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Spinal angiolipomas are benign uncommon neoplasm composed of mature lipocytes admixed with abnormal blood vessels. They account for only 0.04-1.2% of all spinal tumors. We report two cases of lumbar extradural angiolipoma and review previously reported cases. We found 118 cases of spinal epidural angiolipoma (70 females and 48 males; age range 1.5-...
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... reported cases of spinal angiolipoma reported between 1890 and 2006 were reviewed; only cases in which authors Table 1). For example, a review of 48 angio- lipoma cases, 8 of which were surprisingly neither descri- bed in other series nor included in the bibliography, were omitted [54]. ...
Similar publications
Paragangliomas are tumors that arise from the paraganglion system, which is a component of the neuroendocrine system. Approximately 10% are located in the extra-adrenal paraganglion system. Paragangliomas of the spine, however, are rare. They usually present as an intradural tumor in the cauda equina. There are only three reports of primary intraos...
Citations
... The cause of these tumors is not well understood. Proposed mechanisms suggest they may be derived from local pluripotent stem cells that have divergent differentiation caused by an unknown stimulus [3]. Other proposed causes include congenital malformations or disorganized growth of local tissue [3]. ...
... Proposed mechanisms suggest they may be derived from local pluripotent stem cells that have divergent differentiation caused by an unknown stimulus [3]. Other proposed causes include congenital malformations or disorganized growth of local tissue [3]. The most common presenting symptoms are numbness and weakness, and they often escalate to paraparesis. ...
... The most common presenting symptoms are numbness and weakness, and they often escalate to paraparesis. A literature review found the peak incidence of angiolipomas to be around 46 years of age and a time of diagnosis from symptom onset ranging from one day to 17 years [3]. MRI imaging is the best method for detection given its anatomic soft tissue contrast and visualization of spinal cord compression [4]. ...
A spinal epidural angiolipoma is a rare, benign tumor of adipocytes and blood vessels that accounts only for a small percentage of all spinal axis tumors. We report a case of a 44-year-old male who presented with three months of progressive decreased sensation and strength from about six cm above the umbilicus down to his feet bilaterally. He presented to the emergency room when he could no longer walk. He also had neurogenic urinary retention and likely neurogenic constipation. Physical exam was notable for decreased sensation, decreased strength, and increased patellar reflexes bilaterally. MRI of the thoracic spine showed a posterior epidural mass that spanned from T2 to T3, measuring 1.2 x 1.7 x 4.3 cm, and severely compressed the spinal cord posteriorly. The patient underwent an urgent laminectomy for decompression and mass resection. Pathology was consistent with an angiolipoma. Postoperatively, he experienced a drastic improvement in strength and gross motor skills. The sensation had a partial return following surgery and continued to improve over the hospital stay. In general, the literature reports significant symptomatic improvement in patients with spinal epidural angiolipomas after surgical resection.
... Epidural angiolipoma is a rare benign tumor consisting of mature adipocytes, blood sinuses, capillaries, and small blood vessels. They constitute about 0.04%-1.2% of spinal axis tumors and about 2%-3% of extradural spinal tumors [ 1,2 ]. Although it is a benign tumor, when it is large, it can compress the spinal cord or dural sac and even destroy the adjacent bone, causing a series of neurologic or local pain symptoms, so early detection and accurate diagnosis of this disease is of great clinical significance. ...
Epidural angiolipoma is a rare benign tumor consisting of mature adipocytes, blood sinuses, capillaries, and small blood vessels. It constitutes about 0.04%-1.2% of spinal axis tumors and about 2%-3% of extradural spinal tumors. We report a case of thoracic epidural angiolipoma and review the literature. We describe a 42-year-old woman who had weakness and numbness in her lower extremities prior to diagnosis and had an onset of approximately 10 months. The patient was misdiagnosed as schwannoma on preoperative imaging, possibly because neurogenous tumor is the most common intramedullary subdural tumor, and the lesion grew into bilateral intervertebral foramina. However, the lesion demonstrated high signal on the T2-weighted and T2 fat suppression sequences, and the linear low signal at the lesion edge was ignored, leading to misdiagnosis. The patient underwent posterior thoracic 4-6 laminectomy, pathectomy, and spinal decompression/vertebroplasty under general anesthesia. The final pathologic diagnosis was intradural epidural angiolipoma of the thoracic vertebra. Spinal epidural angiolipoma is a rare benign tumor that occurs frequently in middle-aged women and is mostly located on the dorsal side of the thoracic spinal canal. Magnetic resonance imaging findings of spinal epidural angiolipoma depend on the ratio of fat to blood vessels. Most angiolipomas show equal or high signal on T1-weighted images and high intensity on T2-weighted images, with significant enhancement after injection of gadolinium. The treatment of spinal epidural angiolipoma is complete surgical resection with good prognosis.
... Observations Spine angiomyolipoma is rare with an incidence of 0.14%-1.2%. 1 Overall, it most commonly involves the thoracic spine (78%), the lumbar column (10%), and the cervical or sacral column (<1%). 6 The exact pathogenesis of AMLs is unknown. However, there are two major explanations mentioned for the origin of these rare benign tumors: pluripotent mesenchyme cells giving different mesenchyme cells versus congenital malformation classifying them as true hamartomas. ...
... 10 Fast progression (acute) neurological symptoms might occur in some of the cases of these tumors and is attributed to vascular factors such as venous stasis with thrombosis, intratumoral bleeding, or steal phenomenon leading to an acute cord compression. 6,[11][12][13][14] On MRI, the fatty content appears hyperintense on both T1-and T2weighted images and hypointense on fat-suppressed images, whereas the vascular component appears hypointense on T1-weighted and hyperintense on T2-weighted images and shows intense enhancement in postcontrast images. 2,15,16 However, infiltrative properties such as invasion to adjacent bone or extension to the perilesional space and the masses prone to the ventral location have been mentioned as distinctive features of AML compared with major patterns of angiolipoma. ...
... 9,15 No adjuvant treatment is indicated. 6 ...
BACKGROUND
Angiomyolipoma (AML) of the spine is a rare benign neoplasm (accounting for 0.14%–1.2% of all spine tumors) that is often described along with angiolipoma because of their similarities. They occur almost exclusively in the extradural space, with the thoracic spine being the commonest level.
OBSERVATIONS
The authors present the clinical presentation, diagnosis, and treatment of an extremely rare case of thoracic spine AML in a 47-year-old male patient. The patient underwent laminectomy and gross total resection of the tumor and had an excellent immediate postoperative neurological recovery and long-term functional neurological outcome.
LESSONS
It is always wise to consider rare benign spinal epidural neoplasms such as spinal AMLs in the differential diagnosis of spinal epidural mass, despite metastasis being the commonest epidural tumor with variable modes of treatment, because the management of benign spinal epidural masses such as spine AML is always surgical and associated with an excellent long-term outcome.
... Representan entre el 0.04 al 1.2 % de todos los tumores espinales primarios y cuando son hallados en el espacio epidural constituyen entre el 2 al 3 %. (1,2) Macroscópicamente, es una masa blanda rojiza encapsulada o no, que se separa con facilidad de la duramadre, que en su histología está formada por una proliferación de adipocitos y vasos sanguíneos maduros normales, de apariencia normal o que simulan angiomas capilares, angiomas cavernosos o malformaciones arteriovenosas. El tejido graso es de tipo maduro sin ninguna particularidad. ...
... En estos reportes el debut de los síntomas estuvo relacionado con la pérdida progresiva de la fuerza muscular (40 %), el dolor lumbar o torácico (22 %), la alteración de la sensibilidad (20 %) y la ciática uni o bilateral (5 %). Otro grupo de pacientes estuvo paucisintomático en el momento que se hizo el diagnóstico.(2,6) En este caso, se arribó al diagnóstico después que se produjo un trauma axial lumbosacro, que provocó la fractura del cóccix y que permitió detectar el aumento progresivo de volumen en la región sacra e interglútea, acompañado de dolor en el área e impotencia funcional que le impedían realizar actividades habituales. ...
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.
... [8] e majority of spinal angiolipomas are located in the mid-thoracic region or more rarely in cervical and lumbar region, sacral localization had been reported in only 0.8% of cases, [11] with, to the best of our knowledge, only two cases reported in the literature. [1,3] ...
... According to a literature review of 108 spinal angiolipomas in 2009, the majority of the tumors (86%) were located in the thoracic spine, followed by 12 (11%) in the lumbar spine, 2 (1.85%) in the cervical spine, and one in the sacrum. [1,11] An additional case of sacral angiolipoma was reported in 2020 and was associated with tight filum terminal, spina bifida, and spinal arteriovenous fistula. [3] erefore, we consider this case the third case of sacral angiolipoma in the literature [ Table 1]. ...
... Adjuvant radiation should not be used on patients with this benign entity even in the infiltrating type. [1] CONCLUSION Spinal angiolipomas are rare benign tumors of angiomatous and lipomatous tissue. Sacral location is exceptional. ...
Background
Spinal angiolipomas are rare benign tumors composed of mature adipose tissue and anomalous vascular channels. The sacral localization is extremely rare. To the best of our knowledge, there have been only two cases reported in the literature. Herein, we present an additional case of sacral angiolipoma.
Case Description
We present a case of a 54-year-old woman who presented with the right lumbosciatica and distal crural weakness. Spinal MRI showed an epidural lesion at the level of L5-S1 extending to the first right sacral foramen. The patient had total resection of the tumor and the histological study concluded to an angiolipoma. The patients’ neurologic symptoms improved postoperatively and follow-up revealed no signs of tumor recurrence.
Conclusion
Despite the rarity of sacral localization in angiolipomas, it is a diagnosis to be considered in the case of an epidural tumor with foraminal extension. Magnetic resonance imaging is important for detecting and characterizing spinal angiolipomas despite diagnosis is not always obvious. After surgical removal, the functional prognosis is generally favorable.
... They represent 0.14%-1.2% of all spinal tumors, about 2%-3% of epidural spinal tumors and 16%-35% of spinal lipomas. [6] In 1960, Howard and Helwig established it as a separate pathological entity. [7] Till now, only about 200 cases have been reported in literature. ...
Spinal angiolipomas (SALs) are very rare benign extradural neoplasms, representing 0.14%–1.2% of all spinal tumors. Andersson lesion is a localized vertebral or disco-vertebral lesion in ankylosing spondylitis. Co-occurrence of these lesions is very rare and has never been reported. We are reporting one such case of a 61-year-old man presented with features of dorsal compressive myelopathy. On magnetic resonance imaging (MRI), he was diagnosed to have a SAL at D6–D9 and Andersson lesion at D7–D8 and L1–L2. He was managed surgically with a long-segment fixation and decompression and gross total excision of the tumor. Diagnosis of a SAL was confirmed in a biopsy, and postoperatively, he showed good clinical improvement. In conclusion, given the rarity of co-occurrence of these pathologies, a strong clinical suspicion is required. MRI and CT scan help in diagnosis. Both these pathologies warrant surgical management. When diagnosed early and managed appropriately, they have a good prognosis.
... SALs occur mostly in adults aged between 40 and 60 years and have a female predominance, and the duration of disease is usually long. The most common site of SALs is the posterior epidural space at the thoracic level, and multiple segments are often involved 6,14,23,28) . The thoracic predominance of SALs was also found in our series of patients (5/9). ...
... Immunohistochemical assays were performed in seven of our nine patients, and we observed positive staining for CD31, CD34, and S-100 and a low proliferation rate of Ki-67. The results were in agreement with those reported in the literature 6,24,25) . A diagnosis of SALs should be based on a combination of clinical, radiological and pathological examination findings. ...
Objective:
Angiolipomas are usually found in the subcutaneous tissue of the trunk and limbs. Spinal angiolipomas (SALs) are uncommon and have rarely been reported. We report a series of nine SALs patients who received surgical treatment in our department. To summarize the clinical characteristics of SALs, propose our surgical strategies, and evaluate the effects of the operation.
Methods:
This was a retrospective review of nine SALs patients who received surgical treatment from August 2015 to March 2020. Total or subtotal resection was determined by the axial localization (dorsal or ventral) and the extent of intervertebral foramen involvement. The outcomes were assessed based on the modified Japanese Orthopaedic Association (mJOA) scoring system utilized before surgery and at various follow-up points.
Results:
Among the nine patients, the mean mJOA score before surgery was 6.6±2.3, compared with 10.1±1.1 at the last follow-up time point (33.4±11.8 months). All patients achieved good outcomes, even in cases of subtotal resection.
Conclusion:
Early surgical resection of SALs is recommended, and the specific procedures should be determined by the axial localization (dorsal or ventral) and the extent of intervertebral foramen involvement. Most of the patients had a good prognosis, even in cases of subtotal resection.
... Spinal angiolipoma is composed of adipocyte and abnormal vascular elements [1]. It has an incidence of 0.04% to 1.2% among all spinal axis tumors and 2% to 3% among extradural spinal tumors [2]. ...
... 16,67,68 These fatty lesions are much more prevalent in the spine compared to the skull base because there exists a significant, stabilizing fatty matrix. 69 The valveless CSVS in the EDNAC can also be used for dissemination by metastatic cancer. Rao et al. 12 reported a cervical carcinoma that had infiltrated the clival EDNAC via the hematogenous CSVS route. ...
Objective
The extradural neural axis compartment (EDNAC) is an adipovenous zone located between the meningeal and endosteal layers of dura and has been minimally investigated. It runs along the neuraxis from the orbits down to the coccyx and contains fat, valveless veins, arteries and nerves. This review aims to outline the current knowledge regarding the structural and functional significance of the EDNAC.
Methods
A narrative review of EDNAC literature was conducted.
Results
The EDNAC can be organised into four regional enlargements along its length. These are the orbital, lateral sellar, clival and spinal segments with a lateral sellar orbital junction linking the orbital and lateral sellar segments. The orbital EDNAC facilitates movement of the eyeball, while elsewhere it allows limited motility for the meningeal dura. Major nerves and vessels are cushioned and supported by the EDNAC. Raised intraabdominal pressure (IAP) is also conveyed along the spinal EDNAC causing increased venous pressure in the spine and cranium. From a pathological perspective, the EDNAC functions as a low-resistance, extradural passageway that might facilitate tumour encroachment and expansion.
Conclusions
Clinicians should be aware of the extent and significance of the EDNAC which may impact on skull base/spine surgery and present understanding of tumour spread pathways and growth patterns. Comparatively little research has focussed on the EDNAC since its initial description therefore future investigations will provide more information on this underappreciated component of neuraxial anatomy.
... [25] Most of the previous descriptions suggest peak presentation of these lesions in adults. [9] Very few cases have been reported in children of 12 years or below (Table 1). Here, we report a similar case in a one and a halfyear-old child and discuss the relevant literature. ...
... [14] However, the term angiolipoma was coined by Howard and Helwig in 1960 as an anatomopathological entity. [9] They are extremely rare tumors accounting for 2-3% of spinal epidural tumors. [26] Clinical presentation ...
... [11] Spinal angiolipomas differ from spinal lipomas in that the latter is most commonly found in the lumbosacral region and often associated with dysraphic abnormalities. [9] None of the previously reported cases have reported any neural foraminal extension which was unique in this case. ...
Purpose
Spinal angiolipomas are benign spinal epidural tumors consisting of mature fat cells with angiomatous components. These tumors are rare in children and there are no definite management guidelines.
Method
To the best of our knowledge, only five cases of spinal angiolipomas have been reported in children below 12 years. This article discusses the presentation, diagnostics, and surgical tips for the management of a case of spinal angiolipoma.
Result
In this paper, we present a one and half-year-old child presenting with a spontaneous spinal epidural hematoma from a spinal angiolipoma and underscore the importance of early surgery in a benign disease for a good outcome.
Conclusion
Overall neurological recovery and prognosis following timely intervention for spinal angiolipomas are good.
