A Case of Lymphangiomatosis With Infected Lymphangiomas Effectively Treated by Thoracoscopic Debridement and Drainage.

A 40-year-old woman with lymphangiomatosis also had an intrathoracic lymphangioma infection. Since the age of 8 years, the patient had undergone repeated abdominal and mediastinal cyst surgeries and had received a diagnosis of lymphangiomatosis. At this time, she showed a high fever of 38.5°C. Cultures of both blood and fluid aspirated from the cyst were positive for Staphylococcus aureus. Chest CT imaging revealed an enlarged right-sided mediastinal cystic lymphangioma with new septa in it. A chest tube was inserted into the cyst to remove effusion. The patient was then started with the antibacterial drug cefazolin at 3 g/d. But effective drainage was difficult because of the high viscosity of the effusion and septa working as barriers. We removed these components with endoscopic surgical instruments and via a pulsed-lavage system under general anesthesia. Postoperative CT images showed reexpanded lung structure and reduced cyst size. The patient has taken a favorable course for 2 years.

Peripelvic Lymphatic Malformation Presenting With Gross Hematuria and Renal Colic: A Case Report.

Renal lymphangectasia is a benign malformation of the lymphatics which can be perirenal or peripelvic in location. Diagnosis is made on imaging including ultrasound, computed tomography , or MRI. We present an unusual case of renal lymphangectasia presenting with gross hematuria and flank pain diagnosed on computed tomography.

Telangiectatic Carcinoma - Like Lymphangioma Circumscriptum. A Rare Form of Cutaneous Metastasis of Breast Carcinoma: Case Report.

INTRODUCTION: The Breast cancer is the most common malignancy in middle-aged women and that causes skin metastasis. Skin metastasis in internal cancer cases is a very rare condition and may be difficult to diagnose and have poor prognostic marker. Cutaneous metastasis of breast carcinoma is mostly seen as direct invasion and/or local infiltration. However, in addition to the well-known types, cutaneous metastases may mimic many benign skin lesions and therefore may be difficult to diagnose. CASE REPORT: In this article we present a 36-year-old woman with telangiectatic carcinoma-like lymphangioma circumscriptum, a rare form of cutaneous metastasis skin metastases. It can be the first sign of internal malignancies, so early diagnosis is very important at this stage. CONCLUSION: Therefore, solitary lesions or benign dermatoses seen in the skin and not associated with specific disease should be considered as tumor metastasis especially in female patients with a history of breast cancer and differential diagnosis must be made.

A novel case of lymphangiomatosis in HIV.

Diffuse lymphangiomatosis is a rare disorder characterized by abnormal proliferation of lymphatic channels. It can involve just one organ or multiple organs, such as liver, spleen, lungs, and bone. This disorder generally presents in children and young adults, but in rare cases, patients first present with symptoms in adulthood. Here, we describe a 48-year-old HIV-positive man who presented with shortness of breath. Computed tomography scan revealed a large right-sided pleural effusion and a heterogeneously enhancing liver. Thoracentesis demonstrated a chylous effusion and subsequent liver biopsy revealed a proliferation of dilated lymphatics to establish a diagnosis of lymphangiomatosis.

Percutaneous transoral clivoplasty and upper cervical vertebroplasties for multifocal skeletal lymphangiomatosis resulting in complete resolution of pain: case report.

Lymphangiomatosis is a rare congenital disorder that results in multiorgan system lymphatic invasion. Symptoms due to axial skeletal involvement can range from chronic bone pain to severe deformity resulting in radiculopathy, myelopathy, and even paralysis. The authors present a case of lymphangiomatosis of the clivus, C-1, and C-2, resulting in chronic pain. The patient was successfully treated with percutaneous transoral clivoplasty and vertebroplasty, without disease progression or return of symptoms at 2 years.

Lymphangiomatosis: Two cases with unique presentations, salience of nomenclature, and diagnosis.

We are presenting two unique cases of lymphangiomatosis without visceral and bony involvement and critically discussing the nomenclature used in the extant literature. The first case was a 12-year-old boy with ill-defined mass on the right cheek extending into the ipsilateral orbit leading to conjunctival lesion. The second case was of a 14-week-old infant showing diffuse swelling on nape of the neck. In addition there were raised patches on dorsal aspects of bilateral hands and feet. The biopsies from all the lesions showed similar histopathological features consistent with lymphangiomatosis. We propose that the term lymphangiomatosis should be used only in cases with histological features of lymphangiomatosis. The term should not be used in cases of multiple lymphangiomas. We conclude that the lesions clinically diagnosed as lymphangioma may turn out to be lymphangiomatosis. Extensive lymphangiomatosis without visceral or bony involvement may lead to intrauterine death.

[Lymphedema: anatomy, physiology and pathophysiology of lymphedema, definition and classification of lymphedema and lymphatic vascular malformations]. / Das Lymphödem - Anatomie, Physiologie und Pathophysiologie des Lymphödems, Definition und Klassifizierung des Lymphödems und lymphatischer Gefäßmalformationen.

Lymphedema is a chronic disease associated with a congenital or acquired disorder of the lymphatic vessels or lymph nodes. Untreated lymphedema can lead to complications and disability. Clinical Lymphology deals not only with lymphedema of the extremities but also of the head, the genitals and the internal organs (lymphostatic enteropathy, chylaskos, chylothorax, chylopericard etc). Symptoms of this disorder are often misdiagnosed or not recognized. Ignorance and trivialization of lymphedema causes insufficient treatment, which then is not carried out to the extent as it is possible today by scientific findings. Even today delayed or not optimal treatment causes a long ordeal for many patients.The fact that lymphedema for those affected is a major psychological and social burden, which is limiting the quality of life, has also often been unregarded. The knowledge of anatomy, physiology and pathophysiology as well as the knowledge of causes are necessary for diagnosis, so that early treatment can be initiated.

[Anatomy, micro-anatomy and physiology of the lymphatics of the lungs and chest wall]. / Anatomie, microanatomie et physiologie des lymphatiques des poumons et de la paroi thoracique.

The thoracic lymphatic vessels are pulsating channels which drain actively the fluid of lung parenchyma interstitium and pleural cavities. Their unidirectional valves that avoid reflux of contents, direct the current of fluid to the connection of thoracic duct to subclavian vein or to the thoracic duct itself by these pulsations. The ascending parietal and visceral currents have anastomoses between them. The parietal currents (internal thoracic anteriorly, external axillaries in lateral and paravertebral in posterior) drain the lymph of thoracic wall. Pleural cavities and the visceral currents, drain that of lungs and mediastinal organs. The thoracic duct goes upward in the posterior mediastinum and usually connects to the venous confluent of the left subclavian vein. It receives a part of thoracic lymph and also drains the lymph of trunk and inferior limbs. About a half or two thirds of thoracic duct lymph is originated from liver and intestines. The intestines have the lymph of digestion with the fatty elements, i.e., the chyle.

Splenic artery rupture during pregnancy concealed by a pancreatic lymphangioma: a rare co-occurrence.

A pregnant woman presented to a local hospital with abdominal pain and hemorrhagic shock. Emergency caesarean section ruled out an obstetric cause and revealed a large mass, interpreted as a hematoma, with active bleeding of unknown origin. Because of her poor clinical condition, the patient was admitted to our hospital. Computed tomographic findings were suspicious for bleeding originating from the splenic artery. Laparotomy confirmed the presence of a ruptured splenic artery. A splenic artery aneurysm-a relatively well known entity during pregnancy-was absent. Hemostasis was achieved by clipping the artery. A large pancreatic cystic mass, which was misinterpreted earlier as a hematoma, was surgically removed. The pathologic examination revealed a pancreatic lymphangioma, an uncommon benign tumor. The ruptured splenic artery was presumably related to the pancreatic lymphangioma and vascular changes caused by pregnancy. A splenic artery rupture in co-occurrence of a pancreatic lymphangioma is a unique presentation which has not been reported previously.

Pediatric vascular malformations: pathophysiology, diagnosis, and the role of interventional radiology.

The Mulliken and Glowacki classification (1982) differentiated vascular anomalies into two groups based on their endothelial characteristics: hemangiomas and vascular malformations. Vascular anomalies are localized defects of the vasculature that affect a limited number of vessels in a restricted area of the body. These defects are secondary to errors in vascular morphogenesis. Depending on the type of vessel involved, the vascular malformation group was subdivided into high-flow (such as arteriovenous malformation and arteriovenous fistula) and low-flow lesions (such as venous and lymphatic malformations). Depending on the type of lesion, the location and degree of involvement and the clinical effect, different types of treatment would be required. For the purpose of this review, we concentrate solely on vascular malformations: the clinical features, genetics, diagnosis, and current treatment options.

Compatibilización de equipos de microscopía virtual: análisis de alternativas con software de imágenes panorámicas / Compatability of virtual microscopy equipment: an analysis of different panoramic image software

La implantación de los equipos de microscopía virtual ha estado condicionada por factores como su elevado coste o la falta de estándares de software entre los diferentes fabricantes. Hemos analizado cinco diferentes programas de imágenes panorámicas aplicables a microscopía virtual: Zoomify, HDView, Gigapan, Silverlight Deep Zoom y Google Earth, con el objetivo de encontrar alternativas de fácil implantación para compatibilizar diferentes equipos, y evitar costes adicionales en software específico. Todos los programas, con pequeñas diferencias, han demostrado su potencial adaptación a imágenes microscópicas, y la sencillez en su empleo a través de enlaces web accesibles desde cualquier conexión a internet. Ninguno de los programas ha requerido conocimientos de programación informática, y son manejables directamente por patólogos sin necesidad de asistencia técnica. Sí es recomendable un cierto conocimiento acerca de imágenes digitales, de manejo de servidores web y de diseño de páginas web para poder obtener el máximo rendimiento en su aplicación a la microscopía virtual(AU)

The use of virtual microscopy equipment has been limited by its high cost or the lack of standardization of the software from different manufacturers. In order to find alternative programmes that could be easily used with existing equipment and therefore avoid the cost of specific software, we analyzed the following five panoramic image software suitable for virtual microscopy: Zoomify, HDView, Gigapan, Silverlight Deep Zoom and Google Earth. With only minor differences, all of these programmes were found to be potentially adaptable for microscopic images and were simple to use in the building of web links accessible from any internet connection. None require computer programming and the pathologist can use them without technical assistance, although a certain knowledge of digital imaging, web server management and web design optimizes their performance with virtual microscopy(AU)

Diffuse lymphangiomatosis--a fatal case with atypical skeletal features.

Diffuse lymphangiomatosis is a rare idiopathic condition that occurs mostly in children, is characterized by a non-neoplastic proliferation of lymphatic vessels, leading to organ dysfunction, chylous effusions, and death. A closely related condition-the Gorham-Stout syndrome-is also characterized by lymphangiomatosis and chylous effusions, but also with massive osteolytic changes ("vanishing bone disease"). A 33-year-old woman presented with a 5-year history of worsening chylous effusions and organomegaly. An extensive evaluation has ruled out most diagnoses. A complete radiographic skeletal study did not disclose any osteolytic changes. However, a Tc99 bone scan has demonstrated an absence of osteoblastic activity in some bones. An autopsy confirmed the diagnosis of diffuse lymphangiomatosis, but with histologically normal bone. If this unusual imaging pattern will be reproduced in future cases, a much needed diagnostic aid may help decrease the frequent diagnostic delays in diffuse lymphangiomatosis.

The clinical spectrum of lymphatic disease.

Lymphatic disease is quite prevalent, and often not well clinically characterized. Beyond lymphedema, there is a broad array of human disease that directly or indirectly alters lymphatic structure and function. The symptomatic and objective presentation of these patients can be quite diverse. In this review, we have attempted to provide a systematic overview of the subjective and objective spectrum of lymphatic disease, with consideration of all of the categories of disease that primarily or secondarily impair the functional integrity of the lymphatic system. Lymphedema is discussed, along with chromosomal disorders, lymphangioma, infectious diseases, lymphangioleiomyomatosis, lipedema, heritable genetic disorders, complex vascular malformations, protein-losing enteropathy, and intestinal lymphangiectasia.

Pathogenesis of lymphangiomas.

Based on various hypotheses concerning lymphangiogenesis published in the literature, different putative mechanisms of lymphangioma development are discussed including failure of the lymphatic system to connect with or separate from the venous system, abnormal budding of the lymphatic system from the cardinal vein, or acquired processes such as traumata, infections, chronic inflammations, and obstructions. Increasingly, the possible influence of lymphangiogenic growth factors on the development of lymphangiomas is discussed. The proved expression of different growth factors in the endothelium of lymphangiomas leads to new hypotheses regarding the pathogenesis of lymphangiomas. Thus, further studies on the lymphangiogenesis and the development of lymphangiomas will have to clarify as to whether lymphangiomas are true malformations or neoplastic in nature.

Clinical improvement of diffuse lymphangiomatosis with pegylated interferon alfa-2b therapy: case report and review of the literature.

Diffuse lymphangiomatosis is a very rare congenital disease, characterized by diffuse or multifocal lymphangioma in the skeletal tissue, spleen, liver, mediastinum, and/or lung. The prognosis is usually poor, especially for children with thoracic lesion, and treatments for the disease are controversial. The authors report a 9-year-old boy with diffuse lymphangiomatosis involving the thorax with pleural effusions, the spleen, and systemic bone. The patient was treated with pegylated interferon alfa-2b, and achieved good clinical and radiological improvement.

Immune response: A possible role in the pathophysiology of hemangioma.

Hemangioma is a distinct category of benign vascular tumors characterized by presentation within the first weeks of life, rapid growth during the first year and variable degree of spontaneous involution over a period of several years. Recent research reported that CD8+ T cells in hemangiomas, and the endothelia of hemangioma uniquely expressed leukocyte marker FCgammaRII and myeloid cell marker. Presence of high levels of indoleamine 2,3-dioxygenase in proliferating hemangiomas and significantly decreasing during involution was also confirmed. Topical application of imiquimod cream, an immune regulator, to proliferating hemangiomas apparently accelerated regression of the lesions. These findings suggest immune response may be involved in the pathogenesis of hemangioma. The endothelia of hemangioma may express various markers to escape the immune surveillance. An immune response may be one of the mechanisms for hemangioma regression. Strategies with systemically or locally applying immune regulator into the tumor may be an applicable way in accelerating the involution of hemangioma.