Tumeurs médiastinales d'origine embryonnaire

Mediastinal tumours of embryonic origin refer to tumours resulting from a disorder of the embryonic development, or to tumours deriving from germinal cells and of which the primitive localization is the mediastinum. Such tumours are constituted by tissues similar to those that successively develop from the embryonic to the adult stage. These benign or malignant lesions are classified according to their histogenesis and the available therapeutic means. Germinal tumours are constituted by tissues similar to those that succeed one another during the development, by differentiation of primitive laminas and extraembryonic laminas. In terms of therapy, seminomas have to be distinguished from non seminomatous germinal tumours, and from teratomas. Mediastinal cysts are malformations constituted by tissues included normally into the adult thorax. They are distributed in five classes, according to their structure: bronchogenic and paraoesophageal cysts, pleuropericardic cysts, cystic lymphangiomas, benign haemangiomas, and meningeal cysts.     

Neurogenic tumor of small intestine, report of a case with review of literature

A case of neurofibroma of small intestine presented with massive intra-abdominal bleeding was reported. There was a total of 51 cases of neurogenic tumor of small intestine including the present case to be collected from Japanese literature. 33 cases were not associated with Von Recklinghausen's disease. Discussion was given according to their sex and age distribution, symptom and sign, type of development, tendency of malignant degeneration, and treatment.     

Mediastino-hepato-renal cystic lymphangiomas—diagnostic and surgical considerations

Cystic lymphangiomas or hygromas are rare benign vascular tumours, caused by congenital malformation of the lymphatic vessels. It appears as a progressive swelling in the head or neck of children during 2-5 years of life, yet rarely seen in the mediastinum or abdomen. Symptomatic mediastinal cystic lymphangiomas provide symptoms such as chest pain, breathlessness, cough, and dysphagia, making it difficult to differentiate from other mediastinal tumours. The tumour can become larger due to infections, inflammations, obstructions and bleedings. Chest X-ray, ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) provide helpful information but the diagnosis appears merely after surgical resection and histological examination. Only a few cases have been reported. Hence, we report the first case of a mediastinal and asymptomatic renal and multiple hepatic cystic lymphangiomas in a 71-year-old male with respiratory symptoms and sever reduction in lung capacity. The symptoms regressed fully after surgical excision and lung diffusions capacity increased significantly.     

Hepatic haemangiomas: possible association with female sex hormones

BACKGROUND AND AIMS: The association of hepatic haemangiomas with female sex hormones is not entirely clear. We prospectively evaluated the impact of female sex hormones on the natural history of liver haemangiomas. METHODS: We followed 94 women with 181 haemangiomas diagnosed by ultrasound for a period of1-17 years (mean 7.3 (5.5) years). The location, number, size, and ultrasonographic pattern of the lesions were evaluated. Patients were also evaluated by questionnaire for gynaecological and reproductive history. We compared the change in number and size of haemangiomas in patients who received or did not receive exogenous hormonal treatment. RESULTS: Age at first period was inversely associated with the size of haemangiomas (r = 0.181, p = 0.015) while age at menopause was positively correlated with the number of haemangiomas detected at first ultrasound (r = 0.542, p<0.0001). During follow up, no change in the ultrasonographic pattern or number of haemangiomas was observed. An increase in the size of the lesions was demonstrated in 5/22 (22.7%) hormone therapy exposed patients compared with 7/72 (9.7%) controls. Three variables (ultrasonographic pattern, number of haemangiomas, and hormone therapy) predicted whether or not a given haemangioma would increase in size. A hypoechoic pattern increased the risk of progression while a hyperechoic pattern decreases that risk (p = 0.003). The number of haemangiomas was inversely associated with the likelihood of progression (p = 0.006) and hormone therapy increased the risk of haemangioma enlargement (p = 0.05). CONCLUSIONS: Hepatic haemangiomas seem to be influenced by both endogenous and exogenous female sex hormones although significant enlargement occurs only in a minority of patients. Consequently, routine liver ultrasound follow up in women with hepatic haemangiomas receiving hormone therapy appears appropriate.     

Retrocrural cystic lymphangioma

Pure posterior mediastinal cystic lymphangiomas are very rare and have seldom been documented. This is the report of such a case, including the ultrasonographic and computed tomographic manifestations of the lesion, the first documentation of such manifestations in the literature.     

Vascular anomalies of the gastrointestinal tract--an unusual case of multiple intra-abdominal hemangiomas

A case of multiple abdominal haemangiomas (mesentery, intestine, liver) in a 19 year-old patient is described. The anomalies, at first incidentally found during appendectomy, were confirmed by angiographies and vascular surgery. Ascites and bleeding esophageal varices developed in further course. A short review of literature and classifications is given.     

Natural history of hepatic haemangiomas: clinical and ultrasound study.

Hepatic haemangiomas are the most common benign tumours of the liver and commonly present as incidental findings on sonographic examination of the abdomen. Since little is known of the natural course of these tumours, we performed a clinical and sonographic follow up of 123 haemangioma patients. Our prospective study investigated clinical and sonographic findings in 158 haemangiomas for periods of 12 to 60 months. Ninety nine haemangiomas measured less than 2 cm and had an echogenic pattern; 40 were between 2 cm and 5 cm with a mainly echogenic structure; 19 measured greater than 5 cm and showed a mixed echo pattern. At the first examination only eight patients, all with giant haemangiomas, presented symptoms which could be attributed to the tumour. During follow up only one haemangioma changed in shape and size. One patient who was symptom free at the first examination experienced right upper abdominal quadrant pain during follow up. No deterioration occurred in any of the patients with symptoms at the first examination, and all had a satisfactory quality of life. No complications arose during the follow up period. This study shows that in adults haemangiomas remain stable in size and echo patterns rarely change. Only haemangiomas greater than 5 cm may cause symptoms. Prolonged clinical and sonographic follow up of small and medium sized haemangiomas is not warranted.     

Minimally invasive excision of multiple cystic lymphangiomas of the mediastinum: a case report

Mediastinal lymphangioma is one of the rarest benign mediastinal masses. We report the successful minimally invasive surgical treatment of multiple cystic lymphangiomas in a middle-aged man with a familial history of lung cancer. This case report describes cystic lymphangioma as a rare differential diagnosis for a mediastinal mass in an adult.     

Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature

Lymphangiomas are rare benign cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and approximately 186 cases have been reported. They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors, including those arising from the liver, kidney and pancreas. This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient. The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention, abdominal pain, anorexia, fever, nausea and diarrhea. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis. Surgical removal of the cyst was accomplished without incident. A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.     

Endoscopic submucosal dissection of esophageal lymphangioma: a case report with a review of the literature

Esophageal lymphangioma is a very rare disease. We report a case of esophageal lymphangioma successfully treated with endoscopic submucosal dissection (ESD), which yielded definitive histological diagnosis and symptom relief. ESD offers a better option for definitive diagnosis as well as complete resection of large esophageal lymphangiomas with flat configuration.     

Cavernous lymphangioma of the duodenum: case report and review of the literature

Lymphangiomas of the gastrointestinal tract are extremely rare benign tumors. There have been 39 cases of solitary intestinal lymphangiomas reported, 8 of which were found in the duodenum; only 3 of these were seen radiographically. A case of cavernous lymphangioma of the duodenum is presented, the roentgenographic and pathologic findings are described, and the literature is reviewed.     

Lymphangiome kystique rétropéritonéal

The cystic lymphangioma is a vascular tumor that arises at the expense of lymphatic vessels. It is a rare and benign dysplasia that occurs mainly in children. Adult forms are rare. Its expression is mainly cervical or axillary, with a few locations in the abdominal cavity. We report the case of a patient who presented with cystic lymphangiomas located in the retroperitoneal region. Various clinical aspects, diagnosis and treatment were studied.     

A case of hepatocellular carcinoma arising within large focal nodular hyperplasia with review of the literature

Focal nodular hyperplasia (FNH) is a relatively rare benign hepatic tumor, usually presenting as a solitary lesion; however, multiple localizations have also been described. The association of FNH with other hepatic lesions, such as adenomas and haemangiomas has been reported by various authors. We herein report a case of a hepatocellular carcinoma arising within a large focal nodular hyperplasia, in a young female patient.     

6例心脏淋巴管瘤的诊断和手术治疗经验

目的总结心脏淋巴管瘤的诊断及手术治疗情况.方法6例心脏淋巴管瘤患者,首发症状包括心悸、气短及心力衰竭表现.术前诊断包括左心房多发粘液瘤、左心室心肌内海绵状血管瘤、左心室纤维瘤、左心室占位性病变性质不明、左心室假性室壁瘤.术中发现1例仅表现为二尖瓣前乳头肌的肥厚,3例为左心室腔内外生性肿物,l例为左心室壁外肿物,1例为左心房内肿物.处理方法为1例仅取活检病理组织,其余各例切除各肿瘤,1例同期二尖瓣替换术.结果本组6例术前均未能明确诊断,术后病理诊断为淋巴管瘤.无手术早期死亡.随访时间平均17.2个月,超声心动图复查未发现肿瘤复发,患者心功能(NYHA)为I～Ⅱ级.结论心脏淋巴管瘤是罕见的心脏肿瘤,它的临临床特征是非特异性的,手术前常难准确诊断,决定淋巴管瘤性质的可靠依据是病理特征,术中探查对诊断有一定帮助.手术治疗效果良好.     

A case of hepatocellular carcinoma arising within large focal nodular hyperplasia with review of the literature

INTRODUCTIONFocal nodular hyperplasia(FNH)is a relatively rare benign liver tumor,often asymptomatic and discovered incidentally[1,2].It occurs in both men and women,but shows a predilection for young women.FNH presents as a solitary lesion in70%of the ca…     

Gallbladder lymphangioma: a case report and review of the literature

Lymphangiomas are benign neoplasms usually occurring in childhood and located in the head and neck. Intraabdominal lymphangiomas account for less than 5% of cases. The involvement of the gallbladder is rare. We report a case of a 29-year-old woman who presented with right upper quadrant pain that had persisted for 6 months. Imaging with ultrasound and magnetic resonance imaging (MRI) revealed a multiseptated lesion surrounding the gallbladder. The patient underwent an exploratory laporatomy, and the mass was resected en bloc with the gallbladder. Histological evaluation of the cystic mass revealed findings consistent with lymphangioma. The prognosis is generally good after complete surgical excision, as was the case for our patient.     

Diffuse cavernous haemangioma complicated with regional osteoporosis

Capillary and cavernous haemangiomas are the most common benign soft tissue tumours of infancy. Cavernous haemangiomas have larger vascular channels and frequently exist in deep dermal tissues. Many haemangiomas require no special treatment. Development of complications such as cardiac failure, malignant change, ulceration and infection are indications for intervention. In this report we present an 11-year-old girl with a pathological fracture after a minimal trauma that might be a result of secondary regional osteoporosis due to diffuse cavernous haemangioma.     

Risk Factors and Outcome for Massive Intra-Abdominal Bleeding Among Patients With Infected Necrotizing Pancreatitis

The incidence of acute bleeding is reported to be 13.5% in patients with acute necrotizing pancreatitis. However, of all the bleeding events, intra-abdominal bleeding was less studied in the literature and its risk factors have not been well defined yet. The purpose of the present study was to investigate the risk factors for massive intra-abdominal bleeding among the patients with infected necrotizing pancreatitis and assessed the outcome of these patients.Both univariate and multivariate logistic regression models were applied for evaluating risk factors for intra-abdominal bleeding using 33 indices, including age, sex, etiology of acute pancreatitis (AP), APACHE II score, etc. Outcome assessments such as mortality, hospital and intensive care unit (ICU) durations, and cost were also compared between patients with or without intra-abdominal bleeding.Acute kidney injury (AKI) (odds ratio [OR]: 7.54, 95% confidence interval [CI]: 2.53–22.52, P < 0.001) and number of operation (OR: 8.84, 95% CI: 2.01–38.86, P = 0.004) were 2 predictors for massive intra-abdominal bleeding in the patients with infected necrotizing pancreatitis. In addition, AP patients with intra-abdominal bleeding also showed significantly higher mortality rate, prolonged hospital and ICU durations, more complications and invasive treatments, as well as increased cost.Our study revealed that AKI and multiple operations were 2 critical factors increasing the risk of intra-abdominal bleeding among patients with infected necrotizing pancreatitis. Additionally, massive intra-abdominal bleeding was also associated with poor prognosis.     

Intra-abdominal co-infection with Mycobacterium bovis and Actinomyces in an AIDS patient: the first reported case and review

The Mycobacterium tuberculous complex and Actinomyces can both cause indolent intra-abdominal infections that result in hard mass lesion(s), oftentimes fixed to underlying tissue and involving the right ileocecal fossa region. Although Actinomyces can present as a co-pathogen when anatomic barriers are compromised, its intra-abdominal co-infection with Mycobacterium bovis has not previously been reported. We present the first reported case of intra-abdominal co-infection with Actinomyces and Mycobacterium bovis, a member of the Mycobacterium tuberculosis complex (MTB), in an AIDS patient and provide a discussion of the pathogenesis and a review of these infections, both individually and together.     

Cavernous haemangioma of the right atrium

Cardiac haemangiomas are rare benign primitive tumors. We are reporting the case of a 67-year-old woman presenting with a haemangioma of the right atrium. This tumor was discovered by echocardiography because of cerebral strokes. The magnetic resonance imaging determined the characteristics of the tumor. It was completely resected through a right atrial approach. This was a round mobile mass, pediculed and implanted at the inferior area of the interatrial septum. The histopathological analysis revealed a cavernous haemangioma.