International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 4 , Pages 267-270, December 2006

Autoamputation of an extranasopharyngeal angiofibroma of the nasal septum

  • Michael P. Castillo

      Affiliations

    • Department of Otolaryngology-Head & Neck Surgery, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas, 5323 Harry Hines Blvd., Dallas, TX 73235-9035, United States
    • ,
  • Charles F. Timmons

      Affiliations

    • Department of Pathology, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas, Dallas, TX, United States
    • ,
  • John E. McClay

      Affiliations

    • Department of Otolaryngology-Head & Neck Surgery, University of Texas Southwestern Medical Center, Children's Medical Center of Dallas, 5323 Harry Hines Blvd., Dallas, TX 73235-9035, United States
    • Corresponding Author InformationCorresponding author.

Received 6 April 2006; received in revised form 28 May 2006; accepted 28 May 2006.

Article Outline

Summary 

Angiofibromas arising from outside the nasopharynx are rare but have been reported from a variety of sites in the head and neck. We report the case of an angiofibroma originating from the nasal septum and which underwent spontaneous autoamputation prior to intervention.

Keywords: Angiofibroma, Septum, Extranasopharyngeal angiofibroma

 

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1. Introduction 

Angiofibromas normally present as a nasopharyngeal mass arising from the pterygomaxillary fossa behind the maxillary sinus in an adolescent male. Rarely, they may present as an intranasal mass appearing outside of the nasopharynx. These aberrantly arising lesions behave differently from traditional juvenile nasopharyngeal angiofibromas (JNAs) and are seen commonly in females and older patients [1]. We present the case of an extranasopharyngeal angiofibroma (ENA) that arose from the anterior septum in a preteen male and was spontaneously expelled following autoamputation. This is the fifth case reported of an angiofibroma of the nasal septum and the first that we are aware of to resolve in this fashion.

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2. Case report 

A 9-year-old Asian male was referred to our clinic with a several month history of left sided nasal obstruction and recurrent epistaxis. Exam showed a pink-tan mass at the entrance of the left nostril. A nasal endoscope could not be passed on that side. There was no cervical adenopathy and the child had no history of any bleeding disorder.

A CT scan without contrast showed a left nasal cavity mass measuring 4cm×1.8cm filling the anterior and mid nasal cavity, and protruding through the nostril. An MRI with gadolinium showed intense enhancement of this mass and the presence of flow voids (Fig. 1). The mass had no involvement of the pterygopalatine fossa or nasopharynx.

Following these studies and before operative intervention the child presented to our emergency room with an intense episode of epistaxis not relieved by standard measures. En route to the ER the child felt the mass change position in his nose and subsequently felt it pass into his oral cavity. The child was able to pass this mass from his mouth without compromising his airway. The mass was sent to pathology from the ER. Physical exam in the ER of his nasal cavity reveled dark scab in his anterior left nares and a stalk at the anterior portion of his left nasal septum. There was no active bleeding and no intervention was necessary. Initial laboratories showed a hematocrit of 25.

Gross examination documented a 6cm×3cm×2cm pink-tan soft tissue mass with multiple areas of mucosal ulceration and green-tan purulent exudates. Sectioning of the specimen revealed a hemorrhagic interior with an area of necrosis and acute inflammation at the tip, corresponding to an ulceration. The microscopic appearance featured multiple thin-walled vascular channels of varying sizes separated by a fibromyxoid stroma containing fusiform and stellate cells with oval nuclei, lacking atypia or mitotic activity (Fig. 2). Focal necrosis at the tip, with acute inflammation and slight hemorrhage, corresponding to the gross appearance of the lesion, was confirmed. A thrombus was present in an occasional vessel; however, the mass, as a whole, was still viable with no histological evidence of a proximal site where the lesion thrombosed or outgrew its blood supply to autoamputate due to infarction.

  • View full-size image.
  • Fig. 2. 

    (H&E, 10×) The microscopic appearance of the mass consisting of multiple thin-walled vascular channels of varying sizes separated by a fibromyxoid stroma containing fusiform and stellate cells with oval nuclei, lacking atypia or mitotic activity.

He was discharged from the emergency room and treated with iron supplementation for 4 weeks with resolution of his anemia. Follow up examination of his left nasal septum showed improvement (Fig. 3) at 2 weeks and resolution at 4 weeks of his residual tumor stalk. There has been no evidence of any residual masses or recurrence on serial exams in his 9 month follow-up.

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3. Discussion 

Angiofibromas arising outside the nasopharynx are rare, with a total of 65 cases reported in the literature. The most common site of origin is the maxilla but involvement of many structures in the head and neck have been reported, including the ethmoid sinus, nasal cavity, septum, larynx, sphenoid, cheek, conjunctiva, oropharynx, tonsil, retromolar area, middle turbinate and inferior turbinate [1]. There have been 4 prior reports of septal angiofibromas and each was successfully treated surgically [1], [2], [3], [4]. There are no reports of a spontaneous expulsion.

Several cases, however, of spontaneous regression of nasopharyngeal angiofibromas have been published. Weprin and Siemers reported on a child with a biopsy proven JNA arising from the nasopharynx who was followed for 12 years without any surgical intervention and whose lesion showed involution on serial radiography [5]. Herman et al. reported on nine patients who were known to have residual tumor following resection of a JNA and whose remnants either stabilized or involuted on follow-up CT scans [6]. No cases of extranasopharyngeal angiofibromas (ENAs), have been reported to resolve without intervention [1].

Autoamputation of oral, nasal, gastrointestinal and pulmonary lesions have rarely been reported. Antrochoanal polyps have been noted to strangulate and subsequently autoamputate [7], [8], [9]. Pruna et al. reviewed CT scans both before and after passage of an antrochoanal polyp following a vigorous sneeze in a 41-year-old male [8]. Rashid et al. described a 13-year-old asthmatic female who passed a fleshy mass from her mouth discovered to be an ischemic antrochoanal polyp [9].

There have been similar reports involving various types of lesions of the respiratory tract. De Caluwe et al. noted the spontaneous passage of a dermoid from the oropharynx in an 11-week-old baby that resulted in resolution of respiratory distress [10]. Terashima et al. reported a case of an 18-year-old female who coughed up a bronchial adenoma after spontaneous autoamputation [11]. There are several reports of gastrointestinal lipomas and polyps that have spontaneously detached as well [12], [13], [14]. Autoamputation of a nasal angiofibroma has not previously been reported in the literature.

A differential diagnosis for vascular lesions of the nose should include lobular capillary hemangioma (LCH, or pyogenic granuloma), angiomatous polyps, neurofibromas, and hemangiopericytomas. LCH is a hemorrhagic lesion most commonly seen on the skin of pregnant females but can occur on nasal mucosa in adults and children as well [15], [16], [17]. El-Sayed and Al-Serhani reviewed 12 cases of nasal LCH, of which seven arose from the septum [16]. One of these lesions was originally diagnosed as an angiofibroma on pathology and only correctly termed LCH upon re-examination. Histologically, these lesions show capillaries arranged in a lobular fashion surrounded by larger vessels [16], [17]. This lesion in this Asian male was clearly an ENA and not a LCH because it lacked the lobular pattern of vascular proliferation characteristic of LCH.

Angiomatous polyps, which arise from antrochoanal polyps after vessel occlusion, infarction and neovascularization, have also been confused with angiofibromas [18], [19]. MRI findings show the portion of polyp without vascular compromise to be nonenhancing – like a simple polyp – while the portion with vascular compromise shows strong enhancement [20]. Again, histologically, this lesion was more consistent with an angiofibroma than an angiomatous polyp because it lacked the inflammatory component characteristic of angiomatous and antrochoanal polyps.

Neurofibromas may present as a nasal mass but show irregular enhancement with contrast on radiography and on pathology they have a collagenous matrix with spindle cells [21], [22], not dilated vascular channels. Hemangiopericytomas originate from extravascular cells called pericytes and can arise in the nasal cavity. Pathologically these tumors have only a small amount of interstitial stroma with many spindle-shaped cells and dilated vascular spaces [23], while the lesion in this Asian male had fewer cells but more interstitial stroma between his dilated channels.

ENAs clinically act differently from JNAs. A review of all prior reported ENAs by Windfuhr found 27% occur in females and no reports of recurrences [1]. JNAs on the other hand only rarely affect females [24]. Additionally, large JNAs with extension towards or invasion of the skull base have a significant rate of recurrence [6], [25]. The average age of patients with ENAs is 22, while the average age for patients with JNAs is 16 [2], [25], [26]. ENAs tend to be diagnosed earlier than JNAs, often within 6 months, as they arise in more exposed areas of the head and neck [1], [27], [28].

Surgery is the treatment of choice for angiofibromas and has been noted to successfully treat nearly all cases of ENAs [1], [2]. Angiofibromas that arise in the nasal cavity appear ideal for endoscopic resection. It is unclear what role preoperative embolization plays in the treatment of ENAs. Most recently, Somdas et al., reported good results using preoperative embolization prior to excision of a septal angiofibroma via a lateral rhinotomy approach [4]. In this case, our plan prior to autoamputation was to consider preoperative angiography with embolization followed by endoscopic excision of this mass. Follow up examination is essential in these patients to monitor for any signs of recurrence.

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References 

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PII: S1871-4048(06)00062-1

doi:10.1016/j.pedex.2006.05.007

International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 4 , Pages 267-270, December 2006

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