Dilated Vascular Channels Research Articles

A pearly papule with arborizing vessels

Fig 1. A, Clinical picture of a pink papule, tender when touched on the scalp. B, Dermatoscopy image (DermLite II ProHR, 3Gen LLC, California) of the lesion showing branched vessels as well as blue and yellow clods. C, Histopathology: Circumscribed polylobulated basophilic ovoid nodule located in the dermis, well encapsulated with regular borders. The tumor contains 3 cell types: small basaloid cells with dense hyperchromatic nuclei, located at the periphery of the nodules, larger cuboidal cells with pale eosinophilic cytoplasm, located in the center, as well as scattered lymphocytes. Ductal structures, sparse cystic cavities, dilated vascular channels, and homogeneous eosinophilic material are also seen within the nodules. The overlying epidermis is intact. (Hematoxylin and eosin stain; original magnification:3100.)

A Blakemore for the Bile Duct

Case Report: An 86-year-old woman presented with 1 day of epigastric pain, vomiting, and chills. On exam, she was normotensive, afebrile, and had epigastric tenderness. Laboratory findings demonstrated: TBili 3.4 mg/dL, DBili 2.6 mg/dL, SGOT 410, SGPT 316, WBC 9.3 K/uL (90% neutrophils). Ultrasound showed gallstones, gallbladder wall thickening, and a common bile duct (CBD) measuring 8-9 mm. CT revealed gallbladder wall thickening. Choledocholithiasis was suspected and ERCP was performed. Cholangiogram revealed dilated CBD with a small stone, and sphincterotomy was performed without bleeding. With multiple balloon sweeps, 1 gallstone was extracted from the CBD. During extraction, fluoroscopy revealed extravasation of dye outside the distal CBD. Shortly thereafter, copious blood was seen in the duodenum. A 10 x 60-mm Boston Scientific® Wallflex fully covered stent (SEMS) was then deployed into the CBD under fluoroscopic and wire guidance. No source of bleeding was identified with either duodenoscope or gastroscope. After resuscitation, an emergency CTA was performed and showed portal venous gas, portal vein thrombus, and biliary varices. No further bleeding episodes occurred this hospitalization. No attempt was made to remove the SEMS out of concern of rupturing additional varices. Discussion: Choledochal varices form from venous dilation in the setting of increased extrahepatic portal pressures. The extrahepatic bile duct is surrounded by 2 different venous systems. Dilation of the paracholedochal veins of Petren, which run parallel to the bile duct, usually causes extrinsic compression of the bile duct wall. With dilation of the epicholedochal venous plexus of Saint, which line the outer surface of the CBD, bleeding and mechanical obstruction may occur1. Although choledochal varices are uncommon, they can lead to life-threatening consequences during endoscopy or surgery. The proposed mechanism is squeezing of the dilated vascular channels proximally resulting in rupture of distal channels2,3. Jacobson et al first described rupture of a choledochal varix following balloon extraction of a stone, resulting in massive bleeding, and subsequently placing a plastic stent.1 Sharma et al described 3 similar cases3. While choledochal variceal rupture following balloon extraction has been described (4-7), this is the first reported case of choledochal variceal bleeding secondary to balloon extraction and successful treatment with the placement of a fully covered SEMS. The proposed mechanism is tamponade of the choledochal varices, similar to the effect of a Blakemore tube on esophageal varices. Future prospective randomized trials are required to explore the use of SEMS for bleeding choledochal varices.

Immune complex formation and in situ B-cell clonal expansion in human cerebral cavernous malformations

Cerebral cavernous malformations (CCMs) represent clusters of dilated vascular channels, predisposing to hemorrhagic stroke and seizures. They are associated with defective blood brain barrier, hemorrhages of different ages and a robust inflammatory cell infiltrate. We report for the first time evidence of co-localized IgG and complement membrane attack complexes in CCM lesions. CD4+ and CD8+ T-cells are aggregated with CD20+ B-cells. And IgG repertoire analyses demonstrate in situ B-cell clonal expansion and antigen-driven affinity maturation in CCMs. These results suggest an organ-intrinsic adaptive immune response in CCMs that should be further characterized as a potential therapeutic target.

Surgical Treatment of Masseteric Venous Malformations and Outcomes

Intramuscular venous malformations are often misdiagnosed as other types of tumors with similar presentations. We describe here the typical presentation of a venous malformation within the masseter muscle, including the physical findings and imaging, and present our experience with the surgical excision of these lesions. This is a review of 10 patients with venous malformations localized to the masseter muscle who presented to our institution between 2008 and 2011. The patients included 6 females and 4 males. Of these venous malformations, 80% were noted in childhood, and the remainder manifested later in adolescence. Pain, swelling, and facial asymmetry were the presenting complaints. Magnetic resonance imaging, Doppler ultrasonography, and whole-body blood scintigraphy were used to characterize the lesions. All patients were treated by total resection of the lesion while preserving the marginal mandibular branch of the facial nerve. Magnetic resonance imaging showed the lesions to be isointense with surrounding muscle on T1-weighted images and hyperintense on T2-weighted images. Gross examination of the resected specimens revealed multicolored tissue with dilated vascular channels, frequently containing phleboliths. After the operation, all patients showed improvement in both symptoms and appearance. This improvement was sustained at a mean follow-up of 21 months. Masseteric venous malformations typically present with a pattern of clinical and imaging findings that should allow surgeons to distinguish them from other diseases in the cheek area. Complete surgical excision is a treatment option for these patients and can be performed without facial nerve injury or excessive bleeding. This procedure can result in excellent outcomes for localized intramasseteric venous malformation.

Cavernoma of the lateral ventricule presenting as speech arrests

AbstractCavernous malformations (CM) are formed by dilated thin-walled vascular channels without intervening parenchyma and intraventricular lesions are rare. We report a case of an intraventricular cavernoma in a 54-year old female patient. She presented with speech arrests daily for 10 months. Neurological examination revealed no deficits. The brain MRI study revealed an intraventricular lesion at the level of the left atrium, heterogeneously hyperintense on both T1 and T2-weighted images. It was performed an interhemispheric transcallosal approach with gross-total resection of the lesion. The histological examination was compatible with the diagnosis of cavernous haemangioma. In the postoperative period the patient presented with transient right hemiparesis with recovery in two weeks. We briefly review the literature concerning intraventricular cavernomas.

A case review of myopericytoma: radiological, macroscopic and histological correlation

<h3>Case report</h3> A 76-year-old gentleman presented with a 10 year history of a painless solitary nodule on the right hand. The nodule demonstrated homogeneous intense enhancement on contrast-enhanced CT. The nodule measured 45 mm and had a haemor-rhagic cream white cut surface. Histologically it showed a circumscribed encapsulated nodule containing dilated vascular channels, haemorrhage and hyalinisation. The blood vessels were surrounded by short fascicles of myoid appearing plump spindled cells with abundant eosinophilic cytoplasm. Mitoses were inconspicuous and there was no cytological atypica or necrosis. The tumour was positive for smooth muscle actin and calponin and did not stain for S100 and CD34. A diagnosis of myopericytoma was made. <h3>Discussion</h3> Myopericytoma is a benign pericytic tumour commonly occurring in adults especially in the distal extremities. Differential diagnosis would include myfibroma, angioleiomyoma, glomus tumour, solitary fibrous tumour, haemangioma or ancient Schwannoma. The clinical history, pathological appearances and immunoperoxidase studies discussed above are characteristics of this tumour. The tumour rarely recurs if completely excised. There are very few cases of malignant myopericytoma which show infiltrative growth, marked atypia and increased mitoses.

Acquired Microcystic Lymphatic Malformation of the Distal Upper Extremity Mimicking Verrucae Vulgaris

An 18-year-old African American male with a history of congenital lymphedema of the right upper extremity presented for evaluation of multiple verrucous lesions on his right hand. Clusters of 2 to 4-mm dome-shaped vesicles were intermixed with scattered verrucous papules on the right forearm and the dorsal and palmar aspects of the hand. Histopathology of one the verrucous lesions showed well-circumscribed areas of dilated lymphatic vascular channels with lymph in the lumen. The patient was diagnosed with microcystic lymphatic malformation, verrucous type. This article reviews the literature regarding reports of this variant of microcystic lymphatic malformation in the pediatric population.

Adult Diffuse Hepatic Hemangiomatosis: A Rare Cause of Dilated Cardiomyopathy and Sudden Cardiac Arrest

A 53-year-old man presented with two months of fatigue and dyspnea on exertion, and was found to have hepatomegaly and hypotension (71/30 mmHg). Initial tests showed lactic acidosis with normal cardiac enzymes. Computed tomography of the abdomen revealed diffuse, hypodense, hepatic lesions without ischemic bowels. Shortly after admission, he developed cardiopulmonary arrest with ventricular fibrillation and expired despite aggressive medical support. Postmortem examination demonstrated dilated cardiomyopathy without coronary artherosclerosis (heart weight 785 g, normal 365 ± 71 g) and hepatomegaly with diffuse hemangiomatosis (liver weight 4,455 g, normal 1,677 ± 396 g, Fig. 1).1 Figure 1. Multiple irregular, well-circumscribed, dark brown lesions (arrow) were seen in the liver parenchyma. The lesions ranged from 0.5-10 cm in greatest dimension. Diffuse hepatic hemangiomatosis is extremely rare.2 The histologic characteristics include dilated vascular channels lined with flat endothelial cells (Fig. 2). The etiologies are poorly understood, though its association with the use of estrogen and metoclopramide has been described.3,4 The two most common causes of death are cardiomyopathy (from massive hepatic arteriovenous shunting) and cirrhosis.5 Surgical resection is an option in selected cases with small lesions without cirrhosis.5,6 Most cases are inoperable because of poor delineation of the tumor border and bleeding risk. Other anecdotal therapies include hepatic artery ligation and radiation.6 Prognosis depends on the area of hepatic involvement and cardiac function. Figure 2. Microscopic examination revealed cavernous vascular spaces (asterisk) separated by thin fibrous septae lined by endothelial cells (arrow). These findings confirmed the diagnosis of hepatic hemangiomatosis.

Telangiectatic Hyperplastic Nodule Associated with Vascular Malformation in a Patient with Chronic Hepatitis B: Radiologic and Pathologic Features

Recognizing hepatocellular nodules that cannot be classified as typical for hepatocellular carcinoma, hepatocellular adenoma, or focal nodular hyperplasia is important, especially in a patient with high risk for hepatocellular carcinoma. The authors report a case of a 53-year-old man with chronic hepatitis B, who was referred to the hospital with a liver mass found on routine imaging follow-up. Abdominal ultrasound revealed a 2.4-cm hypoechoic lesion. Contrast computed tomography showed homogeneous arterial enhancement and mild hyperdensity on portal venous phase images. Due to the high risk for hepatocellular carcinoma, the patient underwent laparoscopic left lateral segmentectomy that revealed a 2.2-cm poorly defined red-brown lesion. The nodule was diagnosed as a hypervascular/telangiectatic hyperplastic hepatocellular nodule based on histopathologic findings and immunostaining profile with negative glutamine synthetase, diffuse positive CD34 highlighting hyperplastic endothelial cells along the telangiectatic sinusoids and dilated vascular channels, and CK7 and CK19 reactive normal bile ducts within the lesion.

Large lymphaticovenous malformation resection

We report a case of a giant cardiac lymphaticovenous malformation arising from the atrioventricular groove in a 38-year old Caucasian female. Cardiac vascular lesions are rare and tend to be poorly described in the literature. Lymphaticovenous malformations are present at birth and develop due to errors in venolymphatic development. As the tumour enlarged, the patient experienced significant shortness of breath on exertion. At resection, the mass measured 6.0 cm anterior-posterior ×10.4 cm craniocaudal. The mass was found to be adhered tightly to the coronary sinus. Histologically, the lesion was composed of dilated vascular and lymphatic channels within a fatty stroma. The mass was resected without complications.

The comparison of efficacy of different imaging techniques (conventional radiography, ultrasonography, magnetic resonance) in assessment of wrist joints and metacarpophalangeal joints in patients with psoriatic arthritis

SummaryBackground:Vascular anomalies are usually diagnosed through their clinical picture and history. The purpose of this study was to assess the role of MR imaging in initial assessment of cervicofacial vascular anomalies in children.Material/Methods:Twenty pediatric patients with vascular anomalies located in the cervicofacial region underwent MRI examination in our department. Images were evaluated for lesion detectability and its signal characteristics (on T1w, T2w images with fat suppression and contrast enhanced T1w sequences); the extent of the lesions and surrounding tissue involvement were also assessed.Results:In the studied group MR images revealed all anomalies and provided information of their anatomic extent and invasion of surrounding anatomic structures. Nine hemangiomas and six venous malformations were found among studied patients. Two children had multiloculated lesions corresponding to lymphatic malformations. One examination visualized a lesion consisting mainly of dilated vascular channels with an apparent feeding artery, which was consistent with arteriovenous malformation. Two remaining lesions were mixed malformations. Nine patients had lesions limited to subcutaneous tissue. Two masses infiltrated bone structures. There was muscle involvement found in nine cases.Conclusions:MR imaging is a well-established method for detection and monitoring of vascular anomalies in children. With ultrasound used mostly for initial diagnosis and additional flow assessment, angiography viewed as an invasive therapeutic method and computed tomography used only in specific situations due to its high irradiation dose, magnetic resonance is the best imaging method used in differential diagnosis and topographical characterization of vascular malformations and tumors of cervicofacial area in pediatric patients. Noninvasively and without irradiation, it enables evaluation of the extent and characteristics of lesions and planning proper therapeutic strategy.

Atypical vascular lesion after radiation therapy for breast cancer.

184 Background: Atypical vascular lesions (AVLs) are known to occur in the setting of radiation therapy after breast conserving surgery for treatment of breast cancer. Their relationship with potential secondary angiosarcoma has yet not been fully characterized. AVLs typically present as single or multiple pink papules in the skin of the breast. Histologic examination reveals proliferation of dilated vascular channels in the dermis with IHC reactive for CD31, CD34 and D2-40. Management consists of local excision to rule out angiosarcoma. Methods: The pathology records of the NYU Langone Medical Center were queried for patients who were diagnosed with atypical vascular lesions. Results: A total of 3 patients with previous history of breast cancer treated with breast conserving therapy (BCT) and radiation therapy were included in this case series. Two patients were diagnosed with DCIS and one was diagnosed with Invasive ductal carcinoma. The median time between the cancer diagnosis and the development of AVL was 5 years (3-9 years). Presentation was an erythematous skin nodule in two patients and progressive telangectasia in one. Diagnosis was made initially by punch biopsy and it was followed by excisional biopsy in all three cases. Only one patient had residual atypical vascular lesion in the excisional biopsy especimen. Conclusions: AVL is a pathological entity with growing importance over the years due to its questionable progression to angiosarcoma. The improvement in survival of patients diagnosed with breast cancer as well as the widespread use of lumpectomy with breast irradiation for the treatment of the disease have caused an exponential increase in the number of patients seen in daily practice after BCT. Clinicians must implement high surveillance of these patients and have a low threshold for further work up of skin changes in the radiated breast in order to early diagnose these lesions since it is yet unknown which ones will progress to angiosarcoma. There are ongoing studies in the development of immunophenotypical markers such as MYC that will be a useful diagnostic tool to differentiate AVL from its malignant counterpart.

Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features

Eccrine angiomatous hamartoma (EAH) is an exceedingly rare benign tumor-like lesion prevalent in childhood which may produce pain and marked sweating. The histological features include proliferation of eccrine sweat glands and angiomatous capillary channels. We report an 8-year-old girl who had a single lesion on her left lower leg. Physical examination revealed a slightly elevated, 4×7 cm erythematous plaque on the lateral aspect of left leg. Sweating in the lesion was evoked by physical work or emotional stress. There was no pain or tenderness associated with the lesion. The patient had no history of trauma to the site. These lesions were clinically angiomatous, and we obtained the diagnosis by histopathological examination. Histopathological examination of the lesion showed increased numbers of eccrine glands, as well as dilated vascular channels in the deep dermis and subcutaneous tissue. These findings are consistent with EAH.

Tissue Elastography of Uterine Cervix for Prediction of a Successful Labor Induction: Preliminary Results

s S123 of an abnormal finding in the placental sonogram. Our ultrasound examination revealed a huge placental chorioangioma of an anterior placenta in an intact double-ovum twin pregnancy with normal developed fetuses. Imaging Findings or Procedure Details: Blood perfusion in the chorioangioma was checked and Doppler controls were performed (umbilical andmiddle cerebral artery). Pulsatility index (PI) and resistive index (RI) values were in normal limits. At 36 + 6 weeks gestation, a Misgav Ladach cesarean section was carried out. Two female infants were born weighing 2710 g, Apgar 8/9/10, pH 7.24, BE -5.2 and 2300g, Apgar 9/9/ 10, pH 7.23, BE -5.2, respectively. Histologically two placentas were described, 443 and 762 g, each with a three-vessel umbilical cord and small infarcts. The found hamartomous placental tumor, size 10,7 x 9.3 x 1.9, presented with innumerable blood vessels, lobulas, expanded villi, cystic spaces, dilated vascular channels and calcified areas in the proliferating trophoblastic epithelium. Conclusion: Such large clinically significant chorioangiomas occur very rarely (0.2-4:10,000 births). Often, they cause severe fetal complications, but not in this extraordinary case of a chorioangioma of the vascular type.

Multifocal Congenital Lymphangioendotheliomatosis Without Gastrointestinal Bleeding and/or Thrombocytopenia

A 5-month-old male infant presented with an increasing number of widespread asymptomatic violaceous cutaneous macules, papules, and nodules since birth. He is 1 of the 2 identical twins born to unrelated healthy parents. Histology revealed proliferation of dilated thin-walled vascular channels lined by bland endothelial cells in the dermis and subcutis. In some of the vascular channels, there were formations of intravascular papillae surrounded by hobnail endothelial cells. Immunohistochemistry demonstrated positive staining for CD31, CD34 factor VIII and vascular endothelial growth factor-3 (VEGFR-3) and negative staining for D2-40 and latencyassociated nuclear antigen-1 (LANA-1). The clinical and histologic findings were compatible with multifocal congenital lymphangioendothelionmatosis with thrombocytopenia, except that a year of follow-up was uneventful and no gastrointestinal bleeding or thrombocytopenia was recorded.

Wyburn-Mason syndrome

A 14-year-old girl presented with chronic headache and right-side visual difficulties. She had right facial pigmentation since birth. Ophthalmoscopy examination reveals multiple dilated vascular channels over the right retina suggestive of retinal arteriovenous malformation (AVM) (Fig. 1). Cerebral angiography reveals multiple cerebral AVMs on the ipsilateral side (Fig. 2). Features were in keeping with Wyburn-Mason syndrome (WMS). WMS is a rare type of phakomatosis characterized by unilateral vascular abnormalities involving the facial structures, orbits and brain and most commonly associated with Wyburn-Mason, who initially categorized the entity with detailed descriptions of nine case histories [1]. Clinically, patients may present with cerebral or ocular symptoms.

A Step-by-Step Practical Approach to Imaging Diagnosis and Interventional Radiologic Therapy in Vascular Malformations

Within vascular anomalies, vascular malformations are those present at birth that grow with the patient and exhibit abnormal dilated vascular channels lined by mature endothelium. Vascular tumors, the other group of vascular anomalies, demonstrate endothelial hypercellularity. Vascular malformations are further divided into low-flow varieties (capillary, venous, and lymphatic malformations) and high-flow varieties (arteriovenous malformation and fistula). All malformations exhibit a predictable group of clinical patterns that vary in severity and rate of progression. The interventional radiologist must incorporate this clinical data with characteristic ultrasound and magnetic resonance findings to arrive at a diagnosis. One must then decide in a multidisciplinary fashion, based on objective clinical criteria and image-based morphology, if the patent is a candidate for intervention. Sclerotherapy is a technique used to treat vascular malformations whereby an endothelial-cidal agent is introduced into the endoluminal compartment to initiate vascular closure. The high flow rate of an arteriovenous malformation requires the incorporation of superselective transarterial, direct, and transvenous access with flow reduction techniques to deliver adequate dose of sclerosant and embolic to the nidus. Satisfactory outcomes are seen in over half of all malformations patients. Similar treatment-related complications are seen between malformations but are lowest in lymphatic and highest in arteriovenous malformations.

Solitary Fibrous Tumor of the Uterine Cervix

A 68-year-old woman with stage pT1b1 N0 cervical squamous carcinoma had an incidental cervical polyp. The polyp measured 1.7 cm in maximal diameter and histologic evaluation showed it to be composed of spindle-shaped cells with hypercellular and hypocellular foci. The stroma was collagenized and contained several dilated vascular channels that imparted a hemangiopericytic pattern to the lesion. Immunohistochemistry showed the spindle cells to be positive for vimentin, CD99, CD34, bcl-2, ER, PR, and beta-catenin (cytoplasmic) but negative for EMA, S100, factor XIIIa, AE1/AE3, caldesmon, desmin, CD31, and smooth muscle actin. The morphology and immunophenotype was in keeping with a diagnosis of a solitary fibrous tumor (SFT). SFT shares several histologic features of a superficial cervicovaginal myofibroblastoma; the cellular variability, pattern and distribution of vessels, stromal collagenization, and desmin negativity favors SFT.

Extra digital glomangioma

Glomus tumors are relatively rare vascular tumors with reported incidence of 1.6% of all soft tissue tumors. It is variously regarded as a hamartoma or a neoplasm of neuromyoarterial glomus, which consists of dilated vascular channels, surrounded by proliferating glomus, nerve cells, and plays an important role in temperature regulation. We report the case of glomangioma in a 43-year-old male who came to dermatology OPD with a painful lesion over the right elbow of six months duration. Cutaneous examination revealed a single round reddish blue papule of about 0.5 cm in size on the flexor aspect of the right elbow. The papule was firm in consistency and tender. The excision biopsy showed stratified squamous lining with well circumscribed tumor in the dermis. The tumor consisted of sheets of uniform cells with pale cytoplasm; round to ovoid centrally placed punched out nuclei and well defined cell margins. There were numerous dilated thin walled vascular spaces surrounded by a layer of tumor cells and the histopathology was consistent with glomangioma.

Cavernous malformations: natural history, diagnosis and treatment

Cavernous malformations (CMs) consist of dilated vascular channels that have a characteristic appearance on MRI. CMs are usually found intracranially, although such lesions can also affect the spinal cord. Individuals with CMs can present with epilepsy and focal neurological deficits or acute intracranial hemorrhage. In many cases, however, patients with such lesions are asymptomatic at diagnosis. Furthermore, several natural history studies have documented that a substantial proportion of asymptomatic CMs follow a benign course. Surgical resection is recommended for CMs that require intervention. Radiosurgery has been advocated for many lesions that have not been easily accessible by conventional surgery. The outcomes of radiosurgery and surgery for deep lesions, however, vary widely between studies, rendering treatment recommendations for such CMs difficult to make. In addition to reviewing the literature, this article will discuss the current understanding of lesion pathophysiology and explore the controversial issues in the management of CMs, such as when to use radiosurgery or surgery in deep-seated lesions, the treatment of epilepsy, and the safety of anticoagulation.