Recurrent pterygo-palatal angiofibroma with intracranial extension: case report

Abstract

We report the case of a patient with recurrent pterygo-palatal angiofibroma and its treatment. A 21-year-old male patient had a long history of recurrent epistaxis with progressive nasal obstruction. He was diagnosed with an angiofibroma centered in the right pterygo-palatine fossa. Initially, he underwent surgical excision with removal of the entire tumor. The evolution was clinically good with no signs of recurrence on the cervico-facial scan of control (CT). Nine months after, he presented a reappearance of epistaxis. A cervico-facial MRI was performed and showed a recurrence of the tumor process, which this time was considered inextirpable, hence the decision to opt for radiotherapy with intensity modulated radiation therapy (IMRT). He has improved clinically with a clear reduction in tumor mass on CT scan. This technique represents an interesting alternative to overcome anatomical complexity of the region, cover the tumor and preserve the organs at risk.

Diode Laser Management of Primary Extranasopharyngeal Angiofibroma Presenting as Maxillary Epulis: Report of a Case and Literature Review

“…In fact, JNAs are occasionally detected as nasopharyngeal masses on radiograms taken for other medical problems, or manifest unspecific signs such as unilateral nasal obstruction, recurrent epistaxis, etc. [ 6 , 9 , 34 ]. At more advanced stages, patients may show facial swelling, cranial neuropathy, proptosis and limitation of mouth opening [ 1 , 2 , 3 , 5 , 6 , 7 , 33 , 34 ].…”

“…In addition, a biopsy is generally contraindicated in such instances because of the risk of potentially fatal bleeding. Consequently, the diagnosis essentially is based on matching clinical and radiological (CT, MRI, angiography) findings [ 6 , 7 , 9 , 10 , 35 ].…”

“…JNA has been alternatively interpreted as a hamartoma [ 40 , 55 , 56 , 57 , 58 ], a vascular malformation (related to incomplete regression of the first branchial arch artery) [ 28 ], overgrowth of paraganglionic tissue [ 40 ] or extracolonic manifestation of familial adenomatous polyposis. [ 2 , 4 , 8 , 10 , 11 , 40 , 55 , 57 , 58 ] To date, according to the most accepted theory [ 8 , 9 , 10 , 11 , 56 , 57 , 58 , 59 ], JNA may be the consequence of repeated microhemorrhages followed by repair by granulation tissue and subsequent fibrous tissue deposition, and possibly due to sexual hormone stimulation of the vascular erectile tissues located in the proximity of the sphenopalatine foramen.…”

“…Morphologically, JNA would fit the criteria for benign mesenchymal neoplasms but may show a locally aggressive clinical course in view of local extension into the adjacent tissues, which often precludes complete surgical removal, with possible tumor persistence and recurrences in a relatively high number of patients (20%) [ 4 , 5 ]. This represents a true challenge for JNA management, as such tumors frequently arise in the posterior-lateral wall of the nasal cavity close to the superior margin of the sphenopalatine foramen and may cause bone erosion and displacement of adjacent structures [ 5 , 6 , 7 , 8 , 9 , 10 ]. There is accumulating evidence that a morphologically similar angiofibroma, namely extranasopharyngeal angiofibroma (ENA), should be considered as a distinct subtype.…”

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“…J uvenile Angio bromas (JAs), also referred to as Juvenile Nasopharyngeal Angio bromas (JNA) or Pterygopalatine Angio bromas, represent rare benign tumors originating from connective tissue, as indicated by histological ndings. 1,2 The nomenclature is assigned based on the tumor's clinical location. However, JAs occasionally manifest in regions beyond the nasopharynx, including the maxillary sinus area, nasal and paranasal cavities, palatal region, or the mandibular region, and are thus classi ed as Extra-Nasopharyngeal Angio bromas (ENA).…”