Primary Neuroendocrine Carcinoma of the Cerebellopontine Angle: A Case Report and Literature Review

Abstract

Primary intracranial neuroendocrine tumors are extremely rare malignancies with very few cases reported in the world literature. We describe a primary neuroendocrine carcinoma arising from the right cerebellopontine angle, the second case that has been described in this location. The possible origin in this place and treatment are described.A 29-year-old male patient, diagnosed with schwannoma of the right cerebellopontine angle, and treated with radiosurgery at another institution, came to our hospital six months later, The patient presented with a history of rapid progression of numbness on the right side of the face, diplopia, dizziness, vomiting, and facial palsy. On examination, the right cranial nerves V, VI, VII, VIII, and IX were affected. The MRI showed tumor growth occupying the right cerebellopontine angle, with compression of the brain stem and cerebellum. A right retromastoid craniectomy removed the tumor partially and the histopathological examination revealed a high-grade neuroendocrine carcinoma.We describe a primary neuroendocrine tumor of the brain that, despite its rarity, must be considered in the differential diagnosis. There are currently no guidelines for the management of these tumors. According to previously reported cases, surgery is the first line of treatment, followed by radiotherapy or chemotherapy. We consider that such a rare case is needed to be reported for a better understanding of the disease and its neurobiology.

A rare case of primary neuroendocrine carcinoma of the central nervous system

“…This unique property helps in the detection of this tumor. 9 Intracranial NENs are mostly metastatic, and their incidence is <5%, with an extracranial primary tumor. 6 Detection of the primary site is challenging, and radiological investigations, including PET/computed tomography and somatostatin receptor scintigraphy (SRS), proved to be very helpful.…”

“…15 Some authors recommend surgery followed by chemotherapy and radiotherapy, while others suggest platinum-based chemotherapy. 9,16 It is important to correctly diagnose this rare intracranial tumor as its prognosis is better in comparison to metastatic neuroendocrine tumors.…”

“…Neuroendocrine cells are naturally involved in the synthesis and secretion of biologically active amine, as are NENs. This unique property helps in the detection of this tumor 9 . Intracranial NENs are mostly metastatic, and their incidence is <5%, with an extracranial primary tumor 6 .…”

“…However, there is still no consensus regarding the standard protocol for treating intracranial NETs since many factors influence treatment, including age, neurological state, histological differentiation, location, and locoregional extension of the tumor 15 . Some authors recommend surgery followed by chemotherapy and radiotherapy, while others suggest platinum‐based chemotherapy 9,16 …”