Cutaneous sarcomas
Summary Cutaneous sarcomas comprise a heterogeneous group of mesenchymal spindle cell tumors of the dermis and subcutis, one of the best‐known entities being dermatofibrosarcoma protuberans. Other sarcomas addressed in this review include atypical fibroxanthoma, cutaneous undifferentiated pleomorphi...
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| Published in | Journal der Deutschen Dermatologischen Gesellschaft Vol. 15; no. 6; pp. 630 - 648 |
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| Main Authors | , , |
| Format | Journal Article |
| Language | English |
| Published |
Germany
Wiley Subscription Services, Inc
01.06.2017
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| Subjects | |
| Online Access | Get full text |
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| Summary: | Summary
Cutaneous sarcomas comprise a heterogeneous group of mesenchymal spindle cell tumors of the dermis and subcutis, one of the best‐known entities being dermatofibrosarcoma protuberans. Other sarcomas addressed in this review include atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, leiomyosarcoma, liposarcoma, and angiosarcoma. With the exception of dermatofibrosarcoma protuberans, which has its peak incidence in middle‐aged adults, cutaneous sarcomas usually occur in elderly individuals starting in the sixth or seventh decade of life. The pathogenesis of the various disease entities is not uniform and has not been definitively ascertained. Histology is the key to arriving at a correct diagnosis, and should always include ruling out other dermal neoplasms such as melanoma. In recent years, molecular genetic methods have provided greater insight into the pathogenesis, thus paving the way for new targeted therapies. Treatment of choice for cutaneous sarcomas is excision with sufficient surgical margins. Adjuvant and neoadjuvant therapeutic concepts include radiation therapy and the use of targeted therapies or chemotherapies. Local recurrences have frequently been reported in cutaneous sarcomas. Unlike soft tissue sarcomas, the prognosis in terms of survival – with the exception of angiosarcoma – is very good if treated adequately, a fact that should be emphasized to patients. |
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| Bibliography: | The most common primary cutaneous sarcomas include dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), dermal undifferentiated pleomorphic sarcoma, and leiomyosarcoma. Common vascular neoplasms of the dermis and subcutis include Kaposi's sarcoma and angiosarcoma. DFSP usually presents as a plaque with solitary or multiple skin‐colored/brownish‐red tumor nodules; initial lesions may also present as atrophic plaques. Differential diagnoses ought to include squamous cell carcinoma, basal cell carcinoma, and amelanotic melanoma. In terms of differential diagnosis, (amelanotic) melanoma and dedifferentiated squamous cell carcinoma should be ruled out using appropriate markers (S100 and pancytokeratin markers). The disease entity ‘cutaneous undifferentiated pleomorphic sarcoma’ comprises those cutaneous sarcomas that cannot be unequivocally classified despite careful histological assessment. Predilection sites are chronically sun‐exposed areas in the head/neck region. Clinical presentation and histopathological assessment play a key role in the diagnosis of cutaneous sarcomas. Detection of the t(17;22)(q22;q13) translocation using RT‐PCR or FISH can be diagnostically useful. The exact pathogenesis of AFX is still unclear. Atypical fibroxanthoma (AFX) primarily occurs in elderly individuals. Treatment of choice is excision with sufficient surgical margins and histological examination of tumor margins. The risk of distant metastases is increased in leiomyosarcomas that infiltrate the subcutis; in this context, the lungs are most frequently affected. Angiosarcomas of the scalp and face may present as hematoma‐like macules, plaques, or nodules with poor demarcation from the surrounding tissue, thus mimicking lymphoma, hematoma, or inflammatory dermatoses. On the molecular level, DFSP is largely characterized by a t(17;22)(q22;q13) translocation. On the molecular level, mutations in the p53 tumor suppressor gene associated with a UV signature can frequently be demonstrated. The term “malignant fibrous histiocytoma” is no longer used in the current literature. Treatment of choice is excision with wide surgical margins and complete histological examination of tumor margins. Based on patients’ individual risk, follow‐up exams should be carried out on a semiannual basis for at least five years. Treatment of choice for atypical fibroxanthoma is wide excision (surgical margins: 1–2 cm). Complete histological examination of tumor margins reduces the probability of a local recurrence. Imatinib acts as a multikinase inhibitor in the PDGFRB signaling pathway. Both the histological picture as well as immunohistochemical properties essentially correspond to those seen in AFX. Differentiation from other neoplasms such as malignant peripheral nerve sheath tumors, dedifferentiated carcinomas, or melanoma is of foremost importance. The prognosis of cutaneous sarcomas is comparatively good as they primarily tend to recur locally; distant metastases are rare. Given that dermal leiomyosarcomas with a subcutaneous component have a poorer prognosis, the depth of infiltration is a key parameter. Metastatic DFSP or lesions that cannot be resected may be systemically treated with imatinib. decade of life. Radiation therapy and chemotherapy can be incorporated into the treatment as adjuvant modalities. Immunohistochemically, endothelial cell markers (CD31, CD34, podoplanin) and nuclear transcription factors (FLI‐1, ERG) are of diagnostic significance. Treatment of choice is excision with wide surgical margins. Inoperable or metastatic angiosarcoma is treated with chemotherapy, for example, with pegylated liposomal doxorubicin or taxanes. Histopathologically, DFSP is characterized by interwoven (storiform), monomorphic spindle cells. Dermatofibrosarcoma protuberans is one of the most common cutaneous sarcomas, with a peak incidence in the second to fifth decade of life. Angiosarcomas arising in chronic lymphedema of the extremities are designated by the eponym Stewart‐Treves syndrome. Treatment of choice is wide excision (surgical margins: 1–3 cm) as well as complete micrographic control of excisional margins. Cutaneous leiomyosarcoma is an uncommon tumor, with a peak incidence in the 6 In the rare case of metastasis, patients should be treated at specialized centers and, if possible, in the context of a clinical trial. Clinically, atypical fibroxanthoma presents as a firm, erythematous or skin‐colored, barely mobile nodule on actinically damaged skin. A current topic of debate is whether atypical fibroxanthoma might be a superficial variant of cutaneous undifferentiated pleomorphic sarcoma. Given that metastasis is rare, the prognosis is generally favorable. Cutaneous angiosarcomas may occur as primary tumors, or develop in association with ionizing radiation or due to chronic lymphedema. Clinically, cutaneous leiomyosarcoma presents as a firm, skin‐colored or erythematous subcutaneous nodule that may occasionally be tender to pressure. to 7 Angiosarcomas account for approximately 1 % of all sarcomas, with an incidence of 0.01/100,000. The skin is the most common organ involved, with more than one‐third of all cases. The pigmented variant of DFSP is referred to as Bednar tumor. DFSP has a good prognosis in terms of survival, with 10‐year survival rates of up to 99 %. Cutaneous undifferentiated pleomorphic sarcoma Compared to other superficial cutaneous sarcomas, angiosarcomas have a poor prognosis with 5‐year survival rates of 10–30 %, primarily due to early hematogenous dissemination. Cutaneous leiomyosarcomas originate from arrector pili or vascular smooth muscle cells. Treatment of choice for localized angiosarcomas is complete excision. th Leiomyosarcoma consists of atypical spindle cells arranged in fascicles, bundles, or nodules. Transformed adipocytes are regarded as the cell of origin in liposarcoma. Apart from atypical spindle or pleomorphic cells, histiocytic cells and multinucleated giant cells are also present. ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-3 content type line 23 ObjectType-Review-1 |
| ISSN: | 1610-0379 1610-0387 |
| DOI: | 10.1111/ddg.13249 |