Primary Cerebellar Neuroendocrine Tumors: Chimeras or Real Entities A Case Report with a 6-Year Follow-Up

Vernieri, Claudio; Femia, Daniela; Pusceddu, Sara; Capella, Carlo; Rosaí, Juan; Calareso, Giuseppina; Concas, Laura; Prinzi, Natalie; Russo, Giuseppe Lo; Braud, Filippo de; Buzzoni, Roberto

doi:10.1159/000447771

Cited by 6 publications

(7 citation statements)

References 12 publications

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“…Histomorphology in NECA is undifferentiated with solid or diffuse growth and nuclear atypia ( Klimstra et al, 2010 ; Oronsky et al, 2017 ). Previous case reports of primary CNS NETs include seven cases of differentiated NETs ( Porter et al, 2000 ; Deshaies et al, 2004 ; Ibrahim et al, 2010 ; Hood et al, 2014 ; Liu et al, 2016 ; Vernieri et al, 2016 ; Hakar et al, 2017 ) and three cases of NECAs ( Tamura et al, 2014 ; Liu et al, 2016 ; Reed et al, 2019 ). Primary CNS NECAs were described to stain positive for neuroendocrine markers, including chromogranin and synaptophysin, and negative for the glial marker GFAP.…”

Section: Discussionmentioning

confidence: 99%

Unique Finding of a Primary Central Nervous System Neuroendocrine Carcinoma in a 5-Year-Old Child: A Case Report

et al. 2022

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Neuroendocrine tumors (NETs) are rare neoplasms predominantly arising in the gastrointestinal-tract or the lungs of adults. To date, only ten cases of primary central nervous system (CNS) NETs have been reported, with just three of them describing a neuroendocrine carcinoma (NECA) and none occurring in a child. We report on a previously healthy 5-year-old boy, who presented with headaches, nausea and vomiting, and was diagnosed with a left cerebellar solid mass with a cystic component. After gross-total resection, histology revealed a neuroendocrine carcinoma. Molecular analysis of the tumor tissue showed a KRAS-splice-site mutation (c451-3C > T). The KRAS-mutation was discovered to be a maternal germline mutation, previously described as likely benign. After extensive search for an extracranial primary tumor, including Ga-68 DOTANOC-PET-CT, the diagnosis of a primary CNS NECA was established, and proton irradiation was performed. Unfortunately, the patient developed an in-field recurrence just 5 weeks after the end of radiotherapy. The tumor was re-resected with vital tumor tissue. Six cycles of chemotherapy were initiated, consisting of cisplatin, carboplatin, etoposide and ifosfamide. The patient remains disease free 22 months after the end of treatment, supporting the beneficial effect of platinum- and etoposide-based chemotherapy for this tumor entity.

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Section: Discussionmentioning

confidence: 99%

Unique Finding of a Primary Central Nervous System Neuroendocrine Carcinoma in a 5-Year-Old Child: A Case Report

et al. 2022

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“…Whenever the brain shows neuroendocrine carcinoma, around 75% of patients have lymph node metastases and liver metastases in 50% of patients [6]. It is necessary to differentiate primary from the metastatic neuroendocrine tumor, because the primary neuroendocrine carcinoma of the brain has a much better prognosis as compared to metastatic neuroendocrine carcinoma [6,7]. Usually, the patients with metastatic neuroendocrine carcinoma of the brain die due to systemic disease progression as suggested by Mallory et al [8].…”

Section: Discussionmentioning

confidence: 99%

journey of neuroendocrine carcinoma of the brain from the primary to metastatic tumor

Chaudhari¹,

Patel²,

Pradhan³

et al. 2022

Indian J Case Reports

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The neuroendocrine tumors most commonly originate in the lung, liver, and gastrointestinal tract. They can be graded into a three-tiered system depending on their proliferation index. These tumors frequently metastasize to the regional lymph nodes. Neuroendocrine tumors of the brain are very rare. Most of them are metastatic and a handful of cases are primary. Hereby, we present a case of brain neuroendocrine carcinoma, which was solitary with no evidence of the primary tumor in the body at the time of neurosurgery. However, treating physicians vigilantly kept him on regular follow-up and investigated further. The patient was diagnosed to have a lesion in the lung 3 months after surgery. Thus, it was considered primary neuroendocrine carcinoma of the brain; however, regular follow-up and further positron emission tomography scan showed a small mass in the lung and adrenal gland. Based on this, a final diagnosis of metastatic neuroendocrine carcinoma of the brain was made.

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“…4) Primary intracranial NETs are extremely rare entities, and to date, only 12 cases have been reported in the literature. 1,[4][5][6][7][8][9][10][11][12][13] Most were located in the skull base and sellar regions, except for one case each in the third ventricle, 1) frontal convexity, 6) parietal lobe, 9) and cerebellum. 13) Based on the pathological findings of the present case, we consider that the differential diagnosis of NETs should include esthesioneuroblastoma and ectopic sparsely granulated corticotroph adenoma (SGCA).…”

Section: Discussionmentioning

confidence: 99%

“… 4) Primary intracranial NETs are extremely rare entities, and to date, only 12 cases have been reported in the literature. 1 , 4 – 13) Most were located in the skull base and sellar regions, except for one case each in the third ventricle, 1) frontal convexity, 6) parietal lobe, 9) and cerebellum. 13) …”

Section: Discussionmentioning

confidence: 99%

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Primary Intracranial Neuroendocrine Tumor of the Skull Base Complicated with Tension Pneumocephalus after Radiotherapy

Kambe

Nakajima

Fukushima

et al. 2021

NMC Case Report Journal

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Neuroendocrine tumors (NETs) are neoplasms that originate from cells of the endocrine and nervous systems, and are commonly found in the gastrointestinal and respiratory tracts. Primary intracranial NETs are extremely rare and have been the focus of only a few studies thus far. Herein, we report the case of a primary intracranial NET of the skull base complicated with tension pneumocephalus after radiotherapy. An 84-year-old woman visited a local hospital for a head injury, and CT revealed a skull base tumor. MRI showed that the tumor was located mainly on the clivus and extended into the paranasal sinuses and nasal cavity. We biopsied the tumor via the nasal cavity, and the pathological diagnosis was NET, WHO grade 2. We subsequently administered focal intensity-modulated radiation therapy, but the patient developed tension pneumocephalus 1 year after radiotherapy. We therefore performed endoscopic transnasal cerebrospinal fluid leak closure with a nasoseptal flap. The postoperative course was successful, and the patient returned home but died of an unknown cause 2 years after discharge. The optimal postoperative management of primary intracranial NETs remains controversial. Tension pneumocephalus related to radiotherapy is a rare complication. Assessing skull bone erosion before radiotherapy and performing regular radiological follow-up examinations are essential to prevent this rare complication.

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Primary Cerebellar Neuroendocrine Tumors: Chimeras or Real Entities A Case Report with a 6-Year Follow-Up

Cited by 6 publications

References 12 publications

Unique Finding of a Primary Central Nervous System Neuroendocrine Carcinoma in a 5-Year-Old Child: A Case Report

Unique Finding of a Primary Central Nervous System Neuroendocrine Carcinoma in a 5-Year-Old Child: A Case Report

journey of neuroendocrine carcinoma of the brain from the primary to metastatic tumor

Primary Intracranial Neuroendocrine Tumor of the Skull Base Complicated with Tension Pneumocephalus after Radiotherapy

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