Giant Invasive Sacral Schwannoma with Aortic Bifurcation Compression and Hydronephrosis

Journal Pre-proof Giant Invasive Sacral Schwannoma with Aortic Bifurcation Compression and Hydronephrosis K. Dr. Ragurajaprakash, M.D., MRCS., DNB(Surg)., MCh(neuro), Hanakita Dr. Junya, M.D., Ph.D, Takahashi Dr. Toshiyuki, M.D., Ph.D, Ueno Dr. Manabu, M.D., Ph.D, Minami Dr.Manabu, M.D., Ph.D, Tomita Dr. Yosuke, M.D., Ph.D, Tsujimoto Dr. Yoshitaka, M.D., Kanematsu Dr. Ryo, M.D. PII:

S1878-8750(19)33117-1

DOI:

https://doi.org/10.1016/j.wneu.2019.12.088

Reference:

WNEU 13935

To appear in:

World Neurosurgery

Received Date: 29 October 2019 Accepted Date: 15 December 2019

Please cite this article as: Dr. Ragurajaprakash K, Dr. Junya H, Dr. Toshiyuki T, Dr. Manabu U, Dr.Manabu M, Dr. Yosuke T, Dr. Yoshitaka T, Dr. Ryo K, Giant Invasive Sacral Schwannoma with Aortic Bifurcation Compression and Hydronephrosis World Neurosurgery (2020), doi: https://doi.org/10.1016/ j.wneu.2019.12.088. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Elsevier Inc. All rights reserved.

TITLE: Giant Invasive Sacral Schwannoma with Aortic Bifurcation Compression and Hydronephrosis

Giant Invasive Sacral Schwannoma with Aortic Bifurcation Compression and Hydronephrosis Corresponding Author / First author 1. Dr. Ragurajaprakash K, M.D., MRCS., DNB(Surg)., MCh(neuro) Department of Neurosurgery, Royal care super speciality hospital , Neelambur, Coimbatore, Tamilnadu, India -641062. Phone: +91 9865002156; 91-422- 2227000 / 4040000 Co-Authors 2. Dr. Junya Hanakita, M.D., Ph.D Spinal Disorders Center, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289 3. Dr. Toshiyuki Takahashi, M.D., Ph.D Spinal Disorders Center, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289 4. Dr. Manabu Ueno, M.D., Ph.D Department of Urology, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289 5. Dr.Manabu Minami, M.D., Ph.D Spinal Disorders Center, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289 6. Dr. Yosuke Tomita, M.D., Ph.D Spinal Disorders Center, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289 7. Dr. Yoshitaka Tsujimoto, M.D., Spinal Disorders Center, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289 8. Dr. Ryo Kanematsu, M.D., Spinal Disorders Center, Fujieda Heisei Memorial Hospital 123-1 Mizukami, Fujieda City, Shizuoka 426-8662, Japan Phone: 81-54-643-1230; FAX: 81-54-643-1289

Introduction The incidence of spinal tumor is less than 1 in 1,00,000 person per year.1 Rasmussen et al

2

found in his study only 35 (7%) of 557 intraspinal tumors was involved in the sacrum. It has been estimated that the sacral tumors usually occur in only one of 40,000 patients admitted in hospitals, but these giant sacral tumors are considered even more rare now a days

3

.Usual

presentation of these tumors is benign, but malignant transformations have been reported especially with Von Recklinghausen disease. Among all schwannomas only 0.5-5% occur retroperitoneally 4. Large schwannomas are still rare , occurring in only 0.3-5% of retroperitoneal schwannomas 5. Only few cases have been reported in literature for large retroperitoneal tumors causing compression of adjacent structures. One case with bilateral hydronephrosis 4 and the other case with left hydronephrosis 13 have been reported. Our case is the first of its kind to be published , a giant invasive sacral schwannoma with aortic bifurcation compression and bilateral hydronephrosis. The term giant means remarkable size of the tumor

6

and the term invasiveness

means extensive growth into all directions compressing adjacent structures 6. Retroperitoneal tumors when they are situated in the lumbar region are more prone to cause hydronephrosis even if the tumor size is 5cm or less. Because the ureters and aortic bifurcation are located in the retroperitoneal space, they can be easily compressed by the retroperitoneal lumbar tumors. Sacral schwannoma ,

extending retroperitoneally, with compression both of the ureters and the aortic

bifurcation is a rare combination .

Case Report;

A 56 years old female experienced slight fullness of lower abdominal region about 12 years ago, which was diagnosed as sacral tumor on CT scan and MRI. By biopsy , her tumor was confirmed to be a benign schwannoma. 10 years ago, posterior approach was performed at a department of neurosurgery of another university, to remove her tumor resulting in only partial resection due to high vascularity and firmness of the tumor. After the operation , right thigh paresthesia and right leg weakness occurred, which continued for several years. After this operation, she consulted our department 7 years ago. We considered several kinds of therapies including radiotherapy and embolization, which were not indicated due to the remarkable size and volume of the tumor. Because of her slight symptoms without severe neurological disturbances, surgical treatment for the tumor was not planned at that time. She spent an uneventful time until this spring when she complained of acute onset of urinary disturbances and high fever. Her family doctor examined the patient noticing the enlargement of the tumor size with remarkable bilateral hydronephrosis on MRI study, which was not noticed in the previous study. She was referred to our clinic again for fear of renal dysfunction due to remarkable hydronephrosis. (Figure 1- shows previous follow up MRI images, during the last decade, which shows gradual increase in size of the tumor). She underwent all investigations and workup (Figures 2 - 5). There was an heterogenous mass lesion, which is well encapsulated, margins were well defined, which was around 10cm * 10cm * 8cm occupying the entire right hemipelvis, starting from sacrum posteriorly until behind the pubic bone compressing the urinary bladder anteriorly. Entire colon was pushed laterally, compressing the ureters with bilateral hydronephrosis. CT angiogram showed highly vascular tumor with compression of aortic bifurcation. Diagnosed as Type 2 tumor according to Klimo et al classification , and as Type V tumor according to Modified sridhar et al classification of spinal

nerve sheath tumor. This time we decided to remove the tumor as much as we can , to reduce the volume size ,which resolves the bilateral hydronephrosis. Transabdominal anterior approach was performed. Due to its high vascularity and firmness, subtotal excision was performed preserving all vital structures (Figure 6 shows - intraoperative pictures). Residual tumor was irradiated with 50.4 Gy / 28 Fr. she has been regularly followed up with out neurological deficits.

Discussion: These sacral schwannomas have diversity of clinical presentation . A small tumor restricted to sacrum may present as low back pain , numbness over lateral aspect of leg, or in the perineal region. As tumor overgrows , it may erode the sacrum, extend to the retroperitoneal space. For a long duration , tumor may be asymptomatic. Symptoms occur due to compressive effect of large tumors on the adjacent structures . Depending on the structures involved, symptoms differ accordingly. Bladder compression presenting as dysuria, bowel compression as constipation, ureter compression as hydrourethronephrosis. Depending on the size and location of sacral schwannomas, Klimo et al 7 introduced a classification which helps to understand the nature of tumor, and also helps to select the appropriate surgical approach. (Table 1) There is another classification for spinal nerve sheath tumors based on the invasiveness, such as modified Sridhar classification of benign nerve sheath tumors. (Table 2) Our case is classified as Type 2, according to Klimo et al. The tumor of the present case eroded sacral S1 -S2 segments, extensively grew to involve entire pelvis, compressing ureters, aortic bifurcation, bladder, bowel, which was classified as Type V tumor according to modified Sridhar classification. Chandhanarat Chandhanayingyong et al

8

reported 4 cases of sacral small schwannoma.

Due to its benign nature of the tumor ,aggressive tumor resection, which may lead to neurological

deficits, was denied. They planned for intralesional curettage and post operative adjuvent radiation therapy . All patients were followed up regularly with out neurological deficits and relieved from their original symptoms. In our case, we had a giant sacral invasive schwannoma with compressive symptoms of adjacent structures, presenting as hydronephrosis and destruction of sacral bone.We have done subtotal excision of the tumor via transadbominal anterior approach with relief of pain and hydronephrosis. Sacral segment removal or amputation may result in bowel and bladder disturbances, which is not acceptable for benign condition . Ursalan Ahmed khan et al 9 reported giant sacral schwannoma of Type 2, for which they have performed anterior and posterior approach in two stages with good results using intra operative neuromonitoring. Because our patient had undergone posterior approach with neurological deficits and slow recovery from it , reexploration through previous approach may be more morbid. So we adopted trans abdominal anterior approach. According to a systemic review and meta analysis according to PRISMA guidelines (Preferred Reporting items for systemic reviews and meta analyses), only 38 reported cases of sacral schwannomas

9

of Type 2 have been found in

various literatures. Among them only 6 cases were of Giant size (10cm in any one dimension), and all 6 cases except one had neurological deficits. Though complete resection, which brought various severe post-operative complications in the reported cases, is best option, less morbid procedure would be appropriate and we planned for subtotal excision, with complete recovery of symptoms without neurological deficits. We have a literature review for type 2 giant sacral schwannoma’s (Table 3), in which one can find only one case presented with bilateral hydronephrosis. This present giant sacral schwannoma with unique combination of the bilateral hydronephrosis and aortic bifurcation compression seems worthwhile to be reported Conclusion

This present giant sacral invasive schwannoma with aortic bifurcation compression and bilateral hydroureteronephrosis is the first case to be reported in the literature. In literature review there are cases causing vascular compression by the tumors, but most of which are located in the lumbar region. Hydronephrosis is more common in tumors located in lumbar retroperitoneal region, but sacral retroperitoneal tumors causing hydronephrosis are very rare. With subtotal excision , her symptoms improved sufficiently. Conflict of Interest The author reports no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

References 1. Schellinger KA1, Propp JM, Villano JL, McCarthy BJ.Descriptive epidemiology of primary spinal cord tumors. J Neurooncol. 2008 Apr;87(2):173-9. 2. Rasmussen TB, Kernohan JW, Adson AW: Pathologic classifi- cation, with surgical consideration, of intraspinal tumors. Ann Surg 111:513–530, 1940

3. Whittaker LD, Pemberton JD: Tumors ventral to the sacrum. Ann Surg 107:96–106, 1938 4. Girgin C, Ozkan U, Sezer A, Tugyan N. A. Large pelvic schwannoma causing bilateral hydronephrosis. Int J Urol. 2003;10:616-8 5. Li Q., Gao C., Juzi J.T, Hao X., Analysis of 82 cases of retroperitoneal schwannoma, ANZ J

Surg, 2007, 77, 237-240

6. Sridhar K., Ramamurthi R., Vasudevan M.C., Ramamurthi B., Vasudevan M.C., Giant invasive spinal schwannomas: definition and surgical management, J. Neurosurg., 2001, 94, 210-215 7. Klimo P, Jr, Rao G, Schmidt RH, Schmidt MH. Nerve sheath tumors involving the sacrum, case report and classification scheme. Neurosurg Focus. 2003;15:E12 8. Chandhanarat Chandhanayingyong, Apichat Asavamongkolkul, Nittaya Lektrakul, and Sorra nart Muangsomboon, “The Management of Sacral Schwannoma: Report of Four Cases and Review of Literature,” Sarcoma, vol. 2008, Article ID 845132, 6 pages, 2008 9. Khan UA, Ismayl G, Malik I. Giant Sacral Schwannoma Treated with a 360 Approach: A Rare Case and Systematic Review of the Literature.World Neurosurg. 2018 Jul;115:65-72. 10. Fris TL, Friis-andersen H. Benign sacral schwannoma- A case and short review of the literature. Br J Neurosurg.2015;29:595-596 11. Togral G, Arikan M, Hasturk AE, Gungor S. Incidentally diagnosed giant invasive sacral schwannoma. Its clinical features and surgical management without stability. Neurosciences(Riyadh). 2014;19:224-228 12. Chanplakorn Pongsthorn, Hiroshi Ozawa, Toshimi Aizawa, Takashi Kusakabe, Takeshi Nakamura, and Eiji Itoi. Giant sacral schwannoma: A report of six cases. Ups J Med Sci. 2010 May; 115(2): 146–152. 13. Zhou M, Chen K, Wu C, Yang H. Giant sacral schwannoma with pelvic and lumbar spine

extension. Spine J.2013;13:1154-1155 14. Domínguez J, Lobato RD, Ramos A, Rivas JJ, Gómez PA, Castro S. Giant intrasacral schwannomas: report of six cases. Acta Neurochir (Wien). 1997;139(10):954-9; discussion 959-60. 15. Stecken J, Bardaxoglou E, Touquet S, Manzo N, Cherki E, Dorwling-Carter D, Muckensturm B. Giant sacral schwannoma with pelvic extension. Therapeutic strategy. Apropos of a case. Neurochirurgie. 1996;42(6):294-9. 16. Zou F, Dai M, Zhang B, Nie T. Misdiagnosis of a giant intrapelvic schwannoma: A case report. Oncol Lett. 2013 Dec;6(6):1646-1648. 17. Hoarau N , K.Slim, D.Da Ines. CT and MR imaging of retroperitoneal schwannoma. Diagnostic and Interventional Imaging. Volume 94, Issue 11, November 2013, Pages1133-1139.

Figure / Table legends Figure 1 - follow up MRI during the last decade, which shows gradual increase in size of the tumor. Figure 2 - CT angiogram, showing aortic bifurcation vascular compression. Figure 3 - CT contrast abdomen, showing hydronephrosis and vascular compression.

Figure 4 - pre operative MRI contrast, showing large retroperitoneal tumor, involving right hemipelvis, compressing bladder and bowels. Figure 5 - CT bony cuts, showing large retroperitoneal tumor extending to lumbar vertebrae, and sacral segments S1 and S2 erosions. Figure 6 - intra operative pictures of tumor removal.

Table 1 - Klimo et al - Tumor classification for sacral schwannoma’s / Surgical approach. Table 2 - Modified Sridar classification for benign nerve sheath tumors. Table 3 - Literature review for type 2 giant sacral schwannoma’s.

Table 1 Klimo et al Classification - for sacral schwannoma’s / Surgical approach Type 1

Tumors are limited to the sacrum itself. (may invade and track along the spinal canal) / Posterior approach

Type 2

Tumor extends / breach the anterior and posterior bone limits of the sacrum. / Combined approach (anterior and posterior approach)

Type 3

Tumour is located within the presacral / retroperitoneal space. / only anterior transabdominal approach would be sufficient.

Modified Sridhar classification of benign nerve sheath tumours Type I

Intraspinal tumor < 2 vertebral segments in length; a: intradural

b: extradural

Type II

Intraspinal tumor > 2 vertebral segments in length (giant tumor)

Type III

Intraspinal tumor with extension into nerve root foramen

Type IV

Intraspinal tumor with extraspinal extension (dumbbell tumors); a: extraspinal component < 2.5 cm; b: extraspinal component > 2.5 cm (giant tumor)

Type V

Tumor with erosion into vertebral bodies (giant invasive tumor), lateral and posterior extensions into myofascial planes

Type VI

Tumor in entirely intravertebral location without intraspinal portion

Type VII

Intraspinal tumor with erosion into vertebral bodies (invasive tumor) and extension into nerve root foramen

Table 2

Table TABLE -3 Literature review of giant Sacral Schwannoma

Literature review of giant Sacral Schwannoma

Study

Number of Cases

Age Range / Sex

Symptoms

Tumor Size

Surgical Approach

Complete / Partial excision

Remarks

Ursula Ahmed khan et al 9

One case

38 year / M

Constipation, Dysuria, paresthesia of lower limbs

13 * 11 * 9 cm

Combined approach ( two staged procedure)

Complete Excision

No Hydro nephrosis

Cengiz Girgin et al 4

One case

73 / M

constipation Dysuria

13 * 18 * 20 cm

Anterior Approach

Complete Excision

Hydronephrosis present (Bilateral)

Fris et al 10

One case

68 / M

Constipation

10 * 10 * 9 cm

Anterior Approach

Partial Excision

No Hydro nephrosis

Togral et al 11

One case

42 / M

Abdominal pain

13.8 * 9.1 cm

Combined Approach

Complete Excision

No Hydro nephrosis

Chanplakorn P et al 12

Three cases

12- 23 years 1 - Male 2 - Female

Gluteal pain Lower limb pain and paresthesia

11 - 15 cm in any one Dimension

2 - Combined Approach 1- Anterior Approach

2 - Partial 1- Complete Excision

No Hydro nephrosis

Zhou et al 13

One case

62 / M

left lower limb Paresthesia

10 * 15.8 * 17..8 cm

Posterior Approach

Complete Excision

No Hydro nephrosis

Dominguez et al 14

Six cases

17 - 68 years 5 - females 1 - male

Lumbar pain Dysuria

4.5 - 10 cm

2 - Combined Approach 4 - Posterior Approach

Partial Excision

No Hydro nephrosis

Stecken J et al 15

One case

48 / F

Dysuria

15 cm in any one dimension

Combined Approach

Complete Excision

No Hydro nephrosis

Fan Zou et al 16

One case

68 / F

Abdominal pain, Dysuria Constipation

14 * 10 * 15 cm

Anterior Approach

Complete Excision

Hydronephrosis present (left side)

Hoarau N et al 17

One case

40 / M

constipation Dysuria

12.7 * 10 * 11.5 cm

Anterior Approach

Complete Excision

No Hydro nephrosis

Table

Abbreviations CT

- Computer tomography.

MRI - Magnetic resonance imaging. Gy.

- Gray

Fr.

- Fractions of radiation therapy