What is Neuroblastoma?

It is a childhood tumor that develops from neuroblast embryological crest cells of the sympathetic nervous system.

How Frequently is Neuroblastoma Seen?

It is the most common cause of tumoral masses in the abdomen during the neonatal period. Approximately 200 new cases are seen each year in our country. It constitutes approximately 6-8% of all childhood tumors. 25% of patients are diagnosed under the age of 1, 50% under the age of 2, and 97% under the age of 10. The average age of occurrence is 2 years. It is seen twice as often in boys.

What are the General Characteristics of Neuroblastoma?

It is the most common abdominal tumor of the newborn. It is a common retroperitoneal tumor seen under the age of 1. 0% of patients are diagnosed under the age of 2, and 80% under the age of 4. It can be diagnosed in the womb and most of them are benign. Most are sporadic. It most commonly metastasizes to the bone marrow. It may be accompanied by some diseases of nervous origin, such as Hirschsprung disease. This type of cancer is very rare in older children and pre-puberty.

Is the Cause of Neuroblastoma Known?

The cause of neuroblastoma is unknown. Also, no chemical substance or exposure has been found to be associated with neuroblastoma.

Where in the Body Does Neuroblastoma Occur?

Neuroblastomas can occur anywhere there is sympathetic nerve tissue. About two-thirds of neuroblastomas originate in the adrenal glands or in nerve cells near the spinal cord that control heart rate, blood pressure and digestion. Other cases of neuroblastoma originate in the ribcage, neck or pelvis (lower abdomen).

Nöroblastomun Uzak Organlara Yayılır mı (Meteztaz Yapar mı)?

When many neuroblastoma patients are diagnosed, the cancer; has spread (metastasized) to the lymph nodes, liver, bones, bone marrow, skin, brain, or other organs.

Are There Types of Neuroblastoma?

Yes, there are. These are; Ganglioneuroma,: does not spread and grow
• Ganglioneuroblastoma: type between ganglioneuroma and neuroblastoma; can grow and spread, location is similar to ganglioneuroma.

Are There Criteria Indicating Whether Neuroblastoma Is Benign or Malignant?

Yes, there are. For example, if the patient is younger than 1.5 years old, it is a good criterion, if he is older than 5 years old, it is a bad criterion. Also, histopathological structure is important, advanced stage is a bad prognosis. Those that can be completely removed surgically are good

What Are the Clinical Symptoms of Neuroblastoma?

Early symptoms of neuroblastoma are generally vague. Clinical findings of the tumor vary according to the location and chemical substances it secretes. Patients usually apply with painful, immobile, abdominal mass or distant spread findings. In addition, depending on the chemicals released, the patient may experience abdominal pain, diarrhea, excessive sweating, high blood pressure, joint pain due to bone spread, and facial flushing. If located in the chest, there may be difficulty swallowing, respiratory distress, and recurrent infection. In tumors that have spread to the orbital bones, a dark ring around the eye and a swollen eye

In the head and neck region; unilateral palpable mass, Horner syndrome (myosis, ptosis, enophthalmos, anhidrosis) findings may be seen.

In the orbit and eyes; orbital-periorbital hemorrhage (raccoon eye), exophthalmos, supraorbital mass, ecchymosis, edema in the eyelids, ptosis, papilledema due to cerebral involvement, optic atrophy, strabismus, paralysis in the outer eye muscles, iris heterochromia due to cervical sympathetic involvement, anisocoria, Horner syndrome, opsomyoclonus are the findings that may be seen.

In the thoracic region; dyspnea, dysphagia, lung infections, lymphatic compression, superior vena cava syndrome, Horner syndrome may be seen in upper thoracic tumors. There are usually no symptoms in lower thoracic tumors.

In the abdominal region; anorexia, vomiting, abdominal pain, abdominal distension and palpable mass, edema in the scrotum and lower extremities due to the compression of the mass on venous and lymphatic drainage, widespread liver involvement findings can be detected especially in newborns with metastatic disease.

In the paraspinal region; localized waist and back pain and tenderness, paraplegia, weakness in the lower extremities, atrophy in the lower extremities, areflexia or hyperreflexia, walking problems, scoliosis, bladder and anal sphincter dysfunction may be seen.

In the pelvic region; constipation, urinary retention, presacral palpable mass may be detected in rectal examination. In bone involvement; pain, limping, restlessness in young children are present. In the lungs; lungs and pleura are rarely affected (0.7%). Therefore, biopsy should be performed in cases where lung involvement is suspected. In lymph node involvement; lymph node enlargement may be detected.

Neuroblastoma may present with different clinical presentations in the newborn: Subcutaneous nodules, widespread liver metastasis, bone marrow involvement findings, widespread hemorrhage into the tumor, hydrops fetalis and/or erythroblastosis fetalis findings are clinical findings of neonatal neuroblastoma.

There are also cases where neuroblastoma is detected with prenatal USG (26). These patients have a good clinical course and complete cure can be achieved with surgical resection. A point to be noted is that in most of these patients, an abdominal mass cannot be palpated and only 50% have increased urine catecholamines.

Are There Findings and Syndromes of Neuroblastoma When Seen in Physical Examination?

Yes, there are.

Panda Eye; Ecchymosis is seen around the eye in hemorrhages due to spread around the eye.

Horner Syndrome: It is seen when it spreads to the Stellate gagnglion located in the neck and upper chest area. (Ptosis (drooping eyelid) on the affected side, Miosis (constriction of the pupil), Enophthalmos (condition in which the eyeball is located further back than normal in the orbit).

Dumbbel (Hourglass) indication:

Dancing eye syndrome: Involuntary and continuous movement of the eyes.

Pepper Syndrome: Enlargement of the liver to the point of respiratory distress due to spread.

Hutchinson Syndrome: Pain due to spread to the bone and bone marrow and therefore the patient's reluctance to walk and jumping walk.

How is Neuroblastoma Diagnosed?

The disease is diagnosed with history and physical examination. It is confirmed and followed up with blood, urine tests and imaging methods. Rarely, it can be detected in the womb or later in screening ultrasounds and x-rays.

What are the Stages of Neuroblastoma?

There are different stagings;

Staging according to the International Neuroblastoma Staging System-INSS;

Stage 1: Macroscopically completely removed localized tumor (with or without microscopic residue)
Stage 2A: Macroscopically incompletely removed localized tumor, no ipsilateral lymph node involvement.
Stage 2B: Macroscopically completely or partially removed localized tumor with ipsilateral lymph node involvement.
Stage 3: Tumor that crosses the midline and cannot be removed
Stage 4: Distant organ and tissue involvement such as skeletal system, bone marrow, soft tissue, distant lymph nodes
Stage 4S: Skin, liver and/or bone marrow involvement with local disease in Stage 1 or 2 without other distant metastases (without bone metastases).

Which Substances Increase in Blood and Urine Tests in Neuroblastoma?

Catecholamines, Neurospecific Enalase, LDH, Ferritin and Calcium may increase in the blood. Vanillylmandylic and Homovolonic acid values, which are the breakdown products of catecholamines, increase in urine.

In bone marrow examination, the presence of Rost formation is definitive diagnosis. In addition, some disorders may be detected in chromosome examinations.

What are the Imaging Methods for Neuroblastoma?

Direct X-Ray Graphs; Calcifications and repulsion in intestinal gases in the abdomen, mass image in the posterior mediastinum in the chest and lytic erosions in the bones in both can be seen.

Ultrasound: Solid mass is seen, kidneys can be monitored separately.

Computed Tomography: Calcified solid mass is seen separately from kidney and liver.

Magnetic Resonance; It clarifies the relationship of the tumor with neighboring organs, especially large vessels.

Scintigraphy: It shows bone and bone marrow and distant organ spreads.

 

How is Neuroblastoma Treated?

Neuroblastoma treatment; if the tumor can be removed, first surgical removal. Then, if necessary, chemotherapy and radiotherapy are applied.

Is Surgery Sufficient in Neuroblastoma Treatment?

This depends on the histological type, stage, age of the patient and some other parameters of the tumor. It may be sufficient.

What Complications Can Neuroblastoma Surgery Have?

Bleeding and abscess development may occur after surgery. For these reasons, re-surgical intervention or skin interventions may be required.
Rarely, other intra-abdominal organs are at risk of injury. If such an injury occurs, it is usually repaired during surgery. Anastomosis leakage may occur after surgery and a re-operation may be required, if anastomosis is not possible during surgery, a colostomy or ileostomy (making the stool in the bag connected to the abdomen) may be opened.
The risk of Incisional Hernia (Incisional hernia) formation in the surgery area is 0.1-1%.
There may be a long-lasting loss of sensation and tingling around the surgical site, sometimes permanent.
Intestinal adhesion (Brid ileus): Adhesions may form between the intestines even years after surgery. Treatment is primarily surgical if there is no medical improvement.
Complications after neuroblastoma surgery;

• Relapse of the disease
• Organ losses due to bleeding or vascular involvement
• Nerve damage and related functional losses depending on the region of the tumor

What are the Risk Groups of Neurobastoma?

• Low risk group; Stage I and 2A at all ages, Stage 1,2,3, or 4S if under 1 year old
• Medium risk group; Stage 2 and 3 at all ages, Stage 4 if under 1 year old.
• High risk group: Over 1 year old and Stage 4 tumors.

What are the Survival Rates of Neurobastoma?

It is around 90% in low risk groups, 50-60% in medium risk groups and 25% in high risk groups.

*** The information provided here and the content of the website are designed to inform visitors, especially families. No information should be considered as advice by visitors and should not lead to any decision or action. The patient should definitely be examined by a pediatric surgeon, consulted with him/her and made a decision based on his/her personal knowledge.