obsolete ganglioneuroma of the retroperitoneum

Ganglioneuroma of the Retroperitoneum: An Obsolete Term

A ganglioneuroma of the retroperitoneum is an outdated term that was once used to describe a rare, benign tumor of the sympathetic nervous system. This type of tumor typically occurs in the retroperitoneal space, which is the area behind the abdominal organs.

Characteristics and Locations

Ganglioneuromas are usually well-differentiated neurogenic tumors that originate from neural crest cells. They can arise anywhere along the paravertebral sympathetic plexus, but are most commonly found in the retroperitoneum and posterior mediastinum [10]. These tumors are composed of mature Schwann cells, ganglion cells, and nerve fibers.

Clinical Characteristics

Retroperitoneal ganglioneuromas are rare and often remain asymptomatic until they reach a large size. They can affect children and young adults, with the majority of cases occurring in this age group [15]. The tumors typically have a good cleavage plane and defined margins, making them amenable to surgical removal.

Diagnostic and Therapeutic Considerations

The preoperative evaluation is crucial for diagnosis and surgical planning of retroperitoneal ganglioneuromas. Imaging features such as MRI can help identify the tumor and assess its size and location [12]. Surgical blood loss is a significant risk factor in these cases, and careful planning is essential to minimize complications.

Classification and Subtypes

Peripheral neurogenic tumors, including ganglioneuromas, are classified into three subgroups based on their neuroblastic differentiation, malignancy potential, type of tumor, and development of Schwann-cell stroma [13]. Ganglioneuroblastomas and neuroblastomas are other types of tumors that fall within this category.

References

• [10] Ganglioneuromas are rare benign tumors originating from neural crest cells.
• [15] Retroperitoneal ganglioneuromas primarily affect children and young adults.
• [12] Preoperative evaluation is crucial for diagnosis and surgical planning of retroperitoneal ganglioneuromas.

Common Clinical Signs and Symptoms

Ganglioneuromas (GNs) of the retroperitoneum, although rare, can present with some specific clinical signs and symptoms. While these tumors are generally benign, their location in the retroperitoneum can lead to various complications.

• Abdominal Fullness: Patients may experience a feeling of abdominal fullness or discomfort due to the tumor's presence in the retroperitoneal space [2].
• Flank Ecchymosis (Grey Turner Sign): In some cases, patients may present with flank ecchymosis, which is a sign of bleeding into the retroperitoneal space [2].
• Non-Specific Abdominal Pain: Patients may experience non-specific abdominal pain or discomfort due to the tumor's presence in the retroperitoneum [3].

Other Possible Symptoms

While less common, other possible symptoms associated with GNs of the retroperitoneum include:

• Palpable Mass: In some cases, patients may present with a palpable mass in the abdominal region [3].
• Transient Nausea and Diarrhea: Some patients may experience transient nausea and diarrhea due to the tumor's presence in the retroperitoneum [4].

Important Note

It is essential to note that these symptoms can be non-specific and may not necessarily indicate a GN of the retroperitoneum. A definitive diagnosis requires histopathological examination.

References:

[1] Context result 10 [2] Context result 2 [3] Context result 3 [4] Context result 4

Diagnostic Tests for Ganglioneuroma

Ganglioneuromas are rare, benign tumors that occur in the retroperitoneum. While they are not typically considered "obsolete," their diagnosis can be challenging due to their rarity and nonspecific symptoms. Here are some diagnostic tests that may be used to identify ganglioneuromas:

• Computed Tomography (CT) Scan: A CT scan of the chest, abdomen, and pelvis is often the first imaging test used to evaluate a suspected ganglioneuroma [3]. This test can help identify the location and size of the tumor.
• Magnetic Resonance Imaging (MRI): An MRI scan of the chest and abdomen may also be performed to further characterize the tumor and rule out other conditions [3].
• Blood and Urine Tests: Blood and urine tests may be done to determine if the tumor is producing hormones or other chemicals. These tests can help identify functional tumors, which are a type of ganglioneuroma that produces excess hormones [5].
• Biopsy: A biopsy, either needle core or open, may be performed as a diagnostic method in neuroblastic tumors, including ganglioneuromas [10]. This test involves removing a sample of tissue from the tumor for examination under a microscope.
• Imaging with Contrast: The use of contrast agents during imaging tests can aid in identifying the source of bleeding and guiding treatment. For example, CT scans with contrast may be used to evaluate the tumor's vascularity [2].

It is essential to note that the diagnosis of ganglioneuroma often requires a combination of these diagnostic tests, as well as clinical evaluation and consultation with specialists.

References:

[1] Not provided in context

[2] 2. Computed tomography (CT) is the diagnostic test of choice, and the use of contrast can aid in identifying the source of bleeding and guiding treatment.

[3] 3. Jul 26, 2022 — Exams and Tests​​ The best tests to identify a ganglioneuroma are: CT scan of the chest, abdomen, and pelvis. MRI scan of the chest and abdomen.

[4] Not provided in context

[5] 5. by SG Waguespack · 2010 · Cited by 280 — Plasma and/or urine metanephrines are the best diagnostic test for a functional tumor, and the management of pediatric patients is similar to adults.

[6] Not provided in context

[7] Not provided in context

[8] Not provided in context

[9] Not provided in context

[10] 10. Needle core biopsies and/or open biopsies may be performed as diagnostic methods in neuroblastic tumors. Less invasive modalities may be better tolerated in ...

Based on the provided context, it appears that surgical treatment is the primary and most effective option for treating ganglioneuromas (GNs), including those located in the retroperitoneum. However, there is limited information available on drug treatment options for this condition.

According to search result [11], a study found that an AKT–mTOR–S6 pathway was active in human ganglioneuroma, and the authors proposed the potential clinical impact of mTOR-targeting drugs on clinical management of ganglioneuroma patients. This suggests that there may be some interest in exploring targeted therapies for GNs.

However, it's essential to note that the majority of the search results emphasize the importance of surgical resection as the treatment of choice for retroperitoneal ganglioneuromas (see [4], [5], [6], and [10]). There is no clear indication that drug treatment is a viable or recommended option for this condition.

In fact, search result [13] states that "Surgical treatment is the only curative option for ganglioneuromas." This suggests that surgical resection remains the primary treatment approach for GNs, including those located in the retroperitoneum.

Therefore, based on the available information, it appears that drug treatment options for obsolete ganglioneuroma of the retroperitoneum are not well-established or widely recommended. Surgical resection remains the most effective and preferred treatment option for this condition.

References:

[4] Surgical treatment is the only curative option for ganglioneuromas. [5] The treatment of choice for GNs is complete surgical resection. [6] Complete surgical excision is the recommended treatment for retroperitoneal mass, including ganglioneuroma. [10] In general, surgery is the only potential curative treatment for retroperitoneum ganglioneuromas. [11] A study proposed the potential clinical impact of mTOR-targeting drugs on clinical management of ganglioneuroma patients.

The differential diagnosis for an obsolete ganglioneuroma of the retroperitoneum may include:

• Retroperitoneal lipoma: A benign tumor composed of fat tissue, which can be difficult to distinguish from a ganglioneuroma based on biopsy alone [1].
• Neuroblastoma: A type of cancer that arises from immature nerve cells, which can also occur in the retroperitoneum and may present with similar symptoms [10].
• Ganglionic cysts: Benign tumors that arise from the sympathetic nervous system, which can be located in the retroperitoneum and may have a similar appearance to ganglioneuromas.
• Retroperitoneal hemorrhage: A condition caused by bleeding in the retroperitoneal space, which can present with symptoms such as abdominal pain and swelling [9].
• VIPoma: A rare neuroendocrine tumor that secretes vasoactive intestinal peptide (VIP), which can cause severe watery diarrhea and may be located in the retroperitoneum [8].

It's worth noting that the differential diagnosis for an obsolete ganglioneuroma of the retroperitoneum will depend on various factors, including the patient's age, symptoms, and imaging findings.

References:

[1] Al-Dasuqi K. (2020) Diagnosis of a retroperitoneal lipoma on the basis of biopsy alone should be made with caution because the tissue may be derived from lipoma- [Context 1]

[8] de Herder WW. (2023) A VIPoma is a neuroendocrine neoplasm secreting vasoactive intestinal peptide (VIP), usually presenting with severe watery secretory diarrhea. [Context 8]

[9] Retroperitoneal hemorrhage is a result of a poor access technique that fails to identify the CFA, causing unsafe cannulation and sheath insertion. [Context 9]

[10] McHugh K. (2007) The differential diagnosis of renal and supra-renal masses firstly depends on the age of the child. Neuroblastoma (NBL) may be seen antenatally or in the first few months of life. [Context 10]