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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 51-54

Neurofibroma in the head-and-neck region: Our experience


Department of Otorhinolaryngology, Gauhati Medical College and Hospital, Guwahati, Assam, India

Date of Web Publication1-Mar-2018

Correspondence Address:
Dr. Kalpana Sharma
Department of Otorhinolaryngology, Gauhati Medical College and Hospital, Guwahati, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijhas.IJHAS_97_17

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  Abstract 


Neurofibroma in the head-and-neck region is a rare entity. It can occur either as a solitary, slowly progressive tumor or as part of neurofibromatosis. We here report three cases of neurofibroma in the head-and-neck region. Case 1 is a 20-year-old female with a solitary neurofibroma in trachea. Case 2 is a 35-year-old male with neurofibromatosis type 1 and spontaneous hemorrhage in the neurofibroma of the facial region. Case 3 is a 25-year-old female with a solitary neurofibroma in the left posterior triangle of the neck. Multiple neurofibroma in the head-and-neck region may occur in the skin as part of neurofibromatosis and as solitary lesions in intratracheal to posterior triangle region of the neck.

Keywords: Hematoma, head-and-neck, neurofibroma, posterior triangle, trachea


How to cite this article:
Sharma K, Barman D. Neurofibroma in the head-and-neck region: Our experience. Int J Health Allied Sci 2018;7:51-4

How to cite this URL:
Sharma K, Barman D. Neurofibroma in the head-and-neck region: Our experience. Int J Health Allied Sci [serial online] 2018 [cited 2021 Jan 20];7:51-4. Available from: https://www.ijhas.in/text.asp?2018/7/1/51/226264




  Introduction Top


Aneurofibroma is a benign nerve sheath tumor in the peripheral nervous system. The most common form occurs in the skin (cutaneous neurofibroma) or in peripheral nerve (solitary neurofibroma). These arise sporadically or in association with neurofibromatosis type 1 (NF1), an autosomal dominant genetically inherited disease. Plexiform neurofibroma is considered by some to occur only in association with NF1. Neurofibromas arise from nonmyelinating type Schwann cells that exhibit biallelic inactivation of NF1 gene that codes for the protein neurofibromin.[1] Neurofibroma in the head-and-neck region is less common. Here, we present three rare cases of neurofibroma in the head-and-neck region.


  Case Reports Top


Case report 1

A 20-year-old female was admitted to our hospital with a history of a dry cough for the past 2 years and with wheeze and dyspnea on exertion for the past 1 year. Dyspnea was gradually progressive in nature, the symptom of dyspnea got aggravated for the past 1 month. There was no associated history of dysphagia, hemoptysis, or aspiration symptoms. General examination was normal with no evidence of neurofibromatosis. On chest examination, bilateral wheeze was present. Her head-and-neck and cardiovascular examinations were normal. She received initial treatment with antibiotic, bronchodilators, and dexamethasone, but symptoms did not get relieved. On flexible bronchoscopy showed a round well-defined nodular mass arising from the right posterolateral wall of trachea obliterating the three-fourth area of tracheal airway. X-ray soft-tissue neck lateral view revealed a mass in the trachea at C7 level [Figure 1]a. Contrast-enhanced computed tomography (CT) scan of the neck and thorax showed homogeneously enhancing intraluminal soft-tissue mass measuring 14 mm × 14 mm × 11.6 mm in the trachea arising from the right lateral wall at the level of C7 and D1 [Figure 1]b, with pneumothorax, pneumomediastinum, and surgical emphysema. The mass was excised under local anesthesia. The postoperative period was uneventful, and decannulation was done on the 7th postoperative day. Histopathology examination (HPE) report shows a tumor mass lined partly by respiratory epithelium composed of elongated, spindle-shaped Schwann cells arranged in sheets, intercalating fascicles, and plexiform pattern, in a deeply eosinophilic fibrillary background. Stroma shows mild infiltration by lymphocytes, mast cells histiocytes, and few hemosiderin-laden macrophages at places features suggestive of neurofibroma [Figure 2].
Figure 1: (a) The X-ray image showing soft-tissue mass at C7 level. (b) Axial computed tomography scan showing intratracheal soft-tissue mass

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Figure 2: Photomicrograph showing spindle cells arranged in sheets, intersecting fascicles, and plexiform pattern

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Case report 2

A 35-year-old male patient of neurofibromatosis was reported to our department with a history of a fast-growing mass in the left facial region. The patient noticed the mass suddenly at night. There was no history of trauma prior. There was also no associated history of pain. On physical examination, there was a soft mass measuring 8 cm × 7 cm × 5 cm 3 with tenderness on palpation. The mass was grayish-brown colored, nonpulsating, no bruit with well-demarcated border. Café-au-lait spots were noted on the body. CT scan revealed well-defined homogenous high-density images measuring 8 cm × 7.2 cm × 5.4 cm in the right facial region with cystic changes. Surgical resection under general anesthesia was done. A hematoma of approximate 300 ml dark red-colored blood clot was evacuated from the fragile tumor. There were also cystic changes in the lesion. Subsequent histopathological examination revealed fibrocollagenous cyst wall lined by flattened, necrotic, and hemorrhagic epithelium. Cyst wall stroma showed dense infiltration by sheets of hemosiderin-laden macrophages. Surrounding fibrocollagenous tissue shows dense infiltration by chronic inflammatory cells, including focal aggregates of multinucleated giant cells at places.

Case report 3

A 25-year-old female patient presented with a history of swelling in the left posterior triangle of the neck for the past 2 years. The swelling was gradually progressive with an associated history of mild pain over the swelling. On local examination, there was 10 cm × 8 cm, firm in consistency, nontender mass in the left posterior triangle of the neck. CT scan of the neck shows well-defined oval mass lesion measuring 6.3 cm × 5.9 cm × 3.8 cm in the left posterior triangle of the neck [Figure 3]. Fine-needle aspiration cytology of the mass shows few individually lying spindle cells along with lymphocytes and excision biopsy, and histopathology was advised by the pathologist. The mass was excised by surgical exploration, by incision at left lower neck skin crease and by meticulous dissection the tumor from its blood supply was removed. Excision was carried out under general anesthesia. The mass was grayish-white in color, encapsulated, fleshy measuring 6 cm × 5 cm × 3.5 cm, and on the cut surface, it appears relatively avascular. Histopathological examination report shows tumor mass composed of sheets of elongated spindle-shaped Schwann cells arranged in interlacing bundles, in a pale eosinophilic fibrillary background. Stroma shows few dilated and engorged vascular channels. The postoperative period was uneventful. The suture of the wound was removed on the 7th postoperative day and patient was discharged.
Figure 3: Axial computed tomography scan showing the location of tumor in the neck

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  Discussion Top


A neurofibroma in the head-and-neck region is a very rare tumor. These lesions present as slowly progressive masses that can either compress to the adjacent vital structures or interfere with normal physiology.[2] Primary tumor of the trachea is very rare. These may cause airway obstruction, in many cases, the progressive symptoms of airway obstruction are misdiagnosed with asthma or chronic bronchitis.[3] There were only 23 cases of tracheobronchial neurofibromas reported worldwide.[4] The tracheal tumor is to be kept in mind for differential diagnosis during the workup of adult-onset dyspnea and wheeze.[5] In our case as well (case 1) presented with a history of a cough, wheeze, and dyspnea on exertion. First, case had pneumothorax due to chronic airway obstruction and CT scan was done before fiberoptic bronchoscopy. The patient was earlier admitted in the Department of Medicine before her examination in the Department of Otorhinolaryngology. Treatment of tracheal tumor depends on size and location of tumor. The small tumor can be removed endoscopically with or without laser, however, if tumor invades adjacent tissue open surgery should be carried out.[4] High location of the intratracheal tumor, difficult intubation from above the mass, anticipated difficulty in endoscopic resection without a tracheostomy, and possibility of resection through the same vertical tracheostomy incision without general anesthesia should be considered before taking up the surgery under general anesthesia.[6] In our case 1, the patient was also having pneumothorax, pneumomediastinum, and surgical emphysema for which cardiothoracic vascular surgeons opinion was taken and was managed conservatively and intratracheal nodular mass 15 mm × 14 mm × 11.6 mm size was excised by vertical tracheostomy incision under general anesthesia. Patients with neurofibromatosis one have increased the chance of developing a benign and malignant tumor, which may in some cases infiltrate and lead to compression of vital structures and evident deformities.[7] Our case 2 is a case of hematoma in the facial region. Etiology of the hemorrhage is unknown, hemorrhage in NF1 has been thought to result from friable vasculature secondary to arterial dysplasia or vascular invasion by the neurofibroma.[8] The tumor tissue itself also has an abnormal vasculature structure with thin-walled ecstatic blood vessels lying in loose neural stroma that replaces normal adipose tissue.[9] Attenuated platelet sensitivity to collagen in patients with NF1 contributing to a massive intratumoral hemorrhage and excessive bleeding during operation has also been suggested.[10] Destruction of the mural layer could has been the cause of massive hemorrhage in NF1.[11] The second case was relevant to present because the patient had Type I neurofibromatosis. Blood tests showed normal laboratory findings and peroperatively tumor tissue was fragile with cystic changes. Only HPE revealed the diagnosis of a hemorrhagic cyst. Tumor excision and ligation represent viable options for treatment, but open surgery is invasive, and hemostasis is often difficult to achieve using sutures due to fragile nature of vascular tissue in NF1.[12] In our case, we did surgical excision by open procedure and hemostasis was achieved by ligation. In case 3, presenting symptom was swelling in the left lateral neck and mild pain over the swelling without any feature of compression of the neurovascular structure. The tumor was excised under general anesthesia by an open procedure. As it is a benign tumor, complete excision is the treatment of choice. There were no systemic or ophthalmic features in the three cases presented in this report. Café-au-lait spots were present in the second case but absent in first and third cases.


  Conclusion Top


Neurofibroma in the head-and-neck region is a very rare clinical entity, but it should be considered in the differential diagnosis of head-and-neck swellings. Multiple neurofibroma in the head-and-neck region may occur in the skin as part of neurofibromatosis and as solitary lesions from intratracheal to posterior triangle region of the neck.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Muir D, Neubauer D, Lim IT, Yachnis AT, Wallace MR. Tumorigenic properties of neurofibromin-deficient neurofibroma Schwann cells. Am J Pathol 2001;158:501-13.  Back to cited text no. 1
    
2.
Sahota JS, Viswanathan A, Nayak DR, Hazarika P. Giant neurofibroma of the tongue. Int J Pediatr Otorhinolaryngol 1996;34:153-7.  Back to cited text no. 2
    
3.
Wood DE. Management of malignant tracheobronchial obstruction. Surg Clin North Am 2002;82:621-42.  Back to cited text no. 3
    
4.
Nichkaode PB, Umalkar R, Tulaskar N, Panchbhai K, Zamad R. A rare case of endobronchial neurofibroma. J Evol Med Dent Sci 2013;2:2974-9.  Back to cited text no. 4
    
5.
Parrish RW, Banks J, Fennerty AG. Tracheal obstruction presenting as asthma. Postgrad Med J 1983;59:775-6.  Back to cited text no. 5
    
6.
Savana M, Jiten N, Birjit O, Sudheeranjan TH, Sobita P, Anita N. Neurofibroma of trachea: A case report. IOSR J Dent Med Sci 2015;14:53-5.  Back to cited text no. 6
    
7.
Rapado F, Simo R, Small M. Neurofibromatosis type 1 of the head and neck: Dilemmas in management. J Laryngol Otol 2001;115:151-4.  Back to cited text no. 7
    
8.
Rao V, Affifi RA, Ghazarian D. Massive subcutaneous hemorrhage in a chest-wall neurofibroma. Can J Surg 2000;43:459-60.  Back to cited text no. 8
    
9.
Poston GJ, Grace PA, Venn G, Spencer J. Recurrent near-fatal haemorrhage in von Recklinghausen's disease. Br J Clin Pract 1990;44:755-6.  Back to cited text no. 9
    
10.
Rasko JE, North KN, Favaloro EJ, Grispo L, Berndt MC. Attenuated platelet sensitivity to collagen in patients with neurofibromatosis type 1. Br J Haematol 1995;89:582-8.  Back to cited text no. 10
    
11.
Sun ZJ, Zhao YF, Wang SP, He SG. Giant facial haematoma in neurofibromatosis type 1. Dentomaxillofac Radiol 2008;37:52-7.  Back to cited text no. 11
    
12.
Saijo H, Hayashida K, Morooka S, Kuwabara K, Fujioka M. Transcatheter arterial embolization for shock caused by intratumoral hemorrhaging in neurofibromatosis type 1: A report of two cases. Case Rep Dermatol 2014;6:59-65.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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