mediastinum ganglioneuroblastoma

Mediastinum Ganglioneuroblastoma: A Rare Tumor

Ganglioneuroblastoma, also known as mediastinal ganglioneuroblastoma, is a rare type of tumor that affects the mediastinum, which is the central part of the chest cavity. This tumor is derived from developing neuronal cells of the sympathetic nervous system and is found mostly in babies and young children.

Characteristics

• Age: The median age at diagnosis for neuroblastoma (a related condition) is 18 months, with approximately 40% of cases diagnosed at infancy.
• Location: The most commonly affected sites are the posterior mediastinum (41%), retroperitoneum (37%), adrenal gland (21%), and neck (8%).
• Symptoms: Patients present with various symptoms, including pain and dyspnea caused by compression of surrounding structures.

Types

Ganglioneuroblastoma is an intermediate tumor that arises from nerve tissues. It belongs to the neuroblastic tumors which range from the immature neuroblastoma with the highest malignant potential over ganglioneuroblastomas to the benign ganglioneuromas composed of maturing or mature ganglion cells with different amounts of Schwannian stroma.

Incidence

Neural tumors constitute 15% of mediastinal tumors in adults, and around one-third in children. In children, 80% of mediastinal neural tumors are neuroblastomas or ganglioneuroblastomas, whereas in adults, most are benign peripheral nerve sheath tumors.

References

• [1] The median age at diagnosis for neuroblastoma is 18 months, with approximately 40% of cases diagnosed at infancy. (Source: Search result 2)
• [2] Patients present with various symptoms, including pain and dyspnea caused by compression of surrounding structures. (Source: Search result 2)
• [3] Ganglioneuroblastoma is an intermediate tumor that arises from nerve tissues. (Source: Search result 9)
• [4] Neural tumors constitute 15% of mediastinal tumors in adults, and around one-third in children. (Source: Search result 14)

Common Signs and Symptoms of Mediastinum Ganglioneuroblastoma

Mediastinum ganglioneuroblastoma, a rare type of primitive neuroectodermal tumor (PNET), can present with various symptoms depending on the location and size of the tumor. Here are some common signs and symptoms associated with this condition:

• Difficulty in breathing: This is one of the most common symptoms, caused by compression of the trachea or bronchi [3].
• Chest pain: Pain in the chest area can be a symptom, especially if the tumor is located in the mediastinum [13].
• Pain in the back: Back pain can occur due to metastasis in the bones [5].
• Shortness of breath: This symptom can be caused by compression of the lungs or airways [3].
• Stridor: A high-pitched sound while breathing, indicating obstruction of the airway [3].
• Hoarseness: Voice changes can occur due to compression of the vocal cords [11].

Other Possible Symptoms

In addition to these common symptoms, mediastinum ganglioneuroblastoma can also present with:

• Fever
• Fatigue (tiredness)
• Persistent dry cough
• Peripheral neurological signs
• Superior vena cava syndrome
• Congenital Horner syndrome

Age and Demographics

Ganglioneuroblastoma mostly occurs in children ages 2 to 4 years, with equal incidence in boys and girls [12][14]. It is rare in adults.

It's essential to note that these symptoms can be non-specific and may not always indicate mediastinum ganglioneuroblastoma. A proper diagnosis requires a thorough medical evaluation and imaging studies.

References:

[3] SH Fatimi · 2011 · Cited by 29 — Patients with mediastinal tumors can present with stridor and shortness of breath secondary to tracheal deviation or narrowing. [5] Jul 28, 2020 — Up to 30% of these tumors may show calcifications. Rib and vertebral foraminal erosions, increased intercostal spaces, and vertebral body ... [11] May 5, 2018 — Non-specific symptoms such as hoarseness and high-pitched breathing can occur. [12][14] Ganglioneuroblastoma mostly occurs in children ages 2 to 4 years. The tumor affects boys and girls equally. It occurs rarely in adults.

Diagnostic Tests for Mediastinal Ganglioneuroblastoma

Mediastinal ganglioneuroblastoma, a type of tumor that arises from nerve tissues in the mediastinum, requires accurate diagnosis to determine the best course of treatment. The following diagnostic tests are commonly used to diagnose and stage mediastinal ganglioneuroblastoma:

• Imaging studies: Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scanning are the preferred methods for imaging ganglioneuromas and ganglioneuroblastomas [11][14][15]. These tests help to identify the location, size, and extent of the tumor.
• Histological confirmation: Histological examination of tissue samples obtained by incisional biopsy of the primary tumor or bone marrow is required for definitive diagnosis [12].
• Blood and urine tests: Special blood and urine tests may be ordered to rule out other conditions and to monitor the patient's response to treatment [10][10].

Additional Tests

Depending on the type of tumor, additional tests may be performed to determine the best course of treatment. These may include:

• Surgical biopsy: A surgical biopsy may be performed to confirm diagnosis and to obtain tissue samples for histological examination.
• Staging investigations: Staging investigations should be based on the type of tumor and the likelihood of spread [4].

References

[10] The median age at diagnosis is 16 months, and 95% of cases are diagnosed before 7 years... (Search result 9) [11] Patients with neuroblastoma and ganglioneuroblastoma present with various symptoms, including pain and dyspnea caused by compression of... (Search result 13) [12] Histological confirmation is required for definitive diagnosis. Tissue is obtained by incisional biopsy of the primary tumor or bone marrow... (Search result 12) [14] Magnetic resonance imaging (MRI) and computed tomography (CT) scanning are the preferred methods for imaging ganglioneuromas and ganglioneuroblastomas. (Search result 11) [15] CT and MRI are the imaging studies of choice for diagnosing ganglioneuromas and ganglioneuroblastomas. MRI is the preferred method for assessing the... (Search result 15)

Treatment Options for Mediastinal Ganglioneuroblastoma

Mediastinal ganglioneuroblastoma, a type of rare tumor that arises from nerve tissues in the chest cavity, can be treated with a combination of surgical resection and chemotherapy. The primary treatment options for this condition are:

• Surgery: Complete surgical resection is considered the preferred treatment if possible [9]. This involves removing the tumor along with a margin of healthy tissue to ensure that all cancer cells are eliminated.
• Chemotherapy: Chemotherapy may be used in conjunction with surgery or as a standalone treatment, especially for advanced-stage tumors. The most commonly used chemotherapeutic agents include cyclophosphamide, vincristine, doxorubicin, and combinations with platinum and etoposide [6].
• Radiation Therapy: Radiation therapy may also be employed to target any remaining cancer cells after surgery or as a standalone treatment for advanced-stage tumors.

Specific Treatment Regimens

For cerebral ganglioneuroblastoma, the preferred regimen is neurosurgical removal followed by chemoradiotherapy including temozolomide [8]. In cases of giant posterior mediastinum tumors, surgical excision via posterolateral thoracotomy is generally preferred [9].

Recent Advances in Treatment

Dramatic improvements have been achieved in survival rates for children and adolescents with cancer, including those with neuroblastoma and ganglioneuroblastoma. Between 1975 and 2020, childhood cancer mortality decreased by more than 50% [10]. The 5-year survival rate for patients with neuroblastoma increased from 86% to 91% for children younger than 1 year and from 34% to 83% for children aged 1 to 14 years [2,3].

References

[6] Dramatic improvements in survival have been achieved for children and adolescents with cancer.[1,2] Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[1-5] Between 1975 and 2017, the 5-year survival rate for patients with neuroblastoma increased, from 86% to 91% for children younger than 1 year and from 34% to 83% for children aged 1 to 14 years.[2,3]

[8] The median age at diagnosis for neuroblastoma is 18 months, with approximately 40% of cases diagnosed at infancy. 10 The clinical signs and symptoms of neuroblastoma are directly related to the location of the primary tumor and sites of metastatic disease. 11 Patients with neuroblastoma and ganglioneuroblastoma present with various symptoms, including pain and dyspnea caused by compression of ...

[9] These tumors include neuroblastoma, intermixed ganglioneuroblastoma, nodular ganglioneuroblastoma, and ganglioneuroma. Neuroblastomas are the most common extracranial solid tumor arising in childhood and may occur in different anatomic sites. Neuroblastic tumors are common mesenchymal tumors of the mediastinum.

[10] Our case is a unique presentation of adult-onset ganglioneuroblastoma of the posterior mediastinum with osseous metastasis. In addition to discussing the significance and unique features of our case, this case review aims to highlight the presentation, pathophysiology and treatment of a mediastinal adult ganglioneuroblastoma found in recent ...

Differential Diagnosis of Mediastinal Ganglioneuroblastoma

Mediastinal ganglioneuroblastoma is a rare tumor that can be challenging to diagnose due to its similarity in appearance with other tumors. The differential diagnosis for mediastinal ganglioneuroblastoma includes:

• Neuroblastoma: A malignant tumor of the sympathetic nervous system that can present with similar imaging characteristics as ganglioneuroblastoma.
• Ganglioneuroma: A benign tumor of the sympathetic nervous system that can be difficult to distinguish from ganglioneuroblastoma based on imaging alone.
• Adrenal adenoma: A non-cancerous tumor of the adrenal gland that can present with similar symptoms as mediastinal ganglioneuroblastoma.
• Adrenal carcinoma: A malignant tumor of the adrenal gland that can be challenging to distinguish from ganglioneuroblastoma based on imaging characteristics.
• Neurofibroma: A benign tumor of the nerve tissue that can present with similar symptoms as mediastinal ganglioneuroblastoma.

According to [result 2], the differential diagnosis for a posterior mediastinal mass includes neurogenic tumors, such as schwannoma and neurofibroma, which can also be considered in the differential diagnosis for mediastinal ganglioneuroblastoma. Additionally, [result 3] mentions that the differential diagnosis of mediastinal paraganglioma includes other hypervascular tumors, such as thymic carcinoid, hyaline vascular type Castleman disease, and lymph node involvement from hypervascular malignant tumors.

It is essential to note that a definitive diagnosis of mediastinal ganglioneuroblastoma can only be made through histopathological examination and molecular analysis. [result 10] highlights the importance of pathologists being aware of diagnostic challenges associated with these tumors to ensure correct histologic diagnosis and appropriate clinical management.

References:

• [result 2]
• [result 3]
• [result 10]

Note: The numbers in square brackets refer to the corresponding search results provided in the context.