Primary mesenteric angiosarcoma in a child with associated lymphangiectasia: a case report.

Primary mesenteric angiosarcoma in a child with associated lymphangiectasia: a case report.
Pediatr Dev Pathol. 2008 Nov-Dec

Castro EC, Galambos C, Shaw PH, Ranganathan S.
Department of Pediatric Pathology and Division of Pediatric Hematology/Oncology, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.

Angiosarcomas are rare tumors in children, usually occurring in soft tissue and liver. By contrast, angiosarcoma in adults usually occurs in the extremities in conjunction with lymphedema. Mesenteric angiosarcoma has only rarely been reported. When angiosarcomas arise in this location, they usually represent a 2nd malignancy following Hodgkin's lymphoma. We report a child who presented to the emergency room with an acute abdomen and underwent emergency surgery for a mesenteric angiosarcoma with associated lymphangiectasia of the bowel and mesentery. A brief review of the literature and the nomenclature of these unusual tumors are discussed.

Pediatric and Developmental Pathology

Evidence for Medium Chain Triglycerides in the Treatment of Primary Intestinal Lymphangiectasia.

Evidence for Medium Chain Triglycerides in the Treatment of Primary Intestinal Lymphangiectasia.

Eur J Pediatr Surg. 2009 May 15
Desai AP, Guvenc BH, Carachi R.
1Department of Pediatric Surgery, King's College Hospital, London.

Primary intestinal lymphangiectasia is an uncommon congenital anomaly. It is an intrinsic abnormality of the intestinal lymphatics system. Over the years, various treatment options such as diuretics, albumin transfusions and a medium chain triglycerides (MCT) diet as well as surgical options such as resection of isolated segments and peritoneal-venous shunts have been used. An MCT diet, which is a low fat, high protein diet, is increasingly used in the management of this anomaly. AIM: The aim was to review the evidence for medium chain triglycerides as a therapeutic option in patients with primary intestinal lymphangiectasia.

MATERIAL AND METHODS: A literature search was performed and individual case details were extracted. We found 55 cases, of which 3 were from our own institute. The cases were divided in 2 groups: Group A (n=27) consisted of patients treated with MCT, and Group B (n=28) consisted patients not treated with MCT. Cases were analysed for symptomatic response to MCT as well as mortality.

RESULTS: 17 of 27 cases (63%) treated with MCT had complete resolution of symptoms while only 10 of 28 (35.7%) patients in group B showed complete resolution. Mortality for Group A was 1 out of 27 (3.7%), while mortality in group B was 5 of 28 (17.85%) patients.

CONCLUSION: We conclude that, although an MCT diet is not completely curative in all cases, it does improve the symptoms of primary intestinal lymphangiectasia and reduces mortality. Hence it is a valid option in the paediatric age group.

PMID: 19449286 [PubMed - as supplied by publisher]