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Congenital chylous ascites is a rare polietiologic entity, requiring close study of the infant's organism by visualization methods in order to diagnose the bening or malignant underlying pathology. In the article is given a report on case of congenital chylous ascites in infant, caused by lymphangioma in the peritoneal cavity. Atypical clinic and the lack of diagnostic standards led to the later detection of the ascite's origin.

Objective: To add a new case of primary vulvar lymphangioma circumscriptum to the literature and to review the current literature., Materials and Methods: We searched the PubMed/MEDLINE databases for previous case reports using the key words "vulvar lymphangioma circumscriptum" and "primary vulvar lymphangioma circumscriptum.", Results: We found 21 cases of primary vulvar lymphangioma circumscriptum reported until 2013. Mean age was 32 years (range = 3-76 y), and 62 % of the cases had been treated surgically., Conclusions: The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.

Systemic lupus erithematosus (SLE) is an autoimmune disease with multiorgan involvement caused principally by vasculitis of small vessels. The gastrointestinal tract is one of the most frequently affected by SLE, with abdominal pain as the most common symptom. An early diagnosis and treatment of lupus enteritis is essential to avoid complications like hemorrhage or perforation, with up to 50 % of mortality rate. However, differential diagnosis sometimes is difficult, especially with other types of gastrointestinal diseases as digestive involvement of antiphospholipid syndrome (APS), moreover when both entities may coexist. We describe the case of a patient with both diseases that was diagnosed with lupus enteritis and treated with steroid therapy; the patient had an excellent response.

Unlabelled: Lymphangiomas are a common form of vascular malformation of the lymphatic vessels, mainly in the head and neck region. Most cases are progressive evolution and require a multidisciplinary approach. Currently, the first therapeutic option is sclerotherapy, leaving surgery for the treatment of remaining lesions., Objective: To present a case of facial lymphatic malformation (LM) treated with sclerotherapy, surgery and orthodontics in a 15-year follow up., Case Report: A one-year-old female patient who consulted health professionals due to a progressive volume increase of the soft parts of her right cheek. The imaging study confirmed the diagnosis of microcystic lymphatic malformation. It was managed with OK-432 sclerotherapy and Bleomycin. At 2 years of age, the patient response was considered adequate; an intralesional submandibular surgical excision was then performed, with partial resection of the lesion. The biopsy confirmed the diagnosis of microcystic LM. Six months after, a re-resection was planned using the same approach and removing the remaining lesion, with favorable development until the age of 9 years when the patient required surgery and orthodontic management due to intraoral recurrence. No major developments until the age of 13 when a new orthodontic surgery and handling are planned to perform right oral commissure suspension., Conclusion: LM management by sclerotherapy, surgery, and orthodontics has shown the advantages of a multidisciplinary long-term treatment in this case.

Objective: To present two cases of infrequent renal masses, trying to achieve the diagnosis before surgery., Methods: We describe a case referred from the Department of Hematology in which bilateral perirrenal masses were described in the CT scan; after biopsy they where classified as extramedullary hematopoietic tissue. The other case was a patient presenting to the emergency room with dyspnea. CT Scan showed lungs with multiple cysts, chylothorax and a cystic-solid mass in the left perirenal space. In the lung biopsy they reported lung lymphangiomatosis, so we didn't perform renal biopsy., Results: Most renal masses are renal carcinomas (856%). The less common diagnosis are sarcomas, lymphomas, upper urinary tract transitional cell carcinomas, metastases of other primary tumors, the Erdheim-Chester disease, the Castleman disease and benign tumors. All these diseases might show similar images in the CT scan and MRI, being the biopsy and histological study necessary for the diagnosis, Conclusions: Perirenal extramedullary hematopoiesis and perirenal lymphangioma are rare diseases that need a pathologic study for their diagnosis.

Introduction: Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma., Case Presentation: A 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. He had been diagnosed with Klippel-Trenaunay syndrome at the age of six years, with hemihypertrophy of his right lower limb and strawberry naevi over both lower limbs. His blood film results were positive for acanthocytes, which accounted for more than 20% of the red blood cell population. He was also found to have extensive splenic lymphangiomas and a large retroperitoneal lymphangioma encasing the mesentric vessels in the right para-aortic region. An extensive battery of tests to identify a secondary cause for the acanthocytosis failed to show any positive results., Conclusions: Retroperitoneal lymphangioma has been reported in association with Klippel-Trenaunay syndrome once before, but an association with acanthocytosis has never been reported. Given the rarity of all three conditions this is not surprising. The cause of acanthocytosis in this setting is currently unresolved. It is plausible that this may be a primary association with Klippel-Trenaunay syndrome, as an alternative aetiology was not found.

Objectives: Lymphangiomas are relatively uncommon lesions of the lymphatic channels that can arise in virtually any part of the body provided by lymphatic vessels. The most common localization is the head/neck region, with only sporadic reports in other sites. The mediastinum is a rare location, with around 20 cases reported in the literature., Methods: We describe one case of mediastinal lymphangioma with a true intrathymic localization, which to our knowledge has never been described., Results: The gross features and microscopic findings are reported with a discussion of the clinicopathologic signatures of this pathologic entity., Conclusions: Intrathymic lymphangioma should always be taken into consideration in the differential diagnosis of cystic mediastinal lesions in children and adult patients., (Copyright© by the American Society for Clinical Pathology.)

A 12-year-old girl presented with swelling on the lateral aspect of the left foot since early childhood, progressively increasing in size and now causing pain on walking. Pain was relieved with medication only. There was also history of a single episode of blood-tinged discharge from the swelling 3 years prior. There was no history of trauma, diabetes, or hypertension.

Benign lymphangioendothelioma (BL) represents a very rare lymphatic vascular proliferation. Our aim is to be aware that owing to its characteristic features, pathologists can easily misinterpret it as cutaneous low-grade angiosarcoma when examining only small specimens. In the present case, multiple small and yellowish to reddish soft nodules were noticed in the edematous left arm of a 54-year-old Japanese female 4 years after the radical mastectomy with axillary lymph nodes dissection and following radiotherapy to the chest for the left breast carcinoma. The biopsy specimen showed an ill-defined lesion composed of a proliferation of irregular and sometimes anastomosing vascular structures in the dermis, lined by endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no mitotic figures. As the lesion grew within deeper dermis, these proliferating vessels dissected dermal collagenous bands, occasionally arranged in low-papillary projections and/or characteristic hobnail cytomorphology. We first interpreted it as low-grade angiosarcoma following chronic lymphedema due to the operation, i.e., the so-called Stewart-Treves syndrome. Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well. Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%. Therefore, we finally made a diagnosis of BL of the skin. MIB-1 labeling index might be useful and adjunctive aids for reaching the correct diagnosis of cutaneous BL, especially in case of small or inadequate specimens.Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_197.

Cystic lymphangiomas of the adrenal gland are rare. A 79-year-old female presented in the emergency room with epigastric discomfort, and an immovable mass was palpated in her abdomen upon physical examination. Imaging studies revealed a large cystic lesion in the pancreatic tail. The radiologic impression ruled out the possibility of a mucinous cystic neoplasm, or a pseudocyst in the pancreas. The operative findings demonstrated that the cystic mass originated in the left adrenal gland. A laparoscopic excision of the cystic mass was performed, and immunohistochemistry confirmed that this mass was a lymphangioma of the adrenal gland. Several prior reports have suggested that lymphangioma can mimic renal or splenic cysts. However, lymphangioma cases mimicking pancreatic cysts are very rare.

A 47-year-old woman presented with left nipple itch and discomfort. On physical examination she was found to have a 7 mm lesion. She underwent bilateral mammography and bilateral breast ultrasound which were normal. A punch biopsy of the lesion was performed in the office and the specimen submitted to pathology. Histopathological examination showed ectatic vascular spaces lined by flattened, cytologically bland endothelial cells dissecting the dermal collagen. Evident lymphatic valves were present within the vascular spaces confirming that the vessels were lymphatic in nature. The diagnosis of acquired progressive lymphangioma (benign lymphangioendothelioma) was rendered., (2014 BMJ Publishing Group Ltd.)

Malignant cutaneous vascular tumours are very uncommon in children. As in adults, they are aggressive neoplasms with metastatic potential and a high mortality rate. Some nonmalignant vascular tumours may exhibit a locally aggressive behaviour and some of them can be associated with life-threatening systemic complications such as Kasabach-Merritt syndrome. Early diagnosis of these aggressive neoplasms in children is very important in order to start appropriate therapy as soon as possible, as this can have a significant impact on the prognosis of these patients. This review focuses on cutaneous vascular tumours of intermediate dignity (tufted angioma, kaposiform haemangioendothelioma, multifocal lymphangioendotheliomatosis with thrombocytopenia, papillary intralymphatic angioendothelioma, retiform haemangioendothelioma, adult-type haemangioendotheliomas) and malignant vascular tumours in young children (Kaposi sarcoma, angiosarcoma)., (© 2014 British Association of Dermatologists.)

Intra-abdominal cysts have a variety of origins, of which lymphatic and mesothelial types are the most commonly encountered. Here we describe a combined mesothelial cyst and lymphangioma arising within the small bowel subserosa of an 80-year-old woman. This was found incidentally at laparotomy performed for an unrelated condition. To date, such a hybrid lesion has not been previously reported. The ways by which this lesion might have arisen are discussed., (© The Author(s) 2013.)

We report a case of lymphangioma circumscriptum (cavernous lymphangioma with epithelial hyperplasia) in a 12-year-old girl, presenting as a papillary tumor on the right dorsal side of her tongue. Microscopic examination found cavernous vascular channels lined by a single layer of CD31(+), podoplanin-positive, CD34(-) endothelial cells that occupied the papillary lamina propria and were accompanied by epithelial hyperplasia. A review of the literature on oral vascular tumors with epithelial hyperplasia, namely, lymphangioma circumscriptum and angiokeratoma, provided information that draws into question the use of these terms., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Lymphangioma is an uncommon benign tumor that develops in the lymphatic system. Abdominal lymphangiomatosis is extremely rare in adult patients, and the clinical symptoms of this condition are complicated and atypical. We report a case of abdominal lymphangiomatosis in a 38-year-old female who presented with intestinal bleeding and protein-losing enteropathy, as well as lesions in the lung and bones. A computed tomography scan revealed multiple small cystic lesions without enhancement. Histological examination revealed microscopic cysts were submucosal, with walls composed of thin fibrous tissue, and D2-40 stained highlight the lining of the lymphatic channels by immunohistochemical method. We make a comparison with the cases reported before, and also discuss the diagnose of diffuse pulmonary lymphangiomatosis and Gorham's disease.

Oropharyngeal and tonsillar masses are a very rare cause of dysphagia in children. In this article, we describe a rare cause of dysphagia in children, a lymphangiomatous lesion of the palatine tonsil, and discuss the clinical and histopathologic features of these lesions.

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.

Infantile hemangiomas are the most common benign tumor of childhood. Lymphangiomas are benign hamartomatous vascular tumors. Both lesions can be problematic when located in the periocular region. Pediatricians must be familiar with the characteristics of each which would necessitate referral to an ophthalmologist or other subspecialist for evaluation, including obstruction of the visual axis which can lead to amblyopia of the affected eye. Additional potential complications include proptosis, ocular motility limitation, optic nerve injury, and poor eyelid closure with or without corneal surface disease. All children with periocular hemangiomas or lymphangiomas should be referred to an ophthalmologist for further evaluation., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Lymphangioma is a benign proliferation of the lymphatic vessels that accounts for approximately 4% of vascular malformations and 26% of benign vascular tumors. Compared to those arising in nongenital areas, lymphangiomas of the vulva and genital areas are more hyperplastic, possibly due to the loose connective tissue, which can cause a cauliflowerlike appearance and may easily be misdiagnosed as genital warts or molluscum contagiosum. We report a case of acquired progressive lymphangioma (APL) of the inguinal area that mimicked giant condyloma acuminatum and showed favorable results following surgical excision. We also provide a review of the literature regarding the pathogenesis, diagnosis, differential diagnosis, and treatment of APL.

Murine bEnd.3 endothelioma cell line has been widely used in vascular research and here we report the novel finding that bEnd.3 cells express lymphatic vessel endothelial hyaluronan receptor-1 (LYVE-1) and vascular endothelial growth factor receptor-3 (VEGFR-3). Moreover, these cells express progenitor cell markers of Sca-1 and CD133. Upon stimulation with tumor necrosis factor-alpha (TNF-alpha), the bEnd.3 cells demonstrate enhanced formation of capillary-type tubes, which express LYVE-1. As the bEnd.3 cell line is derived from murine endothelioma, we further examined human tissues of endothelioma and identified lymphatic vessels in the tumor samples which express both LYVE-1 and podoplanin. Moreover, a significantly higher number of lymphatic vessels were detected in the endothelioma samples compared with normal control. Taken together, this study not only redefines bEnd.3 cells for vascular research, but also indicates a broader category of human diseases that are associated with lymphatics, such as endothelioma.

Lymphangioma circumscriptum (LC) results from the development of abnormal lymphatic vasculature and is characterized by the presence of grouped vesicles filled with clear or colored fluid. Vulvar localization is uncommon. Abnormalities of the lymphatic system, such as lymphedema and cystic hygroma, are well-known features of genetic disorders such as Noonan syndrome (NS) and Turner syndrome. We report the case of a patient with NS who presented with LC of the vulva. We also discuss the expanding spectrum of clinical anomalies associated with the presentation of NS.

Orbital lymphangiomas are mostly congenital, apparent vascular space-occupying lesions, which can lead to disfiguring swelling of the periorbital soft tissues, ocular motility disorders, optic nerve compression and keratopathy. The treatment is challenging because the disease is principally incurable. Lymphangiomatous tissue can be surgically partially reduced or treated by intralesional injection of various sclerosants. In this review we report the successful use of OK-432 for destruction of a macrocystic orbital lymphangioma.

The scrotum is an extremely rare site for lymphangioma. We report the case of a scrotal lymphangioma in a 20-year-old male patient who developed painless scrotal swelling. Typical sonography and MRI findings are shown. Surgical excision and histopathology confirmed the diagnosis., (Copyright © 2013 Wiley Periodicals, Inc.)

Lymphangiomas are rare congenital malformations which are commonly seen in the head and neck region. The disease can be histologically differentiated from other vascular disorders such as cavernous or capillary hemangioma with the lymphatic endothelium-lined cystic spaces. The onset of lymphangiomas are either at birth (60 to 70%) or up to two years of age (90%). It is rare in adults. The therapeutic strategy is mainly based on the surgical removal of the lesion. The risk of recurrence is high in incomplete removal of the lesion. In this article, we discuss the major clinical manifestations, disease-related impairments and dental problems which patients may face as well as treatment options for lymphangioma of the tongue. Within this context, we present four cases of lymphangioma of tongue, including two with a giant macroglossia leading problems in dentition and related dental problems.

Vulvar lymphangioma circumscriptum (LC) is an unusual benign condition, congenital or acquired, related to interference in the lymph drainage. Acquired cases are usually related to oncological surgical procedures. We report 6 cases of vulvar LC: 3 have been caused by surgery and radiotherapy for gynecologic cancer, whereas the other 3 have originated under benign conditions--Crohn disease, recurring episodes of cellulits, and lower limb lymphedema. In 3 of them, the initial clinical diagnosis was genital wart. Surgical exeresis was performed as the treatment of choice in 5 patients and there were 3 cases of recurrences. Diagnosis of this unusual condition is not always easy even after histopathologic examination. The possibility of vulvar LC should be taken into account as a possible diagnosis in patients with previous oncological surgery or genital warts refractory to the conventional treatment. In these cases, a biopsy of not only the epidermis but also the superficial dermis is recommendable to rule out inflammatory or tumoral processes. Knowledge on the features of this lesion can avoid unnecessary clinical and therapeutic procedures.

Vascular lesions are commonly encountered in routine pathologic practice and often pose diagnostic challenges owing to their morphologic diversity. Although WT-1 expression was reported in some vascular tumors, little is known about its staining patterns in a spectrum of vascular lesions from various locations. We examined WT-1 immunostain in 95 cases of vascular lesions including angiosarcomas (AS, 19 cases), hemangioendotheliomas (HE, 5), Kaposi's sarcomas (KS, 4), cavernous hemangiomas (CVH, 12), capillary hemangiomas (CPH, 7), pyogenic granulomas (PG, 4), lymphangiomas (LA, 4), hemangiopericytomas (HP, 5), glomus tumors (GT, 8), vascular malformation (VM, 13) and granulation tissue (GRT, 14). Strong WT-1 cytoplasmic stain was invariably observed in all cases of malignant and borderline vascular tumors including AS (19/19), KS (4/4) and HE (5/5). WT-1 was also consistently expressed in CPH (7/7), PG (4/4), and GRT (14/14), while it became weaker in VM (10/13) and often negative in CVH (2/12) and LA (0/4). WT1 stain was not demonstrated in HP (0/5) and rarely in GT (2/8). We conclude that consistent and diffuse WT-1 cytoplasmic stain in AS, HE and KS can be useful in distinguishing these tumors from poorly differentiated tumors with mimicking features. On the other hand, reliable WT-1 stain in CPH, PG and GRT may help in differential diagnosis with non-endothelial vascular tumors such as GT and HP. Recognizing the WT-1 cytoplasmic stain in a broad spectrum of benign and neoplastic tissues is critical in formulating appropriate immunohistochemical panels and avoiding misinterpretation of results.

Hemolymphangioma is a malformation of both lymphatic and blood vessels. The incidence of splenic hemolymphangioma is extremely rare. Laparoscopic partial splenectomy is feasible, reproducible, and safe in children with focal splenic tumors. We report on the case of a 12-year-old male with a large splenic hemolymphangioma successfully managed by laparoscopic partial splenectomy. The patient recovered well after operation. Laparoscopic partial splenectomy is a safe and minimally invasive technique for treatment of splenic hemolymphangioma located in the pole of the spleen.

Congenital cystic masses of the neck are uncommon and can present in any age group. Diagnosis of these lesions can be sometimes challenging. Many of these have characteristic locations and imaging findings. The most common of all congenital cystic neck masses is the thyroglossal duct cyst. The other congenital cystic neck masses are branchial cleft cyst, cystic hygroma (lymphangioma), cervical thymic and bronchogenic cysts, and the floor of the mouth lesions including dermoid and epidermoid cysts. In this review, we illustrate the common congenital cystic neck masses including embryology, clinical findings, imaging features, and histopathological findings., (Copyright © 2014 Elsevier, Inc. All rights reserved.)

Background/aims: Spleen lymphangiomas are rare congenital malformations of the lymphatics. There is a paucity of data in the literature on the evaluation and laparoscopic treatment for adult spleen lymphangiomas. The aim of this study is to further elucidate the feature of this tumor and laparoscopic management of spleen lymphangiomas., Methodology: From September 2006 to February 2011, data was collected retrospectively for all patients who underwent laparoscopic splenectomy in our institution with a diagnosis of spleen lymphangiomas. The perioperative details were recorded as well as follow-up., Results: Seven adult patients underwent successfully LS for spleen lymphangiomas. Mean age was 47.9 years and five patients (71.4%) were women. Patients' symptoms included abdominal pain (28.6%), palpable abdominal mass with nausea (14.3%). Four patients (57.1%) were asymptomatic at diagnosis. The preoperative diagnosis depended on ultrasonography, computed tomography and final needle aspiration. No patient had multiple splenic lesions before. Mean operative time was 112 min. Mean estimated blood loss was 78.6 ml. Pathological examinations confirmed the preoperative diagnosis. No preoperative and postoperative complications have occurred. There were no recurrences at a median follow-up of 31 months., Conclusions: Laparoscopic splenectomy is a preferred treatment for patients with suspected cystic lymphangioma. It should be preferred to total splenectomy.

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.

Introduction: Lymphangioma is a rare benign cyst caused by congenital malformation of the lymphatic systems that often occurs in the cervicofacial region. There is no consensus on its management: Observation, aspiration, injection, cryotherapy, electrocautery, radiation, laser, ligation and excision., Methods: We performed a literature search with the keywords "cystic hygroma", "lymphangioma", "management", "OK 432" and "picibanil" from Medline, Embase and PubMed databases., Results: We present a review of the history, signs and symptoms, diagnosis, histology, classification and management options of cystic hygroma., Conclusion: There is no consensus on the treatment options. It should be individualised depending on the size of the lesion, anatomic localisation and complications.

Lymphangioma of the palatine tonsil is a rare, benign lesion that presents as a tonsillar outgrowth and causes symptoms related to irritation and airway obstruction. Histologically, the mass has abundant dilated lymphatic channels amid a fibrous stroma with lymphoid and adipose elements. There are several theories regarding the pathogenesis of these lesions, and the appropriate diagnostic classification is controversial. Because a lymphangioma may resemble a true neoplasm of the palatine tonsil clinically, the lesion must be removed for accurate histologic diagnosis and to rule out malignancy. Lymphangioma of the palatine tonsil is treated with surgical excision and has no recurrence once completely resected.

Background: Benign lymphangioendothelioma represents a rare lymphatic vascular proliferation characterized by proliferation of irregular and thin-walled vessels dissecting amongst dermal collagen. Immunohistochemical analysis has been lacking in most previously reported cases., Methods: Herein, we report the clinical and histopathologic characteristics of four cases of benign lymphangioendothelioma. Immunohistochemical study was completed for all lesions., Results: All lesions presented as large, red to brown patches or plaques. Three lesions were located on the thigh and one lesion was located on the neck. Histopathologically, all lesions showed proliferation of anastomotic or retiform thin-walled vessels with a single layer of endothelial cells that dissect the dermis. D2-40 and Prox1 immunostains were positive and Wilms tumor 1 (WT-1) immunostain was negative in all cases., Conclusion: Benign lymphangioendothelioma represents a lymphatic vascular proliferation. A lack of expression of WT-1 suggests it represents a lymphatic vascular malformation., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Radiation-induced cutaneous vascular neoplasms occur infrequently and comprise benign, so-called atypical vascular lesions (AVL) and angiosarcomas (AS), often being high-grade malignant tumors. Both arise most frequently within previously irradiated skin in breast-conserving-treated mammary cancer patients. Because of the different clinical course and, consequently, different therapeutic approaches, histopathologic distinction of AVL and AS is essential but significant morphologic overlap has been documented. Furthermore, the coexistence of these lesions or progression of AVL into AS has rarely been reported. Whether AVL is a precursor of AS is much debated and unresolved to date. Recent interest has focused on genetic changes and their differences in AS and AVL. MYC amplification and expression of the corresponding protein has been identified in AS in comparison with AVL. Therefore, MYC fluorescent in situ hybridization and anti-MYC immunohistochemical analysis are diagnostically useful in difficult cases. Furthermore, advanced tailored treatment strategies in AS, one of the most aggressive type of sarcoma, rely on identifying genes and proteins involved in malignant angiogenesis.

An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.

Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is extremely rare, and often affects the skin and subcutaneous tissues of children. Since its first description by Dabska, only a few intraosseous cases have been described in the literature and none of them presents with multifocal osteolytic lesion of bones. We present a case of unusual multifocal intraosseous PILA in facial bones occurring in a 1 year 3 month old male child. Computed tomography (CT) scan revealed multifocal osteolytic lesions were located at the left zygoma, left orbital bone and right maxillary. Histologically, the lesions were ill-defined and composed of multiple delicate interconnecting vascular channels with papillae formation which projected into the lumen lined by atypical plumped endothelial cells. The vascular channels were also lined by plump cuboidal endothelial cells with focal hobnailed or "match-head" appearance. In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens. By immunohistochemistry, the tumor cells were positive for CD31, CD34 and D2-40 at varying intensity. A final diagnosis of intraosseous PILA was made. To the best of our knowledge, this case is the first case of primary multifocal osseous PILA., Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1919488629100787.

Maintenance of tissue homeostasis and immune surveillance are important functions of the lymphatic vascular system. Lymphatic vessels are lined by lymphatic endothelial cells (LECs). By gene micro-array expression studies we recently compared human lymphangioma-derived LECs with umbilical vein endothelial cells (HUVECs). Here, we followed up on these studies. Besides well-known LEC markers, we observed regulation of molecules involved in immune regulation, acetylcholine degradation and platelet regulation. Moreover we identified differentially expressed WNT pathway components, which play important roles in the morphogenesis of various organs, including the blood vascular system. WNT signaling has not yet been addressed in lymphangiogenesis. We found high expression of FZD3, FZD5 and DKK2 mRNA in HUVECs, and WNT5A in LECs. The latter was verified in normal skin-derived LECs. With immunohistological methods we detected WNT5A in LECs, as well as ROR1, ROR2 and RYK in both LECs and HUVECs. In the human, mutations of WNT5A or its receptor ROR2 cause the Robinow syndrome. These patients show multiple developmental defects including the cardio-vascular system. We studied Wnt5a-knockout (ko) mouse embryos at day 18.5. We show that the number of dermal lymphatic capillaries is significantly lower in Wnt5a-null-mice. However, the mean size of individual lymphatics and the LEC number per vessel are greater. In sum, the total area covered by lymphatics and the total number of LECs are not significantly altered. The reduced number of lymphatic capillaries indicates a sprouting defect rather than a proliferation defect in the dermis of Wnt5a-ko-mice, and identifies Wnt5a as a regulator of lymphangiogenesis., (© 2013 Elsevier Inc. All rights reserved.)