Cases reported "Skin Neoplasms"

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1/1027. Nasal and nasal-type natural killer/T-cell lymphoma.

    Nasal and nasal-type natural killer (NK)/T-cell lymphomas follow an aggressive course and have a poor prognosis. Recent pathologic studies suggest that the disease is a malignant proliferation of NK cells, which often express CD56. An association with the Epstein-Barr virus has also been reported. Skin involvement occurred in each of the 3 patients studied. radiation therapy provided some benefit to the patients in the early stages. Conventional chemotherapies were not effective. To overcome this multiple-drug resistance of the tumor cells, cyclosporine and high-dose chemotherapy was combined with peripheral-blood stem-cell transplantation. The average life span from the onset of the disease for our patients was 9.6 months. Further improvement in the management of nasal and nasal-type NK/T-cell lymphomas is necessary.
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2/1027. Juvenile hyaline fibromatosis: clinical heterogeneity in three patients.

    BACKGROUND: Systemic hyalinoses are genetic generalized fibromatoses characterized by an accumulation of hyalin in the dermis. Two distinctive syndromes are recognized in the literature: infantile systemic hyalinosis (ISH) and juvenile hyaline fibromatosis (JHF). ISH and JHF are sometimes difficult to separate since they show significant overlap. OBSERVATIONS: We report on 3 children from two unrelated families suffering from JHF. The first child is severely handicapped by joint contracture, massive hyperplasia of the gingivae, diffuse skin papules and subcutaneous nodules occupying the scalp, face, perianal area, palms, soles and chest. At the same age, the second child only shows pearly skin papules on the face, groin and perianal area and gingival hyperplasia without joint stiffness or any other subjective complaint. The third patient, a brother of the second child, developed mild skin abnormalities by the end of the first year. The occurrence in siblings and consanguinity in the second family suggests autosomal recessive inheritance. Histological skin examination in the 3 cases showed hyaline deposition in the dermis and abnormal ultrastructure of fibroblasts. Biochemical findings showed mucopolysaccharide abnormalities in both families. CONCLUSION: Our patients do not only illustrate the different expressions of JHF but also show some overlap with ISH, suggesting a common cause for both disorders. Genetic studies will finally answer this question.
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keywords = nose
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3/1027. Systemic sclerosis revealing T-cell lymphoma.

    We describe a case of systemic sclerosis (SSc) occurring together with malignant lymphoma. A 43-year-old man, who had noticed sclerodactyly 1 month before consultation, was admitted for progressive skin sclerosis on his forearms and chest. SSc was diagnosed. Immediately after admission, skin sclerosis rapidly extended to the neck and trunk, and subcutaneous tumors developed on the neck, chest and back. Skin sclerosis was prominent at the sites where subcutaneous tumors were present. The tumors were diagnosed as non-Hodgkin's lymphoma of T-cell phenotype derived from soft tissue. Following 4 cycles of chemotherapy, he had complete remission and the skin sclerosis remarkably improved. It is possible that cytokines produced by T-cell lymphoma cells were responsible for the development of skin sclerosis in this case.
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keywords = nose
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4/1027. Basal cell carcinoma with massive ossification.

    We report a case of basal cell carcinoma with massive ossification in a 66-year-old white man. Ossification in various benign and malignant neoplasms have been reported including basal cell carcinomas, in which ossifications are seen in small foci or peripheral rim of the tumor. However, in our case, massive ossification is seen throughout the tumor, and only small areas of the periphery of the tumor show diagnostic histology. Therefore, this case might have presented a diagnostic difficulty or been misdiagnosed as an osteoma cutis if a smaller incisional or punch biopsy had been performed. The phenomenon of bone formation itself is not specific for any diagnostic entity, and therefore an underlying lesion should be carefully sought in case of secondary ossification.
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keywords = nose
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5/1027. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease.

    We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease.
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keywords = nose
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6/1027. Subcutaneous panniculitic T-cell lymphoma developing in a child with idiopathic myelofibrosis.

    PURPOSE: Subcutaneous panniculitic T-cell lymphoma is reported in a child with idiopathic myelofibrosis. Both disease entities are rarely seen in children. PATIENT AND methods: A girl aged 5 years and 9 months had pancytopenia and severe constitutional symptoms. Idiopathic myelofibrosis was subsequently diagnosed. RESULTS: A transient response was achieved after treatment with a course of high-dose methylprednisolone therapy. However, proptosis and skin nodules developed during tapering of steroid therapy. A computed tomography scan of the orbit also revealed a mass lesion in the right lacrimal gland region. A skin biopsy specimen showed a subcutaneous panniculitic T-cell lymphoma. The clinical course was marked by high fever, profound pancytopenia, massive gastrointestinal bleeding, and severe, recurrent infections. Her condition rapidly deteriorated, and she died from polymicrobial sepsis 4 months after her initial examination. CONCLUSIONS: Subcutaneous panniculitic T-cell lymphoma is a distinctive clinicopathologic entity that is rarely seen in children. The association of myelofibrosis and peripheral T-cell lymphoma as seen in this has been rarely reported.
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keywords = nose
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7/1027. Epidermal naevus syndrome and hypophosphataemic rickets: description of a patient with central nervous system anomalies and review of the literature.

    The epidermal naevus syndrome (ENS) is a rare dermatological condition consisting of congenital epidermal nevi associated with anomalies in the central nervous system, bones, eyes, hear or genito-urinary system. We report a new case of ENS associated with hypophosphataemic rickets. The girl was born with a mixed-type epidermal naevus and skeletal anomalies. Hypophosphataemic rickets was diagnosed at the age of 2.5 years. At 14 years of age. MRI of the head demonstrated right brain hypotrophy, a left temporal arachnoid cyst and asymmetric lateral ventricles. We reviewed the literature and found 13 reported cases of ENS associated with hypophosphataemic rickets. Conclusion We report a further patient with epidermal naevus syndrome and hypophosphataemic rickets, followed from birth to the age of 15 years, who had structural central nervous system anomalies with normal intellectual functioning. A comprehensive neurological work up is recommended in patients with epidermal naevus syndrome.
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ranking = 0.21831216863816
keywords = nose
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8/1027. Diagnostic relevance of chromosomal in-situ hybridization in Merkel cell carcinoma: targeted interphase cytogenetic tumour analyses.

    AIMS: To resolve the conflicting diagnoses of five pathologists (which included well-differentiated neuroendocrine carcinoma, malignant carcinoid, undifferentiated small-cell carcinoma, primitive neuroectodermal tumour, metastases of small-cell lung carcinoma (SCLC) and Merkel cell carcinoma (MCC)), and tumour-free lungs after necropsy, we investigated an alarmingly metastasizing MCC in a 32-year-old Caucasian man using chromosomal in-situ hybridization (CISH). Differences in incidence and course in males and females also prompted targeted analyses for chromosomes X and Y. The lesion was also analysed for p53 gene mutations. methods AND RESULTS: paraffin sections of the thorax, buccal lymph nodes and scalp tumours were stained with haematoxylin and eosin. immunohistochemistry was performed with antibodies against pancytokeratin, keratin 20, neuron-specific enolase (NSE), chromogranin, neurofilaments and vimentin, among others. Sections (5-6 microm) of the tumours were analysed with alpha-satellite probes for chromosomes 1, 6, 7, 11, 12, 17, 18, X and Y using CrSH; and exons 5-9 of the p53 gene were examined by polymerase chain reaction and single strand conformation polymorphism (PCR-SSCP) methods. Although positive for pancytokeratin, keratin 20, chromogranin, NSE, synaptophysin and vimentin, the similarity in antigen profiles expressed by SCLC and MCC prevented a definitive tumour diagnosis. Chromosomal in-situ hybridization, however, revealed trisomies 1 and 11, two frequent aberrations in MCC, and trisomy 18. Moreover, 71% of the tumour cells had two to three copies of X, whereas 98% of the cell nuclei in the hair follicles and normal epidermis (purported Merkel cell origins) displayed one x chromosome. No mutations were detected in the five exons of the p53 gene examined. CONCLUSIONS: Had CISH been performed earlier, treatment may have been tailored specifically to suit MCC, since MCC and SCLC have different therapeutic strategies. Finally, chromosome X may be of prognostic relevance in MCC, which apparently predominates in females and yet shows poorer prognosis in males, and hence be worthy of further investigation.
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ranking = 0.21831216863816
keywords = nose
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9/1027. Clinicopathological report: mucinous carcinoma of the eyelid.

    BACKGROUND: Mucinous carcinoma of the skin is a rare tumour that may involve the peri-ocular region. methods/RESULTS: A case report is presented of a 73-year-old woman with a right upper lid tumour, initially diagnosed as a basal cell carcinoma. Excisional biopsy of the residual tumour revealed mucinous carcinoma. Re-examination of the original pathology proved to be mucinous carcinoma, originating in the eyelid skin. Further treatment involved wide local resection and reconstruction. Systemic investigations were undertaken to exclude the possibility of metastatic mucinous carcinoma. CONCLUSION: This case is presented to alert ophthalmologists to the occurrence of this tumour in the periocular region, to highlight the importance of surgical excision with wide margins and the need for systemic investigation to exclude a primary malignancy in other sites, in particular the gastrointestinal system and breast.
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ranking = 0.21831216863816
keywords = nose
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10/1027. Treatment of multiple glomangioma with tuneable dye laser.

    BACKGROUND: Multiple glomangiomas are a rare clinical occurrence. In the past, various treatments have been proposed for eradication of these tumours. OBJECTIVE: An alternative and effective therapy for multiple glomangioma is proposed. Results: The effective clinical response of multiple, painful, bluish-black lesions on the soles of the feet and the hands, diagnosed as multiple glomangioma, to flashlamp tuneable dye laser is reported. CONCLUSION: The flashlamp tuneable dye laser is an effective modality in the treatment of multiple glomangioma.
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ranking = 0.21831216863816
keywords = nose
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