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I. NEUROBLASTOMA

  1. Definition

    1. Primitive malignant neoplasm that arises from neural crest tissue.

    2. Usually located in adrenal gland but can occur anywhere neural crest cells migrate.

    3. Most common extracranial solid malignancy of childhood.

    4. Incidence of 1 per 100,000 children in the United States.

  2. Clinical presentation. Typically presents as firm, fixed, irregular mass extending obliquely from costal margin, occasionally across midline, and into lower abdomen.

  3. Diagnosis

    1. Laboratory studies

      1. Twenty-four–hour urine collection should be analyzed for vanillylmandelic acid and other catecholamine metabolites.

      2. Elevated lactate dehydrogenase is associated with poor prognosis.

    2. Radiologic studies

      1. Plain abdominal radiograph may reveal calcifications within tumor.

      2. Computed tomography scan typically shows extrinsic compression and inferolateral displacement of kidney.

      3. Metastatic evaluation involves bone marrow aspiration and biopsy, bone scan, chest radiograph, and chest computed tomography (CT) scan.

  4. Management

    1. Treatment based on tumor stage. Two staging systems are used:

      1. International Neuroblastoma Risk Group Staging System (INRGSS). Uses image-defined risk factors to determine suitability for surgery.

      2. International Neuroblastoma Staging System (INSS). Takes into account the surgical staging.

    2. Complete surgical resection remains best hope for cure.

    3. Infants with type 4S disease (“special” neuroblastoma) are typically younger than 1 year old and may undergo spontaneous regression. Planned therapy should consider this well-recognized, but poorly understood, fact.

    4. Advanced tumors require multimodality therapy with surgery, radiation, and chemotherapy, but this is uncommon in neonates.

II. CONGENITAL MESOBLASTIC NEPHROMA

  1. Definition

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

    2. Most common renal tumor in neonates and young infants.

    3. Pathologically divided into 3 groups: classic, cellular, and mixed.

  2. Clinical presentation

    1. Can be identified on prenatal ultrasound.

    2. More commonly, palpable mass found on abdominal exam.

    3. Paraneoplastic syndromes (hypertension or hypercalcemia) are possible.

  3. Diagnosis

    1. Physical examination

      1. Mass found on examination in newborn period or becomes apparent in first few months of life.

    2. Radiologic studies (Figure 126–1)

      1. Ultrasonography obtained when a solid mass is identified.

      2. Cross-sectional imaging needed to assess tumor origin, local extension, and impact on surrounding structures (magnetic resonance imaging/CT scan).

  4. Management

    1. Nephrectomy and lymph node sampling to assess for rare malignant degeneration.

    2. Classic form effectively treated with complete resection.

    3. Cellular form has propensity to recur locally or metastasize.

III. WILMS TUMOR (NEPHROBLASTOMA)

  1. Definition

    1. Embryonal renal neoplasm with blastemic, stromal, and epithelial cell types. Histology can be either favorable or unfavorable (anaplastic).

    2. Usually unilateral renal involvement, but may be bilateral (5%).

  2. Clinical presentation. Palpable abdominal mass extending from below costal margin is usual presentation.

  3. Risk factors. Aniridia, hemihypertrophy, certain genitourinary anomalies, and family history of nephroblastoma are well recognized.

  4. Diagnosis

    1. Ultrasonography generally followed by computed tomography scan, which reveals intrinsic distortion of caliceal system of involved kidney.

    2. Possibility of tumor thrombus in renal vein and inferior vena cava should be evaluated.

  5. Management

    1. Children’s Oncology Group staging system is used most often.

    2. Unilateral renal involvement

      1. Radical nephrectomy with lymph node sampling.

      2. Surgical staging determines the need for radiotherapy and chemotherapy.

    3. Bilateral renal involvement

      1. Treatment highly individualized.

      2. Neoadjuvant ...

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