Cappellani Alessandro, Di Vita Maria, Lo Menzo Emanuele, Zanghì Antonio, Lanzafame Salvatore, Veroux PierFrancesco, Zanet Ernesto, Cavallaro Andrea, Berretta Massimiliano
Department of Surgery, University of Catania Medical School, Catania, Italy.
Ann Ital Chir. 2011 Sep-Oct;82(5):383-7.
Mesenteric and duodenal leiomyosarcomas are very rare malignancies. Muscular metastases from leiomyosarcoma are even more rare. Surgery is the only chance of cure and should be attempted whenever possible. The relief of symptoms and the prevention of recurrences are ultimately the aims of surgery. We present a unique case of mesocolic and duodenal leiomyosarcoma with muscular metastases.
A 61 year old woman was treated by radical resection including left neftectomy and left hemicolectomy for a leiomyosarcoma of the left mesocolon. Three years after the first surgery a leiomyosarcoma of the duodenal wall was diagnosed. Following a careful evaluation that ruled out the presence of other secondary locations, she underwent pancreatoduodenectomy. Three months later she observed a small, mildly painful swelling in the left thigh, rapidly growing to a diameter of 4 cm over a month period. The MRI showed a low-signal intensity malignancy in T2-weighted images whereas the lesion was homogeneously enhanced by Gadolinium on T1-weighted imaging. The histological examination after excision confirmed the clinical suspicion of a metastasis from high grade leiomyosarcoma. Successively the patient underwent a palliative chemotherapy treatment with epirubicin and ifosfamide for three cycles. The patient experienced a progression of disease with multiple pulmonary and encephalic metastases five months later.
Muscular metastases from leiomyosarcoma are occasionally described in the literature. The apparition of muscular metastases is considered a negative prognostic factor and shortly precedes massive distant diffusion of the malignancy. Denervation syndrome can be a risk factor for muscular metastases. To our knowledge, this is the first report of a skeletal-muscle metastasis following mesenteric and duodenal leiomyosarcoma.
肠系膜和十二指肠平滑肌肉瘤是非常罕见的恶性肿瘤。平滑肌肉瘤的肌肉转移更为罕见。手术是唯一的治愈机会，应尽可能尝试。缓解症状和预防复发是手术的最终目标。我们报告一例罕见的伴有肌肉转移的结肠系膜和十二指肠平滑肌肉瘤病例。
一名61岁女性因左结肠系膜平滑肌肉瘤接受了包括左肾切除术和左半结肠切除术的根治性切除。首次手术后三年，诊断出十二指肠壁平滑肌肉瘤。在仔细评估排除其他继发部位后，她接受了胰十二指肠切除术。三个月后，她发现左大腿有一个小的、轻度疼痛的肿块，在一个月内迅速增大至直径4厘米。MRI显示在T2加权图像上为低信号强度的恶性肿瘤，而在T1加权成像上病变经钆均匀强化。切除后的组织学检查证实了临床怀疑的高级别平滑肌肉瘤转移。随后，患者接受了表柔比星和异环磷酰胺的姑息化疗三个周期。五个月后，患者出现疾病进展，伴有多处肺和脑转移。
平滑肌肉瘤的肌肉转移在文献中偶尔有描述。肌肉转移的出现被认为是一个不良预后因素，且在恶性肿瘤大量远处扩散之前不久出现。去神经综合征可能是肌肉转移的一个危险因素。据我们所知，这是肠系膜和十二指肠平滑肌肉瘤后骨骼肌转移的首例报告。
