(1/214) Merkel cell carcinoma and melanoma: etiological similarities and differences.

Merkel cell carcinoma (MCC) of the skin and cutaneous malignant melanoma can now be compared epidemiologically through the use of population-based data not previously available for MCC. The results may provide new clues to etiology. In this study, United States data covered by the Surveillance, Epidemiology, and End Results (SEER) Program were from nine areas of the United States (approximately 10% of the population). In 1986-1994, 425 cases of MCC were registered. The annual age-adjusted incidence per 100,000 of MCC was 0.23 for whites and 0.01 for blacks; among whites, the ratio of melanoma to MCC was approximately 65 to 1. Only 5% of MCC occurred before age 50, unlike the lifelong risk of nodular and superficial spreading melanoma. Regional incidence rates of both cancers increased similarly with increasing sun exposure as measured by the UVB solar index. The most sun-exposed anatomical site, the face, was the location of 36% of MCC but only 14% of melanoma. Both cancers increased in frequency and aggressiveness after immunosuppression and organ transplantation (36 cases from the Cincinnati Transplant Tumor registry and 12 from published case reports) and after B-cell neoplasia (5 cases in this study; 13 from case series in the literature). The SEER data contained reports of six patients with both types of cancer; 5 melanomas before the diagnosis of MCC and 1 after diagnosis. MCC and melanoma are similarly related to sun exposure and immunosuppression, but they differ markedly from one another in their distributions by age, race, and anatomical site, especially the face.  (+info)

(2/214) Overgrowth of oral mucosa and facial skin, a novel feature of aspartylglucosaminuria.

Aspartylglucosaminuria (AGU) is a lysosomal storage disorder caused by deficiency of aspartylglucosaminidase (AGA). The main symptom is progressive mental retardation. A spectrum of different mutations has been reported in this disease, one missense mutation (Cys163Ser) being responsible for the majority of Finnish cases. We were able to examine 66 Finnish AGU patients for changes in the oral mucosa and 44 of these for changes in facial skin. Biopsy specimens of 16 oral lesions, 12 of them associated with the teeth, plus two facial lesions were studied histologically. Immunohistochemical staining for AGA was performed on 15 oral specimens. Skin was seborrhoeic in adolescent and adult patients, with erythema of the facial skin already common in childhood. Of 44 patients, nine (20%) had facial angiofibromas, tumours primarily occurring in association with tuberous sclerosis. Oedemic buccal mucosa (leucoedema) and gingival overgrowths were more frequent in AGU patients than in controls (p<0.001). Of 16 oral mucosal lesions studied histologically, 15 represented fibroepithelial or epithelial hyperplasias and were reactive in nature. Cytoplasmic vacuolisation was evident in four. Immunohistochemically, expression of AGA in AGU patients' mucosal lesions did not differ from that seen in corresponding lesions of normal subjects. Thus, the high frequency of mucosal overgrowth in AGU patients does not appear to be directly associated with lysosomal storage or with alterations in the level of AGA expression.  (+info)

(3/214) Microsurgical reconstruction of basal cell carcinoma defect of the face: a multidisciplinary approach.

This article describes a 73-year-old white man with a history of dizziness secondary to profound anemia who presented with a large basal cell carcinoma of the left front temple region. A multidisciplinary approach to the extirpation and reconstruction of this defect is presented with a review of histopathologic features and outcomes of basal cell carcinoma excision.  (+info)

(4/214) Malignant schwannoma of the trigeminal nerve.

SUMMARY: We present the MR imaging, CT, and clinical findings of a patient with malignant schwannoma of the trigeminal nerve. Local tumor recurrence is frequent and may be mistaken for lymphatic spread. In this report, we emphasize the natural history of this rare tumor and discuss the importance of imaging in diagnosis and surveillance.  (+info)

(5/214) Inflammatory myofibroblastic tumor involving the pterygopalatine fossa.

SUMMARY: Inflammatory myofibroblastic tumors (IMT) comprise a rare group of lesions characterized histologically by acute and chronic inflammatory cells with a variable degree of fibrous stroma. Occurrence in the extracranial head and neck in children is unusual, and involvement in the pterygopalatine fossa has not, to our knowledge, been reported as occurring in this age group. We present the CT findings of an IMT of the pterygopalatine fossa in a 6-year-old female patient with a 2-week history of fever and a painless swelling of the left cheek. The diagnosis of IMT should be included in the differential diagnosis of a child presenting with an aggressive mass associated with systemic features such as fever, elevated sedimentation rate, and leukocytosis.  (+info)

(6/214) MR imaging in two cases of subacute denervation change in the muscles of facial expression.

SUMMARY: Denervation changes in muscle following damage to cranial and peripheral nerves can be observed on both CT and MR imaging studies. These findings are well described for cranial nerves (CN) V, X, XI, and XII. The CT findings of denervation atrophy due to CN VII dysfunction have been reported. We describe the MR imaging findings in two patients with perineural spread of tumor along CN VII. Both patients showed T2 prolongation and postcontrast enhancement in muscles of facial expression, suggestive of subacute denervation changes.  (+info)

(7/214) Inverted follicular keratosis.

Attention is drawn to a benign skin tumour which has escaped recognition in the British lieterautre. Inverted folliculr keratosis can be mistaken clinically for basal cell carcinoma and a variety of benign skin lesions. Pathologically it is easliy confused with squamous carcinoma, a serious error because this lesion occurs dominantly on the face. The lesion is thought to arise from the infundibulum of hair follicte.  (+info)

(8/214) Cutaneous histiocytic lesions: a clinical dilemma.

The diagnosis of malignant histiocytosis requires a high index of clinical suspicion, awareness of its atypical features and availability of various tissue samples for morphological and special studies. The case reported here highlights the diagnostic difficulties encountered in a patient diagnosed as malignant histiocytosis who presented with cutaneous lesions in multiple foci, which included the face, groin and forearm. Only after repeated biopsies and special stains, a diagnosis of malignant histiocytosis was arrived at. Chemotherapy with CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone) was initiated. The response to chemotherapy was good and the patient is doing well eleven months after initial diagnosis.  (+info)