con el diagnóstico histopatológico de cordoma de la base del cráneo .. magnética cerebral, corte sagital, secuencia T1, cordoma del clivus. Resección endoscópica de cordoma del clivus. Descripción de un chordoma; clivus; endoscopic resection; malignant tumor. RESUMEN. Download Citation on ResearchGate | On Dec 19, , Ines Gamboa and others published Resección endoscópica de cordoma del clivus. Descripción de un.
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C hordomastumors presumably derived from notochordal rests, are rare. Most are located in the sacrococcygeal and clival regions. Extraosseous processes that histologically resemble a chordoma include ecchordosis physaliphora 14, 17, 22, 25 and intradural chordoma. Vel small asymptomatic and largely intradural masses are considered hamartomas. Like intraosseous notochordal rests, they are developmental lesions but, in contrast, ecchordoses often exhibit a minor intraosseous and a dominant intradural component, the connection being a delicate pedicle traversing a dural defect.
Intradural chordomas are extremely rare and, although they resemble classic chordomas in coroma of both their notochordal origin and microscopic features, they are clinicopathologically distinct. Topographically, they resemble ecchordoses in that some demonstrate a delicate bone-associated pedicle; however, they differ in being larger and more cellular than ecchordoses. Furthermore, unlike other chordomas, intradural chordomas are amenable to resection. We report a case of a lumbar extraosseous extradural tumor with histological features of classic chordoma.
The extradural tumor lay within the loose areolar tissue of Batson’s plexus, demonstrated no osseous involvement, and was completely removed. This previously healthy year-old woman presented with a 2-month history of lumbar, right sacroiliac joint, and right thigh pain.
Chordoma – Wikipedia
The initial lumbar pain, which developed after a prolonged period of sitting, was severe for a number of hours then slowly moderated over 5 days without fully abating. During this time, she also developed right lateral thigh pain that did not respond to rest.
Sitting, especially driving, aggravated the pain, whereas standing and lumbar hyperextension provided some relief. The patient was not aware of progressive neurological loss. Physical examination was unremarkable aside from a finding of tenderness over the right sacroiliac joint and a mildly diminished deep-tendon reflex at the right knee. Laboratory investigations were within normal limits for the patient’s age.
Radiological investigations included magnetic resonance MR imaging with gadolinium enhancement of the lumbar region and an iopamidol myelogram with computerized tomography CT of the lower four lumbar vertebrae. Delayed CT of L-2, L-3, and L-4 at 6, 12, and 24 hours postmyelography demonstrated two abnormalities: The disc protrusion at the L-2 interspace was considered clinically significant in that it explained the presence of pain in the right thigh.
Back pain was ascribed to both the extruded disc and the mass effect of the epidural lesion. Laminectomy and discectomy with exploration of the epidural process were recommended. Left parasagittal magnetic resonance image TR msec, TE 15 msec showing expansion of the L-3 neural foramen by a low-intensity lesion arrowwhich erodes the L-3 pedicle and vertebral body.
Postmyelography computerized tomography scan at the level of the L-3 neural foramen confirming a noncalcified extradural extraosseous mass. Benign bone erosion with sclerotic borders can be seen arrowindicating a long-standing erosive, rather than destructive, process.
Computerized tomography scan at the L-2 interspace demonstrating a focal disc herniation on the right arrow. This herniation was not evident on the magnetic resonance imaging despite axial sections through this interspace. Surgery was performed under general anesthesia by one of the authors B.
Extraosseous spinal chordoma
The yellow-brown spherical tumor was situated between the scalloped pedicle of the L-3 vertebra and nerve root.
It lay within the loose areolar tissue of Batson’s plexus and did not involve surrounding structures such as the common dural sac, nerve roots, vertebral body, or pedicle or ligamentous structures. The solid, spherical, circumscribed tumor wall was surrounded by delicate filamentous connective tissue and contained soft material that could be aspirated, as well as viscous fluid.
The position of the tumor at L-3 and the friability of the tumor tissue resulted in piecemeal removal.
Both of these factors raised the possibility of tumor recurrence and seeding. Histological, immunocytochemical, and ultrastructural studies confirmed the diagnosis of chordoma Figs. Photomicrographs of the tumor specimen. A portion of the tumor with sheet-like growth of vacuolated cells.
A portion of the tumor with typical features of physaliphorous cells. A portion of the tumor showing strong uniform keratin reactivity. Electromicrographs of the tumor specimen. Cells with cytoplasmic vacuoles and clear glycogen lakes arrows are present. Section showing cells with an abundance of intermediate filaments F. Note the interdigitation and desmosomal junctions.
Chordoma | Radiology Reference Article |
The patient made an uneventful postoperative recovery and was ambulating in a cast brace. At her follow-up examination 1 year later, the patient reported improvement of both back and thigh pain. The lumbar fusion was considered sound. Biannual MR imaging will be performed to identify tumor recurrence should it occur. The interest of this case lies not only in the nature and location of the incidentally discovered tumor but also in its radiological presentation as an asymptomatic lesion at one lumbar level associated with a symptomatic disc extrusion at another lumbar level.
Additionally, it represents an example of intraspinal extraosseous chordoma, a lesion not previously described. Like their counterparts at other sites, such tumors are considered to arise from notochordal rests. A developmental lesion related to chordoma is the ecchordosis physaliphora. It is uncommon, occurs almost exclusively in the prepontine and clival regions, and is encountered in 0.
Histologically, they are identical to notochordal tissue. Notochordal rests in the true vertebrae are rare 2 and are primarily located centrally or posteriorly within vertebral bodies. A purported example with unusual light microscopic features was reported by Maesen, et al. These foci were micro- rather than macroscopic, were entirely intraosseous, and bore no physical resemblance to ecchordosis physaliphora occurring in the cranial region.
To be analogous to the intracranial ecchordosis, the designation should be expanded to include extraosseous lesions.
In a recent article, Kurokawa, et al. Their illustration demonstrates a chondroid tumor-like nodule that differs histologically from cranial ecchordosis.
Although the histological, immunocytochemical, and ultrastructural features of this tumor are those of ordinary cranial, vertebral, or sacral chordoma, the tumor described in this report exhibits a different topography and growth pattern, being extraosseous and confined to the epidural space. Scalloping of the pedicle and body of the lumbar vertebrae is attributed to compression and remodeling rather than to destruction of bone.
A CT study of axial chordomas by Krol, et al. Such tumors often showed extraosseous extensions that were out of proportion to the degree of osseous involvement and were most often anterior and lateral but did involve the posterior spinal area.
The radiological appearance of our tumor was not diagnosed either on plain radiography, CT, or MR imaging. In fact, the imaging findings were atypical for vertebral chordoma and more characteristic of either a neurogenic neoplasm, such as neurilemoma or neurofibroma, an arachnoid cyst, or diverticulum.
Plain radiographs of the lumbar spine did not demonstrate a destructive lesion, either lytic or blastic both of which are typical of chordomas.
The only abnormality demonstrated on CT was scalloping of the posterior portion of the vertebral body and enlargement of the neural foramen, both frequently seen in neurogenic tumors. As in the case of chordoma of true vertebrae, our patient’s lesion did not show peripheral amorphous calcification, a common feature of sacral lesions.
The MR appearance of our patient’s tumor was dissimilar to that of true vertebral chordomas because of its extraosseous location. The signal changes seen were nonspecific, and the tumor did not enhance with gadolinium, which is unusual for a neoplasm.
Increased T 2 -weighted MR signal relative to cerebrospinal fluid, which was present, has been described in some spinal and cranial chordomas with high mucoid content. Our experience suggests that at least some cranial chordomas enhance unpublished data.
The purported cervical ecchordosis of Kurokawa, et al. The MR images showed a low-intensity cljvus. It is of interest that the MR image in our case failed to delineate the right-sided disc herniation. The tumor in our patient lent itself to complete surgical removal. This is in sharp distinction to chordomas of the vertebral bodies, which are virtually unresectable.
In a series of spinal chordomas reported by Sundaresan, et al. Based on our morphological and immunohistochemical studies, we propose that the tumor presented in this report, a bona fide intraspinal extraosseous extradural chordoma, had its origin in an ectopic notochordal remnant. The radiological characteristics of the lesion were misleading as codoma mimicked an arachnoid cyst or a neurogenic neoplasm.
Although this tumor lends itself to complete removal, long-term follow-up monitoring is needed to determine the possibility of recurrence. Case Report This previously healthy year-old woman presented with a 2-month history of lumbar, right sacroiliac joint, and right thigh pain. Clivua Physical examination was unremarkable aside from a finding of tenderness over the right sacroiliac joint and a mildly diminished deep-tendon reflex at the right knee.
Operation Surgery was performed under general anesthesia by one of the authors B. Postoperative Course The patient made an uneventful postoperative recovery and was ambulating in a cast brace. Discussion The interest of this case lies not only in the nature and location of the incidentally discovered tumor but also in its radiological presentation as an asymptomatic lesion at one lumbar level associated with a symptomatic disc extrusion at another vel level.
Embryological Considerations A developmental lesion related to chordoma is the ecchordosis physaliphora. Radiographic Features Although the histological, immunocytochemical, and ultrastructural features of this tumor are those of ordinary cranial, vertebral, or sacral chordoma, the tumor described in this report exhibits a different topography and growth pattern, being extraosseous and confined to the epidural space.
Surgical Implications The tumor in our patient lent itself to complete surgical removal. Conclusions Based on our morphological and immunohistochemical studies, we propose that cordomaa tumor presented in this report, a bona fide intraspinal extraosseous extradural chordoma, had its origin in an ectopic notochordal remnant.