Symptoms of Neuroblastoma

Symptoms of neuroblastoma are characterized by extreme diversity and depend on the localization and extent of damage to one or another organ.

Most often, the tumor is localized in the adrenal glands (40% of cases), followed by retroperitoneal space (25-30%), posterior mediastinum (15%), small pelvis (3%) and neck region (1%). Rare and unidentified localizations are observed in 5-15% of cases of neuroblastoma.

Pain is a clinically significant symptom of neuroblastoma in 30-35% of patients, fever is observed in 25-30%. Loss of body weight - in 20% of patients. For a long time, asymptomatic flow is possible, the frequency of which depends on the stage of the process: at stage I - 48%, in stage II - 29%, in stage III - 16%, in stage IV - 5%, in stage IVS - 10% of cases.

With the location of the tumor in the cervico-thoracic region of the sympathetic trunk, Horner's syndrome (ptosis, miosis, enophthalmus, anhidrosis on the side of the lesion) was noted in a number of cases. When the process is localized in the retrobulbar space, the symptom of "glasses" with exophthalmos may appear. Obsessive cough, respiratory distress, chest wall deformity, dysphagia and frequent regurgitation are characteristic of a tumor located in the posterior mediastinum. Distribution of the process from the chest cavity to the retroperitoneal space through the diaphragmatic apertures is described as a symptom of "hourglass" or "dumbbell". When localized in the retroperitoneal space palpation, it is possible to determine the rocky density of an almost unbiased tumor with a bumpy surface. Early tumor fixation is due to rapid ingrowth into the spinal canal through the intervertebral foramen. In this case, the neurologic symptoms in the form of paresis and paralysis can come to the fore.

When the bone marrow is affected, mielodepression occurs, manifested by anemic and thrombocytopenic syndromes, as well as infectious episodes in the development of neutropenia.

In rare cases, due to tumor secretion of vaso-intestinal peptides, massive diarrhea becomes one of the leading symptoms.

Metastases in the skin with neuroblastoma have the form of dense knots of cyanotic-purple color.

Relapses with localized forms of neuroblastoma are relatively rare (18.4%), but in half of patients with relapse they have distant metastases. A clear dependence of the frequency of relapses on age is typical: the older the child at the time of establishing a primary diagnosis, the higher the frequency of its recurrence. Most often with recurrence of neuroblastoma, lesions are localized in the bones, bone marrow, lymph nodes. Significantly less affected skin, liver and brain.

Classification of neuroblastoma by stages

The most common current system is the staging of neuroblastoma INSS (International Neuroblastoma Staging System).

• Stage I: a localized macroscopically completely removed tumor without lymph node involvement (it is permissible to involve completely removed lymph nodes immediately adjacent to the tumor); the same for a bilateral tumor.
• Stage II.
  • Stage IIa: macroscopically incompletely removed unilateral tumor without lymph node involvement (it is permissible to involve completely removed lymph nodes immediately adjacent to the tumor).
  • Stage IIb: a unilateral tumor with lymph node involvement on the same side.
• Stage III: an incompletely removed tumor, a transitional median line with or without lymph node involvement, or a unilateral tumor with lymph node involvement on the opposite side, or an incompletely removed median tumor with bilateral growth or bilateral lymph node involvement (the vertebral column takes the median line pillar).
• Stage IV: Dissemination of the tumor into the bone marrow, bones, removed lymph nodes, liver, skin and / or other organs.
  • Stage IVS: localized tumor (stages I, IIa or IIb) with dissemination only in the liver, skin and / or bone marrow in children under the age of one year (bone marrow infiltration does not exceed 10% of tumor cells in the smear, mlBG negative). Unlike other malignant tumors with a microscopically determined residual tumor in situ, a diagnosis of stage I disease is established in connection with the possible in most cases spontaneous regression. This phenomenon is also possible with a macroscopically determined residual tumor of stages II and III.

[1], [2], [3]