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

Martina Piloni; Filippo Gagliardi; Michele Bailo; Lina Raffaella Barzaghi; Marcella Callea; Mariarosa Terreni; Andrea Falini; Antonella Castellano; Pietro Mortini

doi:10.1055/s-0040-1721022

Journal of Neurological Surgery Part A: Central European Neurosurgery, Inhaltsverzeichnis

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

¹Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Filippo Gagliardi

¹Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Michele Bailo

¹Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Lina Raffaella Barzaghi

¹Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Marcella Callea

²Department of Pathology, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Mariarosa Terreni

²Department of Pathology, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Andrea Falini

³Department of Neuroradiology and CERMAC, Vita-Salute San Raffaele University, and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Antonella Castellano

³Department of Neuroradiology and CERMAC, Vita-Salute San Raffaele University, and IRCCS San Raffaele Scientific Institute, Milan, Italy

,

Pietro Mortini

¹Department of Neurosurgery and Gamma Knife Radiosurgery, Vita-Salute San Raffaele University and IRCCS San Raffaele Scientific Institute, Milan, Italy

› Institutsangaben

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

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Referenzen

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