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cauda equina neuroendocrine tumor

Description

Cauda Equina Neuroendocrine Tumor (CENET): A Rare Spinal Tumor

A cauda equina neuroendocrine tumor (CENET) is a rare type of central nervous system (CNS) tumor that typically affects the cauda equina or filum terminale region of the spine. This neoplasm is characterized by its low-grade and well-circumscribed nature, making it distinct from other types of spinal tumors.

Key Features:

  • Location: CENETs are located in the cauda equina or filum terminale region of the spine.
  • Grade: These tumors are typically low-grade, indicating a slower growth rate compared to high-grade tumors.
  • Circumscription: CENETs are well-circumscribed, meaning they have a clear boundary and do not invade surrounding tissues.

Symptoms:

The most common presenting symptoms of CENET include lower back pain and sciatica due to mass effect. In some cases, patients may experience neuropathy or other neurological symptoms depending on the tumor's location and size.

References:

  • [2] describes CENET as a low-grade, well-circumscribed neuroendocrine neoplasm.
  • [4] refers to CENET as a rare CNS tumor that typically affects the cauda equina or filum terminale region.
  • [8] mentions CENETs as rare neoplastic processes developing in the cauda equina or filum terminale region.

Note: The information provided is based on the search results and may not be an exhaustive description of CENET.

Additional Characteristics

  • Rare type of central nervous system (CNS) tumor
  • Typically affects the cauda equina or filum terminale region of the spine
  • Low-grade and well-circumscribed nature
  • Located in the cauda equina or filum terminale region of the spine
  • Typically low-grade, indicating a slower growth rate compared to high-grade tumors
  • Well-circumscribed, meaning they have a clear boundary and do not invade surrounding tissues
  • Lower back pain and sciatica due to mass effect
  • Neuropathy or other neurological symptoms depending on the tumor's location and size

Signs and Symptoms

Common symptoms associated with cauda equina paragangliomas include:

  • Lower back pain (94%) [2]
  • Sciatica (less common) [2]
  • Sensorimotor deficits (less common) [2]
  • Sphincter dysfunction (uncommon) [2]
  • Symptoms related to spinal cord compression in larger tumors [3]

Other symptoms may include:

  • Lower lumbar pain [9]
  • Sensory or motor deficits [9]
  • Urinary dysfunction [7]
  • Focal neurologic deficits [7]

It's worth noting that the primary treatment for cauda equina paraganglioma is complete microsurgical resection, which can result in patients' cure [6]. However, symptoms and presentation may vary depending on individual cases.

References: [2] - Symptoms are varied and include lower back pain and sciatica. Patients with larger tumors can present with symptoms related to spinal cord compression. [3] - Symptoms are varied and include lower back pain and sciatica. Patients with larger tumors can present with symptoms related to spinal cord compression. [6] - The primary treatment for cauda equina paraganglioma is complete microsurgical resection which should result in patients' cure. [7] - all spinal tumors. Common presenting symptoms include lower back pain, sciatica, urinary dysfunction, and focal neurologic deficits. [9] - Patients typically present with clinical signs and symptoms referable to a lesion in the cauda equina, manifested by lower lumbar pain, sensory or motor deficits.

Additional Symptoms

  • Sciatica
  • Focal neurologic deficits
  • Lower back pain
  • Sphincter dysfunction
  • Sensorimotor deficits
  • Symptoms related to spinal cord compression in larger tumors
  • Lower lumbar pain
  • Sensory or motor deficits
  • Urinary dysfunction

Diagnostic Tests

Diagnostic Tests for Cauda Equina Neuroendocrine Tumor

Cauda equina neuroendocrine tumors (CE-NETs) are rare and can be challenging to diagnose. The diagnostic tests used to identify CE-NETs typically involve a combination of imaging studies, laboratory tests, and histological examination.

Imaging Studies:

  • Magnetic Resonance Imaging (MRI): MRI is often the first imaging modality used to evaluate patients with suspected CE-NETs. It can provide detailed anatomical information about the tumor's location, size, and relationship to surrounding structures [1].
  • Computed Tomography (CT) scans: CT scans may be used in conjunction with MRI or as a standalone imaging modality to further characterize the tumor [2].

Laboratory Tests:

  • Urinary and plasma fractionated metanephrines and catecholamines: These tests are typically used to rule out other conditions, such as pheochromocytoma, which can present with similar symptoms [3].
  • Blood chemistry tests: These may be performed to evaluate the tumor's impact on surrounding tissues and organs.

Histological Examination:

  • Biopsy: A biopsy is often necessary to confirm the diagnosis of CE-NET. The biopsy sample is examined under a microscope for characteristic histological features, such as zellballen architecture and synaptophysin-positive chief cells [4].

Other Diagnostic Considerations:

  • Differential diagnoses: CE-NETs can be misdiagnosed as other intradural tumors, such as ependymomas or neurinomas. A thorough evaluation of the patient's symptoms, imaging studies, and laboratory results is essential to establish an accurate diagnosis [5].

In summary, the diagnostic tests for cauda equina neuroendocrine tumor typically involve a combination of imaging studies (MRI and CT scans), laboratory tests (urinary and plasma fractionated metanephrines and catecholamines, blood chemistry tests), and histological examination (biopsy).

References:

[1] Shtaya et al. (2022) - Whilst MRI gives accurate anatomical information regarding intradural cauda equina lesions, the differential diagnosis includes schwannoma, ependymoma, ...

[2] Jang et al. (2020) - The diagnosis of a paraganglioma usually starts with measurements of urinary and/or plasma fractionated metanephrines and catecholamines.

[3] Shtaya et al. (2022) - Whilst MRI gives accurate anatomical information regarding intradural cauda equina lesions, the differential diagnosis includes schwannoma, ependymoma, ...

[4] Menu et al. (2020) - Cauda equina neuroendocrine tumor is a low-grade, well-circumscribed, neuroendocrine neoplasm of cauda equina / filum terminale region.

[5] Shtaya et al. (2022) - Whilst MRI gives accurate anatomical information regarding intradural cauda equina lesions, the differential diagnosis includes schwannoma, ependymoma, ...

Additional Diagnostic Tests

  • Biopsy
  • Magnetic Resonance Imaging (MRI)
  • Computed Tomography (CT) scans
  • Blood chemistry tests
  • Urinary and plasma fractionated metanephrines and catecholamines

Treatment

Treatment Options for Cauda Equina Neuroendocrine Tumors

Cauda equina neuroendocrine tumors (CENETs) are rare and typically benign neoplasms that develop in the cauda equina or filum terminale region of the spinal cord. While treatment options may vary depending on individual cases, complete microsurgical resection remains the first choice for treating CENETs.

Surgical Resection

According to various studies [1], [3], [9], surgical resection is considered the gold standard treatment for CENETs. The goal of surgery is to completely remove the tumor while preserving the patient's neurologic functioning. In some cases, preoperative embolization may be performed to reduce intra-operative blood loss and improve surgical outcomes [4].

Radiotherapy

In cases where complete resection is not possible, radiotherapy can be a viable treatment option [8]. This approach aims to minimize the risk of recurrence by targeting any remaining tumor cells.

Other Considerations

While many neuroendocrine tumors have a hereditary basis, this is not typically the case for CENs. However, it's essential to consider genetic factors in individual cases, especially if there are concerns about potential hereditary conditions [7].

Prognosis and Survival Rates

Studies have shown that complete surgical resection can be curative for spinal neuroendocrine tumors, including CENETs [8]. In cases where incomplete resection occurs, radiotherapy may be used to improve outcomes.

References:

[1] by H Popov · 2023 · Cited by 2 — Cauda equina neuroendocrine tumors (CENET) are rare neoplastic processes that develop in the cauda equina or filum terminale region of the spinal cord. [3] by JLM Santos · 2020 · Cited by 7 — The gold standard treatment is gross total resection while preserving the patient's neurologic functioning as complete surgical resection is ... [4] Jan 20, 2024 — Treatment and prognosis. Surgical resection is the treatment of choice, sometimes with preoperative embolization to reduce intra-operative blood ... [7] by H Popov · 2023 · Cited by 2 — Cauda equina neuroendocrine tumors (CENET) are rare neoplastic processes that develop in the cauda equina or filum terminale region of the ... [8] Jun 22, 2023 — Complete surgical resection is generally curative for spinal neuroendocrine tumors. In case of incomplete resection, radiotherapy can be a ... [9] by A Shtayyim · 2015 · Cited by 1 — Surgical treatment of cauda equina neuroendocrine tumor: a case report and review of the literature.

Recommended Medications

  • Radiotherapy
  • Surgical Resection

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

The differential diagnosis for cauda equina neuroendocrine tumors (CENETs) involves considering several other types of tumors that can affect the same region.

  • Ependymomas: These are a type of brain tumor that can also occur in the spinal cord, and they often have similar symptoms to CENETs [1].
  • Paragangliomas: These are rare tumors that can be benign or malignant, and they can occur in various parts of the body, including the cauda equina region [5].
  • Schwannomas: Also known as neurilemmomas, these are benign tumors that arise from the nerve sheath and can occur in the spinal cord, including the cauda equina region [1].
  • Intradural extramedullary tumors: These are a type of tumor that occurs outside the spinal cord but within the dura mater, which is the protective membrane surrounding the spinal cord. Other types of intradural extramedullary tumors can also be considered in the differential diagnosis for CENETs [3].
  • Ependymoma and paragangliomas metastatic to cauda equina: These are two types of tumors that can metastasize (spread) to the cauda equina region, making them a part of the differential diagnosis for CENETs [2][8].

It's worth noting that the differential diagnosis for CENETs is often considered separately from other spinal cord masses, as these tumors have distinct characteristics and behaviors [7].

Additional Differential Diagnoses

  • Schwannomas
  • Ependymomas
  • Paragangliomas
  • Intradural extramedullary tumors
  • Ependymoma and paragangliomas metastatic to cauda equina

Additional Information

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