Brief Clinical Studies
The Journal of Craniofacial Surgery • Volume 25, Number 5, September 2014
9. Dicerbo M. Blood flow determined by laser Doppler flowmetry in orthognathic surgery. Am J Orthod Dentofacial Orthop 1993;103:488–489 10. Geylikman YB, Artun J, Leroux BG, et al. Effects of Le Fort I osteotomy on human gingival and pulpal circulation. Int J Oral Maxillofac Surg 1995;24:255–260 11. Emshoff R, Kranewitter R, Norer B. Effect of Le Fort I osteotomy on maxillary tooth-type related pulpal blood flow characteristics. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:88–90 12. Emshoff R, Kranewitter R, Gerhard S, et al. Effect of segmental Le Fort I osteotomy on maxillary tooth-type related pulpal blood flow characteristics. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:749–752 13. Justus T, Chang BL, Bloomquist D, et al. Human gingival and pulpal blood flow during healing after Le Fort I osteotomy. J Oral Maxillofac Surg 2001;59:2–7 14. McDonald F, Pitt Ford TR. Blood flow changes in permanent maxillary canines during retraction. Eur J Orthod 1994;16:1–9 15. Tonder KJ. Effect of vasodilating drugs on external carotid and pulpal blood flow in dogs: “stealing” of dental perfusion pressure. Acta Physiol Scand 1976;97:75–87 16. Bell WH. Surgical-orthodontic treatment of interincisal diastemas. Am J Orthod 1970;57:158–163 17. Buckley JG, Jones ML, Hill M, et al. An evaluation of the changes in maxillary pulpal blood flow associated with orthognathic surgery. Br J Orthod 1999;26:39–45 18. Ellingsen RH, Artun J. Pulpal response to orthognathic surgery: a long-term radiographic study. Am J Orthod Dentofacial Orthop 1993;103:338 19. Vedtofte P. Pulp canal obliteration after Le Fort I osteotomy. Endod Dent Traumatol 1989;5:274 20. Meyer MW, Cavanaugh GD. Blood flow changes after orthognathic surgery: maxillary and mandibular subapical osteotomy. J Oral Surg 1976;34:495–501 21. Qeujada JG, Kawamura H, Finn RA, et al. Wound healing associated with segmental total maxillary osteotomy. J Oral Maxillofac Surg 1986;44:366–377 22. Sugg GR, Fonseca RJ, Leeb IJ, et al. Early pulp changes after anterior maxillary osteotomy. J Oral Surg 1981;39:14–20 23. Ramsay DS, Artun J, Martinen SS. Reliability of pulpal blood flow measurements utilizing laser Doppler flowmetry. J Dent Res 1991;70:1427–1430 24. Bell WH. Revascularization and bone healing after anterior maxillary osteotomy: a study using adult rhesus monkeys. J Oral Surg 1969;27:249–255 25. Harada K, Sato M, Omura K. Blood-flow and neurosensory changes in the maxillary dental pulp after differing Le Fort I osteotomies. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97:12–17 26. Zisser G, Gattinger B. Histologic investigation of pulpal changes following maxillary and mandibular alveolar osteotomies in the dog. J Oral Maxillofac Surg 1982;40:332–341 27. Robinson PP. Reinnervation of teeth after segmental osteotomy in the cat. Int J Oral Maxillofac Surg 1986;15:152–159 28. Theisen FC, Guerney LH. Postoperative sequelae after anterior segmental osteotomies. Oral Surg Oral Med Oral Pathol 1971;41:139–151 29. Pepersack WJ. Tooth vitality after alveolar segmental osteotomy. J Oral Maxillofac Surg 1973;1:85–91 30. Vedtofte P, Nattestadt A. Pulp sensibility and pulp necrosis after Le Fort I osteotomy. J Craniomaxillofac Surg 1989;17:167–171 31. Bell WH. Immediate surgical repositioning of one and two tooth dento-osseous segments. Int J Oral Surg 1973;2:265–272 32. Gopikrishna V, Tinagupta K, Kandaswamy K. Comparison of electrical, thermal and pulse oximetry methods for assessing pulp vitality in recently traumatized teeth. J Endod 2007;33:531–535 33. Sailus J, Trowbridge H, Greco M, et al. Sensitivity of teeth subjected to orthodontic forces. J Dent Res 1987;66:556 34. Ingolfsson AER, Tronstad L, Riva CE. Reliability of laser Doppler flowmetry in testing vitality of human teeth. Endod Dent Traumatol 1994;10:185–187
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35. Odor TM, Pitt Ford TR, McDonald F. Effect of wavelength and bandwidth on the clinical reliability of laser Doppler recordings. Endod Dent Traumatol 1996a;12:9–15 36. Hartmann A, Azerad J, Boucher Y. Environmental effects on laser Doppler pulpal blood-flow measurements in man. Arch Oral Biol 1996;41:333–339 37. Soo-ampon S, Vongsavan N, Soo-ampon M, et al. The sources of laser Doppler blood-flow signals recorded from human teeth. Arch Oral Biol 2003;48:353–360 38. Akpinar KE, Er K, Polat S, et al. Effect of gingiva on laser Doppler pulpal blood flow measurements. J Endod 2004;30:138–140 39. Odor TM, Watson TF, Pitt Ford TR, et al. Pattern of transmission of laser light in teeth. Int Endod J 1996b;29:228–234 40. Okabe E, Todoki K, Ito H. Direct pharmacological action of vasoactive substances on pulpal blood flow: an analysis and critique. J Endod 1989;15:473–477 41. Ikawa M, Komatsu H, Ikawa K, et al. Age-related changes in the human pulpal blood flow measured by laser Doppler flowmetry. Dent Traumatol 2003;19:36–40 42. Vongsavan N, Matthews B. Experiments on extracted teeth into the validity of using laser Doppler techniques for recording pulpal blood flow. Arch Oral Biol 1993;38:431–439 43. Anderson KK, Vanarsdall RL, Kim S. Measurement of pulpal blood flow in humans using laser Doppler flowmetry: a technique allowing stability and repeatability of pulpal blood flow measurement during surgical manipulations. Int J Adult Orthod Orthognat Surg 1995;10:247–254 44. Ingle JI, Heithersay GS, Hartwell GR. Endodontic diagnostic procedures. In: Ingle JI, Bakland LK, eds. Endodontics, 5th edition. London, UK: BC Decker Inc, 2002:203–217 45. Olgart LM, Edwall B, Gazelius B. Neurogenic mediators in control of pulpal blood flow. J Endod 1989;15:409–412 46. Matthews B, Vongsavan N. Advantages and limitations of laser Doppler flow meters. Int Endod J 1993;26:9–10
Malignant Peripheral Nerve Sheath Tumor of the Parotid Gland Octavian Chis, MD,* Silviu Albu, MD† Abstract: Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, usually having a poor prognosis, and only a few cases been described in the literature. In this article, we report the diagnostic and therapeutic challenges related to a new case of MPNST of the parotid. Diagnosis was made
From the *Oncology Institute “N. Chiricuta;” and †II-nd Department of Otolaryngology, “Iuliu Hatieganu” University of Medicine and Pharmacy Cluj-Napoca, Cluj-Napoca, Romania. Received December 9, 2013. Accepted for publication February 17, 2014. Address correspondence and reprint requests to Silviu Albu, MD, II-nd Department of Otolaryngology, University of Medicine and Pharmacy Cluj-Napoca, Str. Republicii nr. 18, 400015 Cluj-Napoca, Romania; 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.0000000000000938
© 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 5, September 2014
Brief Clinical Studies
based on clinical, imaging (computed tomography scan), histologic, and immunohistochemistry findings. Despite comprehensive treatment—complete surgical resection and radiotherapy—the tumor displayed a highly aggressive course.
Key Words: Parotid, malignant peripheral nerve sheath tumor, diagnosis, immunohistochemistry
FIGURE 2. Eosinophilic spindle-shaped cells and basophilic matrix (hematoxylin and eosin staining).
M
metastases. Chemotherapy consisting of doxorubicin, cisplatin, and cyclophosphamide was selected. However, the patient died of disease 2 months later.
alignant peripheral nerve sheath tumor (MPNST) refers to sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath.1–3 According to the new World Health Organization classification of soft tissue tumors, spindle cell sarcomas originating from the peripheral nerves have been defined as MPNST.1–3 It is widely recognized that MPNSTs are highly aggressive malignant tumors with a dismal prognosis. The MPNST develops within extremities of the trunk and rarely appears in the head and neck region.1–3 This study describes a patient with MPNST of the parotid gland. To the best of our knowledge, only 8 case reports of MPNST of the parotid gland have been described in the current English-language literature.4–9
CLINICAL PRESENTATION A 60-year-old woman presented with a 4-month history of rapidly developing, painful left parotid swelling. Physical examination revealed a 5 6-cm firm, hardly fixed, immobile, irregular contoured mass associated with severe spontaneous pain and tenderness. There was no facial palsy and no palpable lymph nodes. A computed tomographic scan showed a multilobulated, nonhomogeneous mass within the superficial lobe of the left parotid gland, whereas the deep lobe was normal (Fig. 1). Intraoperatively, a 6 6-cm mass was observed within the left parotid gland. The main trunk and all the branches of the facial nerve were entrapped within the tumor. A total parotidectomy with resection of the main facial nerve trunk, and a modified radical neck dissection was performed. Microscopically, the tumor was composed of spindle cells with coiled or asymmetric oval nuclei. The cells were arranged in sweeping fascicles or a spinning pattern (Fig. 2). There was significant cellular pleomorphism, with curved or twisted nuclei and prominent mitotic activity. Foci of necrosis were also present. Nerve fibers and salivary gland tissue were entrapped within the tumor (Figs. 3, 4). The resection margin was free of tumor. Nodes from the inferior pole of the parotid, posterior jugular chain, and carotid sheath were all negative for metastasis. Immunohistochemistry yielded positive for S-100 protein (Fig. 5) and vimentin; weakly positive for actin; yet negative for CD34, desmin, epithelial membrane antigen, cytokeratin AE1/AE3, and glial fibrillary acidic protein. We counted Ki67 labeling indices in 20% of the tumor cells (Fig. 6). A diagnosis of MPNST was made. The patient underwent a full course of radiation therapy. Six months later, a chest radiograph revealed multiple lesions considered as
FIGURE 1. Patient with tumor of the parotid gland—computed tomographic scan.
DISCUSSION The MPNSTs account for approximately 10% of all soft tissue sarcomas, are located on the extremities and trunk, and are seldom encountered within the head and neck region.1–3 A recent review by Imamura et al6 identified 142 cases of head and neck MPNST published within the past 23 years. The MPNST of the parotid gland is extremely rare, with only 8 cases described in the recent literature.1–9 The MPNSTs derive from Schwann cells, often developing in preexisting von Recklinghausen disease (neurofibromatosis 1-NF1) or as a solitary malignant tumor.5,6 The risk of developing MPNST seems to be 4000 times greater in patients with NF1 than in the general population.6–9 Approximately 5% of the NF1 patients develop MPNSTs, whereas 9% of the head and neck MPNSTs develop from preexisting NF1.3,5–9 Generally, MPNST is a disease of adults, and the onset age shows a significant discrepancy in the literature.1–9 However, most tumors develop in patients aged 20 to 50 years, with a mean of 45 years. The sex ratio of MPNST cases is differently estimated in the published series depending on the inclusion criteria; however, most authors agree that, in sporadic MPNST case, the sex ratio is equal3,5,6. It has been reported that, within the head and neck region, MPNST frequently develops in the neck area—40% to 60% of cases. A recent review revealed that MPNST may arise from any organ of the head and neck region.1–9 Symptoms associated with these tumors include rapidly developing and particularly painful mass and functional disorders such as trismus, dysphagia, dysarthria, and facial palsy in up to 10% of cases.2–9
FIGURE 3. Peripheral nerve branch entrapped within the tumor.
FIGURE 4. Salivary gland encased within the tumor.
© 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 5, September 2014
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FIGURE 5. Immunohistochemistry: positive staining for S-100 protein.
5. Punjabi AP, Haug RH, Chung-Park MJ, et al. Malignant peripheral nerve sheath tumor of the parotid gland: report of case. J Oral Maxillofac Surg 1996;54:765–769 6. Imamura S, Suzuki H, Koda E, et al. Malignant peripheral nerve sheath tumor of the parotid gland. Ann Otol Rhinol Laryngol 2003;112:637–643 7. Nepka C, Karadana M, Karasavvidou F, et al. Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report. Acta Cytol 2009;53:423–426 8. Gogate BP, Anand M, Deshmukh SD, et al. Malignant peripheral nerve sheath tumor of facial nerve: presenting as parotid mass. J Oral Maxillofac Pathol 2013;17:129–131 9. De Stefano A, Kulamarva G, Citraro L, et al. Malignant peripheral nerve sheath tumour (malignant epithelioid Schwannoma) of the parotid gland. Bratisl Lek Listy 2012;113:628–631 10. Wong WW, Hirose T, Scheithauer BW, et al. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998;42:351–360 11. Loree TR, North JHJr, Werness BA, et al. Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors. Otolaryngol Head Neck Surg 2000;122:667–672
FIGURE 6. Ki67 labeling of the tumor.
Microscopically, the tumor is composed of eosinophilic spindleshaped cells and basophilic extracellular matrix. Immunoreactivity with S-100 protein stands for neural differentiation. Although most cases were positive for S-100 protein, the staining intensity varied from case to case. Nevertheless, vimentin was strongly positive in all reported studies, whereas epithelial-derived antigens were negative in most of the case reports.3–6 Positive immunostaining with antigens used to spot nerve sheath differentiation such as Leu 7, myelin basic protein, and nerve cell adhesion molecule was also described.6 It was suggested that staining with p53 and high Ki67 immunoreactivity designates malignant transformation.3,6 It is generally accepted that, in MPNST, the best chance for survival was with wide radical excision or amputation when the tumor involved an extremity.6,10 In the head and neck region, because of the particular anatomy, radical surgery is not always possible. Nevertheless, recurrence is common. If radical tumor removal is not possible, excision combined with high-dose radiation therapy seems to be the best alternative.10,11 Adjuvant chemotherapy should be considered in those MPNST patients who had it in relation with NF1 or at a site of prior radiation.3,8,10 Prognosis in MPNST of the head and neck is generally poorer than that of MPNST developing in the extremities.11 Patients with NF1 had a 5-year survival of 23%, compared with 47% in patients without such disease. The mean survival period was 42 months, and 21 of the 38 patients with head and neck MPNSTs did not survive.5,9 Prognostic factors associated with survival include tumor size, location of the tumor, and surgical margins.11 Invasion of the skull base, carotid artery, and spine predicts meager survival
Assessment of Cricopharyngeal Muscle Aging With Apoptotic Markers Ayşe Pelin Gör, MD,* Zeynep Alkan, MD,† Ozgur Yigit, MD,† Nevra Dursun, MD,‡ Acioglu Engin, MD,† S.M. Akin, MD,§ Bulent Sam, MD§ Objectives: The aims of this study were to evaluate histochemical markers of apoptosis in the cricopharyngeus muscle, which is the gatekeeper of the pharyngoesophageal region during the swallowing process; to investigate the effects of primary aging on this muscle; and to determine whether a relationship exists with gastroesophageal reflux disease. Materials and Methods: The study included 30 fresh cadavers with a time of death of 12 hours or less obtained from the Turkish Ministry of Justice Forensic Medicine Unit. All cadavers were dissected with routine postmortem skin incisions to extract specimens from the cricopharyngeus muscle and the esophagocardiac junction mucosa. Muscle degeneration and primary aging were demonstrated by immunodetection of Bax, Bcl-2, and Caspase-3 proteins as markers of the apoptosis. Esophageal specimens were examined for the presence of reflux esophagitis. Results: The mean age was 41.5 (14 –74) years, and the study included 18 male and 9 female cadavers. Three of them were excluded
REFERENCES 1. Hujala K, Martikainen P, Minn H, et al. Malignant nerve sheath tumors of the head and neck: four case studies and review of the literature. Eur Arch Otorhinolaryngol 1993;250:379–382 2. Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, and ultrastructural study of seven cases. Am J Surg Pathol 1998;22:1368–1378 3. Vege DS, Chinoy RF, Ganesh B, et al. Malignant peripheral nerve sheath tumors of the head and neck: a clinicopathological study. J Surg Oncol 1994;55:100–103 4. Athow AC, Kirkham N. Malignant parotid salivary gland peripheral nerve sheath tumour in a twelve-year-old girl. J Laryngol Otol 1992;106:748–750
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From the *Otorhinolaryngology Clinic, Istanbul Catalca Ilyas Cokay State Hospital; †Otorhinolaryngology Clinic and ‡Pathology Department, Istanbul Research and Educational Hospital; and §T.C. Ministry of Justice Forensic Medicine Unity, Istanbul, Turkey. Received July 29, 2013. Accepted for publication February 17, 2014. Address correspondence and reprint requests to: Ayşe Pelin Gör, MD, Otorhinolaryngology Clinic, Istanbul Catalca Ilyas Cokay State Hospital, Ferhatpasa, 34540 Catalca, Istanbul; 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.0000000000000940
© 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 5, September 2014
9. Dicerbo M. Blood flow determined by laser Doppler flowmetry in orthognathic surgery. Am J Orthod Dentofacial Orthop 1993;103:488–489 10. Geylikman YB, Artun J, Leroux BG, et al. Effects of Le Fort I osteotomy on human gingival and pulpal circulation. Int J Oral Maxillofac Surg 1995;24:255–260 11. Emshoff R, Kranewitter R, Norer B. Effect of Le Fort I osteotomy on maxillary tooth-type related pulpal blood flow characteristics. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:88–90 12. Emshoff R, Kranewitter R, Gerhard S, et al. Effect of segmental Le Fort I osteotomy on maxillary tooth-type related pulpal blood flow characteristics. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;89:749–752 13. Justus T, Chang BL, Bloomquist D, et al. Human gingival and pulpal blood flow during healing after Le Fort I osteotomy. J Oral Maxillofac Surg 2001;59:2–7 14. McDonald F, Pitt Ford TR. Blood flow changes in permanent maxillary canines during retraction. Eur J Orthod 1994;16:1–9 15. Tonder KJ. Effect of vasodilating drugs on external carotid and pulpal blood flow in dogs: “stealing” of dental perfusion pressure. Acta Physiol Scand 1976;97:75–87 16. Bell WH. Surgical-orthodontic treatment of interincisal diastemas. Am J Orthod 1970;57:158–163 17. Buckley JG, Jones ML, Hill M, et al. An evaluation of the changes in maxillary pulpal blood flow associated with orthognathic surgery. Br J Orthod 1999;26:39–45 18. Ellingsen RH, Artun J. Pulpal response to orthognathic surgery: a long-term radiographic study. Am J Orthod Dentofacial Orthop 1993;103:338 19. Vedtofte P. Pulp canal obliteration after Le Fort I osteotomy. Endod Dent Traumatol 1989;5:274 20. Meyer MW, Cavanaugh GD. Blood flow changes after orthognathic surgery: maxillary and mandibular subapical osteotomy. J Oral Surg 1976;34:495–501 21. Qeujada JG, Kawamura H, Finn RA, et al. Wound healing associated with segmental total maxillary osteotomy. J Oral Maxillofac Surg 1986;44:366–377 22. Sugg GR, Fonseca RJ, Leeb IJ, et al. Early pulp changes after anterior maxillary osteotomy. J Oral Surg 1981;39:14–20 23. Ramsay DS, Artun J, Martinen SS. Reliability of pulpal blood flow measurements utilizing laser Doppler flowmetry. J Dent Res 1991;70:1427–1430 24. Bell WH. Revascularization and bone healing after anterior maxillary osteotomy: a study using adult rhesus monkeys. J Oral Surg 1969;27:249–255 25. Harada K, Sato M, Omura K. Blood-flow and neurosensory changes in the maxillary dental pulp after differing Le Fort I osteotomies. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97:12–17 26. Zisser G, Gattinger B. Histologic investigation of pulpal changes following maxillary and mandibular alveolar osteotomies in the dog. J Oral Maxillofac Surg 1982;40:332–341 27. Robinson PP. Reinnervation of teeth after segmental osteotomy in the cat. Int J Oral Maxillofac Surg 1986;15:152–159 28. Theisen FC, Guerney LH. Postoperative sequelae after anterior segmental osteotomies. Oral Surg Oral Med Oral Pathol 1971;41:139–151 29. Pepersack WJ. Tooth vitality after alveolar segmental osteotomy. J Oral Maxillofac Surg 1973;1:85–91 30. Vedtofte P, Nattestadt A. Pulp sensibility and pulp necrosis after Le Fort I osteotomy. J Craniomaxillofac Surg 1989;17:167–171 31. Bell WH. Immediate surgical repositioning of one and two tooth dento-osseous segments. Int J Oral Surg 1973;2:265–272 32. Gopikrishna V, Tinagupta K, Kandaswamy K. Comparison of electrical, thermal and pulse oximetry methods for assessing pulp vitality in recently traumatized teeth. J Endod 2007;33:531–535 33. Sailus J, Trowbridge H, Greco M, et al. Sensitivity of teeth subjected to orthodontic forces. J Dent Res 1987;66:556 34. Ingolfsson AER, Tronstad L, Riva CE. Reliability of laser Doppler flowmetry in testing vitality of human teeth. Endod Dent Traumatol 1994;10:185–187
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35. Odor TM, Pitt Ford TR, McDonald F. Effect of wavelength and bandwidth on the clinical reliability of laser Doppler recordings. Endod Dent Traumatol 1996a;12:9–15 36. Hartmann A, Azerad J, Boucher Y. Environmental effects on laser Doppler pulpal blood-flow measurements in man. Arch Oral Biol 1996;41:333–339 37. Soo-ampon S, Vongsavan N, Soo-ampon M, et al. The sources of laser Doppler blood-flow signals recorded from human teeth. Arch Oral Biol 2003;48:353–360 38. Akpinar KE, Er K, Polat S, et al. Effect of gingiva on laser Doppler pulpal blood flow measurements. J Endod 2004;30:138–140 39. Odor TM, Watson TF, Pitt Ford TR, et al. Pattern of transmission of laser light in teeth. Int Endod J 1996b;29:228–234 40. Okabe E, Todoki K, Ito H. Direct pharmacological action of vasoactive substances on pulpal blood flow: an analysis and critique. J Endod 1989;15:473–477 41. Ikawa M, Komatsu H, Ikawa K, et al. Age-related changes in the human pulpal blood flow measured by laser Doppler flowmetry. Dent Traumatol 2003;19:36–40 42. Vongsavan N, Matthews B. Experiments on extracted teeth into the validity of using laser Doppler techniques for recording pulpal blood flow. Arch Oral Biol 1993;38:431–439 43. Anderson KK, Vanarsdall RL, Kim S. Measurement of pulpal blood flow in humans using laser Doppler flowmetry: a technique allowing stability and repeatability of pulpal blood flow measurement during surgical manipulations. Int J Adult Orthod Orthognat Surg 1995;10:247–254 44. Ingle JI, Heithersay GS, Hartwell GR. Endodontic diagnostic procedures. In: Ingle JI, Bakland LK, eds. Endodontics, 5th edition. London, UK: BC Decker Inc, 2002:203–217 45. Olgart LM, Edwall B, Gazelius B. Neurogenic mediators in control of pulpal blood flow. J Endod 1989;15:409–412 46. Matthews B, Vongsavan N. Advantages and limitations of laser Doppler flow meters. Int Endod J 1993;26:9–10
Malignant Peripheral Nerve Sheath Tumor of the Parotid Gland Octavian Chis, MD,* Silviu Albu, MD† Abstract: Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, usually having a poor prognosis, and only a few cases been described in the literature. In this article, we report the diagnostic and therapeutic challenges related to a new case of MPNST of the parotid. Diagnosis was made
From the *Oncology Institute “N. Chiricuta;” and †II-nd Department of Otolaryngology, “Iuliu Hatieganu” University of Medicine and Pharmacy Cluj-Napoca, Cluj-Napoca, Romania. Received December 9, 2013. Accepted for publication February 17, 2014. Address correspondence and reprint requests to Silviu Albu, MD, II-nd Department of Otolaryngology, University of Medicine and Pharmacy Cluj-Napoca, Str. Republicii nr. 18, 400015 Cluj-Napoca, Romania; 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.0000000000000938
© 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 5, September 2014
Brief Clinical Studies
based on clinical, imaging (computed tomography scan), histologic, and immunohistochemistry findings. Despite comprehensive treatment—complete surgical resection and radiotherapy—the tumor displayed a highly aggressive course.
Key Words: Parotid, malignant peripheral nerve sheath tumor, diagnosis, immunohistochemistry
FIGURE 2. Eosinophilic spindle-shaped cells and basophilic matrix (hematoxylin and eosin staining).
M
metastases. Chemotherapy consisting of doxorubicin, cisplatin, and cyclophosphamide was selected. However, the patient died of disease 2 months later.
alignant peripheral nerve sheath tumor (MPNST) refers to sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath.1–3 According to the new World Health Organization classification of soft tissue tumors, spindle cell sarcomas originating from the peripheral nerves have been defined as MPNST.1–3 It is widely recognized that MPNSTs are highly aggressive malignant tumors with a dismal prognosis. The MPNST develops within extremities of the trunk and rarely appears in the head and neck region.1–3 This study describes a patient with MPNST of the parotid gland. To the best of our knowledge, only 8 case reports of MPNST of the parotid gland have been described in the current English-language literature.4–9
CLINICAL PRESENTATION A 60-year-old woman presented with a 4-month history of rapidly developing, painful left parotid swelling. Physical examination revealed a 5 6-cm firm, hardly fixed, immobile, irregular contoured mass associated with severe spontaneous pain and tenderness. There was no facial palsy and no palpable lymph nodes. A computed tomographic scan showed a multilobulated, nonhomogeneous mass within the superficial lobe of the left parotid gland, whereas the deep lobe was normal (Fig. 1). Intraoperatively, a 6 6-cm mass was observed within the left parotid gland. The main trunk and all the branches of the facial nerve were entrapped within the tumor. A total parotidectomy with resection of the main facial nerve trunk, and a modified radical neck dissection was performed. Microscopically, the tumor was composed of spindle cells with coiled or asymmetric oval nuclei. The cells were arranged in sweeping fascicles or a spinning pattern (Fig. 2). There was significant cellular pleomorphism, with curved or twisted nuclei and prominent mitotic activity. Foci of necrosis were also present. Nerve fibers and salivary gland tissue were entrapped within the tumor (Figs. 3, 4). The resection margin was free of tumor. Nodes from the inferior pole of the parotid, posterior jugular chain, and carotid sheath were all negative for metastasis. Immunohistochemistry yielded positive for S-100 protein (Fig. 5) and vimentin; weakly positive for actin; yet negative for CD34, desmin, epithelial membrane antigen, cytokeratin AE1/AE3, and glial fibrillary acidic protein. We counted Ki67 labeling indices in 20% of the tumor cells (Fig. 6). A diagnosis of MPNST was made. The patient underwent a full course of radiation therapy. Six months later, a chest radiograph revealed multiple lesions considered as
FIGURE 1. Patient with tumor of the parotid gland—computed tomographic scan.
DISCUSSION The MPNSTs account for approximately 10% of all soft tissue sarcomas, are located on the extremities and trunk, and are seldom encountered within the head and neck region.1–3 A recent review by Imamura et al6 identified 142 cases of head and neck MPNST published within the past 23 years. The MPNST of the parotid gland is extremely rare, with only 8 cases described in the recent literature.1–9 The MPNSTs derive from Schwann cells, often developing in preexisting von Recklinghausen disease (neurofibromatosis 1-NF1) or as a solitary malignant tumor.5,6 The risk of developing MPNST seems to be 4000 times greater in patients with NF1 than in the general population.6–9 Approximately 5% of the NF1 patients develop MPNSTs, whereas 9% of the head and neck MPNSTs develop from preexisting NF1.3,5–9 Generally, MPNST is a disease of adults, and the onset age shows a significant discrepancy in the literature.1–9 However, most tumors develop in patients aged 20 to 50 years, with a mean of 45 years. The sex ratio of MPNST cases is differently estimated in the published series depending on the inclusion criteria; however, most authors agree that, in sporadic MPNST case, the sex ratio is equal3,5,6. It has been reported that, within the head and neck region, MPNST frequently develops in the neck area—40% to 60% of cases. A recent review revealed that MPNST may arise from any organ of the head and neck region.1–9 Symptoms associated with these tumors include rapidly developing and particularly painful mass and functional disorders such as trismus, dysphagia, dysarthria, and facial palsy in up to 10% of cases.2–9
FIGURE 3. Peripheral nerve branch entrapped within the tumor.
FIGURE 4. Salivary gland encased within the tumor.
© 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 5, September 2014
Brief Clinical Studies
FIGURE 5. Immunohistochemistry: positive staining for S-100 protein.
5. Punjabi AP, Haug RH, Chung-Park MJ, et al. Malignant peripheral nerve sheath tumor of the parotid gland: report of case. J Oral Maxillofac Surg 1996;54:765–769 6. Imamura S, Suzuki H, Koda E, et al. Malignant peripheral nerve sheath tumor of the parotid gland. Ann Otol Rhinol Laryngol 2003;112:637–643 7. Nepka C, Karadana M, Karasavvidou F, et al. Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report. Acta Cytol 2009;53:423–426 8. Gogate BP, Anand M, Deshmukh SD, et al. Malignant peripheral nerve sheath tumor of facial nerve: presenting as parotid mass. J Oral Maxillofac Pathol 2013;17:129–131 9. De Stefano A, Kulamarva G, Citraro L, et al. Malignant peripheral nerve sheath tumour (malignant epithelioid Schwannoma) of the parotid gland. Bratisl Lek Listy 2012;113:628–631 10. Wong WW, Hirose T, Scheithauer BW, et al. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998;42:351–360 11. Loree TR, North JHJr, Werness BA, et al. Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors. Otolaryngol Head Neck Surg 2000;122:667–672
FIGURE 6. Ki67 labeling of the tumor.
Microscopically, the tumor is composed of eosinophilic spindleshaped cells and basophilic extracellular matrix. Immunoreactivity with S-100 protein stands for neural differentiation. Although most cases were positive for S-100 protein, the staining intensity varied from case to case. Nevertheless, vimentin was strongly positive in all reported studies, whereas epithelial-derived antigens were negative in most of the case reports.3–6 Positive immunostaining with antigens used to spot nerve sheath differentiation such as Leu 7, myelin basic protein, and nerve cell adhesion molecule was also described.6 It was suggested that staining with p53 and high Ki67 immunoreactivity designates malignant transformation.3,6 It is generally accepted that, in MPNST, the best chance for survival was with wide radical excision or amputation when the tumor involved an extremity.6,10 In the head and neck region, because of the particular anatomy, radical surgery is not always possible. Nevertheless, recurrence is common. If radical tumor removal is not possible, excision combined with high-dose radiation therapy seems to be the best alternative.10,11 Adjuvant chemotherapy should be considered in those MPNST patients who had it in relation with NF1 or at a site of prior radiation.3,8,10 Prognosis in MPNST of the head and neck is generally poorer than that of MPNST developing in the extremities.11 Patients with NF1 had a 5-year survival of 23%, compared with 47% in patients without such disease. The mean survival period was 42 months, and 21 of the 38 patients with head and neck MPNSTs did not survive.5,9 Prognostic factors associated with survival include tumor size, location of the tumor, and surgical margins.11 Invasion of the skull base, carotid artery, and spine predicts meager survival
Assessment of Cricopharyngeal Muscle Aging With Apoptotic Markers Ayşe Pelin Gör, MD,* Zeynep Alkan, MD,† Ozgur Yigit, MD,† Nevra Dursun, MD,‡ Acioglu Engin, MD,† S.M. Akin, MD,§ Bulent Sam, MD§ Objectives: The aims of this study were to evaluate histochemical markers of apoptosis in the cricopharyngeus muscle, which is the gatekeeper of the pharyngoesophageal region during the swallowing process; to investigate the effects of primary aging on this muscle; and to determine whether a relationship exists with gastroesophageal reflux disease. Materials and Methods: The study included 30 fresh cadavers with a time of death of 12 hours or less obtained from the Turkish Ministry of Justice Forensic Medicine Unit. All cadavers were dissected with routine postmortem skin incisions to extract specimens from the cricopharyngeus muscle and the esophagocardiac junction mucosa. Muscle degeneration and primary aging were demonstrated by immunodetection of Bax, Bcl-2, and Caspase-3 proteins as markers of the apoptosis. Esophageal specimens were examined for the presence of reflux esophagitis. Results: The mean age was 41.5 (14 –74) years, and the study included 18 male and 9 female cadavers. Three of them were excluded
REFERENCES 1. Hujala K, Martikainen P, Minn H, et al. Malignant nerve sheath tumors of the head and neck: four case studies and review of the literature. Eur Arch Otorhinolaryngol 1993;250:379–382 2. Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, and ultrastructural study of seven cases. Am J Surg Pathol 1998;22:1368–1378 3. Vege DS, Chinoy RF, Ganesh B, et al. Malignant peripheral nerve sheath tumors of the head and neck: a clinicopathological study. J Surg Oncol 1994;55:100–103 4. Athow AC, Kirkham N. Malignant parotid salivary gland peripheral nerve sheath tumour in a twelve-year-old girl. J Laryngol Otol 1992;106:748–750
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From the *Otorhinolaryngology Clinic, Istanbul Catalca Ilyas Cokay State Hospital; †Otorhinolaryngology Clinic and ‡Pathology Department, Istanbul Research and Educational Hospital; and §T.C. Ministry of Justice Forensic Medicine Unity, Istanbul, Turkey. Received July 29, 2013. Accepted for publication February 17, 2014. Address correspondence and reprint requests to: Ayşe Pelin Gör, MD, Otorhinolaryngology Clinic, Istanbul Catalca Ilyas Cokay State Hospital, Ferhatpasa, 34540 Catalca, Istanbul; 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.0000000000000940
© 2014 Mutaz B. Habal, MD
Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
