- Email: [email protected]
Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension
G Model
ARTICLE IN PRESS
ANORL-359; No. of Pages 3
European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2014) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case report
Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension H. Touil a,∗ , S. Briki a , F. Karray a , I. Bahri b a b
Service de stomatologie et de chirurgie maxillo-faciale et esthétique, CHU Habib Bourguiba, route de l’Ain, 3000 Sfax, Tunisia Service de cytologie pathologique, CHU Habib Bourguiba, route de l’Ain, 3000 Sfax, Tunisia
a r t i c l e
i n f o
Keywords: Malignant peripheral nerve sheath tumor Superficial cervical plexus Immunohistochemistry Surgery
a b s t r a c t Introduction: Cervical locations of malignant peripheral nerve sheath tumor (MPNST) are rare, at less than 1% of malignant tumors of this region. Case report: A 53-year-old woman presented with a lateral cervical swelling involving the parotid region. Histology was in favor of MPNST. Adjuvant radiotherapy was indicated because of the infiltrating nature of the tumor. At 2 years’ follow-up, there was no recurrence. Discussion: Clinical diagnosis is difficult in cervical MPNST. Only histology with immunohistochemistry can establish the correct diagnosis. Treatment requires complete surgical resection and regular clinical follow-up. © 2014 Elsevier Masson SAS. All rights reserved.
1. Introduction Malignant peripheral nerve sheath tumor (MPNST), also known as malignant schwannoma and formerly as neurogenic sarcoma or neurofibrosarcoma [1,2], is a peripheral nervous system tumor developing in the nerve sheath [1,3,4], often from major nerve trunks and in the extremities [5]; head and neck locations are rare [4], at less than 10% [6]. The present report details the clinical, paraclinical and therapeutic aspects of MPNST. 2. Case report A 53-year-old woman with no particular history presented with left pretragal swelling of 1 year’s evolution with progressive increase in volume. Physical examination found a painless, firm, nodular left pretragal swelling of 3 cm long-axis adhering to the superficial plane. The covering skin was purplish. A painful left cervical line prolonged the nodular swelling (Fig. 1). The cervical lymph-node areas were uninvolved. Facial mobility was conserved. Oral examination was normal. Ultrasound found a centrally vascularized macrolobular subcutaneous hypoechogenic formation measuring 36 × 10 mm. It extended into the parotid region, in medial contact with the gland, and was then prolonged by a tubular
∗ Corresponding author. Tel.: +00216 55251 842; fax: +00216 74242 167. E-mail address: [email protected] (H. Touil).
cervical formation with non-compressible hypoechogenic contents suggestive of venous thrombosis. Cervical MRI found a rounded superficial upper mandibular mass of low intensity on T1 and high intensity on T2-weighted sequences, with strong gadolinium uptake (Fig. 2), associated with an adjacent left cervical thrombotic vein (Fig. 3). A diagnosis of parotid tumor was suggested, indicating surgical resection. Intra-operative exploration found a tumoral formation in continuity with the superficial cervical plexus branches and infiltrating the parotid gland and covering skin. Frozen section biopsy was in favor of a malignant tumor of sarcomatous aspect, with involvement of the surgical margins. Complementary resection was performed, with free skin flap reconstruction, with healthy final surgical margins. Final histologic analysis of the specimen showed a WHO stage-IV MPNST (Fig. 4). Postoperative course was straightforward (Fig. 5). Thoraco-abdominal CT for extension assessment found no further locations. Adjuvant radiation therapy was prescribed. At 2 year’s follow-up, there had been no recurrence.
3. Discussion MPNST [1,4] is of ectomesenchymatous origin [2]. Prevalence is < 0.001% in the general population [1], and it accounts for < 5% of soft-tissue sarcomas [1,4,7]. Incidence is higher in case of history of irradiation and in type-1 neurofibromatosis (NF1) [2–5,8], where incidence is 5% [4,5] and onset is earlier, in the 3rd decade of life [8]. MPNST degenerates from well-established neurofibromsas, notably giant, multiple or extensive plexiform neurofibroma [6–8]. Clinical diagnosis is difficult due to rarity and a presentation that
http://dx.doi.org/10.1016/j.anorl.2013.11.012 1879-7296/© 2014 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Touil H, et al. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. European Annals of Otorhinolaryngology, Head and Neck diseases (2014), http://dx.doi.org/10.1016/j.anorl.2013.11.012
G Model ANORL-359; No. of Pages 3 2
ARTICLE IN PRESS H. Touil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2014) xxx–xxx
Fig. 3. Coronal cervical MRI, showing cervical prolongation of the tumor.
Fig. 1. Pre-operative aspect, showing a mass in the parotid region, with skin infiltration, prolonged by a lateral cervical line.
is often non-specific and may mimic adenopathy [3,4]. Symptomatology is generally related to nerve compression [3]. This tumor can manifest by pain [4] or rapid change in the neurofibroma’s characteristics [8]. Imaging guides diagnosis and assesses extension [4,7]. MRI is the examination of choice [4,9], and generally finds an irregular multilobular lesion with heterogeneous contrast uptake [7]. Definitive diagnosis is histological, making fine-needle aspiration and biopsy especially useful [10]. In the present case, the
Fig. 2. T2-weighted contrast-enhanced axial cervical MRI, showing mass with strong uptake.
parotid location suggested parotid origin, which is why we opted for histology following total resection. Diagnosis remains difficult, however, due to the great heterogeneity of the tumoral tissue and similarity to other forms of sarcoma [5]. Immunohistochemistry is highly contributive [7], with positive results for S100 protein [1,3,4] and vimentin [7] specific to nerve cells. Histologically, MPNST presents as well-differentiated, anaplastic, epithelial or triton tumor [7]. The main differential diagnoses are fibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, esthesioneuroblastoma, poorly differentiated lymphoma and achromic melanoma [7]. The treatment of choice is complete surgical resection [2,8]. Margins are usually checked in frozen sections, as in the present case, both to confirm diagnosis and to check resection quality. Adjuvant therapies are not codified [2,8]. Histologically, MPNST is radioresistant and chemoresistant [2]. Nevertheless, postoperative radiotherapy has been reported as providing benefit in high-grade or large or deep MPNST or in case of margin invasion, as in the
Fig. 4. Histologic cross-section, positive for S100 protein (HE stain, × 200).
Please cite this article in press as: Touil H, et al. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. European Annals of Otorhinolaryngology, Head and Neck diseases (2014), http://dx.doi.org/10.1016/j.anorl.2013.11.012
G Model ANORL-359; No. of Pages 3
ARTICLE IN PRESS H. Touil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2014) xxx–xxx
3
4. Conclusion Diagnosis of MPNST is founded on a range of histologic and notably immunohistochemical evidence. Surgery is the reference attitude, aiming at resection with healthy margins to avoid recurrence. Disclosure of interest The authors have not supplied their declaration of conflict of interest. References
Fig. 5. Clinical aspect at 3 months’ follow-up.
present case [4,9]. The contribution of chemotherapy is still under assessment [7]. MPNST is often aggressive [2], with a high rate of metastasis and local recurrence [1,2,8]. Dissemination is mainly hematogenic, with metastases to lung, bone and liver [4,5]. Regular prolonged, basically clinical, surveillance is thus mandatory [5]. Prognosis essentially depends on resection quality, histologic stage, tumor size [3,4] and location [4,10], necrosis [7], and association with NF1 [3]. Five-year survival is 20–50% [3,8].
[1] Patnaik A, Sekhar Mishra S, Senapati SB, et al. Primary intraosseous malignant peripheral nerve sheath tumor of spine with a giant paraspinal and retrospinal subcutaneous extension. Surg Neurol Int 2012;3:157. [2] Ali NS, Junaid M, Aftab K. Malignant Peripheral Nerve Sheath Tumour of Maxilla. J Coll Physician Surg Pak 2011;21:420–2. [3] Moumine M, Thiery G, Harroudi T, et al. Schwannome malin primitif du rameaux buccal supérieur du nerf facial. Ann Chir Plast Esthet 2012;57:308–11. [4] Tekaya R, Hamdi W, Azzouz D, et al. Névralgie cervicobrachiale révélatrice d’un neurofibrosarcome (MPNST) cervical. Rev Neurol (Paris) 2008;164:82–6. [5] Younes M, Simon E. Schwannome du nerf mandibulaire. Rev Stomatol Chir Maxillofac 2012;113:465–7. [6] Minovi A, Basten O. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007;29:439–45. [7] Kar M, Suryanarayana SV, Shukla NK. Malignant peripheral nerve sheath tumors (MPNST): clinicopathological study and treatment outcome of twentyfour cases. World J Surg Oncol 2006;4:55. [8] Leroy K, Dumas V, Martin-Garcia N, et al. Malignant peripheral nerve sheath tumors associated with neurofibromatosis Type 1: a clinicopathologic and molecular study of 17 patients. Arch Dermatol 2001;137:908–13. [9] Prem S, Gangothria S, Reddyb KS, et al. Malignant peripheral nerve sheath tumor of the mandible: a case report and review of literature. J Neurol Res 2011;1:219–22. [10] Benevello C, Sommacale D, Palladino E, et al. A rare case of malignant schwannoma of the brachial plexus. World J Surg Proced 2013;28:3.
Please cite this article in press as: Touil H, et al. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. European Annals of Otorhinolaryngology, Head and Neck diseases (2014), http://dx.doi.org/10.1016/j.anorl.2013.11.012
ARTICLE IN PRESS
ANORL-359; No. of Pages 3
European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2014) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case report
Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension H. Touil a,∗ , S. Briki a , F. Karray a , I. Bahri b a b
Service de stomatologie et de chirurgie maxillo-faciale et esthétique, CHU Habib Bourguiba, route de l’Ain, 3000 Sfax, Tunisia Service de cytologie pathologique, CHU Habib Bourguiba, route de l’Ain, 3000 Sfax, Tunisia
a r t i c l e
i n f o
Keywords: Malignant peripheral nerve sheath tumor Superficial cervical plexus Immunohistochemistry Surgery
a b s t r a c t Introduction: Cervical locations of malignant peripheral nerve sheath tumor (MPNST) are rare, at less than 1% of malignant tumors of this region. Case report: A 53-year-old woman presented with a lateral cervical swelling involving the parotid region. Histology was in favor of MPNST. Adjuvant radiotherapy was indicated because of the infiltrating nature of the tumor. At 2 years’ follow-up, there was no recurrence. Discussion: Clinical diagnosis is difficult in cervical MPNST. Only histology with immunohistochemistry can establish the correct diagnosis. Treatment requires complete surgical resection and regular clinical follow-up. © 2014 Elsevier Masson SAS. All rights reserved.
1. Introduction Malignant peripheral nerve sheath tumor (MPNST), also known as malignant schwannoma and formerly as neurogenic sarcoma or neurofibrosarcoma [1,2], is a peripheral nervous system tumor developing in the nerve sheath [1,3,4], often from major nerve trunks and in the extremities [5]; head and neck locations are rare [4], at less than 10% [6]. The present report details the clinical, paraclinical and therapeutic aspects of MPNST. 2. Case report A 53-year-old woman with no particular history presented with left pretragal swelling of 1 year’s evolution with progressive increase in volume. Physical examination found a painless, firm, nodular left pretragal swelling of 3 cm long-axis adhering to the superficial plane. The covering skin was purplish. A painful left cervical line prolonged the nodular swelling (Fig. 1). The cervical lymph-node areas were uninvolved. Facial mobility was conserved. Oral examination was normal. Ultrasound found a centrally vascularized macrolobular subcutaneous hypoechogenic formation measuring 36 × 10 mm. It extended into the parotid region, in medial contact with the gland, and was then prolonged by a tubular
∗ Corresponding author. Tel.: +00216 55251 842; fax: +00216 74242 167. E-mail address: [email protected] (H. Touil).
cervical formation with non-compressible hypoechogenic contents suggestive of venous thrombosis. Cervical MRI found a rounded superficial upper mandibular mass of low intensity on T1 and high intensity on T2-weighted sequences, with strong gadolinium uptake (Fig. 2), associated with an adjacent left cervical thrombotic vein (Fig. 3). A diagnosis of parotid tumor was suggested, indicating surgical resection. Intra-operative exploration found a tumoral formation in continuity with the superficial cervical plexus branches and infiltrating the parotid gland and covering skin. Frozen section biopsy was in favor of a malignant tumor of sarcomatous aspect, with involvement of the surgical margins. Complementary resection was performed, with free skin flap reconstruction, with healthy final surgical margins. Final histologic analysis of the specimen showed a WHO stage-IV MPNST (Fig. 4). Postoperative course was straightforward (Fig. 5). Thoraco-abdominal CT for extension assessment found no further locations. Adjuvant radiation therapy was prescribed. At 2 year’s follow-up, there had been no recurrence.
3. Discussion MPNST [1,4] is of ectomesenchymatous origin [2]. Prevalence is < 0.001% in the general population [1], and it accounts for < 5% of soft-tissue sarcomas [1,4,7]. Incidence is higher in case of history of irradiation and in type-1 neurofibromatosis (NF1) [2–5,8], where incidence is 5% [4,5] and onset is earlier, in the 3rd decade of life [8]. MPNST degenerates from well-established neurofibromsas, notably giant, multiple or extensive plexiform neurofibroma [6–8]. Clinical diagnosis is difficult due to rarity and a presentation that
http://dx.doi.org/10.1016/j.anorl.2013.11.012 1879-7296/© 2014 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Touil H, et al. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. European Annals of Otorhinolaryngology, Head and Neck diseases (2014), http://dx.doi.org/10.1016/j.anorl.2013.11.012
G Model ANORL-359; No. of Pages 3 2
ARTICLE IN PRESS H. Touil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2014) xxx–xxx
Fig. 3. Coronal cervical MRI, showing cervical prolongation of the tumor.
Fig. 1. Pre-operative aspect, showing a mass in the parotid region, with skin infiltration, prolonged by a lateral cervical line.
is often non-specific and may mimic adenopathy [3,4]. Symptomatology is generally related to nerve compression [3]. This tumor can manifest by pain [4] or rapid change in the neurofibroma’s characteristics [8]. Imaging guides diagnosis and assesses extension [4,7]. MRI is the examination of choice [4,9], and generally finds an irregular multilobular lesion with heterogeneous contrast uptake [7]. Definitive diagnosis is histological, making fine-needle aspiration and biopsy especially useful [10]. In the present case, the
Fig. 2. T2-weighted contrast-enhanced axial cervical MRI, showing mass with strong uptake.
parotid location suggested parotid origin, which is why we opted for histology following total resection. Diagnosis remains difficult, however, due to the great heterogeneity of the tumoral tissue and similarity to other forms of sarcoma [5]. Immunohistochemistry is highly contributive [7], with positive results for S100 protein [1,3,4] and vimentin [7] specific to nerve cells. Histologically, MPNST presents as well-differentiated, anaplastic, epithelial or triton tumor [7]. The main differential diagnoses are fibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, esthesioneuroblastoma, poorly differentiated lymphoma and achromic melanoma [7]. The treatment of choice is complete surgical resection [2,8]. Margins are usually checked in frozen sections, as in the present case, both to confirm diagnosis and to check resection quality. Adjuvant therapies are not codified [2,8]. Histologically, MPNST is radioresistant and chemoresistant [2]. Nevertheless, postoperative radiotherapy has been reported as providing benefit in high-grade or large or deep MPNST or in case of margin invasion, as in the
Fig. 4. Histologic cross-section, positive for S100 protein (HE stain, × 200).
Please cite this article in press as: Touil H, et al. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. European Annals of Otorhinolaryngology, Head and Neck diseases (2014), http://dx.doi.org/10.1016/j.anorl.2013.11.012
G Model ANORL-359; No. of Pages 3
ARTICLE IN PRESS H. Touil et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2014) xxx–xxx
3
4. Conclusion Diagnosis of MPNST is founded on a range of histologic and notably immunohistochemical evidence. Surgery is the reference attitude, aiming at resection with healthy margins to avoid recurrence. Disclosure of interest The authors have not supplied their declaration of conflict of interest. References
Fig. 5. Clinical aspect at 3 months’ follow-up.
present case [4,9]. The contribution of chemotherapy is still under assessment [7]. MPNST is often aggressive [2], with a high rate of metastasis and local recurrence [1,2,8]. Dissemination is mainly hematogenic, with metastases to lung, bone and liver [4,5]. Regular prolonged, basically clinical, surveillance is thus mandatory [5]. Prognosis essentially depends on resection quality, histologic stage, tumor size [3,4] and location [4,10], necrosis [7], and association with NF1 [3]. Five-year survival is 20–50% [3,8].
[1] Patnaik A, Sekhar Mishra S, Senapati SB, et al. Primary intraosseous malignant peripheral nerve sheath tumor of spine with a giant paraspinal and retrospinal subcutaneous extension. Surg Neurol Int 2012;3:157. [2] Ali NS, Junaid M, Aftab K. Malignant Peripheral Nerve Sheath Tumour of Maxilla. J Coll Physician Surg Pak 2011;21:420–2. [3] Moumine M, Thiery G, Harroudi T, et al. Schwannome malin primitif du rameaux buccal supérieur du nerf facial. Ann Chir Plast Esthet 2012;57:308–11. [4] Tekaya R, Hamdi W, Azzouz D, et al. Névralgie cervicobrachiale révélatrice d’un neurofibrosarcome (MPNST) cervical. Rev Neurol (Paris) 2008;164:82–6. [5] Younes M, Simon E. Schwannome du nerf mandibulaire. Rev Stomatol Chir Maxillofac 2012;113:465–7. [6] Minovi A, Basten O. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007;29:439–45. [7] Kar M, Suryanarayana SV, Shukla NK. Malignant peripheral nerve sheath tumors (MPNST): clinicopathological study and treatment outcome of twentyfour cases. World J Surg Oncol 2006;4:55. [8] Leroy K, Dumas V, Martin-Garcia N, et al. Malignant peripheral nerve sheath tumors associated with neurofibromatosis Type 1: a clinicopathologic and molecular study of 17 patients. Arch Dermatol 2001;137:908–13. [9] Prem S, Gangothria S, Reddyb KS, et al. Malignant peripheral nerve sheath tumor of the mandible: a case report and review of literature. J Neurol Res 2011;1:219–22. [10] Benevello C, Sommacale D, Palladino E, et al. A rare case of malignant schwannoma of the brachial plexus. World J Surg Proced 2013;28:3.
Please cite this article in press as: Touil H, et al. Malignant peripheral nerve sheath tumor of the superficial cervical plexus with parotid extension. European Annals of Otorhinolaryngology, Head and Neck diseases (2014), http://dx.doi.org/10.1016/j.anorl.2013.11.012