Chapter 122

1. Definition. Neuroblastoma is a primitive malignant neoplasm that arises from neural crest tissue. It is the most common congenital tumor and is usually located in the adrenal gland but can occur anywhere where there are neural crest cells. Estimates are about 1 per 8000–10,000 children in the United States.

2. Clinical presentation. This tumor typically presents as a firm, fixed, irregular mass extending obliquely from the costal margin, occasionally across the midline and into the lower abdomen.

3. Diagnosis

1. Laboratory studies. A 24-h urine collection should be analyzed for vanillylmandelic acid and other metabolites.

1. Radiologic studies. A plain abdominal radiograph may reveal calcification within the tumor. A computed tomography (CT) scan typically shows extrinsic compression and inferolateral displacement of the kidney. Search for possible metastatic deposits involves bone marrow aspiration and biopsy, bone scan, chest radiograph, and chest CT scan.

4. Management. Treatment is based on stage. Complete surgical resection is still the best hope for cure unless the infant has type 4S disease, which is associated with spontaneous regression without resection. Planned therapy should take into account this well-recognized but poorly understood fact. Advanced tumors require multimodality therapy with surgery, radiation, and chemotherapy, but this is uncommon in neonates.

1. Definition. Embryonic solid renal tissue that is not usually malignant.

2. Clinical presentation. Palpable mass or solid kidney mass seen on prenatal ultrasound.

3. Diagnosis

1. Physical examination. Mass present on examination in the newborn period, usually apparent in first few months of life if not felt in the nursery.

1. Radiologic studies. Ultrasonography if there is a solid mass in the neonate.

4. Management

1. Surgery. Nephrectomy is the first step in treatment and includes lymph node sampling in the event of rare malignant degeneration of the mass.

1. Definition. Wilms tumor is an embryonal renal neoplasm in which blastemic, stromal, and epithelial cell types are present. Renal involvement is usually unilateral but may be bilateral (5% of cases).

2. Clinical presentation. A palpable abdominal mass extending from beneath the costal margin is the usual mode of presentation.

3. Risk factors. Aniridia, hemihypertrophy, certain genitourinary anomalies, and a family history of nephroblastoma.

4. Diagnosis

1. Laboratory studies. There is no tumor marker for Wilms tumor.

1. Radiologic studies. Ultrasonography is generally followed by CT scan, which reveals intrinsic distortion of the caliceal system of the involved kidney. The possibility of tumor thrombus in the renal vein and inferior vena cava should be evaluated by ultrasonography and venography, if necessary.

5. Management

1. Unilateral renal involvement. Nephrectomy is the first step in treatment. Surgical staging determines the administration of radiotherapy and chemotherapy; both are very effective.

1. Bilateral renal involvement. Treatment of bilateral Wilms tumor is highly individualized.

1. Definition. Teratoma is a neoplasm containing elements derived from all three germ cell layers: endoderm, mesoderm, and ectoderm. Teratomas in the neonate are primarily sacrococcygeal in location and believed to represent a type of abortive caudal twinning.

2. Clinical presentation. This tumor is usually grossly evident as a large external mass in the sacrococcygeal area. Occasionally, however, it may ...

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