Download PDF
J Neurol Surg A Cent Eur Neurosurg 2022; 83(01): 089-098
DOI: 10.1055/s-0040-1721022
Case Report

Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm

Martina Piloni
1   Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Filippo Gagliardi
1   Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Michele Bailo
1   Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Lina Raffaella Barzaghi
1   Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Marcella Callea
2   Department of Pathology, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Mariarosa Terreni
2   Department of Pathology, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Andrea Falini
3   Department of Neuroradiology and CERMAC, Vita-Salute San Raffaele University, and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Antonella Castellano
3   Department of Neuroradiology and CERMAC, Vita-Salute San Raffaele University, and IRCCS San Raffaele Scientific Institute, Milan, Italy
,
Pietro Mortini
1   Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy
› Author Affiliations
› Further Information
    Permissions and Reprints

Abstract

Background Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways.

Material and Methods A 57-year-old man presenting with acute visual acuity worsening and left homonymous hemianopia was referred to our hospital. Neuroradiologic studies demonstrated an infiltrating, high-grade lesion involving the optic chiasm and right retrochiasmatic pathways with a hemorrhagic area in the ipsilateral pulvinar.

Results The patient underwent microsurgical biopsy. Pathologic assessment confirmed the diagnosis of CNS embryonal tumor, not otherwise specified (NOS) according to the 2016 World Health Organization (WHO) classification of CNS tumors. The patient was referred to a multimodal adjuvant treatment; he eventually died 4 months after surgery. Competent literature has been systematically reviewed in the light of the relevant changes made in the last version of the WHO classification.

Conclusion Embryonal tumors should be considered in the differential diagnosis for sellar and suprasellar space-occupying lesions, despite the rarity of the disease and the uncommon features at time of presentation. As per our knowledge, this is the first case ever described of hemorrhagic suprasellar embryonal tumor with a diffusion pattern along white matter fibers. Histogenesis, biomolecular and neuroradiologic features, and classification of embryonal tumors are an open field of research, with considerable implications for the definition of better diagnostic pitfalls and therapeutic regimens.

Keywords

adult - embryonal tumors - hemorrhage - suprasellar - supratentorial primitive neuroectodermal tumors

Ethical Approval

All the procedures performed in the study involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.


Informed Consent

Informed consent was obtained from all individual participants included in the study.




Publication History

Received: 16 January 2020

Accepted: 19 March 2020

Article published online:
21 January 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Hart MN, Earle KM. Primitive neuroectodermal tumors of the brain in children. Cancer 1973; 32 (04) 890-897
    CrossrefPubMedGoogle Scholar
  • 2 Louis DN, Ohgaki H, Wiestler OD. et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 2007; 114 (02) 97-109
    CrossrefPubMedGoogle Scholar
  • 3 Gaffney CC, Sloane JP, Bradley NJ, Bloom HJ. Primitive neuroectodermal tumours of the cerebrum. Pathology and treatment. J Neurooncol 1985; 3 (01) 23-33
    CrossrefPubMedGoogle Scholar
  • 4 Massimino M, Gandola L, Spreafico F. et al. Supratentorial primitive neuroectodermal tumors (S-PNET) in children: a prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy. Int J Radiat Oncol Biol Phys 2006; 64 (04) 1031-1037
    CrossrefPubMedGoogle Scholar
  • 5 Kim DG, Lee DY, Paek SH, Chi JG, Choe G, Jung HW. Supratentorial primitive neuroectodermal tumors in adults. J Neurooncol 2002; 60 (01) 43-52
    CrossrefPubMedGoogle Scholar
  • 6 Ohba S, Yoshida K, Hirose Y, Ikeda E, Kawase T. A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. J Neurooncol 2008; 86 (02) 217-224
    CrossrefPubMedGoogle Scholar
  • 7 Balafouta MJ, Kouvaris JR, Miliadou AC. et al. Primitive neuroectodermal tumour in a 60-year-old man: a case report and literature review. Br J Radiol 2003; 76 (901) 62-65
    CrossrefPubMedGoogle Scholar
  • 8 Bellis EH, Salcman M, Bastian FO. Primitive neuroectodermal tumor in a 57-year-old man. Surg Neurol 1983; 20 (01) 30-35
    CrossrefPubMedGoogle Scholar
  • 9 Han SR, Sohn MJ, Yoon SW. et al. Extracranial metastases of a supratentorial primitive neuroectodermal tumour. J Clin Neurosci 2007; 14 (01) 55-58
    CrossrefPubMedGoogle Scholar
  • 10 Krampulz T, Hans VH, Oppel F, Dietrich U, Puchner MJ. Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report. J Neurooncol 2006; 77 (03) 291-294
    CrossrefPubMedGoogle Scholar
  • 11 Kuratsu J, Matsukado Y, Seto H, Nonaka N, Sueyoshi N. Primitive neuroectodermal tumor arising from the thalamus of an adult: case report. Neurol Med Chir (Tokyo) 1986; 26 (01) 30-34
    CrossrefPubMedGoogle Scholar
  • 12 Lawandy S, Hariri OR, Miulli DE. et al. Supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. J Med Case Reports 2012; 6: 361
    CrossrefPubMedGoogle Scholar
  • 13 Pickuth D, Leutloff U. Computed tomography and magnetic resonance imaging findings in primitive neuroectodermal tumours in adults. Br J Radiol 1996; 69 (817) 1-5
    CrossrefPubMedGoogle Scholar
  • 14 Sanno N, Shimura T, Takumi I. et al. An adult case of cerebral primitive neuroectodermal tumour. Acta Neurochir (Wien) 1998; 140 (05) 445-446
    CrossrefPubMedGoogle Scholar
  • 15 Shingu T, Kagawa T, Kimura Y, Takada D, Moritake K, Hoshii Y. Supratentorial primitive neuroectodermal tumor in an aged patient: case report. Neurol Med Chir (Tokyo) 2005; 45 (10) 530-535
    CrossrefPubMedGoogle Scholar
  • 16 Abuzayed B, Khreisat W, Maaiah W, Agailat S. Supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in adult. J Neurosci Rural Pract 2014; 5 (02) 176-179
    Article in Thieme ConnectPubMedGoogle Scholar
  • 17 Black-Tiong SP, Sandler SJ, Otto S, Wells AJ. Adult supratentorial primitive neuroectodermal tumour presenting as intracranial haemorrhage: case report. J Clin Neurosci 2017; 37: 41-42
    CrossrefPubMedGoogle Scholar
  • 18 Papadopoulos EK, Fountas KN, Brotis AG, Paterakis KN. A supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in a 42-year-old man: a case report and review of the literature. J Med Case Reports 2013; 7: 86
    CrossrefPubMedGoogle Scholar
  • 19 Berwaerts JVJ, Dua G, Abs R, Neetens I, Mahler C. A pseudo-endocrine suprasellar tumour: review of primitive neuroectodermal tumours in adults. Endocr Relat Cancer 1998; 5: 303-310
    CrossrefPubMedGoogle Scholar
  • 20 Dupuy M, Bonneville F, Grunenwald S. et al. Primary sellar neuroblastoma. A new case and review of literature. Ann Endocrinol (Paris) 2012; 73 (03) 216-221
    CrossrefPubMedGoogle Scholar
  • 21 Lin JH, Tsai DH, Chiang YH. A primary sellar esthesioneuroblastomas with unusual presentations: a case report and reviews of literatures. Pituitary 2009; 12 (01) 70-75
    CrossrefPubMedGoogle Scholar
  • 22 Oyama K, Yamada S, Usui M, Kovacs K. Sellar neuroblastoma mimicking pituitary adenoma. Pituitary 2005; 8 (02) 109-114
    CrossrefPubMedGoogle Scholar
  • 23 Radotra B, Apostolopoulos V, Sandison A. et al. Primary sellar neuroblastoma presenting with syndrome of inappropriate secretion of anti-diuretic hormone. Endocr Pathol 2010; 21 (04) 266-273
    CrossrefPubMedGoogle Scholar
  • 24 Sajko T, Rumboldt Z, Talan-Hranilovic J, Radic I, Gnjidic Z. Primary sellar esthesioneuroblastoma. Acta Neurochir (Wien) 2005; 147 (04) 447-448 , discussion 448
    CrossrefPubMedGoogle Scholar
  • 25 Sarwar M. Primary sellar-parasellar esthesioneuroblastoma. AJR Am J Roentgenol 1979; 133 (01) 140-141
    CrossrefPubMedGoogle Scholar
  • 26 Huang BY, Castillo M. Nonadenomatous tumors of the pituitary and sella turcica. Top Magn Reson Imaging 2005; 16 (04) 289-299
    CrossrefPubMedGoogle Scholar
  • 27 Kleinschmidt-DeMasters BK, Lopes MB, Prayson RA. An algorithmic approach to sellar region masses. Arch Pathol Lab Med 2015; 139 (03) 356-372
    CrossrefPubMedGoogle Scholar
  • 28 Espino Barros Palau A, Khan K, Morgan ML, Powell SZ, Lee AG. Suprasellar primitive neuroectodermal tumor in an adult. J Neuroophthalmol 2016; 36 (03) 299-303
    CrossrefPubMedGoogle Scholar
  • 29 Lach B, Rippstein P, Benott BG, Staines W. Differentiating neuroblastoma of pituitary gland: neuroblastic transformation of epithelial adenoma cells. Case report. J Neurosurg 1996; 85 (05) 953-960
    CrossrefPubMedGoogle Scholar
  • 30 Mariani L, Schaller B, Weis J, Ozdoba C, Seiler RW. Esthesioneuroblastoma of the pituitary gland: a clinicopathological entity? Case report and review of the literature. J Neurosurg 2004; 101 (06) 1049-1052
    CrossrefPubMedGoogle Scholar
  • 31 Roy A, Timothy J, Anthony R, Chakrabarty A. Correspondence: aesthesioneuroblastoma arising in pituitary gland. Neuropathol Appl Neurobiol 2000; 26 (02) 177-179
    CrossrefPubMedGoogle Scholar
  • 32 Schmalisch K, Psaras T, Beschorner R, Honegger J. Sellar neuroblastoma mimicking a pituitary tumour: case report and review of the literature. Clin Neurol Neurosurg 2009; 111 (09) 774-778
    CrossrefPubMedGoogle Scholar
  • 33 Gandhi R, Babu R, Cummings TJ, Adamson C. Adult primitive neuroectodermal tumors: the prognostic value of supratentorial location. J Neurooncol 2013; 114 (01) 141-148
    CrossrefPubMedGoogle Scholar
  • 34 Zheng YC, Jung SM, Lee ST. et al. Adult supratentorial extra-pineal primitive neuro-ectodermal tumors. J Clin Neurosci 2014; 21 (05) 803-809
    CrossrefPubMedGoogle Scholar
  • 35 Smoll NR. Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs). Cancer 2012; 118 (05) 1313-1322
    CrossrefPubMedGoogle Scholar
  • 36 Pomeroy SL, Tamayo P, Gaasenbeek M. et al. Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature 2002; 415 (6870): 436-442
    CrossrefPubMedGoogle Scholar
  • 37 Janss AJ, Yachnis AT, Silber JH. et al. Glial differentiation predicts poor clinical outcome in primitive neuroectodermal brain tumors. Ann Neurol 1996; 39 (04) 481-489
    CrossrefPubMedGoogle Scholar
  • 38 Visée S, Soltner C, Rialland X. et al. Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients. Histopathology 2005; 46 (04) 403-412
    CrossrefPubMedGoogle Scholar
  • 39 Chen L, Wang W, Zhou R. et al. Characteristics of fecal and mucosa-associated microbiota in Chinese patients with inflammatory bowel disease. Medicine (Baltimore) 2014; 93 (08) e51
    CrossrefPubMedGoogle Scholar
  • 40 Yang HJ, Nam DH, Wang KC, Kim YM, Chi JG, Cho BK. Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. Childs Nerv Syst 1999; 15 (08) 377-383
    CrossrefPubMedGoogle Scholar
  • 41 Gessi M, Setty P, Bisceglia M. et al. Supratentorial primitive neuroectodermal tumors of the central nervous system in adults: molecular and histopathologic analysis of 12 cases. Am J Surg Pathol 2011; 35 (04) 573-582
    CrossrefPubMedGoogle Scholar
  • 42 Shih RY, Koeller KK. Embryonal tumors of the central nervous system: from the radiologic pathology archives. Radiographics 2018; 38 (02) 525-541
    CrossrefPubMedGoogle Scholar
  • 43 Erdem E, Zimmerman RA, Haselgrove JC, Bilaniuk LT, Hunter JV. Diffusion-weighted imaging and fluid attenuated inversion recovery imaging in the evaluation of primitive neuroectodermal tumors. Neuroradiology 2001; 43 (11) 927-933
    CrossrefPubMedGoogle Scholar
  • 44 Klisch J, Husstedt H, Hennings S, von Velthoven V, Pagenstecher A, Schumacher M. Supratentorial primitive neuroectodermal tumours: diffusion-weighted MRI. Neuroradiology 2000; 42 (06) 393-398
    CrossrefPubMedGoogle Scholar
  • 45 Chawla A, Emmanuel JV, Seow WT, Lou J, Teo HE, Lim CC. Paediatric PNET: pre-surgical MRI features. Clin Radiol 2007; 62 (01) 43-52
    CrossrefPubMedGoogle Scholar
  • 46 Kovanlikaya A, Panigrahy A, Krieger MD. et al. Untreated pediatric primitive neuroectodermal tumor in vivo: quantitation of taurine with MR spectroscopy. Radiology 2005; 236 (03) 1020-1025
    CrossrefPubMedGoogle Scholar
  • 47 Majós C, Alonso J, Aguilera C. et al. Adult primitive neuroectodermal tumor: proton MR spectroscopic findings with possible application for differential diagnosis. Radiology 2002; 225 (02) 556-566
    CrossrefPubMedGoogle Scholar
  • 48 Gessi M, Giangaspero F, Lauriola L. et al. Embryonal tumors with abundant neuropil and true rosettes: a distinctive CNS primitive neuroectodermal tumor. Am J Surg Pathol 2009; 33 (02) 211-217
    CrossrefPubMedGoogle Scholar
  • 49 Louis DN, Perry A, Reifenberger G. et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 2016; 131 (06) 803-820
    CrossrefPubMedGoogle Scholar
  • 50 Korshunov A, Sturm D, Ryzhova M. et al. Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol 2014; 128 (02) 279-289
    CrossrefPubMedGoogle Scholar
  • 51 Caporlingua A, Armocida D, Caporlingua F. et al. Disseminated cerebrospinal embryonal tumor in the adult. Case Rep Pathol 2016; 2016: 6785459
    PubMedGoogle Scholar
  • 52 Tariq MU, Ahmad Z, Minhas MK, Memon A, Mushtaq N, Hawkins C. Embryonal tumor with multilayered rosettes, C19MC-altered: report of an extremely rare malignant pediatric central nervous system neoplasm. SAGE Open Med Case Rep 2017; 5: X17745208
    PubMedGoogle Scholar
  • 53 Kouyialis AT, Boviatsis EI, Karampelas IK, Korfias S, Korkolopoulou P, Sakas DE. Primitive supratentorial neuroectodermal tumor in an adult. J Clin Neurosci 2005; 12 (04) 492-495
    CrossrefPubMedGoogle Scholar
  • 54 Biswas S, Burke A, Cherian S. et al. Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?. Pediatr Blood Cancer 2009; 52 (07) 796-803
    CrossrefPubMedGoogle Scholar
  • 55 Packer RJ, Sutton LN, Elterman R. et al. Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy. J Neurosurg 1994; 81 (05) 690-698
    CrossrefPubMedGoogle Scholar