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Childhood Solid Tumors

Childhood Solid Tumors. Alexandra Walsh, MD Resident Lecture 1.26.12. Pediatric versus Adult Cancers. NCI, SEER data. Percentage Distribution of Childhood Cancers. Cancer Research UK, 2004. Cancer in Children. Childhood Cancers. 61% of childhood cancers are “solid tumors”.

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Childhood Solid Tumors

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  1. Childhood Solid Tumors Alexandra Walsh, MD Resident Lecture 1.26.12

  2. Pediatric versus Adult Cancers NCI, SEER data

  3. Percentage Distribution of Childhood Cancers Cancer Research UK, 2004

  4. Cancer in Children

  5. Childhood Cancers 61% of childhood cancers are “solid tumors” Abdominal tumors Soft tissue tumors Bone tumors Eye tumors

  6. Case #1 HPI/PMH 3 yo female presents to PMD secondary to a “lump in the belly” that her mom felt while bathing her the night before Child has been well No fever, URI symptoms, N/V/D/C, abdominal pain, urinary complaints or swelling anywhere else PMH unremarkable

  7. Case #1 PE • Afebrile, stable vital signs • Alert, active, playful 3 yo • Shoddy cervical lymphadenopathy • Heart regular rate and rhythym • Lungs clear • 6 x 5 cm firm, smooth, non-tender abdominal mass in the right quadrant, not crossing midline, margins well defined

  8. Case #1 Labs • WBC 6000 • 40% segs, 60% lymphs (ANC 2400) • Hgb 12 • Hct 36 • Plts 180,000 • Normal CMP including BUN, creatinine • Normal LDH, uric acid • HVA, VMA • Normal • AFP, B-HCG • Normal • Urinalysis • 2+ blood

  9. CT Scan of Abdomen

  10. Wilms Tumor • 5-6% of childhood cancers • 5th most common cancer overall and 2nd most common abdominal malignancy • Occurs most commonly in children 2-5 yo • Arises from primitive metanephric blastema • Typically occurs sporadically but can occur in certain syndromes/ congential defects • Beckwith-Wiedemann Syndrome • Denys-Drash syndrome • WAGR syndrome

  11. Beckwith-Wiedemann Syndrome Congenital disorder of growth regulation 1/14,000 children Macrosomia, macroglossia, neonatal hypoglycemia, abdominal wall defects, ear anomalies, visceromegaly May be associated with hemi-hypertrophy 4-21% of patients develop cancer Wilms tumor (43%) and Hepatoblastoma (12%) Q4 month abdominal u/s until age 8

  12. Wilms Tumor Associated Syndromes • Familial tumors- associated with 2 loci • WT1: primary gene implicated in development of Wilms • Located at 11p13 • Deletions noted in WAGR (Wilms, Aniridia, GU anomalies, Retardation) • Missense WT1 mutations in Denys Drash (gonadal dysgenesis, nephropathy)

  13. Wilms Tumor • Presentation: Often asymptomatic increase in size of abdomen • Labs: Usually normal • May have anemia secondary to hemorrhage into the tumor • Microscopic hematuria

  14. Wilms Tumor • Imaging: CT scan or ultrasound • Must evaluate contralateral kidney • Can metastasize to liver and lungs • Staging and histology of tumor important for prognosis • Overall survival rate of 90% (Stage IV, unfavorable histology has 17% overall survival rate)

  15. Wilms’ Tumor Treatment/Outcome

  16. Case #2 HPI/PMH 3 yo male presents to PMD secondary to low-grade fever, fussiness, and decreased PO intake over the past 2-3 weeks Several pound weight loss Irritable and over the past few days wants to be held all the time and does not want to walk No URI symptoms, no N/V/D/C, no sick contacts No significant PMH

  17. Case #2 PE Temp 100.8, P 130, R 25 Irritable, difficult to console, pale, ill-appearing 3 yo Shoddy cervical lymphadenopathy Tachycardia with II/VI SEM Lungs CTA bilaterally 6 x 5 cm firm, fixed, irregularly shaped, right-sided abdominal mass that extends beyond the midline

  18. Case #2 Labs • WBC 4000 • 40% segs, 60% lymphs (ANC 2400) • Hgb 9 • Hct 27 • Plts 95,000 • Retic 0.4 • Normal CMP including BUN, creatinine • elevated LDH, normal uric acid • AFP, B-HCG • Normal • Urinalysis • Normal • HVA, VMA • Elevated

  19. CT Scan of Abdomen

  20. Neuroblastoma • 7-10% of childhood cancers • 50% diagnosed before 2 yo, 90% by 5 yo • Arises from neural crest tissue of the sympathetic ganglia or adrenal medulla • “Small round blue cell” malignancy • Must be differentiated from Ewings sarcoma (PNETs), rhabdomyosarcoma, lymphoma

  21. Neuroblastoma • Post-ganglionic sympathetic neuroblasts (primitive, pluripotent) • Often constitutional symptoms: fever, irritability, weight loss • Other symptoms based on primary site • Cervical: neck mass, Horner’s syndrome • Thoracic: Superior vena cava syndrome • Paraspinous: cord compression • Abdominal: fullness, pain, obstruction • Metastatic disease: marrow, bone, liver, skin

  22. Neuroblastoma Elevated VMA, HVA, elevated ferritin, LDH, Abnormal blood counts if marrow involvement < 18 mo tend to have low stage, curable disease (70-90% survival) > 18 months tend to have metastatic disease (< 15% survival) Unique features: Stage 4S, opsoclonus-myoclonus, raccoon eyes

  23. Neuroblastoma Origin

  24. Neuroblastoma IV-S • Infants with small primary tumors (stage I or II) • Distant metastases: • Liver • Skin (blueberry muffin) • Bone marrow • NO BONE METS • Excellent prognosis with minimal or no therapy

  25. Case #3 HPI/PMH 3 yo male presents to PMD secondary to intermittent vomiting typically in the morning and irritability over the past couple of weeks Crying more, waking up at night, and his head seems to be hurting Over past few days, he seems to be weak and is falling more No fever, URI symptoms No significant PMH

  26. Case #3 PE/Labs Afebrile, vitals stable Fussy, somewhat lethargic 3 yo Normal heart and lungs Abdomen soft, NT/ND, active BS, no HSM or masses Papilledema, CN II-XII normal, Normal motor strength and sensation. Ataxic gait, dysmetria on finger to nose CBC, CMP normal

  27. CT Scan/MRI of Head

  28. Brain Tumors Most common solid tumor in childhood (25% of all childhood cancers) Tumors tend to be infratentorial (posterior fossa) in young patients and supratentorial in older patients

  29. Brain Tumors • Treatment consists of resection if possible, chemotherapy,+/- craniospinal radiation in tumors with high probability of neuroaxis dissemination (≥ 3 yo) • Prognosis variable depending on location, grade of tumor, resectability

  30. Case #4 HPI/PMH 15 yo male presents with L knee pain of approximately 1 month duration Originally began when he fell on his knee while being tackled playing touch-football with friends + swelling above the knee that seems to be getting worse No fever, no redness or warmth to the area PMH unremarkable

  31. Case #4 PE/Labs Afebrile, vitals signs stable Healthy appearing 15 yo in NAD Mass palpated involving L distal femur, no effusion, warmth, erythema noted Normal CBC Elevated alkaline phosphatase and LDH

  32. X-Ray, MRI of Knee

  33. Bone Tumors Signs of bone tumor include pain at site (often following minor trauma), mass, and fracture at area of bone destruction Osteosarcoma and Ewing sarcoma are most common types Outcomes variable depending on whether or not metastases present at diagnosis and location

  34. Case #5 HPI/PMH 6 month old WF presents to her pediatrician for evaluation of fussiness and vomiting. She has been on zantac for GERD. PMH: term SVD, immunizations UTD FH: colon cancer and colon polyps in maternal family members SH: lives with family; only child

  35. Case #5 PE/Labs • Vital signs stable; afebrile • PE notable for hepatomegaly • Abdominal u/s reveals multilobular mass in right lobe of liver measuring 7.6 x 10.9 cm. • CT scans without evidence of metastatic disease. • Labs: • AST mildly elevated 96 • T bili 1.1 (direct bilirubin 0.3) • CBC notable for mild anemia • AFP 2300 (<15)

  36. CT Scan

  37. Hepatoblastoma Malignant liver tumors account for 1.1% of all childhood cancers (1.5 cases/million children under age 15 yearly) Hepatoblastoma accounts for 2/3rds Usually occurs in first 4 yrs of life. More common in males Outcome is linked to stage of disease Associated with prematurity Occurs in association with several cancer genetic syndromes.

  38. Genetic Syndromes leading to Hepatoblastoma • Beckwith-Wiedemann • Reports of Li-Fraumeni (TP53) and trisomy 18 • Familial adenomatous polyposis (FAP): germline mutation of APC gene (chromosome 5q21.22) which is a tumor suppressor gene. • Associated also with colorectal cancer and hepatocellular carcinoma

  39. Case 6: HPI/PMH 16 mo WM who has been tilting his head when trying to look at objects. Appetite has been normal. Rest of ROS is negative. Pediatrician referred to ophthalmology who noted a lesion in the left eye on dilated exam. PMH/FH: unremarkable

  40. Case continues MRI of brain confirms lesion in left eye (1.1 x 0.7 x 1.4 cm). Exam under anesthesia confirms left eye lesion and revealed very small lesion in right eye.

  41. Retinoblastoma Malignant tumor of the embryonic neural retina Most common intraocular tumor of childhood Approx 3% of all childhood cancers 1/15,000 live births per year in US- estimated 200 new diagnosis per year (20-30% are bilateral) Median age at diagnosis is 2 yrs (90% of cases are diagnosed before age 5)

  42. Retinoblastoma presentation Leukocoria Strabismus Lack of pain unless secondary glaucoma or inflammation. Diagnosis is made by ophthalmologic and radiographic evaluation with pathologic confirmation usually not needed. Labs are usually normal

  43. Hereditary Retinoblastoma • Usually presents with bilateral disease/mutifocal tumors and at a younger age (median age at dx is 11 mos). • RB1 gene • Tumor suppressor gene on 13q14 • RB protein- molecular switch controlling passage thru G1 check point between G1 and entry into S phase • Vital for cell division/proliferation

  44. RB1 mutations • Germline mutation- 90% risk of developing RB and 50% risk of passing mutation to offspring • Genetic testing can identify mutations in 80-90% of individuals • Screening: • Fundoscopic exam 1-2 wks after birth and every 2 weeks until 3 months of age. Then space out and continue exams until 4-5 yrs. • Yearly MRI from 6 mos to 5 yrs to screen for pineal tumors

  45. Treatment • 90% overall 5 year survival • Metastatic disease associated with a poor prognosis • Trilateral retinoblastoma: • Primary RB of pineal and parasellar sites • Usually seen in familial disease • Usually fatal • Mgmt: • Local therapy- enucleation, cyrotherapy, thermotherapy • Radiotherapy • Systemic chemotherapy

  46. Case #7 • Presented to PMD with 3-day history of swelling in R neck • Dx’d with sialthiothiasis and rx’d augmentin bid, set him up see ENT if no improvement • 11/24 saw ENT who agreed with diagnosis but obtained CT scan because it was not responding to antibiotics • Increased dose of augmentin • 11/26 CT scan • Large mass in R maxillary, ethmoid and extrachonal orbit • 12/4 CT instatrac and MRI done

  47. Case #7 PE: T 96.4, HR 76, BP 120/57, RR 20, GEN: pleasant, oriented HEENT: +proptosis of R eye, with subjunctival hemorrhage, EOMI, large mass over R max sinus, large palpable mass in R supraclavicular area, nonerythematous, somewhat tender, very firm CV, LUNG, LYMPH, ABD exams unremarkable LABS: CBC wnl, UA 10.3, LDH 1379

  48. Soft tissue sarcoma • Sarcoma: malignant tumor of mesenchymal cell origin • Rhabdomyosarcoma: arises from immature mesenchymal cells that are committed to skeletal muscle lineage (55-60%) • Non-RMS STS: Histology other than RMS (40-55%)

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