Development of a biomarker of efficacy in second-line treatment for lymphangioma of the tongue: a pilot study

Lymphangioma of the tongue is a rare lymphatic malformation, and various authors have reported the successful use of sirolimus for its treatment. However, the safety of sirolimus in children needs further evaluation so that those who do not respond are not necessarily exposed to its potential adverse effects. We hypothesised that assessment of lymphangiogenesis can be used to predict whether the patient will respond to sirolimus, so we organised a prospective study after ethics committee approval had been given. After clinical and histological diagnoses of lymphangioma of the tongue had been confirmed, 16 patients were given sirolimus 0.8 mg/day in three divided doses. Clinical response was assessed and compared with lymphatic microvessel density (LMVD), which was calculated immunohistochemically using the monoclonal antibody D2-40 as the lymphatic endothelial marker. Nine patients responded well, five partially, and two failed to respond. Mean (SD) LVD among the good responders was 21.00 (3.74), whereas among non-responders it was 8.00 (4.24). There was a significant difference in mean LVD between good responders, partial responders, and non-responders (p = 0.04). Sirolimus is effective in treating children with lymphangioma of the tongue, and lymphangiogenesis is a useful therapeutic predictive marker.     

Cavernous lymphangioma of the breast: case report with electron microscopic and immunohistochemical investigations

A rare case of cavernous lymphangioma of the breast in a 16-year-old Japanese girl was investigated by light and electron microscopies, and immunohistochemistry. The patient, who had a soft breast tumor measuring 16 x 14 cm, had undergone a tumorectomy two years after she noticed the tumor. Histologically, the tumor was composed of numerous narrow spaces containing amorphous fluid lined with a monolayer of endothelial cells. Abundant collagen fibers and numerous fibroblasts were found beneath the endothelium. Histological findings confirmed a diagnosis of lymphangioma of the breast. Immunohistochemically, the endothelium showed negative immunoreaction for factor VIII-related antigen, but the discontinuous basal lamina was positive for laminin. Ultrastructurally, numerous microfilaments and pinocytotic vesicles were found in the cytoplasm of the endothelial cells, and intermediate junctions were recognized between adjacent cells. In addition, a discontinuous basal lamina was seen beneath the endothelium. Electron microscopic and immunohistochemical investigations are thus useful in confirming a diagnosis of cavernous lymphangioma of the breast.     

Cystic lymphangioma of the scrotum

A 7-year-old boy who presented with a painful left hemiscrotal mass was diagnosed with acquired lymphangioma of the scrotum. Chronic friction from a cast for Perthes' disease might have been the cause of sudden enlargement of a congenital lymphangioma of the scrotum. Magnetic resonance imaging (MRI) was useful for preoperative diagnosis and determining the extent of the scrotal lesions. Total excision of the mass leaving the overlying skin was successfully performed. The clinical significance of MRI for preoperative diagnosis and planning surgical resection of this lesion is discussed. Accepted: 1 April 1997     

平阳霉素瘤内注射治疗口腔颌面部淋巴管瘤长期临床观察

自1987年11月至1995年9月采用平阳霉素瘤体内注射治疗口腔颌面部各种类型淋巴管瘤208例，随访1～8年200例，其中5年以上80例，总有效率97．5％，治愈率86．5％。未出现肺炎及纤维化等严重并发症。本文对疗效与分型，瘤体大小及年龄的关系，药物作用机制及其副反应作了详细分析。     

儿童体表淋巴管瘤的磁共振影像诊断

目的探讨儿童体表淋巴管瘤的磁共振影像学表现,评价磁共振影像诊断在儿童体表淋巴管瘤的价值,提高对淋巴管瘤的影像诊断水平。方法回顾性分析2014年1月～2018年12月年经手术或穿刺病理证实的的儿童体表淋巴管瘤42例的磁共振影像。结果单纯囊性淋巴管瘤26例,海绵状淋巴管瘤9例,血管淋巴管瘤7例。40例均表现为多房囊性结构,2例表现为单囊结构;18例病例可见液液平面;7例见血管穿行征;4例可见血管挤压征;20例表现为混杂信号,22例表现为长T1长T2信号;颈部及腋下大部分淋巴管瘤呈侵袭性生长,四肢及头皮肿物呈爬行性生长。结论儿童体表淋巴管瘤磁共振影像具有一定的特征,磁共振对儿童淋巴管瘤的诊断有重要的价值,能准确判断病灶的范围,对临床手术评估及内科治疗具有重要的意义。     

Surgery for cystic lymphangioma in Gorlin–Goltz syndrome

INTRODUCTION: We present a case of three major lymphomesenteric cysts in a female patient with known basal cell nevus syndrome (BCNS or Gorlin-Goltz syndrome). DISCUSSION: Although those cysts have been reported to appear in this syndrome, the exact prevalence is not known. In this case, the patient suffered from severe and recurrent abdominal pain due to bowel obstruction. CONCLUSION: By causing severe symptoms, cystic lymphangiomas are an important complication of the BCNS despite of their seldom occurrence. Surgeons confronted with intraabdominal masses in a patient with BCNS should always be aware of this manifestation of the disorder and plan interdisciplinary operations.     

A case of intrapulmonary lymphangioma surrounded by pulmonary hilar structures

A 53-year-old man was referred to our hospital for examination of a nodule in the right hilar region observed on a chest roentgenogram. Computed tomography and magnetic resonance imaging revealed a 34 × 32-mm multilocular cystic mass with partial calcification adjacent to the hilar structures in the right upper lung lobe. The mass was resected using video-assisted thoracoscopic surgery and identified as pulmonary cystic lymphangioma through postoperative histopathological analysis. Intrapulmonary lymphangioma is an uncommon benign tumor, with only few reports of surgically resected cases. We describe our surgical findings and recommend surgical resection where possible for suspected intrapulmonary lymphangioma.     

Lymphangioma in the Epidural Space of the Thoracic Spine

A rare case of solitary intraspinal epidural lymphangioma is described with a review of the literature. A 16-year-old boy was admitted to our hospital with a history of two-year of progressive paraparesis. Magnetic resonance imaging study revealed a 2 × 2 × 6 cm sized epidural cystic mass in the thoracic spine. Surgical total removal and biopsy were performed. The final pathologic report on the mass indicated lymphangioma.     

胎儿颈部水囊状淋巴管瘤3例

患者1,女,35岁,孕20＋6周,孕2产0,既往体健。常规产前超声检查：胎儿发育与孕周相符,脊柱排列整齐,于胎儿颈部皮下可见大小约65mm×38mm的囊性回声,边界清,形态不规则,内部透声尚可,可见分隔（图1）。羊水增多,最大深度90mm,胎儿双侧胸腔可见积液。超声诊断：胎儿畸形,