Kanazawa Ippei, Yamauchi Mika, Yano Shozo, Imanishi Yasuo, Kitazawa Riko, Nariai Yoshiki, Araki Asuka, Kobayashi Keisuke, Inaba Masaaki, Maruyama Riruke, Yamaguchi Toru, Sugimoto Toshitsugu
Department of Internal Medicine 1, Shimane University Faculty of Medicine, 89-1 Enya-cho, Izumo 693-8501, Japan.
Bone. 2009 Sep;45(3):603-8. doi: 10.1016/j.bone.2009.05.018. Epub 2009 May 28.
Malignant transformation of fibrous dysplasia is very rare and has not been previously described in patients with McCune-Albright syndrome in the absence of radiation treatment during gestation. Here, we report a 38-year-old pregnant woman with McCune-Albright syndrome and acromegaly accompanied by osteosarcoma. The patient was in the 6th week of pregnancy, when she visited our hospital. She had multiple fibrous dysplasia, skin pigmentation, and acromegaly. The markedly high bone turnover rate during pregnancy tended to decrease after a normal delivery. Fibrous dysplasia of the lower jaw rapidly increased in the 37th week of pregnancy, and the tumor was surgically resected after delivery. Pathological examination of the resected tumor revealed fibrous dysplasia admixed with osteosarcoma containing chondroblastic and osteoblastic tissue. We firstly reported a case of osteosarcoma in a patient with McCune-Albright syndrome, which rapidly progressed during pregnancy.
纤维发育不良的恶性转化非常罕见,此前在孕期未接受放射治疗的McCune-Albright综合征患者中尚未有过相关描述。在此,我们报告一名38岁患有McCune-Albright综合征和肢端肥大症并伴有骨肉瘤的孕妇。该患者在怀孕第6周时前来我院就诊。她患有多处纤维发育不良、皮肤色素沉着和肢端肥大症。孕期明显较高的骨转换率在正常分娩后趋于下降。下颌骨的纤维发育不良在怀孕第37周时迅速增大,产后对肿瘤进行了手术切除。对切除肿瘤的病理检查显示,纤维发育不良中混杂有包含软骨母细胞和成骨细胞组织的骨肉瘤。我们首次报告了一例McCune-Albright综合征患者发生骨肉瘤且在孕期迅速进展的病例。