Common Pediatric Solid Tumors
-
Upload
abdullatif-al-rashed -
Category
Health & Medicine
-
view
2.099 -
download
1
Transcript of Common Pediatric Solid Tumors
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