To evaluate the expression of D2-40 in normal lymphatic endothelium and vascular tumours or tumour-like lesions of the skin and soft tissue. D2-40 is a novel monoclonal antibody to a Mr 40 000 O-linked sialoglycoprotein that reacts with a fixation-resistant epitope in lymphatic endothelium.
Formalin-fixed paraffin-embedded sections from 30 normal tissue samples, including skin, soft tissue, stomach, and colon, and 84 vascular tumours or vascular tumour-like lesions were immunostained with monoclonal antibodies to D2-40 and CD31. Normal lymphatic endothelial cells in all normal tissues expressed D2-40. Its positive staining delineated flattened channels or open spaces lined by a single layer of endothelial cells whose lumena were sometimes filled with lymphocytes. Ten of 10 cases of lymphangioma, nine of 10 Kaposi's sarcomas (KSs), one of five spindle cell haemangiomas, one of one reactive angioenodotheliomatosis, one of one vascular transformation of lymph node sinuses, three of three Dabska tumours, one of 10 epithelioid haemangioendotheliomas (HEs) and seven of 15 angiosarcomas were positive for D2-40. Positively staining angiosarcomas were characterized by epithelioid or papillary endothelial cells. Twenty-two non-spindle cell haemangiomas, one retiform HE and one Kaposiform HE, and five glomus tumours were negative for D2-40. In comparison, CD31 was expressed in five of 10 lymphangiomas, nine of 10 KSs, 27 of 27 haemangiomas, three of three Dabska tumours, 10 of 10 epithelioid HEs, 15 of 15 angiosarcomas and one of one each of retiform HE, Kaposiform HE, reactive angioendotheliomatosis, and vascular transformation of node sinuses. Five glomus tumours were negative for CD31.
The monoclonal antibody D2-40 is a highly sensitive and specific marker of lymphatic endothelium in normal tissue and a subset of vascular lesions, including KS, Dabska tumour, and lymphangioma. The findings support the concept that these tumours show at least partial lymphatic endothelial differentiation. Subsets of angiosarcomas and HEs show both vascular and lymphatic endothelial differentiation. D2-40 can be used in a panel of markers to classify vascular tumours. There is no requirement for epitope retrieval. This novel monoclonal antibody also has the potential for increasing the accuracy of detection of lymphatic invasion in primary tumours and could be widely applied for this purpose in surgical pathology.
"No clear clinical distinction exists between these diseases, and a definitive diagnosis of CPL can be made only by pathologic examination (3, 11, 14). CPL shows positive staining for D2-40, vimentin, CD31, CD34, and factor VIII-related antigen (15). The patient in the present case was immunohistochemically positive for D2-40 and CD34, which was consistent with a diagnosis of CPL. "
[Show abstract][Hide abstract] ABSTRACT: Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.
Journal of Korean medical science 04/2014; 29(4):609-13. DOI:10.3346/jkms.2014.29.4.609 · 1.27 Impact Factor
"Lymphatics are thin-walled, endothelium-lined channels devoid of red blood cells and a smooth muscle layer, but it is very difficult to distinguish morphologically normal lymphatic channels from venules or capillaries. Currently, the monoclonal antibody immunohistochemical stain, D2-40, is considered to be a highly sensitive and specific marker of the lymphatic endothelium . To the best of our knowledge, our case report is the first to identify lymphatic endothelium at an abnormal paravertebral soft tissue mass and the duodenum in Gorham's disease. "
[Show abstract][Hide abstract] ABSTRACT: We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.
"These tumors primarily affect the skin of children (5), however, in recent years it has been demonstrated that such a lesion may be present in a wider age range and in more organs than originally described (6). The literature describes a category of ‘similar lesions’ (7), which have two distinctive qualities, namely papillary growth and a lymphatic immunophenotype with positivity for D2-40 (8) and VEGFR-3 (7,9). The pathogenesis of such neoplasms remains obscure. "
[Show abstract][Hide abstract] ABSTRACT: Primary vascular tumors of the lymph nodes other than Kaposi's sarcoma are rare. The present study describes the case of a primary hemangioendothelioma in a cervical lymph node in a patient treated for a carcinoma of the large bowel. Microscopically, the structure of the lymph node was completely substituted by tumoral proliferation, with an architectural pattern consisting of anastomosing vascular channels, a number of which contained papillary projections or a tuft-like structure. This study demonstrates the potential diagnostic errors and hypothesizes the pathogenesis of this type of lesion; a vascular endothelial growth factor (VEGF)/VEGF receptor-3 (VEGFR-3) autocrine loop may be activated.
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