Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review
Abstract
:1. Introduction
2. Case Presentation
2.1. Case 1
2.2. Case 2
3. Discussion
4. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
- Ostrom, Q.T.; Gittleman, H.; Truitt, G.; Boscia, A.; Kruchko, C.; Barnholtz-Sloan, J.S. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2011–2015. Neuro Oncol. 2018, 20, iv1–iv86. [Google Scholar] [CrossRef] [Green Version]
- Louis, D.N.; Perry, A.; Wesseling, P.; Brat, D.J.; Cree, I.A.; Figarella-Branger, D.; Hawkins, C.; Ng, H.K.; Pfister, S.M.; Reifenberger, G.; et al. The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary. Neuro Oncol. 2021, 23, 1231–1251. [Google Scholar] [CrossRef]
- Yust Katz, S.; Cachia, D.; Kamiya-Matsuoka, C.; Olar, A.; Theeler, B.; Penas Prado, M.; Gilbert, M.R.; Armstrong, T. Ependymomas Arising Outside of the Central Nervous System: A Case Series and Literature Review. J. Clin. Neurosci. 2018, 47, 202–207. [Google Scholar] [CrossRef]
- Schiavello, E.; Biassoni, V.; Antonelli, M.; Modena, P.; Cesaro, S.; Pierani, P.; Gandola, L. Pediatric Extraspinal Sacrococcygeal Ependymoma (ESE): An Italian AIEOP Experience of Six Cases and Literature Review. Childs Nerv. Syst. 2018, 34, 1291–1298. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Lien, B.V.; Brown, N.J.; Himstead, A.S.; Ball, B.Z.; Guillen, A.; Acharya, N.; Yi Yang, C.; Sahyouni, R.; Perez-Rosendahl, M.; Stitzlein, R.N.; et al. Surgical Management of a Rare Myxopapillary Ependymoma of the Gluteal Region: A Case Report. Surg. Neurol. Int. 2021, 12, 130. [Google Scholar] [CrossRef] [PubMed]
- Batich, K.A.; Riedel, R.F.; Kirkpatrick, J.P.; Tong, B.C.; Eward, W.C.; Tan, C.L.; Pittman, P.D.; McLendon, R.E.; Peters, K.B. Recurrent Extradural Myxopapillary Ependymoma With Oligometastatic Spread. Front. Oncol. 2019, 9, 1322. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Andreiuolo, F.; Puget, S.; Peyre, M.; Dantas-Barbosa, C.; Boddaert, N.; Philippe, C.; Mauguen, A.; Grill, J.; Varlet, P. Neuronal Differentiation Distinguishes Supratentorial and Infratentorial Childhood Ependymomas. Neuro Oncol. 2010, 12, 1126–1134. [Google Scholar] [CrossRef]
- McGuire, C.S.; Sainani, K.L.; Fisher, P.G. Incidence Patterns for Ependymoma: A Surveillance, Epidemiology, and End Results Study. J. Neurosurg. 2009, 110, 725–729. [Google Scholar] [CrossRef]
- Mallory, F.B. Three Gliomata of Ependymal Origin; Two in the Fourth Ventricle, One Subcutaneous over the Coccyx. J. Med. Res. 1902, 8, 1–10.1. [Google Scholar]
- Ciraldo, A.V.; Platt, M.S.; Agamanolis, D.P.; Boeckman, C.R. Sacrococcygeal Myxopapillary Ependymomas and Ependymal Rests in Infants and Children. J. Pediatr. Surg. 1986, 21, 49–52. [Google Scholar] [CrossRef]
- Chou, S.; Soucy, P.; Carpenter, B. Extraspinal Ependymoma. J. Pediatr. Surg. 1987, 22, 802–803. [Google Scholar] [CrossRef]
- Kramer, G.W.; Rutten, E.; Sloof, J. Subcutaneous Sacrococcygeal Ependymoma with Inguinal Lymph Node Metastasis. Case Report. J. Neurosurg. 1988, 68, 474–477. [Google Scholar] [CrossRef]
- Marc’hadour, F.L.; Pasquier, B. Subcutaneous Sacrococcygeal Ependymoma with Incidental Glomus Coccygeum. Histopathology 1991, 18, 570–572. [Google Scholar] [CrossRef] [PubMed]
- Gupta, R.K.; Pratap, D. Metastasising Congenital Subcutaneous Sacrococcygeal Ependymoma. Indian J. Cancer 1992, 29, 76–81. [Google Scholar]
- Serour, F.; Gorenstein, A.; Finci, Y.; Zaidel, L. Subcutaneous Sacrococcygeal Myxopapillary Ependymoma. Pediatr. Surg. Int. 1993, 8, 362–365. [Google Scholar] [CrossRef]
- Botti, G.; Gravina, A.; Cremona, F.; Izzo, F.; Rigutini, M.; Picone, A. Subcutaneous Sacrococcygeal Myxopapillary Ependymoma. A Case Report. Eur. J. Cancer 1994, 30A, 570–571. [Google Scholar] [CrossRef]
- Kline, M.J.; Kays, D.W.; Rojiani, A.M. Extradural Myxopapillary Ependymoma: Report of Two Cases and Review of the Literature. Pediatr. Pathol. Lab Med. 1996, 16, 813–822. [Google Scholar] [CrossRef]
- Webber, E.M.; Fraser, R.B.; Resch, L.; Giacomantonio, M. Perianal Ependymoma Presenting in the Neonatal Period. Pediatr. Pathol. Lab Med. 1997, 17, 283–291. [Google Scholar] [CrossRef]
- Sawyer, J.R.; Miller, J.P.; Ellison, D.A. Clonal Telomeric Fusions and Chromosome Instability in a Subcutaneous Sacrococcygeal Myxopapillary Ependymoma. Cancer Genet. Cytogenet 1998, 100, 169–175. [Google Scholar] [CrossRef]
- Ilhan, I.; Berberoglu, S.; Kutluay, L.; Maden, H.A. Subcutaneous Sacrococcygeal Myxopapillary Ependymoma. Med. Pediatr. Oncol. 1998, 30, 81–84. [Google Scholar] [CrossRef]
- Johnson, J.M.; Jessurun, J.; Leonard, A. Sacrococcygeal Ependymoma: Case Report and Review of the Literature. J. Pediatr. Surg. 1999, 34, 1405–1407. [Google Scholar] [CrossRef]
- Grubnic, S.; MacVicar, D. A Perianal Problem. Br. J. Radiol. 1999, 72, 1235–1236. [Google Scholar] [CrossRef] [PubMed]
- Aktuğ, T.; Hakgüder, G.; Sarioğlu, S.; Akgür, F.M.; Olguner, M.; Pabuçcuoğlu, U. Sacrococcygeal Extraspinal Ependymomas: The Role of Coccygectomy. J. Pediatr. Surg. 2000, 35, 515–518. [Google Scholar] [CrossRef]
- Rao, I.S.; Kapila, K.; Aggarwal, S.; Ray, R.; Gupta, A.K.; Verma, K. Subcutaneous Myxopapillary Ependymoma Presenting as a Childhood Sacrococcygeal Tumor: A Case Report. Diagn. Cytopathol. 2002, 27, 303–307. [Google Scholar] [CrossRef] [PubMed]
- Akpolat, N.; Bozlak, N.; Kazez, A.; Köseoğullari, A.A. Sacrococcygeal Extraspinal Ependymoma: A Case Report. Turk. J. Pediatr. 2003, 45, 276–279. [Google Scholar]
- Tröbs, R.-B.; Sorger, I.; Schütz, A. [Sacrococcygeal extraspinal myxopapillary ependymoma]. Klin. Padiatr. 2006, 218, 243–245. [Google Scholar] [CrossRef]
- Beschorner, R.; Wehrmann, M.; Ernemann, U.; Bonin, M.; Horber, V.; Oehl-Jaschkowitz, B.; Meyermann, R.; Dufke, A. Extradural Ependymal Tumor with Myxopapillary and Ependymoblastic Differentiation in a Case of Schinzel-Giedion Syndrome. Acta Neuropathol. 2007, 113, 339–346. [Google Scholar] [CrossRef]
- Alexiou, G.A.; Sfakianos, G.; Moschovi, M.; Athanasiadou, S.; Stefanaki, K.; Prodromou, N. Myxopapillary Ependymoma of the Sacrococcygeal Region Presenting as a Pilonidal Sinus. Pediatr. Neurosurg. 2012, 48, 64–65. [Google Scholar] [CrossRef] [PubMed]
- Chakraborti, S.; Kini, H.; Pai, K.G.; Upadhyaya, V. Sacrococcygeal Myxopapillary Ependymoma with Anaplastic Ependymoma Component in an Infant. J. Pediatr. Neurosci. 2012, 7, 218–220. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Cimino, P.J.; Agarwal, A.; Dehner, L.P. Myxopapillary Ependymoma in Children: A Study of 11 Cases and a Comparison with the Adult Experience. Pediatr. Blood Cancer 2014, 61, 1969–1971. [Google Scholar] [CrossRef]
- Dogan, M.S.; Collins, R.; Wang, J.; Koral, K. Subcutaneous Sacrococcygeal Ependymoma in a Child. Spine J. 2016, 16, e513–e514. [Google Scholar] [CrossRef]
- Amin, R.; Berdan, E.; Knipstein, J.; Jarzembowski, J.; Siddiqui, S. Extraspinal Sacrococcygeal Ependymoma Masquerading as Sacrococcygeal Teratoma in the Pediatric Patient. Pediatr. Surg. Int. 2018, 34, 109–112. [Google Scholar] [CrossRef]
- Rogers, S.; Jones, D.T.W.; Ireland, A.; Gottardo, N.G.; Endersby, R. Unusual Paediatric Spinal Myxopapillary Ependymomas: Unique Molecular Entities or Pathological Variations on a Theme? J. Clin. Neurosci. 2018, 50, 144–148. [Google Scholar] [CrossRef] [Green Version]
- Gupta, T.; Patel, V.; El-Medani, F.; Gupta, S. An Unexpected Diagnosis of Paediatric Subcutaneous Sacrococcygeal Extraspinal Ependymoma: Lessons Learnt and Review of the Literature. J. Surg. Case Rep. 2020, 2020, rjaa177. [Google Scholar] [CrossRef] [PubMed]
- Thejeel, B.; Rajakulasingam, R.; Balogh, P.; Saifuddin, A. Diagnosis: Subcutaneous Myxopapillary Ependymoma. Skeletal Radiol. 2020, 49, 1175–1176. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Planas, S.; Cruz, O.; Bejarano, M.; Albert, A.; Rovira, C.; Bombi, J.A. Extra-Axial Sacral Soft Tissue Giant Cell Ependymoma Affecting a Child: Case Report and Review of the Literature. Neuropathology 2021, 41, 139–145. [Google Scholar] [CrossRef]
- Weltgesundheitsorganisation. WHO Classification of Tumours of the Central Nervous System; Louis, D.N., Ohgaki, H., Wiestler, O.D., Cavenee, W.K., Eds.; World Health Organization classification of tumours; Revised 4th edition; International Agency for Research on Cancer: Lyon, France, 2016; ISBN 978-92-832-4492-9. [Google Scholar]
- Bale, P.M. Ependymal Rests and Subcutaneous Sacrococcygeal Ependymoma. Pathology 1980, 12, 237–243. [Google Scholar] [CrossRef] [PubMed]
- Maiorana, A.; Fante, R.; Fano, R.A. Myxopapillary Ependymoma of the Sacrococcygeal Region. Report of a Case. Pathologica 1989, 81, 471–476. [Google Scholar]
- Lemberger, A.; Stein, M.; Doron, J.; Fried, G.; Goldsher, D.; Feinsod, M. Sacrococcygeal Extradural Ependymoma. Cancer 1989, 64, 1156–1159. [Google Scholar] [CrossRef]
- Bell, D.A.; Woodruff, J.M.; Scully, R.E. Ependymoma of the Broad Ligament. A Report of Two Cases. Am. J. Surg. Pathol. 1984, 8, 203–209. [Google Scholar] [CrossRef] [PubMed]
- Duggan, M.A.; Hugh, J.; Nation, J.G.; Robertson, D.I.; Stuart, G.C. Ependymoma of the Uterosacral Ligament. Cancer 1989, 64, 2565–2571. [Google Scholar] [CrossRef]
- Kleinman, G.M.; Young, R.H.; Scully, R.E. Ependymoma of the Ovary: Report of Three Cases. Hum. Pathol. 1984, 15, 632–638. [Google Scholar] [CrossRef]
- McEachron, K.R.; Gaertner, W.B. Extradural Sacrococcygeal Subcutaneous Ependymoma Misdiagnosed as Pilonidal Disease: Case Report and Review of the Literature. J. Surg. Case Rep. 2016, 2016, rjw121. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Sonneland, P.R.; Scheithauer, B.W.; Onofrio, B.M. Myxopapillary Ependymoma. A Clinicopathologic and Immunocytochemical Study of 77 Cases. Cancer 1985, 56, 883–893. [Google Scholar] [CrossRef]
- Helwig, E.B.; Stern, J.B. Subcutaneous Sacrococcygeal Myxopapillary Ependymoma. A Clinicopathologic Study of 32 Cases. Am. J. Clin. Pathol. 1984, 81, 156–161. [Google Scholar] [CrossRef]
- Benesch, M.; Mynarek, M.; Witt, H.; Warmuth-Metz, M.; Pietsch, T.; Bison, B.; Pfister, S.M.; Pajtler, K.W.; Kool, M.; Schüller, U.; et al. Newly Diagnosed Metastatic Intracranial Ependymoma in Children: Frequency, Molecular Characteristics, Treatment, and Outcome in the Prospective HIT Series. Oncologist 2019, 24, e921–e929. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Morantz, R.A.; Kepes, J.J.; Batnitzky, S.; Masterson, B.J. Extraspinal Ependymomas. Report of Three Cases. J. Neurosurg. 1979, 51, 383–391. [Google Scholar] [CrossRef] [PubMed]
- D’Angelo, P.; De Pasquale, M.D.; Barretta, F.; Affinita, M.C.; Conte, M.; Dall’Igna, P.; Di Cataldo, A.; Inserra, A.; Provenzi, M.; Quaglietta, L.; et al. Malignant Sacrococcygeal Germ Cell Tumors in Childhood: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Experience. Pediatr. Blood Cancer 2021, 68, e28812. [Google Scholar] [CrossRef]
- De Corti, F.; Sarnacki, S.; Patte, C.; Mosseri, V.; Baranzelli, M.C.; Martelli, H.; Conter, C.; Frappaz, D.; Orbach, D. Prognosis of Malignant Sacrococcygeal Germ Cell Tumours According to Their Natural History and Surgical Management. Surg. Oncol. 2012, 21, e31–e37. [Google Scholar] [CrossRef]
- Miralbell, R.; Louis, D.N.; O’Keeffe, D.; Rosenberg, A.E.; Suit, H.D. Metastatic Ependymoma of the Sacrum. Cancer 1990, 65, 2353–2355. [Google Scholar] [CrossRef]
- Rudà, R.; Reifenberger, G.; Frappaz, D.; Pfister, S.M.; Laprie, A.; Santarius, T.; Roth, P.; Tonn, J.C.; Soffietti, R.; Weller, M.; et al. EANO Guidelines for the Diagnosis and Treatment of Ependymal Tumors. Neuro Oncol. 2018, 20, 445–456. [Google Scholar] [CrossRef] [PubMed] [Green Version]
- Gerstner, E.R.; Pajtler, K.W. Ependymoma. Semin. Neurol. 2018, 38, 104–111. [Google Scholar] [CrossRef] [PubMed]
- Mogler, C.; Kohlhof, P.; Penzel, R.; Grenacher, L.; Haag, G.M.; Schirmacher, P.; Mueller, W. A Primary Malignant Ependymoma of the Abdominal Cavity: A Case Report and Review of the Literature. Virchows Arch. 2009, 454, 475–478. [Google Scholar] [CrossRef] [PubMed]
- Fegerl, G.; Marosi, C. Stabilization of Metastatic Myxopapillary Ependymoma with Sorafenib. Rare Tumors 2012, 4, e42. [Google Scholar] [CrossRef] [Green Version]
Authors | Age (Years) | Sex | Localization | Histology | Metastases | First-Line Treatment | Coccygectomy | Recurrence | Follow-up |
---|---|---|---|---|---|---|---|---|---|
Ciraldo 1986 [10] | 0.75 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | Yes | No | Alive |
Chou 1987 [11] | 16 | F | Subcutaneous sacrococcygeal | Myxopapillary | NA | Surgery | No | NA | NA |
Kramer 1988 [12] | 15 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | Yes, local and metastatic (inguinal nodes) | 20 years |
Marc’Hadour 1991 [13] | 14 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | No | 2 years |
Gupta 1992 [14] | 1.5 | M | Subcutaneous sacrococcygeal | Myxopapillary | Inguinal node | Surgery | No | Yes, local | NA |
Serour 1993 [15] | 8 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | Yes | No | 20 months |
Botti 1994 [16] | 10 | NA | Subcutaneous sacrococcygeal | Myxopapillary | Left buttock | Surgery | No | No | NA |
Kline 1996 [17] | 0.67 | F | Subcutaneous sacrococcygeal | Myxopapillary | Inguinal nodes | Surgery | No | Yes, metastatic (inguinal nodes, peritoneum) | Death |
Webber 1996 [18] | newborn | M | Presacral | NA | No | Surgery | NA | Yes, local | NA |
Sawyer 1998 [19] | 13 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | NA | NA |
Ilhan 1998 [20] | 8 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | Yes | No | 20 months |
Johnson 1999 [21] | 7 | M | Subcutaneous sacrococcygeal | Grade II | No | Surgery | Yes | No | 8 years |
Grubnic 1999 [22] | 8 | M | Subcutaneous sacrococcygeal | Myxopapillary | NA | Surgery | Yes | No | NA |
Aktuǧ 2000 [23] | 5 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | Yes | No | 3 years |
Rao 2002 [24] | 1.33 | F | Presacral | Myxopapillary | No | Surgery + CT | Yes | No | Alive |
Akpolat 2003 [25] | 7 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | No | Alive |
Tröbs 2006 [26] | 9 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | Yes | No | NA |
Beschorner 2007 [27] | 1.2 | M | Subcutaneous sacrococcygeal | Grade IV (Myxopapillary + areas of ependymoblastic differentiation) | No | Surgery | No | No | Alive |
Alexiou 2012 [28] | 13 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery + RT | No | No | 8 months |
Chakraborti 2012 [29] | 1 | F | Subcutaneous sacrococcygeal | Grade IV (Myxopapillary + areas of anaplastic differentiation) | No | Surgery | Yes | Yes, metastatic (inguinal nodes) | Alive |
Cimino 2014 (7 patients) [30] | 0–17 (range), 7.4 (mean) | 4F/3M | NA | Myxopapillary | NA | NA | NA | No | NA |
Dogan 2016 [31] | 9 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | No | Alive |
Amin 2018 [32] | 8 | F | Subcutaneous sacrococcygeal | Myxopapillary | NA | Surgery | Yes | No | Alive |
Schiavello 2018 [4] | 14 | M | Presacral | Grade II | No | Surgery | No | No | 6 years |
8 | F | Presacral | Grade II | inguinal nodes, osseus | CT + RT + Surgery | No | No | 26 years | |
4 | F | Subcutaneous sacrococcygeal | Myxopapillary | Parauterine nodes | Surgery | Yes | Yes, metastatic (neoplastic iliac thrombosis, lung, broad ligament, retroperitoneal and mediastinal nodes) | 15 years | |
12 | M | Subcutaneous sacrococcygeal | Grade II | No | Surgery | No | No | 11 years | |
16 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | No | 11 years | |
7 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | Yes, metastatic (inguinal nodes) | 33 years | |
Rogers 2018 [33] | 12 | F | Subcutaneous sacrococcygeal | Myxopapillary | NA | Surgery | No | No | 1 year |
Gupta 2020 [34] | 9 | M | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | No | No | 18 months |
Thejeel 2020 [35] | 16 | F | Subcutaneous sacrococcygeal | Myxopapillary | No | Surgery | NA | NA | NA |
Planas 2021 [36] | 12 | F | Subcutaneous sacrococcygeal | Giant cell | No | Surgery | No | No | 2 years |
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. |
© 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Share and Cite
Fabozzi, F.; Ceccanti, S.; Cacchione, A.; Colafati, G.S.; Carai, A.; Crocoli, A.; Mastronuzzi, A.; Cozzi, D.A. Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review. Diagnostics 2021, 11, 1680. https://doi.org/10.3390/diagnostics11091680
Fabozzi F, Ceccanti S, Cacchione A, Colafati GS, Carai A, Crocoli A, Mastronuzzi A, Cozzi DA. Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review. Diagnostics. 2021; 11(9):1680. https://doi.org/10.3390/diagnostics11091680
Chicago/Turabian StyleFabozzi, Francesco, Silvia Ceccanti, Antonella Cacchione, Giovanna Stefania Colafati, Andrea Carai, Alessandro Crocoli, Angela Mastronuzzi, and Denis A. Cozzi. 2021. "Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review" Diagnostics 11, no. 9: 1680. https://doi.org/10.3390/diagnostics11091680
APA StyleFabozzi, F., Ceccanti, S., Cacchione, A., Colafati, G. S., Carai, A., Crocoli, A., Mastronuzzi, A., & Cozzi, D. A. (2021). Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review. Diagnostics, 11(9), 1680. https://doi.org/10.3390/diagnostics11091680