accidents; 44%

other; 22%

meningitis; 1%

heart disease; 4%

congenital anomalies; 8%

CANCER; 10%

Suicide, 1%

Pneumonia, 2%

cerebral palsy; 1%

HIV infection; 1%Homicide, 1%

Leading causes of death of children between of 1 and 14

Distribution of cancer in children younger than 15 years of age by diagnosis

Acute lynphoblastic leucemia 23.3 %

CNS tumors 20.7 %

Neuroblastoma 7.3 %

Non-Hodgkin's 6.3 %

Wilms' tumor 6.1 %

Hodgkin's disease 5 %

Acute myeloid leukemia 4.2 %

Rhabdomypsarcoma 3.4 %

Retinoblastoma 2.9 %

Osteosarcoma 2.6 %

Other 2.1 %

Common chief complaints given by parents that suggest a pediatric cancerChief complaints Suggested cancer

Chronic drainage from ear Rhabdomyosarcoma: Langerrhans cell histiocytosis

Morning headache with vomiting Brain tumor

Lump in neck that that does not respond to antibiotics

Hodgkin’s or non- Hodgkin’s lymphomas

Swollen face and neck Non-Hodgkin’s lymphoma, leukemia

Mass in abdomen Wilm’s tumor, neuroblastoma, hepatoma

Bleeding from vagina Yolk sack tumor, rhabdomyosarcoma

Weight loss Hodgkin’s lymphoma

Bone pain Leukemia, neuroblastoma

TERATOMASTERATOMAS • Teratomas are tumors comprising more than a single cell Teratomas are tumors comprising more than a single cell

type derived from more than one germ layer. A significant type derived from more than one germ layer. A significant degree of confusion has arisen regarding nomenclature for degree of confusion has arisen regarding nomenclature for the various subtypes of teratomas. The word itself is derived the various subtypes of teratomas. The word itself is derived from the Greek word from the Greek word teraton,teraton, meaning monster, and was meaning monster, and was used initially by Virchow in the first edition of his book on used initially by Virchow in the first edition of his book on tumors, which was published in 1863. Teratomas range tumors, which was published in 1863. Teratomas range from benign, well-differentiated (mature) cystic lesions to from benign, well-differentiated (mature) cystic lesions to those that are solid and malignant (immature). Besides those that are solid and malignant (immature). Besides being mature, with malignant transformation, teratomas also being mature, with malignant transformation, teratomas also may be monodermal and highly specialized. may be monodermal and highly specialized.

The most common The most common locationlocation

• sacrococcygeal (57%)sacrococcygeal (57%)• gonads (29%)gonads (29%) • mediastinal (7%)mediastinal (7%) • retroperitoneal (4%)retroperitoneal (4%) • cervical (3%)cervical (3%) • intracranial (3%)intracranial (3%)

Classification of the Classification of the

sacrococcygeal teratomassacrococcygeal teratomas • Type I tumorsType I tumors are predominantly external, are predominantly external, attached to the coccyx, and may have a small attached to the coccyx, and may have a small presacral component (45.8%). No metastases presacral component (45.8%). No metastases were associated with this group.were associated with this group.

• Type II tumorsType II tumors have both an external mass have both an external mass and significant presacral pelvic extension and significant presacral pelvic extension (34%) and have a 6% metastases rate.(34%) and have a 6% metastases rate.

• Type III tumorsType III tumors are visible externally, but are visible externally, but the predominant mass is pelvic and the predominant mass is pelvic and intraabdominal (8.6%). A 20% rate of intraabdominal (8.6%). A 20% rate of metastases was found in this group.metastases was found in this group.

• Type IV lesionsType IV lesions are not visible externally but are not visible externally but are entirely presacral (9.6%) and have an 8% are entirely presacral (9.6%) and have an 8% metastases rate.metastases rate.

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Sacrococcygeal teratoma

Malignant sacrococcygeal teratoma

Complications

Differential Differential TreatmentTreatmentdiagnosis:diagnosis:

• мeningomyelocele

• rectal abscess • dermoid cyst • angioma • lipoma• neurogenic

tumor• pilonidal cyst.

Surgical, including removal of the coccyx.Malignant = surgical excision + chemotherapy + radiation (metastases to lung, bone, liver).

RHABDOMYOSARCOMARHABDOMYOSARCOMAA malignant tumor of mesenchimal cell origin is A malignant tumor of mesenchimal cell origin is

called a sarcoma. Mesenchymal cells normaly mature called a sarcoma. Mesenchymal cells normaly mature into skeletal muscle, smooth muscle, fat, fibrous tissue, into skeletal muscle, smooth muscle, fat, fibrous tissue, bone, and cartilage. Rhabdomyosarcoma is thought to bone, and cartilage. Rhabdomyosarcoma is thought to arise from immature mesenchimal cells that are arise from immature mesenchimal cells that are committed to skeletal muscle lineage, but these tumors committed to skeletal muscle lineage, but these tumors can arise in tissues in which striated muscle is not can arise in tissues in which striated muscle is not normally found, such as urinary bladder. normally found, such as urinary bladder.

Rhabdomyosarcoma (RMS), the most common soft Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in infants and children, represents about tissue sarcoma in infants and children, represents about 5-15% of all malignant solid lesions. RMS arises from a 5-15% of all malignant solid lesions. RMS arises from a primitive cell type and occurs in mesenchymal tissue at primitive cell type and occurs in mesenchymal tissue at almost any body site excluding brain and bone. almost any body site excluding brain and bone.

The Intergroup The Intergroup Rhabdomyosarcoma Study Rhabdomyosarcoma Study

divides tumors into 5 types:divides tumors into 5 types:– embryonal (57 %), embryonal (57 %), – alveolar (19 %), alveolar (19 %), – botryoid (6 %), botryoid (6 %), – undifferentiated (17 %), undifferentiated (17 %), – pleomorphic (1 %). pleomorphic (1 %).

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embryonic rhabdomyosarcoma of the vesica urinaria

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Orbital embryonic rhabdomyo-sarcoma

Alveolar rhabdomyosarcoma

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Wilms’ tumor (nephroblastoma)

• Wilms’ tumor is thought to be caused Wilms’ tumor is thought to be caused by alterations of genes responsible for by alterations of genes responsible for normal genitourinary development. normal genitourinary development. Examples of common congenital Examples of common congenital anomalies associated with Wilms’ anomalies associated with Wilms’ tumor are cryptorchidism, double tumor are cryptorchidism, double collecting system, horseshoe kidney, collecting system, horseshoe kidney, and hypospadias. Environmental and hypospadias. Environmental exposures, although considered, seem exposures, although considered, seem less likely to play a role.less likely to play a role.

ClinicalClinical

History.History. The most common presentation is an asymptomatic The most common presentation is an asymptomatic abdominal mass discovered by the parent or physician. abdominal mass discovered by the parent or physician. Occasionally the child presents with haematuria, but Occasionally the child presents with haematuria, but symptoms are often non-specific: abdominal fullness, symptoms are often non-specific: abdominal fullness, abdominal pain, gastrointestinal upset, fever, weight loss, abdominal pain, gastrointestinal upset, fever, weight loss, malaise, and anaemia. Hypertension is sometimes detectable. malaise, and anaemia. Hypertension is sometimes detectable.

A small number of patients who have hemorrhaged into A small number of patients who have hemorrhaged into their tumor may present with signs of hypotension, anemia, their tumor may present with signs of hypotension, anemia, and fever. Rarely, patients with advanced-stage disease may and fever. Rarely, patients with advanced-stage disease may present with respiratory symptoms related to the presence of present with respiratory symptoms related to the presence of lung metastases.lung metastases.

Cytogenetics studiesCytogenetics studies

• An 11p13 deletion as in the WAGR An 11p13 deletion as in the WAGR syndrome (Wilms’, aniridia, syndrome (Wilms’, aniridia, genitourinary abnormalities, mental genitourinary abnormalities, mental retardation)retardation)

• A duplication of the paternal allele A duplication of the paternal allele 11p15 as in BWS11p15 as in BWS

• Mutational analysis of the WT1 gene Mutational analysis of the WT1 gene in cases where Denys-Drash syndrome in cases where Denys-Drash syndrome (intersexual disorders, nephropathy, (intersexual disorders, nephropathy, Wilms’ tumor) is suspectedWilms’ tumor) is suspected

Imaging StudiesImaging Studies

• Renal ultrasonographyRenal ultrasonography (with dynamic imaging of the (with dynamic imaging of the renal vein and interior vena cava).renal vein and interior vena cava).

• CT scanningCT scanning.. Abdominal CT scanning helps determine Abdominal CT scanning helps determine the tumor's origin, lymph node involvement, bilateral the tumor's origin, lymph node involvement, bilateral kidney involvement, and invasion into major vessels (eg, kidney involvement, and invasion into major vessels (eg, inferior vena cava or liver metastases). If findings on inferior vena cava or liver metastases). If findings on chest CT scanning are positive while chest radiographic chest CT scanning are positive while chest radiographic findings are negative, diagnostic biopsy of the lesions findings are negative, diagnostic biopsy of the lesions noted on the chest CT scan is recommended.noted on the chest CT scan is recommended.

• Chest radiographyChest radiography (4-field) - Detects lung metastases (4-field) - Detects lung metastases (Patients with lung lesions on chest radiography receive (Patients with lung lesions on chest radiography receive whole lung radiotherapy.)whole lung radiotherapy.)

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Wilms’ tumor (nephroblas-toma)

Imaging Studies Recommended for Follow-Up of Children with Wilms’ Tumor Free of Metastasis at Diagnosis

Wilms’ Tumor Type Imaging Studies Off-Treatment Schedule

FH (stages I, II, and III) Anaplastic histology (stages I, II, and III)

Chest radiography

After 6 weeks and 3 months postoperatively; then every 3 months (5 times), then every 6 months (3 times), then yearly (2 times)

Patients aged <48 months at diagnosis with nephrogenic rests (all stages)

Abdominal ultrasonography

Every 3 months for 6 years

Patients aged >48 months at diagnosis with nephrogenic rests (all stages)

Abdominal ultrasonography

Every 3 months for 4 years

FH (stage I and II)Abdominal

ultrasonographyYearly (6 times)

FH (stage III)Abdominal

ultrasonography

After 6 weeks and 3 months postoperatively; then every 3 months (5 times), then every 6 months (3 times), then yearly (2 times)

Unfavorable histology (all stages)

Abdominal ultrasonography

Every 3 months (4 times), then every 6 months (4 times)

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nephroblastoma:

angiograma

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Leftside nephroblastoma (CT-scan)

Current approach to Wilms’ tumor by stage and histologyCurrent approach to Wilms’ tumor by stage and histology Stage, Histology Surgery Chemotherapy Radiotherapy*

Stage I or II with FHStage I with anaplasia

NephrectomyVincristine

DactinomycinNone

Stage III or IV with FHStage II, III, or IV with focal anaplasia

NephrectomyVincristine

DactinomycinDoxorubicin

Yes

Stage II, III, or IV with diffuse anaplasiaStage I, II, III, or IV CCSK

Nephrectomy

VincristineDoxorubicinCyclophosphamideEtoposide

Yes

Stage I, II, III, or IV RTK

NephrectomyCyclophosphamide

EtoposideCarboplatin

Yes

NEUROBLASTOMANEUROBLASTOMANeuroblastoma is a tumour of neural Neuroblastoma is a tumour of neural

crest origin which may occur in the crest origin which may occur in the adrenal medulla or anywhere along the adrenal medulla or anywhere along the sympathetic ganglion chain, namely in the sympathetic ganglion chain, namely in the neck, thorax, abdomen, and pelvis. neck, thorax, abdomen, and pelvis. Seventy-five per cent of tumours occur in Seventy-five per cent of tumours occur in the abdomen (adrenal medulla 50 %, the abdomen (adrenal medulla 50 %, paraspinal ganglia 25 %), 20 % occur in paraspinal ganglia 25 %), 20 % occur in the thorax, and 5 % occur in the neck and the thorax, and 5 % occur in the neck and the pelvis.the pelvis.

Localizations of the Localizations of the neuroblastoma

• 75 % of tumours occur 75 % of tumours occur in the abdomen: in the abdomen:

– adrenal medulla 50 adrenal medulla 50 %, %,

– paraspinal ganglia paraspinal ganglia 25 %25 %

• 20 % occur in the 20 % occur in the thorax, thorax,

• 5 % occur in the neck 5 % occur in the neck and the pelvisand the pelvis

The Evans classification for The Evans classification for neuroblastoma neuroblastoma

• Stage I:Stage I: tumor confined to an organ of origin. tumor confined to an organ of origin. • Stage II:Stage II: tumor extending beyond an organ of tumor extending beyond an organ of

origin, but not crossing the midline. origin, but not crossing the midline. Ipsilateral lymph nodes may be involved. Ipsilateral lymph nodes may be involved.

• Stage III:Stage III: tumor extending beyond midline. tumor extending beyond midline. Bilateral lymph nodes may be involved. Bilateral lymph nodes may be involved.

• Stage IV:Stage IV: remote disease involving skeleton, remote disease involving skeleton, bone marrow, soft tissue or distant lymph bone marrow, soft tissue or distant lymph nodes. nodes.

• Stage IVS:Stage IVS: same as stage I or II with same as stage I or II with presence of disease in liver, skin or bone presence of disease in liver, skin or bone marrow.marrow.

DiagnosisDiagnosisUltrasonographyUltrasonography distinguishes neuroblastoma (solid, distinguishes neuroblastoma (solid,

extrarenal) from cystic lesions and renal tumours. The extrarenal) from cystic lesions and renal tumours. The radiographic detectionradiographic detection of calcification in the tumour is of calcification in the tumour is suggestive of neuroblastoma. In children with an suggestive of neuroblastoma. In children with an abdominal neuroblastoma, abdominal neuroblastoma, intravenous urographyintravenous urography shows displacement rather than distortion of the shows displacement rather than distortion of the pelvicaliceal system. A pelvicaliceal system. A skeletal survey skeletal survey and and chest chest radiographradiograph are mandatory to detect possible metastases. are mandatory to detect possible metastases. CTCT gives good anatomical data about the tumour. gives good anatomical data about the tumour. Recent studies suggest that Recent studies suggest that magnetic resonance magnetic resonance imagingimaging (MRI) is useful both to delineate the primary (MRI) is useful both to delineate the primary tumor and to evaluate bone marrow metastasis, vessel tumor and to evaluate bone marrow metastasis, vessel involvement, and extension into spinal cord.involvement, and extension into spinal cord.

Presenting complaints with neuroblastoma

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Mediastinal neuroblastoma

SurgerySurgeryplays the pivotal role in the management of plays the pivotal role in the management of

neuroblastoma. Depending of the timing, neuroblastoma. Depending of the timing, operative procedures can have diagnostic as well operative procedures can have diagnostic as well as therapeutic functions. The goals of primary as therapeutic functions. The goals of primary surgical procedures, performed before any other surgical procedures, performed before any other therapy, are to establish the diagnosis, to provide therapy, are to establish the diagnosis, to provide tissue for biological studies, to stage the tumor tissue for biological studies, to stage the tumor surgically, and to attempt to excise the tumor, if surgically, and to attempt to excise the tumor, if feasible. In delayed primary or second look feasible. In delayed primary or second look surgery, the surgeon determines response to surgery, the surgeon determines response to therapy and removes residual disease when therapy and removes residual disease when possible.possible.

Classification of Vascular Classification of Vascular LesionsLesions

Vascular malformations (flat lesions)

Hemangiomas (raised lesions)

Salmon patch (also known as nevus simplex or nevus telangiectaticus)

Superficial hemangioma (Cherry, strawberry hemangioma)

Port-wine stain (also known as nevus flammeus)

Deep hemangioma (also known as cavernous hemangioma)

Strawberry Strawberry haemangiomashaemangiomas

Cherry haemangiomasCherry haemangiomas

Cavernous haemangiomas

Before treatment

After treatment

Port wine stains

Treatment Indications for HemangiomasTreatment Indications for Hemangiomas Threat to life or functionThreat to life or function

– Kasabach-Merritt syndrome (coagulopathy)Kasabach-Merritt syndrome (coagulopathy)– Anatomic siteAnatomic site– Vision impairment Vision impairment – Respiratory impairmentRespiratory impairment– High-output cardiac failure (mortality up to 50 %)High-output cardiac failure (mortality up to 50 %)– IInternal lesions nternal lesions

Location in scar-prone area Location in scar-prone area – NoseNose, , LipLip, , EarEar, , Glabellar area Glabellar area – Any large facial hemangiomasAny large facial hemangiomas– Pedunculated lesions Pedunculated lesions

Tendency to bleed or to become infectedTendency to bleed or to become infectedRapid rate of growth (tripling in size within weeks)Rapid rate of growth (tripling in size within weeks)

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Cavernous lymphangioma

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naevus melanoma

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melanoma