Biological identity of orbital cavernous venous malformations

ABSTRACT Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

Hemangioma cavernoso / Cavernous Hemangioma

Introducción: El hemangioma cavernoso es una de las neoplasias benignas más frecuentes en la infancia. Objetivo: Describir el caso de un hemangioma cavernoso en edad pediátrica. Caso clínico: Lactante femenina de 6 meses de edad, de procedencia urbana, cuyo nacimiento tuvo lugar en el Hospital Materno Fe del Valle Ramos, del municipio Manzanillo. Al nacer se observa una lesión en forma placa eritematosa violácea infiltrada de ± 10 cm, de borde definido, de superficie liza, con aumento de temperatura al tacto, que se extendía desde la parte medial anterior y posterior hasta la superior de la pierna del miembro inferior izquierdo. Se decidió interconsultar con el Servicio de Angiología, el cual diagnostica la lesión como hemangioma cavernoso. Conclusiones: Se corroboró que el hemangioma cavernoso es una entidad frecuente en la infancia, su diagnóstico y tratamiento oportunos son altamente beneficiosos para la mejoría y la cura, por lo que se impone la realización de un correcto y exhaustivo examen físico, de lo que se deriva también la prevención de complicaciones a corto, mediano y largo plazo(AU)

Introduction: Cavernous hemangioma is one of the most frequent benign neoplasms in childhood. Objective: To describe the case of a cavernous hemangioma at pediatric age. Clinical case: A 6-month-old female infant of urban origin was born at Hospital Materno Fe del Valle Ramos, Manzanillo Municipality, Granma Province. At birth, a violaceous erythematous plaque-like infiltrated lesion was observed, of approximately 10 cm, with a defined border, smooth surface, increased temperature at hand contact, extending from the anterior and posterior medial part to the upper leg of the left lower limb. It was decided to do an interconsultation with the angiology service, which diagnosed the lesion as a cavernous hemangioma. Conclusions: Cavernous hemangioma was corroborated to be a frequent entity in childhood, whose timely diagnosis and treatment are highly beneficial for improvement and cure, so it extremely necessary to carry out a correct and exhaustive physical examination, also deriving the prevention of complications in the short, medium and long terms(AU)

Surgical approach selection and curative effect analysis of orbital cavernous hemangioma / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To investigate the choice of surgical approach, therapeutic effect and complications of orbital cavernous hemangioma(OCH)in different positions of orbit.METHODS: The clinical data of 128 patients of 128 eyes with OCH whose were surgically removed and pathologically diagnosed in the department of ophthalmology of the Second Affiliated Hospital of Air Force Military Medical University from January 2016 to August 2021 were retrospectively analyzed. The position of OCH in the orbit was determined by preoperative imaging examination(CT/MRI), so as to select different surgical approaches and analyze the postoperative curative effect and the incidence of complications.RESULTS: The location of OCH in the orbit: 82 eyes in the muscle cone and 46 eyes outside the muscle cone. According to the quadrant of the orbit where the tumor was located, there were 24 eyes in the upper-outer quadrant, 38 eyes in the lower-outer quadrant, 28 eyes in the upper-inner quadrant, 12 eyes in the lower-inner quadrant, and 26 eyes the intraconal central space. The selection of surgical approach: 1)OCH in the muscle cone: conjunctival approach surgery in 53 eyes, lateral orbital approach surgery in 22 eyes, lateral combined medial conjunctiva orbital approach surgery in 5 eyes, skin approach surgery in 1 eye, transnasal approach under nasal endoscope surgery in 1 eye; 2)OCH outside the muscle cone: skin approach in 29 eyes, conjunctival approach in 12 eyes and lateral orbital approach in 5 eyes. Postoperative efficacy: except for 1 eye of postoperative tumor residue, the other 127 eyes were completely removed. Postoperative complications: 1)Ocular motility disorder: 16 eyes, including conjunctival approach surgery in 11 eyes, lateral orbital approach surgery in 4 eyes, lateral combined medial conjunctiva orbital approach surgery in 1 eye; 2)Visual acuity decreased in 9 eyes: conjunctival approach surgery in 3 eyes, lateral orbital approach surgery in 6 eyes; 3)Mydriasis occurred in 9 eyes, including 4 eyes via conjunctival approach surgery and 5 eyes via lateral orbital approach surgery; 4)Intraorbital hemorrhage occurred in 3 eyes: all occurred through conjunctival approach surgery; 5)Visual acuity was lost in 2 eyes, including conjunctival approach surgery in 1 eye and lateral orbital opening approach surgery in 1 eye; 6)There were 2 eyes of ptosis, including conjunctival approach surgery in 1 case and skin approach surgery in 1 eye; 7)Tumor residue occurred in 1 eye: multiple intraorbital tumors were treated by lateral orbital approach surgery.CONCLUSION: The accurate location of OCH combined with imaging examination and the selection of appropriate surgical approaches according to different locations can successfully remove the tumor and reduce the incidence of complications.

Application value of the contrast-enhanced ultrasound in the diagnosis of orbital retrobulbar tumors / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To analyze the characteristics of the contrast-enhanced ultrasound in orbital retrobulbar tumors, and to explore the application value of the contrast-enhanced ultrasound in the diagnosis of orbital retrobulbar tumors.<p>METHODS: The contrast-enhanced ultrasound data of the 52 patients(52 eyes)with orbital retrobulbar tumors in the Fourth People's Hospital of Shenyang from March 2012 to May 2015 were retrospectively analyzed. According to the pathological results, 41 eyes were divided into benign group and 11 eyes were malignant group. The time intensity(TI)curve and quantitative data were obtained by SonoLiver software.<p>RESULTS: The TI curve of benign group mainly showed fast in and slow out mode, while that of malignant group mainly showed fast in and fast out mode. Quantitative data: rise time(RT)and mean transit time(mTT)in malignant group were lower than those in benign group, while there was no significant difference in maximal intensity(IMAX)between the two groups(<i>P</i>>0.05).<p>CONCLUSION: Contrast-enhanced ultrasound can provide imaging basis for the differential diagnosis of orbital retrobulbar tumors which is helpful for the preoperative qualitative diagnosis.

Adrenal cavernous hemangioma: A case report and literature review / 北京大学学报(医学版)

A 57-year-old male patient was referred to our department with complaints of his right adrenal gland occupancy and hypertension about 6 months. When admitted to the hospital, the blood pressure was about 160/100 mmHg, and the heart rate was 110 beats/min. He was no obvious obesity, acne, abnormal mood, without weakness of limbs, acral numbness, palpitation and headache. He presented with type 2 diabetes for more than 3 years, with oral administration of metformin enteric coated tablets and subcutaneous injection of insulin glargine to control blood glucose, and satisfied with blood glucose control. Enhanced CT showed that: the right adrenal gland showed a kind of oval isodense, slightly hypodense shadow, the edge was clear, lobular change, the size was about 5.8 cm×5.4 cm, uneven density, there were nodular and strip calcification, round lipid containing area and strip low density area, and the CT value of solid part was about 34 HU. Enhanced scan showed heterogeneous nodular enhancement in the solid part of the right adrenal gland, nodular enhancement could be seen inside. The CT values of solid part in arterial phase, venous phase and delayed phase were 45 HU, 50 HU and 81 HU, respectively. Considering from the right adrenal gland, cortical cancer was more likely. No obvious abnormality was found in his endocrine examination. After adequate preoperative preparation, retroperitoneal laparoscopic adrenalectomy was performed under general anesthesia. During the operation, the 6 cm adrenal tumor was closely related to the inferior vena cava and liver, and after careful separation, the tumor was completely removed and normal adrenal tissue was preserved. The operation lasted 180 min and the blood loss was 100 mL, and the blood pressure was stable during and after the operation. There was no obvious complication. The results of pathological examinations were as follows: the size of the tumor was 7.5 cm×6.0 cm×3.5 cm, soft, with intact capsule and grayish-red cystic in section. Pathological diagnosis: (right adrenal gland) cavernous hemangioma, secondary intravascular thrombosis, old hemorrhagic infarction with calcification and ossification. After 6 months of observation, no obvious complications and tumor recurrence were found. In summary, cavernous hemangioma of adrenal gland is a rare histopathological change. Its essence is a malformed vascular mass. Blood retention is the cause of thrombosis and calcification in malformed vessels. The imaging findings were inhomogeneous enhancement of soft tissue masses, and the adrenal function examination showed no obvious abnormalities. Retroperitoneal laparoscopic surgery is feasible after adequate preoperative preparation. It is difficult to diagnose the disease preoperatively and needs to be confirmed by postoperative pathology.

Retinal vascular abnormalities in Sturge-Weber syndrome

Sturge–Weber syndrome (SWS) includes facial, leptomeningeal and choroidal hemangioma. The retinal vasculature is essentially normal. Rare cases of retinal vascular tortuosity and arterio-venous malformations have been reported. We report two cases with rare concomitant retinal vascular abnormalities along with SWS. Both the patients had nevus flammeus, hemifacial hypertrophy, and choroidal hemangioma. In one case, retinal cavernous hemangioma was seen in the affected eye. The other case revealed retinal neovascularization secondary to proliferative diabetic retinopathy in the eye with choroidal hemangioma.

Effects of four surgical approaches on patients with deep cavernous hemangioma of the orbit / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To investigate the effects of conjunctival approach, lateral approach, skin approach and lateral medial conjunctival approach on visual acuity, tumor residual rate and complications in patients with deep cavernous hemangioma of the orbit.<p>METHODS: Totally 114 patients with deep cavernous hemangioma of the orbit were divided into group A(30 cases, conjunctival approach), group B(34 cases, translateral approach)and group C(27 cases, transcutaneous)according to the surgical approach. The approach and the D group(23 cases, combined with the medial conjunctival approach <i>via</i> the lateral side), compared the visual acuity improvement effect, tumor residual rate and postoperative complications in the four groups.<p>RESULTS: There was no difference in visual acuity improvement between the four groups(<i>P</i>=0.918). The residual tumor rates of patients in group A, group B, group C and group D were 0, 2.9%, 3.7%, and 0, respectively. There was no difference in the incidence of postoperative complications between the four groups(<i>P</i>=0.244). <p>CONCLUSION: According to the indications of deep cavernous hemangioma of the orbit, the reasonable choice of minimally invasive surgery can effectively maintain the normal visual acuity level and the safety is consistent.

Cavernous Hemangioma of the Gallbladder: a Case Report

Cavernous hemangioma of the gallbladder is an extremely rare benign tumor. The tumor has only a few cases being reported in literature. However, to the best of our knowledge, no reports focusing on the MRI findings of cavernous hemangioma of the gallbladder have been published. This study reports a case of gallbladder hemangioma with pathologic and radiologic reviews, including MRI findings.

Atypical Cavernous Hemangioma of the Trigeminal Nerve:Report of One Case / 中国医学科学院学报

Extracerebral cavernous hemangioma typically occurs in cavernous sinus in middle cranial fossa,showing iso-or hypointensity on T1WI,obvious hyperintensity on T2WI,and evident enhancement after contrast administration during magnetic resonance imaging(MRI).In this article we report one case of atypical cavernous hemangioma of the trigeminal nerve,with atypical MRI findings including isotense or slight long T2 signal,dotty short T1 signal,and non-enhancement on T1WI.

A cavernous hemangioma located in the axillary area: Challenges in preoperative diagnosis and operation

Cavernous hemangiomas are benign neoplasms of endothelial cells. Although this neoplasm has the potential to develop in all parts of the body, it rarely develops in the axilla; in fact, there are only two case reports of axillary cavernous hemangiomas in the literature. Here, we describe a third case, which occurred in a 30-year-old Korean woman. The patient presented with a palpable mass in the left axilla that was initially thought to be either a phyllodes tumor or a lymphoma based on imaging studies. However, the results of an excisional biopsy led to a diagnosis of cavernous hemangioma. Although uncommon, a cavernous hemangioma can be encountered unexpectedly, presenting as a mass in axilla. Although usually curative, surgery may be challenging not only because of the rarity of the condition, but also because of inconsistent preoperative findings and the involvement of large vessels.

Synchronous Orofacial Granulomatosis in a Patient with Brain Cavernous Hemangioma

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.

Extradural lumbar nerve root cavernous hemangioma: case report and successful surgical resection / Hemangioma cavernoso extradural de raiz nervosa lombar: relato de caso e ressecção cirúrgica bem-sucedida

Extradural lumbar spinal canal cavernous hemangiomas (or cavernomas) are rare lesions that can induce intense back pain and neurological deficit. We present a case report of a patient with a pure radicular lombar extradural cavernoma resembling a benign neurological tumor in imaging exams and a successful surgical resection.

Os hemangiomas cavernosos do canal vertebral lombar extradural (ou cavernomas) são lesões raras que podem induzir dor intensa no dorso e déficit neurológico. Apresentamos um relato de caso de um paciente com um cavernoma extradural lombar radicular puro assemelhando-se a um tumor neurológico benigno em exames de imagem e uma ressecção cirúrgica bem-sucedida.

Hemangioma cavernoso laríngeo en adultos: reporte de un caso y revisión de la literatura / Cavernous hemangioma of the larynx in adults: case report and literature review

RESUMEN Los hemangiomas son uno de los tumores más frecuentes en cabeza y cuello. Los hemangiomas de laringe se pueden dividir clínicamente en formas infantiles y adultas. En niños es una patología frecuente, mientras que en adultos es muy infrecuente. En adultos, tiene mayor incidencia en hombres y su ubicación más frecuente es supraglótica. Su histología más frecuente es el hemangioma cavernoso (HC), que corresponde a una malformación de origen venoso, con paredes delgadas y vasos dilatados. La presentación más habitual es con disfonía o ronquera de meses o años de evolución y se sospecha mediante la nasofaringolaringoscopía o el estudio de imágenes (TC y/o RM). Existen distintas formas de tratamiento, incluidas la observación, la corticoterapia, la radioterapia, la cirugía láser, la cirugía abierta, etc., sin existir un consenso sobre la mejor opción. Se presenta el caso de un paciente de 43 años con antecedentes de tabaquismo que consultó por una historia de disfonía de un año de evolución. Se diagnosticó mediante nasofaringolaringoscopía y TC de cuello una gran masa supraglótica que se extirpó mediante un abordaje de laringofisura. La biopsia diferida informó un HC.

ABSTRACT Hemangiomas are one of the most frequent head and neck tumors. Clinically, they can be divided into childish and adult forms. In children it is a frequent pathology, whereas in adults it is very rare. In adults, it has a higher incidence in men and its more frequent location is supraglottic. Its most frequent histology is the cavernous hemangioma, which corresponds to a malformation of venous origin, with thin walls and dilated vessels. The most common presentation is with the disphony or hoarseness lasting from months to years of evolution and suspicion is made through nasofibroscopy or the imaging study (CT and / or MRI). There are different forms of treatment, including observation, corticotherapy, radiotherapy, laser surgery, open surgery, etc., without a consensus on the best option. We present the case of a 43 year old male with a history of smoking who consulted for dysphonia of a year of evolution. A large supraglottic mass was diagnosed through nasofibroscopy and CT of the neck, which is removed by a laryngopharyngeal approach. Deferred biopsy reported a cavernous hemangioma.

Hemangioma cavernoso como causa de hemorragia digestiva oculta manifiesta: reporte de caso / A Case Report of Cavernous Hemangioma as a Cause of Occult Gastrointestinal Bleeding

Resumen Se realizó un estudio descriptivo tipo reporte de caso de un paciente valorado por el servicio de gastroenterología en la Clínica Nuestra en la ciudad de Cali, por sospecha de sangrado digestivo oculto cuyos estudios previos no habían mostrado causa de sangrado. Se realizó videocápsula endoscópica (MiroCam®) con un hallazgo positivo de lesión en el intestino delgado. Se llevó a enteroscopia en Clínica Farallones de la Ciudad de Cali con enteroscopio Olympus 180 Q que confirmó el hallazgo. Ulteriormente, se llevó a cirugía, en la que se confirmó el diagnóstico. Los datos y las fotografías se obtuvieron en bases de datos de los servicios de endoscopia y las fotos de patología se obtuvieron del registro del médico patólogo. Finalmente, se realizó una búsqueda en PubMed sobre literatura existente de historia clínica y reportes de casos similares.

Abstract This is a descriptive study based on the case report of a patient suspected of occult gastrointestinal bleeding who was evaluated by the gastroenterology service in Clínica Nuestra in Cali. Previous studies had not shown cause of bleeding. Video endoscopy (Mirocam) found a lesion in the small intestine. Enteroscopy at performed at Clínica Farallones in Cali with an Olympus 180 Q enteroscope confirmed the finding. Surgery further confirmed the diagnosis. Data and photographs were obtained from the database of endoscopy services and the pathology photos were obtained from the pathologist's registry. A literature review based on a Pubmed search of the existing literature on clinical histories and reports of similar cases is included.

Surgical Treatment of Cavernous Angiomas in the Pineal Gland ­ Case Report

Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.

Microsurgery of spinal cord Intramedullary cavernous hemangioma / 中华显微外科杂志

Objective To investigate the microsurgical effect of spinal cord Intramedullary cavernous hemangioma.Methods The clinical data of 23 patients with spinal cord Intramedullary cavernous henangioma from January,2003 to March,2017 were analyzed retrospectively.McCormick clinical neurological function classification method and MRI examination were used to analyze the changes of neurological function and imaging changes beforeand-after operation.MRI and assess nerve function were reviewed 6-12 months after discharge.Results Twentytwo cases were totally resected,and subtotal excised in 1 case.The symptoms improved and even disappeared completely in 17 cases.There was no obvious improvement in 4 cases,2 cases were worse and no death cases.Twentyone cases were followed-up.The follow-up time was 3 months-9 years,and no tumor recurrence was found.Among the 14 cases of clinical neurological function Ⅰ-Ⅱ,10 cases recovered rapidly and most of the symptoms improved or even disappeared.Three cases had no obvious change.The other 1 case aggravated symptoms;Six cases of Ⅲ-Ⅳ patients in 7 cases of postoperative recovery,and 1 case of no change.According to preoperative neurological function grade Ⅰ-Ⅱ and Ⅲ-Ⅳ,the treatment effect of patients was compared,the difference was significant (x2=5.25,P＜0.05).Conclusion Microsurgical resection of spinal cord Intramedullary cavernous hemangioma is safe and effective.The preoperative nerve function,the relationship between the tumor and the spinal cord,and the surgical skill and the degree of resection are the important factors that affect the prognosis.

The MRI findings of cavernous hemangioma in the thoracic spinal epidural space / 实用放射学杂志

Objective To explore the MRI features of cavernous hemangioma in the thoracic spinal epidural space,to improve the recognition of the disease.Methods The clinical,pathological and MRI data of 7 cases with cavernous hemangioma in the thoracic spinal epidural space confirmed by surgery and pathology were analyzed retrospectively.Results 7 cases of cavernous hemangioma in the thoracic spinal epidural space were included in our study,all cases underwent MRI contrast enhancement examination and 6 of them underwent conventional MRI examination.On T1WI,4 cases showed hypointensity,2 cases showed slightly hypointensity and 1 case showed mixed slightly hypointensity.On T2WI,5 cases showed slightly hyperintensity,1 case showed slightly hypointensity. On fat suppression sequence,5 cases showed hyperintensity,1 case showed heterogeneous hyperintensity.On diffusion-weighted imaging (DWI),7 cases showed obviously hyperintensity.On contrast-enhanced images,the lesions enhanced homogeneously in 5,enhanced heterogeneously in 2.Conclusion MRI can display the location,appearance and enhancement pattern of thoracic spinal epidural cavernous hemangioma distinctly,while definite diagnosis of which still depends on pathology.

Huge Cavernous Hemangioma of the Chest Wall: A Rarely Arising Benign Tumor of Chest Wall

A 35-year-old female complaining of a painful huge right posterior chest wall mass was admitted. Chest computed tomography showed the pleural invasion of mass and deformed ribs. Suspected diagnosis was hemangioma. Entire mass was removed carefully and pleural biopsy was performed. The operation was finished after bleeding control and chest tube insertion. The mass was diagnosed as a cavernous hemangioma histopathologically. Cavernous hemangioma of chest wall is a rare benign vascular tumor. Surgical operation of hemangioma demands meticulous bleeding control because of risk of hemorrhage.

Clinical analysis of pure spinal epidural cavernous hemangiomas / 局解手术学杂志

Objective To analyze and summarize the clinical manifestations,diagnosis and treatment of pure spinal epidural cavernous hemangiomas.Methods We presented a case series of 6 patients with pure spinal epidural cavernous hemangiomas in our hospital from April 2012 to November 2016 with previous articles discussing their clinical presentation,radiological characteristics,surgical technique,pathological features,and functional outcome.Results All patients were diagnosed as pure spinal epidural cavernous hemangiomas by pathology.The cavernous hemangiomas were totally cut and patients recovered well after operations.All patients gradually improved neurologically and achieved a good outcome with no recurrence during the follow-up for 13 to 68 months.Conclusion Pure spinal epidural cavernous hemangioma is rare.It is easily confused with other spinal diseases.MRI is an important imaging examination method.Pathology is the method of diagnosis.Surgical resection is the main method of treatment,and the prognosis is good.

A Thoracolumbar Pure Spinal Epidural Cavernous Hemangioma: A Case Report

STUDY DESIGN: Case report. OBJECTIVES: We report a case of pure epidural cavernous hemangioma located at the thoracolumbar spine in a 53-year-old woman that mimicked a neurogenic tumor on magnetic resonance imaging (MRI). SUMMARY OF LITERATURE REVIEW: A pure spinal epidural cavernous hemangioma without bony involvement is a very rare lesion about which limited information is available in the literature. MATERIALS AND METHODS: A 53-year-old woman visited our clinic for hypoesthesia with a tingling sensation in the left anterolateral thigh that had begun a month ago. No other neurologic symptoms or signs were present upon a neurologic examination. MRI from an outside hospital showed a 2.0×0.5 cm elongated mass at the T11-12 left neural foramen. The tumor was completely removed in piecemeal fashion. RESULTS: The histopathologic examination revealed a cavernous hemangioma, which was the final diagnosis. The outcome was favorable in that only operation-related mild back pain remained, without any neurologic deficits, after a postoperative follow-up of 2 years and 3 months. No recurrence was observed on MRI at 2 years postoperatively. CONCLUSION: Pure epidural spinal cavernous hemangioma is very rare, and it is very difficult to differentiate from other epidural lesions. However, we believe that it should be included in the differential diagnosis of spinal epidural tumors due to its favorable prognosis.