Document Type : case report

Authors

1 Mohammad Hasanpour , Assistant professor , Department of neurosurgery, Iran university of medical science.

2 Medical Doctor, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

3 Professor of Pathology, Infectious Diseases and Tropical Medicine Research Center, Department of Pathology, Isfahan University of Medical Sciences, Isfahan, Iran

4 Medical Doctor, Digestive disease research institute, Shariati hospital, Tehran University of Medical Sciences, Tehran, Iran

5 Department of neurosurgery,faculty of medicine , Mashhad university of medical sciences , Mashhad ,Iran

Abstract

Case Report: A 2-year-old girl was referred with the chief complaint of limb weakness following a mild trauma. She had been suffering from restlessness and neck pain for a month. Laboratory findings were normal. In MRI, there was evidence of craniocervical junction extra-axial mass lesion arising from the posterior aspect of the dense process ligamentous complex extending from the foramen magnum to the posterior fossa with engulfment of the right vertebral artery. Regarding the compressive effect of the tumor, a right trans-condylar suboccipital surgical approach was used to resection the mass, near totally, and decompress the brain stem. Immune-Histo-Chemical Staining (IHC) showed a grade 2 meningioma. Low-dose radiotherapy was applied; but the pathology result corroborated the tumor’s radiologic features. Due to the follow-up MRI evidence of aggressive tumor recurrence the clinical behavior of the tumor and the patient's progression, there was a possibility that the first diagnosis was not correct; hence, a second operation was performed during which a smaller portion of the tumor could be resected compared with the first operation. Pathological study and IHC staining reported MRT and tumor markers, including pan-cytokeratin (CK), epithelial membrane antigen (EMA), and vimentin, were strongly positive. So, a chemotherapy regimen was added to radiotherapy. Unfortunately, the patient did not respond well to the follow-up treatment, and she expired after one year.
Conclusion: In similar cases, where the radiological and pathological features of the tumor are atypical, the histological examination should include molecular examination, as meningioma in this age group is extremely rare. And, confirming the pathological and molecular characteristics of the tumor by different experts is strongly recommended.

Keywords

  1. Adesina AM, Tihan T, Fuller CE, Poussaint TY. Atlas of pediatric brain tumors: Springer; 2016.
  2. Buscariollo DL, Park HS, Roberts KB, Yu JB. Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis. Cancer. 2012; 118(17):4212-9.
  3. Geller JI, Roth JJ, Biegel JA. Biology and Treatment of Rhabdoid Tumor. Crit Rev Oncog. 2015; 20 (3-4):199-216.
  4. Li D, Heiferman DM, Syed HR, Santos JG, Bowman RM, DiPatri AJ, Tomita T, Wadhwani NR, Alden TD. Pediatric primary spinal atypical teratoid rhabdoid tumor: a case series and review of the literature. Journal of neurosurgery Pediatrics. 2019:1-17.
  5. Abu Arja MH, Patel P, Shah SH, Auletta JJ, Meyer EK, Conley SE, Aldrink JH, Pindrik JA, AbdelBaki MS. Synchronous Central Nervous System Atypical Teratoid/Rhabdoid Tumor and Malignant Rhabdoid Tumor of the Kidney: Case Report of a Long-Term Survivor and Review of the Literature. World neurosurgery. 2018; 111:6-15.
  6. Liu Y-C, Chang Y-S, Liang W-X, Wan K-S. A newborn with malignant Rhabdoid tumors: A visual diagnosis case. IP International Journal of Medical Paediatrics and Oncology. 2018; 4(4).
  7. Saunders J, Ingley K, Wang XQ, Harvey M, Armstrong L, Ng T, Dunham C, Bush J. Loss of BRG1 (SMARCA4) Immunoexpression in a Pediatric Non-Central Nervous System Tumor Cohort. Pediatric and developmental pathology: the official journal of the Society for Pediatric Pathology and the Pediatric Pathology Society. 2020; 23(2):132-8.
  8. Baker TG, Lyons MJ, Leddy L, Parham DM, Welsh CT. Epithelioid Sarcoma Arising in a Long-Term Survivor of an Atypical Teratoid/Rhabdoid Tumor in a Patient With Rhabdoid Tumor Predisposition Syndrome. Pediatric and developmental pathology: the official journal of the Society for Pediatric Pathology and the Pediatric Pathology Society. 2021; 24(2):164-8.
  9. Li J, Zhang W, Hu H, Zhang Y, Wang Y, Gu H, Huang D. Case Analysis of 14 Children with Malignant Rhabdoid Tumor of the Kidney. Cancer Manag Res. 2021; 13:4865-72.
  10. Tsipou H, Roka K, Gavra M, Glentis S, Stefanaki K, Kattamis A. Congenital extracranial extrarenal rhabdoid tumor: a rare clinicopathologic entity and diagnostic challenge. Liver. 2020; 4:5.
  11. Benesch M, Bartelheim K, Fleischhack G, Gruhn B, Schlegel PG, Witt O, Stachel KD, Hauch H, Urban C, Quehenberger F, Massimino M, Pietsch T, Hasselblatt M, Giangaspero F, Kordes U, Schneppenheim R, Hauser P, Klingebiel T, Frühwald MC. High-dose chemotherapy (HDCT) with auto-SCT in children with atypical teratoid/rhabdoid tumors (AT/RT): a report from the European Rhabdoid Registry (EU-RHAB). Bone Marrow Transplant. 2014; 49(3):370-5.
  12. Dardis C, Yeo J, Milton K, Ashby LS, Smith KA, Mehta S, Youssef E, Eschbacher J, Tucker K, Dawes L, Lambie N, Algar E, Hovey E. Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment. Front Neurol. 2017; 8:247.
  13. Ahmeda H, Darwisha EA, Abo-bakrkhattabb OM. Role of Diffusion Mri In Differentiation Between The Common Pediatric Posterior Fossa Brain Tumors. The Egyptian Journal of Hospital Medicine. 2018; 73(2):6090-6.
  14. Sali AP, Epari S, Nagaraj TS, Sahay A, Chinnaswamy G, Shetty P, Moiyadi A, Gupta T. Atypical Teratoid/Rhabdoid Tumor: Revisiting Histomorphology and Immunohistochemistry With Analysis of Cyclin D1 Overexpression and MYC Amplification. Int J Surg Pathol. 2021; 29(2):155-64.
  15. Krishnan C, Vogel H, Perry A. Atypical teratoid/rhabdoid tumor with ganglioglioma-like differentiation: case report and review of the literature. Hum Pathol. 2014; 45(1):185-8.
  16. Tahir F, Majid Z, Qadar LT, Abbas A, Raza M. Co-existent Rhabdoid Tumor of The Kidney and Brain in a Male Infant: A Rare Case. Cureus. 2019; 11(8):e5423.
  17. Shiflett JM, Herrington BL, Joyner DA, Saad AG. Atypical teratoid rhabdoid tumor of the cauda equina in a child: report of a very unusual case. Applied Immunohistochemistry & Molecular Morphology. 2020; 28(7):e58-e62.
  18. Menezes AH. Craniovertebral junction neoplasms in the pediatric population. Childs Nerv Syst. 2008; 24(10):1173-86.
  19. Mocco J, Laufer I, Mack WJ, Winfree CJ, Libien J, Connolly ES, Jr. An extramedullary foramen magnum cavernous malformation presenting with acute subarachnoid hemorrhage: case report and literature review. Neurosurgery. 2005; 56(2):E410; discussion E.
  20. Kumar R. Primary malignant rhabdoid tumors of the brain, clinicoradiological findings of two cases. Neurology India. 1999; 47(4):314-7.
  21. Rahmat K, Kua CH, Ramli N. A child with atypical teratoid/rhabdoid tumor of the posterior cranial fossa. Singapore medical journal. 2008; 49(12):e365-8.
  22. Abolfotoh M, Tavanaiepour D, Hong C, Dunn IF, Lidov H, Al-Mefty O. Primary calcified rhabdoid meningioma of the cranio-cervical junction: A case report and review of literature. J Craniovertebral Junction Spine. 2012; 3(1):32-7.
  23. Wolfe AD, Capitini CM, Salamat SM, DeSantes K, Bradley KA, Kennedy T, Dehner LP, Patel NJ. Neck Rhabdoid Tumors: Clinical Features and Consideration of Autologous Stem Cell Transplant. Journal of pediatric hematology/oncology. 2018; 40(1):e50-e4.
  24. Shih RY, Koeller KK. Embryonal Tumors of the Central Nervous System: From the Radiologic Pathology Archives. Radiographics. 2018; 8(2):525-41.
  25. Sigauke E, Rakheja D, Maddox DL, Hladik CL, White CL, Timmons CF, Raisanen J. Absence of expression of SMARCB1/INI1 in malignant rhabdoid tumors of the central nervous system, kidneys and soft tissue: an immunohistochemical study with implications for diagnosis. Mod Pathol. 2006; 19(5):717-25.
  26. Menke JR, Raleigh DR, Gown AM, Thomas S, Perry A, Tihan T. Somatostatin receptor 2a is a more sensitive diagnostic marker of meningioma than epithelial membrane antigen. Acta neuropathologica. 2015; 130(3):441-3.
  27. Eaton KW, Tooke LS, Wainwright LM, Judkins AR, Biegel JA. Spectrum of SMARCB1/INI1 mutations in familial and sporadic rhabdoid tumors. Pediatr Blood Cancer. 2011; 56(1):7-15.
  28. Stabouli S. Pediatric Atypical Teratoid/Rhabdoid Tumor in the Spine: Diagnosis and Treatment. Pediatric Cancer, Volume 3: Springer; 2012. p. 55-62.