The Journal of Craniofacial Surgery • Volume 25, Number 4, July 2014
Brief Clinical Studies
consistent with others.8,25,26 Basically, the primary trigeminal neuralgia often occurs in middle- or old-aged group because of the fact that brain sulci atrophy and cerebrovascular atherosclerosis with hypertension27 resulting from the aging process result in the contaction between the cranial nerve and surrounding vessels.
CONCLUSIONS Our study showed that surgical management of those patients with CPA cholesteatoma presented as TN may lead to a satisfactory result. The principle of the surgery is to remove the tumor firstly with care of protecting those cranial nerves. Afterwards, the offending artery could be withdrawn from the nerve easily. Sometimes, the offending artery may not be found after the tumor resection for it may have been transposed off while the tumor being removed.
REFERENCES 1. Michaels L. Pathology of cholesteatoma: a review. J R Soc Med 1979;72:366 2. Hao S, Tang J, Wu Z, et al. Natural malignant transformation of an intracranial epidermoid cyst. J Formos Med Assoc 2010;109:390–396 3. Morishita T, Watanabe T, Ohta T, et al. Atypical epidermoid cyst with repetitive hemorrhages in the supracallosal region. Neurol Med Chir (Tokyo) 2009;49:492–494 4. Martinez-Lage JF, Quiñonez MA, Poza M, et al. Congenital epidermoid cysts over the anterior fontanelle. Childs Nerv Syst 1985;1:319–323 5. Roka YB, Bista P, Sharma GR, et al. Cerebellopontine epidermoid presenting with trigeminal neuralgia for 10 years: a case report. Cases J 2009;2:9345 6. Cruccu G, Leandri M, Feliciani M, et al. Idiopathic and symptomatic trigeminal pain. J Neurol Neurosurg Psychiatry 1990;53:1034–1042 7. Barker FG, Jannetta PJ, Bissonette DJ, et al. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med 1996;334:1077–1084 8. Kobata H, Kondo A, Iwasaki K. Cerebellopontine angle epidermoids presenting with cranial nerve hyperactive dysfunction: pathogenesis and long-term surgical results in 30 patients. Neurosurgery 2002;50:276–286 9. Jamjoom AB, Jamjoom ZA, Al-Fehaily M, et al. Trigeminal neuralgia related to cerebellopontine angle tumors. Neurosurg Rev 1996;19:237–241 10. Yasargil GM, Abernathey CD, Sarioglu AÇ. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561–567 11. Diraz A, Kobayashi S, Kyoshima K, et al. Transpetrosal extreme lateral suboccipital approach for extensive posterior fossa epidermoid cyst—case report. Neurol Med Chir (Tokyo) 1992;32:589 12. Nagasawa D, Yew A, Safaee M, et al. Clinical characteristics and diagnostic imaging of epidermoid tumors. J Clin Neurosci 2011;18:1158–1162 13. Ilıca AT, Hıdır Y, Bulakbaşı N, et al. HASTE diffusion-weighted MRI for the reliable detection of cholesteatoma. Diagn Interv Radiol 2011;18:153–158 14. Olson JJ, Beck DW, Crawford SC, et al. Comparative evaluation of intracranial epidermoid tumors with computed tomography and magnetic resonance imaging. Neurosurgery 1987;21:357–360 15. Goru S, Pemberton M. Trigeminal neuralgia: the role of magnetic resonance imaging. Br J Oral Maxillofac Surg 2009;47:228–229 16. Kawamura N, Uesaka T, Inamura T, et al. [Small epidermoid induced trigeminal neuralgia unrecognized by conventional CT and MRI for over 25 years]. No To Shinkei 2000;52:1113 17. Ichimura S, Hayashi T, Yazaki T, et al. Dumbbell-shaped intradiploic epidermoid cyst involving the dura mater and cerebellum. Neurol Med Chir (Tokyo) 2008;48:83–85 18. Ayache D, Williams MT, Lejeune D, et al. Usefulness of delayed postcontrast magnetic resonance imaging in the detection of residual cholesteatoma after canal wall-up tympanoplasty. Laryngoscope 2005;115:607–610
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19. Puca A, Meglio M, Tamburrini G, et al. Trigeminal involvement in intracranial tumours. Anatomical and clinical observations on 73 patients. Acta Neurochir 1993;125:47–51 20. Iwasaki K, Kondo A, Otsuka S, et al. Painful tic convulsif caused by a brain tumor: case report and review of the literature. Neurosurgery 1992;30:916–918 21. Yang X-S, Li S-T, Zhong J, et al. Microvascular decompression on patients with trigeminal neuralgia caused by ectatic vertebrobasilar artery complex: technique notes. Acta Neurochir 2012;154:793–797 22. Zhong J, Li S-T, Zhu J, et al. A clinical analysis on microvascular decompression surgery in a series of 3000 cases. Clin Neurol Neurosurg 2012;114:846–851 23. Zhong J, Zhu J, Li S-T, et al. Microvascular decompressions in patients with coexistent hemifacial spasm and trigeminal neuralgia. Neurosurgery 2011;68:916–920 24. Zhong J, Li S-T, Xu S-Q, et al. Management of petrosal veins during microvascular decompression for trigeminal neuralgia. Neurol Res 2008;30:697–700 25. Schiefer TK, Link MJ. Epidermoids of the cerebellopontine angle: a 20-year experience. Surg Neurol 2008;70:584–590 26. Shulev Y, Trashin A, Gordienko K. Secondary trigeminal neuralgia in cerebellopontine angle tumors. Skull Base 2011;21:287 27. Feng B, Zheng X, Zhang W, et al. Surgical treatment of pediatric hemifacial spasm patients. Acta Neurochir 2011;153:1031–1035
A Rare Solitary Neurofibroma of the Frontal Sinus Hongxing Li, MM,*† Xiaoxiao Peng, MB,‡ Junqing Li, MM,* Kongbin Yang, MD* Abstract: Neurofibromas are common peripheral nerve sheath tumors related to Schwann cell’s proliferation and are usually associated with neurofibromatosis type 1. Although solitary neurofibroma that occurs in the paranasal sinus is reported occasionally, neurofibroma located in the frontal sinus is extremely rare. Here, we report a case of a young woman who had a heterogeneous lesion in the left frontal sinus, eroding its anterior and posterior wall with signs of intracranial invasion. Postoperatively, results of the histologic examination confirmed the diagnosis of solitary neurofibroma. In conclusion, solitary neurofibroma should be considered in the differential diagnosis of frontal sinus masses. Key Words: Solitary neurofibroma, frontal sinus, neurofibromatosis, review
From the *Department of Neurosurgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang; †Department of Neurosurgery, Shengli Oilfield Central Hospital affiliated to Binzhou Medical University; and ‡Department of Intensive Care Unit, Dongying People’s Hospital, Dongying, Shandong, the People’s Republic of China. Received December 2, 2013. Accepted for publication January 17, 2014. Address correspondence and reprint requests to Kongbin Yang, MD, Department of Neurosurgery, The First Affiliated Hospital of Harbin Medical University, 23 Youzheng St, Harbin, Heilongjiang 150001, the People’s Republic of China; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000784
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 25, Number 4, July 2014
Brief Clinical Studies
N
eurofibromas are benign, heterogeneous peripheral nerve sheath tumors, deriving from an admixture of perineural fibroblast proliferations and Schwann cells.1 Clinical features show hard and tough cylindrical or fusiform mass, multiple small nodules, or localized lipoma-like mass, which are usually associated with neurofibromatosis type 1 (NF-1). Solitary neurofibromas that occur in patients without stigmata or family history of NF-1 are probably caused by a somatic mutation. The head and neck region is the common site for neurofibromas, but solitary lesion in the frontal sinus is rare. In the current study, we present an uncommon case of solitary neurofibroma that absorbs the anterior and posterior wall of the frontal sinus, with signs of intracranial invasion in a female patient without any other manifestation or family history of NF-1.
FIGURE 2. T1-weighted postcontrast sagittal MRI: the lesion has been enhanced, with signs of endocranium infiltration and intracranial extension (white arrow).
appearance of solitary neurofibroma (Figs. 3A, B). The 5 months of follow-up examination revealed no evidences of tumor recurrence.
DISCUSSION CLINICAL REPORT A 26-year-old woman with a swelling on the forehead, followed by a progressive enlargement during the past 2 months, was admitted to our department. There was an oval soft tissue mass on the left side of the forehead without local skin changes such as inflammation of the sinus. It was not associated with epistaxis, nasal congestion, or nasal obstruction. No systemic symptoms were observed, such as fever or weight loss. On palpation, the mass was of mild tenderness, hard, and fixed, with no lymph node enlargement. No café au lait spots, similar masses, or other skin lesions were detected in her body. No family or medical history of any similar conditions was obtained. Results of computed tomographic (CT) examination revealed an isolated soft tissue mass located in the left frontal sinus eroding the anterior and posterior wall, with bone destruction and intracranial extension (Figs. 1A, B). Magnetic resonance imaging (MRI) scan also detected an isointense mass in T1-weighted and T2-weighted images (Figs. 1C, D). With contrast-enhanced MRI scan, the mass has been enhanced significantly (Fig. 2). The patient underwent mass resection under general anesthesia via coronal incision within the hairline. On observation, the mass was pale pink, with no encapsulation, mulberry, and 25 20 20 mm in size, located along the frontal sinus wall, with intracranial invasion and adhesions to the endocranium. In addition, a small osteoma (5.0 3.0 3.0 mm) was found below it. After the tumor removal, we observed the defects of the anterior and posterior walls of the frontal sinus, which were approximately 20 20 mm and 20 15 mm, respectively. There were no postoperative complications. Histopathologically, the tumor showed the characteristic
FIGURE 1. A, Soft tissue window CT. A soft tissue mass was located in the left frontal sinus, eroding the frontal bone and protruding into the forehead (black arrow). B, Bone window CT. Note the lesion eroding the anterior and posterior walls of the frontal sinus, with bone destruction and intracranial extension (black arrow). C, T1-weighted axial MRI. Note the oval lesion with isointense signal intensity, with a contour that is vague, with a sign of soft tissue infiltration (white arrow). D, T2-weighted sagittal MRI. Note the mass with isointense signal intensity, with signs of soft tissue infiltration and forehead swelling (white arrow).
Neurofibromas are benign but non–encapsulated tumor. They derive from the connective tissue of peripheral nerve sheaths, especially the endoneurium,1 which are composed of the Schwann cells and fibroblasts with perineurial cells and axons. Findings of electron microscopy examination showed that the mast cells embedded in an extracellular matrix.2 Neurofibromas are rare as solitary lesions and most commonly occur as part of neurofibromatosis, especially the von Recklinghausen disease. Hillstrom et al3 reported that nerve sheath tumors including neurofibromas occur more frequently in the head and neck regions, but only 4% was associated with the paranasal sinus. However, a solitary neurofibroma located in the frontal sinus is much more rare. Sharma and Hong4 first described an isolated lesion arising in the frontal sinus in a young male patient in 2012. Interestingly, that is the only 1 report to date. In this article, we present the second one in an adult female patient. Neurofibromas combine with NF-1, of which the primary clinical manifestations are café au lait patches, iris Lisch nodules, axillary freckling, and distinctive bony dysplasia. However, like any other benign lesions, solitary neurofibromas are nonspecific. The signs and symptoms depend greatly on the exact location of the tumor and the involvement of the surrounding structures. They can occur in any nerve of the body with variable symptoms, such as asymptomatic, progressive pain, weakness, tingling, or numbness. Generally, a palpable mass with a solid texture is the initial finding during physical examination. In the nose and paranasal sinus, neurofibromas usually arise from the first and second divisions of the trigeminal nerve. Bone destruction is a feature in these lesions. However, a solitary neurofibroma with intracranial extension has never been described. Because the disease incidence is low,3 the clinical presentation is a painless mass,5 and it is rarely associated with neurologic symptoms, it is difficult to diagnose a solitary neurofibroma deriving from the paranasal sinuses correctly before an operation. In our case, the initial clinical diagnosis was meningioma. Preoperative imaging plays an important role in confirming the diagnosis and planning the approach for surgery. In addition, bone absorption would be observed in some neurofibromas. Therefore, a bone window CT scan is useful. In our case, the CT scan revealed the lesion equidensite with the brain tissue, which destroyed the anterior and posterior walls of the frontal sinus, with signs of intracranial invasion. Most neurofibromas are usually isointense on T1-weighted images, have high
FIGURE 3. Histologic section. A, Typical neurofibroma containing copious spindle cells (hematoxylin-eosin stain, original magnification 100). B, Immunohistochemical staining for CD34. Some cells in the lesion are positive (100).
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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The Journal of Craniofacial Surgery • Volume 25, Number 4, July 2014
Brief Clinical Studies
signal on T2-weighted images, and are enhanced with contrast medium at MRI.6 However, MRI revealed the mass to be isointense on T1 and T2 in our case. Although imaging scan can help diagnose neurofibromas, the definite diagnosis can be obtained with a pathologic examination.3 Our case showed that parts of the tumor cells were positive for CD99, Ki-67, anti-smooth muscle antibody, B-cell lymphoma-2, and CD34. On the other hand, a small osteoma was detected, located below the lesion during the operation in our patient. It may be caused by the lesion eroding the frontal sinus wall. Generally speaking, the management of neurofibromas is dependent on symptoms and signs. For the small ones, symptoms and signs may not require any intervention. Surgical excision is considered when there is pain, progressive neurologic deterioration, compression of adjacent tissues with loss of functions, and suspected malignant degeneration. Complete resection is considered to be the mainstay of treatment of neurofibromas because they are usually not encapsulated and might have an extensive infiltration.7 For the NF-1 associated with malignant transformation, the primary treatment is surgical removal, and radiotherapy can improve local disease control and delay recurrence.8 In addition, it is moderately sensitive to chemotherapy.9 In summary, we report a rare case of solitary neurofibroma arising from the frontal sinus in a woman. Therefore, when a mass had arisen from the frontal sinus, solitary neurofibroma should be considered. Surgical resection of the lesion was performed with excellent prognosis. Postoperative recurrence is usually rare.
REFERENCES 1. Hajdu SI. Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 1993;72:3549–3552 2. Kimura M, Kamata Y, Matsumoto K, et al. Electron microscopical study on the tumor of von Recklinghausen’s neurofibromatosis. Acta Pathol Jpn 1974;24:79–91 3. Hillstrom RP, Zarbo RJ, Jacobs JR. Nerve sheath tumors of the paranasal sinuses: electron microscopy and histopathologic diagnosis. Otolaryngol Head Neck Surg 1990;102:257–263 4. Sharma SB, Hong P. Solitary neurofibroma of the frontal sinus. Case Rep Otolaryngol 2012;2012:373808 5. Das Gupta TK, Brasfield RD, Strong EW, et al. Benign solitary Schwannomas (neurilemomas). Cancer 1969;24:355–366 6. Majoie CB, Hulsmans FJ, Castelijns JA, et al. Primary nerve-sheath tumours of the trigeminal nerve: clinical and MRI findings. Neuroradiology 1999;41:100–108 7. Gottfried ON, Viskochil DH, Fults DW, et al. Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications. Neurosurgery 2006;58:1–16 8. Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res 2002;62:1573–1577 9. Ferner RE, O’Doherty MJ. Neurofibroma and schwannoma. Curr Opin Neurol 2002;15:679–684
Papillary Meningioma With Vital Abnormal Vessels Hai-Bo Zhang, PhD,* Yi-Ping Zhan, PhD,† Xin-Guang Yu, PhD* Abstract: Papillary meningioma is an uncommon meningioma subtype of World Health Organization grade III. It could show some
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radiologic profiles pointing to malignant behavior, such as a cystic change, a heterogeneous enhancement, and an ill- defined border. However, to date, the radiologic profile described in this article has not been reported in previous literatures, and it is just the characteristic being considered as the major cause for patients’ death. A 16-year-old adolescent boy with a 6-month history of headache was admitted to our department on June 28, 2012. Magnetic resonance imaging showed a giant well-defined mass in the left temporal region, with a severe flow void on T2-weighted image and an abundant stripelike enhancement on T1-weighted contrast-enhanced scan. In view of its middle cranial fossa location (one predilection site for meningioma), meningioma was suspected preoperatively. A regular left frontotemporal craniotomy was performed. Unexpectedly, extreme hemorrhage happened intraoperatively, and it was difficult to stop the bleeding. After identification of no hemorrhage in the operative cavity through intraoperative magnetic resonance imaging, the operation was finished, with an overall blood loss of 15,000 mL. The patient died of brain stem dysfunction the second day after the operation. Key Words: Papillary meningioma, flow void
P
apillary meningioma (PM) is one rare subtype that is included in the World Health Organization grade III meningioma. To date, fewer than 200 cases of PM have been published in the English-language literatures. Many factors (including the extent of resection, the presence of some positive histopathologic index, and adjuvant therapy, etc) had been reported to be significantly related to postoperative outcome,1,2 but no literature demonstrated the influence of vessel voids on survival. Hereby, we present a rare case of PM with hazardous abnormal vessels manifesting marked signal voids on magnetic resonance imaging (MRI) observation. Meanwhile, we discussed some key points from this case.
CLINICAL REPORT A 16-year-old adolescent boy who had a 6-month history of headache was referred to our department on June 28, 2012. No special neurologic dysfunction was found on physical examination. Computed tomographic scan showed a high-density mass of the left temporal region with some ductlike shadow inside (Fig. 1A). Magnetic resonance images revealed marked signal voids intratumorally and 2 conspicuous serpentine signal voids located adjacent to the brain stem on T2-weighted sequence (Fig. 1B) as well as markedly stripelike enhancement on contrast- enhancing sequence (Fig. 1C). Extra-axial neoplasm, with the most possibility of meningioma, was considered preoperatively because of the same enhancement level of tentorium cerebelli margin nearby the tumor base. After the preoperative From the *Institute of Neurosurgery, Chinese PLA General Hospital; and †Department of Neurosurgery, Beijing Sanbo Brain Hospital, Capital Medical University, Beijing, China Received December 13, 2013. Accepted for publication January 17, 2014. Address correspondence and reprint requests to Xin-Guang Yu, PhD, Institute of Neurosurgery, Chinese PLA General Hospital, Beijing, 100853, China; E-mail: [email protected] The authors report no conflicts of interest. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License, where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in anyway or used commercially. ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000792
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
consistent with others.8,25,26 Basically, the primary trigeminal neuralgia often occurs in middle- or old-aged group because of the fact that brain sulci atrophy and cerebrovascular atherosclerosis with hypertension27 resulting from the aging process result in the contaction between the cranial nerve and surrounding vessels.
CONCLUSIONS Our study showed that surgical management of those patients with CPA cholesteatoma presented as TN may lead to a satisfactory result. The principle of the surgery is to remove the tumor firstly with care of protecting those cranial nerves. Afterwards, the offending artery could be withdrawn from the nerve easily. Sometimes, the offending artery may not be found after the tumor resection for it may have been transposed off while the tumor being removed.
REFERENCES 1. Michaels L. Pathology of cholesteatoma: a review. J R Soc Med 1979;72:366 2. Hao S, Tang J, Wu Z, et al. Natural malignant transformation of an intracranial epidermoid cyst. J Formos Med Assoc 2010;109:390–396 3. Morishita T, Watanabe T, Ohta T, et al. Atypical epidermoid cyst with repetitive hemorrhages in the supracallosal region. Neurol Med Chir (Tokyo) 2009;49:492–494 4. Martinez-Lage JF, Quiñonez MA, Poza M, et al. Congenital epidermoid cysts over the anterior fontanelle. Childs Nerv Syst 1985;1:319–323 5. Roka YB, Bista P, Sharma GR, et al. Cerebellopontine epidermoid presenting with trigeminal neuralgia for 10 years: a case report. Cases J 2009;2:9345 6. Cruccu G, Leandri M, Feliciani M, et al. Idiopathic and symptomatic trigeminal pain. J Neurol Neurosurg Psychiatry 1990;53:1034–1042 7. Barker FG, Jannetta PJ, Bissonette DJ, et al. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med 1996;334:1077–1084 8. Kobata H, Kondo A, Iwasaki K. Cerebellopontine angle epidermoids presenting with cranial nerve hyperactive dysfunction: pathogenesis and long-term surgical results in 30 patients. Neurosurgery 2002;50:276–286 9. Jamjoom AB, Jamjoom ZA, Al-Fehaily M, et al. Trigeminal neuralgia related to cerebellopontine angle tumors. Neurosurg Rev 1996;19:237–241 10. Yasargil GM, Abernathey CD, Sarioglu AÇ. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561–567 11. Diraz A, Kobayashi S, Kyoshima K, et al. Transpetrosal extreme lateral suboccipital approach for extensive posterior fossa epidermoid cyst—case report. Neurol Med Chir (Tokyo) 1992;32:589 12. Nagasawa D, Yew A, Safaee M, et al. Clinical characteristics and diagnostic imaging of epidermoid tumors. J Clin Neurosci 2011;18:1158–1162 13. Ilıca AT, Hıdır Y, Bulakbaşı N, et al. HASTE diffusion-weighted MRI for the reliable detection of cholesteatoma. Diagn Interv Radiol 2011;18:153–158 14. Olson JJ, Beck DW, Crawford SC, et al. Comparative evaluation of intracranial epidermoid tumors with computed tomography and magnetic resonance imaging. Neurosurgery 1987;21:357–360 15. Goru S, Pemberton M. Trigeminal neuralgia: the role of magnetic resonance imaging. Br J Oral Maxillofac Surg 2009;47:228–229 16. Kawamura N, Uesaka T, Inamura T, et al. [Small epidermoid induced trigeminal neuralgia unrecognized by conventional CT and MRI for over 25 years]. No To Shinkei 2000;52:1113 17. Ichimura S, Hayashi T, Yazaki T, et al. Dumbbell-shaped intradiploic epidermoid cyst involving the dura mater and cerebellum. Neurol Med Chir (Tokyo) 2008;48:83–85 18. Ayache D, Williams MT, Lejeune D, et al. Usefulness of delayed postcontrast magnetic resonance imaging in the detection of residual cholesteatoma after canal wall-up tympanoplasty. Laryngoscope 2005;115:607–610
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19. Puca A, Meglio M, Tamburrini G, et al. Trigeminal involvement in intracranial tumours. Anatomical and clinical observations on 73 patients. Acta Neurochir 1993;125:47–51 20. Iwasaki K, Kondo A, Otsuka S, et al. Painful tic convulsif caused by a brain tumor: case report and review of the literature. Neurosurgery 1992;30:916–918 21. Yang X-S, Li S-T, Zhong J, et al. Microvascular decompression on patients with trigeminal neuralgia caused by ectatic vertebrobasilar artery complex: technique notes. Acta Neurochir 2012;154:793–797 22. Zhong J, Li S-T, Zhu J, et al. A clinical analysis on microvascular decompression surgery in a series of 3000 cases. Clin Neurol Neurosurg 2012;114:846–851 23. Zhong J, Zhu J, Li S-T, et al. Microvascular decompressions in patients with coexistent hemifacial spasm and trigeminal neuralgia. Neurosurgery 2011;68:916–920 24. Zhong J, Li S-T, Xu S-Q, et al. Management of petrosal veins during microvascular decompression for trigeminal neuralgia. Neurol Res 2008;30:697–700 25. Schiefer TK, Link MJ. Epidermoids of the cerebellopontine angle: a 20-year experience. Surg Neurol 2008;70:584–590 26. Shulev Y, Trashin A, Gordienko K. Secondary trigeminal neuralgia in cerebellopontine angle tumors. Skull Base 2011;21:287 27. Feng B, Zheng X, Zhang W, et al. Surgical treatment of pediatric hemifacial spasm patients. Acta Neurochir 2011;153:1031–1035
A Rare Solitary Neurofibroma of the Frontal Sinus Hongxing Li, MM,*† Xiaoxiao Peng, MB,‡ Junqing Li, MM,* Kongbin Yang, MD* Abstract: Neurofibromas are common peripheral nerve sheath tumors related to Schwann cell’s proliferation and are usually associated with neurofibromatosis type 1. Although solitary neurofibroma that occurs in the paranasal sinus is reported occasionally, neurofibroma located in the frontal sinus is extremely rare. Here, we report a case of a young woman who had a heterogeneous lesion in the left frontal sinus, eroding its anterior and posterior wall with signs of intracranial invasion. Postoperatively, results of the histologic examination confirmed the diagnosis of solitary neurofibroma. In conclusion, solitary neurofibroma should be considered in the differential diagnosis of frontal sinus masses. Key Words: Solitary neurofibroma, frontal sinus, neurofibromatosis, review
From the *Department of Neurosurgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang; †Department of Neurosurgery, Shengli Oilfield Central Hospital affiliated to Binzhou Medical University; and ‡Department of Intensive Care Unit, Dongying People’s Hospital, Dongying, Shandong, the People’s Republic of China. Received December 2, 2013. Accepted for publication January 17, 2014. Address correspondence and reprint requests to Kongbin Yang, MD, Department of Neurosurgery, The First Affiliated Hospital of Harbin Medical University, 23 Youzheng St, Harbin, Heilongjiang 150001, the People’s Republic of China; E-mail: [email protected] The authors report no conflicts of interest. Copyright © 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000784
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery • Volume 25, Number 4, July 2014
Brief Clinical Studies
N
eurofibromas are benign, heterogeneous peripheral nerve sheath tumors, deriving from an admixture of perineural fibroblast proliferations and Schwann cells.1 Clinical features show hard and tough cylindrical or fusiform mass, multiple small nodules, or localized lipoma-like mass, which are usually associated with neurofibromatosis type 1 (NF-1). Solitary neurofibromas that occur in patients without stigmata or family history of NF-1 are probably caused by a somatic mutation. The head and neck region is the common site for neurofibromas, but solitary lesion in the frontal sinus is rare. In the current study, we present an uncommon case of solitary neurofibroma that absorbs the anterior and posterior wall of the frontal sinus, with signs of intracranial invasion in a female patient without any other manifestation or family history of NF-1.
FIGURE 2. T1-weighted postcontrast sagittal MRI: the lesion has been enhanced, with signs of endocranium infiltration and intracranial extension (white arrow).
appearance of solitary neurofibroma (Figs. 3A, B). The 5 months of follow-up examination revealed no evidences of tumor recurrence.
DISCUSSION CLINICAL REPORT A 26-year-old woman with a swelling on the forehead, followed by a progressive enlargement during the past 2 months, was admitted to our department. There was an oval soft tissue mass on the left side of the forehead without local skin changes such as inflammation of the sinus. It was not associated with epistaxis, nasal congestion, or nasal obstruction. No systemic symptoms were observed, such as fever or weight loss. On palpation, the mass was of mild tenderness, hard, and fixed, with no lymph node enlargement. No café au lait spots, similar masses, or other skin lesions were detected in her body. No family or medical history of any similar conditions was obtained. Results of computed tomographic (CT) examination revealed an isolated soft tissue mass located in the left frontal sinus eroding the anterior and posterior wall, with bone destruction and intracranial extension (Figs. 1A, B). Magnetic resonance imaging (MRI) scan also detected an isointense mass in T1-weighted and T2-weighted images (Figs. 1C, D). With contrast-enhanced MRI scan, the mass has been enhanced significantly (Fig. 2). The patient underwent mass resection under general anesthesia via coronal incision within the hairline. On observation, the mass was pale pink, with no encapsulation, mulberry, and 25 20 20 mm in size, located along the frontal sinus wall, with intracranial invasion and adhesions to the endocranium. In addition, a small osteoma (5.0 3.0 3.0 mm) was found below it. After the tumor removal, we observed the defects of the anterior and posterior walls of the frontal sinus, which were approximately 20 20 mm and 20 15 mm, respectively. There were no postoperative complications. Histopathologically, the tumor showed the characteristic
FIGURE 1. A, Soft tissue window CT. A soft tissue mass was located in the left frontal sinus, eroding the frontal bone and protruding into the forehead (black arrow). B, Bone window CT. Note the lesion eroding the anterior and posterior walls of the frontal sinus, with bone destruction and intracranial extension (black arrow). C, T1-weighted axial MRI. Note the oval lesion with isointense signal intensity, with a contour that is vague, with a sign of soft tissue infiltration (white arrow). D, T2-weighted sagittal MRI. Note the mass with isointense signal intensity, with signs of soft tissue infiltration and forehead swelling (white arrow).
Neurofibromas are benign but non–encapsulated tumor. They derive from the connective tissue of peripheral nerve sheaths, especially the endoneurium,1 which are composed of the Schwann cells and fibroblasts with perineurial cells and axons. Findings of electron microscopy examination showed that the mast cells embedded in an extracellular matrix.2 Neurofibromas are rare as solitary lesions and most commonly occur as part of neurofibromatosis, especially the von Recklinghausen disease. Hillstrom et al3 reported that nerve sheath tumors including neurofibromas occur more frequently in the head and neck regions, but only 4% was associated with the paranasal sinus. However, a solitary neurofibroma located in the frontal sinus is much more rare. Sharma and Hong4 first described an isolated lesion arising in the frontal sinus in a young male patient in 2012. Interestingly, that is the only 1 report to date. In this article, we present the second one in an adult female patient. Neurofibromas combine with NF-1, of which the primary clinical manifestations are café au lait patches, iris Lisch nodules, axillary freckling, and distinctive bony dysplasia. However, like any other benign lesions, solitary neurofibromas are nonspecific. The signs and symptoms depend greatly on the exact location of the tumor and the involvement of the surrounding structures. They can occur in any nerve of the body with variable symptoms, such as asymptomatic, progressive pain, weakness, tingling, or numbness. Generally, a palpable mass with a solid texture is the initial finding during physical examination. In the nose and paranasal sinus, neurofibromas usually arise from the first and second divisions of the trigeminal nerve. Bone destruction is a feature in these lesions. However, a solitary neurofibroma with intracranial extension has never been described. Because the disease incidence is low,3 the clinical presentation is a painless mass,5 and it is rarely associated with neurologic symptoms, it is difficult to diagnose a solitary neurofibroma deriving from the paranasal sinuses correctly before an operation. In our case, the initial clinical diagnosis was meningioma. Preoperative imaging plays an important role in confirming the diagnosis and planning the approach for surgery. In addition, bone absorption would be observed in some neurofibromas. Therefore, a bone window CT scan is useful. In our case, the CT scan revealed the lesion equidensite with the brain tissue, which destroyed the anterior and posterior walls of the frontal sinus, with signs of intracranial invasion. Most neurofibromas are usually isointense on T1-weighted images, have high
FIGURE 3. Histologic section. A, Typical neurofibroma containing copious spindle cells (hematoxylin-eosin stain, original magnification 100). B, Immunohistochemical staining for CD34. Some cells in the lesion are positive (100).
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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The Journal of Craniofacial Surgery • Volume 25, Number 4, July 2014
Brief Clinical Studies
signal on T2-weighted images, and are enhanced with contrast medium at MRI.6 However, MRI revealed the mass to be isointense on T1 and T2 in our case. Although imaging scan can help diagnose neurofibromas, the definite diagnosis can be obtained with a pathologic examination.3 Our case showed that parts of the tumor cells were positive for CD99, Ki-67, anti-smooth muscle antibody, B-cell lymphoma-2, and CD34. On the other hand, a small osteoma was detected, located below the lesion during the operation in our patient. It may be caused by the lesion eroding the frontal sinus wall. Generally speaking, the management of neurofibromas is dependent on symptoms and signs. For the small ones, symptoms and signs may not require any intervention. Surgical excision is considered when there is pain, progressive neurologic deterioration, compression of adjacent tissues with loss of functions, and suspected malignant degeneration. Complete resection is considered to be the mainstay of treatment of neurofibromas because they are usually not encapsulated and might have an extensive infiltration.7 For the NF-1 associated with malignant transformation, the primary treatment is surgical removal, and radiotherapy can improve local disease control and delay recurrence.8 In addition, it is moderately sensitive to chemotherapy.9 In summary, we report a rare case of solitary neurofibroma arising from the frontal sinus in a woman. Therefore, when a mass had arisen from the frontal sinus, solitary neurofibroma should be considered. Surgical resection of the lesion was performed with excellent prognosis. Postoperative recurrence is usually rare.
REFERENCES 1. Hajdu SI. Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 1993;72:3549–3552 2. Kimura M, Kamata Y, Matsumoto K, et al. Electron microscopical study on the tumor of von Recklinghausen’s neurofibromatosis. Acta Pathol Jpn 1974;24:79–91 3. Hillstrom RP, Zarbo RJ, Jacobs JR. Nerve sheath tumors of the paranasal sinuses: electron microscopy and histopathologic diagnosis. Otolaryngol Head Neck Surg 1990;102:257–263 4. Sharma SB, Hong P. Solitary neurofibroma of the frontal sinus. Case Rep Otolaryngol 2012;2012:373808 5. Das Gupta TK, Brasfield RD, Strong EW, et al. Benign solitary Schwannomas (neurilemomas). Cancer 1969;24:355–366 6. Majoie CB, Hulsmans FJ, Castelijns JA, et al. Primary nerve-sheath tumours of the trigeminal nerve: clinical and MRI findings. Neuroradiology 1999;41:100–108 7. Gottfried ON, Viskochil DH, Fults DW, et al. Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications. Neurosurgery 2006;58:1–16 8. Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res 2002;62:1573–1577 9. Ferner RE, O’Doherty MJ. Neurofibroma and schwannoma. Curr Opin Neurol 2002;15:679–684
Papillary Meningioma With Vital Abnormal Vessels Hai-Bo Zhang, PhD,* Yi-Ping Zhan, PhD,† Xin-Guang Yu, PhD* Abstract: Papillary meningioma is an uncommon meningioma subtype of World Health Organization grade III. It could show some
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radiologic profiles pointing to malignant behavior, such as a cystic change, a heterogeneous enhancement, and an ill- defined border. However, to date, the radiologic profile described in this article has not been reported in previous literatures, and it is just the characteristic being considered as the major cause for patients’ death. A 16-year-old adolescent boy with a 6-month history of headache was admitted to our department on June 28, 2012. Magnetic resonance imaging showed a giant well-defined mass in the left temporal region, with a severe flow void on T2-weighted image and an abundant stripelike enhancement on T1-weighted contrast-enhanced scan. In view of its middle cranial fossa location (one predilection site for meningioma), meningioma was suspected preoperatively. A regular left frontotemporal craniotomy was performed. Unexpectedly, extreme hemorrhage happened intraoperatively, and it was difficult to stop the bleeding. After identification of no hemorrhage in the operative cavity through intraoperative magnetic resonance imaging, the operation was finished, with an overall blood loss of 15,000 mL. The patient died of brain stem dysfunction the second day after the operation. Key Words: Papillary meningioma, flow void
P
apillary meningioma (PM) is one rare subtype that is included in the World Health Organization grade III meningioma. To date, fewer than 200 cases of PM have been published in the English-language literatures. Many factors (including the extent of resection, the presence of some positive histopathologic index, and adjuvant therapy, etc) had been reported to be significantly related to postoperative outcome,1,2 but no literature demonstrated the influence of vessel voids on survival. Hereby, we present a rare case of PM with hazardous abnormal vessels manifesting marked signal voids on magnetic resonance imaging (MRI) observation. Meanwhile, we discussed some key points from this case.
CLINICAL REPORT A 16-year-old adolescent boy who had a 6-month history of headache was referred to our department on June 28, 2012. No special neurologic dysfunction was found on physical examination. Computed tomographic scan showed a high-density mass of the left temporal region with some ductlike shadow inside (Fig. 1A). Magnetic resonance images revealed marked signal voids intratumorally and 2 conspicuous serpentine signal voids located adjacent to the brain stem on T2-weighted sequence (Fig. 1B) as well as markedly stripelike enhancement on contrast- enhancing sequence (Fig. 1C). Extra-axial neoplasm, with the most possibility of meningioma, was considered preoperatively because of the same enhancement level of tentorium cerebelli margin nearby the tumor base. After the preoperative From the *Institute of Neurosurgery, Chinese PLA General Hospital; and †Department of Neurosurgery, Beijing Sanbo Brain Hospital, Capital Medical University, Beijing, China Received December 13, 2013. Accepted for publication January 17, 2014. Address correspondence and reprint requests to Xin-Guang Yu, PhD, Institute of Neurosurgery, Chinese PLA General Hospital, Beijing, 100853, China; E-mail: [email protected] The authors report no conflicts of interest. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License, where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in anyway or used commercially. ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000792
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
