Cases reported "skin neoplasms"

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1/9430. 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. ( info)

2/9430. Sweet's syndrome associated with chronic myelogenous leukemia: demonstration of leukemic cells within a skin lesion.

    We report a case of acute febrile neutrophilic dermatosis, Sweet's syndrome, associated with chronic myelogenous leukemia (CML) in which we found rearrangement of the bcr gene in dna obtained from a skin lesion as well as in blood dna by Southern blot analysis. This indicated the presence of CML cells within the skin lesion. To our knowledge, this is the first report in which the presence of CML cells is shown within skin lesions of Sweet's syndrome. In our patient, leukocyte alkaline phosphatase activities returned to normal levels when he was suffering from Sweet's syndrome and decreased again to below normal levels after it subsided. Whether the normalization of leukocyte alkaline phosphatase activity is common among CML patients with Sweet's syndrome remains to be determined. ( info)

3/9430. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura.

    We describe the clinical and laboratory findings of a young man with mycosis fungoides. The disease was associated, since the early stages, with autoimmune purpura. Interferon alfa (IFN-alpha) administration improved this patient's condition, both the purpuric eruption and patchy cutaneous lesions, thus suggesting T-cell abnormalities may be responsible for the development of the disease. ( info)

4/9430. Human herpes-virus 8 seropositive patient with skin and graft Kaposi's sarcoma after lung transplantation.

    Kaposi's sarcoma (KS) has been reported after solid organ transplantation mostly in recipients of renal, liver, heart, and bone allografts. We describe the first case of a patient with lung transplantation who developed KS of the skin, but also of the lung graft. The tumors were localized to places of previous trauma, implying the involvement of a Koebner phenomenon. Moreover, a polymerase chain reaction assay revealed the presence of dna sequences of herpesvirus 8 (HHV-8) on tissue of the cutaneous KS. Serological tests showed HHV-8 seronegativity of the graft donor and HHV-8 seropositivity of the patient before lung transplantation suggesting that the latter was already infected before the surgery and that immunosuppression resulted in the development of KS. This case report raises the question of the prevalence of HHV-8 in candidates for transplantation and organ donors, and of the value of an antiviral prophylaxis to lower the risk of KS. ( info)

5/9430. Bednar tumor (pigmented dermatofibrosarcoma protuberans) occurring in a site of prior immunization: immunochemical findings and therapy.

    Bednar tumor is a rare pigmented variant of dermatofibrosarcoma protuberans (DFSP). Because of its rarity, information is lacking regarding the optimal therapy and potential utility of immunohistochemistry in diagnosis. We report a case of Bednar tumor in which the diagnosis was aided by immunohistochemistry for CD34, an antigen known to be expressed in DFSP but not previously reported in Bednar tumor. Our case was also striking because it represents the first reported appearance of a Bednar tumor at a site of prior immunization, a phenomenon previously noted in some cases of DFSP. The patient was treated effectively with mohs surgery and is without recurrence at 9 months. ( info)

6/9430. Recurrent malignant proliferating trichilemmal tumour: local management with ethanol injection.

    We report a 59-year-old woman who exhibited a recurrent malignant proliferating trichilemmal tumour on the scalp for 1 5 years. The tumour was recalcitrant to conventional treatments such as chemotherapy, radiation or hyperthermia and we performed intratumoral ethanol injection as an alternative means of reducing tumour mass and obtaining haemostasis. biopsy specimens obtained after the ethanol injection revealed oedema, haemorrhage in the dermis and degeneration of the tumour cells, showing vacuolization with pyknotic nuclei. For cases of recurrent skin tumours and for patients in poor clinical condition, intratumoral ethanol injection is likely to be a therapeutic alternative to surgery or other conventional treatments. ( info)

7/9430. 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. ( info)

8/9430. Malignant fibrous histiocytoma and atypical fibroxanthoma in renal transplant recipients.

    BACKGROUND: Allograft recipients are at increased risk for skin cancer. The incidence of cutaneous squamous cell carcinoma is 50-250 times higher than in the age-matched control population, and basal cell carcinoma is about 10 times more frequent. The incidence of Kaposi's sarcoma is increased 400 to 500 times over that in a control population of the same ethnic origin. However, the incidence of other types of cutaneous sarcoma in organ allograft recipients is largely unknown. CLINICAL observation: Within a 2-year-period, we observed 2 patients with cutaneous malignant fibrous histiocytoma and 1 patient with atypical fibroxanthoma among a cohort of 642 renal transplant recipients. For comparison, the incidence for dermatofibrosarcoma protuberans which is the commonest type of cutaneous sarcoma, is 0.45/100,000 persons/year in the non-immunocompromised population. Our observation represents an incidence of 156/100,000/ year (95% confidence interval Cl 28-489/100,000/year) for cutaneous malignant fibrous histiocytoma and of 78/100,000/year (95% CI 4-368/ 100,000/year) for atypical fibroxanthoma. CONCLUSION: To our knowledge, this is the first report on an elevated incidence of cutaneous malignant fibrous histiocytoma and of atypical fibroxanthoma in renal transplant recipients. Future cohort studies on malignancies in organ allograft recipients should aim at defining this risk more exactly. ( info)

9/9430. Pleomorphic sclerotic fibroma.

    We report the clinical and histologic features of 2 cutaneous tumors demonstrating many of the usual features of sclerotic fibroma, an entity that is well described in the literature. Our cases differed from the usual form of sclerotic fibroma in that they demonstrated marked focal cellular pleomorphism without increased mitotic activity. We suggest the term pleomorphic sclerotic fibroma for these lesions. ( info)

10/9430. Bone involvement in a case of Kaposi sarcoma.

    BACKGROUND: Extracutaneous involvement is rare in the classical form of Kaposi sarcoma (KS). observation: We report a case of bone involvement revealed by bone pain. Magnetic resonance (MR) images demonstrated the local invasion of bone from cutaneous lesions. Bone biopsy confirmed bone involvement. The patient was treated with vindesine. Bone pain progressively disappeared. CONCLUSION: Bone involvement has rarely been reported in classical KS though 4.5% of the patients were affected when it was systematically sought. Treatment of symptomatic lesions requires radiotherapy or chemotherapy. ( info)
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