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BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is frequently associated with liver cysts, but an association with giant cavernous liver hemangioma is not mentioned in the literature. CASE REPORT We report the case of a 41-year-old man with ADPKD, secondary arterial hypertension, and stage 4 chronic kidney disease who presented with a 2-week history of persistent pain at the base of the right hemithorax and in the right hypochondrium. An ultrasound examination and a contrast-enhanced computed tomography scan revealed a giant cavernous liver hemangioma. Surgery was intially taken into account (however, twice delayed because of the COVID-19 pandemic) but later refused because it would have left the patient with dangerously few liver parenchyma. CONCLUSIONS To our knowledge, this is the first reported case of ADPKD associated with cavernous liver hemangioma. Vascular endothelial growth factor could be the pathophysiological link between the 2 conditions. Further research may unravel the molecular biology that underlies this possible association, pointing to new therapeutic avenues for ADPKD.

Cavernous hemangiomas are the most common benign orbital tumors in the orbit, but radiological differentiation from other solitary orbital masses can still be challenging at times. While there have been previous studies describing the radiological characteristics of cavernous hemangiomas on computed tomography (CT) and magnetic resonance imaging (MRI), there have not been any studies comparing the 2 imaging modalities. The purpose of our study was to evaluate CT and MRI findings of orbital cavernous hemangiomas and compare both modalities.We performed a cross-sectional study of patients with a histopathological diagnosis of cavernous hemangioma over a 20-year period from January 1997 to December 2016 in a single tertiary institution.Our study included 77 patients; mean age was 46.6 ± 11.2 years, and females comprised 68.8%. The lateral orbit (23.4%) was the most common location. The masses were well-defined, with 55.8% being ovoid, 27.3% round, and 16.9% lobulated. The most common enhancement pattern on CT was a small point starting in the periphery, progressing to heterogeneous filling in the late phase. The most common enhancement pattern on MRI was multiple patchy starting points, widespread across the tumor, with a final homogeneous filling in the late phase.Significant differences between CT and MRI were found in terms of area, location, and number of starting points of contrast enhancement in the early phase. We also found that the use of MRI is limited in differentiating fast-filling cavernous hemangiomas from other orbital tumors and in such cases, contrast-enhanced CT would be helpful.

Background: Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies., Objective: To develop guidelines for CCM management., Methods: The Angioma Alliance ( www.angioma.org ), the patient support group in the United States advocating on behalf of patients and research in CCM, convened a multidisciplinary writing group comprising expert CCM clinicians to help summarize the existing literature related to the clinical care of CCM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and radiology standards, (4) neurosurgical considerations, and (5) neurological considerations. The group reviewed literature, rated evidence, developed recommendations, and established consensus, controversies, and knowledge gaps according to a prespecified protocol., Results: Of 1270 publications published between January 1, 1983 and September 31, 2014, we selected 98 based on methodological criteria, and identified 38 additional recent or relevant publications. Topic authors used these publications to summarize current knowledge and arrive at 23 consensus management recommendations, which we rated by class (size of effect) and level (estimate of certainty) according to the American Heart Association/American Stroke Association criteria. No recommendation was level A (because of the absence of randomized controlled trials), 11 (48%) were level B, and 12 (52%) were level C. Recommendations were class I in 8 (35%), class II in 10 (43%), and class III in 5 (22%)., Conclusion: Current evidence supports recommendations for the management of CCM, but their generally low levels and classes mandate further research to better inform clinical practice and update these recommendations. The complete recommendations document, including the criteria for selecting reference citations, a more detailed justification of the respective recommendations, and a summary of controversies and knowledge gaps, was similarly peer reviewed and is available on line www.angioma.org/CCMGuidelines ., (© 2017 Angioma Alliance.)

Purpose: Cavernous hemangioma of the retina (CHR) is a rare retinal vascular hamartoma. The objective of this study was to summarize the current knowledge of this rare condition based on a detailed review of 96 documented cases., Methods: The incidence, clinical and histological appearance, differential diagnosis, treatment, and prognosis of CHR were analyzed by evaluating clinical data obtained from 96 cases in the literature since the first case report in 1934., Results: More than 90% of cases of CHR have been reported in white individuals with even gender distribution. A majority of patients presented a unilateral unifocal lesion without positive family history. Our analysis showed bilateral cases were strongly correlated with family history, which suggests the importance of screenings for family members in bilateral CHR cases. Intracranial involvement was reported in 14% of CHR cases. Most patients were asymptomatic, but in patients with history of headaches, transient visual disturbance, or seizures 80% had intracranial involvement., Conclusion: It is important to screen family members in bilateral CHR cases, and it is critical to exclude cerebral vascular abnormalities in view of relative high incidence of intracranial involvement and high morbidity.

Background: Liver haemangiomas are the most common benign tumours, commonly presented in women and considered giant when their diameter surpasses 4cm. They are mostly asymptomatic and incidental findings. They manifest with abdominal pain and mass effect. These tumours can be managed by observation, enucleation, resection, and embolisation., Objective: To determine the experience in our unit as regards the treatment and post-surgical outcomes of patients with liver haemangiomas., Materials and Methods: A retrospective study was performed on 14 patients with a histopathological diagnosis of liver haemangioma. An analysis was made using the sociodemographic, tumour-related and surgical related variables, as well as any complications., Results: Of the 14 patients analyse, there were 7 males and 7 females, with a median age of 43.43±15.03 years, and a mean tumour size of 6.86±3.5cm. Eight (51.7%) of the tumours were located in the right lobe, 3 (21.4%) in the left lobe, and 3 (21.4%) in the caudate lobe. Resection was performed in 7 patients (50%), enucleation in 5 patients (35.7%), and biopsy in 2 patients (14.3). No relationship was found between sex, pathology, or tumour location. No morbidity or mortality was found., Conclusions: Liver haemangiomas in our unit have similar characteristics to those described in other studies. Surgical treatment in our hospital offers a positive outcome., (Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.)

Orbital tumors are a rather frequent pathology. Their diagnosis and treatment may be difficult. They can be benign or malignant. All the tissues of the orbit can give rise to a tumor, resulting in their large number. Among the benign tumors, we have meningiomas and cavernous hemangiomas, and for the malignant tumors, lymphomas, metastasis, ENT tumors and lacrimal gland tumors in the adult. Usually the signs are nonspecific, with proptosis, oculomotor disturbance, inflammatory signs, pain and sometimes a mass. Imaging (CT, MRI and color Doppler ultrasound) shows the tumor, its location, extent and possible metastases. Biopsy and anatomic and cytopathologic examination confirm the type of benign or malignant tumor. Based on these three elements: clinical appearance, imaging and histology, the tumor will be treated, usually by a surgical approach according to the recommendations of a multidisciplinary tumor conference. Radiation therapy and chemotherapy may supplement the treatment., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Spinal cavernous malformations (SCM) are rare lesions often presenting with acute onset of symptoms and progressive neurological deterioration due to hemorrhage into the spinal cord. With the aid of modern techniques, their surgical removal became much safer. The present study was undertaken to analyze the outcome of our series of surgically and conservatively treated patients with SCM. Over a period of 20 years, 20 surgically treated and 5 conservatively managed patients with spinal cavernous malformations were identified and enrolled into this analysis. Demographic data, clinical symptoms, localization and extension of the cavernoma, as well as pre- and postoperative neurological status were obtained. The clinical status was assessed using the Frankel score. Patients were followed up clinically and by MRI. Before surgery, 90% (18/20) of our surgical patients were classified as Frankel D (93.8%), whereas two patients (10%) were graded C. None of the patients had a worse Frankel score at the time of discharge. Eighty percent of them (16 cases) remained unchanged, and 20% (4 patients) improved during the first follow-up (mean 6.3 months, range 2-17 months). All improved patients had a superficially located SCM and were operated early (≤3 months). No worsening was observed during extended follow-up (range 9-134 months, mean 44.7 months). Five nonsurgically treated patients showed no significant clinical deterioration over a period of 6.7 years (mean, range 2.9-8 years). SCM localization and number of involved segments had no influence on outcome. Our data show that SCM can be resected with favorable neurological outcome by using intraoperative neuromonitoring. Within the follow-up period, patients treated conservatively remained in a stable neurological condition.

Cavernous hemangioma is the most frequent benign orbital tumor in adults. The purpose of this study was to examine its clinical features, to define surgical indications, and to determine the roles of the various surgical approaches praticed in ophthalmology: transconjunctival (increasingly utilized), anterior transcutaneous, and lateral orbitotomy. The records of all patients treated for orbital cavernous hemangioma (OCH) since 2004 at the Fondation Rothschild (Paris, France) were retrospectively reviewed. Forty-three patients were treated for orbital cavernous hemangioma. Fifty-eight percent were women, mean age 50.2; 79 % of the tumors were intraconal. Among those patients, 36 underwent surgical removal, 5 were followed periodically, and 2 were lost to follow-up. The main surgical indications were: optic nerve compression (26 patients), proptosis (24 patients) and diplopia (3 patients). Transconjunctival, anterior transcutaneous and Kronlein approaches were used in 16, 12 and 4 patients respectively. Four patients had intrapalpebral hemangiomas easily reached transcutaneously. Two patients demonstrated transient partial 3rd nerve palsy (one with the lateral orbitotomy approach and one with the transconjunctival approach), one patient with the lateral orbitotomy approach developed a palsy of the superior branch of the 3rd nerve, and one patient with the transcutaneous anterior approach developed mydriasis. Surgical excision of OCH's is required in the presence of clinical complications. The transconjunctival approach is a safe technique which can lead to complete resection of the tumor in most cases., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Object: Functional MRI (fMRI) has proven to be an effective component of pretreatment planning in patients harboring a variety of different brain lesions. The authors have recently reported significant relationships concerning distances between brain tumor borders and areas of functional activation (lesion-to-activation distance; LAD) with regard to patient morbidity and mortality. This study further examines the relationship between LAD, focusing on a host of vascular lesions and pre- and posttreatment morbidity., Methods: This study included a sample population of patients with vascular lesions (n = 106), primarily arteriovenous malformations (AVMs) and cavernomas. These patients underwent pretreatment fMRI-based motor mapping (n = 72) or language mapping (n = 84). The impact of LAD and other variables derived from the patient medical record were analyzed with respect to functional deficits in terms of morbidity (weakness and/or aphasia)., Results: In patients with no pretreatment deficits, there was trend for a significant relationship between the Wernicke area LAD and posttreatment language deficits. In patients with or without pretreatment deficits, a trend toward significance was observed between sensorimotor LAD and posttreatment motor deficits. Additionally, lesion type (AVMs or cavernomas) affected posttreatment deficits, with more patients with cavernomas showing posttreatment language deficits than patients with AVMs. However, this difference was not observed for posttreatment motor deficits., Conclusions: These findings suggest that the proximity of a vascular lesion to sensorimotor and language areas is a relevant parameter in estimating patient prognosis in the perioperative period. Additionally, vascular lesion type and existence of pretreatment deficits play a significant role in outcomes.

Purpose: To evaluate the frequencies of orbital space-occupying lesions seen at single orbital unit in a period of 35 years., Methods: In this retrospective case series, the authors reviewed the medical records of 2,480 consecutive patients referred to the authors' Orbital Unit for evaluation of an orbital mass between 1976 and 2011. The final diagnosis in each case was established by a combination of history, ocular findings, diagnostic imaging, and histopathologic analysis, when available. The number and percentage of benign and malignant tumors were determinated, also according to the age of the patients and the tumor location within the orbit. This study adhered to the principles of the Declaration of Helsinki., Results: Of the 2,480 lesions, 1,697 (68%) were benign and 783 (32%) were malignant. The most frequent benign tumors were dermoid cyst (14%) and cavernous hemangioma (9%). The most common malignant tumors were non-Hodgkin lymphoma (12%), basal cell carcinoma (3%), and orbital metastases (3%). In patients younger than 60 years, benign tumors are more frequent, whereas in patients older than 60 years, the frequency of malignant tumors increased. Regarding the distribution in the orbit, the most common tumors were dermoid cyst (206 cases) in the upper-outer quadrant, mucocele (155 cases) in the upper-inner quadrant, basal cell epithelioma (35 cases) in the lower-inner quadrant, cavernous hemangioma (68 cases) in the lower-outer quadrant, and meningioma (90 cases) in central space. Most of the tumors were located in the upper-outer quadrant. In the lower-inner quadrant, malignant tumors were more frequent than benign tumors., Conclusions: The authors' findings highlight the distinctive nature of the orbital oncology experience at an ocular oncology department.

Background: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments., Materials and Methods: A nationwide survey of critical IHH patients treated between 2005 and 2010 was performed in all 117 registered pediatric surgical hospitals in Japan. As a result, 19 patients were identified and reviewed using a statistical analysis., Results: Abdominal distention (47.4%), high-output cardiac failure (47.4%), coagulopathy (42.1%), and respiratory distress (31.6%) were the major symptoms. Three patients died (1 of coagulopathy, 1 of cardiac failure, and 1 of both). An accompanying portovenous shunt was also highlighted. Infantile hepatic hemangioma was totally insensitive to steroid treatment in 3 (23.1%) of the 13 patients, and 9 (47.4%) of the 19 patients required other treatments. Surgical resection and β-blocker improved the hematologic data, whereas hepatic arterial ligation and embolization seemed to produce a limited effect. Among the dead patients, several hematologic parameters were significantly worse: the thrombocyte count (pretherapeutic: 73,000 vs 300,000/mm(3), dead vs survivor, respectively [P < .03]; posttherapeutic: 66,000 vs 388,700/mm(3) [P < .003]) and the prothrombin time (posttherapeutic, 35.0 vs 12.1 seconds [P < .0001], dead vs survivor, respectively)., Conclusion: For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including β-blocker therapy, surgery, and liver transplantation should be considered., (Copyright © 2011 Elsevier Inc. All rights reserved.)

There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

The aim of this study was to provide an overview of the central nervous system (CNS) tumours epidemiology in Karlovac region, over the 1995-2010 period. We analyzed data on 359 patients (194 men and 165 women), diagnosed with CNS tumours according to the World Health Organization's diagnostic criteria, in period 1995-2010. The data were obtained from the Neurology and Neurosurgery Department, including other medical records. The data were analysed with t-test and chi-square test. A total of 359 cases of tumours in CNS were recorded for the period of 1995-2010, with slight predominance of men (194;54.0%) over women (165;46.0%). Under the assumption of gender equality, we did not detect a significant gender difference in tumour diagnosis (p = 0.279). Mean age at the diagnosis was 64.1 +/- 12.6 years, with significant gender difference: mean age at diagnosis for men was 62.8 +/- 11.6 years, while for women it was 65.7 +/- 13.5 (p = 0.029). The commonest type of all tumours was metastases (144;40.1%). When only primary tumours were analysed, the commonest type was glioblastoma (125;58.15%), followed by meningeoma (44;20.5%). The remaining types were much less frequent, with i.e. 5 recorded cases of the following three types: astrocytoma, ependimoma and oligodendroglyoma (2.3%). These results suggest a commonly encountered epidemiological profile in the region, with commonest metastases, and glioblastoma as the most common primary tumour. Due to difficulties related to patient gravitating hospitals admittance and overall small sample size for more detailed analyses, it remains for future studies to determine potential association of the Homeland war (1991-1995) and the occurrence of CNS tumours.

Objective: The purpose of our study was to evaluate the association of hepatic hemangiomatosis with giant cavernous hemangioma (GCH) and describe the imaging appearances and clinical relevance., Materials and Methods: Forty-one patients who had undergone CT or MRI with reported GCH (> 8 cm) between 1997 and 2009 were identified retrospectively. Three readers interpreted 27 MRI studies, 36 CT studies, and 16 ultrasound studies of these patients. Prevalence, extent, and imaging appearance of coexistent hemangiomatosis in the surrounding liver parenchyma were evaluated., Results: Forty-two GCHs were identified in 41 patients and hemangiomatosis was present in 18 of 41 patients (44%) with GCH. Twelve patients had a diffuse pattern of hemangiomatosis (67%), and six patients showed a nodular pattern consisting of multiple coalescent nodules measuring < 5 mm (33%). There was no association between the size of the GCH and presence and extent of hemangiomatosis. The common hepatic artery was enlarged (> 5 mm) in 14 patients with GCH, of whom 12 had associated hemangiomatosis. There was a statistically significant association between the size of the hepatic artery and presence of hemangiomatosis (p < 0.001)., Conclusion: Hemangiomatosis is not rare in the liver parenchyma adjacent to a GCH. The presence and extent of hemangiomatosis must be specifically communicated to referring physicians. Surgical candidates have to be carefully selected to avoid complications, such as excessive blood loss and diminished risk of postoperative liver decompensation from apparent overestimation of functional residual volume due to oversight of involved liver areas by hemangiomatosis.

Background: Radiation-induced tumors (RIT) are increasingly recognized as delayed complications of brain irradiation during childhood. However, the true incidence is not established, their biology is poorly understood, and few guidelines exist regarding the long-term follow-up of irradiated children., Methods: We studied retrospectively patients irradiated for brain tumor under 18 years and followed in our institution since 1970. RIT were defined as new masses, different from the original tumor, occurring after delay in irradiated areas, and not related to phacomatosis., Results: Among 552 irradiated patients, 42 (7.6%) developed one or more RIT, 26 months to 29 years after irradiation (mean 12.8 years). The cumulated incidence was 2.0% at 5 years and 8.9% at 10 years. Of the patients, 73.8% were adult at the time of diagnosis of RIT, and 75% were diagnosed within 18.1 years after irradiation. We identified 60 cavernomas, 26 meningiomas, 2 malignant gliomas, 1 meningosarcoma, and 6 thyroid tumors. Compared with meningiomas, cavernomas appeared earlier, in children irradiated at an older age, and with a male predominance. Although RIT were correlated with higher irradiation doses, 80.9% of these occurred at some distance from the maximum irradiation field. Twenty-five lesions were operated in 20 patients; three patients died because of progression of the RIT., Conclusion: A significant number of patients undergoing irradiation for brain tumor during childhood develop a RIT, often during adulthood. Our data suggest that radiation-induced cavernomas result from angiogenetic processes rather than true tumorigenesis. Protracted follow-up with MRI is warranted in children irradiated for brain tumor., (© Springer-Verlag 2011)

Intramedullary spinal cord cavernous hemangiomas (angiomas) are occult vascular malformations characterized by rare lesions consisting of closely packed capillary-like vessels. In general, patients with this disease become symptomatic because of hemorrhage leads to progressive neurological deficits. Therefore, surgical tumor resection should be considered for symptomatic patients should be considered a surgical tumor resection.

We conducted a retrospective study to determine the incidence and characteristics of submandibular gland hemangioma at our institution. We reviewed the records of all patients who had undergone submandibular gland excision from January 1998 through December 2006. We found a total of 230 such cases. Of these, submandibular gland hemangioma was found in 4 patients (1.7%)-3 women and 1 man, aged 20 to 47 years (mean 34.8). Their duration of symptoms had ranged from 26 to 78 months (mean: 49.3). These symptoms had included submandibular swelling, pain or discomfort, and features of sialadenitis. Computed tomographic angiography had revealed that the hemangiomas were supplied by the facial and lingual arteries. Two of the 4 patients had undergone preoperative vascular embolization, but it had failed to significantly reduce the amount of intraoperative blood loss. Final histopathologic examination had revealed that all 4 lesions were cavernous hemangiomas and that they had replaced the normal glandular structure. No recurrence was seen during a follow-up that ranged from 47 to 72 months (mean: 56.3).

Objective: To describe the clinical presentation, diagnosis, and management of patients presenting with intracranial cavernous angiomas (CAs) at a tertiary care hospital in Pakistan., Study Design: Case series., Place and Duration of Study: Section of Neurosurgery, The Aga Khan University Hospital (AKUH), Karachi, from January 1990 to September 2008., Methodology: A retrospective case note review of all patients diagnosed with intracranial CAs at AKUH during the study period. The studied variables included patient demographics, clinical presentation, family history of intracranial CAs or other space occupying lesions, modality of diagnosis, management, and outcome. Results were described as frequency percentages., Results: During the review period, 18 patients (11 males and 7 females) were diagnosed with intracranial CAs. The median age at diagnosis was 28.5 years. The most common location of malformations was cerebrum (n=13) followed by brain stem (n=3), and cerebellum (n=2). Nine patients had multiple lesions. Family history was present in 2 patients. Seizures and focal neurologic deficits were the main clinical manifestations. The detection rate was 93.8% with magnetic resonance imaging, but less with angiography and computerized tomography. Ten cases were treated surgically; 8 were managed conservatively. The outcome was satisfactory except for one patient, who died within 6 months of diagnosis. Surgery was performed for gross haemorrhage, rapidly increasing neurologic deficits, and intractable or long-standing seizures., Conclusion: Cavernomas tend to occur at younger age in females than males. This data raises a possibility of a higher frequency of multiple cavernomas in Pakistani patients. The main clinical manifestations are seizures and focal neurologic deficits. MRI is most sensitive and specific neuroradiologic modality for detecting this vascular malformation.

Background: Cavernous angioma is a vascular malformation which can be found in any region within the central nervous system., Objectives: There are few clinical and demographic cavernous angioma studies with large sample sizes. Therefore, the present study was designed to provide further information on the clinical and demographic characteristics of cavernous angioma using a relatively large sample of Persian patients., Methods: Patients with cavernous angioma were recruited from the outpatient neurology clinics in Isfahan, Iran, from October 2003 to October 2006., Results: In all cases, the diagnosis of cavernous angioma was based on brain magnetic resonance imaging. There were 35 patients (female/male: 17 : 18) identified with cavernous angioma. The mean age at presentation was 28.8 years. Initial manifestations included seizures in 16, headache in 11 and intracranial hemorrhage in eight patients. During follow-up, all patients experienced seizures and 19 developed headaches. Depression, vertigo, nausea, vomiting, disequilibrium, loss of consciousness and sensorimotor symptoms were also observed., Conclusion: Some of the findings of the present study were in accordance with previous studies. However, more of our patients with positive family history had solitary rather than multiple lesions, and more of our patients had generalized tonic-clonic seizures rather than partial seizures. Moreover, our data demonstrated that if there is a history of cavernous angioma with intracranial hemorrhage in family members, the presenting cavernous angioma patient is more prone to intracranial hemorrhage.

Purpose: A representative compilation of data on the incidence and prevalence of benign cutaneous vascular neoplasms and malformations in childhood has been made., Patients, Materials and Methods: A review of the literature has been made and basic data from an own epidemiological survey in the province of Tyrol (Austria) in 6-year-old children are evaluated., Results: For capillary malformations with spontaneous regression (salmon patches), the reported numbers usually vary between 20 and 30% of the newborns, while true, persisting capillary malformations (port-wine stains) can be found in about 1%. Strawberry angiomas are found in about 3% of mature newborns but in up to 12.5% of preterm children. Complex vascular malformations or severe cases of vascular tumours are very rare., Conclusion: All in all, vascular lesions are very common findings in childhood, but in most cases harmless and transient in nature. The number of lesions that may require adequate but usually uncomplicated treatment amounts to about one percent of children (strawberry angiomas and port-wine stains). Complex and/or life-threatening severe vascular tumours and malformations that represent a considerable therapeutic challenge are extremely rare events.

Objective: The presentation and natural history of untreated, symptomatic intramedullary spinal cavernomas at our institution were analyzed. The objective is to provide additional information regarding the natural history of conservatively managed, symptomatic, intramedullary spinal cord cavernous malformations., Methods: The medical records of patients treated in our institution between 1989 and 2002 were reviewed to identify those with intramedullary cavernomas. The medical, radiological, surgical, and pathological records from these patients were retrospectively reviewed and analyzed., Results: Fourteen patients were included in the study. The mean age at presentation was 42 years. Four lesions (29%) were located in the cervical region and 10 lesions (71%) were present in the thoracolumbar spinal cord. All patients were symptomatic at the time of presentation. In this cohort of 14 patients, 10 patients (71%) were conservatively managed. For these patients, the mean duration of symptoms before presentation was 10 months. The mean duration of follow-up from the time of presentation was 80 months. The median McCormick grade for conservatively treated patients at presentation was II. During this period, none of the conservatively managed patients had an acute intramedullary bleed. In nine patients, the McCormick grade at the last follow-up evaluation was the same as or better than their score at presentation. Four patients (29%) were treated surgically. The mean duration of symptoms before presentation was 33 months. The mean duration of follow-up from the time of presentation was 42 months. In two surgical patients, the McCormick grade at the last follow-up evaluation remained unchanged compared with their score at presentation, whereas the McCormick grade improved in one patient and deteriorated in another patient., Conclusion: This cohort of conservatively managed patients with symptomatic, intramedullary spinal cord cavernomas was clinically stable throughout the follow-up period. In this series, patients harboring symptomatic spinal cord cavernous malformation did not have significant, permanent neurological decline during the follow-up period when treated with the conservative approach of observation. This data provides additional information for determining the appropriate treatment strategy for patients with intramedullary spinal cavernomas.

Object: Cavernous hemangiomas (cavernomas) are benign vascular malformations that may cause seizures and/or hemorrhage when they develop in the brain. The incidence of cavernoma development after brain radiotherapy is unknown. The aim of this study was to assess the prevalence of cavernoma formation in patients who had previously received radiotherapy for brain tumors during childhood., Methods: All patients were identified who were younger than 16 years of age and had undergone radiotherapy for brain tumors within a 16-year period (January 1, 1988-December 31, 2003). The patient data that were ascertained included age at diagnosis, sex, histopathology results, initial preoperative magnetic resonance (MR) imaging results, date of radiotherapy, and date of detection of cavernoma. Children who were followed up for less than 1 month after radiotherapy or who died during treatment were excluded, as were children with brainstem tumors. All patients had undergone diagnostic MR imaging before receiving radiotherapy, and no vascular malformations were revealed. Of the 379 patients identified, 297 satisfied the inclusion criteria. Ten patients (3.4%) developed cavernomas after radiation therapy. The ages of these patients ranged from 2 to 11 years at the time of radiotherapy (median 7 years), and the latency interval between radiotherapy and cavernoma development was 3 to 102 months (median 37 months)., Conclusions: The prevalence of cavernomas in the present study was more than six times greater than the prevalence rate cited in the literature for this population. The authors conclude that there is an increased risk of developing an intracranial cavernoma after radiotherapy for brain tumors. The possibility of this complication arising should be mentioned when informed consent is sought before treatment using radiotherapy.

Objectives: To define the frequency of retinal lesions in a large panel of patients with familial cerebral cavernomas and to screen the cerebral cavernous malformation genes in patients with cerebral and retinal lesions., Methods: Fundus examination was proposed to each of the index patients of 70 families with cerebral cavernous malformation who have been included in a prospective clinical and neuroradiological follow-up. All of the coding exons of the KRIT1, MGC4607, and PDCD10 genes were screened as previously described., Results: Of the 70 index patients, 60 were consecutively examined. The 10 remaining patients refused the fundus examination. Three of the 60 examined patients had a retinal cavernoma diagnosis. Three mutations were found: a point mutation within exon 5 of the KRIT1 gene, a large deletion that encompassed exons 1 and 2 of the MGC4607 gene, and a large genomic de novo deletion encompassing the whole PDCD10 gene., Conclusions: Retinal cavernoma frequency can be estimated to be about 5% of the patients with familial cerebral cavernomas. Retinal cavernomas are not restricted to KRIT1 mutation carriers but can be observed in patients carrying a mutation in any of the 3 cerebral cavernous malformation genes., Clinical Relevance: Five percent of patients with familial cerebral cavernomas have retinal cavernomas. These lesions are clinically asymptomatic. They can be associated with any of the 3 cerebral cavernous malformation genes.

Object: The goal of this study was to determine the incidence of radiation-induced cavernomas in children treated for medulloblastoma., Methods: A retrospective chart and film review was performed for all patients treated for medulloblastoma at the Insitute for Neurology and Neurosurgery/Beth Israel Medical Center between August 1996 and the present. The clinical and radiographic histories of pediatric patients (ages 3-21 years at diagnosis) with a histologically confirmed diagnosis of medulloblastoma who received craniospinal radiation therapy were reviewed. Fifty-nine patients were identified, with a mean age at radiation treatment of 7.7 years and a mean follow-up time of 7.2 years. The dose to the craniospinal axis was 24 Gy (31 patients) or 36 Gy (28 patients). The radiation energy in the craniospinal axis was provided by photons in 55 patients and protons in four. All patients received a posterior fossa boost of 54 Gy (46 patients) or 72 Gy (13 patients). Twenty-six lesions developed in 18 patients (31%) during the observation period. The cumulative incidence of lesion development was 5.6, 14, and 43%, at 3, 5, and 10 years, respectively. The sites of occurrence were cerebral (20 cases) and cerebellar (six cases). There was no significant correlation between age at diagnosis, sex, craniospinal radiation dose or energy source, and lesion development. Only one patient required surgical intervention for a symptomatic hemorrhagic lesion in the frontal lobe. Histological analysis in this case was consistent with cavernoma., Conclusions: Cavernomas are common after cranial irradiation in children, and their incidence increases over time. Most of these lesions follow a benign course and do not require intervention.

Cavernous hemangiomas of the adrenal glands are extremely rare benign lesions and most are incidental findings. The increasing frequency of the adrenal masses detection is due to the widespread use of the contemporary imaging techniques. We review incidence, pathology, clinical features, diagnostic and therapeutic treatment of the adrenal cavernous hemangiomas.

166 patients with arterio-venous malformations (AVM) were treated in the Department of Neurosurgery at Silesian School of Medicine from 1987 to 2002. There were 30 (18%) patients diagnosed as cavernous angioma (CA). The oldest treated patient was 73 years old and the youngest one was 5 years old. 16 (55%) male and 14 (45%) female were examined and the results of this examination are presented below. The location of CA was as follows: supratentorially--19 patients (64%), infratentorially--6 patients (20%), extracranially--5 patients (16%). During the admission the clinical status of patients were evaluated according to Glasgow Coma Scale (GCS) and Hunt-Hess scale (H-H). Epilepsy occurred in 5 patients (16%), neurological focal deficits--14 (45%), intracranial hemorrhage--7 (23%). All patients were examined using CT (computer tomography) scan, cerebral angiography was carried out in 15 (50%) patients and MRI (magnetic resonance imaging) in 16 (55%). All patients were operated on in our medical centre. The results of treatment were presented according to Glasgow Outcome Scale (GOS).

A case of asymptomatic right atrial cavernous hemangioma in a 50-year-old man is reported. With right atriotomy under total cardiopulmonary bypass, the right atrial mass was identified as arising from the noncoronary Sinus of Valsalva. The mass was successfully resected with the Sinus of Valsalva closed primarily. The patient is alive and well 9 months following the operation. The incidental discovery of the mass, clinical evaluation, operative procedure, and pathologic findings are reported. We also briefly discuss the epidemiology, natural history, and the diagnostic and therapeutic approaches to cardiac tumors.

Introduction: Cavernous hemangioma is the most frequent focal liver lesion. It affects mainly women and may cause symptoms such as abdominal pain, mass, and early satiety, or complications such as heart failure or coagulopathy. There are several options for treatment in symptomatic patients. However, it seems that surgical resection is the only curative treatment., Aim: Evaluate indications and results of liver resection in patients with cavernous hemangiomas and hepatic hemangiomatosis., Patients and Methods: We conducted a retrospective analysis of clinical files of patients treated at the Instituto Nacional de Cancerología (INCan) and the Centro Médico ISSEMYM during a 8-year period. Epidemiological data as well as diagnostic work-up and treatment were analyzed., Results: From August 1995 to May 2003, 24 patients with liver hemangiomas were resected at both institutions. Twenty three were female (95.8%) and one, male (4.1%). Indications for surgery were presence of symptoms in 20 patients (83.3%), undefined diagnosis in three (12.5%), and rapid growth in one (4.1%). Most frequent symptoms were abdominal pain in 20 (83.3%) patients, followed by abdominal mass in five (20.8%), and early gastric satiety in four (16.6%). Abdominal computed tomography (CT) scan was the most frequent imaging study used in 22 patients (91.6%), followed by ultrasound in 20 (83.3%). Size of lesion ranged from 4-30 cm (X: 8.7 cm), 16 patients were submitted to formal liver resection (66.6%), and eight to enucleation (33.3%). Four patients presented operative complications (16.6%) that included postoperative bleeding in two (8.3%), fever in one (4.1%) and abdominal haematoma in one (4.1%). There was no operative mortality. Twenty two patients were asymptomatic at time of evaluation (91%)., Conclusions: This lesion affects mainly women, and presence of symptoms is the most common indication for treatment. Choice of surgical procedure to be carried out depends on location and morphology of the lesion. Liver resection or enucleation are safe forms of treatment that properly controlled symptomatology.

Vascular malformations of the orbit can best be understood within the context of their hemodynamics, of which there are three types. Type 1 (no flow) lesions have essentially little connection to the vascular system and include lymphangiomas or combined venous lymphatic malformations. Type 2 (venous flow) lesions appear as either distensible lesions with a direct and rich communication with the venous system or nondistensible anomalies that have minimal communication with the venous system. Types 1 and 2 can be combined with both features of distensible and nondistensible hemodynamics. Arterial flow lesions, type 3, include arteriovenous malformations characterized by direct antigrade high flow through the lesion to the venous side. Cavernous hemangiomas are also malformations that demonstrate direct low flow through the lesion.

We report our experience with 9 cases of brain stem cavernoma in children aged 15 years during the period 1987-2000. All patients were admitted with lesion-related symptoms, except in one where the brain stem cavernoma was discovered on a routine MR scan. A female predominance was noted in the present study. In 2 children, brain stem cavernoma was associated with multiples lesions. All patients except two with multiple cavernomas underwent surgery. One child died 2 months after surgery from recurrent hemorrhage. The eight others are alive. Among the 8 survivors, only one has worsened. Children with brain stem cavernoma who develop persistent or progressive deficit are candidates for surgery. When surgery is indicated, total removal is mandatory to avoid the risk of recurrence and rebleeding.

The authors review the pertinent literature dealing with all aspects of cerebral cavernous malformations in the adult. Clinical, neuroradiological, pathological, and epidemiological aspects are presented. The clinical significance of bleeding from cavernous malformations and various hemorrhage patterns are discussed in relation to the factors that influence hemorrhage rates. Recent reports describing the genetic mechanisms of inheritance, de novo formation, and angiogenesis of cavernomas are reviewed as well. Brainstem cavernomas have received special attention, since their clinical management is controversial in the literature. Presently, microsurgical removal is favored by the majority of authors and stereotactic radiosurgery appears to be inappropriate for prevention of bleeding from a cavernoma. Our own case material consists of data of 72 patients operated upon during the past 5 years. Twenty-four patients harbored the lesion within the brainstem, 18 within the deep white matter of the hemispheres, 12 in the basal ganglia or thalamus, 11 in superficial areas of the hemisphere, and seven within the cerebellum. The perioperative morbidity rate was 29.2% (21/72) while the rate of long-term morbidity was 5.5% (4/72), with no mortality in this series. It is concluded that cerebral cavernous malformations, including lesions in critical regions of the brain, can be treated microsurgically with excellent results and an acceptable morbidity.

Purpose: To determine the types and frequency of orbital tumors in the older adult population., Design: Retrospective consecutive case series., Participants: Two hundred consecutive patients aged 60 years or older with an orbital tumor managed at a tertiary referral center., Main Outcome Measures: The relative incidence and management of orbital tumors in patients aged 60 years or older., Results: Of approximately 950 consecutive patients with orbital space-occupying lesions managed over a 25-year period on the Oncology Service, 200 patients (21%) were 60 years or older at the time of diagnosis. The orbital tumor was unilateral in 183 patients (92%) and bilateral in 17 patients (8%). The most common clinical features at presentation included mass in 51 patients (26%), proptosis in 36 (18%), and pain in 30 (15%). The mean duration of symptoms was 11 months before referral. The orbital tumor was malignant in 125 patients (63%) and benign in 75 (27%). The most common diagnoses were malignant lymphoma in 47 patients (24%), idiopathic orbital inflammation in 19 (10%), and cavernous hemangioma in 15 (8%). The diagnosis was made with clinical findings and imaging studies in 58 patients (29%) and additional histopathologic examination in 142 patients (71%). Of 217 tumors, the main tumor location was in the superior half of the orbit in 106 lesions (49%), in the anterior orbit in 100 (46%), and in the extraconal space in 135 (62%). Imaging studies showed a mass with well-defined margins in 90 tumors (41%) and ill-defined margins in 127 (59%). The tumors were solid in 203 cases (94%), cystic in 6 (3%), and mixed in 8 (3%). Over a mean of 31 months follow-up, 50 (25%) patients had orbital tumor-related systemic involvement (metastasis or lymphoma), and 150 (75%) remained free of systemic involvement., Conclusions: Orbital tumors in the senior adult population are malignant in 63% of cases. Malignant lymphoma is the most common tumor in this age group, accounting for 24% of cases. Overall, 25% of patients have systemic problems related to the orbital process develop, so systemic evaluation is warranted.

From 1983 through 1997, our center diagnosed 130 cases of benign neoplasms: 27 with focal nodular hyperplasia (FNH), 25 with hepatic adenoma, 71 with cavernous hemangioma, and seven with mixed tumors of different diagnoses. Most often these lesions were seen in females [female-to-male ratio (f/m): 5.5/1]. Hepatic adenomas and mixed tumors were seen exclusively in females and FNH predominantly in females (f/m: 26/1). Hemangiomas, however, were not uncommon in men (f/m: 52/19) relative to the other tumors (P < 0.001). Furthermore patients with hemangioma were older (mean age: 49 years) whereas patients with hepatic adenoma, FNH, and mixed tumors were often younger (mean age: 33, 35, and 44 years respectively; P < 0.004). Oral contraceptive steroid use was related by 21 of 25 patients (84%) with hepatic adenoma, 22 of 26 (85%) females with FNH, five of seven (71%) females with mixed tumors, and 10 of 52 (19%) patients with hemangioma. Ninety-five of the 130 patients (73%) had one or more symptoms. There was no statistically significant correlation between symptoms and the size of the lesion, the final diagnosis, and whether there were solitary or multiple masses. Three of 25 (12%) with hepatic adenoma presented with rupture, and one of 27 (4%) with FNH had such a consequence. None of the hemangiomas presented with rupture or progressed to such a state. One patient with hepatic adenoma (4%) had a focus of malignancy. Surgical removal of benign tumors was performed in 82 of 130 patients (63%), and there was one operative mortality (1.2%) in a patient who had a caudate lobe FNH. The types of surgical procedures included segmentectomy (62%), lobectomy (34%), and trisegmentectomy (4%). In two of 84 patients who had undergone laparotomy resection was not technically possible. Resection is recommended in all cases of hepatic adenoma because of fear of rupture or associated focus of malignancy. FNH was not observed to undergo a malignant transformation and will rarely rupture. Surgery is only recommended for symptomatic hemangioma, and size of the lesion is not a criterion for excision.

The case of an ovarian cavernous hemangioma with torsion in an 8-year-old girl is described. Current literature records less than 50 cases of which only 8 are in children. The presenting symptoms of acute abdomen and the ultrasonographic study led to the preoperative diagnosis of torsion of an ovarian tumor. Salpingo-oophorectomy and appendicectomy were performed with an uneventful postoperative course. The histological pattern of the tumor was that of an entirely cavernous hemangioma. The case is reported in view of its rarity.

Intramedullary spinal cord cavernous haemangiomas are rare lesions that can cause severe myelopathic symptoms. The purpose of the present study was to define the pattern of clinical presentation, part of natural history, prognostic factors and therapeutic strategies considering both our own experience and reports from the literature. The data of 48 studies (published between 1903 and 1996), presenting information of all together 107 patients (108 lesions) regarding pre-treatment clinical and radiological factors, treatment strategies, and the outcome, plus our own experience of nine patients were retrospectively re-analyzed. The prognostic influence of pretreatment factors was estimated with the chi-square statistics. Clinical evaluation before/after treatment was performed using the Frankel scale. The average bleeding rate was obtained from the ratio of percentage of first bleeding events in the population to the mean age of the population. There were 47 males and 69 females (aged from twelve to 88 years). Thirty nine percent of the lesions were found in the cervical, 54% in the thoracic (30% upper, 24% lower) and 7% in the lumbar cord. The peak age of presentation was in the fourth decade, the median duration of symptoms was 32 months. Clinical symptoms before treatment were progressive in all cases. Three patterns of clinical presentation could be identified: a) episodes of stepwise clinical deterioration (30%), b) slow progression of neurological decline (41%), c) acute onset with rapid or gradual decline over weeks or months (26%). 58% of the lesions showed clinical or radiological signs of haemorrhage. In 66% of surgical patients (91 efficiently documented cases), clinical improvement was achieved, 28% remained unchanged and 6% deteriorated. Whereas age, sex and lesion location had no influence on the results, duration of symptoms (< three years) correlated significantly to a better outcome (p < 0.02). Surgical management in symptomatic patients is recommended. Once clinical signs caused by the malformation have appeared, the patients tend to experience progressive neurological deterioration.

Background: Cavernous angiomas, which are vascular malformations mostly located in the central nervous system, may be inherited as an autosomal dominant disorder known as familial cerebral cavernoma (FCC). FCC has been studied in Hispanoamerican families, in which a strong founder effect was shown. We studied the families of 57 non-Hispanic patients with cavernous angiomas., Methods: All 28 neurosurgery centres in France collaborated in the study. Inclusion criteria were: families of index patients known to have at least one clinically affected relative, and families of index patients with multiple cavernous angiomas who initially presented as sporadic cases. Clinical and cerebral magnetic resonance imaging (MRI) investigations were done in all patients and in other at-risk individuals who consented to take part., Findings: On MRI, 16 of 22 sporadic index patients had relatives with cavernous angiomas. 51 multiple-case families, including 100 patients with symptoms and 164 symptom-free individuals had MRI lesions. Most FCC patients had multiple lesions and there was a strong correlation between number of lesions and age (p<0.01). The sensitivity of gradient-echo sequences was higher than that of standard MRI for detection of small cavernous angiomas. Pattern of inheritance was autosomal dominant, with incomplete clinical penetrance. The occurrence of de-novo mutations was strongly suggested in some families., Interpretation: Neuroimaging penetrance of FCC is much higher than clinical penetrance. 75% of sporadic cases with multiple lesions are in fact familial cases. The proportion of patients developing clinical symptoms is higher in the hereditary form than in the sporadic form of the disorder.

Objectives: Anticipation has been linked to unstable trinucleotide repeats in many neurological disorders. We examined the hypothesis of genetic anticipation in familial cavernous angioma (FCA) of the central nervous system., Material and Methods: The mean ASO of affected individuals was compared between successive generations in 55 families. Intergenerational pair-wise comparisons were employed to avoid several ascertainment biases. Regarding severity of disease both type of manifestation and number of cavernous angiomas were compared between generations., Results: The mean ASO decreased significantly both from the first to the second generation (31.6 vs 17.8 years; P = 0.000) and from the second to the third generation (17.8 vs 6.7 years; P = 0.002). The pair-wise comparisons also showed significantly earlier ASO. No clear evidence for anticipation with regard to severity of disease was found., Conclusions: Molecular genetic studies will determine whether trinucleotide repeats are the underlying mechanism for our observation of anticipation in FCA.

This is a report of an extremely rare case of isolated cavernous hemangioma of the stomach found in a neonate. Hematemesis developed in a 7-day-old baby boy weighing 2.8 kg at birth. This was followed by melena. Endoscopic examination findings showed a large hemorrhagic mass on the lesser curvature of the stomach. During laparotomy, a large vascular tumor was identified and subtotal gastrectomy was carried out. On section, the tumor extended through the submucosa to the serosal surface. Histological diagnosis was cavernous hemangioma. The authors could collect only 10 cases of hemangioma of the stomach diagnosed in childhood from the world literature.

Although hepatic hemangioma is common, its rupture after trauma is rare. We report a 47-year-old woman with a traumatic hepatic hemangioma rupture following a traffic accident who successfully underwent operation. Only four cases of hepatic hemangioma rupture caused by blunt trauma have been reported including this one. Each patient (two men and two women) had a giant right lobe hemangioma. The causal injury included a traffic accident in two, a fall in one, and minor trauma in another. Three patients underwent surgery, and one was conservatively managed. All the patients survived.

One case of small bowel polypoid hemangioma of the jejunum presenting as recurrent gastrointestinal blood loss is reported. The presenting symptoms and diagnostic methods are discussed. The importance of intraoperative enteroscopy in diagnosing and treating this condition is emphasized.

Cavernous malformations (angiomas) affecting the central nervous system and retina can be inherited in autosomal dominant pattern (OMIM 116860). These vascular lesions may remain clinically silent or lead to a number of neurological symptoms including seizure, intracranial hemorrhage, focal neurological deficit, and migraine. We have mapped a gene for this disorder in two families, one of Italian-American origin and one of Mexican-American origin, to markers on proximal 7q, with a combined maximum lod score of 3.92 (theta of zero) with marker D7S479. Haplotype analysis of these families places the locus between markers D7S502 proximally and D7S515 distally, an interval of approximately 41 cM. The location distinguishes this disorder from an autosomal dominant vascular malformation syndrome where lesions are primarily cutaneous and that maps to 9p21.