Late childhood neuroblastoma

Description: Neuroblastoma (NB) evolved from primitive neural crest cells, the most common primary site of the sympathetic chain and adrenal medulla. Different ages, tumor sites and different degrees of tissue differentiation make their biological characteristics and clinical manifestations very different, some of which can naturally subside or transform into benign tumors, but other patients are very refractory and have a poor prognosis. In the past 30 years, the prognosis of infants or early NB has improved significantly, but the prognosis of patients with advanced age is still very poor. There are many factors in NB that can affect prognosis, and age and stage are still the most important factors. Etiology: (1) The cause of the disease is an embryonic tumor, mostly located in the cerebral hemisphere. (B) Pathogenesis NB comes from primitive pluripotent sympathetic nerve cells originating from neural crest, in the form of blue small round cells. Different degrees of differentiation, type and transition site of cells after neural crest migration form different normal tissues of sympathetic nervous system, including spinal sympathetic ganglia and adrenal chromaffin cells. The NB histological subtype is consistent with the normal differentiation model of the sympathetic nervous system. Classical pathological classification classifies NB into type 3, namely neuroblastoma, ganglion cell tumor, and ganglion cell tumor. These three types reflect the differentiation and maturation process of NB. A typical NB consists of consistent small cells, about 15% to 50% of cases, with eosinophilic neural fibers around the mother cells. Another fully differentiated, benign NB is a ganglioneuroma, composed of mature ganglion cells, neural fiber networks, and Schwann cells. Ganglioblastoma is between the first two and contains neuroblasts and ganglion cell components. The Shimada classification combined with age divided the pathology into 4 subtypes and clinically divided into 2 groups. The four subtypes include NB (Schwannin less matrix type); GNB mixed type (matrix-rich); GN mature type and (3NB nodular type (including less matrix type and matrix rich type). The first 3 types represent NB The maturity process, while the last type is polyclonal. For NB, cell differentiation is divided into 3 levels, including undifferentiated, poorly differentiated, and differentiated; the mitotic index (MKI) of cells is also divided into low, medium, and high. Grade 3. Shimada classifies the differentiation degree, mitotic index and age of tumor cells, and divides NB into clinical prognosis group (FH) and poor prognosis group (UFH): 1. FH includes the following types (1) NB, MKI is Low to moderate, age <1.5 years old. (2) Differentiated NB, MKI is low, age 1.5 to 5 years old. (3) GNB hybrid type. (4) GN 2. UFH includes (1) NB, MKI advanced (2) NB, MKI is intermediate, age 1.5 to 5 years old. (3) Undifferentiated or poorly differentiated NB, age 1.5 to 5 years old. (4) All NBs >5 years old. (5) GNB nodule type In pathology, in addition to HE staining, immunohistochemical electron microscopy can be further performed to distinguish from other small round cell tumors. Neuron-specific esterase (NSE) is positive at NB, and a typical dense nucleus can be seen under electron microscopy. The neurosecretory granules bound to the membrane have microfilaments and microtubules arranged in parallel in the nerve fiber network. Symptoms: Clinical manifestations and original The location, age, and stage were related. 65% of the children had primary tumors in the abdominal cavity. The primary adrenal glands accounted for 40% of the older children, but only 25% of the infants. Other common sites were the chest and neck. About 10% The primary site of the case is unclear. About 70% of NB is ill before the age of 5, and very few cases occur after the age of 10. 1. The most common symptoms of different parts of the mass are the masses in different parts. (1) Primary in the abdomen: The sympathetic chain of the adrenal gland and the spine are common in the primary, and the symptoms usually appear when the mass is large. There may be abdominal pain, increased abdominal circumference, full back, lumps and masses, and gastrointestinal symptoms. (2) Primary In the chest: there are symptoms associated with mediastinal compression and respiratory symptoms, such as shortness of breath, cough, etc. 2. Patients with advanced manifestations often have limb pain, anemia, fever, weight loss, orbital metastasis. The orbital metastasis forms a characteristic panda eye. Expressed as an eye The ball is prominent, and the periorbital cyanosis is purple. Others may have hypertension and mass related compression symptoms, such as dyskinesia, urinary incontinence, etc. when there is intraspinal infiltration and compression. 3. Metastatic route NB The main metastatic route is lymphatic and blood. About 35% of patients with localized lesions have local lymph node infiltration. Hematogenous metastasis mainly occurs in bone marrow, bone, liver and skin. Brain and lung metastasis may occur in terminal or recurrence, but it is rare. Localized lesions in infant cases. Localized lymph node metastasis and disseminated lesions were 39%, 18%, and 25%, respectively, but 19%, 13%, and 68%, respectively, in older children, that is, when they were seen in older children. Most of them are in advanced stage of the disease. Diagnosis: 1. Diagnostic method Histopathological examination is the most important means of NB diagnosis, sometimes combined with immunohistochemistry and electron microscopy to confirm the diagnosis. Imaging studies revealed a mass consistent with NB characteristics, and NB tumor cells were found in the bone marrow, and a significantly elevated catecholamine metabolite (HVA or VMA) could also be diagnosed. If pathological diagnosis is difficult, chromosome examination revealed a 1p deletion or N-myc amplification to support NB diagnosis. 2. Diagnostic staging should also include diagnostic staging. The US Childhood Tumor Collaborative System (CCSG) is as follows: (1) Stage I: The tumor is confined to the primary organ. (2) Stage II: The tumor is beyond the primary organ, but does not exceed the midline, and the ipsilateral lymph node may be involved. (3) Stage III: The tumor exceeds the midline, and bilateral lymph nodes may be involved. (4) Stage IV: distant transfer. (5) VIs: <1 year old, the primary tumor is stage I and II, but there are metastases limited to liver, skin and bone marrow. Identification and identification of other tumors, mainly based on pathological examination. Complications: Anemia, weight loss, high blood pressure, and movement disorders can occur. Transfer can occur. Treatment: Western medicine treatment (1) treatment due to the large difference in NB prognosis, some patients such as small age, early NB prognosis is significantly better than the older age group, so should be based on patient prognostic factors, such as age, stage, N-myc amplification, Graded treatment is used for 1p deletion. Early patients without N-myc amplification and 1p deletion, only surgery, follow-up after surgery. Larger age, advanced stage, accompanied by N-myc amplification, 1p loss, need to receive strong chemotherapy and surgery until bone marrow transplantation. Surgery, chemotherapy and radiotherapy are still the three main methods of NB treatment, and different intensity treatment plans are adopted according to their clinical prognostic factors. Generally, localized tumors are advocated for surgical resection and chemotherapy. The strategy of first chemotherapy, reoperation, re-chemotherapy or radiotherapy is recommended for patients who cannot be surgically resected. Drugs sensitive to NB include cyclophosphamide, vincristine, etoposide (VP-16), carboplatin, cisplatin, antitumor antibiotics (doxorubicin), and isocyclic amides. Combination chemotherapy for advanced patients, but the prognosis is still unsatisfactory. The US CCSG Collaboration Group reported that the survival rate of advanced NB in the 4 years after receiving autologous bone marrow transplantation was 38%, and the results of various treatment programs did not show any difference. For those with other prognostic factors in stage IV (such as N-myc amplification, age > 2 years, induction therapy was not relieved), the prognosis of the autologous bone marrow transplantation group was better than conventional treatment. There was no difference between the results of allogeneic transplantation and autologous transplantation. The recovery of hematopoietic function during autologous peripheral blood stem cell transplantation is faster than that of bone marrow stem cell transplantation, and the chance of tumor cell contamination is relatively reduced. Read more...