The Journal of Craniofacial Surgery
Brief Clinical Studies
FIGURE 3. Preoperative view of dissected components. Lateral component (LC) is transferred towards the anterior component (AC), posteromedial component (PMC) is transferred medially to align to LC.
FIGURE 4. Patient’s appearance on postoperative first year.
Germany) and skin was sutured separately by using 6.0 Prolene (Ethicon). Light dressing was applied. 6. Sutures were removed on fifth postoperative day. There were no problems on postoperative follow-up (Fig. 4).
DISCUSSION Many classifications were described for CELC.2 –4 Classifications that were described do not bring a common solution. The most appropriate classification is of Kitayama et al2 in appearance, earlobe clefts are divided into 4 groups longitudinal, transverse, triple lobe, and defective types. This classification reveals anatomical problem clearly, so that the problem can be described more accurately. There are many techniques for CELC correction,1,5–12 but only a few authors have discussed on triple lobe deformity clearly.13,14 A simple skin excision to reach lobes and transferring components together is the main principle of our technique. If lobes are very large, minimal excision can be performed. It should be noted that the different anatomical planes and poor soft tissue connection are issues that need to be tackled. Components can be presented in various ways. The most important points are to reconstruct a rounded free edge and minimal tissue sacrifice for earlobe, to establish a rounded free edge for triple lobe deformity, surgeon needs to dominate the three-dimensional anatomy and imagine how to combine the components of triple lobe.
REFERENCES 1. Fearon J, Cuadros CL. Cleft earlobe repair. Ann Plast Surg 1990;24:252–257 2. Kitayama Y, Yamamoto M, Tsukada S. Classification of congenital cleft ear lobe. Jpn J Plast Reconstr Surg 1980;23:663 3. Matsumoto K. Surgical repair of the congenital ear lobe cleft. Br J Plast Surg 1981;34:410–413 4. Yamada A, Fukuda O, Soeda S, et al. The evaluation of cleft earlobe. Jpn J Plast Reconstr Surg 1976;19:171 5. Hyakusoku H, Fumiiri M. The square flap method. Br J Plast Surg 1987;40:40–46 6. Attalla MF. Congenital earlobe cleft: a new technique for repair. Br J Plast Surg 1990;43:223–225 7. Fujiwara T, Matsuo K, Taki K, et al. Triangular flap repair of the congenital earlobe cleft. Ann Plast Surg 1995;34:402–405 8. Hochberg J, Ardenghy M. Repair of incomplete cleft earlobe. Ann Plast Surg 1996;37:170–172 9. Maral T, Tuncali D, Ozgu¨r F, et al. A technique for the repair of simple congenital earlobe clefts. Ann Plast Surg 1996;37:326–331 10. Bhandari PS. Congenital cleft earlobe: surgical repair. Plast Reconstr Surg 2001;108:986–987 11. Borman H, Deniz M, Ertas NM, et al. 7-plasty technique for the surgical treatment of congenital longitudinal ear lobe cleft. J Craniofac Surg 2008;19:1643–1644
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12. Qing Y, Cen Y, Xu X, et al. A new technique for correction of simple congenital earlobe clefts: diametric hinge flaps method. Ann Plast Surg 2013;70:657–658 13. Park C, Roh T. Button-down procedure for correction of cleft earlobe malformation. Plast Reconstr Surg 1997;99:1429–1432 14. Park C. Lower auricular malformations: their representation, correction, and embryologic correlation. Plast Reconstr Surg 1999;104:29–40
Schwannoma of the Servical Vagus with Intrathoracic Extension Selahattin Tugrul, MD, Erol Senturk, MD, Orhan O¨zturan, MD, Abdu¨laziz Ko¨k, MD,y and Nur Buyukpinarbasili, MDz Abstract: Schwannomas are the rare benign tumors of the neck. Patients developing from the brachial plexus and cervical truncus and extending to the posterior mediastinum have been reported, but for the first time the following case report describes the extension of a vagal origin schwannoma from the anterior and middle mediastinum and its extraction with thoracotomy without sternotomy. Key Words: Intrathoracic extension, neurilemmoma, vagus nerve
S
chwannomas are rare benign tumors that develop from Schwann cells located in peripheral nerve sheaths. They typically present as encapsulated, solitary, and slowly growing masses. They can develop from peripheral, cranial, or autonomous nerve sheaths in different anatomic locations, but 25–40% are detected in the head and neck regions.1 They can be independently sporadic, or found as part of a syndrome such as Neurofibromatosis 1 and 2. In the latter case, attention should be paid to other examination findings such as skin stigmata and family history. Probable diagnosis of schwannomas can be made by physical examination, ultrasound, CT, and MR, but definitive diagnosis can only be made by histopathology. According to their microscopic cellularity, schwannomas can be divided into 2 groups: Antony A or Antony B. Treatment is surgical enucleation to protect the affected nerve. Neck schwannomas can originate from the brachial and cervical plexus, sympathetic truncus, nervus facialis, nervus glossopharyngeus, nervus hypoglossus, or, quite rarely, the nervus vagus. Intrathoracic extension of a vagus-originated schwannoma has never been reported. This case report describes the symptoms of a vagus-originated giant schwannoma and the surgical extraction of its intrathoracic extension. From the Department of Otorhinolaryngology; yDepartment of Thoracic Surgery; and zDepartment of Pathology, Bezmialem Vakif University, Fatih, Istanbul, Turkey. Received January 30, 2015. Accepted for publication February 8, 2015. Address correspondence and reprint requests to Erol Senturk, MD, Department of Otorhinolaryngology, Medical Faculty, Bezmialem Vakif University, Fatih, 34080, Istanbul, Turkey. E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001704 #
2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 4, June 2015
Brief Clinical Studies
CASE REPORT A 28-year-old male patient presented to our clinic with hoarseness and swelling on the left side of his neck. History disclosed that this swelling has been present for a few years but had recently got larger. Hoarseness had started the month before. The 10 cm long and 5 cm wide mass on the left side of his neck could be moved horizontally with palpation, but not vertically. No other finding arose in physical examination. During indirect laryngoscopy, the left vocal cord was immobile in respiration and phonation. No other mass was observed in the oropharynx, nasopharynx, or hypopharynx. In ultrasonography (USG), a smooth contour solid mass was observed between the internal jugularis vein and common artery in which calcifications, fibrous septae, and nodules were present. In the contrast-enhanced neck MR, a heterogeneous mass of greater than 20 cm was detected. It started from the lower border of the parotid gland in the left anterior paracervical region, and extended to the anterior mediastinum (Fig. 1). A large number of nodular constructions were hypointense in the lesion T1 sections, hypo-hyperintensity in the T2 sections, and had peripheral contrast involvement inside the lesion. Spindle cells, considered to be part of a nerve sheath tumor, were observed during fine needle aspiration (FNA) biopsy accompanied by USG. Our otorhinolaryngology and thoracic surgery team operated on the patient with the probable of vagal schwannoma. The operation was started under general anesthesia in the posterior-lateral thoracotomy position. Left anterior thoracotomy was performed. The 7-cm-diameter mass under the phrenic nerve was dissected up to the thoracic outlet (Fig. 2). After that, the neck was positioned in the supine position and a carotid-type incision was performed. With a lateral cervical approach, the mass was dissected from vagal nerve fibers by the microsurgical technique and extracted in almost a block shape (Fig. 3). After hemostasis, a drain was placed in the neck and thorax, and the operation was completed. No electrocardiographic (EKG) abnormality or hemodynamic instability occurred during the procedure. Cheilothorax that had perhaps formed postoperatively because of damage to the thoracic duct had disappeared after 1 week on a fat-free diet. The patient was administered antibiotherapy and then discharged. Pathologic diagnosis was cellular schwannoma. Immunohistochemically, tumor cells S-100 were common positive (þ). The Ki-67 score was found to be 8% (Fig. 4). During monthly routine controls that followed, the patient had no complaint except hoarseness. Laryngoscopy disclosed left vocal cord paralysis.
FIGURE 1. T2 magnetic resonance image shows the tumor.
FIGURE 2. A picture of the mass during operation shows the intrathoracic extension of the schwannoma. D: heart. o: schwannoma.
FIGURE 3. It is a picture of the whole mass after the operation. D: intrathoracic part. o: cervical part.
#
2015 Mutaz B. Habal, MD
FIGURE 4. A, A microscopic picture of the tumor shows mild cellularity (hematoxylin and eosin; original magnification, 100). B, Immunohistochemically, tumor cells are painted S-100 common positive (þ).
DISCUSSION A nerve sheath tumor was first defined by Verocay in 1908 who called it a neurinoma.2 Neurinomas are generally benign, encapsulated, and have distinct boundaries. Because approximately one third of all schwannomas are formed in the head and neck region, they should therefore be kept in mind during differential diagnosis for neck masses. For massive neck and thorax masses, sarcoma, lymphoma, and pulmonary tumors should also be considered as well as neurogenic tumors; however, final diagnosis should be made histopathologically. Vagal schwannomas are rarely seen among head–neck schwannomas. In a series of 21 diseases listed by Langner et al, the origin of schwannoma was found to be the brachial plexus (23.8%), vagus (14.2%), and sympathetic chain (19%), or lingual, recurrent laryngeal, and cervical plexus nerves (4.7% each). No site of origin was found in 6 patients (28.6%).3 In their series, vagal schwannomas were limited to the neck. In our patient, the schwannoma revealed an intrathoracic extension. Schwannomas revealing intrathoracic extension have been defined before; however, because such schwannomas originate from the brachial or cervical plexus, they extend to the posterior mediastinum and are called ‘‘dumbbell schwannomas.’’4,5 The schwannoma in our patient revealed extension to the middle and anterior mediastinum, with proximity to the heart, phrenic nerve, and major veins. In the literature, we could find no reported patient of a vagal schwannoma revealing introthoracic extension similar to ours. A schwannoma can be considered as part of the von Reclinghausen syndrome. In an interesting and yet unknown manner, such tumors are usually located on the right side.6 In our patient, no skin stigmata or family history that could suggest this syndrome was present, and the tumor was on the opposite (ie, left) side. Cervical vagal schwannomas generally reveal asymptomatic findings such as slowly growing lateral neck masses, with hoarseness as its most common symptom. Our patient had complaints only of hoarseness and the swelling on his neck. Dyspnea, dysphagia, coughing, syncopal episodes, and Horner syndrome may accompany, but none of these symptoms was present in our patient. During the operation, cardiac arrhythmia could be observed depending on the manipulation. For a patient with a mass in the lateral area of the neck, other causes should also be considered for differential diagnosis from neurogenic tumors, including branchial cleft cyst, lipoma, lymphadenopathy, sarcoma, carotid body tumor, salivary gland tumor, and abscess. In terms of skin stigmata, a complete physical examination with ultrasonography, MR, and FNA helped in diagnosis. A neurogenic tumor originating from the vagus nerve can be shown via ultrasonography that it extends between the carotid artery and jugular vein, but MR monitoring should help clarify the situation. In our patient, the MR disclosed a heterogeneous mass with sharp edges and nodules inside of a hold contrast. Some authors recommend an FNA; however, due to possible hemorrhaging and scar forming, others do not.7 Even authors who do not suggest a biopsy reported that they biopsied their cases. We wanted to support our
e321
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Brief Clinical Studies
diagnosis with FNA before the operation, and to see that it did not cause any additional symptoms. For limited neck patients, the common treatment is gross total resection by protecting the vagal nerve with a/the lateral cervical approach. For an introthoracic extension of neurogenic tumors that originated in the brachial and cervical plexus, posterior thoracotomy is performed, also with the same lateral cervical approach.8 In this patient, we were able to extract the tumor extending to the anterior and middle mediastinum by left anterior thoracotomy after left lung extinction. No sternotomy was needed. After resection, definitive diagnosis of schwannoma can be made histopathologically and classified by 1 of 2 microscopic patterns. In the Antoni A pattern, Verocay corpuscles are present and hypercellularity and nuclear palisading are also present. Antoni B exhibits a hypocellular pattern consisting mostly of loose tissue. To support the diagnosis of either, S-100 staining can be used. In our case, the tumor was hypercellular and stained thoroughly with S-100. Vagal schwannoma should be considered in the differential diagnosis of a lateral neck mass. Hoarseness is its most known frequent symptom. Considering possible intrathoracic extension beside the neck mass, the neck and thorax should be reviewed together by MR. For intrathoracic extension, a multidisciplinary approach is necessary together with thoracic surgery. Even a tumor with more than a 5 cm intrathoracic extension can be extracted by anterior thoracotomy without sternotomy.
ACKNOWLEDGMENT The authors appreciate the contributions and editorial assistance made by S. Delacroix, a native English speaker.
REFERENCES 1. Colreavy MP, Lacy PD, Hughes J, et al. Head and neck schwannomas: a 10 years review. J Laryngol Otol 2000;114:119–124 2. Whittam DE, Morris TM. Neurilemmoma of the larynx. J Laryngol Otol 1970;84:747–750 3. Langner E, Del Negro A, Akashi HK, et al. Schwannomas in the head and neck: retrospective analysis of 21 patients and review of the literature. Sao Paulo Med J 2007;125:220–222 4. Kanzaki M, Ohtsuka T, Obara T, et al. Surgically treated dumbbell schwannoma arising in the brachial plexus with intrathoracic extension. Jpn J Thorac Cardiovasc Surg 2003;51:62–64 5. Gyhra A, Israel J, Santander C, et al. Schwannoma of the brachial plexus with intrathoracic extension. Thorax 1980;35:703–704 6. Kline DG, Hudson AR. Tumors involving nerve. In: Kline DG, Hudson AR, eds. Nerve Injuries: Operative Results for Major Nerve Injuries, Entrapments, and Tumors. Philadelphia: WB Saunders; 1995:525–574 7. Gilmer-Hill HS, Kline DG. Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature. Neurosurgery 2000;46:1498–1503 8. Rubio Correa I, Monje F, Gonza´lez-Garcı´a R, et al. Lateral cervical approach combined with posterior thoracotomy for the treatment of giant neurilemmoma of thechest involving the neck. J Oral Maxillofac Surg 2013;71:433–436
Recurrent Atypical Fibroxanthoma Versus Malignant Fibrous Histiocytoma Leslie G. Branch, MD, John G. Albertini, MD,y and Barry Leshin, MDy
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Abstract: Atypical fibroxanthoma (AFX) has been characterized variously as a benign noninvasive neoplasm with rare recurrence. We report 2 cases of recurrent AFX. There is ongoing controversy over the diagnosis of AFX versus malignant fibrous histiocytoma (MFH) in the clinical context of recurrent AFX. Histopathologic diagnosis of fibrohistiocytic neoplasms can be quite challenging and small foci of cancer can be easily overlooked. This is particularly problematic when scarring associated with recurrent tumors is present. The utility of en face sections, as utilized in Mohs micrographical surgery, and the employment of immunohistochemical stains may be helpful in diagnosis. Recurrence of AFX can occur but is difficult to distinguish from MFH. Key Words: Atypical fibroxanthoma, malignant fibrous histiocytoma, recurrent atypical fibroxanthoma
A
typical fibroxanthoma (AFX) is usually a firm erythematous ulcerated cutaneous nodule measuring 1.5 to 2 cm in diameter. AFX occurs most frequently on sun-exposed areas of the head and neck in elderly Caucasian males.1–4 AFX is thought to be a superficial variant of malignant fibrous histiocytoma (MFH) and is characterized by an indolent growth pattern confined to the dermis. Lesions with similar histologic appearance and immunohistochemical profile but extending into the subcutaneous tissue with necrosis, intravascular invasion, or neural invasion are considered MFH.1,3–7 Others regard AFX and MFH as distinct entities due to their differing risk factors or immunohistochemical markers and have reclassified cases of recurrent AFX as MFH.5,6 We report 2 cases of recurrent AFX and discuss the diagnosis of AFX versus MFH in the clinical context of recurrent AFX.
PATIENT 1 A 73-year-old male patient presented with a 1.1 0.8 cm superficially eroded nodule on the left temple (Fig. 1A) excised by Mohs micrographic surgery (MMS). Two years later, the patient presented with a recurrent 3.5 2.5 cm mass 2 cm below the scar on the left temple. Due to its depth of invasion, the lesion was reclassified as MFH and excised with MMS. The patient has been free of recurrence for 8 months.
PATIENT 2 An 87-year-old male patient presented with a slowly enlarging ulcerated plaque measuring 1.5 1.4 cm on the left concha and antihelix that was previously treated with curettage and electrodessication (Fig. 1B). The lesion was excised with MMS and closed with island pedicle flap from the postauricular sulcus. The patient had 3 recurrences, each about 1 1 cm in size, over the next 10.5 months and every time MMS was used for excision. The last excision included the entire upper half of the ear and AFX with foci of perineural involvement was found. A split-thickness skin From the Department of Plastic and Reconstructive Surgery, Wake Forest Baptist Health, Winston-Salem; and yThe Skin Surgery Center, Winston-Salem, NC. Received June 25, 2014. Accepted for publication January 5, 2015. Address correspondence and reprint requests to Leslie Branch, MD, 1 Medical Center Blvd, Winston-Salem, NC 27157; E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001550 #
2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
FIGURE 3. Preoperative view of dissected components. Lateral component (LC) is transferred towards the anterior component (AC), posteromedial component (PMC) is transferred medially to align to LC.
FIGURE 4. Patient’s appearance on postoperative first year.
Germany) and skin was sutured separately by using 6.0 Prolene (Ethicon). Light dressing was applied. 6. Sutures were removed on fifth postoperative day. There were no problems on postoperative follow-up (Fig. 4).
DISCUSSION Many classifications were described for CELC.2 –4 Classifications that were described do not bring a common solution. The most appropriate classification is of Kitayama et al2 in appearance, earlobe clefts are divided into 4 groups longitudinal, transverse, triple lobe, and defective types. This classification reveals anatomical problem clearly, so that the problem can be described more accurately. There are many techniques for CELC correction,1,5–12 but only a few authors have discussed on triple lobe deformity clearly.13,14 A simple skin excision to reach lobes and transferring components together is the main principle of our technique. If lobes are very large, minimal excision can be performed. It should be noted that the different anatomical planes and poor soft tissue connection are issues that need to be tackled. Components can be presented in various ways. The most important points are to reconstruct a rounded free edge and minimal tissue sacrifice for earlobe, to establish a rounded free edge for triple lobe deformity, surgeon needs to dominate the three-dimensional anatomy and imagine how to combine the components of triple lobe.
REFERENCES 1. Fearon J, Cuadros CL. Cleft earlobe repair. Ann Plast Surg 1990;24:252–257 2. Kitayama Y, Yamamoto M, Tsukada S. Classification of congenital cleft ear lobe. Jpn J Plast Reconstr Surg 1980;23:663 3. Matsumoto K. Surgical repair of the congenital ear lobe cleft. Br J Plast Surg 1981;34:410–413 4. Yamada A, Fukuda O, Soeda S, et al. The evaluation of cleft earlobe. Jpn J Plast Reconstr Surg 1976;19:171 5. Hyakusoku H, Fumiiri M. The square flap method. Br J Plast Surg 1987;40:40–46 6. Attalla MF. Congenital earlobe cleft: a new technique for repair. Br J Plast Surg 1990;43:223–225 7. Fujiwara T, Matsuo K, Taki K, et al. Triangular flap repair of the congenital earlobe cleft. Ann Plast Surg 1995;34:402–405 8. Hochberg J, Ardenghy M. Repair of incomplete cleft earlobe. Ann Plast Surg 1996;37:170–172 9. Maral T, Tuncali D, Ozgu¨r F, et al. A technique for the repair of simple congenital earlobe clefts. Ann Plast Surg 1996;37:326–331 10. Bhandari PS. Congenital cleft earlobe: surgical repair. Plast Reconstr Surg 2001;108:986–987 11. Borman H, Deniz M, Ertas NM, et al. 7-plasty technique for the surgical treatment of congenital longitudinal ear lobe cleft. J Craniofac Surg 2008;19:1643–1644
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Volume 26, Number 4, June 2015
12. Qing Y, Cen Y, Xu X, et al. A new technique for correction of simple congenital earlobe clefts: diametric hinge flaps method. Ann Plast Surg 2013;70:657–658 13. Park C, Roh T. Button-down procedure for correction of cleft earlobe malformation. Plast Reconstr Surg 1997;99:1429–1432 14. Park C. Lower auricular malformations: their representation, correction, and embryologic correlation. Plast Reconstr Surg 1999;104:29–40
Schwannoma of the Servical Vagus with Intrathoracic Extension Selahattin Tugrul, MD, Erol Senturk, MD, Orhan O¨zturan, MD, Abdu¨laziz Ko¨k, MD,y and Nur Buyukpinarbasili, MDz Abstract: Schwannomas are the rare benign tumors of the neck. Patients developing from the brachial plexus and cervical truncus and extending to the posterior mediastinum have been reported, but for the first time the following case report describes the extension of a vagal origin schwannoma from the anterior and middle mediastinum and its extraction with thoracotomy without sternotomy. Key Words: Intrathoracic extension, neurilemmoma, vagus nerve
S
chwannomas are rare benign tumors that develop from Schwann cells located in peripheral nerve sheaths. They typically present as encapsulated, solitary, and slowly growing masses. They can develop from peripheral, cranial, or autonomous nerve sheaths in different anatomic locations, but 25–40% are detected in the head and neck regions.1 They can be independently sporadic, or found as part of a syndrome such as Neurofibromatosis 1 and 2. In the latter case, attention should be paid to other examination findings such as skin stigmata and family history. Probable diagnosis of schwannomas can be made by physical examination, ultrasound, CT, and MR, but definitive diagnosis can only be made by histopathology. According to their microscopic cellularity, schwannomas can be divided into 2 groups: Antony A or Antony B. Treatment is surgical enucleation to protect the affected nerve. Neck schwannomas can originate from the brachial and cervical plexus, sympathetic truncus, nervus facialis, nervus glossopharyngeus, nervus hypoglossus, or, quite rarely, the nervus vagus. Intrathoracic extension of a vagus-originated schwannoma has never been reported. This case report describes the symptoms of a vagus-originated giant schwannoma and the surgical extraction of its intrathoracic extension. From the Department of Otorhinolaryngology; yDepartment of Thoracic Surgery; and zDepartment of Pathology, Bezmialem Vakif University, Fatih, Istanbul, Turkey. Received January 30, 2015. Accepted for publication February 8, 2015. Address correspondence and reprint requests to Erol Senturk, MD, Department of Otorhinolaryngology, Medical Faculty, Bezmialem Vakif University, Fatih, 34080, Istanbul, Turkey. E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001704 #
2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 4, June 2015
Brief Clinical Studies
CASE REPORT A 28-year-old male patient presented to our clinic with hoarseness and swelling on the left side of his neck. History disclosed that this swelling has been present for a few years but had recently got larger. Hoarseness had started the month before. The 10 cm long and 5 cm wide mass on the left side of his neck could be moved horizontally with palpation, but not vertically. No other finding arose in physical examination. During indirect laryngoscopy, the left vocal cord was immobile in respiration and phonation. No other mass was observed in the oropharynx, nasopharynx, or hypopharynx. In ultrasonography (USG), a smooth contour solid mass was observed between the internal jugularis vein and common artery in which calcifications, fibrous septae, and nodules were present. In the contrast-enhanced neck MR, a heterogeneous mass of greater than 20 cm was detected. It started from the lower border of the parotid gland in the left anterior paracervical region, and extended to the anterior mediastinum (Fig. 1). A large number of nodular constructions were hypointense in the lesion T1 sections, hypo-hyperintensity in the T2 sections, and had peripheral contrast involvement inside the lesion. Spindle cells, considered to be part of a nerve sheath tumor, were observed during fine needle aspiration (FNA) biopsy accompanied by USG. Our otorhinolaryngology and thoracic surgery team operated on the patient with the probable of vagal schwannoma. The operation was started under general anesthesia in the posterior-lateral thoracotomy position. Left anterior thoracotomy was performed. The 7-cm-diameter mass under the phrenic nerve was dissected up to the thoracic outlet (Fig. 2). After that, the neck was positioned in the supine position and a carotid-type incision was performed. With a lateral cervical approach, the mass was dissected from vagal nerve fibers by the microsurgical technique and extracted in almost a block shape (Fig. 3). After hemostasis, a drain was placed in the neck and thorax, and the operation was completed. No electrocardiographic (EKG) abnormality or hemodynamic instability occurred during the procedure. Cheilothorax that had perhaps formed postoperatively because of damage to the thoracic duct had disappeared after 1 week on a fat-free diet. The patient was administered antibiotherapy and then discharged. Pathologic diagnosis was cellular schwannoma. Immunohistochemically, tumor cells S-100 were common positive (þ). The Ki-67 score was found to be 8% (Fig. 4). During monthly routine controls that followed, the patient had no complaint except hoarseness. Laryngoscopy disclosed left vocal cord paralysis.
FIGURE 1. T2 magnetic resonance image shows the tumor.
FIGURE 2. A picture of the mass during operation shows the intrathoracic extension of the schwannoma. D: heart. o: schwannoma.
FIGURE 3. It is a picture of the whole mass after the operation. D: intrathoracic part. o: cervical part.
#
2015 Mutaz B. Habal, MD
FIGURE 4. A, A microscopic picture of the tumor shows mild cellularity (hematoxylin and eosin; original magnification, 100). B, Immunohistochemically, tumor cells are painted S-100 common positive (þ).
DISCUSSION A nerve sheath tumor was first defined by Verocay in 1908 who called it a neurinoma.2 Neurinomas are generally benign, encapsulated, and have distinct boundaries. Because approximately one third of all schwannomas are formed in the head and neck region, they should therefore be kept in mind during differential diagnosis for neck masses. For massive neck and thorax masses, sarcoma, lymphoma, and pulmonary tumors should also be considered as well as neurogenic tumors; however, final diagnosis should be made histopathologically. Vagal schwannomas are rarely seen among head–neck schwannomas. In a series of 21 diseases listed by Langner et al, the origin of schwannoma was found to be the brachial plexus (23.8%), vagus (14.2%), and sympathetic chain (19%), or lingual, recurrent laryngeal, and cervical plexus nerves (4.7% each). No site of origin was found in 6 patients (28.6%).3 In their series, vagal schwannomas were limited to the neck. In our patient, the schwannoma revealed an intrathoracic extension. Schwannomas revealing intrathoracic extension have been defined before; however, because such schwannomas originate from the brachial or cervical plexus, they extend to the posterior mediastinum and are called ‘‘dumbbell schwannomas.’’4,5 The schwannoma in our patient revealed extension to the middle and anterior mediastinum, with proximity to the heart, phrenic nerve, and major veins. In the literature, we could find no reported patient of a vagal schwannoma revealing introthoracic extension similar to ours. A schwannoma can be considered as part of the von Reclinghausen syndrome. In an interesting and yet unknown manner, such tumors are usually located on the right side.6 In our patient, no skin stigmata or family history that could suggest this syndrome was present, and the tumor was on the opposite (ie, left) side. Cervical vagal schwannomas generally reveal asymptomatic findings such as slowly growing lateral neck masses, with hoarseness as its most common symptom. Our patient had complaints only of hoarseness and the swelling on his neck. Dyspnea, dysphagia, coughing, syncopal episodes, and Horner syndrome may accompany, but none of these symptoms was present in our patient. During the operation, cardiac arrhythmia could be observed depending on the manipulation. For a patient with a mass in the lateral area of the neck, other causes should also be considered for differential diagnosis from neurogenic tumors, including branchial cleft cyst, lipoma, lymphadenopathy, sarcoma, carotid body tumor, salivary gland tumor, and abscess. In terms of skin stigmata, a complete physical examination with ultrasonography, MR, and FNA helped in diagnosis. A neurogenic tumor originating from the vagus nerve can be shown via ultrasonography that it extends between the carotid artery and jugular vein, but MR monitoring should help clarify the situation. In our patient, the MR disclosed a heterogeneous mass with sharp edges and nodules inside of a hold contrast. Some authors recommend an FNA; however, due to possible hemorrhaging and scar forming, others do not.7 Even authors who do not suggest a biopsy reported that they biopsied their cases. We wanted to support our
e321
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Brief Clinical Studies
diagnosis with FNA before the operation, and to see that it did not cause any additional symptoms. For limited neck patients, the common treatment is gross total resection by protecting the vagal nerve with a/the lateral cervical approach. For an introthoracic extension of neurogenic tumors that originated in the brachial and cervical plexus, posterior thoracotomy is performed, also with the same lateral cervical approach.8 In this patient, we were able to extract the tumor extending to the anterior and middle mediastinum by left anterior thoracotomy after left lung extinction. No sternotomy was needed. After resection, definitive diagnosis of schwannoma can be made histopathologically and classified by 1 of 2 microscopic patterns. In the Antoni A pattern, Verocay corpuscles are present and hypercellularity and nuclear palisading are also present. Antoni B exhibits a hypocellular pattern consisting mostly of loose tissue. To support the diagnosis of either, S-100 staining can be used. In our case, the tumor was hypercellular and stained thoroughly with S-100. Vagal schwannoma should be considered in the differential diagnosis of a lateral neck mass. Hoarseness is its most known frequent symptom. Considering possible intrathoracic extension beside the neck mass, the neck and thorax should be reviewed together by MR. For intrathoracic extension, a multidisciplinary approach is necessary together with thoracic surgery. Even a tumor with more than a 5 cm intrathoracic extension can be extracted by anterior thoracotomy without sternotomy.
ACKNOWLEDGMENT The authors appreciate the contributions and editorial assistance made by S. Delacroix, a native English speaker.
REFERENCES 1. Colreavy MP, Lacy PD, Hughes J, et al. Head and neck schwannomas: a 10 years review. J Laryngol Otol 2000;114:119–124 2. Whittam DE, Morris TM. Neurilemmoma of the larynx. J Laryngol Otol 1970;84:747–750 3. Langner E, Del Negro A, Akashi HK, et al. Schwannomas in the head and neck: retrospective analysis of 21 patients and review of the literature. Sao Paulo Med J 2007;125:220–222 4. Kanzaki M, Ohtsuka T, Obara T, et al. Surgically treated dumbbell schwannoma arising in the brachial plexus with intrathoracic extension. Jpn J Thorac Cardiovasc Surg 2003;51:62–64 5. Gyhra A, Israel J, Santander C, et al. Schwannoma of the brachial plexus with intrathoracic extension. Thorax 1980;35:703–704 6. Kline DG, Hudson AR. Tumors involving nerve. In: Kline DG, Hudson AR, eds. Nerve Injuries: Operative Results for Major Nerve Injuries, Entrapments, and Tumors. Philadelphia: WB Saunders; 1995:525–574 7. Gilmer-Hill HS, Kline DG. Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature. Neurosurgery 2000;46:1498–1503 8. Rubio Correa I, Monje F, Gonza´lez-Garcı´a R, et al. Lateral cervical approach combined with posterior thoracotomy for the treatment of giant neurilemmoma of thechest involving the neck. J Oral Maxillofac Surg 2013;71:433–436
Recurrent Atypical Fibroxanthoma Versus Malignant Fibrous Histiocytoma Leslie G. Branch, MD, John G. Albertini, MD,y and Barry Leshin, MDy
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Volume 26, Number 4, June 2015
Abstract: Atypical fibroxanthoma (AFX) has been characterized variously as a benign noninvasive neoplasm with rare recurrence. We report 2 cases of recurrent AFX. There is ongoing controversy over the diagnosis of AFX versus malignant fibrous histiocytoma (MFH) in the clinical context of recurrent AFX. Histopathologic diagnosis of fibrohistiocytic neoplasms can be quite challenging and small foci of cancer can be easily overlooked. This is particularly problematic when scarring associated with recurrent tumors is present. The utility of en face sections, as utilized in Mohs micrographical surgery, and the employment of immunohistochemical stains may be helpful in diagnosis. Recurrence of AFX can occur but is difficult to distinguish from MFH. Key Words: Atypical fibroxanthoma, malignant fibrous histiocytoma, recurrent atypical fibroxanthoma
A
typical fibroxanthoma (AFX) is usually a firm erythematous ulcerated cutaneous nodule measuring 1.5 to 2 cm in diameter. AFX occurs most frequently on sun-exposed areas of the head and neck in elderly Caucasian males.1–4 AFX is thought to be a superficial variant of malignant fibrous histiocytoma (MFH) and is characterized by an indolent growth pattern confined to the dermis. Lesions with similar histologic appearance and immunohistochemical profile but extending into the subcutaneous tissue with necrosis, intravascular invasion, or neural invasion are considered MFH.1,3–7 Others regard AFX and MFH as distinct entities due to their differing risk factors or immunohistochemical markers and have reclassified cases of recurrent AFX as MFH.5,6 We report 2 cases of recurrent AFX and discuss the diagnosis of AFX versus MFH in the clinical context of recurrent AFX.
PATIENT 1 A 73-year-old male patient presented with a 1.1 0.8 cm superficially eroded nodule on the left temple (Fig. 1A) excised by Mohs micrographic surgery (MMS). Two years later, the patient presented with a recurrent 3.5 2.5 cm mass 2 cm below the scar on the left temple. Due to its depth of invasion, the lesion was reclassified as MFH and excised with MMS. The patient has been free of recurrence for 8 months.
PATIENT 2 An 87-year-old male patient presented with a slowly enlarging ulcerated plaque measuring 1.5 1.4 cm on the left concha and antihelix that was previously treated with curettage and electrodessication (Fig. 1B). The lesion was excised with MMS and closed with island pedicle flap from the postauricular sulcus. The patient had 3 recurrences, each about 1 1 cm in size, over the next 10.5 months and every time MMS was used for excision. The last excision included the entire upper half of the ear and AFX with foci of perineural involvement was found. A split-thickness skin From the Department of Plastic and Reconstructive Surgery, Wake Forest Baptist Health, Winston-Salem; and yThe Skin Surgery Center, Winston-Salem, NC. Received June 25, 2014. Accepted for publication January 5, 2015. Address correspondence and reprint requests to Leslie Branch, MD, 1 Medical Center Blvd, Winston-Salem, NC 27157; E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001550 #
2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
