Cavernous Hemangioma Between the Elastomer and Fibrous Capsule of a Breast Implant.

Hemangiomas are described in many locations, but breast hemangioma (BH) is rare, accounting for only 0.4% of all breast tumors. These tumors are difficult to diagnose preoperatively using conventional imaging modalities because they lack pathognomonic characteristics. Mammographic and sonographic appearances of BH were described in just a few case reports, and breast implant-related hemangiomas are even rarer. We report a case of the tumor arising in an atypical location-between the elastomer and fibrous capsule of a breast implant.Level of Evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Lacunar Stroke, Cavernous Angioma, and Fusiform Aneurysm Due to Irradiation for Pilocytic Astrocytoma-A Case Report.

Radiotherapy is a useful modality for the treatment of brain tumors, but may induce brain degeneration, tumor formation, and vasculopathy in the irradiated field. We describe a rare case of a pediatric patient who presented multiple different types of vascular events consecutively in the irradiated field including lacunar stroke because of occlusion of perforating artery, intraventricular hemorrhage from cavernoma, and subarachnoid hemorrhage because of the rupture of fusiform aneurysm, 6 years after radiotherapy against pilocytic astrocytoma. The life-threatening aneurysm was resected, and its histologic findings revealed the radiation-induced vasculopathy. We should avoid irradiation, and repeat surgical resection for the pediatric cases with pilocytic astrocytoma. Once irradiation was indicated for them, however, we should carefully follow-up not only tumor recurrence but also angiograms to predict any cerebrovascular events.

Electromagnetic navigation-guided neuroendoscopic removal of radiation-induced intraforniceal cavernoma as a late complication of medulloblastoma treatment.

INTRODUCTION: Medulloblastoma is the most frequent malignant brain tumour in children. Radiation-induced cavernous haemangiomas (RICHs) are a known late complication of radiation exposure, especially in young children. CASE REPORT: We present a patient who underwent subtotal resection of posterior fossa medulloblastoma with subsequent chemotherapy and radiotherapy at the age of 10 years. A new lesion in the region of the left foramen of Monro appeared 16 years later. Based on the imaging results, metastasis or radiation-induced cavernoma was considered. The lesion had the same appearance on imaging as a rarely published intraventricular cavernoma of the foramen of Monro. Unlike the cavernoma of the foramen of Monro, this lesion was subependymal and intraforniceal. Using electromagnetic navigation and neuroendoscopy, the lesion was completely removed. Histopathological examination revealed a cavernous haemangioma. CONCLUSION: This is a unique case of intraforniceal paraforaminal cavernoma that was successfully removed endoscopically using electromagnetic neuronavigation and without neurological sequelae.

Neurocutaneous Melanosis Presenting as Cavernous Hemangioma Persistent Abdominal Pain.

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the presence of multiple congenital melanocytic nevi and the proliferation of melanocytes in the central nervous system. The authors present a 9-year-old Chinese boy whose clinical manifestations are intermittent headache for 2 months and persistent abdominal pain for 10 days. 3D-reconstruction computed tomography angiography image, digital subtraction angiography, and magnetic resonance imaging plus angiography (MRI+MRA) examinations results suggested that cavernoma at left frontal lobe potentially associated with hemorrhage. In addition, miliary abnormal signals were widely scattered on MRA image so that other malignant metastatic diseases cannot be ruled out. GI physical examination had not any abnormal findings, antispasmodic drugs were ineffective but antiepilepsy drugs were effective to abdominal pain. In surgery, no cavernoma was noticed but an accumulation of densely melanocytic mass located at the lesion on radiology images. The lesions spread along with perivascular of sylvian veins and leptomeningeal. Pathology investigation demonstrated brain metastatic malignant melanoma associated with hemosiderosis. The lesion of brain parenchyma was totally removed but the spread lesions could not be treated with surgery. Adjuvant radiotherapy was performed but failed to control the malignant development, still the patient died in 3 months postinitial operation. The authors conclude that abdominal pain was a manifestation of epilepsy related to the frontal lobe lesion. Neurocutaneous melanosis is a rare disease, brain metastases result in abdominal pain is rare even more, and it is worth the attention of clinicians.

[Treatment of non-cirrhotic, non-tumoural portal vein thrombosis]. / Actuación ante la trombosis portal no cirrótica no tumoral.

Thrombosis of the splenoportal axis not associated with liver cirrhosis or neoplasms is a rare disease whose prevalence ranges from 0.7 to 3.7 per 100,000 inhabitants. However, this entity is the second most common cause of portal hypertension. Prothrombotic factors are present as an underlying cause in up to 70% of patients and local factors in 10-50%. The coexistence of several etiological factors is frequent. Clinical presentation may be acute or chronic (portal cavernomatosis). The acute phase can present as abdominal pain, nausea, vomiting, fever, rectorrhagia, intestinal congestion, and ischemia. In this phase, early initiation of anticoagulation is essential to achieve portal vein recanalization and thus improve patient prognosis. In the chronic phase, symptoms are due to portal hypertension syndrome. In this phase, the aim of treatment is to treat or prevent the complications of portal hypertension. Anticoagulation is reserved to patients with a proven underlying thrombophilic factor.

Progressive Multiple Cavernous Angiomas after Radiotherapy in a Pediatric Patient with Medulloblastoma: A Case Report.

BACKGROUND: We report a case of progressive multiple cavernous angiomas. PATIENT AND METHOD: A 16-year-old boy presented with a 2-month history of headache and dizziness. Six years earlier, he underwent surgery for cerebellar medulloblastoma and subsequent chemoradiotherapy according to the M-051 protocol of the Korean Society of Pediatric Neuro-Oncology. Follow-up brain magnetic resonance imaging (MRI) revealed a tiny hemorrhage on the cerebellum 3 months after 23.4-Gy craniospinal and 32.4-Gy boost radiotherapy. RESULT: The multiple hemorrhagic lesions had progressively developed on the whole brain without any symptoms for 6 years. On admission, MRI revealed a 1.5-cm enlarged mass with subacute hemorrhage on the right frontal area. The mass was totally removed and diagnosed as cavernous angioma. However, 5 months later, the patient complained of a headache. MRI revealed 1.4- and 0.7-cm enlarged masses on the left frontal and right temporal areas with internal hemorrhage, respectively. The left frontal mass was totally removed, and the histopathological finding was suggestive of cavernous angioma. CONCLUSION: This case showed early-developed multiple hemorrhagic lesions after radiotherapy, which had been progressive and were associated with some symptomatic cavernous angiomas. Pediatric patients with brain radiotherapy should undergo radiological check-up to identify vascular lesions, especially symptomatic patients.

Massive splenomegaly caused by cavernous hemangiomas associated with Klippel-Trenaunay syndrome: report of a case.

The etiology of Klippel-Trenaunay syndrome (KTS) is not well understood. Although splenic involvement is very rare in KTS, life-threatening events such as spontaneous rupture of a splenic hemangioma may occur. We recently performed elective splenectomy for massive splenomegaly causing uncontrollable abdominal pain in a woman with KTS. The extracted spleen weighed 4260 g, and cavernous hemangiomas in the spleen were found to be the cause of the splenomegaly. The patient's abdominal pain resolved after surgery and her postoperative course was uneventful, except for persistent bleeding from the bladder. This is a rare case of KTS with associated severe splenomegaly caused by hemangiomas.

Posttraumatic cavernous hemangioma of the skull.

A 59-year-old man presented with a slowly enlarging mass in the lateral orbital rim of the left eye for 1 year. He also reported a history of crania defect in the left front bone (6 years earlier) which was confirmed on the former computed tomographic (CT) scan. On examination, the solid mass lesion located in the left frontal extends to the lateral orbital rim of the left eye. CT scan showed a mass with poorly defined margins, which invaded the neighboring tissue and nearly damaged the bone, and located on the exact site of the preceding crania defect. Complete resection was performed, and the clinical diagnosis of cavernous hemangioma was confirmed on histopathologic examination. Skull cavernous hemangiomas are rare tumors for which the origin is not yet clear. We report a case in which the intraosseous hemangiomas developed at the same site of the preceding crania defect.

A case of more than 20 years survival with glioblastoma, and development of cavernous angioma as a delayed complication of radiotherapy.

Glioblastoma (GBM) is the most common malignant CNS neoplasm, the prognosis of which remains poor even after multidisciplinary treatment. The 5-year overall survival rate of GBM is less than 10% and has remained unchanged for more than 50 years. Because GBM patients rarely survive over a decade, only very few cases of delayed complications caused by therapy have been reported. Here, we report the case of a 24-year-old man who is still alive 21 years after surgical resection and chemoradiotherapy for GBM. This patient developed a cavernous angioma 19 years after the initial surgery as a delayed complication of radiotherapy. The diagnosis of the initial tumor was confirmed by histopathological review, which indicated that the tumor had immunohistochemical and genetic profiles consistent with GBM. Long-term survival in the case of this GBM patient likely resulted from a combination of factors, including hypermethylation of the MGMT (O(6)-methyl guanine methyl transferase) CpG island, young age at diagnosis, good performance status, and complete surgical resection of the tumor. To the best of our knowledge, this case report describes one of the longest-surviving GBM patients and is the first on radiation-induced cavernous angioma in a GBM patient.

[Secondary tumors following radiation therapy and chemotherapy : Incidence of cavernous hemangiomas]. / Zweittumoren nach Bestrahlung und Chemotherapie : Auftreten von Kavernomen.

PROBLEM: There is a risk of developing secondary central nervous system (CNS) tumors after cranial radiation therapy. Meningiomas and pituitary tumors are also increasingly treated with radiation therapy, which means that the risk of secondary tumors from radiation in children and adults must be communicated. METHODS: Studies in children show that radiation causes a 7- to 10-fold increase in subsequent CNS tumors with a cumulative incidence over 20 years ranging from 1.03 to 28.9. The latency period for the occurrence of secondary tumors ranged from 5.5 to 30 years, with gliomas developing after 5-10 years and meningiomas around 15 years after irradiation. The latency period for secondary CNS tumors in adults ranged from 5 to 34 years. CONCLUSION: After radiation treatment, tumors can rarely occur as secondary sequelae, mostly meningiomas and gliomas, but also cavernomas. The treatment and long-term results of radiation-induced CNS tumors showed no worse results than primary CNS tumors over the course of time.

High incidence of radiation-induced cavernous hemangioma in long-term survivors who underwent hematopoietic stem cell transplantation with radiation therapy during childhood or adolescence.

Radiation-induced cavernous hemangioma (RICH) is a late complication of cerebral radiation therapy. Long-term survivors of hematopoietic stem cell transplantation (HSCT) who underwent radiation therapy could be at increased risk for RICH. We investigated records of 68 patients who underwent HSCT during childhood or adolescence and were assessed by magnetic resonance imaging (MRI), including T2*-weighted imaging of the brain, annually for 5 years over a range of 6 to 29 years after HSCT. We developed a scoring and grading system for RICH to monitor the process and the progress of radiologic changes. Among the 68 patients investigated, 28 (41.2%) were diagnosed with CH. All 28 patients had received total body irradiation as a conditioning treatment for HSCT and/or cranial radiation therapy before HSCT as part of the treatment of their primary disease. RICH was diagnosed in none of the patients who did not receive radiation (n = 19), in 46.2% of those who received 6 to 12 Gy (n = 39), and in all of those who received 18 to 36 Gy (n = 10). Total RICH scores were correlated with higher radiation doses. Careful and long-term evaluation with MRI, including T2*-weighted imaging, is necessary for HSCT recipients who received radiation therapy before and/or during HSCT.

Therapy-associated secondary tumors in patients with non-germinomatous malignant germ cell tumors.

We report three patients with non-germinomatous malignant germ cell tumor (NGMGCT) who developed therapy-associated secondary tumors. They were diagnosed as having NGMGCT by elevated serum levels of α-fetoprotein (AFP), human chorionic gonadotropin (HCG), or ß-HCG. Preoperatively, all patients received a combination of etoposide and platinum-based chemotherapy and radiotherapy; neo-adjuvant therapy (NAT) was followed by complete excision of the residual tumor. Postoperatively, all underwent maintenance chemotherapy and all remained free of NGMGCT without recurrence. However, they developed therapy-associated secondary tumors, i.e. glioblastoma, meningioma, or cavernous angioma after 10.1, 9.8, and 8.2 years, respectively. The patient with glioblastoma died one year after its detection. The other two patients are currently alive; the meningioma was completely removed and the cavernous angioma is being monitored without additional treatment. To the best of our knowledge, therapy-associated secondary tumors in patients treated for NGMGCT are rare.

Radiation-induced tumors in children irradiated for brain tumor: a longitudinal study.

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.

Cerebral cavernoma: an emerging long-term consequence of external beam radiation in childhood.

The long-term effects of cranial external beam radiotherapy are emerging as survival rates for cerebral tumours improve. Cerebral cavernoma are a recognized consequence of cranial irradiation. Endocrinologists managing the life-long complications of hypopituitarism associated with irradiation need to be aware and vigilant of the risks of cavernoma formation, in particular in the population with a history of childhood irradiation. We present three cases of young patients who were diagnosed with cerebral cavernoma many years after childhood irradiation treatment and review the current literature on this condition. We discuss implications for endocrine practice as rising numbers of patients survive childhood cancer and irradiation and are now attending adult endocrine services for long-term management of secondary hypopituitarism.

Synchronous radiation-induced orbital meningioma and multiple cavernomas.

Synchronous radiation-induced tumours are extremely rare. We present the first reported case of synchronous radiation-induced orbital meningioma and cavernomas of the cerebellum and bilateral basal ganglia, presenting 16 years after ionizing radiation therapy for parietal anaplastic ependymoma, at the age of five. This case again underscores the risks of radiotherapy to children.

Incidental resolution of a radiation-induced cavernous hemangioma of the brain following the use of bevacizumab in a child with recurrent medulloblastoma.

Radiation-induced cavernous hemangiomas (RICH) are a known complication of radiation exposure, especially in young children. The current treatment approaches to these lesions include observation and surgical resection. We report the case of a 4-year-old male with recurrent medulloblastoma who had resolution of an incidental RICH lesion while being treated with bevacizumab for his recurrent brain tumor. There was no evidence of worsening hemorrhage with this therapy and the RICH did not recur upon discontinuation of the chemotherapy regimen. This is the first documented case of a RICH lesion responding to antiangiogenic therapy, suggesting the possible use of this class of agents in the treatment of symptomatic patients who are not considered appropriate candidates for surgical resection. Although the risk of bleeding must be taken into consideration, antiangiogenic therapies have the potential to be a novel treatment modality for symptomatic RICH lesions.

[Intramedullary tumours and pseudotumours]. / Moelle épinière tumorale et pseudo-tumorale.

Swelling of the spinal cord and/or enhancement after intravenous gadolinium administration are not always specific features of intramedullary tumour. These may also be seen in association with several diseases of inflammatory, infectious, granulomatous or vascular origin. A tumour is characterized by its sagittal location, axial topography: central, lateral or exophytic, its size and size of the spinal canal, macroscopic components: calcium, fat, methemoglobin, melanin, hemosiderin, vascular pedicle, cystic component, enhancement after intravenous gadolinium administration, effect on the spinal cord tracts and edema. Characteristics: astrocytoma is lateral and infiltrative, ependymoma is central with white matter tract displacement and hemosiderin cap, hemangioblastoma is postero-lateral and shows enhancement with a vascular pedicle, metastases are very edematous or leptomeningeal in location.

Radiation-induced cavernous hemangiomas: case report and literature review.

The case of a 51-year-old man diagnosed with two acquired cavernous hemangiomas 17 years after cranial irradiation for a cerebellar astrocytoma is reported. A review of 84 cases of radiation-induced cavernous hemangiomas found in the literature is presented. In this series the mean age at the time of irradiation (+/- SD) was 10.4 +/- 2.0 years (median = 8 years), while the mean time to cavernous hemangioma diagnosis (+/- SD) was 10.3 +/- 1.9 years (median = 8 years). Time to cavernous hemangioma diagnosis was found to be inversely related to radiation dose. Hemorrhage from radiation-induced cavernous hemangiomas was found in 40.0% of patients, with an incidence of 3.9% per patient year. An inverse trend was identified between radiation dose and symptomatic presentation, cavernous hemangioma hemorrhage or surgical resection. This review of radiation-induced cavernous hemangiomas confirms that both younger patients and those who received a larger dose of radiation are at increased risk of radiation-induced cavernous hemangiomas. Our results suggest that, based on an assessment of CT or MR images, there may be an increased risk of hemorrhage when comparing radiation-induced to congenital cavernous hemangiomas. Increasing radiation doses appear to stabilize these lesions, decreasing the risk of a symptomatic presentation, cavernous hemangioma hemorrhage and surgical intervention.