Marroquín-Herrera, Omar¹; Rosales-Camargo, Santiago Andrés²; Taub-Krivoy, Alex³; Fuentes-Reyes, Carlos⁴; Ramon-Cuellar, Juan Fernando⁵; Alvarado-Gómez, Fernando⁶

Language: English
References: 6
Page: 250-253
PDF size: 239.56 Kb.

ABSTRACT

Introduction: Giant spinal thoracic dumbbell schwannoma is a benign tumor extremely rare in pediatric age, which may be associated with neurofibromatosis. Case description: A 14-year-old girl who presented paresthesia in the lower extremities and back pain of 6 months onset, with 2 weeks of neurological deficit progression, associated with clinical data of neurofibromatosis. Posterior total excision was performed in a single surgery with transpedicular instrumentation without complications. Conclusion: This is the second case of type IVb pediatric thoracic giant spinal schwannoma reported, and the largest extracted by single posterior route in a single surgical time without complications.

INTRODUCTION

Spinal schwannoma is a slow-growing encapsulated benign tumor that originates in a myelinated nerve sheath with a diagnostic peak between 4 and 5 decades of life in a male: female ratio of 1:1, constituting up to 30% of all spinal tumors and are usually located in the intradural extramedullary region (72%), they can also be located extradurally (13%), intradural and extradural (13%) and intramedullary (1%).¹ Giant Schwannomas can be dumbbell shaped tumors that invade other body cavities or vertebral bodies as K. Sridhar mentioned in 2001 when developed the current morphological classification.²

CASE PRESENTATION

A 14-year-old girl who presented paresthesia in the lower extremities and back pain of six months onset, with two weeks of neurological deficit progression, strength 3/5 and sensitivity 1/2 in the lumbosacral plexus with adequate sphincter control. Cafe-au-lait spots on back, abdomen and legs, rest of neurological examination normal. Magnetic resonance study was taken where the tumor is visualized at T5-T6 level that compromises 80% of the medullary canal and extends to the mediastinum and thorax, in the shape of a giant dumbbell with defined edges with a size of 81 × 67 × 55 mm (Figure 1). Emergency surgery was performed due to progressive deficit with a posterior approach, placing transpedicular instrumentation of bilateral T2-T4, right T6, bilateral T7-T8 plus left costotransversectomy at left T5-T6 level that improved the entire intracanal and thoracic tumor without present complications (Figure 2). The tumor was sent to pathology and the genetic study was carried out; the result was positive for Schwannoma with association to Neurofibromatosis type 2. Currently with 9 months of neurologically complete follow-up and without local recurrences of the tumor (Figure 3).

DISCUSSION

Spinal tumors are a relatively rare diagnosis, accounting for 1 to 10% of all pediatric central nervous system tumors. Pediatric spinal schwannomas constitute 2.5 to 4% of all pediatric spinal tumors, with a female: male ratio of 2:1, data that contrast with the adult population.³ Additionally, giant spinal schwannoma is an even rarer presentation, which may or may not be associated with neurofibromatosis.⁴ Within the Sridhar classification, there is only one 14 years old male with a schwannoma type IVb (dumbbell) reported at T7-T8 level that invaded the thorax requiring a single posterior approach by costotransversectomy for its complete macroscopic excision, complementing with transpedicular instrumentation, which had complete clinical improvement at six months.⁵ Case like ours that was treated with a single posterior approach plus transpedicular instrumentation to avoid risk of instability,⁶ despite being larger, we obtained excellent postoperative results, complete neurological recovery without recurrence at nine-month follow-up (Table 1).

CONCLUSIONS

Giant thoracic type IVb spinal schwannoma is extremely rare in pediatric age. At the present time, only 1 case has been reported in the world literature, this is the second report. Both cases were treated by a single posterior approach, with total macroscopic resection plus transpedicular instrumentation with good postoperative outcomes.

REFERENCES

1. Ogrenci A, Koban O, Sentürk S, Yaman O, Sasani M, Dalbayrak S, et al. Giant spinal schwannomas. Clin Surg. 2017; 2: 1593.

2. Sridhar K, Ramamurthi R, Vasudevan MC, Ramamurthi B. Giant invasive spinal schwannomas: Definition and surgical management. J Neurosurg. 2001; 94 (2 Suppl): 210-215.

3. Kulkarni A, Srinivas D, Somanna S, Indira DB, Ananthakrishna CB. Pediatric spinal schwannomas: an institutional study. J Pediatr Neurosci. 2012; 7 (1): 1-3.

4. Shaikh ST, Thareja V, Mohanty CB, Deopujari CE. Giant extradural spinal schwannoma in a non-neurofibromatosis child-case report and review of literature. Child's Nerv Syst. 2021; 37 (4): 1327-1331.

5. Vadivelu S, Prasad P, Adesina AM, Kim E, Luerssen TG, Jea A. Giant invasive spinal schwannoma in children: A case report and review of the literature. J Med Case Rep. 2013; 7: 1-6.

6. Alvarado GF, Marroquín HO, Rosales CS, Velásquez SG. Técnica manos libres para colocación de tornillos transpediculares en columna torácica, revisión narrativa de la literatura. Ortho-tips. 2021; 17 (3): 155-161.

AFFILIATIONS

¹Spine Surgeon. ORCID ID: https://orcid.org/0000-0003-4159-0222. Hospital Universitario Fundación Santa Fe de Bogotá.

²Medical Research, Spine Surgery. ORCID ID: https://orcid.org/0000-0002-4591-0134. Hospital Universitario Fundación Santa Fe de Bogotá.

³Medical Research, Spine Surgery. ORCID ID: https://orcid.org/0000-0002-2635-3432. Hospital Universitario Fundación Santa Fe de Bogotá.

⁴Medical Research, Spine Surgery. ORCID ID: https://orcid.org/0000-0001-9826-5055. Hospital Universitario Fundación Santa Fe de Bogotá.

⁵Neurosurgeon. ORCID ID: https://orcid.org/0000-0002-6116-2033. Hospital Universitario Fundación Santa Fe de Bogotá.

⁶Chief of Spine Surgery. ORCID ID: https://orcid.org/0000-0002-8854-0356. Hospital Universitario Fundación Santa Fe de Bogotá.

Conflict of interest: The authors declare no conflict of interest.

CORRESPONDENCE

Omar Marroquín-Herrera . E-mail: dr.omarmhspine@gmail.com

Received: 11-29-2021. Accepted: 12-21-2021.