GANGLIONEUROMA MAXILLA RARE CASE, INNOVATIVE SURGERY Dr.RAJGOVIND SHARMA Dr.Prashant Sharma Dr.Harsh Deora Division of surgical oncology, SMS MEDICAL COLLEGE,JAIPUR

BACKGROUND Ganglioneuroma(GN)- Arise from PNS Originate from neural crest sympathogonia of sympathetic nervous system Neuroblastic tumours: (Shimada,1999) Neuroblastoma(primarily blasts) Ganglioneuromas(mature ganglion cells) Ganglioneuroblastomas(both)

Frequency: rare tumours-1 in 1,00,000 (mostly children) Sex: male: female:: 1:1.5 Age: Children and young adults. Mean age is 7 yrs(O Adam, Jurnalul pediatrului,2007) LOCATION: Adrenal Para spinal retroperitonium Posterior mediastinum RARE: Head and neck, urinary bladder, bowel wall, gall bladder.

SUMMARY: BENIGN TUMORS, SURGICAL EXCISION IS CURATIVE CLASSIFICATION TWO SYSTEMS 1. Pediatric oncology group(POG): Based on differentiation of cellular and stromal elements Eg: Neuroblastoma<50% differentiated elements. 2. Shimada Classification Histology: stroma grade and differentiation Age: <1yrs; 1-5yr; >5yrs SUMMARY: BENIGN TUMORS, SURGICAL EXCISION IS CURATIVE

CASE REPORT 15 yr old female Presented with c/o Gradually increasing swelling rt.side of face x 12y swelling rt.temporal region x 2y No asso symptoms of Vision loss Deglutation difficulties Neuro-sensory loss

CASE REPORT On examination 1. Larger swelling 8 x 6cm Firm Nontender Local temperature not raised Nonmobile Fixed to underlying bone Skin over swelling stretched 2. Smaller temporal swelling: 6 x 4 cm size Firm Nontender Local temperature not raised Mobile and not fixed to underlying bone & muscle Skin over swelling normal

CASE REPORT Intraoral examination: Lips, buccal mucosa, gums, teeth, tongue movements. Retro molar trigone normal on appearance and palpation. Swelling seen protruding into the oral cavity at Rt. Upper GBS. Vision: 6/24 right eye and 6/6 left eye.

CT SCAN 3D RECONSTRUCTION TEMPORAL BONE SPARED LYTIC LESION INVOLVING MAXILLA

FINAL TISSUE Dx: GANGLIONEUROMA BIOPSY TRANS-ORAL TRU-CUT : SPINDLE CELL NEOPLASM MARKER STUDY: POSTIVE STAIN OF S-100 & SYNAPTOPHYSIN FINAL TISSUE Dx: GANGLIONEUROMA

OPERATIVE PROCEDURE Total maxillectomy + excision of mass in temporal region + reconstruction by Rt. Temporalis muscle flap + nylone darning of orbital floor. Per op findings: 1. A 9 x 6 x 5 CM soft, whitish tumor invading the anterior maxillary wall, sparing the orbit. 2. A second tumor 6 x 8 x 2 cm in the temporal fossa, in subcutaneous plane, well defined, superficial to temporalis muscle.

WEBER FERGUSSON INCISION

Procedure Skin flap raised laterally over the tumor and underlying bone cut keeping capsule intact.

Anterior wall of maxilla separated from lateral wall of nose medially, palatine bone below, floor of orbit above and zygomatic bone laterally.

Main specimen taken out taking care not to damage the orbital apex and eye ball A 6 cm incision taken over the temporal swelling just above the cranial extent of the tumor

MAIN TUMOR SPECIMEN, GROSSLY COMPLETE RESECTION ACHIEVED SMALLER TEMPORAL SWELLING

Nylone Darning of orbital floor done. Temporalis muscle lifted of underlying bone with help of periosteum elevator and inserted under zygomatic tunnel so as to support the eye ball. Nylone Darning of orbital floor done. (Prolene darning has also been tried) TEMPORAL MUSCLE FLAP

POD-2 TUBE FEEDING STARTED AT FOLLOW UP, AFTER SUTURE REMOVAL

Repeat vision testing: right eye 6/24, left eye 6/6 Repeat vision testing: right eye 6/24, left eye 6/6. Eye closure of right eye was almost complete with 0.3 cm gap remaining (ectropion). NO DIPLOPIA CONJUGATE EYE MOV NORMAL

DISSCUSSION MAXILLECTOMIES FOR MAXILLARY NEOPLASMS Due to resection of tumors involving orbit, nasal cavity, palate, paranasal sinuses, intraoral mucosa Cause major functional consequences Deglutition Speech Orbital function Aesthetics

Maxillectomy – A Historical Perspective Total maxillectomies performed by Dupuytren and Gensoul in 1820 and 1824? First recorded maxillectomy by Liston in 1841 Extensive review published by Ohngren in 1933

MAXILLARY BONE Two horizontal and three vertical buttresses Insertion for most muscles of facial expression and mastication Geometrical structure with 6 walls (hexahedron) Roof supports orbital contents Floor forms anterior hard palate.

Classification System (Santamaria & Cordeiro) Type I (Limited maxillectomy) One or two walls, preservation of palate Type II (Subtotal maxillectomy) Lower 5 walls, preservation of orbital floor Type III (Total maxillectomy) Resection of all six walls Orbital preservation (IIIa) vs exoneration (IIIb) Type IV (Orbitomaxillectomy) Upper 5 walls, preservation of palate Santamaria & Cordeiro, 2000. Plast Recon Surg Santamaria & Cordeiro, 2000. Plast Recon Surg

Santamaria & Cordeiro, 2000. Plast Recon Surg

Reconstruction- the past Skin grafts Cervicofacial flaps Pectoralis myocutaneous flap Usually requires two stage procedure

Local and Regional Flaps Palatal mucoperichondrial island flap Up to 15 cm2 surface area Strong enough for through-and-through defects Can rotate 180 deg on pedicle Buccal fat pad Rich vascular supply Best for defects up to 4 cm in diameter Can be used in combination with free bone grafts Submental island 7-15 cm in size Well hidden donor site scar Temporalis Good for orbital support

Free Flaps Indicated for large defects Matching to three-dimensional shape of defect Provide bone, palatal and nasal lining, skin, soft tissue Requires vascular pedicle 10-15 cm long Multiple different options Myocutaneous Osteomyocutaneous Combination with free bone grafts

Prosthetics (Obturation) Advantages Shorter operative time Shorter postop hospital stay Better visualization of maxillectomy cavity for surveilance Disadvantages Hypernasal speech Regurgitation of foods and liquids into nasal cavity Difficulty maintaining hygeine Need for repeated adjustments

CONCLUSION GN are rare benign tumors requiring preoperative tissue diagnosis,surgical excision is curative. Maxillectomy and midface defects result in major functional and aesthetic abnormalities Reconstruction depends on the size and individual components of the resected tissue Nylone(or prolene) darning can be employed for orbit support in cases where intra-op infection is a concern.