Juel Jacob, Ibrahim Rami Mossad
Department of Plastic and Reconstructive Surgery, Aalborg University Hospital, Aalborg, Denmark.
Department of Plastic and Reconstructive Surgery, Odense University Hospital, Odense, Denmark.
Int J Surg Case Rep. 2017;36:151-154. doi: 10.1016/j.ijscr.2017.05.034. Epub 2017 Jun 1.
Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made this designation obsolete. The true incidence remains unknown, but CCS is believed to represent less than one percent of all sarcomas.
A 22-year-old patient presented with a mass sized 2.6×2.7×2.7cm of the left gluteal region, pain, and malaise. Initially, the symptoms were interpreted as an infection. Subsequent, pathological diagnosis after surgical removal was tentatively MM albeit definitive pathological diagnosis was CCS.
The patient of this case underwent definitive surgical treatment with 2cm margin. In spite of time delay, because of prolonged time for definitive diagnosis, PET-CT and sentinel lymph node biopsy did not show any metastasis. One-year postoperatively, multidisciplinary follow-up is without suspicion of relapse.
Accurate and timely diagnosis of CCS are imperative, as initial misdiagnosis, may cause delay and further tumour growth, which is correlated to the prognosis.
透明细胞肉瘤（CCS）是一种罕见的软组织肿瘤，常被误诊，因为它与恶性黑色素瘤（MM）有共同特征。以前，CCS被描述为软组织恶性黑色素瘤，但当代免疫组化技术已使这一命名过时。其真实发病率尚不清楚，但CCS被认为占所有肉瘤的比例不到1%。
一名22岁患者出现左侧臀区一个大小为2.6×2.7×2.7cm的肿块、疼痛和不适。最初，这些症状被解释为感染。随后，手术切除后的病理诊断初步为MM，尽管最终病理诊断为CCS。
该病例患者接受了切缘2cm的确定性手术治疗。尽管由于明确诊断时间延长存在时间延迟，但PET-CT和前哨淋巴结活检未显示任何转移。术后一年，多学科随访未怀疑复发。
准确及时地诊断CCS至关重要，因为最初的误诊可能导致延误和肿瘤进一步生长，这与预后相关。
