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Neuroblastoma Staging, from Campbell's

International Neuroblastoma Risk Group classification, from Campbell's

Wilms tumor COG staging, from Campbell's

Location of rhabdomyosarcoma lymph node metastases, from Campbell's

Preop rhabdomyosarcoma staging, from Campbell's

Postop rhabdomyosarcoma clinical grouping, from Campbell's

Rhabdomyosarcoma risk stratification (includes preop staging and postop clinical group), from Campbell's

Testis GCT staging, from Campbell's

Algorithm for management of prepubertal testis tumors, from Campbell's

Renal and adrenal tumors

Neuroblastoma

• Most common extracranial solid tumor of childhood, 50-70% present with metastatic disease
• Low stage tumors may spontaneously regress
• Stroma poor tumors have poor prognosis (< 10%)
• Presentation: abdominal pain or mass, bone/joint pain, ecchymosis, cough, neurologic symptoms, retention, constipation
• Catecholamine secretion: paroxysmal hypertension, palpitations, flushing headache
• VIP secretion: diarrhea and hypokalemia
• Acute myoclonic encephalopathy: myoclonus, opsoclonus, ataxia, can lead to prolonged neurologic impairment in 70-80%
• Labs: elevated urinary catecholamine metabolites (VMA/HVA) found in 90-95%
• Marrow aspirate x2 and biopsy x2 are recommended for staging
• Management: immediate surgery for localized tumors, otherwise reasonable to treat with neoadjuvant chemotherapy

Nephroblastoma (Wilms tumor)

• Most common primary malignant renal tumor of childhood
• If high risk (WAGR, Denys-Drash, Fanconi anemia, Perlman, Mosaic variegated aneuploidy), recommend screening renal US
• Nephrogenic rests: seen in 1/3 Wilms, increase need for surveillance, can form thick kidney rind
• 85% present with mass, pain, or hematuria
• Labs: obtain CBC, CMP, calcium, UA, coags (5-10% have von Willebrand disease), urine catecholamines (if concern for neuroblastoma)
• Imaging: usually discovered with US, obtain CT/MR A/P with PO+IV contrast and CT with contrast, bone scan only if bone pain or elevated AlkPhos
• Management: radical nephrectomy with regional LN dissection, unless surgically inoperable (per surgeon)
• Surgical contraindications: tumor thrombus above hepatic veins, invasion into local structures requiring extensive resection, pulmonary compromise, severe risk morbidity/mortality
• Bilateral tumors: up to 5%, give chemotherapy then bilateral partial nephrectomy
• Chemotherapy: vincristine, dactinomycin, +/- doxorubicin, cyclophosphamide, etoposide
• Recurrence: most occur within 2yr treatment, lung most common site

Other renal tumors

• Clear cell sarcoma: 3% of renal tumors, 50% present with bone metastases (evaluate with bone scan), 30% at risk for relapse, may require XRT, managed with doxorubicin and surgery
• Rhabdoid tumor: 2% of renal tumors, high risk for brain mets (screen with brain imaging), aggressive, resistant to chemo/XRT, high mortality rate
• Congenital mesoblastic nephroma: most common renal tumor in infants and diagnosed via antenatal ultrasound, usually managed with surgery only
• Solitary multilocular cyst, cystic partially differentiated nephroblastoma: benign tumor with multiple various sized cysts compressing normal parenchyma, considered on spectrum with nephroblastoma, managed with surgery
• Renal cell carcinoma: higher incidence of pRCC, most common renal tumor of second decade, manage with surgery
• Angiomyolipoma (AML): rare in children, treat if > 3-4cm, everolimus then embolization/surgery

Bladder and prostate tumors

Rhabdomyosarcoma

• Most common soft tissue sarcoma in children, third most common pediatric solid tumor (5-15% all tumors), 5-10% occur in bladder/prostate
• Symptoms: retention, urgency, frequency, incontinence, hematuria, occasionally prolapse via urethra
• Stage with CT/MR A/P, CT chest - bone scan and marrow biopsy/aspirate may be indicated
• Management: diagnostic biopsy (usually unresectable at presentation), chemo/XRT
• Residual mass does not necessarily represent residual tumor and may not require removal

Other bladder tumors

• Urothelial cell carcinoma: uncommon, present with hematuria/dysuria, manage with resection (no intravesical therapy), surveil with ultrasound (q3mo x1yr, q6mo x1yr, then annually)
• Nephrogenic adenoma: more common if recurrent infections, prior radiation or bladder surgery, appear similar to bladder cancer, require resection for diagnosis and treatment, are benign but can recur (surveillance recommended)
• Cancer in bladder augments: higher incidence than general population, but likely secondary to long-term smoking or immunosuppression

Female genital tumors

Rhabdomyosarcoma

• Incidence: vaginal -> cervical/uterine -> vulvar/clitoral
• Presents with vaginal bleeding, discharge, or protruding mass
• Management: biopsy, chemotherapy, possible surgery as indicated

Ovarian tumors

• 98% newborns have small ovarian cysts, usually related to maternal hormones
• Adolescent ovarian cysts usually secondary to dysfunctional ovulation, manage with hormonal therapy, surgery only if persistent symptoms or concern for malignancy
• Ovarian mass: 50/50 benign/malignant
• Ovarian germ cell tumors: present with pain, distention, precocious puberty, manage with surgery and chemotherapy

Testicular and paratesticular tumors

Prepubertal testis tumors

• Teratoma and yolk sac tumor most common causes of childhood testis tumors
• Present with painless mass or on US for scrotal pain (rule out torsion), hydrocele present 15-20%
• US cannot differentiate benign from malignant tumors
• AFP may be falsely elevated if < 1yo
• Teratoma: US may show heterogenous complex lesion (cystic + solid), manage with partial orchiectomy, tend to be benign if prepubertal
• Epidermoid cysts: US shows layered mixed appearance, manage with partial orchiectomy
• Yolk sac: elevated AFP (marker for recurrence), 20% spread to lungs (skipping RP nodes), 20% Stage I recurrence with orchiectomy alone
• Leydig: causes 10% total precocious puberty cases, virilizing feature persist despite partial orchiectomy
• Sertoli: present with painless mass, may cause virilization/feminizaiton, manage with orchiectomy, obtain staging if > 5cm, vascular invasion, necrosis, atypia, increased mitotic activity - may require further treatment
• Partial orchiectomy: should be offered if presumed teratoma/benign tumor and confirmed with frozen section, although radical orchiectomy may be required intraop or if recurrence

Paratesticular rhabdomyosarcoma

• 7-10% all GU RMS, 40% all paratesticular lesions
• Present with painless scrotal mass, heterogenous solid mass on US
• Staging: STMs, LFTs, CT C/A/P (20% have LN metastases)
• Management: inguinal orchiectomy, RPLND warranted if < 10yo and positive nodes, all > 10yo require RPLND