local invasion and metastases to para-aortic lymph nodes and bone. Neuroendocrine differentiation has rarely been associated with basal cell carcinoma. The histologic, immunohistochemical, and electron microscopic studies of this rare tumor are described.
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PMID:Neuroendocrine differentiation of a metastatic basal cell carcinoma in a patient with basal cell nevus syndrome. 1047 60

The hedgehog signalling pathway plays a vital role in Drosophila embryonic patterning and development. Hedgehog is a secreted protein, unrelated to classical growth factors, which seems to form concentration gradients across those tissues involved in pattern formation. Cloning of vertebrate homologues of hedgehog and other genes has illustrated the remarkable conservation of function of this pathway throughout evolution. The human homologue of patched, a receptor for the hedgehog protein, was cloned as the gene responsible for naevoid basal cell carcinoma syndrome (NBCCS/'Gorlin Syndrome'), an autosomal dominant condition in which patients suffer from multiple basal cell carcinomas and a wide spectrum of developmental abnormalities. Its role as a tumour suppressor gene in both NBCCS and sporadic basal cell carcinoma led to the suggestion that mutation or inactivation of human patched may be an essential step in development of basal cell carcinomas and other skin tumours. This review describes our current understanding of hedgehog signalling in Drosophila and vertebrates and its relation to the development of human basal cell carcinoma and other skin tumours, together with a discussion of future avenues of research into this critical and intriguing pathway.
Cancer Metastasis Rev 1999
PMID:The hedgehog signalling pathway and its role in basal cell carcinoma. 1072 88


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