Abstract
Objectives
To describe a clear and simplified classification system for juvenile nasopharyngeal angiofibroma (JNA), and to describe suitable management options.
Study design
Retrospective medical record review.
Methods
The clinical and imaging materials of 51 cases of JNA diagnosed at our hospital between 1981 and 2011 were collected and studied. Based on our experiences, we prefer to divide JNAs into three types. Type I includes JNAs fundamentally localized to the nasal cavity, paranasal sinus, nasopharynx, or pterygopalatine fossa. Type II is a JNA extending into the infratemporal fossa, cheek region, or orbital cavity, with anterior and/or minimal middle cranial fossa extension but intact dura mater. Type III is a calabash-like massive tumor lobe in the middle cranial fossa. The management and prognosis for the three types of JNA were compared and evaluated.
Results
Among cases of type I JNA ( n = 16), the entire mass was removed by the initial operation in 15 cases and by a repeat operation in 1 case. Among cases of type II JNA ( n = 29), the entire mass was removed by the first operation in 24 cases and by repeat operation in 5 cases. In cases of type III JNA ( n = 6), the huge calabash-like lobe in the middle cranial fossa could not be completely excised; 4 cases underwent radiotherapy and 2 cases were lost to follow-up.
Conclusions
1) The transnasal cavity approach with endoscopic guidance is suitable for type I JNA resection. 2) The transantral–infratemporal fossa–nasal cavity combined approach is reliable for resection of a type II JNA, which extends into the deep anterior cranial fossa and/or minimally into the middle cranial fossa, with intact dura mater. 3) The complete removal of a type III JNA is difficult, even through a combined extracranial and intracranial approach. Radiotherapy is useful for treating the residual intracranial tumor. The successful or failed experiences of 6 typical cases prove that this revised classification system is reasonable and reliable.
1
Introduction
Juvenile nasopharyngeal angiofibroma (JNA) occurs primarily in adolescent males and accounts for 0.05% of all head and neck neoplasms. Sessions et al , Fisch , Chandler et al , Radkowski et al , and Onerci et al have proposed staging or classification systems for JNA. Previously, we used Fisch’s system to study the diagnosis and management of JNAs . However, we feel that defining some JNAs using the previously proposed typing and staging protocols can be difficult. Based on our retrospective research of 51 cases, we prefer to classify JNAs into three types, simplifying clinical treatment. Table 1 contains our classification schemes for comparing with other classification schemes in the literatures. The Table 2 is used for introducing our treatment recommendation and the prognosis of our patients. Meanwhile, we present six cases here to characterize this problem and its solution sufficiently. Type I tumors were removed by endoscopy via the nasal cavity approach, type II JNAs by a transantral–infratemporal fossa–nasal cavity combined approach via an extended Caldwell–Luc incision, and type III JNAs by a combined intracranial and extracranial approach. However, open craniotomy for resection of a calabash-like huge tumor lobe in the middle cranial fossa remains difficult. Complementary radiotherapy is indicated for intracranial tumor remnants in such cases.
| Type or stage | Fisch 1983 | Chandler et al. 1984 | Sessions et al. 1981 | Radkowski et al. 1996 | Onerci et al. 2006 | Revised | ||
|---|---|---|---|---|---|---|---|---|
| 1 | Limited to nasopharynx and nasal cavity | Tumor confined to NV | IA | Limited to nose and/or NV | Same as in Sessions et al. | Nose, NV, sinus, or minimal ext. to PMF | Localized in nasal cavity, nasopharynx, sinus, PMF. Minimal extension in ITF, orbit, or cranial fossa. | |
| IB | One or more sinuses | Same as in Sessions et al. | ||||||
| II | PMF, maxillary, ethmoidal, and sphenoidal sinuses | Tumor ext. into nasal cavity or sphenoidal sinus | IIA | Minimal ext. into PMF, full PMF | Same as in Sessions et al. | Maxillary sinus, full occupation of PMF, ext. to anterior cranial fossa, and limited ext. to ITF | Localized in ITF, cheek, deep or minimal ACF extension, minimal MCF extension. With or without cavernous sinus and ICA compression, but dura mater intact | |
| IIB | PMF with or without erosion of orbital bones | Same as in Sessions et al. | ||||||
| IIc | ITF with or without cheek | Or posterior to pterygoid plates | ||||||
| III | ITF, orbit and parasellar region remaining lateral to cavernous sinus | Antrum, ethmoidal sinus, PMF, ITF, orbit, and/or cheek | III | Intracranial extension | IIIA | Erosion of skull base, minimal intracranial | Deep. ext. into cancellous bone at base of pterygoid or body and GWS, significant lateral ext. to ITF or to pterygoid plates posteriorly or orbital region, cavernous sinus obliteration | From PMF and superior orbital fissure extending into MCF as a large gourd-shaped lobe. |
| IIIB | Extensive intracranial with or without cavernous sinus | |||||||
| IV | Cavernous sinus, optic chiasm, or pituitary fossa region | Intracranial tumor | Intracranial ext. between pituitary gland and ICA, tumor localization lateral to ICA, middle fossa ext., and extensive intracranial ext. | |||||
| Type | Tumor size | Case | Surgical approach | Blood loss (ml) | Median blood loss (ml) | Result | Follow-up |
|---|---|---|---|---|---|---|---|
| I | Localized in nasal cavity, nasopharynx, sinus, PMF. Minimal extension in ITF, orbit, or cranial fossa. | 16 | Transnasal cavity approach | 20–1500 | 437 | Removed by first ( n = 15) or repeated ( n = 1) operation | 1–17 years |
| II | Localized in ITF, cheek, deep or minimal ACF extension, minimal MCF extension. With or without cavernous sinus and ICA compression, but dura mater intact | 29 | Transantral–infratemporal fossa–nasal cavity approach | 100–8000 | 1893 | Removed by first ( n = 24) or repeated ( n = 5) operation | 1–25 years |
| III | From PMF and superior orbital fissure extending into MCF as a large gourd-shaped lobe. | 6 | EC & IC approach | 1500–5200 | 1975 | Residual tumor in MCF ( n = 6) | 5–24 years |
2
Materials, methods, and results
Between 1981 and 2011, 51 patients with JNA, aged 8–44 (mean, 22.3) years, were treated at the First Affiliated Hospital of Fujian Medical University. Sixteen type I JNAs were removed using the nasal cavity approach (under endoscopic guidance in 12 cases). Twenty-nine type II lesions were accessed via the transantral–infratemporal fossa–nasal cavity combined approach; a Caldwell–Luc incision was used in 24 cases, and methods such as lateral rhinotomy or Weber’s incision were used in 5 cases. Six type III masses were treated via the transantral–infratemporal fossa–nasal cavity combined approach for the extracranial portion, and via the temporal epidural approach (pterional craniotomy) for the intracranial portion. However, the intracranial portions of these type III tumors could not be removed completely, and 4 patients underwent radiotherapy (~ 40 Gy) with a good prognosis. Two patients were hospitalized before 1997. At that time, digital subtraction angiography (DSA) technology was not available in our hospital, and serious intraoperative blood loss resulted in the presence of residual tumor; these two patients were lost to follow-up. With the approval of the Institutional Review Board, 51 JNAs are described herein and 6 typical cases of successes or failures are reported.
2.1
Perioperative management
- 1.
Imaging examinations were performed to evaluate tumor size, arterial supply, and venous drainage.
Since 1996, computed tomography angiography (CTA) with three-dimensional reconstruction, magnetic resonance imaging (MRI), DSA, and other tests have been used to evaluate tumor size, arterial supply, and venous drainage. Two or three days after DSA-guided embolization of the external carotid artery (ECA) tumor blood supply, suitable surgical management was performed.
- 2.
Ophthalmic examinations (routine visual examinations and Schirmer’s tear tests) were performed to evaluate visual function.
- 3.
Surgical cavities were filled as necessary with iodoform or Vaseline gauze, NasoPore, or absorbable hemostatic gauze, or biological glue spray was used.
2.2
Surgical approach
Endoscopic nasal cavity approach for resection of type I JNA
- 1.
Under endoscopy, the tumor in the nasal cavity, nasopharynx, sinus, or pterygomaxillary fossa was removed via a trans-nasal cavity approach, provided it did not extend into the deep intracranial and infratemporal fossa.
- 2.
A type II JNA can be removed using a transantral–infratemporal fossa–nasal cavity combined approach via a Caldwell–Luc incision. Prior to 1986, we used this approach with different incisions, such as lateral rhinotomy or Weber’s incision. To preserve the patient’s physical appearance, we have preferred to use an extended Caldwell–Luc incision (midfacial degloving) for this combined approach since 1986. Previously, we defined this approach as a transantral approach via a midfacial degloving incision , but it can be defined more precisely as a transantral–infratemporal fossa–nasal cavity combined approach via an extended Caldwell–Luc incision . The length of the Caldwell–Luc incision is determined by the size of the tumor. The transantral approach offers the best safe and minimally invasive access to the infratemporal fossa, temporal fossa, and cheek region; the transnasal cavity approach is the best way to access the perinasal region, including the anterior and middle cranial fossae. This approach can be used to directly visualize and remove a type II JNA, allowing careful sectioning of the tumor to prevent serious bleeding and complications. The surgery can be divided into two main steps: 1) use of the transantral–infratemporal fossa approach to strip the tumor from the maxillary sinus, infratemporal and temporal fossae, and cheek region; and 2) use of the nasal cavity approach under endoscopic guidance to strip the tumor from the nasal cavity, nasopharynx, orbit, and sphenoidal sinus. Generally, the tumor lobe in the infratemporal fossa, cheek, and temporal fossa is a laterally expanding lesion extending from the pterygomaxillary fissure. Hence, an isthmus can be observed at this site by imaging and intraoperative visualization. If this huge lateral lobe impedes the surgical field, the isthmus must be tightly sutured and ligated before removal to gain a wider operative field. Afterward, endoscopy via the maxillary sinus and nasal cavity can be used to aid the removal of tumor portions localized to the pterygomaxillary fossa, nasopharynx, sphenoidal sinus, orbital cavity, and anterior cranial fossa. If the optic nerve, optic chiasm, and cavernous sinus are compressed and displaced but the dura mater is intact, the tumor can still be removed completely .
- 3.
A tumor with broad intracranial extension can be removed using a combined intracranial and extracranial approach: the extracranial portion can be accessed via the transantral–infratemporal fossa–nasal cavity combined approach, and the intracranial portion via a pterional approach.
In 8/51 cases, the tumor extended widely into the cranial fossa. The epidural pterional approach was used to explore the intracranial lobe, but no dura mater perforation was found. Two cases of type II JNA were accessed initially via the epidural pterional approach, but ultimately required the use of the transantral–infratemporal fossa–nasal cavity combined approach for complete removal. Six cases with huge gourd-shaped lobes extending into the middle cranial fossa could not be completely removed. Two cases were lost to follow-up, and four cases had intracranial tumor remnants that were treated successfully with radiotherapy.
2.3
Typical case reports: experiences and lesions of types I–III
Case 1, type I: a JNA was completely removed by an endoscopic transnasal cavity approach.
A 15-year-old boy was hospitalized because of a tumor causing bilateral nasal obstruction. CTA showed that the primary tumor was a spherical lesion in the outer wall of the left nasal cavity, extending into the nasopharynx ( Fig. 1 ). Three days after DSA-guided embolism of the branches of the bilateral ECAs supplying the mass, the tumor (5.5 × 3.5 × 3 cm) was removed completely through a transnasal cavity approach under endoscopic guidance, causing the loss of about 100 ml blood. Follow-up more than 1.5 years the patient was in good health.
Case 2, type II: a JNA extending deeply into the infratemporal fossa with minimal erosion of the middle cranial base was completely removed using a transantral–infratemporal fossa–nasal cavity combined approach.
A 17-year-old boy suffered from right nasal obstruction and intermittent epistaxis for 3 years, and was hospitalized in July 2008. The right nasal cavity contained a reddish tumor. CT imaging demonstrated a tumor occupying the nasal cavity, nasopharynx, pterygomaxillary fossa, and infratemporal fossa. The medial and posterior walls of the right maxillary sinus, as well as the right pterygoid process, were partially damaged ( Fig. 2 ). Three days after DSA-guided embolism of the bilateral ECAs, the tumor was removed using a transantral–infratemporal fossa–nasal cavity combined approach via an extended Caldwell–Luc incision. After the anterior wall of the maxillary sinus was partially excised, a semicircular prominence was revealed on the posterior wall. The mucosa and bone were stripped, revealing the smooth surface of the tumor in the infratemporal fossa. Partial destruction of the greater wing of the sphenoid and the superior orbital fissure were discovered intraoperatively. The tumor pedicle was located in the region of the pterygopalatine fossa and the sphenopalatine foramen. Due to the restriction of tumor growth by the pterygomaxillary fissure, an obvious isthmus had formed. Under direct vision and careful separation, the 5 × 4 × 3-cm tumor was removed completely. Intraoperative blood loss was about 1400 ml. Four years later, the patient was in good condition.
Case 3, type II: initial surgical resection provided easier resection of the tumor from peripheral tissue than would repeated surgical resection. Although this primary tumor was very large, the intraoperative blood loss was only 100 ml.
An 8.5-year-old boy suffered from repeated left nasal bleeding for 5 years, with obstructive sleep apnea/hypopnea syndrome (OSAHS). He was hospitalized on August 3, 2011. Physical examination revealed slight swelling on the left side of the face, and a red tumor with a smooth surface occupying the left nasal cavity and spreading into the nasopharynx. CTA showed a huge tumor in the infratemporal fossa, cheek region, pterygomaxillary fossa, nasal cavity, and nasopharynx, with slight involvement of the orbital apex and middle cranial fossa ( Fig. 3 ). Two days after DSA-guided embolization of the ECA branches supplying the tumor, a transantral–infratemporal fossa–nasal cavity combined approach via an extended Caldwell–Luc incision was used to remove it. First, the tumor lobe in the infratemporal fossa and cheek region was removed, and then endoscopy was used to guide removal of the tumor lobe involving the pterygomaxillary fossa, nasal cavity, and sphenoidal sinus. Intraoperative observation identified the tumor pedicle in the sphenopalatine foramen and pterygopalatine fossa. Given the lack of scar tissue from a previous operation, complete tumor resection was easily accomplished within 2.5 hours. The intraoperative blood loss was about 100 ml. The OSAHS disappeared after surgery. Fig. 3 shows the completely excised tumor, which weighed 32.8 g. Follow-up more than one year, the boy was in good health.
