Thoracic Extradural Paraganglioma Localized on 68Ga-DOTANOC PET/CT

Sachani, Hemant; Tripathi, Madhavi; Madhusudan, Kumble S.; Semalti, Kapil; Shanker, Shiv; Arunraj, Sreedharan Thankarajan; Bal, Chandrasekhar

doi:10.1097/rlu.0000000000003643

Cited by 2 publications

(1 citation statement)

References 8 publications

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“…Cauda equina neuroendocrine tumors usually affect adults, with a peak of incidence in the fourth–sixth decades of life, with a median age of 46 years (range 6–85) [ 58 ]. Notably, when neuroendocrine tumors affect the spinal cord, the cauda equina represents the most frequent location, but uncommon locations in the cervical and thoracic regions have been reported [ 59 , 60 , 61 ]. Most of cauda equina neuroendocrine tumors are considered sporadic, with slow-growing behavior, and neurological symptoms correlated to the site of presentation, including low back and/or radicular pain, numbness, paraparesis, sphincter symptoms, or complete cauda equina syndrome [ 62 , 63 ].…”

Section: Epidemiology and Clinical Featuresmentioning

confidence: 99%

Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

Pellerino

Verdijk

Nichelli

et al. 2023

Cancers

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The 2021 WHO classification of the CNS Tumors identifies as “Peripheral nerve sheath tumors” (PNST) some entities with specific clinical and anatomical characteristics, histological and molecular markers, imaging findings, and aggressiveness. The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is particularly low due to the rarity, and drawn recommendations accordingly. Tumor diagnosis is primarily based on hematoxylin and eosin-stained sections and immunohistochemistry. Molecular analysis is not essential to establish the histological nature of these tumors, although genetic analyses on DNA extracted from PNST (neurofibromas/schwannomas) is required to diagnose mosaic forms of NF1 and SPS. MRI is the gold-standard to delineate the extension with respect to adjacent structures. Gross-total resection is the first choice, and can be curative in benign lesions; however, the extent of resection must be balanced with preservation of nerve functioning. Radiotherapy can be omitted in benign tumors after complete resection and in NF-related tumors, due to the theoretic risk of secondary malignancies in a tumor-suppressor syndrome. Systemic therapy should be considered in incomplete resected plexiform neurofibromas/MPNSTs. MEK inhibitor selumetinib can be used in NF1 children ≥2 years with inoperable/symptomatic plexiform neurofibromas, while anthracycline-based treatment is the first choice for unresectable/locally advanced/metastatic MPNST. Clinical trials on other MEK1-2 inhibitors alone or in combination with mTOR inhibitors are under investigation in plexiform neurofibromas and MPNST, respectively.

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