Abstract：
BACKGROUND: Although infantile hepatic hemangioma and hepatic mesenchymal hamartoma are relatively common in benign pediatric liver tumors, coexistence of the two tumors is rare. Placental mesenchymal dysplasia is also a rare disorder. We report the case of a baby girl born after a pregnancy complicated by placental mesenchymal dysplasia, who developed both infantile hepatic hemangioma and hepatic mesenchymal hamartoma.
METHODS: The patient was born at 32 weeks and 5 days of gestation for impending placental abruption, weighing 1450 g. Liver tumors, composed of both hypervascular solid and large cystic lesions, were detected after birth and markedly increased to create abdominal distention within 9 months. Diagnostic imaging suspected the coexistence of infantile hepatic hemangioma and cystic hepatic mesenchymal hamartoma. Following propranolol therapy for infantile hepatic hemangioma and needle puncture of a large cyst, the cystic lesions and adjacent hypervascular lesions were partially resected via laparotomy. Pathological findings confirmed the coexistence of hepatic mesenchymal hamartoma and infantile hepatic hemangioma, which had no association with androgenetic/biparental mosaicism. The postoperative course was uneventful, and the tumor had not regrown after 3 years.
CONCLUSIONS: Although the coexistence of infantile hepatic hemangioma and hepatic mesenchymal hamartoma associated with placental mesenchymal dysplasia is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could have derived from similar embryologic origins rather than being a mere coincidence. Further follow-up is required, with careful attention to the potential for malignant hepatic mesenchymal hamartoma transformation.
METHODS: The patient was born at 32 weeks and 5 days of gestation for impending placental abruption, weighing 1450 g. Liver tumors, composed of both hypervascular solid and large cystic lesions, were detected after birth and markedly increased to create abdominal distention within 9 months. Diagnostic imaging suspected the coexistence of infantile hepatic hemangioma and cystic hepatic mesenchymal hamartoma. Following propranolol therapy for infantile hepatic hemangioma and needle puncture of a large cyst, the cystic lesions and adjacent hypervascular lesions were partially resected via laparotomy. Pathological findings confirmed the coexistence of hepatic mesenchymal hamartoma and infantile hepatic hemangioma, which had no association with androgenetic/biparental mosaicism. The postoperative course was uneventful, and the tumor had not regrown after 3 years.
CONCLUSIONS: Although the coexistence of infantile hepatic hemangioma and hepatic mesenchymal hamartoma associated with placental mesenchymal dysplasia is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could have derived from similar embryologic origins rather than being a mere coincidence. Further follow-up is required, with careful attention to the potential for malignant hepatic mesenchymal hamartoma transformation.
摘要：
背景：尽管婴儿肝血管瘤和肝间质错构瘤在小儿良性肝肿瘤中相对常见，这两种肿瘤的共存是罕见的。胎盘间充质发育不良也是一种罕见的疾病。我们报道了一个怀孕后出生的女婴并发胎盘间充质发育不良的病例，同时发展为婴儿肝血管瘤和肝间质错构瘤。
方法：患者出生在妊娠32周零5天因即将发生胎盘早剥，重1450克。肝肿瘤，由高血管实性和大的囊性病变组成，在出生后检测到，并在9个月内明显增加腹胀。诊断影像学怀疑婴儿肝血管瘤和囊性肝间叶性错构瘤共存。普萘洛尔治疗婴儿肝血管瘤和大囊肿的针穿刺后，通过剖腹手术切除了部分囊性病变和邻近的高血管病变。病理结果证实肝间叶性错构瘤和婴儿肝血管瘤并存，与雄激素/双亲镶嵌无关。术后进展顺利，3年后肿瘤没有再生长。
结论：尽管婴儿肝血管瘤和与胎盘间质发育不良相关的肝间质错构瘤并存极为罕见，这些疾病在病理和发病机制上的相似性表明，它们可能源于相似的胚胎起源，而不是仅仅是巧合。需要进一步的后续行动，仔细注意恶性肝间叶错构瘤转化的潜力。
方法：患者出生在妊娠32周零5天因即将发生胎盘早剥，重1450克。肝肿瘤，由高血管实性和大的囊性病变组成，在出生后检测到，并在9个月内明显增加腹胀。诊断影像学怀疑婴儿肝血管瘤和囊性肝间叶性错构瘤共存。普萘洛尔治疗婴儿肝血管瘤和大囊肿的针穿刺后，通过剖腹手术切除了部分囊性病变和邻近的高血管病变。病理结果证实肝间叶性错构瘤和婴儿肝血管瘤并存，与雄激素/双亲镶嵌无关。术后进展顺利，3年后肿瘤没有再生长。
结论：尽管婴儿肝血管瘤和与胎盘间质发育不良相关的肝间质错构瘤并存极为罕见，这些疾病在病理和发病机制上的相似性表明，它们可能源于相似的胚胎起源，而不是仅仅是巧合。需要进一步的后续行动，仔细注意恶性肝间叶错构瘤转化的潜力。
