Cases reported "Skin Neoplasms"

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1/110. Molecular analysis of glioma and skin-tumour alterations in a xeroderma-pigmentosum child.

    xeroderma pigmentosum (XP) is a rare hereditary disease characterized by a very high frequency of skin tumours due to a defect in the nucleotide-excision-repair process. Some of these patients have also been reported to develop internal tumours with higher frequency than the normal population. Reported here are the clinical features and molecular analysis of an XP patient who developed multiple skin cancers as well as a thalamic glioma. Complementation analysis with recombinant retrovirus, cloning efficiency and unscheduled DNA synthesis after UV-C indicate that the patient belongs to the C group. Characterization of the p53 mutations in the 2 tumours of the patient leads to speculation on the aetiological agents involved in tumour initiation. The skin tumour is clearly induced by the presence of unrepaired UVB-induced dna damage on the non-transcribed strand of the p53 gene, while the glioma may be induced by unrepaired DNA lesions produced by free radicals.
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2/110. Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.

    PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. methods: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.
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3/110. xeroderma pigmentosum variant associated with multiple cancers.

    A 62-year-old Japanese man with xeroderma pigmentosum (XP) variant is reported. The patient had developed at least 6 basal cell carcinomas, a squamous cell carcinoma, and a malignant melanoma on sun-exposed areas, and an atypical carcinoid on the right lung. In vivo phototesting showed a normal response. The minimal erythema dose of ultraviolet B (UVB) was not lowered and no delayed peaking of the erythema reaction was observed. His skin fibroblasts exhibited higher sensitivity to UV irradiation, but a normal level of unscheduled DNA and rna synthesis. Cell fusions with XP group A, C, D, E, F, and G cells after UV irradiation were all complemented. Previous reports together with this case suggest that older XP variant patients have a high frequency of not only skin cancers, but also internal malignancies.
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4/110. Cancer protection in xeroderma pigmentosum variant (XP-V).

    We describe herein a brother and sister diagnosed with xeroderma pigmentosum variant (XP-V) in early adult life, who presented with increased sensitivity to sunlight and with cutaneous carcinomas on sun-damaged skin. The 27-year-old male farmer (Case 1.) was diagnosed with advanced squamous cell carcinoma (SCC) and multiple actinic lesions. Surgical removal of these lesions was performed. Three months later he died of multiple pelvic metastases of SCC. His 29-year-old sister (Case 2.) was operated on for different tumors, histologically SCC-s or basal cell carcinomas (BCC), or praecancerous conditions many times. After a two year interval she was treated with low dose isotretinoin (2 mg/body weight). diagnosis of XP-V was based on unscheduled DNA analysis (USD) and on clinical symptoms. We observed that during the long lasting isotretinoin treatment the tumor frequency dropped to a quarter. Therefore, the isotretinoin treatment seems to be a good approach for cancer prevention in conditions with high predisposition to skin cancer, such as in XP-V.
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5/110. Multiple glomus tumor: a case report and review of the literature.

    Multiple glomus tumors are extremely rare and differ from the more common solitary glomus tumors in their clinical presentation and histological features. The authors report a case of multiple glomus tumors of the right hand in a 65-year-old man, its treatment, and a review of the features of this uncommon tumor. The tumor usually presents as a painful, firm, purplish, solitary nodule of the extremities, especially in the nail bed. Multiple glomus tumors are described as softer, more compressible, bluish nodules and they occur with less frequency than solitary tumors. They are often inherited in an autosomal dominant pattern. The authors present a case of multiple glomus tumors of the right hand, in which many small, painful, red papules were grouped in the right hypothenar region. The patient was treated by wide excision of the lesion and coverage of the defect with an ulnar artery forearm flap.
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6/110. Metastatic cutaneous plasmacytoma: a case report associated with IgA lambda multiple myeloma and a review of the literature of metastatic cutaneous plasmacytomas associated with multiple myeloma and primary cutaneous plasmacytomas.

    We present the case of a 67-year-old Japanese woman with immunoglobulin a lambda (IgA lambda) multiple myeloma (MM). She had firm nodular cutaneous lesions on the trunk and scalp without adjacent bone involvement. The patient was diagnosed as having IgA lambda MM of stage IIIA with 52% plasmacytosis in the bone marrow six months before the appearance of the cutaneous lesions. The abnormal plasma cells showed moderate to marked dysplasia in both the bone marrow and skin lesions. The abnormal plasma cells in the bone marrow exhibited abnormal karyotypes: 41, XX, der (1) t (1p; 1q), -4, -10, -14, -16, -17, 17p , that differed from the "unfavorable" karyotype reported previously. We reviewed the cases of metastatic cutaneous plasmacytoma in MM and cases of primary cutaneous plasmacytoma that have been reported in English or Japanese and identified the Ig class. Among the 83 cases of metastatic cutaneous plasmacytomas in MM, IgG, IgA, IgD, and Bence-Jones protein were found in 52%, 23%, 16%, and 6%, respectively. A disproportionately high frequency of IgD lambda MM was found to have spread to the skin, compared with the frequency of IgD MM itself, which was present in only around 2% of the MM cases. Among the 18 primary cutaneous plasmacytomas, IgG, IgA, and Bence-Jones protein were found in 56%, 11%, and 17%, respectively, but no IgD was found.
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7/110. Cutaneous metastatic lung cancer: literature review and report of a tumor on the nose from a large cell undifferentiated carcinoma.

    Cutaneous metastatic disease is a prognostically important diagnosis. We report the case of a 64-year-old man who had an uncommon histologic type of lung cancer--a large cell undifferentiated carcinoma, which was metastatic to the skin of the nose. The relative frequency of cutaneous metastasis is similar to that of primary cancers. Cutaneous disease as the first sign of metastasis is most often seen in cancer of the lung. However, its appearance as a large tumor on the nose, which was observed in this case, is unusual.
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8/110. A case of classic Kaposi's sarcoma in a Japanese man: detection of human herpes virus 8 (HHV-8) infection by means of polymerase chain reaction and immunofluorescence assay.

    The recently discovered human herpes virus 8 (HHV-8) has been implicated in the pathogenesis of Kaposi's sarcoma (KS). Because classic KS in japan is rare and the detection of HHV-8 DNA by polymerase chain reaction (PCR) has been successful only in limited cases, the frequency and role of HHV-8 infection in KS in japan remain unclear. Herein we report a case of classic KS in a Japanese man whose HHV-8 infection was confirmed by the detection of lesional viral DNA and serum antibodies against lytic antigen.
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9/110. Pigmented squamous cell carcinoma of the skin: morphologic and immunohistochemical study of five cases.

    Invasive pigmented squamous cell carcinoma (PSCC) of the skin is reportedly rare. Herein, we evaluate an additional five cases and compare their relative frequency with non-pigmented squamous cell carcinoma (SCC). Of 46,791 archived cases of SCC, a total of five cases of PSCC were discovered for a relative frequency of approximately 0.01%. Grossly, each tumor presented as a rapidly growing crusted papule on actinic damaged skin of the face. Microscopically, all were composed of a mixture of keratininized squamous cells and melanin-producing dendritic melanocytes. The squamous cells stained for epithelial membrane antigen, and both low and high molecular keratins. The melanocytes stained for S-100 and HMB-45. A matched series of 31 SCCs were subjected to an identical immunohistochemical battery of stains to determine if a histologically subtle and unsuspected number of intratumoral melanocytes existed in SCC. Each of the cases failed to show intratumoral melanocytes. The differential diagnosis and possible histogenesis of PSCC is discussed and the importance of extensive pathologic examination to prevent misdiagnosis is emphasized. Despite the histologic dissimilarity, the long-term prognosis of the reported cases was similar to conventional SCC.
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10/110. Multiple metastases of carcinoma basocellulare into spinal column.

    Basal cell carcinoma presents a relatively low potential and local malignancy and very slow growth giving only occasionally metastatic spreading. The frequency of occurrence of metastatic dissemination is estimated in the literature depending on examined population from 0.028% to 0.55%. Metastases are most often found in lymph nodes, lungs bones and internal organs: liver, spleen, kidneys, adrenal glands, pleura and the peritoneum. Authors present a case of a 69-years old female with an extensive basal cell carcinoma of the head convexity, infiltrating the subcutaneous tissue, periostium, bone and dura mater, giving distant metastases to other bone and soft tissue structures of a thoracic spine, which was confirmed by biopsy and histopathological findings of neoplasm tissue in spine. The primary lesion was successfully treated surgically. Despite administered radiotherapy of metastases in spine, progress of the disease during 1-year period was observed. The patient was alive with metastatic tumours present at last follow-up. Basing on the review of the literature and our case report we can distinguish following factors which may increase the risk of occurrence of basal cell carcinoma metastases: the great extent of the primary lesion, deep penetration to stromal tissue, blood and lymph vessel invasion, long history of tumour occurrence and the presence of metatypia in histopathological findings. The above-mentioned case fulfils the criteria of carcinoma basocellulare metastases proposed by Latters and Kessel and may be included to the general registration list of this cancer in the world.
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