Fig 13 - uploaded by Louis Wetzel
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-Anterior sacral meningocele in 47year-old woman. A, Axial MR image, 500/17, shows well-defined, low-intensity fluid collection (arrowheads) anterior to sacrum. B, Sagittal MR image, 500/17, just left of midline reveals narrow channel (arrowheads) extending from meningocele (M) via defect in S4 to distal thecal sac.
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Chordoma is a rare slow-growing, locally invasive primary malignant bone tumour arising from notochord remnants. It is characterised by a high local recurrence rate. Most chordomas (60%) are found in the sacrococcygeal region: only 15% originate in the mobile spine. CT-scan and MRI can help evaluate the tumour extension. En bloc resection of the tu...
Sacrococcygeal teratomas (SCTs) are rare congenital tumors. With the improvement of diagnostic imaging methods and follow-up protocols in pregnancies, in utero detection of these tumors has increased. Despite these progresses, SCTs may present difficulties in establishing in utero diagnosis and subsequent management. We present two cases of SCT in...
To evaluate pre-intervention echocardiographic parameters of cardiac function in fetuses who survive without hydrops as compared to fetuses who develop hydrops or perinatal death in the setting of sacrococcygeal teratoma (SCT) and twin-reversed arterial perfusion sequence (TRAP).
Clinical, echocardiographic and sonographic data of fetuses with SCT...
The excision of sacrococcygeal teratoma (SCT) may be associated with significant long-term morbidity for the child. We reviewed our experience with SCT in a tertiary health care facility in a developing country with particular interest on the long-term sequelae.
Between January 1990 and May 2008 inclusive, 38 consecutive children with the diagnosis...
A 60 year old male presented with unremitting complaints of acute urinary retention and constipation. Computerized Tomography of the abdomen and pelvis confirmed a sacral mass. The biopsy diagnosed dedifferentiated sacral chordoma; an extremely rare entity, seldom reported and frequently misdiagnosed. We present a unique case report after an extens...
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... Sacral masses may arise from a number of structures around the sacral region. They can represent malignant lesions that may arise from the axial skeleton as chondromas, from cartilaginous tissues as chondrosarcomas, from the medullary cavity of long bones as an Ewing sarcoma [7][8][9][10]. They can also represent a bone-forming tumour as an osteosarcoma. ...
Sacrococcygeal masses may arise from the axial skeleton, cartilaginous tissue, connective tissue nerve cells, germ cells, or plasma cells. The aid of clinical examinations, imaging, and collaboration with other disciplines is often needed. In this case report, we report the case of a new-born male who was born with a sacrococcygeal mass which was diagnosed pathologically as a low-grade spindle cell tumour. The literature on the demographics, pathology, and guidance on spindle cell tumours is sparse due to their rarity. The prognosis and survival of patients with such tumours are greatly influenced by the primary location's site, stage, pathology, and the treatment selected. Further research is needed to study spindle cell tumours.
... Because most are asymptomatic, their real incidence remains unknown [9]. Primary sacral malignant tumors consist of sacral chordoma, chondrosarcoma, Ewing sarcoma/primitive neuroectodermal tumors (PNET), osteosarcoma, and multiple myeloma/plasmacytoma. Common benign tumors include giant cell tumor (GCT), aneurysmal bone cyst, osteoid osteoma, osteoblastoma, and cavernous hemangioma of the sacrum [12]. ...
Epithelioid hemangioma (EH) is an uncommon benign vascular tumor of mesenchymal origin. It mainly presents as expanding nodules around the ear, the forehead, and long bones. Only a handful of cases have been found in cervical, thoracic, lumbar, and sacral vertebrae as lytic lesions with pain and neurological impairment. We present the case of a 36-year-old female with an incidental finding of a sacral mass along with inguinal lymphadenopathy on imaging. Initially, there were no symptoms. The mass gradually progressed and later showed an extraosseous extension with involvement of sacral neural foramina and nerve roots causing severe low back pain and weakness of the left lower extremity. Differential diagnoses initially included secondary metastases and chordoma. However, the biopsy of the mass revealed findings consistent with an EH. To our knowledge, this is the first case of EH presenting as an isolated mass in the sacrum and the third case of EH involving the sacrum in continuation with other vertebrae. EH should be in our differential diagnoses when there is a sacral mass.
... 7 Chordoma is the most common primary sacral neoplasm. 8 More than one vertebral segment is often involved. ...
BACKGROUND Chordomas are tumours presumed to originate from the remnants of embryonic notochord. They present a diagnostic challenge as they can occur in any region within the craniospinal axis and are rare with an incidence of less than 0.1 / 100,000 per year. Imaging is required for their evaluation and magnetic resonance imaging (MRI) is the imaging modality of choice due to excellent soft tissue resolution, multiplanar imaging capabilities and precise anatomical delineation. METHODS MRI scans of 10 patients, who had proven chordomas histologically, were evaluated retrospectively in the Department of Radiodiagnosis, Bangur Institute of Neurosciences from July 2012 to June 2018. Patients without histological proof of chordomas and those lost to follow-up were not included in the study. Clinical information such as age, sex, presenting symptoms were noted. Imaging parameters assessed included tumour location, bone destruction, extraspinal soft tissue component, spinal canal and neural foramina encroachment, arterial encasement, signal intensity, morphology and enhancement pattern by MRI. All the cases were scanned in a 1.5 Tesla MRI machine. Intravenous contrast were used in all cases. RESULTS Of the 10 cases, 7 were male and 3 were female. There were 7 cases of sacrococcygeal chordomas and 3 cases of clival chordomas. The tumours appeared as multilobulated masses with bone destruction. All sacrococcygeal lesions involved more than one vertebral segment with extraspinal soft tissue component encroaching the pelvic cavity and showed cystic spaces containing hypointense septae. All 3 cases of clival chordomas showed compression of the pons and basilar artery. In one case there was compression of optic chiasma with displacement of internal carotid arteries laterally. On T1 weighted imaging, the chordomas were isointense in 4 and hypointense in 6 cases. These tumours were hyperintense in T2WI in 8 and intermediate to high in signal intensity in 2 cases. All demonstrated heterogenous enhancement on contrast with moderate enhancement in 8 patients and mild enhancement in 2 cases. CONCLUSIONS Chordomas are rare tumours that can occur anywhere in the craniospinal axis. MRI is the modality of choice for imaging of chordomas. Characteristic findings in MRI include low to intermediate signal intensity on T1w images and high signal intensity on T2w images. Sacral chordomas have T2 hyperintense cystic masses with hypointense septa. Enhancement is heterogenous ranging from mild to moderate. MRI is invaluable for a pre-operative diagnosis, delineation of tumour extent and as roadmap for surgery and radiotherapy. KEYWORDS Chordomas, Magnetic Resonance Imaging, Clivus, Sacrococcygeal
... The majority of these are benign, however, malignant transformation has been reported in duplication and epidermoid cysts [2,3]. The less common primary retroperitoneal tumors in the pelvic region include chordomas and the tumors arising from neural elements such as neurofibromas, paragangliomas and schwannomas as well as extraintestinal gastrointestinal stromal tumors (GIST) and primary bone tumors [1,3,4]. Primary retroperitoneal sarcomas in the pelvic region are extremely rare and are listed amongst the miscellaneous group of presacral tumors in most reports [3,5,6]. ...
... Solid tumors in the pre-sacral region include tumors of a neurogenic origin, chordomas and other osseous tumors [2][3][4]. A neural origin of a tumor is suggested when there is a neural foraminal widening or erosion. ...
... On imaging, chordomas typically show sacral destruction with intra sacral (bony) components, along with the pre-and post sacral components. This tumor type shows a mixed signal intensity on T2W sequences [4]. Other osseous tumors extending into the retrorectal region are osteomas, osteogenic sarcomas, giant cell tumors and the Ewing's sarcomas of sacral origin [3,4,6]. ...
Background
Primary tumors in the presacral (retrorectal) space are extremely rare in adults, with an estimated incidence of 0.0025 to 0.014 in large referral centers. Congenital varieties are most common and comprise two thirds of these tumors. Primary retroperitoneal sarcomas in the pelvic region are extremely rare. We report the ultrasound and the corresponding Computed Tomography (CT) features of a rare presacral fibrosarcoma in an adult woman, in whom the diagnosis was confirmed by a CT-guided biopsy.
Case Report
A 54-year-old woman presented with a history of lumbar and perineal pain and painful defecation. Rectal examination revealed a hard mass in the retrorectal space. The patient was referred for imaging and a laboratory evaluation with a clinical diagnosis of a presacral mass. An abdominal ultrasound was followed by laboratory evaluation of routine hematological and hepato-renal parameters, abdomino-pelvic CT and a CT-guided biopsy.
The imaging studies showed a presacral solid mass with nodular calcifications. There was conspicuous absence of cystic or adipose contents and of sacral erosion/destruction. However, hepatic metastasis was present. All imaging features suggested a retroperitoneal sarcoma in the pelvic region with metastases to the liver. The diagnosis was confirmed by CT-guided biopsy.
Conclusions
We are reporting a new case of a very rare entity, a presacral fibrosarcoma. The imaging approach and the analysis leading to an appropriate differential diagnosis and final diagnosis is highlighted in our case report.
... 7 Sacrococcygeal teratoma is the most common presacral mass in children containing all three germ-cell lineages. 10 Benign mature teratomas tend to be predominantly cystic containing fat, sebum, calcification and soft tissue from dermoid plugs. ...
Objectives:
Describe an approach to retrorectal/presacral mass evaluation on imaging with attention to imaging features allowing for refinement of the differential diagnosis of these masses. Elaborate on clinically relevant features that may affect biopsy or surgical approach, of which the radiologist should be aware.
Methods:
A review of current literature regarding diagnosis and treatment of retrorectal/presacral masses was performed with attention to specific findings which may lend refinement of the differential diagnosis of these masses. Cases were obtained by searching through a current database at a single institution after IRB approval.
Results:
Recent advances in imaging and treatment methods have led to increased role of radiology in both imaging and tissue diagnosis of retrorectal masses. Surgical philosophies surrounding treatment of these masses have not significantly changed in recent years but there are a few key factors of which the radiologist must be aware.
Conclusions:
The radiologist can offer refinement of the differential diagnosis of retrorectal masses and can elaborate on salient findings which could alter the need for neoadjuvant chemoradiation therapy, presurgical tissue diagnosis, and surgical approach. Advances in Knowledge: This paper presents an imaging approach to retrorectal/presacral masses with emphasis on findings which can dictate ultimate need for neoadjuvant therapy, presurgical tissue diagnosis and alter the preferred surgical approach. This paper consolidates key findings so radiologists can become more clinically relevant in evaluation of these masses.
... The tumor is often discovered after producing pain and showing neurological symptoms. [14][15][16] Although it is rare, presacral lesions can include various types with almost 50% of presacral tumors are malignant or have areas of malignant changes within them with solid tumors having a higher probability of malignancy than the cystic lesions (60% vs 10%). 5,17 Magnetic resonance imaging (MRI) technique is helpful in detecting specific characteristic and specific radiological features associated with schwannomas. ...
Objective: To demonstrate the feasibility of using da Vinci robotic surgical system to perform spinal surgery.
Methods: Magnetic resonance imaging (MRI) of a 29-year-old female patient complaining right pelvic pain for 1 month revealed a 17x8x10 cm non-homogeneous dumbbell shape encapsulated mass with cystic change located in the pelvic cavity and caused an anterior displacement of urinary bladder and colon.
Results: There was no systemic complication and pain decrease 24 hours after surgery and during 2 years of follow up. The patient started a diet 6 hours after the surgery and was discharged 72 hours after the surgery. The pathological diagnosis of the tumor was schwannoma.
Conclusions: Giant dumbbell shape presacral schwannomas are rare tumours and their surgical treatment is challenging because of the complex anatomy of the presacral. Clinical application of da Vinci robotic surgical system in the spinal surgical field is currently confined to the treatment of some specific diseases or procedures. However, robotic surgery is expected to play a practical future role as it is minimally invasive. The advent of robotic technology will prove to be a boon to the neurosurgeon.
... 3 Although rare, chordoma is the most common tumour of the sacrum. 4 The presenting symptoms and signs depend upon the location of the tumour. Because of the slow growing nature of these tumours, they are usually not discovered until they have become very big. ...
A 57-year-old man presented with features suggestive of a non-functioning pituitary tumour. He underwent trans-sphenoidal resection of the tumour which was found to be a chordoma and not a pituitary adenoma. This case demonstrates that a chordoma may directly involve the sellar region and mimic a pituitary adenoma. We also discuss the aetiology, areas of involvement, management and prognosis of this unusual tumour.
... Sacrococcygeal teratoma is a rare, congenital germ cell tumour and is the most common presacral mass in children. 40 Most are diagnosed in the newborn and are benign, but the risk of malignant transformation increases as the child gets older. Malignant transformation occurs in 5e10% of cases diagnosed before 2 months of age and increases to 50e90% for those diagnosed at 2e4 months of age. ...
... Cystic areas, fat, fluidefluid levels, and calcification are common. 39,40 Malignant sacrococcygeal teratomas cause sacrococcygeal destruction and metastases to the lung, liver, bone, and brain. MRI is superior for the detection of spinal canal invasion. ...
The objective of this review is to discuss the less common causes of rectal and perirectal disease with an emphasis on their differentiating radiological features and the importance of a multimodality imaging approach. Radiologists should be aware of the ultrasound, computed tomography, and magnetic resonance imaging appearances of the wide variety of uncommon lesions arising from the rectal and perirectal regions that may simulate adenocarcinoma in order to render an accurate diagnosis and facilitate appropriate management.
Congenital anomalies of the pediatric spine are ideally evaluated with MR imaging. The wide spectrum of pathology in this category of disease is dramatically displaced for both diagnosis and treatment planning for the neurosurgeon. An understanding of embryology and knowledge of the clinical presentation combined with the MR imaging findings allows the radiologist to play a key role in the evaluation of these complex anomalies.
