Cases reported "Skin Neoplasms"

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1/50. B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behaviour.

    The term Richter's syndrome is used to describe the transformation of chronic lymphatic leukaemia (CLL) into a high-grade systemic lymphoma and is associated with a poor prognosis. We have undertaken detailed molecular studies in two patients with cutaneous B-cell lymphoma (CBCL) and CLL. Patient 1 exhibited a low-grade CBCL with different immunoglobulin gene rearrangements in blood and skin. By contrast, patient 2 showed identical gene rearrangements, confirmed by gene sequencing, and died within 4 months of presentation. The latter patient fulfilled the criteria for a diagnosis of cutaneous Richter's syndrome, whereas the former patient demonstrated the coincidence of CLL with a primary CBCL. Our results highlight the importance of gene rearrangement studies with sequencing for the accurate diagnosis of cutaneous Richter's syndrome.
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2/50. Myoepitheliomas of the skin and soft tissues. Report of 12 cases.

    We describe 12 cutaneous and soft tissue myoepitheliomas, most of them in elderly patients. Morphologically the cutaneous and soft tissue myoepitheliomas revealed the same spectrum as their salivary gland counterparts. They were composed of a mixture of spindle, epithelioid and clear myoepithelial cells. Immunohistochemically they were positive to keratins and S-100 protein and reacted inconsistently with antibodies to smooth muscle actin. Morphologically they lacked any folliculo-sebaceous or apocrine differentiation. We believe that they are related to the eccrine type of cutaneous mixed tumours. Most cases had a benign behaviour, but 1 tumour metastasized, and the patient died of the tumour. Myoepitheliomas of soft tissues should be distinguished from other neoplasms with epithelial differentiation and from ossifying fibromyxoid tumour of soft parts, parachordoma and extraskeletal myxoid chondrosarcoma.
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3/50. Sentinel-node guided lymph-node dissection for merkel cell carcinoma.

    Merkel cell carcinoma is an aggressive neuroendocrine skin tumour. Treatment is still debatable. Merkel cell carcinoma resembles malignant melanoma in its cutaneous presentation and its embryonic origin; both have unpredictable biological behaviour, early regional lymph node involvement, early distant metastases and a high recurrence rate. In light of these common features, we used pre-operative lymphoscintigraphy, intraoperative lymph-node mapping and sentinel-node biopsy-a well-described technique for the treatment of melanoma-in a 60-year-old man with Merkel cell carcinoma in the right buttock. Following frozen section identification of a metastatic first-order sentinel node, radical right groin dissection was performed. All the other lymph nodes in this basin proved to be disease-free, including the second-order sentinel node and Cloquet node. The patient is now being treated with adjuvant chemotherapy and radiotherapy. This case shows that sentinel-node guided dissection is applicable to Merkel cell carcinoma.
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4/50. Cutaneous malignant melanoma: Tabuk experience.

    Cutaneous melanoma (CM) has a rising morbidity and mortality in the western world but is rare in certain geographical areas including the middle east. The aim of this study is to define the pattern of CM in this environment over a period of about two decades. A review of associated histological reports, dermatology, plastic general surgical admissions and outpatient census statistic of the North West Military Hospital (N.W.A.F.H.) were carried out from January 1978 to June 1996. The clinico-therapeutic information from both the review case and newly discovered CM was then studied. The result shows that CM is probably rare in the Tabuk military environment and possibly has a low mortality among the affected individuals. The presence of only 2 cases of CM among 73,955 patients over about 20 years suggests that this neoplasm is rare in N.W.A.F.H. Surgery, with localised expert reconstruction, probably offers the best cure for uncomplicated CM in this area. It is suggested that the geographical environment, genetic attributes, custom, attitude, presence of white, painted, sun-reflecting buildings, traditional dress-code and behaviour of the indigenes probably contribute to the suppression of and protection against CM in Tabuk. It is recommended that regular, antimlanoma education awareness programmes among the indigenes and avoidance of sunbathing attitude of the expatriate community should be encouraged in order to maintain this suggested natural selection protection.
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5/50. Common blue naevus with satellite lesions: possible perivascular dissemination resulting in a clinical resemblance to malignant melanoma.

    We report a case of common blue naevus with polymorphous guttate and linear satellite lesions, thereby mimicking peripherally spreading malignant melanoma. Histopathologic examination showed that the naevus cells are clustered around blood vessels in the primary as well as satellite lesions, suggestive of spreading of the naevus cells along the perivascular space. Such biological behaviour resulting in a clinical manifestation of a malignant melanoma-like lesion is a rarity in common blue naevus, a benign cutaneous disorder that is devoid of a malignant potential, and has not been described before.
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6/50. Giant pilomatricoma simulating carcinoma of the male breast.

    We present the case of a 65-year-old man with a large calcifying epithelioma in the right breast. The case is unusual not only for the size of the tumour but also for its location in a male breast. Despite its large size (12 x 10 x 7 cm), the tumour did not show any clinical, radiological or histological evidence of malignancy or aggressive behaviour. One year after surgery there was no recurrence or metastatic disease. The possibility of malignancy, recurrence or metastasis of these large tumours, as well as the necessity of follow-up and investigations, are emphasised.
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7/50. Pseudovascular squamous cell carcinoma of the skin.

    The presence of acantholysis in squamous cell carcinomas (SCC) may rarely be so extreme that, histologically, it mimics a vascular tumour. However, careful histological examination and immunohistochemical study usually lead to the correct diagnosis. We describe such a case to highlight the clinico-pathological features of this rare form of cutaneous malignancy and to emphasize the difficulties in establishing the correct diagnosis. We also review similar cases reported in the literature. Pseudovascular SCC shows a higher degree of recurrence and metastasis than other variants of SCC. Acantholytic foci in these tumours may demonstrate changes in keratinocyte differentiation markers, and this may explain the more aggresive biological behaviour in the pseudovascular variant of SCC.
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8/50. An unusual cutaneous T cell lymphoma presenting as leonine facies.

    We report an unusual case of a cutaneous T cell lymphoma with the clinical picture of leonine facies as the only skin symptom appearing during the first years of the disease. Small atypical lymphocytes with partly pleomorphic, partly indented cerebriform nuclei are present in the facial skin as well as in the peripheral blood and in the bone marrow. The lymphoma shows an indolent clinical behaviour without rapid progression of the disease. It shares features with both pleomorphic small/medium-sized T cell lymphoma and mycosis fungoides, but cannot be classified according to the criteria of the European Organization for research and Treatment of Cancer (EORTC) classification for Primary Cutaneous Lymphomas. At present the lymphoma has to be ranged among the group of so-called unspecified peripheral T cell lymphomas according to the proposal of the International lymphoma Study Group.
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9/50. Gingival and cutaneous angiosarcoma.

    Multiple oral and cutaneous nodular and papular reddish-blue lesions are described in the case of a 60-year-old woman. The duration of the lesions was more than 1 year, with the oral lesion preceding the skin lesions. Histopathological examination revealed malignant vascular tumour with changes consistent with angiosarcoma. Angiosarcoma is an extremely rare malignant tumour of the oral cavity, and the present case describes oral and skin lesions with a unique clinical behaviour.
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10/50. Dermatomyofibroma: a report of two cases, one occurring in a child.

    Dermatomyofibroma is a recently described plaque-like dermal tumour composed of myofibroblasts that usually presents around the shoulder, axilla and posterior neck, often in young adult females. Here, we present two cases, one from the posterior axilla of a 33-year-old female and one from the posterior neck of a 7-year-old male. Both were clinically red-brown lesions with histological and immunohistochemical features diagnostic of dermatomyofibroma. There was no evidence of aggressive biologic behaviour with 3 months and 2 months follow up, respectively. While the majority of dermatomyofibromas present in postpubescent females, the 7-year-old male exemplifies a subgroup occurring in male children which appears to show a particular predilection for the posterior neck.
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