Cases reported "Skin Neoplasms"

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1/481. adenocarcinoma with signet ring cells of the axilla showing apocrine features: a case report.

    A 74-year-old Japanese man developed a reddish, indurated plaque composed of multiple nodules on his right axilla. Histopathologic examination showed a solid tumor that extended from the upper dermis into the subcutis, with both inter- and intracellular lumen formation, cellular arrangement in single files, a fibrotic reaction around the tumor cells, and the presence of mucinous material in the cytoplasm. There was both nuclear and cytoplasmic pleomorphism. Both lysozyme and GCDFP-15 were identified in the tumor cells. Electron microscopic examination showed periluminal condensation of the cytoplasm. Because thorough clinical and laboratory examinations were unremarkable, we regarded this to be a case of primary adenocarcinoma with signet ring cells of the axilla. The neoplasm might have differentiated toward the apocrine sweat glands or the mammary glands. radiation therapy was effective to some degree. This seems to be the first reported case in which adenocarcinoma with signet ring cells of the skin affected a site other than the eyelids.
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2/481. A case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified COL1A1 and PDGFB sequences.

    dermatofibrosarcoma protuberans (DFSP) is a cutaneous tumour of borderline malignancy, the cytogenetic features of which include the translocation t(17;22)(q22;q13) or, more commonly, supernumerary ring chromosomes containing material from 17q22 and 22q13. These rearrangements result in the COL1A1/PDGFB fusion gene. Here, we describe a case of DFSP displaying a ring chromosome 5 together with a large marker chromosome composed of chromosome 22 alphoid dna, material from distal 12q and amplified COL1A1 and PDGFB sequences. This is the first case of DFSP with multiple copies of COL1A1 and PDGFB not confined to ring chromosomes, showing that DFSP is similar to other borderline malignant mesenchymal tumours, where rings and giant markers are alternative vehicles for amplified material.
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3/481. Inflammatory metastatic melanoma.

    An 87-year-old woman developed erythema, induration and tenderness of the skin overlying each breast. One year before, she had undergone an axillary lymph node dissection because of metastases from melanoma. The primary site was unknown. A skin biopsy showed pigmented tumor nests within the dermal lymphatic vessels, and immunohistochemistry confirmed the melanocytic origin. The diagnosis of inflammatory metastatic melanoma was made.
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keywords = dura
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4/481. CO 2 laser surgery in hemophilia treatment.

    The use of CO 2 laser surgery between 1985 and 1991 in south africa and portugal for treatment of disorders in patients with mild to moderate cases of hemophilia A is discussed. Six cases of oral procedures and excision of skin tumors performed during this period are reported. In most of the cases of mild hemophilia no pre- or postoperative infusion of factor viii or desmopressin (DDAVP) was required. In some cases of moderate hemophilia, patients were infused with desmopressin (0.3 mug/kg body weight) and were treated postoperatively with the use of nasal desmopressin spray (150 mug to each nostril for four weeks following surgery). factor viii levels were measured before surgery. Follow up of four weeks was uneventful. The mean average power of the CO 2 laser was 20 W continuous and the pulse duration was 0.1 s for ablational procedures. For dermatologic procedures, a flexible plastic CO 2 laser hollow fiber was used (Flexilase, Sharplan, Allandale, NJ). We concluded that CO 2 laser surgery for hemophiliacs has a confirmed place in modern laser technology provided the standard precautions are taken and facilities are available.
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5/481. Linear epidermal nevus and nevus sebaceus syndromes: a clinicopathologic study of 3 patients.

    BACKGROUND: Linear epidermal nevus syndrome and linear sebaceus nevus syndrome are rare neurocutaneous syndromes characterized by epidermal nevi, epilepsy, and mental retardation. Pathologic descriptions of the central nervous system findings in such patients are rare. DESIGN: We examined the clinicopathologic features of 2 patients with linear epidermal nevus syndrome and 1 with nevus sebaceus syndrome who underwent surgical resections for chronic epilepsy in a tertiary referral center with a high volume of epilepsy surgery. RESULTS: patients included 3 females, aged 11 months (patient 1), 8 years (patient 2), and 2 1/2 years (patient 3) at the time of surgery. The duration of seizures prior to surgery was 11 months, 6 years, and 28 months, respectively. Two patients had epidermal nevi involving the head region (patients 1 and 3), and 1 had a nevus sebaceus of Jadassohn (patient 2); patient 2 had a choristoma, and patient 3 had a dermoid cyst in the eye region. Patient 1 demonstrated hemimegalencephaly radiographically. Histologic examination of resected cortical tissue in patients 1 and 2 demonstrated severe diffuse cortical dysplasia characterized by a disorganized cortical architectural pattern, a haphazard orientation of cortical neurons, and increased molecular layer neurons. Gyral fusion was seen in patient 1. Pial glioneuronal hamartomas were observed in patient 1. Prominent cortical astrocytosis was seen in patients 1 and 2, and foci of microcalcification were evident in patient 1. Cortical dysplasia was milder in patient 3 and consisted of an increased number of molecular layer neurons. Neuronal heterotopia was observed in all 3 patients. CONCLUSION: The spectrum of neuronal migration abnormalities in the setting of these syndromes may be variable in terms of its histologic phenotypic manifestations.
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6/481. Malignant melanoma showing ganglioneuroblastic differentiation: report of a unique case.

    We report a case of metastatic malignant melanoma in an inguinal lymph node, expressing ganglioneuroblastic differentiation. This was characterized by the presence of discrete nests and islands of large ganglion cells with abundant cytoplasm and eccentric nuclei with prominent nucleoli admixed with smaller primitive neuroblasts. The cells were separated by pale pink fibrillar material representing neuritic cell processes. These foci of ganglioneuroblastoma were seen over a background of an otherwise typical metastatic epithelioid, focally melanotic, malignant melanoma. immunohistochemistry showed positivity for neurofilament, synaptophysin, chromogranin, vasoactive intestinal peptide, and glial fibrillary acidic protein in the areas with ganglioneuroblastic differentiation, but not in the melanocytic component. Conversely, HMB45 positivity was expressed by the melanocytic cells only. S-100 protein and Melan-A, a putative melanocytic marker, showed positivity in both melanocytic and ganglioneuroblastic components. Ultrastructurally, neuritic cell processes and dense core neurosecretory granules were identified in the ganglionic and neuroblastic cells. A subsequent nodal metastasis in the same region showed focal neuroblastic differentiation without the ganglionic element. No evidence of neuronal or ganglionic differentiation was seen in the primary skin melanoma.
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keywords = mater
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7/481. Cellular neurothekeoma with possible neuroendocrine differentiation.

    We report a case of cellular neurothekeoma showing unusual immunohistochemical findings and occurring on the left upper arm of a healthy 48-year-old woman. She presented with a 1 cm, red, asymptomatic, dermal nodule of 1 year duration. A biopsy showed dermal proliferation of plexiform fascicles of spindle-shaped or polygonal cells with plentiful eosinophilic cytoplasms. The immunohistochemical profile included negative stains for S-100, CD34, factor xiiia, CD68, HMB45, cytokeratins, and EMA, strongly positive stains for neuron specific enolase (NSE), synaptophysin, and chromogranin a, and focally positive ones for NKI/C3 and alpha-smooth muscle actin. Ultrastructural analysis showed undifferentiated mesenchymal cells with cytoplasmic projections and abundant RER. Although we couldn't find any confirmative cell type in this cellular tumor, we believe that cellular neurothekeoma is predominantly composed of undifferentiated cells that can exhibit features of neuroendocrine cells in addition to fibroblastic or myofibroblastic ones, suggesting a divergent cell origin.
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keywords = dura
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8/481. Q-switched ruby laser treatment of a congenital melanocytic nevus.

    BACKGROUND: The treatment of medium-sized (1.5-20 cm diameter) congenital melanocytic nevi (CMN) has been the concern of dermatologists for decades. Although many techniques have been described and utilized, no single treatment has emerged as applicable under all circumstances. methods: The Q-switched ruby laser (QSRL) at 694 nm, a wavelength well absorbed by melanin relative to other optically absorbing structures in skin, causes highly selective destruction of pigment-laden cells. In addition, the 20-nanosecond pulse duration produced by this laser approximates the thermal relaxation time for melanosomes, thereby confining the energy to the targeted cells. RESULTS: In the present report, treatment using the QSRL resulted in complete clinical removal of a biopsy-documented medium-sized compound CMN with no recurrence after 5 years. In contrast to other therapeutic modalities, complications such has hypertrophic scarring, dyspigmentation, or atrophy were not observed.
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keywords = dura
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9/481. Reconstruction of large defects in the scalp with fasciocutaneous flaps.

    Although recent reports have emphasised free microsurgical transfer for reconstruction of extensive defects in the scalp, in our experience a carefully planned scalp flap is a simpler and safer method than a free transfer. Twenty-one patients with defects as large as 10%-60% of the scalp surface area were reconstructed; the calvarium was resected in five cases and the dura mater in two. In 18 cases the flaps were based on a single pedicle: the superficial temporal artery. In three cases the blood supply of the flaps was based on three major homolateral arteries: the superficial temporal, the posterior auricular, and the occipital. The blood supply of all scalp flaps was based on the interconnected network of the aponeurotic plexus and the pedicles were included into flap in 18 cases. The principles of fasciocutaneous flaps were applied for all 21 scalp flaps. The reconstruction of the skull was delayed in all cases, and the dura was replaced by free autogenous periosteum. The donor area was covered with a skin graft in all cases. In all patients the aesthetic and functional results were considered excellent by them and by us. There were no postoperative complications.
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keywords = dura mater, mater, dura
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10/481. Durable loss of a malignant T-cell clone in a stage IV cutaneous T-cell lymphoma patient treated with high-dose interferon and photopheresis.

    Stage IV cutaneous T-cell lymphoma (CTCL) has a notoriously poor prognosis and many treatment options exist. We describe the successful treatment of a case of stage IV CTCL with combination photopheresis and high-dose interferon alfa (IFNalpha). The patient was treated with combination therapy of monthly photopheresis and daily doses of IFNalpha up to 36 MU. Response to therapy was followed by clinical observation and Southern blot analysis for the detection of a malignant clone. The findings of this case were compared with others using a computer-based literature review. A complete clinical response lasting 64 months was achieved. Clinical relapse was preceded by an increase in the CD4/CD8 ratio and by reappearance of the T-cell receptor gene rearrangement. Combined photopheresis and high-dose IFNalpha led to a durable and sustained complete response in stage IV CTCL. CD4/CD8 ratios and T-cell gene rearrangements may be helpful in patient management.
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