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PubMed2004

Hereditary vascular anomalies: new insights into their pathogenesis.

Increased understanding of the mechanisms of angiogenesis and lymphangiogenesis has provided a glimpse at some of the molecules involved in the pathophysiology of hemangiomas and vascular malformations. This review focuses on recent advances in our understanding of the mechanisms of angiogenesis/lymphangiogenesis and the differentiation of arterial, venous, and lymphatic vessels. We integrate this knowledge with new data obtained from genetic studies in humans, which have revealed a number of heretofore-unsuspected candidates involved in the development of familial vascular anomalies. We present a common infantile vascular tumor, hemangioma, and then focus on hereditary familial vascular and lymphatic malformations. We also summarize transgenic mouse models for some of these malformations. It seems reasonable to believe that novel therapeutic strategies will soon emerge for the treatment of hemangiomas and vascular malformations.

Animals↗
PubMed1993

Periportal halo: a CT sign of liver disease.

Periportal halos are defined as circumferential zones of decreased attenuation identified around the peripheral or subsegmental portal venous branches on contrast-enhanced computed tomography (CT). These halos probably represent fluid or dilated lymphatics in the loose areolar zone around the portal triad structures. While this CT finding is nonspecific, it is abnormal and should prompt close scrutiny of the liver in search of an underlying etiology. Periportal halos which may be due to blood are commonly seen in patients with liver trauma. Periportal edema may cause this sign in patients with congestive heart failure and secondary liver congesion, hepatitis, or enlarged lymph nodes and tumors in the porta hepatis which obstruct lymphatic drainage. This CT sign has also been observed in liver transplants (probably secondary to disruption and engorgement of lymphatic channels) and in recipients of bone marrow transplants who might develop liver edema from microvenous occlusive disease. While the precise pathophysiologic basis of periportal tracking has not been proven, it represents a potentially important CT sign of occult liver disease.

Humans↗
PubMed1992

[A case of mediastinal lymphatic cyst possibly originating from the right thoracic duct].

A 33-year-old male was admitted to our hospital because of an abnormal shadow in the right superior mediastinum on chest X-ray, which was first noted about half a year before his admission. Several diagnostic procedures, including fiber bronchoscopy, CT scan and MRI; however, no final diagnosis could be made. Thoracotomy was performed with the presumed diagnosis of benign mediastinal cystic tumor. Entry of the right thoracic duct into the cyst was observed at operation. Histological examination revealed that the cyst was lined by mono-layered cuboidal epithelial cells with lymphocytic infiltration in the wall of the cyst. The mono-layered cuboidal epithelium of the thoracic duct gradually changed to the epithelium of the cyst. Twenty ml of colorless transparent fluid was aspirated from the cyst. Analysis of aspirated fluid revealed low concentration of several substances except LDH compared to the values of the previously reported normal standards and other reported cases. From these findings, the cyst was diagnosed as mediastinal lymphatic cyst. Entry of the thoracic lymphatic duct into the mediastinal lymphatic cyst is a rare occurrence, and it is of interest to speculate on its relationship to the origin of the lymphatic cyst.

Adult↗
PubMed1995

Prognostic significance of peritumoural blood and lymphatic vessel invasion by tumour cells in T4 non-small cell lung cancer following induction therapy.

We investigated the impact of new biological prognostic factors is in 28 patients receiving a median of two courses of cisplatin-based chemotherapy with (n = 14) or without (n = 14) radiation and operation for stage IIIB (T4) non-small cell lung cancer (NSCLC). After induction therapy, 5 patients had a complete and 21 a partial response; 2 had a stable disease. A complete resection was made in 26 patients (93%). Five patients (18%) had their primary tumour and involved vestiges completely sterilized. In the remaining 23, the majority of the tumours showed abnormalities in the p53 gene expression (56%), harboured proliferating cells (91%) and induced angiogenesis (91%). Peritumoural blood and lymphatic vessel invasion (PBLVI) by tumour emboli was observed in 6 tumours. With a median follow-up of 25 months, overall 3-year survival was 48%; disease-free survival (DFS) has not been reached yet. The only significant factor influencing DFS in multivariate analysis was PBLVI by tumour cells; PBLVI-positive patients had a significantly higher likelihood ratio (P = 0.000001) of developing metastasis than their PBLVI-negative counterparts. This study documents the prognostic implication of PBLVI by tumour cells in T4 NSCLC.

Adult↗
PubMed1993

Hemangiomas and vascular malformations of infancy and childhood.

The vast majority of vascular anomalies of infancy and childhood can be biologically classified as hemangioma or vascular malformation. Hemangiomas are benign neoplasms that proliferate rapidly in infancy only to involute in early childhood. The majority of hemangiomas do not need treatment. Pharmacologic therapy, with corticosteroids or interferon-alpha-2a, is indicated for lesions that threaten vital function or are grossly deforming. Vascular malformations are not tumors, but rather vessel abnormalities due to errors of vascular morphogenesis. They derive from embryonal capillary, venous, arterial, or lymphatic channels, or combinations thereof. The appearance, clinical behavior, and therapy differ based on their channel types. All too often, unfortunately, children with vascular anomalies are shuffled from physician-to-physician because a single practitioner, even a specialist, does not have sufficient knowledge to properly treat the vascular lesion. The authors recommend that every major referral center have a multidisciplinary "Vascular Anomalies Team." We also endorse a biologic classification of vascular lesions to facilitate interspecialty communication regarding diagnosis, natural history, and therapy.

Blood Vessels↗
PubMed1980

Defective DNA repair and increased lethality in ataxia telangiectasia cells exposed to 4-nitroquinoline-1-oxide.

Ataxia telangiectasia (AT) is a rare autosomal recessive disorder in man characterized by progressive loss of muscular coordination (due to neurodegeneration) and permanent dilation of the small blood vessels of the eyes and skin. AT patients also have repeated sinopulmonary infections, immune defects associated with thymus underdevelopment, and abnormal endocrine functions. Affected patients are at high risk of developing malignancy, particularly lymphomas and lymphatic leukaemias, and there are clinical indications that AT patients are hypersensitive to conventional radiotherapy administered for treatment of malignancy. Cultured diploid fibroblasts from AT donors are consistently hypersensitive to ionizing radiation, apparently due to defective enzymatic repair of radiogenic DNA damage. We have determined the survival responses and DNA repair abilities of AT cells exposed to 4-nitroquinoline-1-oxide (4NQO), a chemical carcinogen whose DNA-damaging properties partially mimic those of ionizing radiation. We report here that certain AT cell strains show hypersensitivity to inactivation by 4NQO and defective repair of 4NQO-induced adducts in DNA.

4-Nitroquinoline-1-oxide↗
PubMed2001

VEGF receptor signal transduction.

The family of vascular endothelial growth factors (VEGFs) currently includes VEGF-A, -B, -C, -D, -E, and placenta growth factor (PlGF). Several of these factors, notably VEGF-A, exist as different isoforms, which appear to have unique biological functions. The VEGF family proteins bind in a distinct pattern to three structurally related receptor tyrosine kinases, denoted VEGF receptor-1, -2, and -3. Neuropilins, heparan-sulfated proteoglycans, cadherins, and integrin alphavbeta3 serve as coreceptors for certain but not all VEGF proteins. Moreover, the angiogenic response to VEGF varies between different organs and is dependent on the genetic background of the animal. Inactivation of the genes for VEGF-A and VEGF receptor-2 leads to embryonal death due to the lack of endothelial cells. Inactivation of the gene encoding VEGF receptor-1 leads to an increased number of endothelial cells, which obstruct the vessel lumen. Inactivation of VEGF receptor-3 leads to abnormally organized vessels and cardiac failure. Although VEGF receptor-3 normally is expressed only on lymphatic endothelial cells, it is up-regulated on vascular as well as nonvascular tumors and appears to be involved in the regulation of angiogenesis. A large body of data, such as those on gene inactivation, indicate that VEGF receptor-1 exerts a negative regulatory effect on VEGF receptor-2, at least during embryogenesis. Recent data imply a positive regulatory role for VEGF receptor-1 in pathological angiogenesis. The VEGF proteins are in general poor mitogens, but binding of VEGF-A to VEGF receptor-2 leads to survival, migration, and differentiation of endothelial cells and mediation of vascular permeability. This review outlines the current knowledge about the signal transduction properties of VEGF receptors, with focus on VEGF receptor-2.

Animals↗
PubMed1983

A possible route for lymphocyte migration into diseased tissues.

Vessels within the lymphocytic infiltrate in 161 examples of 24 different diseases have been studied using light and electron microscopy and histochemistry. Hitherto undescribed venules have been identified within lymphocyte aggregates which exhibit a structure, ultrastructure and cytochemistry previously believed to be restricted to the high endothelial venules of lymphoid tissues. The significance of these findings is discussed within the context of lymphocyte migration into abnormal tissue.

Cell Movement↗
PubMed1989

Angioimmunoblastic lymphadenopathy--an update.

Angioimmunoblastic lymphadenopathy (AILD) is a relatively rare serious disorder of the lymphatic system with various organ manifestations and a significant immunological background. It probably represents an abnormal reaction to different chemical and biological antigens. The etiopathogenesis of the disease is still poorly understood. The authors report on findings in a series of five patients with AILD. Stress is laid on polymorphous symptomatology, unpredictable course and on resulting diagnostic as well as therapeutic difficulties in dealing with the disease.

Adult↗
PubMed1988

[Practical importance of capillary microscopy].

Vital capillaroscopy has been successfully used for many years to study the microcirculation of human skin capillaries. The presence of typical capillary abnormalities is helpful in distinguishing scleroderma from other connective tissue diseases. Application of a local cold exposure test to finger nailfold capillary microscopy reveals the functional difference between normal subjects and patients with Raynaud's syndrome on the level of the microcirculation. Transcutaneous intravital fluorescence videomicroscopy may be helpful in the sometimes difficult differential diagnosis of lower limb edema. Microlymphangiography offers a new approach to depiction of the intravital anatomy of human lymphatic skin capillaries. Further the technique of intravital fluorescence videomicroscopy provides a noninvasive method of studying morphologic and functional abnormalities in diabetic microangiopathy.

Capillaries↗
PubMed2006

Cutaneous lymphatic malformations in disappearing bone (Gorham-Stout) disease: a novel clue to the pathogenesis of a rare syndrome.

BACKGROUND: Gorham-Stout disease is an unusual, progressive syndrome of unknown etiology characterized by mono- or polyostotic osteolysis most often affecting children and young adults. The onset is insidious and the disease progresses to extensive and potentially disabling osteolysis often unresponsive to therapeutic intervention. Although bone and soft tissue lesions are the most frequent manifestations of Gorham-Stout disease, skin lesions can occur and may provide a clue to the pathogenesis of this rare syndrome. OBJECTIVE: Our aim was to describe characteristics of vascular skin lesions of this rare condition using magnetic resonance imaging and histomorphological analysis. METHODS: The case of a 36-year-old man with Gorham-Stout disease of the left leg and foot is reported. RESULTS: This case was remarkable for its prominent lymphatic vascular malformations involving the skin and soft tissues adjacent to the diseased bone-a previously undescribed abnormality, which preceded osteolysis for several years. Magnetic resonance imaging played a key role in defining the extent of disease in skin and soft tissues. LIMITATIONS: It is difficult to assess the true incidence of hemangiomatosis in the earlier reports on Gorham-Stout disease in which hemangiomatous cutaneous lesions were mentioned but not described or illustrated. CONCLUSION: A vascular process with angiomatous histological features is considered to be the pathological hallmark of Gorham-Stout disease, but the specific type of this vascular process is still under debate. Our report highlights a lymphatic malformative nature of Gorham-Stout disease, thereby contributing to a better understanding and characterization of this rare disease entity.

Adult↗
PubMed1976

Alkaline phosphatase in the thymus.

(1) Fetal thymuses, organs from patients who died from diseases that are not clinically known to be associated with concomitant lymphoid tissue involvement, as well as thymuses from patients dying from diseases which effect the lymphatic complex of the body, one way or another, have been investigated for their alkaline phosphatase activity, using Gomori technique and applying four different phosphate esters as substrates. (2) Three substrates (beta-glycerophophate, riboflavin 5-phosphate and adenosine triphosphate) showed essentially the same pattern of activity in which the cortex and Hassall's corpuscles were reactive, while the medulla was negative. A reversal of this pattern was demonstrated with 5-monophosphoric acid. (3) Before the age of 32-36 weeks of intra-uterine life there is no alkaline phosphatase activity in the thymus; therafter, the enzyme begins to make its first appearance. (4) There is a definite increase in the intensity of the reaction with advance of intra-uterine life. This increase in phosphatase content is continued postnatally, to reach its maximum at about the age of 10 years: after that, the enzyme activity gradually subsides. (5) There is a tremendous augmentation of phosphatase activity in the case of disease which are known to affect the lymphoid complex. (6) The phosphatase activity of the thymus has been discussed in relation to the prevailing concepts about the function of the thymus, with special emphasis on a possible association with 'lymphocyte-stimulating factor' production and/or secretion.

Adolescent↗
PubMed1991

[Hygroma colli--sonographic indication of fetal chromosome abnormality. A case report].

Transvaginal sonography has been developed as an useful tool to recognize and define structural defects in the first trimester fetus. Fetal cystic hygromas as an manifestation of malformation of the lymphatic system are often the only sign of an severe fetal anomaly. In 70-80% of cases cystic hygroma is associated with a Turner-Syndrome (45,XO). We report a case in which transvaginal sonography has been the only instrumental in the prenatal diagnosis of a nuchal cystic hygroma. Based on this sonographic finding the pregnancy has been terminated. We point to the value of early and subtle ultrasound examination as an excellent method in finding hints for chromosome abnormalities.

Abortion, Induced↗