The Journal of Craniofacial Surgery
& Volume 25, Number 2, March 2014
recommended in asymptomatic patients, even if radiology has demonstrated optic canal narrowing.17 However, our patient complained of a sudden vision loss, and the coronal CT scan revealed the narrowing of the right optic nerve canal after the first surgery. Improvement of impaired vision after the second surgery demonstrated the efficacy of optic nerve decompression. Although TM reconstruction in craniofacial FD is popular, curative treatment plans should be made because the growth of FD does not follow a well-defined pattern. Although total excision and successive craniofacial reconstruction can be achieved, close follow-up for early diagnosis of relapse is highly recommended.
REFERENCES 1. DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848Y1864 2. Chen YR, Noordhoff MS. Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive? Plast Reconstr Surg 1990;86:835Y842 3. Chen YR, Noordhoff MS. Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive? Plast Reconstr Surg 1991;87:799Y800 4. Choi JW, Lee SW, Koh KS. Correction of proptosis and zygomaticomaxillary asymmetry using orbital wall decompression and zygoma reduction in craniofacial fibrous dysplasia. J Craniofac Surg 2009;20:326Y330 5. Mendelsohn DB, Hertzanu Y, Cohen M, et al. Computed tomography of craniofacial fibrous dysplasia. J Comput Assist Tomogr 1984;8:1062Y1065 6. Chen M,Yang C, Fang B, et al. Treatment of hemimandibular fibrous dysplasia with radical excision and immediate reconstruction with free double costochondral graft. J Oral Maxillofac Surg 2010;68:2000Y2004 7. Valentini V, Cassoni A, Marianetti TM, et al. Craniomaxillofacial fibrous dysplasia : conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg 2009;123:653Y660 8. Boni P, Ferri A, Corradi D, et al. Fibro-osseous dysplasia localized to the zygomatic arch: case report. J Craniomaxillofac Surg 2011;39:138Y140 9. Janecka IP. New reconstructive technologies in skull base surgery: role of titanium mesh and porous polyethylene. Arch Otolaryngol Head Neck Surg 2000;126:396Y401 10. Assaf AT, Benecke AW, Riecke B, et al. Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: a case report. J Craniomaxillofac Surg 2012;40:788Y792 11. Ozek C, Gundogan H, Bilkay U, et al. Craniomaxillofacial fibrous dysplasia. J Craniofac Surg 2002;13:382Y389 12. Sakata T, Takahashi K, Kang Y, et al. Repeated surgical reduction of the mandible over a 19-year period due to fibrous dysplasia: a case report. Asian J Oral Maxillofac Surg 2010;22:33Y36 13. Sadeghi SM, Hosseini SN. Spontaneous conversion of fibrous dysplasia into osteosarcoma. J Craniofac Surg 2011;22:959Y961 14. Wei Y, Jiang S, Cen Y. Fibrous dysplasia of skull. J Craniofac Surg 2010;21:538Y542 15. Eldaly A, Nour YA, Ibrahim AA, et al. Radical resection of fibrous dysplasia involving the anterior cranial base. Neurosurg Q 2012;22:126Y132 16. Cheng J, Wang Y, Yu H, et al. An epidemiological and clinical analysis of craniomaxillofacial fibrous dysplasia in a Chinese population. Orphanet J Rare Dis 2012;7:80 17. Schreiber A, Villaret AB, Maroldi R, et al. Fibrous dysplasia of the sinonasal tract and adjacent skull base. Curr Opin Otolaryngol Head Neck Surg 2012;20:45Y52
Brief Clinical Studies
Study of Malignant Peripheral Nerve Sheath Tumor in Cerebellopontine Angle WenMing Hong, MSc,* HongWei Cheng, MD,* XiaoJie Wang, MD,Þ XiaoPeng Hu, MD,þ ChunGuo Feng, BS* Abstract: Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNSTwas made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas. Key Words: Malignant peripheral nerve sheath tumor (MPNST), neurofibrosarcoma, soft tissue sarcoma, cerebellopontine angle (CPA), treatment
T
he term malignant peripheral nerve sheath tumor (MPNST) was coined by the World Health Organization to represent neoplasms, which were previously recognized by terms such as ‘‘malignant neurilemmoma,’’ ‘‘neurofibrosarcoma,’’ ‘‘neurogenic sarcoma,’’ and ‘‘malignant schwannoma.’’ Malignant peripheral nerve sheath tumor with an incidence of 0.001% and a peak incidence in the seventh decade of life in the general population is a rare soft tissue sarcoma arising from the neoplastic transformation of Schwann cells.1,2 Malignant peripheral nerve sheath tumor may arise from any peripheral nerve, and the primary symptoms depend on its anatomic location. Some study of MPNST reported that the largest numbers of tumors were seen in the shoulder, thigh, and pelvis, while with less frequent of occurrence in the thorax, abdomen, hand, forearm, and foot.3 It is evident from the current literature that the intracranial is seldom affected: to our knowledge, there is only 1 published clinical report in cerebellopontine angle (CPA) involvement by MPNST.
From the Departments of *Neurosurgery, †Pathology, and ‡Radiology, First Affiliated Hospital of AnHui Medical University, Hefei, AnHui, China. Received September 21, 2013. Accepted for publication November 27, 2013. Address correspondence and reprint requests to HongWei Cheng, MD, Department of Neurosurgery, First Affiliated Hospital of AnHui Medical University, No. 218 JiXi Rd, Hefei, AnHui 230032, China; E-mail: [email protected] HW Cheng and WM Hong contributed equally to this work. The authors report no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000622
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
699
The Journal of Craniofacial Surgery
Brief Clinical Studies
FIGURE 1. Preoperative cranial MRI scan showing a 3.0 2.0 2.0-cm mass with mixed T1 and T2 signals in the left CPA, which the area of necrotic could be seen and no surrounding edema (A). The mass had heterogeneous enhancement in T1-weighted images enhanced with gadolinium. The fourth ventricle and pons were compressed by the tumor (B).
The following description will report the rare case of MPNST in CPA and illustrated the difficulties in the diagnosis and treatment.
CLINICAL REPORT A 25-year-old man was admitted to our hospital with dizziness for 2 months, left facial numbness, and tinnitus for 10 days. The patient’s medical history and his family history were insignificant. In physical and neurological examination, left superficial facial hypoesthesia and hearing impairment were found. The routine biochemical examination was normal. The brain magnetic resonance imaging (MRI) scan showed a 3.0 2.0 2.0-cm mass with mixed T1 and T2 signals, which the area of necrotic could be seen and no edema surrounded. The mass was enhancement heterogeneous in T1-weighted images enhanced with gadolinium. The fourth ventricle and pons were compressed by the tumor (Figs. 1A, B). The x-ray of internal auditory canal found no bone destruction, the diameter of left internal auditory was 0.4 cm, and the right was 0.3 cm. The left brainstem auditory-evoked potential was abnormal. After the relevant examination was complete, the patient underwent retrosigmoid approach surgery under anesthesia. The tumor was light red, soft material with rich blood supply, which has tight adhesion with brainstem, facial nerve, and trigeminal nerve. The tumor was removed totally under the microscope. The postoperative pathology (Fig. 2) was MPNST, and the immunohistochemistry was vimentin (+), S-100 (+) (Fig. 3), glial fibrillary acidic protein (+), individual cells epithelial membrane antigen (+), cytokeratin (+), synaptophysin (+); the positive rate of Kit was 20%. Teniposide chemotherapy for 3 days and gamma radiotherapy twice were given postoperatively. During 2 years’ follow-up, the patient’s dizziness and left facial numbness disappeared; auditory sense improved.
DISCUSSION Malignant peripheral nerve sheath tumor derived from Schwann cells, fibroblasts, and supporting cells known as perineural cells. The incidence of this rare tumor is approximately 0.001%. Malignant
FIGURE 2. Photomicrograph showing the tumor was constituted by highly heterogeneous short spindle cells and nuclear division, and hence necrosis is easily seen; there are thick-walled blood vessels in the interstitial (hematoxylin-eosin stain, original magnification 200).
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& Volume 25, Number 2, March 2014
FIGURE 3. Immunohistochemistry showing some cells were positive (S-100 stain, original magnification 200).
peripheral nerve sheath tumors usually arise from peripheral nerves or somatic soft tissues.4 They constitute 5% of all sarcomas, and 70% are associated with neurofibromatosis type 1. Malignant peripheral nerve sheath tumors may involve proximal portions of major nerve trunks of the neck, extremities, retroperitoneum, mediastinum, and viscera. Since the first report of MPNST of Gasserian ganglion by Hedeman et al5 in 1978, the reports of MPNST involving the intracranial nerve are still very rare, no more than 32 cases, and most of them are clinical reports. The tumor of CPA is even rarer; only 1 case was reported by Melliou et al6 in 2006. The clinical signs depend on the location and the involved nerve. There is a high incidence of corneal reflex loss in the MPNST of the trigeminal nerve.7 Malignant peripheral nerve sheath tumors are highly malignant and grow rapidly along nerves with infiltration of surrounding tissue and develop hematogenous metastasis. In our case, the patient presented with dizziness, left facial numbness, tinnitus, left superficial facial hypesthesia, and hearing impairment, and the MRI scan showed the fourth ventricle and pons were compressed by the tumor. His clinical symptoms were associated with the involved nerves. Also, his clinical symptoms were very similar with acoustic neurilemmoma, but the MRI scan did not coincide with this common tumor. The possible diagnosis of MPNST was considered, and the wrong diagnosis was avoided. Magnetic resonance imaging is mostly used for MPNST, showing when present signs of malignancy-like tumor progression or multiple enhancing intradural lesions.8 The imaging findings correspond to heterogeneous enhancement, irregularity of contour, and necrosis in some cases. Adjacent structure compression and local invasion are also noted. Gadolinium enhancement has occurred in all cases to variable degrees.9 However, MPNST cannot be distinguished from other sarcomas by MRI and can be misleading when the tumor exhibits signs of benign lesion. In our case, necrotic could been see in the MRI scan, and the possibility of the tumor malignancy had been considered. The computed tomography scan set to a bone window can show invasion of bony structures and increase chance of detecting marginal erosion and that may help the diagnosis of MPNST, but the erosion of the bone was not been found in our case. The histological features of MPNSTs are those of highly cellular, spindle-cell neoplasm resembling a soft tissue sarcoma, but with differentiation toward elements of the nerve sheath, Schwann cell, and perineural cell.10 Frequent mitoses and focal necrosis are typical. Cytological features also vary and may include fibroblastic, schwannian, epithelioid, clear cell, or frankly anaplastic-pleomorphic cells. Rarely are heterologous mesenchymal or epithelial elements present. In our case, the tumor can include heterologous mesenchymal and epithelial elements. Such atypical components show hypercellularity, an increased nuclear-to-cytoplasmic ratio, cytological atypia, and increased mitotic activity. The diagnosis of MPNST is very difficult, and the spectrum of differential diagnosis includes schwannoma, paraganglioma, hemangioma, ependymoma, cavernous angioma, angiolipoma, solitary fibrous tumor, lymphoma, hemangiopericytoma, hemangioendothelioma, and * 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 2, March 2014
the rarely occurring intracranial sarcomas such as fibrosarcoma and synovial sarcoma. So far, there are no direct signs of clinical symptoms, and imaging could aid in the diagnosis of this rare tumor; the postoperative pathology is the only way. The treatment of choice is surgery, but postoperative radiotherapy and chemotherapy are part of adjunctive therapy.11 Gross total resection of the tumor is the most important therapeutic goal. Wide excision or amputation may be required in some cases. When radical tumor removal is not possible, excision combined with high-dose radiation and chemical therapy seems to be the best alternative treatment. In our case, the patient underwent once of chemotherapy and twice of gamma radiotherapy. During the 2 years’ follow-up, the patient is still alive and without any complication. The survival rate is very low. In the review, 66% of patients died, whereas 19% are alive with or without complications.12 In general, grade II tumors typically survive more than 5 years, and those with grade III tumors survive 2 to 3 years. The prognosis of patients with World Health Organization grade IV tumors depends largely on whether effective treatment regimens are available. For MPNST, survival rate was better in patient treated with adjuvant chemotherapy and radiotherapy. Our patient had an uneventful postoperative course and had remained functional for 2 years, and we attribute this favorable outcome to the complete resection of tumor and the chemotherapy and radiotherapy he received after.
CONCLUSIONS Malignant peripheral nerve sheath tumor is a rare and aggressive tumor. Compared with other tumors, there is no typical representation in symptoms, signs, and image. The pathology is the only way to diagnose this kind of tumor. Because it is aggressive, we should consider the possibility of an MPNSTwhen a tumor occurs in some special location, such as CPA in our case. A need for a multidisciplinary approach with the surgery of gross total resection, high-dose of chemotherapy, and radiotherapy is anticipated in the management of MPNST.
ACKNOWLEDGMENTS The authors thank the patient and his family members who agreed to report this rare disease to help more patients. They also thank the doctors in the Departments of Pathology and Radiology for providing clear pictures and also the staff of Journal of Craniofacial Surgery for reviewing the article. REFERENCES 1. Hajdu SI. Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 1993;72:3549Y3552 2. Than KD, Ghori AK, Wang AC, et al. Metastatic malignant peripheral nerve sheath tumor of the cauda equine. J Clin Neurosci. 2011;18:844Y846 3. Cashen DV, Parisien RC, Raskin K, et al. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res 2004;426:69Y73 4. Aydin MD, Yildirim U, Gundogdu C, et al. Malignant peripheral nerve sheath tumor of the orbit: case report and literature review. Skull Base 2004;14:109Y113 5. Hedeman LS, Lewinsky BS, Lochridge GK, et al. Primary malignant schwannoma of the Gasserian ganglion. Report of two cases. J Neurosurg 1978;48:279Y283 6. Melliou A, Karamouzis J, Helis L, et al. Malignant peripheral nerve-sheath tumor of the left cerebello-pontine angle: case report. J BUON 2006;11:367Y368 7. Lee JH, Lee HK, Choi CG, et al. Malignant peripheral nerve sheath tumor in the parapharyngeal space: tumor spread through the eustachian tube. AJNR Am J Neuroradiol 2001;22:748Y750 8. Ziadi A, Saliba I. Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. Auris Nasus Larynx 2010;37:539Y545
Brief Clinical Studies
9. Auclair PL, Langloss JM, Weiss SW, et al. Sarcomas and sarcomatoid neoplasms of the major salivary gland regions. A clinicopathologic and immunohistochemical study of 67 cases and review of the literature. Cancer 1986;58:1305Y1315 10. Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg 2009;249:1014Y1022 11. Chao BH, Stogner-Underwood KA, Kiev J, et al. Intrathoracic malignant peripheral nerve sheath tumor in neurofibromatosis 1. J Clin Oncol 2008;26:2216Y2218 12. Frahm S, Mautner VF, Brems H, et al. Genetic and phenotypic characterization of tumor cells derived from malignant peripheral nerve sheath tumors of neurofibromatosis type 1 patients. Neurobiol Dis 2004;16:85Y91
Surgical Ciliated Cyst of the Medial Canthal Region After the Management of a Midfacial Fracture: A Case Report Jingang An, MD, Yi Zhang, MD Abstract: Surgical ciliated cysts are an uncommon complication of maxillary surgery. We report a case of such a cyst that developed at the right medial canthal region 10 years after the surgical treatment of a severe midfacial fracture. The cyst presented as a round cystic lesion protruding through a small bony defect. The lesion was completely excised, and histologic result showed a pseudostratified ciliated epithelial lining. Key Words: Ciliated cyst, maxillofacial trauma, facial fracture, case report, surgery
S
urgical ciliated cysts were first reported in the Japanese literature, and instances have since been reported with increasing frequency by clinicians in Western countries. Generally, it is believed that surgical ciliated cysts occur at the maxilla, that they are related to maxillary sinus surgery, and that the maxillary sinus mucosa is implanted into the surgical region; therefore, the cyst is generally known as a postoperative maxillary cyst.1 Surgical ciliated cysts after maxillofacial trauma and cysts that occur at the medial canthal area are relatively rare. Herein, we report a case of a patient who developed a surgical ciliated cyst at the medial canthal region 10 years after the surgical management of a midfacial fracture.
CLINICAL REPORT A 42-year-old man presented with a slow-growing mass at the right medial canthal region. Three years ago, he had found the lesion at From the Department of Oral and Maxillofacial Surgery, School and Hospital of Stomatology, Peking University, Beijing, People_s Republic of China. Received November 28, 2013. Accepted for publication December 28, 2013. Address correspondence and reprint requests to Jingang An, MD, Department of Oral and Maxillofacial Surgery, School and Hospital of Stomatology, Peking University, 22 Zhongguancun Nandajie, Haidian District, Beijing, 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.0000000000000675
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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& Volume 25, Number 2, March 2014
recommended in asymptomatic patients, even if radiology has demonstrated optic canal narrowing.17 However, our patient complained of a sudden vision loss, and the coronal CT scan revealed the narrowing of the right optic nerve canal after the first surgery. Improvement of impaired vision after the second surgery demonstrated the efficacy of optic nerve decompression. Although TM reconstruction in craniofacial FD is popular, curative treatment plans should be made because the growth of FD does not follow a well-defined pattern. Although total excision and successive craniofacial reconstruction can be achieved, close follow-up for early diagnosis of relapse is highly recommended.
REFERENCES 1. DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848Y1864 2. Chen YR, Noordhoff MS. Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive? Plast Reconstr Surg 1990;86:835Y842 3. Chen YR, Noordhoff MS. Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive? Plast Reconstr Surg 1991;87:799Y800 4. Choi JW, Lee SW, Koh KS. Correction of proptosis and zygomaticomaxillary asymmetry using orbital wall decompression and zygoma reduction in craniofacial fibrous dysplasia. J Craniofac Surg 2009;20:326Y330 5. Mendelsohn DB, Hertzanu Y, Cohen M, et al. Computed tomography of craniofacial fibrous dysplasia. J Comput Assist Tomogr 1984;8:1062Y1065 6. Chen M,Yang C, Fang B, et al. Treatment of hemimandibular fibrous dysplasia with radical excision and immediate reconstruction with free double costochondral graft. J Oral Maxillofac Surg 2010;68:2000Y2004 7. Valentini V, Cassoni A, Marianetti TM, et al. Craniomaxillofacial fibrous dysplasia : conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg 2009;123:653Y660 8. Boni P, Ferri A, Corradi D, et al. Fibro-osseous dysplasia localized to the zygomatic arch: case report. J Craniomaxillofac Surg 2011;39:138Y140 9. Janecka IP. New reconstructive technologies in skull base surgery: role of titanium mesh and porous polyethylene. Arch Otolaryngol Head Neck Surg 2000;126:396Y401 10. Assaf AT, Benecke AW, Riecke B, et al. Craniofacial fibrous dysplasia (CFD) of the maxilla in an 11-year old boy: a case report. J Craniomaxillofac Surg 2012;40:788Y792 11. Ozek C, Gundogan H, Bilkay U, et al. Craniomaxillofacial fibrous dysplasia. J Craniofac Surg 2002;13:382Y389 12. Sakata T, Takahashi K, Kang Y, et al. Repeated surgical reduction of the mandible over a 19-year period due to fibrous dysplasia: a case report. Asian J Oral Maxillofac Surg 2010;22:33Y36 13. Sadeghi SM, Hosseini SN. Spontaneous conversion of fibrous dysplasia into osteosarcoma. J Craniofac Surg 2011;22:959Y961 14. Wei Y, Jiang S, Cen Y. Fibrous dysplasia of skull. J Craniofac Surg 2010;21:538Y542 15. Eldaly A, Nour YA, Ibrahim AA, et al. Radical resection of fibrous dysplasia involving the anterior cranial base. Neurosurg Q 2012;22:126Y132 16. Cheng J, Wang Y, Yu H, et al. An epidemiological and clinical analysis of craniomaxillofacial fibrous dysplasia in a Chinese population. Orphanet J Rare Dis 2012;7:80 17. Schreiber A, Villaret AB, Maroldi R, et al. Fibrous dysplasia of the sinonasal tract and adjacent skull base. Curr Opin Otolaryngol Head Neck Surg 2012;20:45Y52
Brief Clinical Studies
Study of Malignant Peripheral Nerve Sheath Tumor in Cerebellopontine Angle WenMing Hong, MSc,* HongWei Cheng, MD,* XiaoJie Wang, MD,Þ XiaoPeng Hu, MD,þ ChunGuo Feng, BS* Abstract: Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNSTwas made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas. Key Words: Malignant peripheral nerve sheath tumor (MPNST), neurofibrosarcoma, soft tissue sarcoma, cerebellopontine angle (CPA), treatment
T
he term malignant peripheral nerve sheath tumor (MPNST) was coined by the World Health Organization to represent neoplasms, which were previously recognized by terms such as ‘‘malignant neurilemmoma,’’ ‘‘neurofibrosarcoma,’’ ‘‘neurogenic sarcoma,’’ and ‘‘malignant schwannoma.’’ Malignant peripheral nerve sheath tumor with an incidence of 0.001% and a peak incidence in the seventh decade of life in the general population is a rare soft tissue sarcoma arising from the neoplastic transformation of Schwann cells.1,2 Malignant peripheral nerve sheath tumor may arise from any peripheral nerve, and the primary symptoms depend on its anatomic location. Some study of MPNST reported that the largest numbers of tumors were seen in the shoulder, thigh, and pelvis, while with less frequent of occurrence in the thorax, abdomen, hand, forearm, and foot.3 It is evident from the current literature that the intracranial is seldom affected: to our knowledge, there is only 1 published clinical report in cerebellopontine angle (CPA) involvement by MPNST.
From the Departments of *Neurosurgery, †Pathology, and ‡Radiology, First Affiliated Hospital of AnHui Medical University, Hefei, AnHui, China. Received September 21, 2013. Accepted for publication November 27, 2013. Address correspondence and reprint requests to HongWei Cheng, MD, Department of Neurosurgery, First Affiliated Hospital of AnHui Medical University, No. 218 JiXi Rd, Hefei, AnHui 230032, China; E-mail: [email protected] HW Cheng and WM Hong contributed equally to this work. The authors report no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000622
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
699
The Journal of Craniofacial Surgery
Brief Clinical Studies
FIGURE 1. Preoperative cranial MRI scan showing a 3.0 2.0 2.0-cm mass with mixed T1 and T2 signals in the left CPA, which the area of necrotic could be seen and no surrounding edema (A). The mass had heterogeneous enhancement in T1-weighted images enhanced with gadolinium. The fourth ventricle and pons were compressed by the tumor (B).
The following description will report the rare case of MPNST in CPA and illustrated the difficulties in the diagnosis and treatment.
CLINICAL REPORT A 25-year-old man was admitted to our hospital with dizziness for 2 months, left facial numbness, and tinnitus for 10 days. The patient’s medical history and his family history were insignificant. In physical and neurological examination, left superficial facial hypoesthesia and hearing impairment were found. The routine biochemical examination was normal. The brain magnetic resonance imaging (MRI) scan showed a 3.0 2.0 2.0-cm mass with mixed T1 and T2 signals, which the area of necrotic could be seen and no edema surrounded. The mass was enhancement heterogeneous in T1-weighted images enhanced with gadolinium. The fourth ventricle and pons were compressed by the tumor (Figs. 1A, B). The x-ray of internal auditory canal found no bone destruction, the diameter of left internal auditory was 0.4 cm, and the right was 0.3 cm. The left brainstem auditory-evoked potential was abnormal. After the relevant examination was complete, the patient underwent retrosigmoid approach surgery under anesthesia. The tumor was light red, soft material with rich blood supply, which has tight adhesion with brainstem, facial nerve, and trigeminal nerve. The tumor was removed totally under the microscope. The postoperative pathology (Fig. 2) was MPNST, and the immunohistochemistry was vimentin (+), S-100 (+) (Fig. 3), glial fibrillary acidic protein (+), individual cells epithelial membrane antigen (+), cytokeratin (+), synaptophysin (+); the positive rate of Kit was 20%. Teniposide chemotherapy for 3 days and gamma radiotherapy twice were given postoperatively. During 2 years’ follow-up, the patient’s dizziness and left facial numbness disappeared; auditory sense improved.
DISCUSSION Malignant peripheral nerve sheath tumor derived from Schwann cells, fibroblasts, and supporting cells known as perineural cells. The incidence of this rare tumor is approximately 0.001%. Malignant
FIGURE 2. Photomicrograph showing the tumor was constituted by highly heterogeneous short spindle cells and nuclear division, and hence necrosis is easily seen; there are thick-walled blood vessels in the interstitial (hematoxylin-eosin stain, original magnification 200).
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& Volume 25, Number 2, March 2014
FIGURE 3. Immunohistochemistry showing some cells were positive (S-100 stain, original magnification 200).
peripheral nerve sheath tumors usually arise from peripheral nerves or somatic soft tissues.4 They constitute 5% of all sarcomas, and 70% are associated with neurofibromatosis type 1. Malignant peripheral nerve sheath tumors may involve proximal portions of major nerve trunks of the neck, extremities, retroperitoneum, mediastinum, and viscera. Since the first report of MPNST of Gasserian ganglion by Hedeman et al5 in 1978, the reports of MPNST involving the intracranial nerve are still very rare, no more than 32 cases, and most of them are clinical reports. The tumor of CPA is even rarer; only 1 case was reported by Melliou et al6 in 2006. The clinical signs depend on the location and the involved nerve. There is a high incidence of corneal reflex loss in the MPNST of the trigeminal nerve.7 Malignant peripheral nerve sheath tumors are highly malignant and grow rapidly along nerves with infiltration of surrounding tissue and develop hematogenous metastasis. In our case, the patient presented with dizziness, left facial numbness, tinnitus, left superficial facial hypesthesia, and hearing impairment, and the MRI scan showed the fourth ventricle and pons were compressed by the tumor. His clinical symptoms were associated with the involved nerves. Also, his clinical symptoms were very similar with acoustic neurilemmoma, but the MRI scan did not coincide with this common tumor. The possible diagnosis of MPNST was considered, and the wrong diagnosis was avoided. Magnetic resonance imaging is mostly used for MPNST, showing when present signs of malignancy-like tumor progression or multiple enhancing intradural lesions.8 The imaging findings correspond to heterogeneous enhancement, irregularity of contour, and necrosis in some cases. Adjacent structure compression and local invasion are also noted. Gadolinium enhancement has occurred in all cases to variable degrees.9 However, MPNST cannot be distinguished from other sarcomas by MRI and can be misleading when the tumor exhibits signs of benign lesion. In our case, necrotic could been see in the MRI scan, and the possibility of the tumor malignancy had been considered. The computed tomography scan set to a bone window can show invasion of bony structures and increase chance of detecting marginal erosion and that may help the diagnosis of MPNST, but the erosion of the bone was not been found in our case. The histological features of MPNSTs are those of highly cellular, spindle-cell neoplasm resembling a soft tissue sarcoma, but with differentiation toward elements of the nerve sheath, Schwann cell, and perineural cell.10 Frequent mitoses and focal necrosis are typical. Cytological features also vary and may include fibroblastic, schwannian, epithelioid, clear cell, or frankly anaplastic-pleomorphic cells. Rarely are heterologous mesenchymal or epithelial elements present. In our case, the tumor can include heterologous mesenchymal and epithelial elements. Such atypical components show hypercellularity, an increased nuclear-to-cytoplasmic ratio, cytological atypia, and increased mitotic activity. The diagnosis of MPNST is very difficult, and the spectrum of differential diagnosis includes schwannoma, paraganglioma, hemangioma, ependymoma, cavernous angioma, angiolipoma, solitary fibrous tumor, lymphoma, hemangiopericytoma, hemangioendothelioma, and * 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 2, March 2014
the rarely occurring intracranial sarcomas such as fibrosarcoma and synovial sarcoma. So far, there are no direct signs of clinical symptoms, and imaging could aid in the diagnosis of this rare tumor; the postoperative pathology is the only way. The treatment of choice is surgery, but postoperative radiotherapy and chemotherapy are part of adjunctive therapy.11 Gross total resection of the tumor is the most important therapeutic goal. Wide excision or amputation may be required in some cases. When radical tumor removal is not possible, excision combined with high-dose radiation and chemical therapy seems to be the best alternative treatment. In our case, the patient underwent once of chemotherapy and twice of gamma radiotherapy. During the 2 years’ follow-up, the patient is still alive and without any complication. The survival rate is very low. In the review, 66% of patients died, whereas 19% are alive with or without complications.12 In general, grade II tumors typically survive more than 5 years, and those with grade III tumors survive 2 to 3 years. The prognosis of patients with World Health Organization grade IV tumors depends largely on whether effective treatment regimens are available. For MPNST, survival rate was better in patient treated with adjuvant chemotherapy and radiotherapy. Our patient had an uneventful postoperative course and had remained functional for 2 years, and we attribute this favorable outcome to the complete resection of tumor and the chemotherapy and radiotherapy he received after.
CONCLUSIONS Malignant peripheral nerve sheath tumor is a rare and aggressive tumor. Compared with other tumors, there is no typical representation in symptoms, signs, and image. The pathology is the only way to diagnose this kind of tumor. Because it is aggressive, we should consider the possibility of an MPNSTwhen a tumor occurs in some special location, such as CPA in our case. A need for a multidisciplinary approach with the surgery of gross total resection, high-dose of chemotherapy, and radiotherapy is anticipated in the management of MPNST.
ACKNOWLEDGMENTS The authors thank the patient and his family members who agreed to report this rare disease to help more patients. They also thank the doctors in the Departments of Pathology and Radiology for providing clear pictures and also the staff of Journal of Craniofacial Surgery for reviewing the article. REFERENCES 1. Hajdu SI. Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 1993;72:3549Y3552 2. Than KD, Ghori AK, Wang AC, et al. Metastatic malignant peripheral nerve sheath tumor of the cauda equine. J Clin Neurosci. 2011;18:844Y846 3. Cashen DV, Parisien RC, Raskin K, et al. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res 2004;426:69Y73 4. Aydin MD, Yildirim U, Gundogdu C, et al. Malignant peripheral nerve sheath tumor of the orbit: case report and literature review. Skull Base 2004;14:109Y113 5. Hedeman LS, Lewinsky BS, Lochridge GK, et al. Primary malignant schwannoma of the Gasserian ganglion. Report of two cases. J Neurosurg 1978;48:279Y283 6. Melliou A, Karamouzis J, Helis L, et al. Malignant peripheral nerve-sheath tumor of the left cerebello-pontine angle: case report. J BUON 2006;11:367Y368 7. Lee JH, Lee HK, Choi CG, et al. Malignant peripheral nerve sheath tumor in the parapharyngeal space: tumor spread through the eustachian tube. AJNR Am J Neuroradiol 2001;22:748Y750 8. Ziadi A, Saliba I. Malignant peripheral nerve sheath tumor of intracranial nerve: a case series review. Auris Nasus Larynx 2010;37:539Y545
Brief Clinical Studies
9. Auclair PL, Langloss JM, Weiss SW, et al. Sarcomas and sarcomatoid neoplasms of the major salivary gland regions. A clinicopathologic and immunohistochemical study of 67 cases and review of the literature. Cancer 1986;58:1305Y1315 10. Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg 2009;249:1014Y1022 11. Chao BH, Stogner-Underwood KA, Kiev J, et al. Intrathoracic malignant peripheral nerve sheath tumor in neurofibromatosis 1. J Clin Oncol 2008;26:2216Y2218 12. Frahm S, Mautner VF, Brems H, et al. Genetic and phenotypic characterization of tumor cells derived from malignant peripheral nerve sheath tumors of neurofibromatosis type 1 patients. Neurobiol Dis 2004;16:85Y91
Surgical Ciliated Cyst of the Medial Canthal Region After the Management of a Midfacial Fracture: A Case Report Jingang An, MD, Yi Zhang, MD Abstract: Surgical ciliated cysts are an uncommon complication of maxillary surgery. We report a case of such a cyst that developed at the right medial canthal region 10 years after the surgical treatment of a severe midfacial fracture. The cyst presented as a round cystic lesion protruding through a small bony defect. The lesion was completely excised, and histologic result showed a pseudostratified ciliated epithelial lining. Key Words: Ciliated cyst, maxillofacial trauma, facial fracture, case report, surgery
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urgical ciliated cysts were first reported in the Japanese literature, and instances have since been reported with increasing frequency by clinicians in Western countries. Generally, it is believed that surgical ciliated cysts occur at the maxilla, that they are related to maxillary sinus surgery, and that the maxillary sinus mucosa is implanted into the surgical region; therefore, the cyst is generally known as a postoperative maxillary cyst.1 Surgical ciliated cysts after maxillofacial trauma and cysts that occur at the medial canthal area are relatively rare. Herein, we report a case of a patient who developed a surgical ciliated cyst at the medial canthal region 10 years after the surgical management of a midfacial fracture.
CLINICAL REPORT A 42-year-old man presented with a slow-growing mass at the right medial canthal region. Three years ago, he had found the lesion at From the Department of Oral and Maxillofacial Surgery, School and Hospital of Stomatology, Peking University, Beijing, People_s Republic of China. Received November 28, 2013. Accepted for publication December 28, 2013. Address correspondence and reprint requests to Jingang An, MD, Department of Oral and Maxillofacial Surgery, School and Hospital of Stomatology, Peking University, 22 Zhongguancun Nandajie, Haidian District, Beijing, 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.0000000000000675
* 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
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