Symptoms of neuroblastoma

Symptoms of neuroblastoma are extremely varied and depend on the location and extent of damage to a particular organ.

The most common location of the tumor is the adrenal glands (40% of cases), followed by the retroperitoneal space (25-30%), posterior mediastinum (15%), small pelvis (3%) and neck area (1%). Rare and unspecified localizations are observed in 5-15% of neuroblastoma cases.

Pain is a clinically significant symptom of neuroblastoma in 30-35% of patients, fever is observed in 25-30%. Weight loss - in 20% of patients. Asymptomatic progression is possible for a long time, the frequency of which depends on the stage of the process: at stage I - 48%, at stage II - 29%, at stage III - 16%, at stage IV - 5%, at stage IVS - in 10% of cases.

When the tumor is located in the cervicothoracic region of the sympathetic trunk, Horner's syndrome (ptosis, miosis, enophthalmos, anhidrosis on the affected side) is observed in some cases. When the process is localized in the retrobulbar space, the "glasses" symptom with exophthalmos may appear. Obsessive cough, respiratory disorders, chest wall deformation, dysphagia and frequent regurgitation are characteristic of a tumor located in the posterior mediastinum. The spread of the process from the chest cavity to the retroperitoneal space through the diaphragmatic openings is described as the "hourglass" or "dumbbell" symptom. When localized in the retroperitoneal space, a stony, practically immovable tumor with a tuberous surface can be palpated. Early fixation of the tumor occurs due to rapid ingrowth into the spinal canal through the intervertebral openings. In this case, neurological symptoms in the form of paresis and paralysis may come to the fore.

When the bone marrow is damaged, myelodepression occurs, manifested by anemic and thrombocytopenic syndromes, as well as infectious episodes with the development of neutropenia.

In rare cases, due to the secretion of vasointestinal peptides by the tumor, one of the leading symptoms becomes massive diarrhea.

Skin metastases from neuroblastoma appear as dense, bluish-purple nodules.

Relapses in localized forms of neuroblastoma are relatively rare (18.4%), but distant metastases are found in half of patients with relapse. There is a clear dependence of the frequency of relapses on age: the older the child is at the time of the initial diagnosis, the higher the frequency of relapse. Most often, in case of relapses of neuroblastoma, lesions are localized in the bones, bone marrow, and lymph nodes. Skin, liver, and brain are affected much less often.

Classification of neuroblastoma by stages

The most widely used staging system for neuroblastoma at present is the International Neuroblastoma Staging System (INSS).

• Stage I: localized, macroscopically completely removed tumor without involvement of the lymph nodes (involvement of the immediately adjacent completely removed lymph nodes is acceptable); the same for bilateral tumors.
• Stage II.
  • Stage IIa: macroscopically incompletely removed unilateral tumor without involvement of lymph nodes (involvement of completely removed lymph nodes immediately adjacent to the tumor is acceptable).
  • Stage IIb: Unilateral tumor with lymph node involvement on the same side.
• Stage III: incompletely resected tumor that crosses the midline with or without lymph node involvement, or unilateral tumor with lymph node involvement on the contralateral side, or incompletely resected midline tumor with bilateral growth or bilateral lymph node involvement (the spinal column is taken as the midline).
• Stage IV: tumor dissemination to bone marrow, bones, distant lymph nodes, liver, skin and/or other organs.
  • Stage IVS: localized tumor (stages I, IIa or IIb) with dissemination only to the liver, skin and/or bone marrow in children under one year of age (bone marrow infiltration does not exceed 10% of tumor cells in a smear, mlBG is negative). Unlike other malignant tumors, with microscopically detectable residual tumor in situ, a diagnosis of stage I disease is established due to the possibility of spontaneous regression in most cases. This phenomenon is also possible with macroscopically detectable residual tumor of stages II and III.

[ 1 ], [ 2 ], [ 3 ]