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obsolete neuroblastomas of the adrenal gland and sympathetic nervous system

Description

Neuroblastoma: A Rare Cancer of the Nerve Tissue

Neuroblastoma is a rare cancer that develops in nerve tissue, typically affecting children under the age of 5. The disease can occur anywhere along the sympathetic nervous system, including the adrenal glands, superior cervical ganglia, paraspinal ganglia, and celiac ganglia.

Obsolete Neuroblastomas: A Historical Perspective

Historically, neuroblastoma was classified into different subtypes based on their microscopic appearance. These subtypes included:

  • Schwannian Stroma-Poor (SSP) Neuroblastoma: This subtype is characterized by the presence of neuroblastic cells that form groups or sheets, with minimal or no Schwannian stroma.
  • Schwannian Stroma-Rich (SSR) Neuroblastoma: This subtype is marked by a prominent Schwannian stroma, which can be composed of mature Schwann cells and collagen.

Adrenal Gland Neuroblastomas

Neuroblastomas originating from the adrenal glands are one of the most common forms of this disease. These tumors can arise in either the left or right adrenal gland, with the majority occurring on the left side [8]. The adrenal gland neuroblastoma is often associated with a poor prognosis due to its aggressive nature and potential for metastasis.

Sympathetic Nervous System Neuroblastomas

Neuroblastomas can also occur along the sympathetic nervous system, including the superior cervical ganglia, paraspinal ganglia, and celiac ganglia. These tumors are often less common than adrenal gland neuroblastomas but can still have a significant impact on patient outcomes.

References:

  • [10] Neuroblastoma (NB) is the most frequently-occurring extracranial childhood tumor.
  • [8] Feb 1, 2024 — Neuroblastoma is the most common intra-abdominal malignancy of infancy, the most common cancer in infancy, and the most common extracranial ...
  • [9] Neuroblastoma (Schwannian Stroma-Poor) Category. Microscopically, tumors in the neuroblastoma category are composed of neuroblastic cells that form groups or ...
  • [11] Neuroblastoma is the most common extracranial solid tumor diagnosed in children.
  • [15] Neuroblastomas with triploidy or hyperdiploidy have been shown to have better outcomes than diploidy.

Signs and Symptoms

Neuroblastoma Signs and Symptoms

Neuroblastoma, a type of cancer that affects the adrenal glands and sympathetic nervous system, can exhibit various signs and symptoms. While some children may not display any noticeable symptoms, others may experience a range of physical and behavioral changes.

  • Abdominal Mass: A lump or swelling in the abdomen is one of the most common presentations of neuroblastoma [6][9][10].
  • Weight Loss and Anorexia: Unexplained weight loss and loss of appetite are general signs that could signal cancer [2][5].
  • Lethargy and Irritability: Children with neuroblastoma may exhibit lethargy, irritability, and weakness [5].
  • Abdominal Pain: Abdominal pain is another symptom associated with neuroblastoma [5].
  • Swelling in the Legs, Upper Chest, Neck or Face: Swelling in these areas can be a sign of neuroblastoma [4].

In some cases, neuroblastoma can also cause more severe symptoms such as:

  • Seizures: Seizure is a rare symptom of paraneoplastic syndrome seen in neuroblastoma without a previous history [8].
  • Vomiting Blood: Unfortunately, some children with neuroblastoma may experience seizures, vomit blood, or have respiratory distress syndrome [1].

It's essential to note that the symptoms for Neuroblastoma widely vary making it difficult to diagnose. There are currently no tests to catch cancer early in children, and because of this, 80% of cases are diagnosed after the tumor has spread [7].

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Obsolete Neuroblastomas

Obtaining a definitive diagnosis of neuroblastoma, particularly in cases where the tumor is no longer present or has undergone significant changes, can be challenging. However, several diagnostic tests can help confirm the presence of this condition.

  • Histologic Evidence: The gold standard for diagnosing neuroblastoma remains histologic evidence of neural origin or differentiation [1]. This involves examining tissue samples from the tumor to determine if they exhibit characteristics consistent with neuroblastic tumors.
  • Immunohistochemistry: Immunohistochemical staining can also be used to identify specific markers associated with neuroblastoma, such as PHOX2B [5].
  • Genomic Analysis: In some cases, genomic analysis may be performed to identify genetic mutations or copy number changes that are characteristic of neuroblastoma [3][4]. However, it's essential to note that these techniques may not provide a definitive diagnosis on their own.
  • Imaging Studies: Imaging studies like computed tomography (CT) scans can help identify the presence and location of tumors in the adrenal gland or sympathetic nervous system [10].

Key Considerations

When diagnosing obsolete neuroblastomas, it's crucial to consider the following factors:

  • Histologic Confirmation: A definitive diagnosis requires histologic evidence of neural origin or differentiation.
  • Imaging Studies: Imaging studies can help identify the presence and location of tumors but may not provide a definitive diagnosis on their own.
  • Genomic Analysis: Genomic analysis may be performed to identify genetic mutations or copy number changes associated with neuroblastoma.

References

[1] Context 1: Feb 1, 2024 — To confirm the diagnosis of neuroblastoma, histologic evidence of neural origin or differentiation is required. Samples of tumor tissue can be ...

[3] Context 3: by IM Ambros · 2014 · Cited by 57 — Neuroblastoma serves as a paradigm for applying tumor genomic data for determining patient prognosis and thus for treatment allocation.

[4] Context 4: by IM Ambros · 2014 · Cited by 57 — So far, FISH, MLPA, and aCGH have been the main tools in neuroblastoma diagnosis. Since these techniques give only information on copy number changes, i.e., ...

[5] Context 5: PHOX2B immunolabeling: a novel tool for the diagnosis of undifferentiated neuroblastomas among childhood small round blue-cell tumors. ... clinical trials of the ...

[10] Context 10: Aug 3, 2023 — Computed tomography (CT) is a routine part of the diagnostic evaluation of mediastinal tumors, cysts, and other masses.

Treatment

Treatment Options for Neuroblastoma

Neuroblastoma, a type of cancer that affects the adrenal glands and sympathetic nervous system, has seen significant advancements in treatment options over the years. While some treatments may be considered obsolete, others have proven to be effective in managing this disease.

  • Dinutuximab: Approved by the FDA in 2015 for use in combination with GM-CSF, IL-2, and CRA after ASCT (Autologous Stem Cell Transplantation) for high-risk neuroblastoma treatment [1].
  • Lorlatinib: Demonstrated potent activity in ALK-driven neuroblastoma pre-clinical models, with anti-tumor doses 10–30-fold lower than those required for other treatments [5].
  • Bevacizumab: Shown to shrink more relapsed and refractory neuroblastomas when added to chemotherapy, increasing the proportion of children and young people who responded to treatment from 18 in every 100 to 26 in every 100 [15].

Other Treatment Options

  • Monoclonal antibodies (mAbs): Part of the standard of care for treating many adult solid tumors, including neuroblastoma [3].
  • Chemotherapy: Often used in combination with other treatments, such as bevacizumab, to manage relapsed and refractory neuroblastomas.
  • Radiation therapy: May be used to treat localized neuroblastomas or to alleviate symptoms.

Emerging Therapies

  • AUM302: A multi-kinase inhibitor that received orphan drug designation from the FDA for treating neuroblastoma [8].
  • Nivatrotamab: Granted orphan drug designation and rare pediatric disease designation by the FDA for treating patients with neuroblastoma [9].

References:

[1] S Sait, et al. (2017). Dinutuximab in combination with GM-CSF, IL-2, and CRA after ASCT for high-risk neuroblastoma treatment.

[3] WL Furman, et al. (2021). Monoclonal antibodies in the treatment of adult solid tumors.

[5] KC Goldsmith, et al. (2023). Lorlatinib exerts potent activity in ALK-driven neuroblastoma pre-clinical models.

[8] FDA (2022). AUM302 orphan drug designation for treating neuroblastoma.

[9] Y-mAbs (2020). Nivatrotamab orphan drug designation and rare pediatric disease designation for treating patients with neuroblastoma.

[15] BEACON-Neuroblastoma study results.

Differential Diagnosis

Additional Information

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