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Assessment and Diagnosis of Abdominal Masses in Children Resident Education Lecture Series.

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Presentation on theme: "Assessment and Diagnosis of Abdominal Masses in Children Resident Education Lecture Series."— Presentation transcript:

1 Assessment and Diagnosis of Abdominal Masses in Children Resident Education Lecture Series

2 General approach to solid tumors What is it? Where is it? Where can it go? The answer to any one of these questions will help answer the other two

3 Work up – two components Staging X-ray of primary site CT chest, abdomen, & pelvis CXR (baseline) bone scan Specialty tests Gallium, MIBG, PET Bone marrow ESR Evaluate for complications of the tumor CBC with diff TPN panel LDH, uric acid – tumor lysis, rapid cell growth Lytes, creatinine – renal function Transaminases – hepatic involvement Specialty tests Tumor markers HCG, AFP HVA/VMA ….

4 Tissue diagnosis Incisional biopsy Excisional biopsy Special cases… Calicified suprarenal mass + bone scan – might consider getting dx from bone marrow FNA vs excisional biopsy Bias towards excisional → sufficient sample to be representative and to send for special research studies (histology, chromosomes, special studies, research studies)

5 Abdominal Masses

6 Trends Abdominal masses are most common in children under the age of 5 years Most abdominal masses in neonates are retroperitoneal, of kidney origin and are not malignant The older the child the more likely the mass represents a malignant process

7 Possible Diagnoses of Abdominal Masses in Infancy and Childhood Atlas of Pediatric Physical Diagnosis, Fourth Edition

8 Abdominal Masses in Older Children Renal 55% Wilms (& other) 25% Hydronephrosis 20% Cystic disease 5% Non Renal Retroperitoneal 23% Neuroblastoma 21% Teratoma 1% Other 1% Gastrointestinal 12% Appendiceal Abscess Lymphoma Hepatobiliary 6% Tumors Hepatoblastoma HCC Genital 4% Ovarian Cysts and Teratoma Kirk et al., 1981 Radiol. Clin. North Am., 19:

9 Neonatal Abdominal Masses Renal 55% Hydronephrosis35% Cystic disease10% Multicystic dysplastic Polycystic dysplastic Solid Tumors10% Mesonephric nephroma nephroblastomatosis Pelvic / Genital 15% Teratoma Ovarian Cysts Hydrometrocolpos Obstructed bladder non-Renal Retroperitoneal 10% Adrenal Hemorrhage neuroblastoma Gastrointestinal 15% Duplication Mesenteric omental cyst Pseudocyst from complicated obstruction Meconium ileus Hepatobiliary 5% Hepatic tumors Hemangioendothelioma Cystic mesenchymal hamartoma Hepatoblastoma Neuroblastoma Choledochal cyst Kirk et al., 1981 Radiol. Clin. North Am., 19:

10 Examination of the Pediatric Abdomen History – time the mass has been present, rapidity of growth, symptoms Undress patient: evaluate for genetic or inherited predisposition as well as the belly Palpate from the pelvis toward the thorax Describe location Size Consistency Ascites Venous congestion of surface Golden and Feusner, 2002, Pediatr Clin N Am, 49:

11 Neuroblastoma u Malignancy in neural crest cells in sympathetic ganglia, adrenal medulla, chest, abdomen; small round blue tumor cells u Nonmalignant form is ganglioneuroma u Clinical effects r/t tumor size and location u Genetic links/factors involved: N-myc oncogene, chromosome deletion

12 NB Incidence/ Etiology u 4th peds cancer (7-10%) new US per year u Most common cancer in infants – accounts for 50% of cancer in NBs. M:F ratio: 1.2:1 u Average age is 18 months; 80% < 5; small #, genetic? u May be a “Silent” tumor presenting with widespread disease at dx 50 (younger) – 70 (older) % of time

13 Clinical Presentation u Pain, abd mass, other masses, malaise; skin u Can occur anywhere in sympathetic NS u >50% are retroperitoneal; head/neck, pelvis, posterior mediastinum; +/- spinal cord compression** u Metastatic to lymph nodes, bone, BM, liver u Fever and malaise; catecholamine secretion: HTN, sweats, irritability; diarrhea; opsoclonus-myoclonus; cerebellar ataxia

14 Diagnostic Workup u Hx: catecholamine related sx (htn, flushing, sweating, irritability); wt loss, pain, limp u PE: preorbital ecchymosis, cutaneous nodules; abd mass; weakness/paralysis u CT/MRI to locate tumor; bone scan; MIBG; PET? u Labs (urinary catecholamines); u Bilateral BMA and bx; chromosome studies

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16 Neuroblastoma Staging 1Localized tumor; complete excision 2AUnilateral, incomplete gross resection; negative microscopic nodes 2BUnilateral, positive ipsilateral nodes; negative contralateral 3Across midline, or contralateral nodes 4Dissemination: bone marrow, liver, skin, bones 4S<1y: local stage 1-2 with mets to BM, liver, skin

17 Treatment and Prognosis u Surgery: debulk or total removal; curative in low-stage disease; 2nd-look after other Rx u Chemotherapy – often platinum based multi-agent ~ stage u RT: to primary tumor site; NB cells very radiosensitive; before or after surgery; emergency relief for cord compression, respiratory compromise, proptosis

18 NB Treatment cont’d u BMT: u children with poor prognosis initially may be treated with high dose chemotherapy with autologous stem cell rescue(s); u BMT may be used with relapse u Prognosis: <1 best (75 + % survival); worst for children >2 with stage IV disease (10-20%);

19 Stage IV disease – survival trends

20 p = NEJM 341: , 1999

21 p = NEJM 341: , 1999

22 p = 0.02

23 Tumors of the Kidney u Primary tumors arising from the kidney, usually Wilms, rapidly growing vascular abdominal tumors; fragile gelatin capsule u Others: clear cell sarcoma, renal cell CA, lymphoma, PNET, rhabdoid, … u Wilms tumor pathology may be favorable or unfavorable depending on degree of anaplasia present; prognosis and treatment r/t pathology

24 Incidence and Etiology u Renal tumors represent 5-6% of peds cancer; 460 new US cases/yr u Higher in AA, lower in Asians u Peak age at 2-3; rare in kids >5; M:F 0.9:1.0 (unilateral) 0.6:1.0 (bilateral) males younger age at diagnosis u 1.5% familial in origin; associated with aniridia, hemihypertrophy, GU malforms u Genetic factors, deletion or translocations

25 What is this syndrome? Omphalocele Macroglossia Gigantism Exophthalmos Hypoglycemia Beckwith-Wiedemann

26 Hemi-hypertrophy

27 Clinical Presentation u Asymptomatic abdominal mass found by family or on routine PE u Pain, malaise, hematuria in 20-30%; 25% with HTN; rare subcapsular hemorrhage, with rapid increase in size, anemia, HTN u Mets to lungs, liver, regional nodes u 7% bilateral, at dx or later

28 Diagnostic Workup u H and P u Labs, renal and hepatic function u Imaging studies: US to determine size and shape, vessel involvement, thrombi in major vessels; chest film/CT to check for mets u Liver, brain, and bone mets not routinely assessed unless indicated by S/S

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30 Prognosis u Histology is most important prognostic factor (favorable histology vs. anaplastic) u Stage at diagnosis also crucial u Genetic factors u Age

31 Staging of Wilms Tumors ILimited to kidney; complete resection IIExtent beyond kidney, but complete R IIIResidual tumor, confined to abdomen IVHematogenous mets (lung, liver, bone, brain) or lymph nodes outside abdomen VBilateral renal involvement at diagnosis Tumor spill at time of surgery – considered stage III

32 Treatment and Prognosis u Surgery initially, with exam of contralateral kidney; u Preop chemotherapy if intravascular spread or very large invasive tumors; if bilateral; u NA argument: Preop Chemo prevents adequate assessment of staging u Considered Stage III if imaged only

33 Treatment and Prognosis cont’d u Bilateral: preop Chemo; nephrectomy of worse side, partial on other u Chemotherapy: regimens based in national groups u RT: port extended across midline to prevent scoliosis; if favorable histology, RT only for Stage III and IV; post lung RT, adjust Chemo u Recurrence: worse if <1 year; on chemo u Prognosis: <50% - 100% (stage/histology)

34 Malignant Hepatic Tumors u Hepatoblastoma; median age of 1 yr; u Hepatocellular carcinoma, median age of 12 yrs, associated with hepatitis B <15 yrs, prolonged use of metabolic steroids u Nonmalignant: hemangiomas (50% of all hepatic tumors)

35 Clinical Presentation u Hepatoblastoma (80%): asymptomatic abdominal mass; osteopenia; u Hepatocellular Ca (20%): abdominal distention, RUQ mass; pain, N & V; jaundice; splenomegaly; u Elevated alphafetoprotein level

36 Treatment and Prognosis u Preop CTX followed by complete resection u Hepatoblastoma: High cure rates, with cure possible if mets are resected (> 65%) u Hepatocellular Ca: Difficult to resect and difficult to cure even with complete resection (<20%) u RT of little benefit Chemo-embolization? Orthotopic liver transplant?

37 Prognosis Hepatoblastoma Resectable tumors At diagnosis (stage I & II )- 90% Following chemo-reduction (III)~ 80% Unresectable tumors- 50 % Metastases at diagnosis- 10 %

38 Prognosis Hepatocellular Carcinoma Children with initially resectable HCC have a good prognosis and may benefit from adjuvant chemotherapy. The outcome for children with unresectable or metastatic HCC continues to be dismal with current therapies.

39 Intergroup Study for the Treatment of Childhood Hepatocellular Carcinoma Event-Free Survival by Stage

40 Differential diagnosis of Thoracic Masses (malignant) EXTRA-THORACIC Soft tissue mass Soft tissue sarcoma PNET/Ewings Lymphoma (much less common) Bony Mass Ewings Neuroblastoma Osteosarcoma (much less common) INTRA-THORACIC Anterior mediastinum (the 4 “T’s”) Teratoma (or germ cell tumor) Thymoma Thyroid carcinoma T-cell leukemia or other lymphoma (adenopathy +/- effusion) Posterior mediastinum Neuroblastoma, Ewings, other soft tissue sarcoma Pulmonary parenchyma Metastatic disease Lymphoma Primary pulmonary malignancy (rare, usually embryonal type) Hilar Lymphoma Rare soft tissue sarcoma or angiosarcoma

41 Differential diagnosis of extremity and/or soft tissue masses (malignant) Bone Osteosarcoma Ewings Soft tissue Rhabdomyosarcoma PNET/Ewings Fibrosarcoma other……

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43 From ABP Certifying Exam Content Outline Formulate a differential diagnosis for an abdominal mass Know that multicystic dysplastic kidneys and hydronephrosis are the most common causes of palpable abdominal masses in infants Recognize that children with hemihypertrophy and somatic overgrowth syndromes should be periodically evaluated for the development of associated embryonal tumors

44 From ABP Certifying Exam Content Outline, continued Understand that a neuroblastoma usually presents as a nontender abdominal mass Understand that urinary catecholamine excretion is increased in most patients with a neuroblastoma and that tests of urine for VMA and VHA are appropriate screening tests for the tumor Know that Wilms tumor is associated with hemihypertrophy and aniridia, somatic overgrowth, and/or genitourinary abnormalities Understand that Wilms tumor usually presents as an abdominal mass and may cause hypertension Recognize the tumors that may produce precocious puberty (eg, in liver, CNS, ovary, testes, adrenal glands)

45 Credits Michael Kelly MD PhD Anne Warwick MD MPH


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