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Intradural suprasellar chondroid chordoma
402
Case Reports / Journal of Clinical Neuroscience 17 (2010) 402–403
Intradural suprasellar chondroid chordoma Liu Jiagang a, Liu Yanhui a, Su Xueying b, Mao Qing a,* a b
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu wai nan guo xue xiang 37#, Chengdu, Sichuan 610041, China Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
a r t i c l e
i n f o
Article history: Received 26 March 2009 Accepted 6 May 2009
Keywords: Chondroid chordoma Intradural Suprasellar
a b s t r a c t We report a 51-year-old man with an unusual intradural suprasellar chondroid chordoma. He presented with headache and diminution of vision in both eyes. MRI demonstrated the suprasellar tumor as an isointense mass with heterogeneous enhancement after intravenous administration of contrast agent. There was neither bony nor dural association, and gross-total removal of the mass was performed using a left extended pterional approach. Based on the histological characteristics of the tumor, which was composed of typical chordoma cells and islands of chondroid elements, we diagnosed a chondroid chordoma. We believe this is the first report of an entirely intradural chondroid chordoma on the suprasellar region. Clinical, radiological, and pathological features of the tumor are described. Ó 2009 Elsevier Ltd. All rights reserved.
1. Case report A 51-year-old previously healthy man presented following 6 months of frontal headaches and progressive diminution of vision in both eyes. His right eye was almost blind and visual acuity on the left side had deteriorated with a temporal hemianopia. Laboratory examinations, including endocrinological investigations, were within the normal limits. An MRI of the brain showed a 4 5 6 cm heterogeneous solid mass, mainly in the suprasellar region but also extending to the inferior right frontal lobe. The lesion was isointense on T1-weighted MRI and showed heterogeneous enhancement after injection of contrast medium (Fig. 1A). T2-weighted MRI showed both high-signal areas, as seen in classical chordoma, and low-signal areas that indicated the calcified chondroid component (Fig. 1B). Cerebral angiography demonstrated no tumor stain and normal vessels. The tumor was resected via an extended pterional approach. The lesion appeared to be totally intradural and compressed the inferior aspect of the right frontal lobe and temporal lobe. The right internal carotid artery and its branches were carefully dissected and preserved. Although the calcified component made the mass difficult to remove, radical excision was achieved (Fig. 1C). Histological studies of the resected specimen revealed the tumor was composed of both chordoma tissue with dense vacuolated cells and cartilaginous tissue (Fig. 2a). Immunohistochemical examination showed the tumor was positive for, epithelial membrane antigen (EMA) (Fig. 2b), cytokeratin (Fig. 2c) and S100 protein, which confirmed our diagnosis of a chondroid chordoma (Fig. 2). Postoperatively, the patient regained limited vision in the left eye. Because the tumor was completely resected, no postoperative radiotherapy was administered. At the 10-month follow-up, the patient remained well with no tumor recurrence on MRI. 2. Discussion Chordomas are slow-growing midline tumors that tend to occur at either end of the primitive notochord and account for approximately 1% of intracranial tumors.1 They occur mostly
* Corresponding author. Tel.: +86 028 85596336. E-mail address: [email protected] (M. Qing).
in the sacrococcygeal region (49% of patients), followed by the spheno-occipital region (clival) (36%), and the vertebral region (15%).2 The vast majority of these lesions are locally aggressive, with extradural or transdural extension, and bone destruction has often been described as a hallmark of chordomas.3 Chondroid chordoma is a subtype of chordoma originally described by Heffelfinger as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue.2 These tumors range from being mostly chordoma, with small scattered foci of cartilaginous differentiation, to mostly cartilaginous tumors with small areas of chordoma.4 In our patient, T1-weighted MRI typically demonstrated a suprasellar mass that was isointense with heterogeneous contrast enhancement. T2-weighted MRI showed hyperintense areas indicating the classical chordoma component. A large, lobulated, low-signal calcified area indicated the cartilaginous component comprising approximately 70% of the mass. Chondroid chordoma must also be differentiated from chondrosarcoma, which has a similar clinical and histological appearance. Immunohistochemical markers are useful to distinguish these tumors; chordomas stain positively for epithelial markers, such as cytokeratin and EMA, which are negative in chondrosarcomas of mesodermal origin.1 Our patient’s tumor showed positive staining for cytokeratin, EMA, and S100 protein. Intradural cranial chordomas must be distinguished from ecchordosis physaliphora (EP), which is a non-neoplastic notochordal remnant.5,6 This benign lesion is usually a small, gelatinous nodule, but the size can vary from a few millimeters to 2 cm.7 EP is usually asymptomatic and found incidentally in 2% of autopsies or imaging studies. To date, only 9 symptomatic EPs have been reported.6,8,9 Intradural chordoma and EP are thought to originate from the same embryological remnant. They occur in the same anatomic location, and have identical microscopic, immunohistochemical and ultrastructural characteristics.9,10 MRI is useful to determine the differential diagnosis. In all reported patients, EP appears homogeneously hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI without contrast enhancement after gadolinium injection. The lack of enhancement might be considered a distinctive feature of EP.7,8 T1-weighted MRI showed our patient had a large mass with heterogeneous enhancement, thus indicating it was not an EP. Primary intradural cranial chordomas are rare; only 25 patients with intradural and extra-ossous cranial chordomas have
Case Reports / Journal of Clinical Neuroscience 17 (2010) 402–403
403
Fig. 1. (A) Coronal T1-weighted MRI showing the heterogeneous intradural mass in the suprasellar region. (B) Axial T2-weighted MRI showing the high-signal chordoma component in the midline and the low-signal chondroid component extending to right and compressing the right temporal lobe (arrows). (C) Intraoperative photograph showing two different components of the tumor: a conventional grayish lobulated chordoma (white arrow) and a chondroid calcified element (black arrow).
Fig. 2. Histopathological features of the chondroid chordoma showing (a) typical physaliphorous cells arranged in nests (hematoxylin and eosin 100), (b) positive immunochemistry staining for epithelial membrane antigen (400), and (c) positive immunochemistry staining for cytokeratins (400).
been reported.5,6,11 No guidelines exist for optimal management of primary intradural chordomas; however, it is generally agreed that patients benefit from aggressive, but safe, surgery. In typical extradural chordomas, complete resection is usually not feasible even with extensive surgery because a few tumor cells often remain in the bone, and local recurrences are common. Compared with typical extradural chordomas, intradural chordomas are more suitable for complete resection because of the well-circumscribed margins in addition to the absence of bone invasion.3,4,6 Interestingly, no signs of recurrence or metastasis associated with intradural chordomas have been reported at the follow-up examinations.5,6 It is possible that the degree of resection plays a major role in influencing the duration of the recurrence-free period. In our patient, the tumor was radically resected via an extended pterional approach without postoperative radiotherapy or chemotherapy. At the 10-month follow-up there was no evidence of recurrence. Nevertheless, the information describing pure intradural chordomas is so limited that we are unable to provide statistically significant data to either support or deny the observation that intradural chordomas have a better prognosis than typical extradural chordomas. In conclusion, safe maximal resection of suprasellar intradural chondroid chordoma is warranted to promote a good outcome.
doi:10.1016/j.jocn.2009.05.017
References 1. Rosenberg AE, Brown GA, Bhan AK, et al. Chondroid chordoma–a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol 1994;101:36–41. 2. Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410–20. 3. Rich TA, Schiller A, Suit HD, et al. Clinical and pathologic review of 48 cases of chordoma. Cancer 1985;56:182–7. 4. Jeffrey PB, Biava CG, Davis RL. Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. Am J Clin Pathol 1995;103:271–9. 5. Roberti F, Sekhar LN, Jones RV, et al. Intradural cranial chordoma: a rare presentation of an uncommon tumor. J Neurosurg 2007;106:270–4. 6. Ciarpaglini R, Pasquini E, Mazzatenta D, et al. Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report. Neurosurgery 2009;62:E387–8. 7. Rodriguez L, Colina J, Lopez J, et al. Intradural prepontine growth: giant ecchordosis physaliphora or extraosseous chordoma? Neuropathology 1999;19:336–40. 8. Mehnert F, Beschorner R, Küker W, et al. Retroclival ecchordosis physaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol 2004;25:1851–5. 9. Rotondo M, Natale M, Mirone G, et al. A rare symptomatic presentation of ecchordosis physaliphora: neuroradiological and surgical management. J Neurol Neurosurg Psychiatry 2007;78:647–9. 10. Ling SS, Sader C, Robbins P, et al. A case of giant ecchordosis physaliphora: a case report and literature review. Otol Neurotol 2007;28:931–3. 11. Ito E, Saito K, Nagatani T, et al. Intradural cranial chordoma. Surg Neurol 2009. doi:10.1016/j.surneu.2009.01.003.
Case Reports / Journal of Clinical Neuroscience 17 (2010) 402–403
Intradural suprasellar chondroid chordoma Liu Jiagang a, Liu Yanhui a, Su Xueying b, Mao Qing a,* a b
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu wai nan guo xue xiang 37#, Chengdu, Sichuan 610041, China Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
a r t i c l e
i n f o
Article history: Received 26 March 2009 Accepted 6 May 2009
Keywords: Chondroid chordoma Intradural Suprasellar
a b s t r a c t We report a 51-year-old man with an unusual intradural suprasellar chondroid chordoma. He presented with headache and diminution of vision in both eyes. MRI demonstrated the suprasellar tumor as an isointense mass with heterogeneous enhancement after intravenous administration of contrast agent. There was neither bony nor dural association, and gross-total removal of the mass was performed using a left extended pterional approach. Based on the histological characteristics of the tumor, which was composed of typical chordoma cells and islands of chondroid elements, we diagnosed a chondroid chordoma. We believe this is the first report of an entirely intradural chondroid chordoma on the suprasellar region. Clinical, radiological, and pathological features of the tumor are described. Ó 2009 Elsevier Ltd. All rights reserved.
1. Case report A 51-year-old previously healthy man presented following 6 months of frontal headaches and progressive diminution of vision in both eyes. His right eye was almost blind and visual acuity on the left side had deteriorated with a temporal hemianopia. Laboratory examinations, including endocrinological investigations, were within the normal limits. An MRI of the brain showed a 4 5 6 cm heterogeneous solid mass, mainly in the suprasellar region but also extending to the inferior right frontal lobe. The lesion was isointense on T1-weighted MRI and showed heterogeneous enhancement after injection of contrast medium (Fig. 1A). T2-weighted MRI showed both high-signal areas, as seen in classical chordoma, and low-signal areas that indicated the calcified chondroid component (Fig. 1B). Cerebral angiography demonstrated no tumor stain and normal vessels. The tumor was resected via an extended pterional approach. The lesion appeared to be totally intradural and compressed the inferior aspect of the right frontal lobe and temporal lobe. The right internal carotid artery and its branches were carefully dissected and preserved. Although the calcified component made the mass difficult to remove, radical excision was achieved (Fig. 1C). Histological studies of the resected specimen revealed the tumor was composed of both chordoma tissue with dense vacuolated cells and cartilaginous tissue (Fig. 2a). Immunohistochemical examination showed the tumor was positive for, epithelial membrane antigen (EMA) (Fig. 2b), cytokeratin (Fig. 2c) and S100 protein, which confirmed our diagnosis of a chondroid chordoma (Fig. 2). Postoperatively, the patient regained limited vision in the left eye. Because the tumor was completely resected, no postoperative radiotherapy was administered. At the 10-month follow-up, the patient remained well with no tumor recurrence on MRI. 2. Discussion Chordomas are slow-growing midline tumors that tend to occur at either end of the primitive notochord and account for approximately 1% of intracranial tumors.1 They occur mostly
* Corresponding author. Tel.: +86 028 85596336. E-mail address: [email protected] (M. Qing).
in the sacrococcygeal region (49% of patients), followed by the spheno-occipital region (clival) (36%), and the vertebral region (15%).2 The vast majority of these lesions are locally aggressive, with extradural or transdural extension, and bone destruction has often been described as a hallmark of chordomas.3 Chondroid chordoma is a subtype of chordoma originally described by Heffelfinger as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue.2 These tumors range from being mostly chordoma, with small scattered foci of cartilaginous differentiation, to mostly cartilaginous tumors with small areas of chordoma.4 In our patient, T1-weighted MRI typically demonstrated a suprasellar mass that was isointense with heterogeneous contrast enhancement. T2-weighted MRI showed hyperintense areas indicating the classical chordoma component. A large, lobulated, low-signal calcified area indicated the cartilaginous component comprising approximately 70% of the mass. Chondroid chordoma must also be differentiated from chondrosarcoma, which has a similar clinical and histological appearance. Immunohistochemical markers are useful to distinguish these tumors; chordomas stain positively for epithelial markers, such as cytokeratin and EMA, which are negative in chondrosarcomas of mesodermal origin.1 Our patient’s tumor showed positive staining for cytokeratin, EMA, and S100 protein. Intradural cranial chordomas must be distinguished from ecchordosis physaliphora (EP), which is a non-neoplastic notochordal remnant.5,6 This benign lesion is usually a small, gelatinous nodule, but the size can vary from a few millimeters to 2 cm.7 EP is usually asymptomatic and found incidentally in 2% of autopsies or imaging studies. To date, only 9 symptomatic EPs have been reported.6,8,9 Intradural chordoma and EP are thought to originate from the same embryological remnant. They occur in the same anatomic location, and have identical microscopic, immunohistochemical and ultrastructural characteristics.9,10 MRI is useful to determine the differential diagnosis. In all reported patients, EP appears homogeneously hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI without contrast enhancement after gadolinium injection. The lack of enhancement might be considered a distinctive feature of EP.7,8 T1-weighted MRI showed our patient had a large mass with heterogeneous enhancement, thus indicating it was not an EP. Primary intradural cranial chordomas are rare; only 25 patients with intradural and extra-ossous cranial chordomas have
Case Reports / Journal of Clinical Neuroscience 17 (2010) 402–403
403
Fig. 1. (A) Coronal T1-weighted MRI showing the heterogeneous intradural mass in the suprasellar region. (B) Axial T2-weighted MRI showing the high-signal chordoma component in the midline and the low-signal chondroid component extending to right and compressing the right temporal lobe (arrows). (C) Intraoperative photograph showing two different components of the tumor: a conventional grayish lobulated chordoma (white arrow) and a chondroid calcified element (black arrow).
Fig. 2. Histopathological features of the chondroid chordoma showing (a) typical physaliphorous cells arranged in nests (hematoxylin and eosin 100), (b) positive immunochemistry staining for epithelial membrane antigen (400), and (c) positive immunochemistry staining for cytokeratins (400).
been reported.5,6,11 No guidelines exist for optimal management of primary intradural chordomas; however, it is generally agreed that patients benefit from aggressive, but safe, surgery. In typical extradural chordomas, complete resection is usually not feasible even with extensive surgery because a few tumor cells often remain in the bone, and local recurrences are common. Compared with typical extradural chordomas, intradural chordomas are more suitable for complete resection because of the well-circumscribed margins in addition to the absence of bone invasion.3,4,6 Interestingly, no signs of recurrence or metastasis associated with intradural chordomas have been reported at the follow-up examinations.5,6 It is possible that the degree of resection plays a major role in influencing the duration of the recurrence-free period. In our patient, the tumor was radically resected via an extended pterional approach without postoperative radiotherapy or chemotherapy. At the 10-month follow-up there was no evidence of recurrence. Nevertheless, the information describing pure intradural chordomas is so limited that we are unable to provide statistically significant data to either support or deny the observation that intradural chordomas have a better prognosis than typical extradural chordomas. In conclusion, safe maximal resection of suprasellar intradural chondroid chordoma is warranted to promote a good outcome.
doi:10.1016/j.jocn.2009.05.017
References 1. Rosenberg AE, Brown GA, Bhan AK, et al. Chondroid chordoma–a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol 1994;101:36–41. 2. Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410–20. 3. Rich TA, Schiller A, Suit HD, et al. Clinical and pathologic review of 48 cases of chordoma. Cancer 1985;56:182–7. 4. Jeffrey PB, Biava CG, Davis RL. Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. Am J Clin Pathol 1995;103:271–9. 5. Roberti F, Sekhar LN, Jones RV, et al. Intradural cranial chordoma: a rare presentation of an uncommon tumor. J Neurosurg 2007;106:270–4. 6. Ciarpaglini R, Pasquini E, Mazzatenta D, et al. Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report. Neurosurgery 2009;62:E387–8. 7. Rodriguez L, Colina J, Lopez J, et al. Intradural prepontine growth: giant ecchordosis physaliphora or extraosseous chordoma? Neuropathology 1999;19:336–40. 8. Mehnert F, Beschorner R, Küker W, et al. Retroclival ecchordosis physaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol 2004;25:1851–5. 9. Rotondo M, Natale M, Mirone G, et al. A rare symptomatic presentation of ecchordosis physaliphora: neuroradiological and surgical management. J Neurol Neurosurg Psychiatry 2007;78:647–9. 10. Ling SS, Sader C, Robbins P, et al. A case of giant ecchordosis physaliphora: a case report and literature review. Otol Neurotol 2007;28:931–3. 11. Ito E, Saito K, Nagatani T, et al. Intradural cranial chordoma. Surg Neurol 2009. doi:10.1016/j.surneu.2009.01.003.