Fish F S
Department of Dermatology/Cutaneous Surgery, St. Paul Ramsey Medical Center, MN 55101, USA.
Dermatol Surg. 1996 Mar;22(3):268-73. doi: 10.1111/j.1524-4725.1996.tb00318.x.
Dermatofibrosarcoma protuberans, atypical fibroxanthoma, and cutaneous leiomyosarcoma are uncommon soft tissue sarcomas that tend to have a high recurrence rate when removed with standard excisional surgery, and in some cases have been reported to metastasize. These tumors have been reported in the surgical, medical, and pathology literature. The diagnosis and treatment of these tumors is still evolving. Wide excision and surgical techniques involving meticulous surgical margin control appear to be the most efficacious treatment for these problematic tumors.
The surgical, medical, and pathology literature was extensively reviewed to collate the observations of multiple investigators, and to summarize their findings. Literature from medical journals of multiple subspecialties as well as textbooks were reviewed with particular emphasis on diagnosis, histologic features including immunohistochemistry, and useful surgical techniques that facilitate the removal of these difficult tumors.
Dermatofibrosarcoma protuberans is a soft tissue neoplasm of intermediate malignancy that shows a high recurrence rate when treated with standard excision. Wide surgical excision using a 3-cm margin greatly reduces the risk of recurrence, but is not practical in some cases. Evidence is continuing to accumulate that Mohs micrographic surgery may be the treatment of choice for this tumor. Atypical fibroxanthoma is best thought of as a superficial form of malignant fibrous histiocytoma. It is histologically indistinguishable from certain forms of malignant fibrous histiocytoma and differs primarily in its size and superficial location. These tumors are best removed surgically. For most smaller tumors a 1-cm margin, which is carried down to the subcutaneous tissue, provides adequate treatment. In cases where tissue conservation is important, Mohs micrographic surgery has been shown to be very effective. Cutaneous leiomyosarcoma is a rare superficial soft tissue sarcoma that has a high incidence of recurrence after excision and can metastasize. Because of the small number of patients treated, treatment recommendations are still evolving. Currently, wide local excision is recommended with a 3-5 cm margin and removal of the underlying subcutaneous tissue. Mohs micrographic surgery has been used in a small number of cases with good success.
Dermatofibrosarcoma protuberans, atypical fibroxanthoma, and cutaneous leiomyosarcoma are soft tissue sarcomas that have a high rate of recurrence when treated with standard surgical techniques and may occasionally metastasize. When removing these tumors it is very important to take adequate margins. Mohs micrographic surgery is proving to be very useful in treating these difficult neoplasms.
隆突性皮肤纤维肉瘤、非典型纤维黄色瘤和皮肤平滑肌肉瘤是罕见的软组织肉瘤，采用标准切除手术切除时往往复发率很高，且在某些情况下有转移的报道。这些肿瘤在外科、医学和病理学文献中均有报道。这些肿瘤的诊断和治疗仍在不断发展。广泛切除以及涉及严格控制手术切缘的手术技术似乎是治疗这些疑难肿瘤最有效的方法。
广泛查阅外科、医学和病理学文献，整理多位研究者的观察结果，并总结他们的发现。查阅了多个亚专业医学期刊的文献以及教科书，特别强调了诊断、包括免疫组织化学在内的组织学特征，以及有助于切除这些疑难肿瘤的实用手术技术。
隆突性皮肤纤维肉瘤是一种中度恶性的软组织肿瘤，采用标准切除治疗时复发率很高。采用3厘米切缘的广泛手术切除可大大降低复发风险，但在某些情况下并不实用。越来越多的证据表明，莫氏显微外科手术可能是治疗该肿瘤的首选方法。非典型纤维黄色瘤最好被视为恶性纤维组织细胞瘤的一种浅表形式。它在组织学上与某些形式的恶性纤维组织细胞瘤无法区分，主要区别在于其大小和浅表位置。这些肿瘤最好通过手术切除。对于大多数较小的肿瘤，1厘米切缘并切至皮下组织即可提供充分的治疗。在组织保留很重要的情况下，莫氏显微外科手术已被证明非常有效。皮肤平滑肌肉瘤是一种罕见的浅表软组织肉瘤，切除后复发率很高，且可发生转移。由于治疗的患者数量较少，治疗建议仍在不断发展。目前，建议进行广泛局部切除，切缘为3 - 5厘米，并切除下方的皮下组织。莫氏显微外科手术已在少数病例中应用并取得了良好效果。
隆突性皮肤纤维肉瘤、非典型纤维黄色瘤和皮肤平滑肌肉瘤是软组织肉瘤，采用标准手术技术治疗时复发率很高，偶尔可能发生转移。切除这些肿瘤时，获取足够的切缘非常重要。事实证明，莫氏显微外科手术在治疗这些疑难肿瘤方面非常有用。
