Esophageal Angiolipoma:Report of One Case and Literature Review / 中国医学科学院学报

Esophageal angiolipoma is a rare disease with unspecific clinical manifestations.This paper reported a case of esophageal angiolipoma confirmed by upper gastrointestinal endoscopy and summarized the clinical manifestations,endoscopic and pathological features,treatment and prognosis of the patients by reviewing the relevant literature,aiming to provide references for clinical diagnosis and treatment of this disease in the future.

Angiolipoma sacro. Reporte de un caso / Sacral angiolipoma. A case report

Fundamento: Los angiolipomas son tumores benignos que se presentan en adultos con una localización, preferentemente, en el espacio epidural posterior torácico. Objetivo: Presentar un caso que debutó con un traumatismo axial lumbosacro donde se evidenció una localización poco común de la lesión y sin relación con las estructuras del canal raquídeo. Presentación del caso: Hombre de 25 años que se cayó y debido a ello se le hizo un traumatismo directo en la región sacrococcígea con dolor y aumento de volumen regional, asociado a parestesias glúteas. Los estudios radiológicos evidenciaron una fractura del cóccix y la presencia de una lesión ubicada en las partes blandas, de aspecto redondeado, homogéneo, sólido, de poco más de 50 mm de diámetro. Se le realizó tratamiento quirúrgico que consistió en coccigectomía subperióstica y exéresis macroscópica de la masa. El estudio histológico concluyó el diagnóstico de un angiolipoma. Conclusiones: Los angiolipomas son tumores raros que tienen características radiológicas peculiares, requieren de alta sospecha clínico-imagenológica para indicar los estudios y el tratamiento. La exéresis total es recomendada para evitar la recurrencia y mejorar el pronóstico.

Background: Angiolipomas are benign tumors that appear in adults with special location in the posterior thoracic epidural position. Objective: To present a case that appeared with a lumbosacral axial trauma where a non-common lesion location was evidenced with no relation among the structures of the spinal canal. Case presentation: 25-year-old man who fell down, consequently suffered a painful direct trauma to the sacrococcygeal region and increased regional volume, associated with gluteal paresthesias. Radiological studies showed a fracture of the coccyx and presence of a lesion located in the soft tissues, with a rounded, homogeneous, solid aspect, a little more than 50 mm in diameter. Surgical treatment consisted of subperiosteal coccygectomy and macroscopic excision of the mass. Histological study concluded the diagnosis of an angiolipoma. Conclusions: Angiolipomas are rare tumors with peculiar radiological features, they require high clinical-imaging suspicion for studies and treatment. Total excision is recommended to avoid recurrence and improve prognosis.

Causa infrecuente de odinofagia: angiolipoma laríngeo / Uncommon cause of odynophagia: laryngeal angiolipoma

Laryngeal angiolipoma is a benign and rare tumor at this level. It is a type of unique lipoma, which is located in aritenoepiglottic retraction, epiglottis, and postcricoid region, and it is usually diagnosed after surgical exeresis. It is usually silent until it reaches a large size, causing dysphagia as the most common symptom; so it should be part of the differential diagnosis of odynophagia. We present a case in a male patient of supraglottic angiolipoma as an unusual cause of odynophagia

El angiolipoma laríngeo es un tumor benigno e infrecuente a este nivel. Es un tipo de lipoma único, que se localiza en el repliegue aritenoepiglótico, la epiglotis y la región poscricoidea, y suele diagnosticarse tras su exéresis quirúrgica. Suele ser silente hasta que alcanza gran tamaño, ocasionado odinofagia como síntoma más frecuente; de ahí que deba formar parte del diagnóstico diferencial de la odinofagia. Presentamos un caso en un paciente varón de angiolipoma supraglótico, como causa inusual de odinofagia

Angiomiolipoma de cavidad nasal: reporte de un caso y revisión de la literatura / Angiomyolipoma of the nasal cavity: case report and literature review

El angiomiolipoma de cavidad nasal es un tumor hamartomatoso extremadamente infrecuente compuesto por adipocitos maduros, espacios vasculares con escaso tejido elástico y la presencia de haces de células musculares lisas maduras. Se manifiesta principalmente por obstrucción nasal unilateral y epistaxis recurrente. Se presenta el caso de una paciente de 73 años con historia crónica de obstrucción nasal y epistaxis recurrente izquierda asociada a rinorrea y descarga posterior intermitente. La tomografía computarizada (TC) y resonancia nuclear magnética (RNM) demuestran una lesión vascular circunscrita en fosa nasal izquierda. La angiografía demostró irrigación exclusiva de la arteria esfenopalatina izquierda. Se realizó extirpación de la lesión por abordaje endonasal endoscópico previa embolización arterial. La revisión de la literatura mundial muestra que es el duodécimo caso de angiomiolipoma de cavidad nasal reportado.

Angiomyolipoma of nasal cavity is an extremely rare hamartoma tumor composed of mature adipocytes, vascular spaces with lack of elastic tissue, and the presence of bundles of mature smooth muscle cells. It manifests itself mainly by unilateral nasal obstruction and recurrent epistaxis. We present the case of a 73-years-old woman with chronic history of left-sided nasal obstruction and recurrent epistaxis associated with rhinorrhea and intermittent post nasal drip. Computed tomography and magnetic resonance imaging demonstrate a vascular lesion inside the left nasal cavity. Angiography showed irrigation exclusively by the left sphenopalatine artery Surgical excision was performed by endoscopic endonasal approach after arterial embolization. World literature review showed that this is the twelfth reported case of angiomyolipoma of the nasal cavity.

Angiomyxolipoma of tongue

Angiomyxolipoma is a rare histological variant of lipoma, characterized by proliferation of adipose tissue associated with a variable amount of myxoid stroma with numerous thick- and thin-walled blood vessels. An exhaustive literature review could reveal only 12 cases of angiomyxolipoma affecting subcutaneous tissue, subungual area and spermatic cord. In oral cavity, only 2 cases of angiomyxolipoma have been reported to date, one case in the buccal mucosa and one case in the floor of the mouth. Hence, this is only the third case of angiomyxolipoma affecting oral cavity and the first case of angiomyxolipoma of the tongue. We report a case of a 51-year male presenting with a swelling on the right lateral border of the tongue. The lesion was excised and microscopical examination confirmed the diagnosis of angiomyxolipoma

Angiolipoma on the Lumbar Spine

Angiolipomas are composed of mature adipose tissue and blood vessels. Spinal angiolipomas are rare benign tumors with a good prognosis, and most symptoms are due to progressive compression of the spinal cord and root. The treatment of choice is total resection without any additional therapy. We report a case of an epidural angiolipoma on the lumbar spine in a 69-year-old man with weakness of the lower extremities. Lumbar magnetic resonance images revealed a well-enhanced epidural mass. He underwent laminectomy, and the tumor was completely removed. Pathologic examination confirmed the tumor was an angiolipoma. The patient’s symptoms gradually improved postoperatively.

Síndrome de Klippel-Feil con fistula traqueoesofagica, pulgar bifido y angiolipoma cerebral / Klippel-Feil syndrome with tracheoesophageal fistula, bifid thumb and cerebral angiolipoma

El síndrome de Klippel-Feil es una malformación congénita de la charnela cráneo-cervical compleja que involucra vértebras y visceras, caracterizada por la tríada clásica de cuello corto, limitación de movimientos de la cabeza por la fusión de vértebras cervicales e implantación baja del cabello en la región occipital. Se presenta por falla de segmentación en el esqueleto axial del embrión. Su incidencia se estima en 1/40 000-42 000nacimientos y predomina en el sexo femenino. El objetivo del presente trabajo es describir el cuadro clínico de un paciente con síndrome de Klippel-Feil y múltiples malformaciones asociadas, entre ellas, fístula traqueoesofágica, pulgar bífido y lipomas/angiolipomas intracraneales, las cuales, hasta ahora, no han sido descritas en el síndrome, por lo que se considera un hallazgo excepcional.

The Klippel-Feil syndrome is a congenital malformation of the skull flap involving complex cervical vertebrae and organs, characterized by a classic triad: short neck, limitation of movement of the head due to cervical spine fusion and low hairline in occipital region. It results from an error in the axial skeleton segmentation of the embryo; its incidence is estimated at 1/40,000-42,000 births and predominates in females. The aim of this paper is to describe the clinical picture of a patient with Klippel-Feil syndrome and multiple malformations, including tracheoesophageal fistula, bifid thumb and intracranial lipomas/angiolipomas,that have not been previously described in the syndrome, so it is considered an exceptional finding.

Angiolipoma intraóseo del calcáneo. Reporte de un caso y revisión de la literatura / Calcaneous intraosseous angiolipoma. A Case Report and literatura review

Los lipomas intraóseos son considerados como los tumores óseos primarios benignos más raros por su muy poca frecuencia. Su incidencia es menor del 1 por 1.000 entre los tumores primarios y para 2002 se habían publicado menos de 100 casos a nivel mundial. El angiolipoma, es una variante histológica del lipoma aún menos frecuente, representando sólo del 5 al 17% de todos los lipomas intraóseos. Generalmente se trata de una lesión asintomática, un hallazgo radiológico en exámenes realizados por otras razones suele ser la forma de su diagnóstico. Sin embargo, algunos lipomas pueden ser sintomáticos. Presentamos el caso de paciente femenino de 36 años quien posterior a presentar dolor del tobillo derecho se diagnostica una lesión en el calcáneo, que posterior a su análisis anatomopatológico resultó ser un angiolipoma intraóseo. Presentamos su tratamiento con médula ósea autóloga y su evolución(AU)

Intraosseous lipomas are considered the rarest and a very infrequently benign primary bone tumors. Its incidence is less than 1 per 1,000 between primary tumors and by 2002 had published fewer than 100 cases worldwide. The angiolipoma, is a histological variant of lipoma even rarer, representing only 5 to 17% of all intraosseous lipomas. This is usually an asymptomatic lesion, radiological finding in tests performed for other reasons is usually the way to diagnosis. However, some may be symptomatic lipomas. We report the case of a female patient aged 36 who after presenting pain in the right calcaneus, which after pathologic analysis proved diagnosed intraosseous angiolipoma. We present treatment with autologous bone marrow and evolution(AU)

Angiolipoma intracraniano adjacente a aneurisma sacular cerebral: relato de caso e revisão da literatura / Intracranial Angiolipoma Associated with Cerebral Saccular Aneurysm: Case Report and Review of Literature

Angiolipomas são neoplasias benignas compostas por adipócitos maduros e proliferação vascular angiomatosa de rara ocorrência no Sistema Nervoso Central. É relatado o caso de angiolipoma em cisterna silviana adjacente a aneurisma sacular de artéria cerebral média. São discutidas as características imagenológicas do tumor e a possibilidade de uma origem comum com lesões vasculares intracranianas. Este é o primeiro caso relatado de angiolipoma intracraniano associado a aneurisma de artéria cerebral média.

Angiolipomas are benign neoplasms composed of mature fat cells and vascular angiomatous proliferation of rare incidence in Central Nervous System.It's related a case of angiolipoma in Sylvian fissure associated with cerebral saccular aneurysm of medial cerebral artery. Imagenologic characteristics of the tumor and a possible common origin with intracranial vascular lesions are discussed. This is the first reported case of intracranial angiolipoma involving medial cerebral artery aneurysm.

Infiltrating Epidural Angiolipoma Involving Lumbar Spine / 대한정형외과학회잡지

We report on an unusual case with infiltrating extradural spinal angiolipoma. Most spinal angiolipomas involve the thoracic spine and infiltrating ones are also located mainly at the thoracic levels rather than lumbar lesion. In particular, there are few cases of lumbar extradural infiltrating type spinal angiolipoma. One case is that of a 52-year-old female with infiltrating extradural spinal angiolipoma involving lumbar 4 (L4) vertebra, who underwent a L4-5 laminectomy and surgical removal of the tumor. We achieved satisfactory results with surgical treatment of the patient. Spinal angiolipoma has a benign course with a good postoperative outcome.

Acute paraplegia by spinal angiolipoma. Case report and literature review / Paraplegia aguda por angiolipoma espinal. Relato de caso e revisão da literatura / Paraplejía aguda por angiolipoma espinal. Reporte de caso y revisión de la literatura

Spinal angiolipoma is an uncommon form of benign tumor. There are 142 reported cases and only one of acute paraplegia. We describe a case of a 39 year old male with acute spinal paraplegia angiolipoma in T4-T5 with surgical resolution three weeks after the onset of the neurological syndrome. Postoperative neurologic recovery was complete. The angiolipoma consists of mature fat cells and abnormal blood vessels. There are 2 types: non-infiltrating and infiltrating. Its clinical course is slow and progressive, it can be accelerated by vascular phenomena, intratumoral abscess, and pregnancy. There is just one report of spontaneous intratumoral hemorrhage and acute paraplegia, coinciding with our case. There is no consensus as to treatment, and release and complete resection are suggested, as it is a disease with good prognosis. Angiolipoma spinal epidural is a rare form of benign tumor, and the treatment of this pathology continues to be release and resection of the tumor, with a favorable prognosis, despite the delay in surgery, as in the case reported. Intratumoral bleeding should be considered a cause of acute spinal compression syndrome, as ocurred in our patient.

O angiolipoma espinhal é um tumor benigno incomum. Há 142 casos notificados, com apenas um de paraplegia aguda. Descrevemos o caso de um paciente de 39 anos com paraplegia aguda decorrente de angiolipoma espinal em T4-T5 com resolução cirúrgica três semanas após o início da síndrome neurológica. A recuperação neurológica pós-operatória foi completa. O angiolipoma consiste em células adiposas maduras e vasos sanguíneos anormais. Existem dois tipos: os não infiltrantes e os infiltrantes. Seu curso clínico é lento e progressivo, e pode ser acelerado por fenômenos vasculares, abcessos intratumorais e gravidez. Há somente um relato de hemorragia intratumoral espontânea e paraplegia aguda, coincidindo com o nosso caso. Não há consenso quanto ao tratamento, sugerindo-se a liberação e ressecção completa por ser uma doença de bom prognóstico. O angiolipoma espinal epidural é um tumor benigno incomum e o tratamento dessa patologia continua a ser a liberação e ressecção do tumor, que apresenta prognóstico favorável, apesar da demora da cirurgia, como no caso relatado. A hemorragia intratumoral deve ser considerada como causa da síndrome de compressão espinal aguda, como ocorreu com nosso paciente.

El angiolipoma espinal es una neoplasia benigna infrecuente. Hay 142 casos reportados y uno sólo de paraplejía aguda. Describimos un caso de un paciente de 39 años de edad de sexo masculino con paraplejía aguda por angiolipoma espinal T4-T5 con resolución quirúrgica tres semanas posteriores al inicio del síndrome neurológico. A recuperación neurológica postoperatoria fue completa. El angiolipoma se compone de células adiposas maduras y vasos sanguíneos anormales. Hay 2 tipos: no infiltrantes e infiltrantes. De curso clínico lento y progresivo que puede acelerarse mediante fenómenos vasculares, absceso intratumoral y el embarazo. Hay un solo reporte de hemorragia intratumoral espontánea y paraplejía aguda, coincidiendo con nuestro caso. No existe consenso para el tratamiento, sugiriéndose la liberación y resección total por ser una patología de buen pronóstico. Angiolipoma espinal epidural es un tumor benigno infrecuente siendo el tratamiento de ésta patología la liberación y resección tumoral con un pronóstico favorable, a pesar de la demora del acto quirúrgico como en el caso reportado. El sangrado intratumoral debe considerarse como causal de síndrome de compresión espinal agudo como ocurrió con nuestro paciente.

Intussuscepção intestinal em adulto jovem secundária à angiolipoma de cólon: relato de caso / Intestinal intusception secondary to an angiolipoma of the colon in an young adult: case report

Intussuscepção intestinal é rara em adultos. Angiolipomas são causas incomuns da doença. Manifestações clínicas inespecíficas e acurácia limitada dos exames de imagem disponíveis dificultam o diagnóstico pré-operatório. A confirmação de intussuscepção é baseada em achados cirúrgicos. Ressecção intestinal é mandatória quando o cólon é acometido devido ao alto risco de malignidade. Relatamos um caso de intussuscepção colônica em um adulto jovem, secundária à angiolipoma.

Intestinal intussusception is rare in adults. Angiolipomas are uncommon causes of disease. Nonspecific clinical manifestations and limited accuracy of imaging available hinder the preoperative diagnosis. Confirmation of intussusception is based on surgical findings. Bowel resection is mandatory when the colon is affected due to the high risk of malignancy. We report a case of colonic intussusception in a young adult, secondary to angiolipoma.

MRI Features of Spinal Epidural Angiolipomas

OBJECTIVE: To describe the MRI findings in ten patients of spinal epidural angiolipoma for differentiated diagnosis presurgery. MATERIALS AND METHODS: Ten surgically proved cases of spinal epidural angiolipomas were retrospectively reviewed, and the lesion was classified according to the MR findings. RESULTS: Ten tumors were located in the superior (n = 4), middle (n = 2), or inferior (n = 4) thoracic level. The mass, with the spindle shape, was located in the posterior epidural space and extended parallel to the long axis of the spine. All lesions contained a fat and vascular element. The vascular content, correlating with the presence of hypointense regions on T1-weighted imaging (T1WI) and hyperintense signals on T2-weighted imaging, had marked enhancement. However, there were no flow void signs on MR images. All tumors were divided into two types based on the MR features. In type 1 (n = 3), the mass was predominantly composed of lipomatous tissue (> 50%) and contained only a few small angiomatous regions, which had a trabeculated or mottled appear. In type 2 (n = 7), the mass, however, was predominantly composed of vascular components (> 50%), which presented as large foci in the center of the mass. CONCLUSION: Most spinal epidural angiolipomas exhibit hyperintensity on T1WI while the hypointense region on the noncontrast T1WI indicates to be vascular, which manifests an obvious enhancement with gadolinium administration.

Imaging Findings of Angiomyxolipoma of the Spermatic Cord Mimicking Inguinal Hernia

We report the case in a 72-year-old man who presented with a right inguinal mass and with a one month history that was initially interpreted as an inguinal hernia. Ultrasonography (US) and computed tomography (CT) demonstrated a right inguinal mass, including myxoid and fat component, extending from the right spermatic cord to the right inguinal subcutaneous layer. Mass excision was performed, and the diagnosis turned out to be angiomyxolipoma. Angiomyxolipoma is a rare tumor and the preoperative diagnosis of this disease is very difficult. However, angiomyxolipoma of the spermatic cord should be considered in the differential diagnosis in patients with an irreducible inguinal mass. Imaging diagnosis, such as US and CT may help to make a preoperative diagnosis.

Unusual Case of Sialoangiolipoma.

Sialoangiolipoma is extremely rare in adults. We report a case with an extremely unusual and atypical presentation of sialangolipoma of submandibular gland. The diagnosis of which is done on histopathology in a case with high index of clinical suspicion of pleomorphic adenoma.

Infiltrating Spinal Angiolipoma

Spinal angiolipomas are rare lesions usually found in the epidural space of the thoracic spine. The infiltrating type of spinal angiolipomas is extremely rare. This report presents the case and reviews the related literature. A 58-year-old man was presented with a 7-month history of progressive weakness and sensory change of lower extremities. Magnetic resonance images showed a well-enhanced mass infiltrating the vertebral foramen at the T4-5 level. Resection of the tumor was performed. Histological study revealed the tumor as an angiolipoma. The patient was relieved from symptoms after tumor resection.

Complejo de la esclerosis tuberosa, revisión de tema y presentación de caso / The tuberous sclerosis complex, review and case presentation

La esclerosis tuberosa fue descrita por primera vez en 1880 y corresponde a un trastorno autosómico dominante, en el que se ha identificado la mutación de dos genes responsables de la enfermedad. Desencadena una enfermedad sistémica que cursa con convulsiones y manifestaciones cutáneas, además de compromiso de riñón, pulmón y corazón. El compromiso del sistema nervioso central genera una gran morbilidad en los pacientes que la padecen, usualmente ante la presencia de tubérculos corticales, y el desarrollo a mediano plazo de las capacidades cognitivas depende su manejo temprano. El diagnóstico se establece por el cuadro clínico a partir de criterios mayores y menores establecidos, además del apoyo de estudios de imágenes y moleculares que permiten identificar las mutaciones de los genes TSC1 y TSC2. El manejo quirúrgico de las lesiones mejora la evolución clínica y el aspecto físico del paciente.

Soft Tissue Masses in the Extremities: The Accuracy of an Ultrasonographic Diagnosis

PURPOSE: We wanted to retrospectively determine the accuracy of an ultrasonographic diagnosis of superficial soft tissue masses in the extremities by using the histologic results as the reference standard. MATERIALS AND METHODS: From January 2005 to June 2010, 154 patients with soft tissue masses in the extremities and who underwent ultrasonographic evaluation followed by biopsy or resection were retrospectively evaluated. The ultrasonographic and histologic diagnoses of the soft tissue masses were lipoma, ganglion cyst, hemangioma, neurogenic tumor, giant cell tumor of the tendon sheath, epidermoid cyst, fibroma, glomus tumor, Baker's cyst and neurofibromatosis. RESULTS: Out of 154 patients, 114 (74%) patients showed concordance between the histologic diagnosis and the ultrasonographic diagnosis, and the remaining 40 (26%) patients did not. The diagnostic accuracy of each soft tissue mass was 95% for lipoma, 83% for ganglion cyst, 75% for hemangioma, 72% for neurogenic tumor, 50% for giant cell tumor of the tendon sheath, 43% for epidermoid cyst, 33% for fibroma and 100% each for glomus tumor, fibromatosis and Baker's cyst. Aside from these tumors, there were also sarcoma, malignant melanoma, elastofibroma, Kimura disease and pilomatricoma. Among the cases that showed discordance between the histologic diagnosis and the ultrasonographic diagnosis, three of them were notable; pilomatricoma being misdiagnosed as dermatofibroma protuberans, angiolipoma being misdiagnosed as vascular leiomyoma and malignant fibrous histiocytoma being misdiagnosed as a malignant soft tissue mass. CONCLUSION: The accuracy of an ultrasonographic diagnosis for soft tissue masses in the extremities varies greatly according to each type of mass. Lipoma, ganglion cyst, hemangioma, glomus tumor, neurogenic tumor and Baker's cyst showed a relatively high rate of concordance between the ultrasonographic diagnosis and the histologic diagnosis, but epidermoid cyst and fibroma showed a relatively lower rate of concordance.

Neoplastic Stromal Cells of Intracranial Hemangioblastomas Disclose Pericyte-derived Mesenchymal Stromal Cells-like Phenotype

BACKGROUND: Stromal cells (SCs) of hemangioblastomas (HBs) have been regarded as true neoplastic components, but their ontogeny remains unclear. Convincing evidence suggests that embryonic mesenchymal cells may be the cells of origin of HBs. The aim of the present study was to investigate the immunophenotypic characteristics of neoplastic SCs using a set of markers against endothelial cells (ECs), vascular smooth muscle cells (vSMCs), mesenchymal stromal cells (MSCs), and pericytes. METHODS: Intracranial HBs (n=46), angiolipoma (n=9), and pyogenic granuloma (n=11) were retrieved and the immunophenotypic profile of SCs was determined by immune stainings. RESULTS: The MIB-1 labeling index was significantly higher in SCs compared to that of ECs and vSMCs, regardless of the type of lesion. The neoplastic SCs of HBs consistently expressed both MSC and pericyte markers, but did not express markers of ECs and vSMCs. Double immunofluorescent staining demonstrated that the neoplastic SCs of HBs expressing MSC or pericyte markers directly abutted onto the ECs of capillaries/venules. CONCLUSIONS: The results suggest that the neoplastic SCs of HBs share the immunophenotypic profile and distribution with those of pericyte-derived MSCs. Thus, HBs might originate from a distinctive population of pericyte-derived MSCs in the central nervous system.

Angiolipoma de recto. Presentación de un caso. / Rectal angiolipoma. A case report

Se presenta el caso de un paciente masculino de 75 años de edad que consulta por obstrucción defecatoria y dolor anal. Por anatomía patológica se diagnostica Angioli-poma Submucoso de Recto. Es un tipo de tumor cuyo diagnóstico preoperatorio es difícil y el tratamiento de elección es el quirúrgico. El paciente fue operado en nuestro Instituto.

We report the case of a 75 years- old male patient, with obstructed defecation and anal pain. The final diagno-sis in the biopsy was submucosal rectal angiolipoma. This kind of tumor is rare and with a difficult preoperatory diagnosis.