皮肤粒细胞肉瘤

报告1例皮肤粒细胞肉瘤.患者男,50 岁.3 年前不慎被竹片刺伤,皮损处出现结节,逐渐增大、增多,沿淋巴管方向分布于双上肢.曾行外周血常规及骨髓穿刺检查均正常.入院行组织病理检查示低分化髓细胞髓外(皮肤和皮下脂肪组织)浸润.免疫病理示髓过氧化物酶(MPO)(++)、Ki67(++)、CD43(+).组织病理诊断为皮肤粒细胞肉瘤.确诊后行骨髓穿刺提示急性粒细胞白血病(AML,M2).转血液科接受化疗,化疗无效很快死亡.     

Isolated granulocytic sarcoma of the skin in an elderly patient: good response to treatment with local radiotherapy and low-dose methotrexate

Granulocytic sarcoma of the skin is frequently associated with haematological diseases and is rarely isolated. The disease generally develops into acute systemic myeloid leukaemia and is associated with a poor prognosis. We report an elderly patient presenting isolated granulocytic sarcoma of the skin who showed a very good response to treatment with local radiotherapy and low-dose methotrexate.     

Cutaneous granulocytic sarcoma arising at the site of radiotherapy for breast carcinoma

Carcinogenic effects of radiotherapy in breast cancer are well-known. Long-term follow-up of these patients shows a significantly increased risk of leukemia. Cutaneous granulocytic sarcoma is an uncommon leukemia cutis that usually occurs in association with acute myelocytic leukemia or myeloproliferative disorders. We report a case of cutaneous granulocytic sarcoma in a 44-year-old woman who had been treated six months earlier for breast adenocarcinoma. The treatment had associated lumpectomy, axillary lymph node dissection and radiotherapy. Skin lesions appeared firstly and predominantly on the irradiated area. Haematological investigations were normal and the diagnosis of isolated sarcoma was made. The uncommon features of this case were the short interval between radiotherapy and the occurrence of leukemia skin lesions and the fact that, to our knowledge, this is the first report of leukemia cutis localised on the irradiated area. The responsibility of radiation in the distribution of cutaneous lesions of granulocytic sarcoma is discussed.     

A case of CD56+ cutaneous aleukaemic granulocytic sarcoma with myelodysplastic syndrome

We describe a 70-year-old man with cutaneous granulocytic sarcoma who presented with numerous cutaneous nodules but without any leukaemic involvement of the peripheral blood. The tumour cells were positive for lysozyme, peroxidase, CD11a, CD11c, CD33 and HLA-DR, and weakly positive for CD4 and CD14, suggesting granulocytic differentiation. The bone marrow at admission showed dysplasia of the erythrocytic and granulocytic lineage and complex chromosomal abnormalities in association with an increase in monocytes. The patient was diagnosed as having granulocytic sarcoma of monocytic lineage with concomitant myelodysplastic syndrome. In this case, tumour cells also expressed the neural cell adhesion molecule (CD56), which has been suggested as a possible risk factor for developing granulocytic sarcoma in acute myelogenous leukaemia.     

Polyamines in malignant lymphoma of the skin and in Kaposi's sarcoma

High-pressure liquid chromatography was used to detect polyamines, the markers of lymphoproliferative diseases of the skin and of Kaposi's sarcoma. The urine polyamine detection may become one of the diagnostic tests in these conditions, but its findings should be confirmed by clinical and histologic data. Measurements of the urine polyamines may be helpful in assessment of the treatment efficacy.     

Extramedullary (skin) presentation of acute monocytic leukemia resembling cutaneous lymphoma: morphological and immunulogical features

Acute monocytic leukemia has been noted to exhibit a predilection for extramedullary involvement (gums, skin, and lymph nodes) at presentation. More unusual is the occurrence in an extramedullary site in the absence of bone marrow involvement. A case is reported with initial presentation in skin preceding a subsequent evolution to a leukemic phase by one year. The skin tumor was initially diagnosed and treated as a lymphoma. A second skin tumor, biopsied one year later was immunophenotyped as a T cell lymphoma using a screening panel of antisera (OKT4 positive, OKMI negative). Shortly thereafter a monocytic leukemia (M5) was discovered. Using a larger panel of antisera and enzyme markers on the second skin biopsy confirmed the monocytic rather than lymphocytic nature of the skin tumor. This case illustrates the importance of using an expanded panel of monoclonal antisera in certain hematopoietic tumors.     

Aberrant Melan-A expression in atypical fibroxanthoma and undifferentiated pleomorphic sarcoma of the skin

Background: Atypical fibroxanthoma (AFX) is a distinctive clinicopathologic entity presenting on sun‐damaged skin of the elderly. Its behavior is benign if strict diagnostic criteria are applied. Tumors showing invasion of deeper structures or perineural/lymphovascular invasion are best regarded as undifferentiated pleomorphic sarcoma of the skin. The diagnosis requires immunohistochemical studies to exclude melanoma, squamous cell carcinoma, angiosarcoma and leiomyosarcoma. Methods: Two AFX and one undifferentiated pleomorphic sarcoma showing aberrant expression of Melan‐A were identified. Clinical data were obtained and histopathological features, immunohistochemical profile and electron microscopy were assessed. Results: All tumors arose on sun‐damaged skin of elderly males. Two AFX showed pushing growth into superficial subcutis only. The undifferentiated pleomorphic sarcoma was characterized by infiltrative growth into galea as well as perineural invasion. Multifocal expression of Melan‐A and MART‐1 was largely limited to tumor giant cells in the absence of S100 or HMB‐45 labeling. No melanosomes or premelanosomes were identified by electron microscopy. Conclusions: Aberrant expression of Melan‐A and MART‐1 in AFX and undifferentiated pleomorphic sarcoma of the skin represents an important diagnostic pitfall with potential for misdiagnosis as melanoma. Melan‐A expression limited to tumor giant cells, in the absence of S‐100 positivity, is a helpful diagnostic feature. Thum C, Hollowood K, Birch J, Goodlad JR, Brenn T. Aberrant Melan‐A expression in atypical fibroxanthoma and undifferentiated pleomorphic sarcoma of the skin.     

The immune status of patients with lymphoproliferative diseases of the skin and Kaposi's sarcoma

Manifest disorders of cellular and humoral immunity are detectable with the use of immunologic tests in patients with mycosis fungoides, skin reticulosis, and Kaposi's sarcoma. The pattern of changes in Kaposi's sarcoma differs from that in malignant lymphomas of the skin. The detected immune status disorders necessitate the use of immunomodulators in combined therapy of such patients.     

Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases

BACKGROUND: A number of malignant soft tissue tumors, particularly those of fibroblastic and fibrohistiocytic derivation, have been found to display myofibroblastic differentiation focally. The term myofibroblastic sarcoma, a controversial presumably distinctive entity, defines a malignant soft tissue tumor in which myofibroblasts are quantitatively the predominant cell type. METHODS: Five cases of cutaneous spindle-cell sarcomas showing fibroblastic-myofibroblastic differentiation with predominance of fibroblasts were retrieved from the files of three large centers of dermatopathology. Tumors were analyzed histopathologically, immunophenotypically, and, in two cases, ultrastructurally. Results were compared with those previously reported in fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma. RESULTS: Immunophenotypic and ultrastructural profiles of the cases analyzed in this series were closer to fibrosarcoma and to malignant fibrous histiocytoma than to myofibroblastic sarcoma by virtue of quantitative predominance of fibroblasts over myofibroblasts. On the other hand, histopathologic findings were in keeping with those reported in myofibroblastic sarcoma. CONCLUSIONS: Our series highlights the intrinsic problems in attaching certain cutaneous sarcomas with fibroblastic-myofibroblastic differentiation to one of the recognized entities and gives support to the hypothesis that fibrosarcoma, malignant fibrous histiocytoma, and myofibroblastic sarcoma are related histogenetically.