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Christopher F. Thompson, M.D. UCLA Head & Neck Surgery chrthompson@mednet.ucla.edu

Pediatric esthesioneuroblastomas are rare tumors with only about 100 reported cases. Standard surgical treatment is the open craniofacial resection. Pre-treatment imaging is critical for planning, and surgery remains the primary treatment modality followed by adjuvant therapy. We report a 12 year-old female who underwent endoscopic-assisted resection of an extensively calcified Kadish C and Dulguerov-Calcaterra T3N0M0 esthesioneuroblastoma. Following radiation therapy, she is disease-free 18 months post-treatment. Although there are few reported cases in pediatric patients, our case supports the use of endoscopic endonasal approaches for select pediatric skull base malignancies.

Endoscopic Craniofacial Resection for Pediatric Esthesioneuroblastoma Christopher F. Thompson, M.D.1; Sunita M. Bhuta, M.D. 2; Marvin Bergsneider, M.D.3; Jeffrey D. Suh, M.D. 1

1Department of Head and Neck Surgery, 2Department of Pathology and Laboratory Medicine, 3Department of Neurosurgery University of California, Los Angeles, CA

CT scan and MRI revealed a coarsely calcified, erosive, 3.8 x 3.5 x 4.1 cm mass involving the right nasal vault, maxillary sinus, and ethmoid air cells (Figures 1). There was erosion of the lamina papyracea with subsequent mass effect on the orbital contents. There was also tumor spread through the cribriform plate, but without dural enhancement. Due to the extensive calcifications seen on CT, the initial diagnosis was suggestive of a fibroossesus lesion such as a fibrosarcoma.

In addition to decreased morbidity and being less invasive, recent reports demonstrate the effectiveness of endoscopic approaches in the oncologic outcomes when compared to open approaches for a wide variety of skull base malignancies, such as esthesioneuroblastoma, but these series do not include the pediatric population. [6,7]. A recent meta-analysis actually showed a significantly better survival curve in patients with esthesioneuroblastomas treated with an endoscopic or endoscopic assisted approach compared to an open approach [6]. However, this benefit is slightly misleading since the open surgical approach cohort had more patients with higher tumor stage. Nevertheless, a recent systematic review revealed similar disease and survival outcomes among adult patients having either endoscopic or open craniofacial resection for various sinonasal malignancies [7].

A 12-year-old female presented with a two-year history of recurrent right-sided epistaxis and a four-month history of increasing right nasal airway obstruction, right facial pain, and hyposmia. Nasal endoscopy demonstrated a large right-sided nasal tumor extending from the apex of the middle meatus to the nasal floor. The mass was smooth and covered with multiple blood vessels coursing on the inferior surface.

Our case of a successful endoscopic assisted craniofacial resection of a Kadish C and Dulguerov-Calcaterra T3N0M0 esthesioneuroblastoma highlights the use of this approach for select pediatric anterior skull base malignancies. More experience is required to identify the ideal patients for this minimally invasive approach, and to determine long-term treatment outcomes.

Esthesioneuroblastoma is an uncommon malignant neuroectodermal tumor arising from olfactory epithelium that was first described in 1924. There are only about 100 reported cases in the pediatric population and patients usually present with nasal obstruction, pain, hyposmia and epistaxis [1]. Surgical treatment for anterior skull base malignancies, such as esthesioneuroblastomas, has traditionally been performed by craniofacial resection (CFR) or a transfacial transnasal approach. Typically, a head and neck surgeon resects the intranasal tumor via a lateral rhinotomy, while the neurosurgeon performs a frontal craniotomy. In children, this approach has a reported postoperative complication risk of 25%, which includes CSF leaks, wound infections, and death [2]. Furthermore, the open surgery can lead to facial asymmetry, especially in children, by disrupting craniofacial growth centers. Other side effects from the craniotomy and retraction of the frontal lobe include seizures, mental status changes, frontal lobe abscess, meningitis, hemorrhage, and pneumocephalus [3]. With the improvement in endoscopic surgical techniques and instruments designed for endonasal skull base surgery, endoscopic approaches have been showing significant promise as new treatment modality for select sinonasal and skull base tumors [4]. Due to the rarity of anterior skull base malignancies in children, there is a need to report on outcomes of this less invasive and promising modality in pediatric pts.

INTRODUCTION

Case Presentation [1] Kumar M, Fallon RJ, Hill JS, Davis, MM. Esthesioneuroblastoma in

Children. J Pediatr Hematol Oncol. 2002;24:482-487. [2] Gil Z, Patel SG, Cantu G, Fliss DM, Kowalski LP, Singh B, et al.

Outcome of craniofacial surgery in children and adolescents with malignant tumors involving the skull base: an international collaborative study. Head Neck. 2009;31(3):308-317.

[3] Howard DJ, Lund VJ, Wei WI. Craniofacial resection for tumors of the nasal cavity and paranasal sinuses: a 25-year experience. Head Neck. 2006;28(10):867-73.

[4] Villaret AB, Yakirevitch A, Bizzoni A, Bosio R, Bignami M, Pistochini A, et al. Endoscopic transnasal craniectomy in the management of selected sinonasal malignancies. Am J Rhinol Allergy. 2010;24(1):60-65.

[5] Ramakrishnan VR, Suh JD, Chiu AG, Palmer JN. Addition of a minimally invasive medial orbital approach in the endoscopic management of advanced sino-orbital disease: cadaver study with the clinical correlations. Laryngoscope. 2011;121(2):437-441.

[6] Devaiah AK, Andreoli MT. Treatment of esthesioneuroblastoma: a 16-year meta-analysis of 361 patients. Laryngoscope. 2009;119(7):1412-1416.

[7] Higgins TS, Thorp B, Rawlings BA, Han JK. Outcome results of endoscopic vs craniofacial resection of sinonasal malignancies: a systematic review and pooled-data analysis. Int Forum Allergy Rhinol. 2011;1(4):255-261.

CONCLUSIONS

DISCUSSION cont.

REFERENCES

ABSTRACT

CONTACT

Our patient underwent endoscopic-assisted craniofacial resection (CFR) followed by radiation therapy. No transfacial or bicoronal incisions were used. The intranasal tumor was resected completely via an endoscopic endonasal CFR, which included resection of the ipsilateral anterior skull base. The tumor originated from the cribriform plate and invaded through the lamina papyracea. Intranasal and dural margins were negative. The olfactory bulb was biopsied and negative for tumor. A transcaruncular approach was used to remove the tumor in the orbit, including the periorbital, nasolacrimal sac and duct, and lamina papyracea as previously described [5]. The skull base was reconstructed using tensor fascia lata, septal bone, and a free mucosal graft. The patient underwent adjuvant radiation therapy with a total of 54 Gy and is disease-free 18 months post-treatment (Figure 3).

Surgical Treatment

Figure 2: (A) Well-formed lobules of closely packed neoplastic cells separated by highly vascularized stroma. (B) Tumor cells with fairly uniform round to ovoid nuclei set in a fine fibrillary stroma.

Figure 1: (A) Coronal and (B) axial CT scans revealing coarsely calcified esthesioneuroblastoma with orbital invasion and (C) MRI showing no intracranial spread.

A biopsy was performed and revealed an esthesioneuroblastoma, Hyams grade 1 (Figure 2). Immunohistochemical staining of the intranasal specimen was positive for synaptophysin, chromogranin, CD56, and S-100.

Although open craniofacial resection has been successful, this approach has potential for significant morbidity. In a recent review including 84 pediatric patients, from 17 institutions, undergoing craniofacial resections for anterior skull base malignancies from 1956 to 2000, the postoperative complication rate was 25% (n=21). Postoperative complications included wound complications (13%), postoperative death (4.8%), orbital complications (3.6%), CNS complications including meningitis or cerebrospinal fluid leak (2.4%), and systemic complications (1.2%) [3]. We cannot compare the complication rate to the endoscopic approach in pediatrics because there are too few reported cases. However, in adults, the largest series of endoscopic resected skull base tumors included 62 patients, and the incidence of similar complications was 15% [4].

DISCUSSION

Figure 3: MRI T2 coronal image 13 months post-treatment showing no evidence of recurrence.