[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.

Resección laparoscópica de tumor hepático / Laparoscopic Resection of Hepatic Tumor

Introducción: La cirugía laparoscópica desde su introducción en 1987 ha experimentado un enorme desarrollo. La cirugía hepática laparoscópica de los tumores hepáticos plantea diversas controversias: los cirujanos deben estar formados en cirugía hepática y cirugía laparoscópica, la técnica laparoscópica más adecuada no está bien definida (totalmente laparoscópica o asistida con la mano), el abordaje o disección de grandes vasos entraña riesgos importantes, los métodos de sección parenquimatosa están menos desarrollados que en la cirugía hepática abierta entre otros retos. A pesar de estos criterios existen situaciones en que la cirugía puede llevarse a cabo con seguridad, sobre todo en tumores benignos y malignos cuando las lesiones se localizan principalmente en segmentos anteriores del hígado. Objetivo: Presentar una paciente portadora de una lesión hepática intervenida quirúrgicamente por videolaparoscopía con buenos resultados y evolución excelente. Caso clínico: Paciente femenina de 42 años de edad; antecedentes de salud con presencia de dolor abdominal de tres meses de evolución en región de hipocondrio derecho y trastornos dispépticos esporádicos. Se confirmó imaginológicamente un tumor de 5 cm de diámetro en segmento III, de aspecto hemangiomatoso con componente mixto y sospecha de malignidad. Se realizó resección laparoscópica, el diagnóstico anatomopatológico fue nódulo regenerativo, tipo nódulo cirrótico con hemangioma cavernoso de localización periférica en hígado sano, sin evidencias de malignidad en los estudios realizados. Conclusiones: Las resecciones quirúrgicas de lesiones hepáticas abordables por vía laparoscópica son una opción terapéutica segura y muy válida en pacientes con indicaciones específicas(AU)

Introduction: Laparoscopic surgery, since its introduction in 1987, has undergone enormous development. Laparoscopic hepatic surgery of liver tumors raises several controversies: surgeons must be trained in hepatic surgery and laparoscopic surgery, the most appropriate laparoscopic technique is not well defined (totally laparoscopic or hand-assisted), the approach to or dissection of large vessels involves significant risks, the methods for parenchymal section are less developed than in open hepatic surgery, among other challenges. Despite these criterions, there are situations in which the surgery can be performed safely, above all in benign and malignant tumors when the lesions are located mainly in anterior segments of the liver. Objective: To present the case of a patient with a hepatic lesion and operated on by videolaparoscopy with good outcomes and excellent evolution. Clinical case: A 42-year-old female patient with a family history of disease presented abdominal pain of three months of evolution in the right hypochondrium and sporadic dyspeptic disorders. Imaging confirmed a tumor of 5 cm of diameter in segment III, with hemangiomatous aspect, mixed component and suspicion of malignancy. Laparoscopic resection was performed; the anatomopathological diagnosis was regenerative nodule, similar to a cirrhotic nodule with cavernous hemangioma of peripheral location in healthy liver, with no evidence of malignancy in the performed studies. Conclusions: Surgical resections of hepatic lesions that can be laparoscopically approached are a safe and very valid therapeutic option in patients with specific indications(AU)

Hemangiomas of the tongue and the oral cavity in a myotonic dystrophy type 1 patient: A case report.

RATIONALE: Myotonic dystrophy type 1 (DM1) is an autosomal dominant disease caused by a cytosine, guanine, thymine (CTG) trinucleotide repeat expansion in the non-coding region of dystrophia myotonica protein kinase gene, causing a multisystem involvement. To date, few studies have been performed to evaluate skin features in DM1 patients, but none reported on the possible association between the disease and tongue hemangiomas. PATIENTS CONCERNS: We report a case of a 63-year-old woman affected by DM1 and presenting, at the intraoral examination, several swelling and buish lesions occurring on buccal and palatal mucosa, and in the anterior two-thirds and margins of the tongue. DIAGNOSIS: Multiple tongue hemangiomas in DM1 patient. INTERVENTIONS: Color Doppler ultrasound revealed hypoechoic lesions with intermittent color picking suggestive of vascular lesion. Surgical excision was performed under general anesthesia. Histopathological examination was compatible with the diagnosis of cavernous hemangiomas. OUTCOMES: At 6 months follow-up, a part from the cosmetic deformity, patient's hemangiomas did not bleed, but caused functional problems with speaking, mastication, and deglutition, in addition to the same symptoms induced by DM1. LESSONS: This case may add new details to better characterize the DM1 phenotype, suggesting that even tongue hemangiomas may be part of the DM1 multisystem involvement.

Cavernous liver hemangioma complicated with spontaneous intratumoral hemorrhage: a case report and literature review.

Hemangiomas, the most common benign tumors of the liver, have a prevalence of approximately 20% and are more frequent in women. According to previous studies, the size and location of the tumor are correlated with the appearance of symptoms and complications. Cases of hemangiomas complicated by spontaneous intratumoral hemorrhage have been rarely reported in the literature. Here, we report the case of a 70-year-old woman admitted for persistent upper abdominal pain. The patient showed signs of anemia, inflammatory markers and a transient increase in creatinine levels, which were corrected by conservative treatment. Our patient denied the previous use of estrogen derivatives, smoking or alcohol consumption. Native computed tomography identified a liver mass measuring 73×63 mm, located in segment IV and bulging out of the anterior contour of the liver. The mass was surgically removed by hepatic segmentectomy, and histopathological examination identified a cavernous hemangioma complicated by intratumoral hemorrhage. The postoperative outcome was favorable. After a literature review, we identified 19 other cases of hepatic cavernous hemangioma complicated by intratumoral hemorrhage reported worldwide.

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.

Case report of a modified Meso-Rex bypass as a treatment technique for late-onset portal vein cavernous transformation with portal hypertension after adult deceased-donor liver transplantation.

RATIONALE: Portal vein thrombosis is a complication after liver transplantation and cavernous transformation of the portal vein (CTPV) is a result of portal vein thrombosis, with symptoms of portal hypertension revealed by an enhanced CT scan. Meso-Rex bypass is an artificial shunt connecting the left portal vein to the superior mesenteric vein and is mainly used for idiopathic cavernomas. This technique is also used for post-transplant portal vein thrombosis in pediatric patients thereby bypassing obstructed sites of the extrahepatic portal vein. Here we report about an adult patient who was treated by connecting the cystic part of the portal vein to the splenic vein instead of the superior mesenteric vein. PATIENTS CONCERN: An adult male patient with post-liver transplantation portal vein cavernous transformation suffered from hypersplenism and elevated hepatic enzymes. DIAGNOSIS: The last follow up revealed irregular and obvious hypersplenism, and splenomegaly had occurred, while an enhanced CT scan revealed serious esophagogastric varices and CTPV in addition to occluded right and common PV trunks. INTERVENTION: The patient was treated by connecting the cystic part of the portal vein to the splenic vein instead of the superior mesenteric vein. OUTCOME: After the operation, a satisfactory velocity was confirmed 1 month postoperatively and the shunt still remained patent at the 6-month postoperation follow-up. LESSONS: A Meso-Rex bypass intervention connecting the left portal vein to the splenic vein instead of the superior mesenteric vein after liver transplantation in an adult patient with right and common portal vein occlusions has been successfully performed as an alternative approach.

Microbleeds and cavernomas after radiotherapy for paediatric primary brain tumours.

BACKGROUND: With the expected growth and aging of the population of primary central nervous system tumours (PCNST) survivors, attention to the radiation-induced late brain injury is fundamental. Late focal hemosiderin deposition (FHD) lesions, namely microbleeds and cavernomas, are among the presumable late cerebrovascular complications associated with radiotherapy for PCNST. OBJECTIVE: To explore association between PCNST radiotherapy and the occurrence FHD lesions and to address the correlation between the topographic location of these microvascular lesions with the focal radiotherapy location. METHODS: Retrospective cohort study of 190 paediatric patients being followed for PCNST in a single referral oncological centre. The frequency of FHD lesions was compared between paediatric PCNST treated (n=132) and not treated (n=58) with brain radiation. Microbleed Anatomical Rating Scale (MARS) was used for systematic identification of these cerebrovascular lesions and to address the consistency between the topographic location of each lesion and the location of the focal radiotherapy area. Univariate analysis to address the role of variables such as tumour histology, location, gender and age of children at the beginning of radiotherapy, duration of follow-up and chemotherapy was performed. RESULTS: FHD lesions (microbleeds and cavernomas) occurred exclusively and in a high percentage (41.6%) in PCNST survivors treated with brain radiation. Younger age at the diagnosis (p=0.031), duration of follow-up (p=0.010) and embryonal histology (p=0.003) positively correlated with the occurrence FHD lesions. FHD lesions were topographically concordant with the brain focal irradiation area in 3/19 (15.8%) patients from the focal RT subgroup and in 22/111 (19.8%) patients from the WBRT plus focal RT subgroup. CONCLUSION: Our study, which is one of the largest to date on the topic, shows that FHD lesions are a common complication after radiotherapy for childhood PCNST. The young brain is probably more susceptible to radiation-induced late cerebrovascular injury. Diffuse small vessel disease and ceiling effect may account for the low topographic concordance we found. The clinical implications of FHD lesions in this specific population are yet to be clarified.

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.

Cavernous Malformation of the Seventh Cranial Nerve: Case Report and Review of Literature.

BACKGROUND: Cavernous malformations (CMs) arising in the cranial nerves are rare. Only a few cases of facial nerve CMs have been reported in the literature. CASE DESCRIPTION: A 52-year old woman presented with headache, unilateral worsening facial weakness, and hearing loss. Imaging was suggestive of a CM of the cerebellopontine angle. Intraoperatively, a mulberry-shaped lesion was seen arising entirely within the seventh nerve. The patient underwent complete resection with sural nerve interposition graft. CONCLUSIONS: CMs are very rare but should be included in the differential diagnosis of cerebellopontine angle masses.

[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.