The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015

Correspondence

Alessandra Marcondes Aranega, DDS, MSc Rodrigo dos Santos Pereira, DDS, MSc Department of Surgery and Integrated Clinics Araçatuba School of Dentistry University of Estadual Paulista Araçatuba Sao Paulo, Brazil

REFERENCES 1. Kohlhof JK, Driemel O, Müller-Richter UD. Traumatic dislocation of the globe into the maxillary sinus—is a rehabilitation possible? Klin Monbl Augenheilkd 2005;224:867–870 2. Magarakis M, Mundinger GS, Kelamis JA, et al. Ocular injury, visual impairment, and blindness associated with facial fractures: a systematic literature review. Plast Reconstr Surg 2012;129:227–233 3. Guly CM, Guly HR, Bouamra O, et al. Ocular injuries in patients with major trauma. Emerg Med J 2006;23:915–917 4. Flower JG. Spontaneous luxation of the eyeballs. JAMA 1941;116:1206–1208 5. Xu B, Xu XL, Yan J. Treatment of traumatic globe dislocated completely into the maxillary sinus. Int J Ophthalmol 2013;6:106–107 6. Haggerty CJ, Roman P. Repositioning of a traumatically displaced globe with maxillary antrostomy: review of the literature and treatment recommendations. J Oral Maxillofac Surg 2013;71:1915–1922 7. Tyers AG, Collin JRO. Orbital implants and postenucleation socket syndrome. Trans Ophthalmol Soc 1982;102:90–92

Malignant Peripheral Nerve Sheath Tumor of the Forehead To the Editor: Malignant peripheral nerve sheath tumor (MPNST) originates from the peripheral nerves or their sheaths, and it has replaced the previous confusing terminologies of a neurogenic sarcoma, neurofibrosarcoma, or malignant schwannoma.1–5 The estimated frequency of MPNSTs in the population is 0.001%; on the other hand, it can increase from 2 to 5% to 42% in patients with neurofibromatosis type 1 (NF1) with an aggressive course.3 The highest incidences of these tumors occur about the ages of 20 to 50 years.1 Malignant peripheral nerve sheath tumors derive from the peripheral nerve root trunk, extremities, and the head and neck area.6 Malignant peripheral nerve sheath tumor is extremely occasional tumor with an incidence of 1 per 100,000 population, which is between 3% and 10% of all the soft tissue sarcomas. So, this entity is often managed as a subgroup of soft tissue sarcomas.1,7 Here, we are reporting a case of a sporadic, malignant, peripheral nerve sheath tumor of the forehead in a 65-year-old man. In our case, there was no clinical sign of NF1, and NF1-independent MPNSTs are extremely infrequent.

FIGURE 2. Postoperative view of the patient.

and examined in another clinic. The excision margins were tumor positive, and the defect was reconstructed with a full-thickness skin graft, which was taken from the supraclavicular area (Fig. 1).There was blunt sensitive pain over the graft area. There was a previous history of trauma that was triggered by a blunt object to the forehead 3 years ago. There were no clinical data that were indicative of NF1 and no family history of any NF1 lesion. On physical inspection, the scarring and swelling were found to be on the forehead area, which measured 2.52.0 cm in the middle one third of the forehead (Fig. 1). The swelling was soft and painful, and it was fixed to the overlying skin, soft tissue, and the muscle, but it was free from bone. The swelling was nonpulsatile, with normal temperature. The laboratory results, which included blood count, urine analysis, and chest x-ray/ultrasonography of the abdomen were all unremarkable. Computed tomography and magnetic resonance imaging were suggestive of a 2.0  1.8 cm soft tissue mass that was free from bone. The patient underwent a wide local excision of the lesion. Ther defect was reconstructed with a scalping flap, and the donor site of the flap was reconstructed with split-thickness skin graft (Fig. 2). The wide excised mass (6.6  3.2  0.8) was solid and cystic in nature, and it was adherent to the supraorbital nerve (Fig. 3). By gently detaching it from the nerve, the lesion was excised, and it was sent for histopathologic analyses. The postoperative interval was uneventful.

GROSS FINDING We took the excised mass of the forehead, which completely measured 6.6  3.2  0.8 cm (Fig. 3). Its outer surface was well restricted, nodular, and irregular, with crowded vessels. On cutting it open, a gray-white mass with a spotted form, with an area of a hemorrhage, necrosis, and cystic alterations, was observed.

LIGHT MICROSCOPY

A 65-year-old male patient was referred to our clinic with a diagnosis of malign peripheral nerve sheets tumor that was excised

Multiple slices, which were explored, showed a well-demarcated tumor with hypercellular and hypocellular parts. The hypercellular parts were arranged in fascicles, sheets, and nodules. The specific tumor cells were spindle formed, with curly cores and buckling. The hypocellular parts were composed of large centers of necrosis with a standard palisading look and with myxoid extents. Focal regions of hemorrhage and necrosis were noticed. Multiple nerves like curls were also distinguished (Fig. 4A). The underlying muscle was free from tumor. An absolute histopathologic conclusion of a malignant soft tissue tumor, a malignant peripheral nerve sheet tumor,

FIGURE 1. Preoperative view of the patients.

FIGURE 3. Excised material.

CLINICAL REPORT

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© 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

The Journal of Craniofacial Surgery • Volume 26, Number 1, January 2015

FIGURE 4. A, Highly cellular spindle cell tumor (hematoxylin-eosin stain, original magnification 100). B, The tumor cells express s100 protein (immunohistochemistry, S100, original magnification 100).

was given. The patient was free from the residual tumor/recurrence for the past 1 year after the follow-up. The immunohistochemical analysis verified the diagnosis of MPNST in view of the diffuse positivity of vimentin and neuron-specific enolase with focal positivity for S-100 and negativity for MDM2 (Fig. 4B). Consequently, the differential diagnosis of a dedifferentiated liposarcoma was ruled out.

DISCUSSION An MPNST is rare neoplasm and an occasional variation of a soft tissue sarcoma of ectomesenchymal origin. It develops from the peripheral nerve sheaths or the branches of the peripheral nerve fibers.2–8 This tumor may arise spontaneously in adult patients, although 5% to 42% of the MPNSTs have an association with multiple NF1’s. Thus, a combination of the macroscopic, pathological, and also immunohistological research is used for diagnosing these tumors. Malignant peripheral nerve sheath tumors are frequently related to NF1’s, and these are aggressive tumors that cause important therapeutic and diagnostic difficulties.3 Approximately 10% of the NF1 patients may have MPNSTs that display bad prognoses.4,5,8 The pathogenesis of MPNSTs has been inadequately known owing to their complicated histopathologies, except a biallelic NF1 gene inactivation is crucial for the tumor occurrence.3,6 Laskim et al7 draw attention to radiation of the peripheral nerve as one of the potential etiologies. Such tumors commonly develop following a long dormant episode of 10 to 20 years after irradiation, and they comprise 11% of MPNSTs.9 Our case, a 65-year-old man, was a farmer with no history of exposure to irradiation and not including any clinical proof of an NF1 (a multiple neurofibroma, café-au-lait spots, congenital abnormalities, or other tumors, etc) in the patient or in the family. Hence, our case was of a sporadic, non-NF1 or NF1independent MPNST of the forehead with a favorable prognosis. Complete surgical removal is a basis of treatment and a strong predictor of the survival regardless of its biological destructive character. In our case, local excision of the tumor with wide resection margins was performed. The patient is free of symptoms after 1 year of follow-up. The tumors are categorized as low and high grade on the basis of their cellular variations, mitotic amounts, and tumor necrosis. In excess of 50% necrosis with exceeding 5 mitotic figures each 10 highpower field has been thought as indicative of a high-grade tumor.1,9–11 In our patient, the MPNST was of low grade considering, the 1 to 3 mitotic figures with 10% of tumor necrosis. These tumors often create diagnostic struggles owing to their cellular sources and histopathologic resemblances with those of other spindle cell tumors.9 The tumor’s distinction diagnosis is monophasic undifferentiated sarcoma, synovial liposarcoma, fibrosarcoma, and leiomyosarcoma. The different distinguishing morphology of the tumor with the special diagnostic patterns helps in the diagnoses of the above stated tumors, and the diagnoses can be confirmed by immunohistopathologic investigations.3,12 In 2006, Miller et al12 noticed that the MPNST lines were heterogeneous in their in vitro growth rates and that they exhibited

Correspondence

diverse alterations in the expressions of the pRb, p53, p14 (Arf ) along with the p16 (INK4a) amino acids. All of the MPNST cell lines expressed the epidermal growth factor receptor, and they lacked the S100 β protein. They determined that the Schwann cell differentiation markers (SOX10, CNP, PMP22, and NGFR) were down-regulated in the MPNSTs; however, the neural crest cell markers, SOX9 and TWIST1, were overexpressed. They supposed that gene expression profiling was a possible biomarker and/or a therapeutic target for the management of MPNSTs and that it could be used as a main analytic instrument.1,13 In the present case, a morphodiagnosis of an MPNST was made with the help of the hypocellular and hypercellular areas with the characteristic nerve-like whorls of the tumor tissues and fascicles and also the nuclear criteria of the tumor cells. The particular mitotic figures and the tumor necrosis favored its low-grade nature, and the size of the tumor suggested its giant type. There was no family history or any kind of clinical proof of an NF1. And so, a final histopathologic analysis of a giant sporadic low-grade MPNST of the forehead was made. The above outcomes were recognized on immunohistopathologic analyses, by way of S-100, neuron-specific enolase, and vimentin, which were positive. The patient has been on standard follow-up for the past 1 year and is free of any residual or recurrent tumor.

CONCLUSIONS In the present case, we have brought to light the sporadic character and the MPNSTs, which are occasional clinical entities, as most MPNSTs exist with NF1’s. As a result of their non-NF1 or NFindependent character, they have good prognoses. A combination of clinical and pathological, in addition to immunohistochemical, studies facilitates in the diagnoses of such uncommon tumors. Wide local excision and reconstruction are the current treatment.

Saime Irkoren, MD Heval Selman Ozkan, MD Department of Plastic and Reconstructive Surgery Adnan Menderes University Faculty of Medicine Aydin, Turkey [email protected] Canten Tataroglu, MD Department of Pathology Adnan Menderes University Faculty of Medicine Aydin, Turkey

REFERENCES 1. Nikumbh DB, Suryawanshi KH, Dravid NV, et al. Giant sporadic low grade malignant peripheral nerve sheath (MPNST) of left thigh. J Clin Diagn Res 2013;7:1155–1158 2. Akhavan A, Moghimi M, Karimi-Zarchi M, et al. Malignant peripheral nerve sheet tumour of cervix. BMJ Case Rep 2012;30:2012 3. Pabiszczak M, Woźniak A, Wierzbicka M, et al. Diagnostic difficulties in the laryngeal malignant peripheral nerve sheath tumor (MPNST). Otolaryngol Pol 2004;58:1133–1136 4. Kar M, Deo SVS, Shukla WK, et al. Malignant peripheral nerve sheath. Clinicopathological study and treatment outcome of 24 cases. World J Surg Oncol 2006;4:55 5. Friedrich RE, Hartmann N, Mautner VF. Malignant peripheral nerve sheath tumor MPNST in NF1 affected children. Anticancer Res 2007;27:1957–1960 6. Hanabusa K, Morikawa A, Murata T, et al. Acoustic neuroma with malignant transformation. Case report. J Neurosurg 2001;95:518–521

© 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 26, Number 1, January 2015

Correspondence

7. Laskim WB, Silvermann TA, Enzinger PM. Post radiation soft tissue sarcoma. An analysis of 53 cases. Cancer 1988;11:2330–2340 8. Minovi A, Basten O, Hunter B, et al. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007;29:439–445 9. Adamson DC, Cummings TJ, Friedman AH. Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin’s lymphoma. Clin Neuropathol 2004;23:245–255 10. Gupta G, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Focus 2007;22:E12 11. Stark A, Buhl R, Hiugo H, et al. Malignant peripheral nerve sheath tumours—report of 8 cases and review of the literature. Acta Neurochir (Wien) 2001;143:357–364 12. Khan RJ, Asgher J, Sohail MT, et al. Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of literature. Pathology 1998;30:237–241 13. Miller SJ, Rangwala F, Williams J, et al. Large scale molecular comparison of human Schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Cancer Res 2006;66:2584–2591

Oro Nasal Communication Closure in Smoker Patient To the Editor: Buccal fat pad in recent years has become a wellaccepted graft for covering intraoral defects.1 This structure is also known by the eponym Bichat, because he was the French anatomist to discover this tissue in 1801.1,2 The buccal fat pad is a supple and lobulated mass; easily accessible and mobilized3; located between the buccinator muscle and the mandibular ramus, separating the masticatory muscles from each other; and often considered to be a nuisance when encountered in intraoral procedures such as facial bone osteotomies, elevation of buccal flap, or procedure on Stensen duct.1 Egyedi,4 in 1977, was the first to describe the use of Bichat fat pad with surgical description for closure of oroantral communications. This type of complication is quite usual and described in the clinical literature after the extraction of maxillary posterior teeth, especially in the presence pneumatization of the maxillary sinus.5,6 The authors aimed to describe a case of closure of oroantral communication with challenging clinical frame, because the patient is a smoker at 2-month latency time of the bucosinus fistula.

FIGURE 2. Preoperative panoramic radiograph.

limits, including periodic visits to the cardiologist, and this case has already been evaluated by the otolaryngologist. Intraoral physical examination has shown lack of healing of the socket corresponding to the tooth 26, with the presence of chronic purulent secretion in alveolar extraction. For purposes of evidence, the Valsalva test was performed, which was positive, therefore confirming the clinical diagnosis of oroantral communication. Immediately, a panoramic radiograph was obtained, which noted the absence of bone continuity on the floor of the maxillary sinus (Figs. 1 and 2). Possible forms of treatment were explained to the patient, always warning of the risk of failure, regardless of the therapeutic approach chosen, because the patient is a heavy smoker with presence of fully formed fistula, and sinusitis. After administration of local anesthesia with articaine hydrochloride 4% with 1:100,000 epinephrine, the fistula was removed through an incision skirting corresponding to the tooth socket 26. After extensive washing of the area with 0.9% saline solution, 2 relaxing incisions were made, an earlier and a more posterior incision to the region. Following the detachment, several incisions were held into the periosteum and muscle layer in order to expose the fat pad of Bichat. After exposure, the tissue was pulled carefully with Dietrich forceps until fully rested and covering the defect without tension. The buccal fat pad was sutured with 4-0 Vicryl, and retail was sutured with 4-0 Vicryl, and some points were given with 5-0 nylon (Figs. 3 and 4). After surgery, antibiotic therapy with 400 mg metronidazole associated with 500 mg amoxicillin 3 times a day for 15 days was prescribed to the patient. The postoperative instructions were to avoid blowing the nose, sneezing open mouth, sucking (such as avoiding using straws), and smoking, which should be light and puffing fewer cigarettes per day. Moreover, with continued use of the prescribed antibiotics, 750 mg of paracetamol every 6 hours for 2 days and 100 mg of nimesulide every 12 hours for 4 days were also prescribed. Periodic visits were scheduled for 1 year, and at the end, we can infer the success of treatment (Figs. 5 and 6), with total closure of

CLINICAL REPORT A 47-year-old woman was referred to the dental clinic of Araçatuba Dental School complaining of severe headaches, especially when waking up, and development of sinusitis with an unpleasant taste in the mouth 2 months ago. The patient is a smoker, without any disease or allergy. All her laboratory tests were reported to be within normal

FIGURE 1. Initial clinical image.

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FIGURE 3. A, Removal of the fistula. B, Exposure of buccal fat pad. C and D, Closing the first layer.

© 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

Malignant peripheral nerve sheath tumor of the forehead.

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