Ganglioneuroma
| Classification according to ICD-10 | |
|---|---|
| D35 | Benign neoplasm of other and unspecified endocrine glands |
| D35.0 | Adrenal gland |
| D36 | Benign neoplasm in other and unspecified locations |
| D36.1 | Peripheral nerves and autonomic nervous system |
| ICD-10 online (WHO version 2019) | |
| Classification according to ICD-O-3 | |
|---|---|
| 9490/0 | Ganglioneuroma |
| ICD-O-3 first revision online | |
Ganglioneuromas are rare, generally benign tumors of the sympathetic nervous system , which are composed of mature ganglion cells , Schwann cells , connective tissue and nerve fibers . In principle, they can arise wherever sympathetic nerve tissue is found, especially in the adrenal medulla , the sympathetic ganglia on both sides of the spine, the posterior mediastinum , head or neck. Rare locations include the urinary bladder, the intestinal or abdominal wall and the gallbladder. The tumor arises either sporadically or by maturation from the more immature ganglioneuroblastomas or neuroblastomas. The tumor is rarely associated with syndromic diseases such as neurofibromatosis type 1 , Beckwith-Wiedemann syndrome , Hirschsprung's disease or DiGeorge syndrome . Almost 40% of ganglioneuromas produce catecholamines . While ganglioneuromas are tumors of the peripheral nervous system, the name is ganglioneuroma of the central nervous system. an obsolete term for a rare benign brain tumor, the gangliocytoma .
Epidemiology
Although ganglioneuromas can manifest themselves at any age, in the majority of cases children, adolescents and young adults are affected. The mean age of onset is 7 years. Congenital ganglioneuromas have also been described in individual cases. The tumor shows no clear sex preference.
morphology
Ganglioneuromas often appear as well-circumscribed, rounded or lobed tumors of a firm consistency. The cut surface appears gray-white to yellow-gray and can show cystic or fatty areas as well as calcifications. Histologically, spindle-like cell proliferates (Schwann cells) can be found similar to neurofibroma , although ganglioid tumor cells reminiscent of ganglion cells, often grouped and multinucleated, can also be seen. Lymphocytic infiltrates are often visible focal. Necrosis is usually absent. It is considered a sign of benignity that the tigroid clods of the ganglioid cells are still recognizable and therefore no chromatolysis is present.
Clinical symptoms
Regardless of their size, ganglioneuromas are often asymptomatic and are not infrequently discovered by chance during routine examinations. Possible symptoms are abdominal pain, shortness of breath, cough, or a palpable resistance in the abdomen. Hormone-producing ganglioneuromas can lead to high blood pressure, reddening of the skin, or masculinization. Watery diarrhea can result from secretion of vasoactive intestinal polypeptide (VIP) by the tumor.
diagnosis
Imaging methods such as magnetic resonance imaging (MRI), computed tomography (CT) or MIBG scintigraphy are suitable for diagnosis . The latter method makes use of the fact that at least some of the ganglioneuromas take up the substance MIBG and accumulate within the tumor cells. In these cases, marking with a radioactive iodine isotope ensures that it can be detected by means of scintigraphy . The MIBG-enriching tumors are those ganglioneuromas that also produce catecholamines at the same time. A biopsy or complete removal of the tumor followed by a histological examination of the tissue obtained may be necessary to confirm the diagnosis. In the case of hormone production by the tumor, laboratory tests are useful.
Therapy and prognosis
The treatment of choice is usually surgical removal of the tumor. Relapses are rarely observed, so that an excellent prognosis can usually be assumed. Metastasis of ganglioneuromas as well as malignant transformation into ganglioneuroblastoma or neuroblastoma has been described in individual cases. The possibility is discussed that ganglioneuroma-typical differentiated metastases could also arise from tissue maturation from settlements of neuroblastomas and ganglioneuroblastomas, so that ganglioneuromas themselves may not have any metastatic potential.
Differential diagnosis
Other tumors of the adrenal gland or the sympathetic nervous system, such as adrenal adenoma and carcinoma, neuroblastoma and ganglioneuroblastoma, mixed neuroblastic tumors or pheochromocytoma must be excluded from the diagnosis .
literature
- AR Jedynak et al: Imaging in Ganglioneuroma and Ganglioneuroblastoma. 2008; (online at: Medscape )
Individual evidence
- ↑ C. Dyke et al.: Maturation of ganglioneuroblastoma to ganglioneuroma. In: Cancer . 2006; 20 (8), pp. 1343-1349.
- ↑ B. Geoerger et al .: Metabolic activity and clinical features of primary ganglioneuromas. In: Cancer. 2001; 91 (10), pp. 1905-1913.
- ^ MA Ponce-Camacho et al .: A 5-year-old girl with a congenital ganglioneuroma diagnosed by fine needle aspiration biopsy: A case report. In: CytoJournal. 2008; 5, p. 5.
- ^ Mediastinum. on: PathologyOutlines.com , November 4, 2007.
- ↑ Tigroid clods. In: Hans Ulrich Zollinger : Pathological Anatomy . Volume 1: General Pathology. Georg Thieme, Stuttgart 1968, p. 276.
- ^ PG Swift et al.: Watery diarrhea and ganglioneuroma with secretion of vasoactive intestinal peptide. In: Archives of Disease in Childhood . 1975; 50, pp. 896-899.
- ↑ JH Garvin et al .: Ganglioneuroma presenting with differentiated skeletal metastases. Report of a case. In: Cancer. 2006; 54 (2), pp. 357-360.
- ↑ A. Goya et al .: Mediastinal ganglioneuroma with lymph node metastasis in a boy presenting upper airway infection. In: Journal of Chinese Clinical Medicine. 2006; 1 (6), pp. 326-329.
- ↑ AV Kulkani et al .: Malignant transformation of ganglioneuroma into spinal neuroblastoma in an adult. Case report. In: Journal of Neurosurgery . 1998; 88 (2), pp. 324-327.
