Shaham D, Ramu N, Bar-Ziv J
Department of Radiology, Hadassah University Hospital, Kiryath Hadassah, Jerusalem, Israel.
Acta Radiol. 1992 Sep;33(5):444-6.
We describe a 56-year-old female with absence of the right pectoralis muscles, aplasia of the right breast, and skeletal deformities of the right hand, typical of Poland's syndrome. Following complaints of lower abdominal pain, a CT examination revealed an 8-cm mass in the right anterior pelvic wall. Surgical resection of the mass revealed a high-grade, poorly differentiated leiomyosarcoma. Poland's syndrome is known to be associated with a high incidence of leukemia but this is the first description of its association with leiomyosarcoma. Although we cannot exclude the possibility of a chance association, it is reasonable to assume that, similar to other syndromes with multiple congenital anomalies, the association with an increased incidence of malignancy is an integral part of the underlying genetic abnormality.
我们描述了一名56岁女性，其右侧胸肌缺如、右乳发育不全以及右手骨骼畸形，这是典型的波兰综合征。在出现下腹部疼痛的症状后，CT检查发现右前盆腔壁有一个8厘米的肿块。对该肿块进行手术切除后发现是高级别、低分化平滑肌肉瘤。已知波兰综合征与白血病的高发病率相关，但这是其与平滑肌肉瘤关联的首次描述。虽然我们不能排除偶然关联的可能性，但有理由推测，与其他具有多种先天性异常的综合征类似，与恶性肿瘤发病率增加的关联是潜在基因异常的一个组成部分。
