- Email: [email protected]
Signet-ring cell carcinoma of the colon with leptomeningeal involvement
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1051–1053
Although it is difficult to exclude the possibility of coincidental association, the high frequency of multiple and irregular aneurysms in these patients may indicate that the pathogenesis of the cerebral aneurysms is related to CREST syndrome. CREST syndrome/SSc is described as an autoimmune collagen vascular disease, and vascular damage is a major component. Although vascular damage is prominent in the small- and medium-sized vessels of the digits, lungs and gastrointestinal tract, the involvement of cerebral vessels has also been reported. Multiple segmental narrowing of cerebral arteries was reported in two patients with CREST syndrome, which were considered to be caused by cerebral vasculopathy.4,5 Histological examination in autopsied patients with CREST syndrome revealed significant calcification of the walls of the small arteries in the brain, indicating that CREST syndrome may have induced primary cerebrovascular changes.6 The mechanisms of vascular damage in SSc are poorly understood. Recent studies suggest that endothelial cell injury underlies the development of vascular damage.7 Various mechanisms capable of producing endothelial cell damage have been described, including specific endothelial cell autoantibodies, inflammatory cytokines or reactive oxygen radicals.8 Similarly, endothelial cell injury has been found to play a primary role in the pathogenesis of ordinary cerebral aneurysms. Chronic hemodynamic stress initially affects the endothelium, and endothelial erosion triggers macrophage infiltration into the vessel wall. Progression of the inflammatory reaction leads to proteolytic destruction of the vessel wall, resulting in aneurysm formation.9 Thus, similar early pathological changes may take place in the development of SSc vasculopathy and cerebral aneurysm. Moreover, a recent study reported a higher prevalence of cerebral aneurysms in SSc patients than in the general population.10 SSc is a heterogeneous disorder in terms of disease symptoms and clinical course. Many autoantibodies have been identified in the sera of SSc patients and specific autoantibody profiles associate strongly with distinct clinical phenotypes.11 Several recent reports
1051
have suggested that the presence of anti-centromere antibodies correlates with severe vascular damage, including ulcers and gangrene, and that anti-centromere antibodies may be directly toxic to endothelial cells.12 However, it is not known whether patients with CREST syndrome have a significantly increased risk of cerebral aneurysm. The true incidence of cerebral aneurysms associated with CREST syndrome should be elucidated to clarify the relationship between autoantibodies and aneurysm formation.
References 1. Wollheim FA. Classification of systemic sclerosis. Visions and reality. Rheumatology 2005;44:1212–6. 2. Ortiz JR, Newman NJ, Barrow DL. CREST-associated multiple intracranial aneurysms and bilateral optic neuropathies. J Clin Neuroophthalmol 1991;11:233–40. 3. Zoumalan RA, Bendok BR, Parkinson RJ, et al. Association of an irregularly shaped anterior choroidal aneurysm with CREST syndrome. Case report. J Neurosurg 2004;101:854–7. 4. Pathak R, Gabor AJ. Scleroderma and central nervous system vasculitis. Stroke 1991;22:410–3. 5. Terajima K, Shimohata T, Watanabe M, et al. Cerebral vasculopathy showing moyamoya-like changes in a patient with CREST syndrome. Eur Neurol 2001;46:163–5. 6. Heron E, Fornes P, Rance A, et al. Brain involvement in scleroderma: two autopsy cases. Stroke 1998;29:719–21. 7. Abraham D, Distler O. How does endothelial cell injury start? The role of endothelin in systemic sclerosis. Arthritis Res Ther 2007;9(Suppl. 2):S2. 8. Kahaleh B. The microvascular endothelium in scleroderma. Rheumatology 2008;47(Suppl. 5):14–5. 9. Jamous MA, Nagahiro S, Kitazato KT, et al. Endothelial injury and inflammatory response induced by hemodynamic changes preceding intracranial aneurysm formation: experimental study in rats. J Neurosurg 2007;107:405–11. 10. Kaku Y, Kouda K, Yoshimura S, et al. Cerebral aneurysms in scleroderma. Cerebrovasc Dis 2004;17:339–41. 11. Gabrielli A, Svegliati S, Moroncini G, et al. Pathogenic autoantibodies in systemic sclerosis. Curr Opin Immunol 2007;19:640–5. 12. Takahashi M, Okada J, Kondo H. Six cases positive for anti-centromere antibodies with ulcer and gangrene in the extremities. Br J Rheumatol 1997;36:889–93.
doi:10.1016/j.jocn.2009.12.008
Signet-ring cell carcinoma of the colon with leptomeningeal involvement Yesim Yildirim a,*, Zafer Akcali b, Ozgur Ozyilkan b a b
Acibadem Kozyatagi Hospital Medical Oncology Department, Istanbul, Turkey Baskent University Department of Medical Oncology, Ankara, Turkey
a r t i c l e
i n f o
Article history: Received 19 August 2009 Accepted 6 October 2009
a b s t r a c t Primary signet-ring cell carcinoma of the colon is rare. Most patients with this type of cancer have a poor prognosis. We describe a patient with signet-ring cell carcinoma of the colon, for whom leptomeningeal metastasis presented a variety of clinical symptoms. Ó 2009 Elsevier Ltd. All rights reserved.
Keywords: Signet-ring carcinoma Colon Leptomeningeal metastasis
* Corresponding author. Address: Acibadem Kozyatagi Hospital Medical Oncology Department, Inonu Caddesi Okur Sokak No: 20, Istanbul 34742, Turkey. Tel.: +90 532 571 56 07; fax: +90 216 571 40 00. E-mail address: [email protected] (Y. Yildirim).
1. Introduction Most colorectal carcinomas (CRC) are adenocarcinomas, although several other histological subtypes, including signet-ring
1052
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1051–1053
cell carcinoma (SRCC) also occur. The incidence of SRCC of the colon is 0.6/100 000.1 The most common metastatic sites for CRC are the liver and lung; metastasis to other sites is rare. However, rather than hepatic metastasis, SRCC shows a high incidence of peritoneal metastasis.1 The incidence of leptomeningeal involvement (LMI) in patients with CRC has not been established, but in a cohort study the cumulative incidence of cranial metastasis in patients with CRC was calculated as 1.2%.2 We describe a patient with a rare pathologic subtype of colon cancer and an equally rare metastatic presentation. 2. Case report A 58-year-old man with a history of vomiting and abdominal pain was admitted to the emergency department. Abdominal ultrasound examination showed a thickening in the splenic flexure. Colonoscopic biopsy showed SRCC characterized by abundant intracytoplasmic mucin, pushing the nuclei to the periphery of the cell, characteristic for SRCC. The patient’s preoperative carcinoembryonic antigen (CEA) level was 22 ng/mL (normal range 0– 4 ng/mL). A left-sided hemicolectomy and lymph node dissection were performed. Microscopic examination showed the tumor consisted of more than 50% signet-ring cells. Tumor cells were arranged in loose clusters and spread diffusely through the bowel wall. Metastatic involvement in 10 of 13 lymph nodes was identified. The patient completed a 6-cycle regimen of the adjuvant 5-fluorouracil and folinic acid. An increase in the patient’s CEA level of 6 ng/mL was noted 18 months after the last cycle of chemotherapy. At that time, colonoscopy showed a polypoid lesion 30 cm from the anal canal. A biopsy again revealed SRCC. Subtotal colectomy, appendectomy and ileoproctostomy were performed. The tumor was found in the muscular layer and in the appendectomy specimen lymph nodes. After the second operation CEA levels returned to normal. The patient received the same regimen of adjuvant therapy.
One month after the last cycle of chemotherapy, the patient’s CEA level increased to 6.0 ng/mL. To avoid a possible recurrence, the patient was evaluated with a colonoscopy and an abdominal CT scan. No abnormalities were found. One month later, owing to a rise in the CEA level, the patient was evaluated with positron emission tomography (PET) but nothing was found. Three months later the patient was referred to the otorhinolaryngology and neurology departments because of headaches and a decrease in vision and hearing. An internal acoustic MRI showed bilateral metastasis of cranial nerves VII and VIII (Fig. 1). At that time the CEA level was 13.1 ng/mL. Lumbar puncture revealed malignant cells in the cerebrospinal fluid (CSF). Craniospinal radiotherapy was commenced. However, the patient’s condition deteriorated gradually and he died 2 months after radiotherapy.
3. Discussion Most SRCCs develop in the stomach; the remainder develop in other primary organs including the colon, rectum, pancreas, urinary bladder and breast. Primary SRCC carcinoma of the colon and rectum forms nearly 1% of all adenocarcinomas of the large intestine.1 These carcinomas show more malignant biological behaviour than typical CRC.3 SRCC can be recognized as a stageindependent prognostic factor for an adverse outcome. CRCs can spread locally or distantly via the lymphatic and venous system. The most frequent sites of metastasis from CRC are the liver, lung, peritoneum, bone, ovaries, and adrenal glands.4 However, a low incidence of hepatic metastasis and high incidence of peritoneal spread are the distinguishing features of SRCC. Cranial metastases are rare and a sign of terminal disease. LMI occurs in 3% to 8% of all cancer patients; however, it is usually seen in patients with leukemia, breast cancer, lymphoma, or lung cancer.5 Central nervous system (CNS) metastasis can occur via communication between the portal system and paravertebral veins. When a tumor spreads to the leptomeninges it gains access to the entire CNS. Patients usually present with a myriad of symptoms, including altered mental status, spinal sensory changes, lower extremity weakness, headache, nausea and vomiting. On neurological examination, cranial nerve palsy and lower extremity weakness are common. Due to the infrequency of LMI in CRC, and the fact that similar symptoms can be caused by chemotherapy itself, it is not easy to attribute all these signs and symptoms to a particular clinical syndrome. Gadoliniumenhanced MRI could be helpful in two-thirds of patients. Enhancement of the dura and nodular lesions on the MRI can indicate LMI, which is usually not detected by an enhanced CT scan of the brain. Additionally, CSF examination may show malignant cells. Craniospinal radiotherapy may improve some of the symptoms but LMI generally results in death.
4. Conclusion SRCC carcinomas of the colon and LMI in CRC are both rare. Each has been presented in several case reports; however, until now, a patient with SRCC of the colon with LMI has not been reported. In this patient, a continuous CEA rise suggested metastasis although a PET scan was negative. Gadolinium-enhanced MRI may aid the diagnosis of LMI.
References Fig. 1. An coronal internal acoustic T1-weighted contrast-enhanced MRI showing bilateral metastasis to cranial nerves VII and VIII.
1. Kang H, O’Connell JB, Maggard MA, et al. A 10-year outcomes evaluation of mucinous and signet-ring cell carcinoma of the colon and rectum. Dis Colon Rectum 2005;48:1161–8.
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1053–1056 2. Schouten LJ, Rutten J, Huveneers HA, et al. Incidence of brain metastases in a cohort of patients with carcinoma of the breast, colon, kidney and lung and melanoma. Cancer 2002;94:2698–705. 3. Shimaoka S, Niihara T, Tashiro K, et al. Signet-ring cell carcinoma of the colon 7 mm in size with peritonitis carcinomatosa. J Gastroenterol 2002;37:550–5.
1053
4. August DA, Ottow RT, Sugarbaker PH. Clinical perspective of human colorectal cancer metastasis. Cancer Metastasis Rev 1984;3:303–42. 5. Grossman SA, Krabak MJ. Leptomeningeal carcinomatosis. Cancer Treat Rev 1999;25:103–19.
doi:10.1016/j.jocn.2009.10.014
Intraoperative monitoring during surgery for hypoglossal schwannoma Mami Ishikawa *, Gen Kusaka, Kouichi Takashima, Haruna Kamochi, Soji Shinoda Department of Neurosurgery, Saitama Medical Center, Jichi Medical University, 847 Amanumacho, Omiyaku, Saitama-shi, 330-8503 Saitama-ken, Japan
a r t i c l e
i n f o
Article history: Received 12 June 2009 Accepted 14 September 2009
Keywords: Electromyography Evoked potential Hypoglossal nerve Operative monitoring Schwannoma
a b s t r a c t A 54-year-old man presented with an intracranial schwannoma of the hypoglossal nerve between the medulla and the left hypoglossal canal. The condylar fossa approach was used with intra-operative electromyography (EMG) monitoring of the lower cranial nerves. The tumor was then removed carefully without decreasing the tongue EMG responses. EMG monitoring enabled us to remove the tumor while maintaining the function of the hypoglossal nerve. Tongue EMG was easily recorded by stimulating the hypoglossal nerve fibers, which was useful in identifying the hypoglossal nerve and evaluating its function. This suggests that tongue EMG is a useful monitoring tool to enhance neurological outcome following removal of tumors in this region. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
2.1. Operative procedure
Schwannoma of the hypoglossal nerve is rare, as reported by Piccirilli et al., whose review found only 105 patients with hypoglossal schwannoma prior to 2007.1 We often operate on intracranial schwannomas, most frequently vestibular schwannomas, and use electrophysiological monitoring to avoid facial nerve palsy. Intraoperative electromyographic (EMG) monitoring of the lower cranial nerves (CN: IX, X, XI, XII) is also useful for their identification and localization.2–4 We present a patient with an intracranial hypoglossal schwannoma. This schwannoma was removed with monitoring of the lower cranial nerves using EMG responses elicited by the stimulation of the hypoglossal nerve and the vagus nerve, to minimize additional neurological deficits.
Informed consent was obtained from the patient before the operation. General anesthesia was induced with succinyl choline and propofol, which was maintained with fentanyl and propofol. The patient was connected to the equipment (Neuropack, Nihon Kohden, Tokyo, Japan) for EMG monitoring of the lower cranial nerves and short latency somatosensory evoked potentials (SSEP) monitoring. The condylar fossa approach5–8 was performed following a left median hockey-stick incision. Incision of the dura mater exposed the tumor, over which the vagal nerve and the accessory nerve were slightly stretched (Fig. 2A). However, these nerves did not adhere to the tumor and were easily moved, along with the arachnoid, to the side of the medulla. These nerves were identified as the vagal nerve, the accessory nerve and the hypoglossal nerve by the EMG response from the vocal cords (Fig. 3A), movement of the patient’s shoulder and the EMG response from the tongue (Fig. 3B), respectively. Stimulation of some of the roots of the hypoglossal nerve fibers elicited EMG responses from the tongue whereas stimulation of other roots did not elicit EMG responses. The tumor was removed carefully without causing EMG response changes from the tongue (Figs. 2B, 3C). Parts of the tumor connected to hypoglossal nerve fibers that did not elicit EMG responses from the tongue following stimulation were removed. The hypoglossal nerve fiber roots associated with EMG responses were not seen to merge with the tumor. The hypoglossal nerve arises from 3–15 roots to form the rostral and caudal trunks.9,10 In our patient, the tumor arose from the caudal trunk, which was also reported in Kikkawa’s study.10 Although radiologically the tumor appeared to have been removed totally (Figs. 1C, 2C), a small amount of tumor that adhered to hypoglossal nerve fibers, and which elicited EMG responses from the tongue when stimulated, could not be removed. There was no decrease in SSEP during the operation.
2. Case report A 54-year-old man with a 1-year history of hoarseness and weakness of the left side of the tongue is presented. He noticed hemiatrophy of the left side of the tongue in November 2007 and was admitted to our hospital on April 2008. CT scans showed slight enlargement of the left hypoglossal canal (Fig. 1A). Gadoliniumenhanced T1-weighted MRI (Fig. 1B) revealed a faintly enhanced lesion between the medulla and the left hypoglossal canal, extending into the canal with a cystic component. Angiograms showed no tumor stain or feeder.
* Corresponding author. Tel.: +81 48 6472111; fax: +81 48 6485180. E-mail address: [email protected] (M. Ishikawa).
Although it is difficult to exclude the possibility of coincidental association, the high frequency of multiple and irregular aneurysms in these patients may indicate that the pathogenesis of the cerebral aneurysms is related to CREST syndrome. CREST syndrome/SSc is described as an autoimmune collagen vascular disease, and vascular damage is a major component. Although vascular damage is prominent in the small- and medium-sized vessels of the digits, lungs and gastrointestinal tract, the involvement of cerebral vessels has also been reported. Multiple segmental narrowing of cerebral arteries was reported in two patients with CREST syndrome, which were considered to be caused by cerebral vasculopathy.4,5 Histological examination in autopsied patients with CREST syndrome revealed significant calcification of the walls of the small arteries in the brain, indicating that CREST syndrome may have induced primary cerebrovascular changes.6 The mechanisms of vascular damage in SSc are poorly understood. Recent studies suggest that endothelial cell injury underlies the development of vascular damage.7 Various mechanisms capable of producing endothelial cell damage have been described, including specific endothelial cell autoantibodies, inflammatory cytokines or reactive oxygen radicals.8 Similarly, endothelial cell injury has been found to play a primary role in the pathogenesis of ordinary cerebral aneurysms. Chronic hemodynamic stress initially affects the endothelium, and endothelial erosion triggers macrophage infiltration into the vessel wall. Progression of the inflammatory reaction leads to proteolytic destruction of the vessel wall, resulting in aneurysm formation.9 Thus, similar early pathological changes may take place in the development of SSc vasculopathy and cerebral aneurysm. Moreover, a recent study reported a higher prevalence of cerebral aneurysms in SSc patients than in the general population.10 SSc is a heterogeneous disorder in terms of disease symptoms and clinical course. Many autoantibodies have been identified in the sera of SSc patients and specific autoantibody profiles associate strongly with distinct clinical phenotypes.11 Several recent reports
1051
have suggested that the presence of anti-centromere antibodies correlates with severe vascular damage, including ulcers and gangrene, and that anti-centromere antibodies may be directly toxic to endothelial cells.12 However, it is not known whether patients with CREST syndrome have a significantly increased risk of cerebral aneurysm. The true incidence of cerebral aneurysms associated with CREST syndrome should be elucidated to clarify the relationship between autoantibodies and aneurysm formation.
References 1. Wollheim FA. Classification of systemic sclerosis. Visions and reality. Rheumatology 2005;44:1212–6. 2. Ortiz JR, Newman NJ, Barrow DL. CREST-associated multiple intracranial aneurysms and bilateral optic neuropathies. J Clin Neuroophthalmol 1991;11:233–40. 3. Zoumalan RA, Bendok BR, Parkinson RJ, et al. Association of an irregularly shaped anterior choroidal aneurysm with CREST syndrome. Case report. J Neurosurg 2004;101:854–7. 4. Pathak R, Gabor AJ. Scleroderma and central nervous system vasculitis. Stroke 1991;22:410–3. 5. Terajima K, Shimohata T, Watanabe M, et al. Cerebral vasculopathy showing moyamoya-like changes in a patient with CREST syndrome. Eur Neurol 2001;46:163–5. 6. Heron E, Fornes P, Rance A, et al. Brain involvement in scleroderma: two autopsy cases. Stroke 1998;29:719–21. 7. Abraham D, Distler O. How does endothelial cell injury start? The role of endothelin in systemic sclerosis. Arthritis Res Ther 2007;9(Suppl. 2):S2. 8. Kahaleh B. The microvascular endothelium in scleroderma. Rheumatology 2008;47(Suppl. 5):14–5. 9. Jamous MA, Nagahiro S, Kitazato KT, et al. Endothelial injury and inflammatory response induced by hemodynamic changes preceding intracranial aneurysm formation: experimental study in rats. J Neurosurg 2007;107:405–11. 10. Kaku Y, Kouda K, Yoshimura S, et al. Cerebral aneurysms in scleroderma. Cerebrovasc Dis 2004;17:339–41. 11. Gabrielli A, Svegliati S, Moroncini G, et al. Pathogenic autoantibodies in systemic sclerosis. Curr Opin Immunol 2007;19:640–5. 12. Takahashi M, Okada J, Kondo H. Six cases positive for anti-centromere antibodies with ulcer and gangrene in the extremities. Br J Rheumatol 1997;36:889–93.
doi:10.1016/j.jocn.2009.12.008
Signet-ring cell carcinoma of the colon with leptomeningeal involvement Yesim Yildirim a,*, Zafer Akcali b, Ozgur Ozyilkan b a b
Acibadem Kozyatagi Hospital Medical Oncology Department, Istanbul, Turkey Baskent University Department of Medical Oncology, Ankara, Turkey
a r t i c l e
i n f o
Article history: Received 19 August 2009 Accepted 6 October 2009
a b s t r a c t Primary signet-ring cell carcinoma of the colon is rare. Most patients with this type of cancer have a poor prognosis. We describe a patient with signet-ring cell carcinoma of the colon, for whom leptomeningeal metastasis presented a variety of clinical symptoms. Ó 2009 Elsevier Ltd. All rights reserved.
Keywords: Signet-ring carcinoma Colon Leptomeningeal metastasis
* Corresponding author. Address: Acibadem Kozyatagi Hospital Medical Oncology Department, Inonu Caddesi Okur Sokak No: 20, Istanbul 34742, Turkey. Tel.: +90 532 571 56 07; fax: +90 216 571 40 00. E-mail address: [email protected] (Y. Yildirim).
1. Introduction Most colorectal carcinomas (CRC) are adenocarcinomas, although several other histological subtypes, including signet-ring
1052
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1051–1053
cell carcinoma (SRCC) also occur. The incidence of SRCC of the colon is 0.6/100 000.1 The most common metastatic sites for CRC are the liver and lung; metastasis to other sites is rare. However, rather than hepatic metastasis, SRCC shows a high incidence of peritoneal metastasis.1 The incidence of leptomeningeal involvement (LMI) in patients with CRC has not been established, but in a cohort study the cumulative incidence of cranial metastasis in patients with CRC was calculated as 1.2%.2 We describe a patient with a rare pathologic subtype of colon cancer and an equally rare metastatic presentation. 2. Case report A 58-year-old man with a history of vomiting and abdominal pain was admitted to the emergency department. Abdominal ultrasound examination showed a thickening in the splenic flexure. Colonoscopic biopsy showed SRCC characterized by abundant intracytoplasmic mucin, pushing the nuclei to the periphery of the cell, characteristic for SRCC. The patient’s preoperative carcinoembryonic antigen (CEA) level was 22 ng/mL (normal range 0– 4 ng/mL). A left-sided hemicolectomy and lymph node dissection were performed. Microscopic examination showed the tumor consisted of more than 50% signet-ring cells. Tumor cells were arranged in loose clusters and spread diffusely through the bowel wall. Metastatic involvement in 10 of 13 lymph nodes was identified. The patient completed a 6-cycle regimen of the adjuvant 5-fluorouracil and folinic acid. An increase in the patient’s CEA level of 6 ng/mL was noted 18 months after the last cycle of chemotherapy. At that time, colonoscopy showed a polypoid lesion 30 cm from the anal canal. A biopsy again revealed SRCC. Subtotal colectomy, appendectomy and ileoproctostomy were performed. The tumor was found in the muscular layer and in the appendectomy specimen lymph nodes. After the second operation CEA levels returned to normal. The patient received the same regimen of adjuvant therapy.
One month after the last cycle of chemotherapy, the patient’s CEA level increased to 6.0 ng/mL. To avoid a possible recurrence, the patient was evaluated with a colonoscopy and an abdominal CT scan. No abnormalities were found. One month later, owing to a rise in the CEA level, the patient was evaluated with positron emission tomography (PET) but nothing was found. Three months later the patient was referred to the otorhinolaryngology and neurology departments because of headaches and a decrease in vision and hearing. An internal acoustic MRI showed bilateral metastasis of cranial nerves VII and VIII (Fig. 1). At that time the CEA level was 13.1 ng/mL. Lumbar puncture revealed malignant cells in the cerebrospinal fluid (CSF). Craniospinal radiotherapy was commenced. However, the patient’s condition deteriorated gradually and he died 2 months after radiotherapy.
3. Discussion Most SRCCs develop in the stomach; the remainder develop in other primary organs including the colon, rectum, pancreas, urinary bladder and breast. Primary SRCC carcinoma of the colon and rectum forms nearly 1% of all adenocarcinomas of the large intestine.1 These carcinomas show more malignant biological behaviour than typical CRC.3 SRCC can be recognized as a stageindependent prognostic factor for an adverse outcome. CRCs can spread locally or distantly via the lymphatic and venous system. The most frequent sites of metastasis from CRC are the liver, lung, peritoneum, bone, ovaries, and adrenal glands.4 However, a low incidence of hepatic metastasis and high incidence of peritoneal spread are the distinguishing features of SRCC. Cranial metastases are rare and a sign of terminal disease. LMI occurs in 3% to 8% of all cancer patients; however, it is usually seen in patients with leukemia, breast cancer, lymphoma, or lung cancer.5 Central nervous system (CNS) metastasis can occur via communication between the portal system and paravertebral veins. When a tumor spreads to the leptomeninges it gains access to the entire CNS. Patients usually present with a myriad of symptoms, including altered mental status, spinal sensory changes, lower extremity weakness, headache, nausea and vomiting. On neurological examination, cranial nerve palsy and lower extremity weakness are common. Due to the infrequency of LMI in CRC, and the fact that similar symptoms can be caused by chemotherapy itself, it is not easy to attribute all these signs and symptoms to a particular clinical syndrome. Gadoliniumenhanced MRI could be helpful in two-thirds of patients. Enhancement of the dura and nodular lesions on the MRI can indicate LMI, which is usually not detected by an enhanced CT scan of the brain. Additionally, CSF examination may show malignant cells. Craniospinal radiotherapy may improve some of the symptoms but LMI generally results in death.
4. Conclusion SRCC carcinomas of the colon and LMI in CRC are both rare. Each has been presented in several case reports; however, until now, a patient with SRCC of the colon with LMI has not been reported. In this patient, a continuous CEA rise suggested metastasis although a PET scan was negative. Gadolinium-enhanced MRI may aid the diagnosis of LMI.
References Fig. 1. An coronal internal acoustic T1-weighted contrast-enhanced MRI showing bilateral metastasis to cranial nerves VII and VIII.
1. Kang H, O’Connell JB, Maggard MA, et al. A 10-year outcomes evaluation of mucinous and signet-ring cell carcinoma of the colon and rectum. Dis Colon Rectum 2005;48:1161–8.
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1053–1056 2. Schouten LJ, Rutten J, Huveneers HA, et al. Incidence of brain metastases in a cohort of patients with carcinoma of the breast, colon, kidney and lung and melanoma. Cancer 2002;94:2698–705. 3. Shimaoka S, Niihara T, Tashiro K, et al. Signet-ring cell carcinoma of the colon 7 mm in size with peritonitis carcinomatosa. J Gastroenterol 2002;37:550–5.
1053
4. August DA, Ottow RT, Sugarbaker PH. Clinical perspective of human colorectal cancer metastasis. Cancer Metastasis Rev 1984;3:303–42. 5. Grossman SA, Krabak MJ. Leptomeningeal carcinomatosis. Cancer Treat Rev 1999;25:103–19.
doi:10.1016/j.jocn.2009.10.014
Intraoperative monitoring during surgery for hypoglossal schwannoma Mami Ishikawa *, Gen Kusaka, Kouichi Takashima, Haruna Kamochi, Soji Shinoda Department of Neurosurgery, Saitama Medical Center, Jichi Medical University, 847 Amanumacho, Omiyaku, Saitama-shi, 330-8503 Saitama-ken, Japan
a r t i c l e
i n f o
Article history: Received 12 June 2009 Accepted 14 September 2009
Keywords: Electromyography Evoked potential Hypoglossal nerve Operative monitoring Schwannoma
a b s t r a c t A 54-year-old man presented with an intracranial schwannoma of the hypoglossal nerve between the medulla and the left hypoglossal canal. The condylar fossa approach was used with intra-operative electromyography (EMG) monitoring of the lower cranial nerves. The tumor was then removed carefully without decreasing the tongue EMG responses. EMG monitoring enabled us to remove the tumor while maintaining the function of the hypoglossal nerve. Tongue EMG was easily recorded by stimulating the hypoglossal nerve fibers, which was useful in identifying the hypoglossal nerve and evaluating its function. This suggests that tongue EMG is a useful monitoring tool to enhance neurological outcome following removal of tumors in this region. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
2.1. Operative procedure
Schwannoma of the hypoglossal nerve is rare, as reported by Piccirilli et al., whose review found only 105 patients with hypoglossal schwannoma prior to 2007.1 We often operate on intracranial schwannomas, most frequently vestibular schwannomas, and use electrophysiological monitoring to avoid facial nerve palsy. Intraoperative electromyographic (EMG) monitoring of the lower cranial nerves (CN: IX, X, XI, XII) is also useful for their identification and localization.2–4 We present a patient with an intracranial hypoglossal schwannoma. This schwannoma was removed with monitoring of the lower cranial nerves using EMG responses elicited by the stimulation of the hypoglossal nerve and the vagus nerve, to minimize additional neurological deficits.
Informed consent was obtained from the patient before the operation. General anesthesia was induced with succinyl choline and propofol, which was maintained with fentanyl and propofol. The patient was connected to the equipment (Neuropack, Nihon Kohden, Tokyo, Japan) for EMG monitoring of the lower cranial nerves and short latency somatosensory evoked potentials (SSEP) monitoring. The condylar fossa approach5–8 was performed following a left median hockey-stick incision. Incision of the dura mater exposed the tumor, over which the vagal nerve and the accessory nerve were slightly stretched (Fig. 2A). However, these nerves did not adhere to the tumor and were easily moved, along with the arachnoid, to the side of the medulla. These nerves were identified as the vagal nerve, the accessory nerve and the hypoglossal nerve by the EMG response from the vocal cords (Fig. 3A), movement of the patient’s shoulder and the EMG response from the tongue (Fig. 3B), respectively. Stimulation of some of the roots of the hypoglossal nerve fibers elicited EMG responses from the tongue whereas stimulation of other roots did not elicit EMG responses. The tumor was removed carefully without causing EMG response changes from the tongue (Figs. 2B, 3C). Parts of the tumor connected to hypoglossal nerve fibers that did not elicit EMG responses from the tongue following stimulation were removed. The hypoglossal nerve fiber roots associated with EMG responses were not seen to merge with the tumor. The hypoglossal nerve arises from 3–15 roots to form the rostral and caudal trunks.9,10 In our patient, the tumor arose from the caudal trunk, which was also reported in Kikkawa’s study.10 Although radiologically the tumor appeared to have been removed totally (Figs. 1C, 2C), a small amount of tumor that adhered to hypoglossal nerve fibers, and which elicited EMG responses from the tongue when stimulated, could not be removed. There was no decrease in SSEP during the operation.
2. Case report A 54-year-old man with a 1-year history of hoarseness and weakness of the left side of the tongue is presented. He noticed hemiatrophy of the left side of the tongue in November 2007 and was admitted to our hospital on April 2008. CT scans showed slight enlargement of the left hypoglossal canal (Fig. 1A). Gadoliniumenhanced T1-weighted MRI (Fig. 1B) revealed a faintly enhanced lesion between the medulla and the left hypoglossal canal, extending into the canal with a cystic component. Angiograms showed no tumor stain or feeder.
* Corresponding author. Tel.: +81 48 6472111; fax: +81 48 6485180. E-mail address: [email protected] (M. Ishikawa).