Common Pediatric Solid Tumors

Abdullatiff Sami Al-RashedCollege of Medicine, King Faisal University Al-Ahsa, Saudi Arabia

Objectives

• Definition.• Epidemiology.• Etiology.• Types.• Symptoms &

Signs.• Staging.• Diagnosis.• Treatment.• Prognosis.

• Definition.• Epidemiology.• Etiology.• Types.• Symptoms &

Signs.• Staging.• Diagnosis.• Treatment.• Prognosis.

• Definition.• Treatment.

Lymphoma(Hodgkin's & Non-Hodgkin's)

Definition• Neoplasia of the lymphatic system and its precursor cells

with genetically distrusted regulation of proliferation, differentiation and apoptosis.

• Characterized by painless, progressive enlargement of the lymph nodes with continues extension between lymph node regions.

Epidemiology • Lymphoma accounts 10% of all pediatric neoplasia.

Hodgkin's lymphoma Non-Hodgkin's lymphoma 7 in 1 million children < age of 16 newly diagnosed with HL annually

Ratio of boys to girls 2:1

Boys > Girls , but during adolescence the incidence is the same

Peak incidence between 5 to 15 years old

Peak incidence between 15 to 35 years old

Etiology

• Unknown etiology in humans.

• It has been found that there is correlation with infection “e.g Epstein-barr Virus”.

• Correlation with Socioeconomic status: the higher SES the more frequently HL occur.

• Genetic causes.

• Unknown etiology in humans.

• Predisposing factors include:– Autoimmune disorders.– Infections ( HIV, EBV, HTLV-1 )– Congenital B-Cell defect.– Congenital T-Cell defect.– Drug induced

(immunosuppressive treatment)

HL NHL

Types

Lymphoma

Hodgkin's lymphoma

Classical Hodgkin’s

lymphoma (90-95%).

Nodular lymphocyte

predominant HL

Non Hodgkin's lymphoma

Diffuse large B-cell lymphoma

(30%)Peripheral T-call

lymphoma

Burkitt Lymphoma

(Highly aggressive)

Other types

Symptoms & SignsPainless enlarged

lymph nodes (commonest

cervical)

Symptoms & Signs

Staging

Diagnosis

Treatment • Treatment plan is assigned based on

the type and stage of lymphoma.

• Traditionally, management consists of a combination of chemotherapy drugs.

• Combination of radiotherapy and chemotherapy can be used in Hodgkin's lymphoma.

Prognosis

Nephroblastoma(Wilms tumor)

Definition

• Malignant embryonal tumor of renal tissue.

• First described in 1899 by max wilms.

Epidemiology

Etiology• Wilms tumor has been associated with loss of function mutations of

a number of tumor suppressor and transcription genes.

• These include mutations of the WT1, p53, FWT1, and FWT2 genes, and at the 11p15.5 locus.

• Associated with some congenital syndromes. These syndromes include:

– WAGR syndrome.– Denys-Drash syndrome.– Beckwith-Wiedemann syndrome.

Types• Histologically, the classic favorable histology Wilms

tumor is comprised of three cell types:

Symptoms & Signs

Staging

Diagnosis

Diagnosis

THE DEFINITIVE DIAGNOSIS OF WILMS TUMOR IS MADE BY HISTOLOGIC

CONFIRMATION AT THE TIME OF EITHER SURGICAL EXCISION OR BIOPSY.

Treatment • Treatment plan is assigned based on results of the initial

staging, histological, and molecular studies. 

• In General , lines of treatment include:

Surgery Chemotherapy Radiotherapy

Prognosis

Neuroblastoma

Definition • Malignant embyronal tumor of precursor cells of sympathetic

ganglia and adrenal medulla.

• Commonly used to refer to a spectrum of neuroblastic tumors (including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) 

Epidemiology

Etiology• Etiology is unknown.

Risk factors:• Maternal factors: These include the following:

– Opiate consumption– Folate deficiency– Toxic exposures – Congenital abnormalities – Gestational diabetes mellitus 

• Genetic factors:– A higher incidence of neuroblastoma has been suggested in girls with Turner

syndrome, Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1 (NF1) 

Types• Neuroblastomas can arise anywhere throughout the

sympathetic nervous system. 

• The common primary sites: 

Adrenal gland(40%)

Abdominal(25%)

Thoracic(15%)

Types

Symptoms & Signs

Staging

Diagnosis

Diagnosis• Diagnostic criteria — Minimum criteria for establishing a diagnosis

of neuroblastoma have been agreed upon by an international consensus panel. A definitive diagnosis of neuroblastoma requires one of the following:

– An unequivocal histologic diagnosis from tumor tissue by light microscopy, with or without immunohistochemistry, electron microscopy, or increased urine (or serum) catecholamines or their metabolites.

– Evidence of metastases to bone marrow on an aspirate or trephine biopsy with concomitant elevation of urinary or serum catecholamines or their metabolites.

Treatment • Treatment plan is assigned based on:

– Stage of the disease. – Patient age.– Histologic appearance of the tumor.– Presence or absence of amplification of the MYCN oncogene.– Quantitative DNA content of the tumor (DNA index or ploidy). 

Treatment

•In General , lines of treatment include:

Surgery Chemotherapy Radiotherapy

Prognosis

hepatoblastoma

Definition

• Hepatoblastoma is the most common primary hepatic malignancy in early childhood.

• The majority of hepatoblastomas occur in the first two years of life and rarely in children older than five years.

Epidemiology

• One percent of all pediatric neoplasias

• The incidence of hepatoblastoma in boys is twice that in girls. 

Etiology• Exact etiology is unknown. • Syndromes with an increased incidence of

hepatoblastoma include:– Beckwith Wiedmann syndrome.– Trisomy 18 & 21.– Acardia syndrome.– Li-Fraumeni syndrome.– Goldenhar syndrome (a type of craniofacial microsomia).– Type 1a glycogen storage disease (von Gierke’s disease).– Familial adenomatous polyposis (FAP).

Types

Symptoms & Signs

StagingStage Characteristics Stage I • The tumor is completely resectable via

wedge resection or lobectomy.• The tumor has PFH results.• The AFP level is within reference range

within 4 weeks of surgery.

Stage IIA • The tumor is completely resectable.• The tumor has histologic results other than

PFH (UH).

Stage IIB • The tumor is completely resectable.• AFP findings are negative at time of diagnosis

(ie, no marker to follow).

Stage III (any of the following) • The tumor is initially unresectable but is confined to one lobe of liver.

• Gross residual disease is present after surgery.

• Tumor ruptures or spills preoperatively or intraoperatively.

• Regional lymph nodes are involved.

Stage IV Distant bone or lung metastasis is present.

Diagnosis

Treatment • Treatment plan is assigned based on results of the initial

staging, histological, and molecular studies. 

• In General , lines of treatment include:

Surgery Chemotherapy Radiotherapy

Prognosis

Hemangioma

Definition• Infantile hemangiomas are benign vascular neoplasms

that have a characteristic clinical course marked by early proliferation and followed by spontaneous involution.

• Hemangiomas are the most common tumors of infancy and usually are medically insignificant.

Treatment• The vast majority of infantile hemangiomas do not

require any medical or surgical intervention.

• Intervention may be required for lesions with potential to interfere with a vital structure or function. These include, but are not limited to:– Lesions in the airway, liver, or gastrointestinal tract.– Lesions in the periorbital region.– Very large, rapidly growing cutaneous hemangiomas.

Treatment

• Treatment choices are:– Drugs: Steroids and Beta-Blocker– Surgery: laser surgery or surgical excesion

References