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Ganglioneuroma of the Sacrum https://doi.org/10.14245/kjs.2017.14.3.106 KJS Print ISSN 1738-2262 On-line ISSN 2093-6729 CASE REPORT Korean J Spine 14(3):106-108, 2017 www.e-kjs.org Ganglioneuroma of the Sacrum Donguk Lee1, Presacral ganglioneuromas are extremely rare benign tumors and fewer than 20 cases have been reported in the literature. Ganglioneuromas are difficult to be differentiated preoperatively Woo Jin Choe1, from tumors such as schwannomas, meningiomas, and neurofibromas with imaging modalities. 2 So Dug Lim The retroperitoneal approach for resection of presacral ganglioneuroma was performed for gross total resection of the tumor. Recurrence and malignant transformation of these tumors is rare. 1 Departments of Neurosurgery and Adjuvant chemotherapy or radiation therapy is not indicated because of their benign nature. 2Pathology, Konkuk University Medical Center, Konkuk University We report a case of a 47-year-old woman with a presacral ganglioneuroma. School of Medicine, Seoul, Korea Key Words: Ganglioneuroma, Presacral, Anterior retroperitoneal approach Corresponding Author: Woo Jin Choe Department of Neurosurgery, Konkuk University Medical Center, displacing the left sacral nerve roots, without 120-1 Neungdong-ro, Gwangjin-gu, INTRODUCTION Seoul 05030, Korea any evidence of bony invasion (Fig. 2). We performed surgery via anterior retrope- Tel: +82-2-2030-7625 Ganglioneuroma is an uncommon benign tu- ritoneal approach and meticulous adhesiolysis Fax: +82-2-2030-7359 mor of neural crest origin which is mainly loca- was necessary because of massive abdominal E-mail: [email protected] lized in the posterior mediastinum, retroperito- adhesion due to the previous gynecologic sur- 1,6) Received: August 16, 2017 neum, and adrenal gland . Presacral ganglio- gery. Especially, the left internal iliac vein and Revised: September 6, 2017 neuromas are extremely rare and known as pre- tumor capsule were adherent and were care- Accepted: September 11, 2017 dominantly occuring in women15). Patients are fully dissected. We could discern the left S1 usually young and show neurological signs and root using a nerve stimulator and the tumor symptoms when the tumor developing a signifi- was attached to the S1 nerve in the posterior cant size4,7). As imaging modalities have become side. Since the tumor was partly in the left S1 more widely performed, the number of ganglio- sacral foramen, the foramen was unroofed with neuromas found has increased. However, it is drill and Kerisson punch to expose and remove very difficult to diagnose ganglioneuroma early the mass completely. The encapsulated retro- in the evaluation of the lesion using image mo- peritoneal tumor located on the left side of the dalities only14). We report our experience with sigmoid colon was excised by piecemeal pattern a case of presacral ganglioneuroma. and complete resection was performed (Fig. 3). Postoperative histopathologic study confirmed CASE REPORT the lesion as a ganglioneuroma (Fig. 4). After the surgery, the patient recovered and discha- A 47-year-old woman presented to hospital rged without any neurological deterioration. with a month’s duration of intermittent flank and iliac fossa pain, abdominal discomfort, and DISCUSSION tingling sensation on left thigh. She had a his- tory of transvaginal myomectomy with symp- Ganglioneuromas are well-differentiated, rare toms of mild, temporary diarrhea and abdomi- benign tumors of neural crest origin. Especially, nal discomfort 3 years prior to admission. There presacral location of ganglioneuroma is extre- was no specific family history. There were no mely rare10,13,17,18). Ganglioneuromas often lo- palpable masses observed on abdominopelvic cate in the posterior mediastinum and retro- examination. Computed tomography (CT) scans peritoneum. The tumors usually show a slow- of the abdomen and pelvis revealed a finely growing pattern with a predominance in wom- Copyright © 2017 by The Korean 15) Spinal Neurosurgery Society demarcated, oval shaped mass arising separa- en . Most tumors are diagnosed at the pro- This is an open access article distributed tely from the ovary on the right side of her gressed state of the tumor in patients between under the terms of the Creative Commons pelvis (Fig. 1). Pelvic magnetic resonance (MR) 10 and 30 years of age because symptoms only Attribution Non-Commercial License (http://creativecommons.org/licenses/by- images showed an elliptical tumor on the left appear when the mass becomes large enough 4,7) nc/4.0/) which permits unrestricted non- side of the pelvis which were well enhanced by to exert a mass effect . Ganglioneuromas can commercial use, distribution, and reproduction in any medium, provided the contrast (dimensions: 3.4 cm×2.4 cm×4.5 cm). occur anywhere in the autonomic nervous sys- original work is properly cited. The tumor was adjacent to the left sacrum and tem and can cause a mass effect on the spinal 106 | Volume 14 | Number 3 | September 2017 www.e-kjs.org Ganglioneuroma of the Sacrum Fig. 3. Intraoperatively, a tumor is apparent after unroofing the left S1 sa- Fig. 1. A pelvic computed tomography scan shows a well-demarcated cral foramen. and heterogeneous mass arising from the left S1 sacral foramen (arrow). Axial (A) and sagittal (B) view. Fig. 4. Histopathologic findings of ganglioneuroma. (A) Hematoxylin and Eosin staining with ×200 magnification. The specimen was composed of fibrous and edematous matrix with nests of ganglion cells and Schwann cells in the matrix. (B) S-100 and neurofilament Fig. 2. Magnetic resonance images show a presacral 3.4×2.4×4.5-cm staining at ×100 magnification. mass (arrow). Contrast enhanced T1-weighted magnetic resonance image with intermediate high signal intensity. Axial (A) and sagittal the surgery. (B) view. CT scans of ganglioneuromas may have dot-like calcifications in two-thirds of cases (this was not seen in the reported case), nerves and sympathetic nerves, leading to neurological abnor- which can be a clue for differential diagnosis with neuroblas- 11) malities such as back pain, neurogenic bladder, urinary frequency, tomas . MRI helps in planning treatments by distinguishing 14,16) constipation, and bowel obstruction. Spontaneous or radiothe- lesions of spinal origin from lesions of pelvic origin . On MR rapy-induced malignant transformation of ganglioneuromas has images, ganglioneuromas may show a high signal intensity dur- been reported but this is extremely uncommon2). ing late enhancement and a heterogeneous mass on T2-weighted According to our literature search, fewer than 20 cases of pre- image. The structural and morphological characteristics of gan- sacral ganglioneuroma have been reported worldwide. Ganglio- glioneuromas, such as the presence of mature sympathetic gan- neuromas arise from sympathoblasts derived from the embryonic glion cells, can help distinguish them from other pelvic benign neural crest. Histologically, ganglioneuromas consist of Schwann tumors such as schwannomas, meningiomas, or neurofibro- 4,6,7,11) cells with mature ganglion and are considered to be part of the mas . Appropriate therapy for ganglioeneuromas is com- 2,5) neuroblastoma group, along with neuroblastomas and ganglio- plete surgical excision . The complete resection of presacral neuroblastomas3,9,10,13,14,19). Preoperative evaluation could be per- ganglioneuroma provides for both effective diagnosis and treat- formed by using fine-needle aspiration biopsy (FNAB), but the ment. Adjuvant chemotherapy or radiation therapy is not in- 12) approach is often technically difficult because of the anatomical dicated due to the nature of this benign disease . location. Also, using a single FNAB sample can lead to inaccurate In our case, an anterior retroperitoneal approach was used, diagnoses and cells should be sampled at multiple sites within which is a effective method for mass removal of presacral lesions the tumor8). because it provides a sufficient field of view to identify the origin It is not easy to differentiate between ganglioneuroma and of the lesion. This approach provided excellent access to the other tumors (such as schwannoma), only with clinical or ima- lesion and facilitated complete resection. Other groups have also ging findings. There is no case report that patient was diagnosed reported that the posterior transsacral approach was proposed with ganglioneuroma preoperatively with imaging modalities14). to remove a tumor completely involving the sacral nerve root. 14) Magnetic resonance imaging (MRI) is a tool that not only helps Modha et al. prefer the anterior approach, but a staged oper- to assess the nature of the lesion but also helps in planning of ation of laminectomy and foraminotomy can be added to manage Korean J Spine Volume 14 | Number 3 | September 2017 | 107 Lee DU et al. any radicular symptoms after removal of the tumor. ganglioneuroma: a case report, review of the literature, and immu- Recurrence and malignant transformation of this tumor have nohistochemical study. Hum Pathol 23:72-75, 1992 been rarely reported2). In that case, the only treatment available 7. Hayes FA, Green AA, Rao BN: Clinical manifestations of ganglio- is surgical resection. The patients’ prognosis is usually good and neuroma. Cancer 63:1211-1214, 1989 there is no consensus as to a follow-up plan. 8. Jain M, Shubha BS, Sethi S, Banga V, Bagga D: Retroperitoneal ganglioneuroma: report of a case diagnosed by fine-needle aspira- tion cytology, with review of the literature. Diagn Cytopathol 21: CONCLUSION 194-196, 1999. 9. Kaufman MR, Rhee JS, Fliegelman
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