Cases reported "Skin Neoplasms"

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1/918. 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 = chest
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2/918. 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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ranking = 5.6340580079389
keywords = back, chest
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3/918. melanosis in association with metastatic malignant melanoma: report of a case and a unifying concept of pathogenesis.

    An unusual case of melanosis associated with metastatic malignant melanoma is reported. This was characterized by progressive blue/gray discoloration of the skin of the chest and abdomen in an elderly patient, 1 year after removal of a polypoid malignant melanoma from the right arm. A biopsy of involved skin revealed perivascular aggregates of melanin-laden histiocytes throughout the dermis, the histopathologic hallmark of melanosis. An unusual aspect of the case was the coincidental finding of a tumor embolus within a small dermal vessel, probably a lymphatic. To date, neoplastic melanocytes have been detected in only a small minority of skin biopsies with features of melanosis. This case and a distillation of related information in the literature lead to the conclusion that the essence of melanosis, and the feature that distinguishes this from conventional metastatic melanoma, is the persistent and cumulative dissemination of melanin, via the bloodstream, throughout the body. This in turn leads to progressive pigmentation of all internal organs and the skin. Only continuous access to the circulation by neoplastic melanocytes could explain such a phenomenon. Potential mechanisms by which this could arise are discussed in the context of existing knowledge.
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keywords = chest
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4/918. 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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ranking = 1.5456158375329
keywords = upper
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5/918. 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 = 1.5456158375329
keywords = upper
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6/918. Papillary formations in metastatic melanoma.

    Cytomorphologic features of melanoma can be extremely variable, in that they can mimic other poorly differentiated neoplasms. Ten cases of metastatic melanoma with distinct, cohesive, papillary tissue fragments observed in fine-needle aspiration (FNA) specimens are reported. These papillary fragments exhibited a central fibrovascular core with attached tumor cells, in a background of single scattered malignant cells, macrophages, and focal necrosis. The aspiration sites included regional or distant palpable lymph nodes, pancreas, bone, and skin. Nine cases had a histologic diagnosis of primary cutaneous melanoma, and in one case the primary skin tumor was detected after the diagnosis was established by FNA of the metastasis. Immunohistochemical studies (S-100 protein, HMB-45 antigen, and factor viii) were performed in four cases, and electron microscopy in one, confirming the diagnosis of melanoma. An awareness of this cytomorphologic variation of papillary formations in cytology preparations from metastatic melanoma is important and can prevent potential inaccurate interpretation.
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keywords = back
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7/918. A case of a malignant melanoma with late metastases 16 years after the initial surgery.

    We report a case of a pulmonary metastasis 16 years after the initial surgery for a malignant melanoma. The patient was a 58-year-old Japanese man. In 1976, he had a pigmented skin lesion with a diameter of 8 mm on his right third finger. He received an amputation of the finger and a dissection of the right axillary. Histological examinations of the tumor revealed a feature of a malignant melanoma with infiltration of the papillary layers of the dermis, 1.5 mm in thickness. The histological subtype was considered to be an acral lentiginous melanoma with a mixed spindle-epithelioid cell pattern. There was no regional lymph node metastasis. In December 1992, when he was 74-years-old, a round tumor in the left lower lung was discovered by chest radiography. In February 1993, he received a left lower lobectomy of the lung. Histological examination revealed a feature of a malignant melanoma with predominantly epithelioid cells and this was considered to be a metastasis from the initial skin lesion. Five months after the lobectomy, he died from a hemorrhage of a metastatic brain tumor. This case indicated the importance of periodic, life-long follow-up in treating malignant melanomas.
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ranking = 1
keywords = chest
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8/918. dermatofibrosarcoma protuberans: an early non-protuberant phase of the tumour.

    A 40-year-old female patient presented with a flat scar like plaque on the upper chest. The patient's perception of subtle change in the lesion was of paramount importance in the decision to biopsy this lesion of innocuous clinical appearance. Histopathological findings were those of dermatofibrosarcoma protuberans. This case illustrates the early clinical features of the tumour and a recommendation for taking a biopsy of any scar-like plaque where there is no clear history of preceding trauma.
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ranking = 2.5456158375329
keywords = upper, chest
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9/918. 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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ranking = 3.6340580079389
keywords = back
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10/918. Cerebriform intradermal nevus: a case of scalp expansion on the galea.

    We report here a 7-year-old Japanese girl with cerebriform intradermal nevus (CIN). By placement of expanders on the galea, her scalp was expanded more easily with less discomfort than is expected when the expanders are placed under the galea. An immunohistochemical study on the expression of proliferating cell nuclear antigen suggested higher proliferative activity of nevus cells from the CIN lesion than that of cells from congenital or acquired intradermal nevi. The high proliferative activity appeared to be associated with a growth spurt of the lesion.
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ranking = 1.4906866563651
keywords = discomfort
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