Case Report
Chordoma of skull base presenting as nasopharyngeal mass Sant Prakash Kataria, Ashima Batra, Gajender Singh, Sanjay Kumar, Rajeev Sen Department of Pathology, Pt B.D. Sharma PGIMS, Rohtak, Haryana, India
ABSTRACT While the nasopharynx is most commonly regarded by the otolaryngologist as a primary site of neoplastic involvement, it is also an avenue of spread of base‑of‑the‑skull tumors presenting as bulging nasopharyngeal masses. Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base/clivus are typically locally aggressive with lytic bone destruction. The optimal treatment may be photon/proton radiotherapy alone or combined with a gross total resection, when feasible. We report a case of intracranial chordoma presenting as nasopharyngeal mass. Key words: Chordoma, nasopharyngeal mass, skull base
Introduction
Case Report
Intracranial chordomas are relatively rare locally aggressive tumors thought to originate along the course of the embryonic remnant of the notochord. The most common location is in the sacrum; however, the skull base/clivus region is the second most common location along the course of the notochord.[1]
A 28 year‑old female sought medical attention for pain in throat and difficulty in deglutition for past 2 months. On anterior rhinoscopy, a pale mass was seen occupying right nasal cavity. The left nasal cavity was normal. Oral cavity examination revealed a smooth bulge on right‑sided soft palate and anterior pillar of right tonsil, because of which the right tonsil could not be visualized. Uvula was in the midline. Indirect laryngoscopy could not be done. On central nervous system (CNS) examination, bilateral occulomotor and right hypoglossal nerves were involved. Fibreoptic nasopharyngoscopy was done under local anesthesia. A mass was seen in superior part of nasopharynx. Other sites of nasopharynx including fossa of Rosenmuller, eustacian tube opening, and torus tubarius were unremarkable.
Skull base chordomas usually arise in the clivus and are rarely completely resectable. [2] They may cause extensive bony erosion of the petrous apex, sphenoid sinus, and clivus and may suggest a more rapidly growing and aggressive tumor type. The extent of the tumor may be accurately determined by conventional tomography, computerized axial tomography, and arteriography.[3] Most are treated with radiotherapy (RT) because of extensive involvement making them unresectable. Because of the risk of severe late complications, the dose is often limited with conventional photon RT, and the probability of cure is low.[2] Access this article online Quick Response Code:
Website: www.ruralneuropractice.com
DOI: 10.4103/0976-3147.116426
Computed tomography (CT) scan of head and neck [Figure 1a and b] showed that base of skull was eroded with extracranial extension of bilateral temporal lobes and occipital lobe. The mass was compressing and pushing brainstem and extending into orbit and bilateral pterygopalatine and infratemporal fossae. Biopsy of the mass was performed. Histological examination revealed round to oval cells with central nuclei and a vacuolated cytoplasm (physaliphorous cells) [Figure 2a]. The cells stained positive with Periodic acid‑Schiff (PAS) [Figure 2b] and monoclonal staining
Address for correspondence: Dr. Ashima Batra, H. No. 901 Ward No. 22, Jhang Colony, Rohtak, Haryana, India. E‑mail: [email protected] Journal of Neurosciences in Rural Practice | 2013 | Vol 4 | Supplement 1
S95
Kataria, et al.: Chordoma as nasopharyngeal mass
a
a
b
Figure 1: (a and b) CT scan showing erosion of base of skull with extracranial extension
for epithelial membrane antigen (EMA) [Figure 2c]. A histopathological diagnosis of chordoma was made. The patient was advised RT but the patient did not comply and became lost to follow up.
Discussion Chordoma is a rare, low‑grade, primary malignant bone tumor arising from primitive notochord remnants of the axial skeleton. It accounts for 1-4% of all primary skeletal tumors and its incidence rate is inferior to 0.1 per 100,000 inhabitants per year. [4] They arise from the sacrum in approximately 50-60% of cases, from the skull base region (spheno‑occipital/nasal) in approximately 25-35% of cases, from the cervical vertebrae in approximately 10% of cases, and from the thoracolumbar vertebrae in approximately 5% of cases. The median age at presentation is around 60 years; however, presentation with skull base tumors may occur at a younger age and has been reported in children and adolescents.[5] Intracranial chordomas have a male (2:1) predilection.[6] The clinical features of chordoma will depend on the site and line of spread of the tumor. In the cranial case, which arises in the region of the basi‑sphenoid, the growth spreads intracranially at an early stage and death eventually results from compression of vital structures. Apart from invading the cranial cavity, the growth sometimes projects into the nasopharynx as well when symptoms of obstruction will be produced. The prominent complaints will therefore consist of headache and cranial nerve palsies due to intracranial spread, nasal obstruction with discharge, and dysphagia in those with pharyngeal spread. Epistaxis as in nasopharyngeal carcinoma is not uncommon.[7] Chordomas are usually relatively slow‑growing, low‑grade malignancies. [5] They have been considered of low S96
b
c
Figure 2: Photomicrograph showing round to oval cells with central nuclei and vacuolated cytoplasm (physaliphorous cells) (a) H and E (×40). These cells stained positive with (b) PAS (×40) and (c) monoclonal staining for EMA (×100)
metastatic potential; however, distant metastasis to lung, bone, soft tissue, lymph node, liver, and skin has been reported in up to 43% of patients.[8] However, metastatic sites usually occur late in the course of the disease.[4] Both CT and magnetic resonance (MR) imaging are used in the evaluation of chordoma. CT is ideal for evaluating the bony involvement, whereas MR imaging is useful in evaluating the surrounding soft tissues and extension into adjacent structures.[1] Histologically, they display lobules and vacuolated (physaliphorous), moderately atypical, neoplastic cells across a myxoid stroma separated by fibrous bands.[9] Adequate wide surgery still remains the cornerstone of chordoma treatment even though safe margins are often hard to obtain because of its anatomical sites of origin.[8] Surgery for skull base and upper cervical spine tumors poses a risk of damage to normal structures, including the spinal cord and cranial nerves. These same normal tissue structures also impose a limitation on the external‑beam RT dose, which adds additional difficulty. The advent of advanced imaging, planning, and delivery of photon RT over the past one to two decades has provided opportunities for delivering high doses of radiation safely to patients with skull base and cervical spine tumors.[4] Proton RT alone or combined with photon RT (proton/photon RT) offers the advantage of improved dose distribution and the ability to treat the tumor to a higher dose without exceeding normal tissue tolerance.[2] Sensitivity to chemotherapy is very low and generally reported in the small subgroup of patients with high‑grade dedifferentiated chordomas with agents active in high‑grade sarcomas.[10] The best results in the treatment of chordomas of the skull base are reported when using surgery and adjuvant high‑dose proton RT.[4] Journal of Neurosciences in Rural Practice | 2013 | Vol 4 | Supplement 1
Kataria, et al.: Chordoma as nasopharyngeal mass
Conclusion Chordoma of skull base is a rare entity. It can sometimes present as nasopharyngeal mass which can be mistaken for primary nasopharyngeal mass. To minimize delay in diagnosis, nasopharyngeal extension of an intracranial chordoma should be considered in the differential diagnosis of any mass forming lesion in nasopharynx.
References 1. 2. 3. 4.
Nguyen RP, Salzman KL, Stambuk HE, Ahuja AT, Harnsberger HR. Extraosseous Chordoma of the Nasopharynx. AJNR Am J Neuroradiol 2009;30:803‑7. Mendenhall WM, Mendenhall CM, Lewis SB, Villaret DB, Mendenhall NP. Skull base chordoma. Head Neck 2005;27:159‑65. Eisemann ML. Sphenooccipital chordoma presenting as a nasopharyngeal mass. A case report. Ann Otol Rhinol Laryngol 1980;89:271‑5. Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH.
Chordoma: The nonsarcoma primary bone tumor. Oncologist 2007;12:1344‑50. 5. Mirra JM, Della Rocca C, Nelson SD, Mertens F. Chordoma. In: Fletcher CD, Unni K, Mertens F, editors. Pathology and genetics of tumours of soft tissue and bone. Lyon, France: IARC Press; 2002. p. 316‑7. 6. Yan ZY, Yang BT, Wang ZC, Xian JF, Li M. Primary chordoma in the nasal cavity and nasopharynx: CT and MR imaging findings. AJNR Am J Neuroradiol 2010;31:246‑50. 7. Cheng TB. Nasopharyngeal Chordoma. Singapore Med J 1963;3:168‑70. 8. Ferraresi V, Nuzzo C, Zoccali C, Marandino F, Vidiri A, Salducca N, et al. Chordoma: Clinical characteristics, management and prognosis of a case series of 25 patients. BMC Cancer 2010;10:22. 9. Baratti D, Gronchi A, Pennacchioli E, Lozza L, Colecchia M, Fiore M, et al. Chordoma: Natural history and results in 28 patients treated at a single institution. Ann Surg Oncol 2003;10:291‑6. 10. Fleming GF, Heimann PS, Stephens JK, Simon MA, Ferguson MK, Benjamin RS, et al. Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer 1993;72:714‑8. How to cite this article: Kataria SP, Batra A, Singh G, Kumar S, Sen R. Chordoma of skull base presenting as nasopharyngeal mass. J Neurosci Rural Pract 2013, 4(Suppl 1):s95-7. Source of Support: Nil. Conflict of Interest: None declared.
Commentary Nasopharynx is the uppermost portion of the pharynx. It is part of upper respiratory tract serving as a conduit between the nasal cavity and the oropharynx. It houses few important structures namely from lateral, the openings of the Eustachian tube (ET), torus tubarius, Fossa of Rosenmuller (FOR), and the adenoid (nasopharyngeal tonsil) in the midline. As the location of the region is posterior to the nasal cavity, examination of the nose by anterior rhinoscopy for a suspected lesion in the nasopharynx often reveals a negative finding. The use of post‑nasal space mirror in posterior rhinoscopy is considered suboptimal in these days to evaluate the nasopharyngeal region. The subtle changes of the mucosal lesion may be easily overlooked. Thus, a nasoendoscopy using a zero degree rigid endoscope or a flexible nasopharyngolaryngoscope should always be performed. In the index case, owing to the big size, the mass was detected from the oral cavity inspection, as a bulged on the soft palate.[1] If the mass was smaller at presentation, it would have been easily missed. On the contrary, the most common pathology in the nasopharynx is the nasopharyngeal carcinoma (NPC), which is almost always originates from the FOR. The special feature of NPC is that it produces classical clinical features before it can attain the size as big as the visualized chordoma in the index case. The Journal of Neurosciences in Rural Practice | 2013 | Vol 4 | Supplement 1
commonest presentation of an NPC is the cervical lymphadenopathy. It constituted more than 80% of clinical signs at presentation of NPC patients in our center.[2] This figure is consistent with reported findings in other reports as well. Besides that, unilateral conductive hearing loss (CHL) can be the sole presenting feature, owing to the primary site, the FOR which is located just lateral to the ET opening. The growing mass from the FOR can easily impinge or occlude, leading to the impairment of the ET function. Pressure different or collection of effusion in the middle ear leads to CHL. In this part of the world (Asia particularly South East Asia) where the NPC is endemic, an adult patient with unilateral CHL should be suspected NPC until proven otherwise. In our local series, more than 50% of NPC patients were having otitis media effusion, mostly unilateral.[2] Other rare presentations include abducens nerve palsy causing diplopia, obstructive symptoms leading to snoring, hyponasality, and sleep apnea. Traumatized surface of the mass or exophytic lesion of NPC may produce epistaxis. In the absence of the mass in FOR but with strong clinical suspicious of NPC, the radiological imaging such as computed tomography scan or magnetic resonance (MR) imaging is valuable to pick up the sub‑mucosal type of NPC.[3] In areas of NPC endemics, for example China, the use of MR imaging as a screening S97
Copyright of Journal of Neurosciences in Rural Practice is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Chordoma of skull base presenting as nasopharyngeal mass Sant Prakash Kataria, Ashima Batra, Gajender Singh, Sanjay Kumar, Rajeev Sen Department of Pathology, Pt B.D. Sharma PGIMS, Rohtak, Haryana, India
ABSTRACT While the nasopharynx is most commonly regarded by the otolaryngologist as a primary site of neoplastic involvement, it is also an avenue of spread of base‑of‑the‑skull tumors presenting as bulging nasopharyngeal masses. Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base/clivus are typically locally aggressive with lytic bone destruction. The optimal treatment may be photon/proton radiotherapy alone or combined with a gross total resection, when feasible. We report a case of intracranial chordoma presenting as nasopharyngeal mass. Key words: Chordoma, nasopharyngeal mass, skull base
Introduction
Case Report
Intracranial chordomas are relatively rare locally aggressive tumors thought to originate along the course of the embryonic remnant of the notochord. The most common location is in the sacrum; however, the skull base/clivus region is the second most common location along the course of the notochord.[1]
A 28 year‑old female sought medical attention for pain in throat and difficulty in deglutition for past 2 months. On anterior rhinoscopy, a pale mass was seen occupying right nasal cavity. The left nasal cavity was normal. Oral cavity examination revealed a smooth bulge on right‑sided soft palate and anterior pillar of right tonsil, because of which the right tonsil could not be visualized. Uvula was in the midline. Indirect laryngoscopy could not be done. On central nervous system (CNS) examination, bilateral occulomotor and right hypoglossal nerves were involved. Fibreoptic nasopharyngoscopy was done under local anesthesia. A mass was seen in superior part of nasopharynx. Other sites of nasopharynx including fossa of Rosenmuller, eustacian tube opening, and torus tubarius were unremarkable.
Skull base chordomas usually arise in the clivus and are rarely completely resectable. [2] They may cause extensive bony erosion of the petrous apex, sphenoid sinus, and clivus and may suggest a more rapidly growing and aggressive tumor type. The extent of the tumor may be accurately determined by conventional tomography, computerized axial tomography, and arteriography.[3] Most are treated with radiotherapy (RT) because of extensive involvement making them unresectable. Because of the risk of severe late complications, the dose is often limited with conventional photon RT, and the probability of cure is low.[2] Access this article online Quick Response Code:
Website: www.ruralneuropractice.com
DOI: 10.4103/0976-3147.116426
Computed tomography (CT) scan of head and neck [Figure 1a and b] showed that base of skull was eroded with extracranial extension of bilateral temporal lobes and occipital lobe. The mass was compressing and pushing brainstem and extending into orbit and bilateral pterygopalatine and infratemporal fossae. Biopsy of the mass was performed. Histological examination revealed round to oval cells with central nuclei and a vacuolated cytoplasm (physaliphorous cells) [Figure 2a]. The cells stained positive with Periodic acid‑Schiff (PAS) [Figure 2b] and monoclonal staining
Address for correspondence: Dr. Ashima Batra, H. No. 901 Ward No. 22, Jhang Colony, Rohtak, Haryana, India. E‑mail: [email protected] Journal of Neurosciences in Rural Practice | 2013 | Vol 4 | Supplement 1
S95
Kataria, et al.: Chordoma as nasopharyngeal mass
a
a
b
Figure 1: (a and b) CT scan showing erosion of base of skull with extracranial extension
for epithelial membrane antigen (EMA) [Figure 2c]. A histopathological diagnosis of chordoma was made. The patient was advised RT but the patient did not comply and became lost to follow up.
Discussion Chordoma is a rare, low‑grade, primary malignant bone tumor arising from primitive notochord remnants of the axial skeleton. It accounts for 1-4% of all primary skeletal tumors and its incidence rate is inferior to 0.1 per 100,000 inhabitants per year. [4] They arise from the sacrum in approximately 50-60% of cases, from the skull base region (spheno‑occipital/nasal) in approximately 25-35% of cases, from the cervical vertebrae in approximately 10% of cases, and from the thoracolumbar vertebrae in approximately 5% of cases. The median age at presentation is around 60 years; however, presentation with skull base tumors may occur at a younger age and has been reported in children and adolescents.[5] Intracranial chordomas have a male (2:1) predilection.[6] The clinical features of chordoma will depend on the site and line of spread of the tumor. In the cranial case, which arises in the region of the basi‑sphenoid, the growth spreads intracranially at an early stage and death eventually results from compression of vital structures. Apart from invading the cranial cavity, the growth sometimes projects into the nasopharynx as well when symptoms of obstruction will be produced. The prominent complaints will therefore consist of headache and cranial nerve palsies due to intracranial spread, nasal obstruction with discharge, and dysphagia in those with pharyngeal spread. Epistaxis as in nasopharyngeal carcinoma is not uncommon.[7] Chordomas are usually relatively slow‑growing, low‑grade malignancies. [5] They have been considered of low S96
b
c
Figure 2: Photomicrograph showing round to oval cells with central nuclei and vacuolated cytoplasm (physaliphorous cells) (a) H and E (×40). These cells stained positive with (b) PAS (×40) and (c) monoclonal staining for EMA (×100)
metastatic potential; however, distant metastasis to lung, bone, soft tissue, lymph node, liver, and skin has been reported in up to 43% of patients.[8] However, metastatic sites usually occur late in the course of the disease.[4] Both CT and magnetic resonance (MR) imaging are used in the evaluation of chordoma. CT is ideal for evaluating the bony involvement, whereas MR imaging is useful in evaluating the surrounding soft tissues and extension into adjacent structures.[1] Histologically, they display lobules and vacuolated (physaliphorous), moderately atypical, neoplastic cells across a myxoid stroma separated by fibrous bands.[9] Adequate wide surgery still remains the cornerstone of chordoma treatment even though safe margins are often hard to obtain because of its anatomical sites of origin.[8] Surgery for skull base and upper cervical spine tumors poses a risk of damage to normal structures, including the spinal cord and cranial nerves. These same normal tissue structures also impose a limitation on the external‑beam RT dose, which adds additional difficulty. The advent of advanced imaging, planning, and delivery of photon RT over the past one to two decades has provided opportunities for delivering high doses of radiation safely to patients with skull base and cervical spine tumors.[4] Proton RT alone or combined with photon RT (proton/photon RT) offers the advantage of improved dose distribution and the ability to treat the tumor to a higher dose without exceeding normal tissue tolerance.[2] Sensitivity to chemotherapy is very low and generally reported in the small subgroup of patients with high‑grade dedifferentiated chordomas with agents active in high‑grade sarcomas.[10] The best results in the treatment of chordomas of the skull base are reported when using surgery and adjuvant high‑dose proton RT.[4] Journal of Neurosciences in Rural Practice | 2013 | Vol 4 | Supplement 1
Kataria, et al.: Chordoma as nasopharyngeal mass
Conclusion Chordoma of skull base is a rare entity. It can sometimes present as nasopharyngeal mass which can be mistaken for primary nasopharyngeal mass. To minimize delay in diagnosis, nasopharyngeal extension of an intracranial chordoma should be considered in the differential diagnosis of any mass forming lesion in nasopharynx.
References 1. 2. 3. 4.
Nguyen RP, Salzman KL, Stambuk HE, Ahuja AT, Harnsberger HR. Extraosseous Chordoma of the Nasopharynx. AJNR Am J Neuroradiol 2009;30:803‑7. Mendenhall WM, Mendenhall CM, Lewis SB, Villaret DB, Mendenhall NP. Skull base chordoma. Head Neck 2005;27:159‑65. Eisemann ML. Sphenooccipital chordoma presenting as a nasopharyngeal mass. A case report. Ann Otol Rhinol Laryngol 1980;89:271‑5. Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH.
Chordoma: The nonsarcoma primary bone tumor. Oncologist 2007;12:1344‑50. 5. Mirra JM, Della Rocca C, Nelson SD, Mertens F. Chordoma. In: Fletcher CD, Unni K, Mertens F, editors. Pathology and genetics of tumours of soft tissue and bone. Lyon, France: IARC Press; 2002. p. 316‑7. 6. Yan ZY, Yang BT, Wang ZC, Xian JF, Li M. Primary chordoma in the nasal cavity and nasopharynx: CT and MR imaging findings. AJNR Am J Neuroradiol 2010;31:246‑50. 7. Cheng TB. Nasopharyngeal Chordoma. Singapore Med J 1963;3:168‑70. 8. Ferraresi V, Nuzzo C, Zoccali C, Marandino F, Vidiri A, Salducca N, et al. Chordoma: Clinical characteristics, management and prognosis of a case series of 25 patients. BMC Cancer 2010;10:22. 9. Baratti D, Gronchi A, Pennacchioli E, Lozza L, Colecchia M, Fiore M, et al. Chordoma: Natural history and results in 28 patients treated at a single institution. Ann Surg Oncol 2003;10:291‑6. 10. Fleming GF, Heimann PS, Stephens JK, Simon MA, Ferguson MK, Benjamin RS, et al. Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer 1993;72:714‑8. How to cite this article: Kataria SP, Batra A, Singh G, Kumar S, Sen R. Chordoma of skull base presenting as nasopharyngeal mass. J Neurosci Rural Pract 2013, 4(Suppl 1):s95-7. Source of Support: Nil. Conflict of Interest: None declared.
Commentary Nasopharynx is the uppermost portion of the pharynx. It is part of upper respiratory tract serving as a conduit between the nasal cavity and the oropharynx. It houses few important structures namely from lateral, the openings of the Eustachian tube (ET), torus tubarius, Fossa of Rosenmuller (FOR), and the adenoid (nasopharyngeal tonsil) in the midline. As the location of the region is posterior to the nasal cavity, examination of the nose by anterior rhinoscopy for a suspected lesion in the nasopharynx often reveals a negative finding. The use of post‑nasal space mirror in posterior rhinoscopy is considered suboptimal in these days to evaluate the nasopharyngeal region. The subtle changes of the mucosal lesion may be easily overlooked. Thus, a nasoendoscopy using a zero degree rigid endoscope or a flexible nasopharyngolaryngoscope should always be performed. In the index case, owing to the big size, the mass was detected from the oral cavity inspection, as a bulged on the soft palate.[1] If the mass was smaller at presentation, it would have been easily missed. On the contrary, the most common pathology in the nasopharynx is the nasopharyngeal carcinoma (NPC), which is almost always originates from the FOR. The special feature of NPC is that it produces classical clinical features before it can attain the size as big as the visualized chordoma in the index case. The Journal of Neurosciences in Rural Practice | 2013 | Vol 4 | Supplement 1
commonest presentation of an NPC is the cervical lymphadenopathy. It constituted more than 80% of clinical signs at presentation of NPC patients in our center.[2] This figure is consistent with reported findings in other reports as well. Besides that, unilateral conductive hearing loss (CHL) can be the sole presenting feature, owing to the primary site, the FOR which is located just lateral to the ET opening. The growing mass from the FOR can easily impinge or occlude, leading to the impairment of the ET function. Pressure different or collection of effusion in the middle ear leads to CHL. In this part of the world (Asia particularly South East Asia) where the NPC is endemic, an adult patient with unilateral CHL should be suspected NPC until proven otherwise. In our local series, more than 50% of NPC patients were having otitis media effusion, mostly unilateral.[2] Other rare presentations include abducens nerve palsy causing diplopia, obstructive symptoms leading to snoring, hyponasality, and sleep apnea. Traumatized surface of the mass or exophytic lesion of NPC may produce epistaxis. In the absence of the mass in FOR but with strong clinical suspicious of NPC, the radiological imaging such as computed tomography scan or magnetic resonance (MR) imaging is valuable to pick up the sub‑mucosal type of NPC.[3] In areas of NPC endemics, for example China, the use of MR imaging as a screening S97
Copyright of Journal of Neurosciences in Rural Practice is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
