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 leucemia23.3 % CNS tumors20.7 % Neuroblastoma7.3 % Non-Hodgkin's6.3 % Wilms' tumor6.1 % Hodgkin's disease5 % Acute myeloid leukemia4.2 % Rhabdomypsarcoma3.4 % Retinoblastoma2.9 % Osteosarcoma2.6 % Other2.1 %

Common chief complaints given by parents that suggest a pediatric cancer Chief complaintsSuggested cancer Chronic drainage from earRhabdomyosarcoma: Langerrhans cell histiocytosis Morning headache with vomitingBrain tumor Lump in neck that that does not respond to antibiotics Hodgkin’s or non- Hodgkin’s lymphomas Swollen face and neckNon-Hodgkin’s lymphoma, leukemia Mass in abdomenWilm’s tumor, neuroblastoma, hepatoma Bleeding from vaginaYolk sack tumor, rhabdomyosarcoma Weight lossHodgkin’s lymphoma Bone painLeukemia, neuroblastoma

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

The most common location sacrococcygeal (57%) gonads (29%) mediastinal (7%) retroperitoneal (4%) cervical (3%) intracranial (3%)

Classification of the sacrococcygeal teratomas Type I tumors are predominantly external, attached to the coccyx, and may have a small presacral component (45.8%). No metastases were associated with this group.Type I tumors are predominantly external, attached to the coccyx, and may have a small presacral component (45.8%). No metastases were associated with this group. Type II tumors have both an external mass and significant presacral pelvic extension (34%) and have a 6% metastases rate.Type II tumors have both an external mass and significant presacral pelvic extension (34%) and have a 6% metastases rate. Type III tumors are visible externally, but the predominant mass is pelvic and intraabdominal (8.6%). A 20% rate of metastases was found in this group.Type III tumors are visible externally, but the predominant mass is pelvic and intraabdominal (8.6%). A 20% rate of metastases was found in this group. Type IV lesions are not visible externally but are entirely presacral (9.6%) and have an 8% metastases rate.Type IV lesions are not visible externally but are entirely presacral (9.6%) and have an 8% metastases rate.

<> Sacrococcygeal teratoma Malignant sacrococcygeal teratoma Complications

Differential Treatment 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).

RHABDOMYOSARCOMA A malignant tumor of mesenchimal cell origin is called a sarcoma. Mesenchymal cells normaly mature into skeletal muscle, smooth muscle, fat, fibrous tissue, bone, and cartilage. Rhabdomyosarcoma is thought to arise from immature mesenchimal cells that are committed to skeletal muscle lineage, but these tumors can arise in tissues in which striated muscle is not normally found, such as urinary bladder. Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in infants and children, represents about 5-15% of all malignant solid lesions. RMS arises from a primitive cell type and occurs in mesenchymal tissue at almost any body site excluding brain and bone.

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

<> embryonic rhabdomyosarcoma of the vesica urinaria <>

Orbital embryonic rhabdomyo- sarcoma

Alveolar rhabdomyosarcoma <>

Wilms’ tumor (nephroblastoma)

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

Clinical Clinical History. The most common presentation is an asymptomatic abdominal mass discovered by the parent or physician. Occasionally the child presents with haematuria, but symptoms are often non-specific: abdominal fullness, abdominal pain, gastrointestinal upset, fever, weight loss, malaise, and anaemia. Hypertension is sometimes detectable. A small number of patients who have hemorrhaged into their tumor may present with signs of hypotension, anemia, and fever. Rarely, patients with advanced-stage disease may present with respiratory symptoms related to the presence of lung metastases.

Cytogenetics studies An 11p13 deletion as in the WAGR syndrome (Wilms’, aniridia, genitourinary abnormalities, mental retardation)An 11p13 deletion as in the WAGR syndrome (Wilms’, aniridia, genitourinary abnormalities, mental retardation) A duplication of the paternal allele 11p15 as in BWSA duplication of the paternal allele 11p15 as in BWS Mutational analysis of the WT1 gene in cases where Denys-Drash syndrome (intersexual disorders, nephropathy, Wilms’ tumor) is suspectedMutational analysis of the WT1 gene in cases where Denys-Drash syndrome (intersexual disorders, nephropathy, Wilms’ tumor) is suspected

Imaging Studies Renal ultrasonography (with dynamic imaging of the renal vein and interior vena cava).Renal ultrasonography (with dynamic imaging of the renal vein and interior vena cava). CT scanning. Abdominal CT scanning helps determine the tumor's origin, lymph node involvement, bilateral kidney involvement, and invasion into major vessels (eg, inferior vena cava or liver metastases). If findings on chest CT scanning are positive while chest radiographic findings are negative, diagnostic biopsy of the lesions noted on the chest CT scan is recommended.CT scanning. Abdominal CT scanning helps determine the tumor's origin, lymph node involvement, bilateral kidney involvement, and invasion into major vessels (eg, inferior vena cava or liver metastases). If findings on chest CT scanning are positive while chest radiographic findings are negative, diagnostic biopsy of the lesions noted on the chest CT scan is recommended. Chest radiography (4-field) - Detects lung metastases (Patients with lung lesions on chest radiography receive whole lung radiotherapy.)Chest radiography (4-field) - Detects lung metastases (Patients with lung lesions on chest radiography receive whole lung radiotherapy.)

<> Wilms’ tumor (nephroblas- toma)

Imaging Studies Recommended for Follow-Up of Children with Wilms’ Tumor Free of Metastasis at Diagnosis Wilms’ Tumor TypeImaging StudiesOff-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 ultrasonographyEvery 3 months for 6 years Patients aged >48 months at diagnosis with nephrogenic rests (all stages) Abdominal ultrasonographyEvery 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)

<> nephroblastoma: angiograma

<> Leftside nephroblastoma (CT-scan)

Current approach to Wilms’ tumor by stage and histology Stage, HistologySurgeryChemotherapyRadiotherapy* Stage I or II with FH Stage I with anaplasia Nephrectomy Vincristine Dactinomycin None Stage III or IV with FH Stage II, III, or IV with focal anaplasia Nephrectomy Vincristine Dactinomycin Doxorubicin Yes Stage II, III, or IV with diffuse anaplasia Stage I, II, III, or IV CCSK Nephrectomy Vincristine Doxorubicin Cyclophosphami de Etoposide Yes Stage I, II, III, or IV RTK Nephrectomy Cyclophosphamide Etoposide Carboplatin Yes

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

Localizations of the Localizations of the neuroblastoma 75 % of tumours occur in the abdomen: –adrenal medulla 50 %, –paraspinal ganglia 25 % 20 % occur in the thorax, 5 % occur in the neck and the pelvis

The Evans classification for neuroblastoma Stage I: tumor confined to an organ of origin.Stage I: tumor confined to an organ of origin. Stage II: tumor extending beyond an organ of origin, but not crossing the midline. Ipsilateral lymph nodes may be involved.Stage II: tumor extending beyond an organ of origin, but not crossing the midline. Ipsilateral lymph nodes may be involved. Stage III: tumor extending beyond midline. Bilateral lymph nodes may be involved.Stage III: tumor extending beyond midline. Bilateral lymph nodes may be involved. Stage IV: remote disease involving skeleton, bone marrow, soft tissue or distant lymph nodes.Stage IV: remote disease involving skeleton, bone marrow, soft tissue or distant lymph nodes. Stage IVS: same as stage I or II with presence of disease in liver, skin or bone marrow.Stage IVS: same as stage I or II with presence of disease in liver, skin or bone marrow.

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

Presenting complaints with neuroblastoma

<> Mediastinal neuroblastoma

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

Classification of Vascular Lesions 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 haemangiomas

Cherry haemangiomas

Cavernous haemangiomas

Before treatment After treatment

Port wine stains

Treatment Indications for Hemangiomas Threat to life or function –Kasabach-Merritt syndrome (coagulopathy) –Anatomic site –Vision impairment –Respiratory impairment –High-output cardiac failure (mortality up to 50 %) –Internal lesions Location in scar-prone area –Nose, Lip, Ear, Glabellar area –Any large facial hemangiomas –Pedunculated lesions Tendency to bleed or to become infected Rapid rate of growth (tripling in size within weeks)

<> Cavernous lymphangioma

<> naevus melanoma

<> melanoma