Long-term follow-up of nineteen cystic lymphangiomas treated with fibrin sealant.

BACKGROUND: Surgical exeresis is regarded as the first choice treatment for cystic lymphangioma. Surgery may be extremely complex, giving rise to complications. Several therapeutic methods have been described to avoid the complications derived from the conventional surgical approach. The idea of treating lymphangioma by means of suction and injection of fibrin sealant (Tissucol), is an alternative to surgery. METHODS: The authors present 19 cases of cystic lymphangioma, treated with fibrin sealant injected into the lesion, during the 1991 to 1997 period. Two of the patients had been treated surgically and experienced recurrence of the tumor previous to treatment. In the other 17 cases, puncture was the only therapy applied. One patient required 3 punctures, another 6 patients required 2 punctures, and only 1 of them, after 2 unsuccessful punctures, was treated with surgical resection. The rest of the 10 cases subsided after the first puncture. Follow-up ranged between 3 and 72 months, with a mean of 40 months. RESULTS: The ultrasonographic (US) follow-up showed a complete remission in 17 patients treated with puncture. One patient remained with a small intermittent tumor, the appearance of which is related to catarrhal processes, and another patient rejected further puncture after the second one. No complications appeared. CONCLUSION: These results support the fact that the puncture, aspiration, followed by injection of Tissucol, is a choice in the surgical treatment of cystic lymphangioma.     

超声引导囊内注射平阳霉素治疗小儿囊状淋巴管瘤的疗效评价

目的探讨超声引导下平阳霉素注射治疗小儿囊状淋巴管瘤的方法及疗效。方法采用B超引导下囊内注射平阳霉素治疗 ,剂量为 0 4mg·kg-1·次 -1,最大量 8mg。每 4周 1次 ,共1～ 5次。结果本组 31例中治愈 2 5例 ,占 81% ;显效 3例 ,占 10 % ;好转 2例 ,占 6 % ;无效 1例 ,占3%。治愈显效率为 90 %。结论该疗法治疗小儿囊状淋巴管瘤的优点是定位准确、损伤小、并发症少、疗效好 ,不影响外观和功能。     

Total laparoscopic excision of retroperitoneal cystic lymphangioma

Retroperitoneal cystic lymphangioma is a rare benign tumor of the retroperitoneal lymphatics that usually manifests in infancy. If surgical excision is used in treatment, it needs to be as complete as possible to reduce the risk of recurrence. Two pediatric patients, an 18-month-old girl and a 4-yearold boy, underwent laparoscopic excision of symptomatic retroperitoneal cystic lymphangiomas. Macroscopically, the resection was complete in both cases. The postoperative course in both cases was uneventful. Both children remained asymptomatic and no recurrence was observed at 18-month follow-up. Complete laparoscopic excision should be considered as a therapeutic option to treat retroperitoneal cystic lymphangioma.     

肝囊性淋巴管瘤2例报告并国内文献回顾

目的:探讨肝囊性淋巴管瘤的临床特点和诊治方法,以提高诊断率及治疗效果。方法:回顾性分析广东医科大学附属医院收治的2例成人肝囊性淋巴管瘤患者临床资料及诊疗过程,并检索、复习中文期刊数据库中肝囊性淋巴管瘤的文献。结果:笔者收治的2例患者,均为女性,入院诊断分别为肝囊性占位和肝囊肿;患者完善相关检查后行肝切除术,术后病理诊断肝囊性淋巴管瘤;患者术后随访均无复发,治疗满意。检索1984—2017年期间国内共报道6例患者,其中男4例,女2例;入院诊断肝囊淋巴管瘤2例(2/6);其他均误诊(4/6),6例患者均行手术切除后明确诊断,术后患者恢复满意。结论:肝囊性淋巴管瘤为临床罕见的肝脏良性疾病。临床症状、体征及影像学表现无特异性,临床易误诊为肝囊肿及其他囊性疾病;MRl有助于鉴别诊断;有明确症状、体征或不能排除恶性的患者应结合个体情况制定治疗方案,病理检查是明确诊断的唯一方法。     

Cystic lymphangioma of the lesser omentum in an adult

Abdominal lymphangiomas are uncommon congenital benign tumors that occur mainly in children. The authors report the case of a 37-year-old woman with a cystic lymphangioma of the lesser omentum. The lesion was removed surgically with a complete resection. The histologic diagnosis was omental lymphangioma. Complete surgical resection with negative surgical margins is the treatment of choice, and the results are excellent. Incomplete resection may lead to recurrence.     

Surgical therapy of abdominal cystic lymphangioma in adults and children

BACKGROUND: Cystic lymphangioma is a rare malformative benign tumor of the lymphatic vessels. In the abdomen it generally develops as a mesenteric and/or retroperitoneal cyst, but any organ can be involved. The present retrospective study aims to define the symptoms, complications and differences noted between adults and children suffering from abdominal cystic lymphangioma; it is based on patients who underwent surgery for this condition at the Geneva University Hospital. PATIENTS AND METHODS: Since 1995, 16 patients (9 adults and 7 children) were surgically treated for abdominal cystic lymphangioma. Their medical files were reviewed retrospectively. The follow-up was based either on the last physical examination or on a telephone interview with the patients. RESULTS: The mean follow-up was 45 months. The most common presenting symptom was abdominal pain (38%). Ultrasonography was the most efficacious diagnostic modality. The lesions were mostly micropolycystic (44%), and found in retroperitoneal locations (50%). The surgical excision was complete in 14 patients and partial in 2 patients. These last 2 were the only ones who developed complications after the surgery, including one recurrence. CONCLUSIONS: A total surgical excision, if feasible without a major sacrifice of adjacent organs, seems to be the best therapeutic option to minimize the risk of recurrence of symptomatic abdominal cystic lymphangiomas. In our clinical experience, the presentation and evolution of the abdominal cystic lymphangioma seemed to be similar in adults and children.     

Cavernous lymphangioma of the leg in children treated by the injection of OK-432 after resection

We describe the treatment of three boys with cavernous lymphangioma of the legs. The suggested guidelines for treatment are extensive surgical resection, although complete resection is usually impossible, and approximately two weeks after the operation aspiration of serous fluid which has accumulated at the operation site, followed by injection of OK-432 (Picibanil). There was pyrexia and local inflammation for several days but the accumulation of serous fluid disappeared after the injection. There were no complications with no recurrence, joint contracture, or pain during a mean follow-up of 48 months (24 to 72). We conclude that an injection of OK-432 after surgical resection of cavernous lymphangioma of the legs in children is an effective treatment.     

Local injection of bleomycin A5 in lymphangioma in children. Analysis of 100 cases

From Jan. 1985 to Dec. 1988, 100 newborn and small infants suffering from lymphangioma were treated with bleomycin A5 injecting into lymphangioma. The cases were divided into three types: (1) Bleomycin A5 local injection into the lymphangioma were practiced on 70 cases; (2) Surgical partial resection of lymphangioma plus local injection on 26 cases; (3) A previous excision but recurred postoperatively were 4 cases. The dosage of bleomycin A5 was 10 mg per time. The therapeutic course was not more than 5 times. The efficacy of bleomycin A5 were satisfactory in all cases except only one case with a large lymphangioma of previous palliative excision but recurred. There was not a recurrent patient at following-up from 3 months to 4 years.     

后腹腔镜治疗腹膜后囊性淋巴管瘤2例报告并文献复习

目的提高对腹膜后囊性淋巴管瘤的认识,探讨其诊治方法。方法报告2例经本院收治的腹膜后淋巴管瘤患者的临床资料,回顾性分析其临床特征及影像学表现。2例均为女性,年龄分别为39岁及45岁。患者均无特征性临床表现。超声及CT均表现为边缘光整的囊性肿块。2例患者均在全麻下行腹腔镜腹膜后肿物切除术,并检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果 2例均成功切除病灶并经病理诊断为腹膜后囊性淋巴管瘤。术后随访5和24个月,未发现肿瘤复发。结论腹膜后囊性淋巴管瘤临床上少见,该病多无特征性临床表现,B超及CT检查对协助术前诊断及手术均有重要指导意义。腹腔镜手术治疗腹膜后囊性淋巴管瘤安全有效。     

Benign cystic mesothelioma in a child

An 11-month-old male presented with massive ascites and respiratory distress with no known etiology. Laparotomy confirmed ascites with multiple large cystic structures that originated in the left retroperitoneum. Grossly, it resembled lymphangioma; however, histopathologic diagnosis was benign cystic mesothelioma (BCM), an entity that presents mainly in women of childbearing age. After resection, the child had 2 recurrences for the following year, the first of which required resection. He also underwent resection of a congenital cystic adenomatoid malformation of the right lung. Only 4 other confirmed pediatric cases of BCM have appeared in the literature. As BCM may be confused with lymphangioma, it is important to be aware of BCM because of its propensity for recurrence and possible malignant degeneration.     

Huge cystic lymphangioma of the pancreas: report of a case

We report herein the case of a 33-year-old woman who presented with palpable abdominal swelling found to be caused by a huge lymphangioma of the pancreas. An abdominal computed tomographic (CT) scan showed a large multilocular cystic mass with water-dense contents, which was derived from the pancreatic head. A pancreaticoduodenectomy (PD) was performed because the tumor had invaded the duodenum. The resected tumor, which was 23 × 12 × 23 cm in size with 2 l of serous fluid, was pathologically diagnosed as a cystic lymphangioma. The endothelial cells lining the internal surface of the cystic spaces were immunohistochemically positive for factor VIII-R antigen and CD31. Our review of the literature revealed 45 reports of lymphangioma of the pancreas, including this one, but to the best of our knowledge this is only the fifth case that required a PD. Nevertheless, we recommend that a complete resection be performed to reduce the risk of recurrence. Received: April 3, 2000 / Accepted: March 6, 2001     

腹膜后淋巴管瘤的诊断与治疗

腹膜后囊性淋巴管瘤临床少见，这是一种淋巴管畸形或发育障碍所致的疾病。作者近２０年间收治６例。首发症状为腹部肿块及消化道功能障碍。术前确诊仅１例，其余误诊为胰腺囊肿，畸胎瘤及卵巢囊肿。６例均行手术治疗，单纯囊肿切除３例，囊肿大部分切除２例，囊肿并胰尾切除１例。手术均获成功。术后随访１～１９年，均未复发。作者认为，Ｂ超、ＣＴ对此病的诊断有重要意义，手术切除是目前治疗本病的理想方法。     

Surgery for ileal mesenteric lymphangioma during pregnancy: Case report and review of the literature

Mesenteric lymphangioma is one of the least frequently encountered types of benign tumor. This case report concerns a 31-year-old pregnant woman with a mesenteric cystic lymphangioma in the ileum. The multiloculated cystic mass was noted near the uterus by CT before the patient became pregnant. After becoming pregnant, she was followed without treatment for the asymptomatic mass. At 25 weeks’ gestation, however, she underwent emergency surgical treatment for small bowel obstruction. Concomitant small bowel resection was performed to remove the cyst. Herein we review seven reported cases of mesenteric benign tumor in pregnancy and explore the clinical features.     

小儿颈部巨大囊状淋巴管瘤的手术治疗

目的 探讨小儿颈部囊状淋巴管瘤的治疗方法,尤其是巨大囊状淋巴管瘤的手术治疗,评价其根治性及美容效果.方法 回顾性分析2001至2010年收治的25例小儿颈部巨大囊状淋巴管瘤的病例资料,瘤体长径均大于10 cm,其中24例行瘤体切除,总结术后并发症,评价治疗效果,从瘤体根治性及美容学要求讨论小儿颈部巨大囊状淋巴管瘤的治疗方法.结果 24例患儿的瘤体均完整切除,术后随访1～5年,颈部包块无复发,均实现根治.一期术后外观满意22例;2例瘢痕愈合,行二期修复整形术后外观满意.术后并发症包括淋巴液渗出5例;切口不良愈合2例,其中有1例并发术区感染;舌体水肿2例.结论 采用手术完整切除瘤体的方法,可以根治小儿颈部巨大囊状淋巴管瘤,并能达到外观满意的美容学要求.     

Cystic lymphangioma of the kidney: Diagnosis and management

INTRODUCTION: Cystic lymphangioma is a rare benign vascular tumor that may arise in various sites, revealed at any age. Abdominal locations represent less than 10% of the cases preferentially involving the mesentery. We report a case of primary lymphangioma arising from the kidney. PRESENTATION OF CASE: A 50-year-old woman was admitted for severe left lumbar pain. Abdominal ultrasonography and computed tomography revealed a 6×10 cm multiloculated cystic mass with hydronephrosis, extending along the left renal hilum. Surgical exploration revealed a retroperitoneal cystic tumor. Anatomopathologic examination concluded it to be a cystic lymphangioma. DISCUSSION: The cystic lymphangioma is a benign malformative tumor of the lymphatic system. Surgery is the best curative treatment with complete excision, the prognosis is excellent. CONCLUSION: Primary renal lymphangioma is exceedingly rare. Medical imaging has certain limits for the diagnosis which required histological confirmation. The treatment of choice is surgical.     

Omentum minus cystic lymphangioma: report of a case and a literature review

Lymphangiomas are rare benign tumors. In most cases, resection is necessary to obtain a precise histopathological analysis. There are capillary, cavernous and cystic lymphangiomas. The therapy of choice is a complete excision. Recurrence has been reported after incomplete resection. We present the case of a 45-year-old man with a lymphangioma of the omentum minus and -review the literature.     

Abdominal cystic lymphangiomas in children. Clinical, diagnostic and therapeutic aspects: apropos of 21 cases]

Twenty-one abdominal cystic lymphangiomas were observed in paediatric patients during a 15-year period, in 11 boys and 10 girls. Diagnosis was prenatal in 2 cases; the mean age of the other 19 children was 4.7 years (range: 3 months-8 years). Tumours were intraperitoneal in 16 cases and retroperitoneal in 5 cases. Symptoms were variable: abdominal pain in 15 cases, palpable tumour in 6 cases (excluding the two cases of prenatal diagnosis). Complications included obstruction in 7 cases (including 3 by volvulus), infection in 6 cases, and intracystic haemorrhage in 3 cases. Abdominal ultrasonography correctly established the diagnosis in all children. Surgical treatment included 20 complete resections and one incomplete resection, including 6 with bowel resections. With a follow-up ranging from 6 months to 10 years, one recurrence occurred and was successfully reoperated. Intraabdominal cystic lymphangioma in childhood is a rare tumour with a variable presentation. An accurate diagnosis can be established by abdominal ultrasound. Complete resection should be performed whenever possible.     

Bleomycin therapy for lymphangioma

Lymphangiomas are uncommun congenital malformations of the lymphatic system, that involve the skin and subcutaneous tissues. Of the several types of treatment, surgical excision has been the preferred. There is a high recurrence rate because lymphangiomas tend to infiltrate the surrounding tissues. The bleomycin is a cytotoxic antitumoral antibiotic, that causes modifications of DNA. It has been also successfully used in intralesional injection treatment of cystic hygromas and haemangiomas, based specifically on a high sclerosing effect on vascular endothelium. We report the cases of five patients, with congenital lymphangioma, localized on the leg, in cervical and latero-thoracal region, treated with repeated intralesional bleomycin injections. The treatment indication was given by the location of this lesions and the infiltration of the surrounding vital tissues, that made the complete surgical excision impossible. Intralesional injection of bleomycin into the lymphangiomas was given at a dose, not exceeding 0,5 mg/kg of body weight, at intervals of 4 weeks. Complete resolution (n = 4) or significant improvement (n = 1) occurred in all patients treated. No other treatment was needed. We didn't notice local or general adverse effects. With this method we set the purpose to treat effectively this congenital malformations, obviating the need for invasive primary surgery or systemic treatment regimens. Toward other methods, intralesional bleomycin injections have a minimal risk of side effects (ulceration, pulmonary fibrosis).     

Retroperitoneal lymphangioma

Lymphangiomas are rare cystic tumours that may present as cystic masses in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. Confusion with other cystic tumours of the retroperitoneum including those arising from the liver, kidney and pancreas is common. A case of a retroperitoneal cystic lymphangioma occurring in a 41-year-old woman raising interesting diagnostic and management issues is reported. The patient presented with vague abdominal pain and persistent nausea. Radiological imaging demonstrated a large multiloculated thin-walled cyst involving the upper retroperitoneum. Surgical resection was complete, revealing a benign cavernous lymphangioma. The patient made a complete recovery and was disease-free 30 months postoperatively.