- Email: [email protected]
Bronchogenic carcinoid metastasis to the intramedullary spinal cord
1196
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1196–1198
Bronchogenic carcinoid metastasis to the intramedullary spinal cord Dianna Li a,*, Jeffrey W. Brennan a, Michael Buckland b, Jonathon F. Parkinson a a b
Department of Neurosurgery, Royal Prince Alfred Hospital, Missenden Road, Camperdown, New South Wales 2050, Australia Department of Anatomical Pathology, University of Sydney, Camperdown, New South Wales, Australia
a r t i c l e
i n f o
Article history: Received 15 December 2009 Accepted 17 December 2009
Keywords: Carcinoid Intramedullary Metastasis Palliation Spine
a b s t r a c t Carcinoid tumours are malignant tumours of neuroendocrine origin. Spinal-cord compression from carcinoid metastasis is uncommon, and intradural spinal carcinoid is rare. We report an instance of intramedullary carcinoid metastasis of the conus medullaris in the context of stable, asymptomatic systemic disease. To our knowledge this is the first reported case of intramedullary carcinoid metastasis. Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved.
1. Case report
2. Discussion
A 33-year-old woman presented with a short history of lower back pain followed by acute incomplete paraplegia at the level of the conus medullaris. She was known to have a bronchogenic neuroendocrine tumour of 7 cm diameter, which had been diagnosed 4 years previously and was being monitored with stable asymptomatic systemic disease including multiple brain metastases (Fig. 1). She had previously received radiotherapy to the chest lesion, whole brain radiotherapy and several courses of etoposide and topotecan chemotherapy. Physical examination demonstrated flaccid arreflexia of both lower limbs, as well as a loss of anti-gravity hip flexion and knee extension with relatively preserved strength distally. There was saddle anaesthesia, urinary retention and a loss of sensation to light touch and pinprick bilaterally up to and including the L3 dermatome. There were no symptoms or signs in the upper limbs. A MRI scan demonstrated a mildly enhancing haemorrhagic intramedullary lesion extending from T11/12 to L1 (Fig. 2A). An additional cervical intramedullary lesion was found, extending from C2/3 to C5/6 (Fig. 2B). Urgent decompression of the conus tumour was performed. A T11–L1 laminectomy and midline myelotomy revealed a partly haemorrhagic tumour poorly delineated from normal spinal cord. An internal subtotal resection was performed. The histological examination revealed a moderately cellular tumour composed of sheets of plump spindled cells with focal loose rosette formations. There were up to four mitotic figures per 2 mm2, but no tumour necrosis. Immunohistochemical tests revealed positive staining for synaptophysin, chromogranin and pan-cytokeratin. The Ki-67 labelling index was 10%. These features were considered most consistent with an atypical carcinoid tumour (Fig. 3A). The patient’s lower-back pain resolved immediately after surgery, and her lower-limb strength began to improve within a few days. She had further treatment with 30 Gy of adjuvant radiotherapy, targeting both the conus and asymptomatic cervical lesion. At the time of discharge to rehabilitation, the patient was ambulating with two sticks, but had not regained bladder or sensory function.
Bronchopulmonary carcinoid tumours are malignant neuroendocrine neoplasms arising from enterochromaffin cells distributed throughout the lung mucosae.1 They are the most common type of primary carcinoid to affect the Caucasian population,2 and represent 25% to 30% of all carcinoid tumours.3,4 The World Health Organisation classifies these tumours into typical and atypical groups based on their histopathology and clinical course.5 Atypical carcinoids are more aggressive and likely to metastasise than their typical counterparts, with an estimated 5-year survival rate of 50% to 75%.6–8 Female gender, primary tumour size of 3.5 cm or greater, and higher mitotic rate are negative predictors of atypical carcinoid survival, and the presence of rosettes is an independent positive predictor.9 Carcinoid metastasis to the spine is not common. Metastasis causes spinal cord compression by growing from bony vertebrae,10–13 encroaching the epidural space14 or occurring intradurally.15,16 In this instance, acute haemorrhage may have precipitated the patient’s sudden onset of paraparesis. A report of spinal teratoma with an associated carcinoid nodule was the only example of intramedullary carcinoidosis found in the literature.17 When feasible, total surgical resection is the preferred treatment for local disease, and may result in cure. Radiotherapy is reserved for unresectable or partially resected surgical tumours.6,18 Our example is noteworthy both for the relative stability of the patient’s widespread tumour load, and the acute presentation with rare intramedullary spinal carcinoid disease (Fig. 3B). Her improvement from bed-bound paraparesis to self-ambulation within 2 weeks supports an aggressive stance in the palliative treatment of her cord lesion.
* Corresponding author. Tel.: +61 2 9515 6111; fax: +61 2 9515 6133. E-mail address: [email protected] (D. Li).
References 1. Gould PM, Bonner JA, Sawyer TE, et al. Bronchial carcinoid tumors: importance of prognostic factors that influence patterns of recurrence and overall survival. Radiology 1998;208:181–5. 2. Yao JC, Hassan M, Phan A, et al. One hundred years after ‘‘carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26:3063–72. 3. Rea F, Rizzardi G, Zuin A, et al. Outcome and surgical strategy in bronchial carcinoid tumors: single institution experience with 252 patients. Eur J Cardiothorac Surg 2007;31:186–91. 4. Pinchot SN, Holen K, Sippel RS, et al. Carcinoid tumors. Oncologist 2008;13:1255–69. 5. Travis W, Colby T, Corrin B, et al. Histological typing of lung and pleural tumors. WHO International histological classification of tumor. Berlin: Springer; 1999.
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1196–1198
1197
Fig. 1. Sagittal (left) and axial (right) T1-weighted MRI scans with gadolinium enhancement demonstrating small supratentorial lesions and larger cerebellar lesions associated with cystic change.
Fig. 2. (A) MRI scans showing an intramedullary conus lesion which is (left) isointense with the spinal cord on T1-weighted imaging, (middle) mildly enhancing and (right) associated with a superior syrinx as well as an area of hypointensity on T2-weighted imaging, most likely representing acute haemorrhage. (B) (left) T1- and (right) T2weighted MRI scans showing an asymptomatic cervical intramedullary lesion.
Fig. 3. (A) Histological samples of the tumour showing (left) plump spindled cells with a rosette formation (arrow; haematoxylin and eosin, magnification 200) and (right) Ki-67 proliferation labelling (magnification 200). (B) Positron emission tomography (PET) scans (left) 8 months prior to admission and (right) during admission showing new spinal lesions, indicating a rapid progression of spinal disease despite stable systemic disease. (This figure is available in colour at www.sciencedirect.com.)
1198
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1198–1201
6. Gustafsson BI, Kidd M, Chan A, et al. Bronchopulmonary neuroendocrine tumors. Cancer 2008;113:5–21. 7. Mezzetti M, Raveglia F, Panigalli T, et al. Assessment of outcomes in typical and atypical carcinoids according to latest WHO classification. Ann Thorac Surg 2003;76:1838–42. 8. Fink G, Krelbaum T, Yellin A, et al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest 2001;119:1647–51. 9. Beasley MB, Thunnissen FB, Brambilla E, et al. Pulmonary atypical carcinoid: predictors of survival in 106 cases. Hum Pathol 2000;31:1255–65. 10. Patchell RA, Posner JB. Neurologic complications of carcinoid. Neurology 1986;36:745–9. 11. Poole CJ. Myelopathy secondary to metastatic carcinoid tumour. J Neurol Neurosurg Psychiatry 1984;47:1359–60. 12. Gowitt GT, Mirra SS. Malignant carcinoid causing spinal cord compression. Neurosurgery 1985;17:801–6.
13. Gray JA, Nishikawa H, Jamous MA, et al. Spinal cord compression due to carcinoid metastasis. Postgrad Med J 1988;64:703–5. 14. Kirkpatrick D, Dawson E, Haskell C, et al. Metastatic carcinoid presenting as a spinal tumour. Surg Neurol 1975;4:283–7. 15. Blondet E, Dulou R, Camparo P, et al. Lumbar intradural metastasis of a primary carcinoid tumor of the lung. Case illustration. J Neurosurg Spine 2005;2: 231. 16. Rao KC, Jhaveri HS, Gellad FE. Carcinoid tumor with intradural spinal metastases. J Comput Tomogr 1988;12:258–60. 17. Fan X, Turner JE, Turner TM, et al. Carcinoid tumor development in an intramedullary spinal cord mature teratoma. AJNR Am J Neuroradiol 2001;22:1778–81. 18. Chakravarthy A, Abrams RA. Radiation therapy in the management of patients with malignant carcinoid tumors. Cancer 1995;75:1386–90.
doi:10.1016/j.jocn.2009.12.021
Reversible sensorineural hearing loss with normal brainstem auditory evoked potentials in pontine hemorrhage due to capillary telangiectasia Munish Kumar Goyal, Gyanendra Kumar *, Pradeep Kumar Sahota Department of Neurology, University of Missouri School of Medicine, CE507, 5 Hospital Drive, Columbia, Missouri 65212, USA
a r t i c l e
i n f o
Article history: Received 21 September 2009 Accepted 17 January 2010
Keywords: Audiometry Brainstem auditory evoked potentials Capillary telangiectasia MRI Pontine hemorrhage Sensorineural hearing loss
a b s t r a c t Capillary telangiectasias are vascular malformations most commonly found in the pons that are rarely associated with hemorrhage. We describe a unique case of pontine capillary telangiectasia causing central brainstem hemorrhage leading to reversible sensorineural deafness associated with a normal brainstem auditory evoked response. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case description
Capillary telangiectasis are vascular malformations of unknown origin and clinical significance1,2 which first appeared in the anatomical and pathological literature in 1959.3 In vivo diagnosis has only been possible since the advent of MRI, because these lesions are so small that they are undetectable by either angiography or computed tomography.4 Capillary telangiectasias account for 16%–20% of all intracerebral vascular malformations at autopsy and are being increasingly recognized by imaging,5 found most commonly, but not exclusively, in the pons.6 Their estimated prevalence at autopsy is 0.4%.7 Most are incidental findings growing very slowly or not at all,8 rarely presenting with mild symptoms such as headache, vertigo, or mild facial weakness. Hemorrhage seen in association with capillary telangiectasia may arise from an associated vascular malformation and rarely from the capillary telangiectasia.9,10 We report an unusual case of central pontine hemorrhage due to capillary telangiectasia that led to sensorineural hearing loss which resolved in 6 months in the absence of an abnormality of brainstem auditory evoked potentials (BAEP).
A 51-year-old Caucasian female developed sudden onset deafness of left ear with weakness and numbness of her right arm and leg, slurring of speech and diplopia. Neurological examination revealed bilateral horizontal gaze evoked nystagmus, left sixth nerve palsy, and bilateral sensorineural hearing loss more pronounced on the left side. Right-sided hypertonic hemiparesis (Medical Research Council [MRC] grade 4/5), hemisensory loss, dysmetria and truncal ataxia were present.
* Corresponding author. Tel.: +1 573 882 4141; fax: +1 573 884 4249. E-mail address: [email protected] (G. Kumar).
2.1. Radiology Noncontrast head CT at presentation showed a central pontine hyperdensity consistent with blood (Fig. 1A). Brain MRI at presentation is shown in Fig. 1(B–D). MR angiography was normal. In retrospect, our patient’s pre-hemorrhage MRI done one year prior showed faint enhancement on T1-weighted contrast enhanced sequences and (Fig. 2A) a subtle hyperintensity on FLAIR images (Fig. 2B). The lesion on follow up MRI scan at 6 months was hypointense on T2-weighted, isointense and and nonenhancing on T1weighted contrast enhanced images, suggesting hemosiderin deposition and resolution of hemorrhage (Fig. 3iiA–C).
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1196–1198
Bronchogenic carcinoid metastasis to the intramedullary spinal cord Dianna Li a,*, Jeffrey W. Brennan a, Michael Buckland b, Jonathon F. Parkinson a a b
Department of Neurosurgery, Royal Prince Alfred Hospital, Missenden Road, Camperdown, New South Wales 2050, Australia Department of Anatomical Pathology, University of Sydney, Camperdown, New South Wales, Australia
a r t i c l e
i n f o
Article history: Received 15 December 2009 Accepted 17 December 2009
Keywords: Carcinoid Intramedullary Metastasis Palliation Spine
a b s t r a c t Carcinoid tumours are malignant tumours of neuroendocrine origin. Spinal-cord compression from carcinoid metastasis is uncommon, and intradural spinal carcinoid is rare. We report an instance of intramedullary carcinoid metastasis of the conus medullaris in the context of stable, asymptomatic systemic disease. To our knowledge this is the first reported case of intramedullary carcinoid metastasis. Crown Copyright Ó 2010 Published by Elsevier Ltd. All rights reserved.
1. Case report
2. Discussion
A 33-year-old woman presented with a short history of lower back pain followed by acute incomplete paraplegia at the level of the conus medullaris. She was known to have a bronchogenic neuroendocrine tumour of 7 cm diameter, which had been diagnosed 4 years previously and was being monitored with stable asymptomatic systemic disease including multiple brain metastases (Fig. 1). She had previously received radiotherapy to the chest lesion, whole brain radiotherapy and several courses of etoposide and topotecan chemotherapy. Physical examination demonstrated flaccid arreflexia of both lower limbs, as well as a loss of anti-gravity hip flexion and knee extension with relatively preserved strength distally. There was saddle anaesthesia, urinary retention and a loss of sensation to light touch and pinprick bilaterally up to and including the L3 dermatome. There were no symptoms or signs in the upper limbs. A MRI scan demonstrated a mildly enhancing haemorrhagic intramedullary lesion extending from T11/12 to L1 (Fig. 2A). An additional cervical intramedullary lesion was found, extending from C2/3 to C5/6 (Fig. 2B). Urgent decompression of the conus tumour was performed. A T11–L1 laminectomy and midline myelotomy revealed a partly haemorrhagic tumour poorly delineated from normal spinal cord. An internal subtotal resection was performed. The histological examination revealed a moderately cellular tumour composed of sheets of plump spindled cells with focal loose rosette formations. There were up to four mitotic figures per 2 mm2, but no tumour necrosis. Immunohistochemical tests revealed positive staining for synaptophysin, chromogranin and pan-cytokeratin. The Ki-67 labelling index was 10%. These features were considered most consistent with an atypical carcinoid tumour (Fig. 3A). The patient’s lower-back pain resolved immediately after surgery, and her lower-limb strength began to improve within a few days. She had further treatment with 30 Gy of adjuvant radiotherapy, targeting both the conus and asymptomatic cervical lesion. At the time of discharge to rehabilitation, the patient was ambulating with two sticks, but had not regained bladder or sensory function.
Bronchopulmonary carcinoid tumours are malignant neuroendocrine neoplasms arising from enterochromaffin cells distributed throughout the lung mucosae.1 They are the most common type of primary carcinoid to affect the Caucasian population,2 and represent 25% to 30% of all carcinoid tumours.3,4 The World Health Organisation classifies these tumours into typical and atypical groups based on their histopathology and clinical course.5 Atypical carcinoids are more aggressive and likely to metastasise than their typical counterparts, with an estimated 5-year survival rate of 50% to 75%.6–8 Female gender, primary tumour size of 3.5 cm or greater, and higher mitotic rate are negative predictors of atypical carcinoid survival, and the presence of rosettes is an independent positive predictor.9 Carcinoid metastasis to the spine is not common. Metastasis causes spinal cord compression by growing from bony vertebrae,10–13 encroaching the epidural space14 or occurring intradurally.15,16 In this instance, acute haemorrhage may have precipitated the patient’s sudden onset of paraparesis. A report of spinal teratoma with an associated carcinoid nodule was the only example of intramedullary carcinoidosis found in the literature.17 When feasible, total surgical resection is the preferred treatment for local disease, and may result in cure. Radiotherapy is reserved for unresectable or partially resected surgical tumours.6,18 Our example is noteworthy both for the relative stability of the patient’s widespread tumour load, and the acute presentation with rare intramedullary spinal carcinoid disease (Fig. 3B). Her improvement from bed-bound paraparesis to self-ambulation within 2 weeks supports an aggressive stance in the palliative treatment of her cord lesion.
* Corresponding author. Tel.: +61 2 9515 6111; fax: +61 2 9515 6133. E-mail address: [email protected] (D. Li).
References 1. Gould PM, Bonner JA, Sawyer TE, et al. Bronchial carcinoid tumors: importance of prognostic factors that influence patterns of recurrence and overall survival. Radiology 1998;208:181–5. 2. Yao JC, Hassan M, Phan A, et al. One hundred years after ‘‘carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26:3063–72. 3. Rea F, Rizzardi G, Zuin A, et al. Outcome and surgical strategy in bronchial carcinoid tumors: single institution experience with 252 patients. Eur J Cardiothorac Surg 2007;31:186–91. 4. Pinchot SN, Holen K, Sippel RS, et al. Carcinoid tumors. Oncologist 2008;13:1255–69. 5. Travis W, Colby T, Corrin B, et al. Histological typing of lung and pleural tumors. WHO International histological classification of tumor. Berlin: Springer; 1999.
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1196–1198
1197
Fig. 1. Sagittal (left) and axial (right) T1-weighted MRI scans with gadolinium enhancement demonstrating small supratentorial lesions and larger cerebellar lesions associated with cystic change.
Fig. 2. (A) MRI scans showing an intramedullary conus lesion which is (left) isointense with the spinal cord on T1-weighted imaging, (middle) mildly enhancing and (right) associated with a superior syrinx as well as an area of hypointensity on T2-weighted imaging, most likely representing acute haemorrhage. (B) (left) T1- and (right) T2weighted MRI scans showing an asymptomatic cervical intramedullary lesion.
Fig. 3. (A) Histological samples of the tumour showing (left) plump spindled cells with a rosette formation (arrow; haematoxylin and eosin, magnification 200) and (right) Ki-67 proliferation labelling (magnification 200). (B) Positron emission tomography (PET) scans (left) 8 months prior to admission and (right) during admission showing new spinal lesions, indicating a rapid progression of spinal disease despite stable systemic disease. (This figure is available in colour at www.sciencedirect.com.)
1198
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1198–1201
6. Gustafsson BI, Kidd M, Chan A, et al. Bronchopulmonary neuroendocrine tumors. Cancer 2008;113:5–21. 7. Mezzetti M, Raveglia F, Panigalli T, et al. Assessment of outcomes in typical and atypical carcinoids according to latest WHO classification. Ann Thorac Surg 2003;76:1838–42. 8. Fink G, Krelbaum T, Yellin A, et al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest 2001;119:1647–51. 9. Beasley MB, Thunnissen FB, Brambilla E, et al. Pulmonary atypical carcinoid: predictors of survival in 106 cases. Hum Pathol 2000;31:1255–65. 10. Patchell RA, Posner JB. Neurologic complications of carcinoid. Neurology 1986;36:745–9. 11. Poole CJ. Myelopathy secondary to metastatic carcinoid tumour. J Neurol Neurosurg Psychiatry 1984;47:1359–60. 12. Gowitt GT, Mirra SS. Malignant carcinoid causing spinal cord compression. Neurosurgery 1985;17:801–6.
13. Gray JA, Nishikawa H, Jamous MA, et al. Spinal cord compression due to carcinoid metastasis. Postgrad Med J 1988;64:703–5. 14. Kirkpatrick D, Dawson E, Haskell C, et al. Metastatic carcinoid presenting as a spinal tumour. Surg Neurol 1975;4:283–7. 15. Blondet E, Dulou R, Camparo P, et al. Lumbar intradural metastasis of a primary carcinoid tumor of the lung. Case illustration. J Neurosurg Spine 2005;2: 231. 16. Rao KC, Jhaveri HS, Gellad FE. Carcinoid tumor with intradural spinal metastases. J Comput Tomogr 1988;12:258–60. 17. Fan X, Turner JE, Turner TM, et al. Carcinoid tumor development in an intramedullary spinal cord mature teratoma. AJNR Am J Neuroradiol 2001;22:1778–81. 18. Chakravarthy A, Abrams RA. Radiation therapy in the management of patients with malignant carcinoid tumors. Cancer 1995;75:1386–90.
doi:10.1016/j.jocn.2009.12.021
Reversible sensorineural hearing loss with normal brainstem auditory evoked potentials in pontine hemorrhage due to capillary telangiectasia Munish Kumar Goyal, Gyanendra Kumar *, Pradeep Kumar Sahota Department of Neurology, University of Missouri School of Medicine, CE507, 5 Hospital Drive, Columbia, Missouri 65212, USA
a r t i c l e
i n f o
Article history: Received 21 September 2009 Accepted 17 January 2010
Keywords: Audiometry Brainstem auditory evoked potentials Capillary telangiectasia MRI Pontine hemorrhage Sensorineural hearing loss
a b s t r a c t Capillary telangiectasias are vascular malformations most commonly found in the pons that are rarely associated with hemorrhage. We describe a unique case of pontine capillary telangiectasia causing central brainstem hemorrhage leading to reversible sensorineural deafness associated with a normal brainstem auditory evoked response. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case description
Capillary telangiectasis are vascular malformations of unknown origin and clinical significance1,2 which first appeared in the anatomical and pathological literature in 1959.3 In vivo diagnosis has only been possible since the advent of MRI, because these lesions are so small that they are undetectable by either angiography or computed tomography.4 Capillary telangiectasias account for 16%–20% of all intracerebral vascular malformations at autopsy and are being increasingly recognized by imaging,5 found most commonly, but not exclusively, in the pons.6 Their estimated prevalence at autopsy is 0.4%.7 Most are incidental findings growing very slowly or not at all,8 rarely presenting with mild symptoms such as headache, vertigo, or mild facial weakness. Hemorrhage seen in association with capillary telangiectasia may arise from an associated vascular malformation and rarely from the capillary telangiectasia.9,10 We report an unusual case of central pontine hemorrhage due to capillary telangiectasia that led to sensorineural hearing loss which resolved in 6 months in the absence of an abnormality of brainstem auditory evoked potentials (BAEP).
A 51-year-old Caucasian female developed sudden onset deafness of left ear with weakness and numbness of her right arm and leg, slurring of speech and diplopia. Neurological examination revealed bilateral horizontal gaze evoked nystagmus, left sixth nerve palsy, and bilateral sensorineural hearing loss more pronounced on the left side. Right-sided hypertonic hemiparesis (Medical Research Council [MRC] grade 4/5), hemisensory loss, dysmetria and truncal ataxia were present.
* Corresponding author. Tel.: +1 573 882 4141; fax: +1 573 884 4249. E-mail address: [email protected] (G. Kumar).
2.1. Radiology Noncontrast head CT at presentation showed a central pontine hyperdensity consistent with blood (Fig. 1A). Brain MRI at presentation is shown in Fig. 1(B–D). MR angiography was normal. In retrospect, our patient’s pre-hemorrhage MRI done one year prior showed faint enhancement on T1-weighted contrast enhanced sequences and (Fig. 2A) a subtle hyperintensity on FLAIR images (Fig. 2B). The lesion on follow up MRI scan at 6 months was hypointense on T2-weighted, isointense and and nonenhancing on T1weighted contrast enhanced images, suggesting hemosiderin deposition and resolution of hemorrhage (Fig. 3iiA–C).