Solid Organ Tumors Flashcards
What is the incidence of cancer in children under 15 years of age?
Cancer is relatively rare in childhood, with 110–130 cases per million children per year in Europe and North America.
What is the commonest cause of death in children aged 4–15 years?
Accidents are the most common cause of death, followed by cancer as the second leading cause.
What is the overall lifetime risk of developing a malignant disease in children?
About 1 in 600 children will develop a malignant disease before the age of 15 years.
Why is childhood cancer considered one of the most challenging aspects of paediatric practice?
Due to its lethal nature, insidious onset, emotional impact, and the increasing prospects of cure.
What has contributed to the improvement in survival rates of children with cancer?
The improvement has been achieved through clinical trials evaluating combinations of multi-agent chemotherapy, radiotherapy, and surgery.
What are the most common types of cancers in children?
Leukaemia and brain tumours are the most common malignancies.
What are the main types of solid tumours in children?
Solid tumours in children are mainly composed of embryonal malignancies (e.g., nephroblastoma, neuroblastoma) and sarcomas, rather than carcinoma.
How do cancers in children generally respond to treatment?
Cancers in children are generally responsive to one or more of the available treatment interventions, including chemotherapy, radiotherapy, and surgery.
What is the annual incidence of malignant tumours in children under 15 years in the Western Cape?
The annual incidence is about 75 per 1,000,000 children under 15 years of age.
What may contribute to the lower reported incidence of malignant tumours in the Western Cape?
The lower incidence may be due to under ascertainment or may represent a real difference in epidemiology compared to other regions.
How does the spectrum of cancer in children differ from that in adults?
In children, tumours are usually deep-seated and often diagnosed at advanced stages with metastases, while adult cancers are more often epithelial in origin and can be screened for.
Why is screening for childhood cancers not effective or practical?
Because childhood tumours are often deep-seated and diagnosed at advanced stages with metastases, making early detection difficult.
What is the general cure rate for childhood cancers?
Around 70% of childhood cancers are curable with good response to surgery, radiotherapy, and chemotherapy, usually in combination.
What is an indicator of cure in childhood cancer?
A 5-year tumour-free period is usually indicative of cure.
What prognostic factors influence the outcome of childhood cancers?
Prognostic factors include age at diagnosis, histological grade, and stage of the disease.
When do many paediatric tumours peak in incidence?
Many paediatric tumours have their peak incidence in children less than 5 years of age.
How has the prognosis for most malignant tumours in children improved?
Advances in paediatric surgical techniques, radiotherapy, and effective chemotherapy have significantly improved the prognosis over the last few decades.
Which childhood cancer has a poor prognosis?
Neuroblastoma has a poor prognosis, with an overall survival rate of less than 30%.
What is the aetiology of cancer in children?
The aetiology of cancer is complex, usually multifactorial, and not fully understood at present.
What genetic predisposing factors increase the risk of childhood cancer?
• Hereditary cutaneous syndromes (e.g., albinism with squamous cell carcinoma).
• Hereditary neurocutaneous syndromes (e.g., neurofibromatosis: rhabdomyosarcoma, pheochromocytoma, or medullary thyroid cancer).
• Chromosomal abnormalities (e.g., Down Syndrome with 15 times higher incidence of leukaemia).
• Cancer family pattern (e.g., SBLA syndrome: sarcoma, breast, brain, leukaemia, lung, adrenal carcinoma).
• Gastro-intestinal syndromes (e.g., hereditary bowel disease: polyposis coli and adenocarcinoma, ulcerative colitis and carcinoma).
• Hemi-hypertrophy and Beckwith-Wiedemann syndrome (e.g., Wilms tumour, hepatic tumours, and adrenocortical carcinoma).
What percentage of children with Beckwith-Wiedemann syndrome have an associated neoplasm?
As many as 25% of children with Beckwith-Wiedemann syndrome have an associated neoplasm.
What are oncoviruses?
Oncoviruses are viruses that can cause cancer, including human papillomavirus (cervical carcinoma), Epstein–Barr virus (B-cell lymphoma, Kaposi’s sarcoma), herpes virus, hepatitis B and C viruses (hepatocellular carcinoma), and Human T-cell leukaemia virus-1 (T-cell leukaemia).
What is the risk of cancer in children with immunodeficiency syndromes?
• HIV-infected children are at risk for lymphoma and Kaposi sarcoma.
• Transplant patients on immunosuppression have 100 times greater incidence of cancer in their lifetime.
What is the risk of developing a second malignancy in childhood cancer survivors?
Survivors of childhood malignancy have a 20-year cumulative probability of 12% for developing a second malignancy, with the peak occurring 15-19 years after the initial diagnosis.
Why is follow-up important for childhood cancer survivors?
Continuous follow-up is needed for childhood cancer survivors due to the increased risk of developing a second malignancy, with a 12% risk 20 years after the initial diagnosis.
What is the chief characteristic of a malignant tumour?
The chief characteristic of a malignant tumour is the ability of its cells to grow and multiply at a rapid, uncontrollable rate.
When does a malignant tumour become clinically manifest?
A malignant tumour becomes clinically manifest when it reaches a cell size of 1x10^9.
What factors influence the clinical picture of a malignant tumour
The clinical picture depends on the tumour’s anatomical localisation, size, secretory function, and complications.
What happens after a tumour undergoes malignant transformation?
After malignant transformation, the tumour becomes life-threatening, and clumps of cells can leave the parent organ, metastasising to other areas via direct spread, blood vessels, or lymphatic channels.
What is the critical factor affecting the prognosis of a malignant tumour?
The time period between malignant transformation, clinical detection, and the institution of therapy is a critical factor affecting prognosis.
What is essential in the evaluation of a child suspected of having a malignant tumour?
What is essential in the evaluation of a child suspected of having a malignant tumour?
How does cancer in children typically present?
Cancer in children presents in one of three ways, but the specific types of presentation are not detailed in the provided information.
What is mandatory in every child suspected of having a malignant tumour?
A complete personal and family history and physical examination are mandatory.
How does cancer in children typically present?
Cancer in children presents in one of three ways: constitutional symptoms, local findings, and specific age-related presentations.
What are constitutional symptoms in childhood malignancy?
Constitutional symptoms include malignant malaise, pallor, pyrexia of unknown origin, weight loss, lassitude, lethargy, tiredness, irritability, loss of appetite, hypertension, diarrhoea, and aches and pains in the limbs, which may be transient and recurrent.
What should be noted when a mother says, “my child is sick”?
This is a critical statement, indicating possible constitutional symptoms related to malignancy.
What are local findings in childhood cancer?
Local findings include symptoms related to the anatomical presence and size of the tumour or complications, such as gastrointestinal haemorrhage, neurological signs (from brain/spinal cord lesions), haematuria (from Wilms tumour or bladder rhabdomyosarcoma), or endocrine symptoms (from hormone production by the tumour).
Where are many solid tumours in children situated?
Many solid tumours in children are located in the flank area on abdominal examination.
What is the “dictum of sixes” in relation to age and pathology?
• Between 0 and 6 months: Lumbar masses are usually benign, typically in the retroperitoneal space, often related to congenital anomalies like hydronephrosis or renal dysplasia.
• Between 6 months and 6 years: Malignant abdominal tumours are more common, and any abdominal mass is assumed to be malignant until proven otherwise.
• Over 6 years: A broader differential diagnosis is considered, including inflammatory, benign, and malignant conditions.
What should a mass lesion in a child alert a physician to?
A mass lesion should alert the possibility of malignancy, as only a few mass lesions can be considered benign based on location or appearance alone.
What might the sudden and spontaneous appearance of metastases indicate?
It may be the first indication of an underlying malignant process.
Give an example of metastases in childhood cancer.
Neuroblastoma metastases can appear in the orbit, leading to ecchymosis, a pathological fracture, subcutaneous nodules, dyspnoea, and haemoptysis.
What are warning signs of childhood cancer related to pallor and bleeding?
Pallor plus bleeding such as purpura, unexplained bruises, or persistent oozing from the nose or mouth.
What persistent symptoms may indicate childhood cancer?
Fever, apathy, or weight loss that is persistent or unexplained after excluding TB or HIV infection.
What type of bone pain is a warning sign of childhood cancer?
Bone pain that is often poorly localized and may wake the child at night. It may present as a limp in an older child or refusal to walk in a toddler.
What does persistent backache in a child suggest?
Persistent backache may denote a spinal tumour and should always be fully investigated.
When is localized lymphadenopathy a warning sign of cancer?
Lymphadenopathy is concerning when it is persistent and unexplained, with discreet non-tender nodes >2cm in diameter that do not respond to antibiotic therapy within 2 weeks, and all supraclavicular nodes should be biopsied.
What unexplained mass is a warning sign of childhood cancer?
Any unexplained mass in the abdomen, testis, head and neck, or limbs. An abdominal mass in children under 6 years is likely to be malignant.
What neurological signs may indicate childhood cancer?
Unexplained neurological signs like longstanding or early morning headaches (>2 weeks), early morning vomiting, ataxia, or cranial nerve palsies.
What eye signs should raise concern for childhood cancer?
Eye signs such as leukocoria (white spot in the eye or white light reflex), recent onset squint, proptosis, and loss of vision.
What laboratory assays are important in the diagnosis of childhood malignancies?
Laboratory assays include urinalysis, FBC, ESR, LFTs, and tumour markers.
Which tumour markers are associated with Wilms tumour and Rhabdomyosarcoma?
LDH (Lactate dehydrogenase).
Which tumour markers are associated with Neuroblastoma?
LDH and Ferritin.
Which tumour markers are associated with Non-Hodgkin lymphoma?
LDH and uric acid.
Which tumour markers are associated with Germ Cell tumours?
αFP (alpha-fetoprotein) and βHCG (beta-human chorionic gonadotropin).
Which tumour marker is associated with Hepatoblastoma?
αFP (alpha-fetoprotein).
What radiographic investigations are used for diagnosing childhood malignancies?
Chest x-ray, tomography, skeletal survey, sonography, computed tomography (CT), MRI, and radioisotope scanning.
What types of biopsies may be needed for diagnosing and staging childhood malignancies?
Biopsy methods include FNAB (fine needle aspiration biopsy), needle core (Tru-cut), open wedge biopsy, or partial/complete resection of the tumour.
How does early diagnosis affect the outcome of childhood malignancies?
Early diagnosis improves the outcome of childhood malignancies.
Where should children with suspected malignancies be managed?
Children should be managed at a paediatric cancer centre and referred to paediatric oncologists for definitive care.
What has contributed to the improvement in the outcome of childhood malignancies over the last 20 years?
Standardized treatment protocols, multimodal therapy, better understanding of pathophysiology and host immune responses, and advances in surgical, anaesthetic, and intensive care have all contributed.
What are the key prognostic factors in childhood malignancies?
Prognostic factors include age, tumour site and size, extension to local tissues and lymph nodes, presence of metastases, and histological grading and genetics of the tumour.
What should be done when a child is suspected of having a malignancy?
The case must be discussed promptly with a paediatric oncologist to avoid unnecessary delays in diagnosis and referral.
What basic investigations are helpful for diagnosing childhood malignancies?
Basic investigations include chest X-ray, HIV test, full blood count with differential, coagulation screen, ESR, urea, creatinine, electrolytes, LDH, and serum urate.
What additional investigations are helpful for suspected germ cell tumours or Hepatoblastoma?
Tumour markers like α-fetoprotein (αFP) and/or serum s-chorionic gonadotropin (sHCG) levels are helpful and are also used for follow-up to detect recurrence.
What is the ultimate aim of managing childhood malignancies?
The ultimate aim is the restoration of health, free of disease.
What is necessary before starting treatment for childhood malignancies?
A proven diagnosis of malignancy is required before treatment can be given to justify the risks.
What approach is required for the optimal treatment of childhood malignancies?
A multi-disciplinary approach in a specialized centre equipped to treat childhood cancer is necessary.
What is the obligation of the primary physician when managing childhood malignancies?
The primary physician must maintain a high index of suspicion, diagnose early, confirm the diagnosis, and participate in the multidisciplinary team for treatment.
What factors should be evaluated before initiating treatment for a child with cancer?
The general clinical status of the child and whether the child will be able to tolerate various procedures.
What is involved in the assessment of the disease?
A careful, systematic assessment of the extent of the disease.
What is important when planning the approach for treatment?
Determining the time, site of biopsy or surgery, and confirming the diagnosis (favourable/unfavourable histology and stage of disease).
How is treatment for childhood malignancies decided?
Treatment is determined by the management team, based on the sequence and intensity appropriate for the child’s condition.
What is an important aspect of management after treatment?
Careful follow-up is essential
What role does surgery play in the treatment of childhood malignancies?
Surgery helps determine histology, the extent of the disease, and resectability.
What is the purpose of biopsy and staging procedures in surgery?
Biopsy and staging procedures help assess the area most involved with the disease, and the biopsy material must be representative.
How can chemotherapy or radiotherapy impact surgery?
Chemotherapy or radiotherapy can reduce tumour bulk and vascularity, making surgery safer. The histologic assessment after chemotherapy helps indicate the degree of response to treatment.
What is the purpose of second look surgery after chemotherapy?
Second look surgery helps resect previously unresectable tumours, re-evaluate the extent of residual disease, and assess the response to treatment.
What is the role of surgery in palliative care for children with no reasonable chance of cure?
Surgery can improve comfort in the terminal stages, e.g., through procedures like gastrostomy, colostomy, or tracheostomy.
How is surgery used in the treatment of metastatic disease?
Surgery may involve biopsy or excision of metastases in organs like the lung, bone, or other solid organs. Resection of single metastases may be curative.
What role does surgery have in treating complications of malignancies?
Surgery may be needed to manage complications like bowel obstruction.
How does surgery support long-term treatment in childhood cancer patients?
Surgery may provide long-term venous access through ports and tunnelled lines for ongoing treatment.
What is the role of radiotherapy in the treatment of paediatric neoplasms?
Radiotherapy is still used in the therapy of paediatric neoplasms but is restricted due to long-term effects on bone and soft tissue growth.
When is chemotherapy most effective in treating childhood malignancies?
Chemotherapy is most effective during cell division and when the tumour burden is small.
What supportive therapies are used in the treatment of childhood cancer?
Blood, platelets, nutritional support, and psychiatric therapies are used.
What is the goal of detailed follow-up care in childhood cancer patients?
Follow-up allows for the prevention and treatment of complications and timely identification and management of recurrence and second malignancies.
What are acute problems that can arise during cancer therapy?
Acute problems include tumour lysis syndrome, spinal cord compression, superior vena cava syndrome, anaemia and haemorrhage, and neutropenia with opportunistic infections.
What are some long-term sequelae of childhood cancer therapy?
Long-term sequelae include second malignancies, skeletal issues (osteopaenia, osteoporosis, osteogenic sarcoma), cardiac toxicity, lung fibrosis, GIT complications, renal toxicity, endocrine issues (stunted growth, thyroid cancer), infertility, and psycho-social abnormalities.
What is tumour lysis syndrome?
Tumour lysis syndrome is the rapid breakdown of tumour cells, releasing intracellular components and causing hyperkalaemia, hyperuricaemia, hyperphosphataemia, and hypocalcaemia.
What is the risk of second malignancies in childhood cancer survivors?
Survivors have a much higher incidence of developing a second tumour.
How does cancer therapy affect the skeletal system?
Cancer therapy can cause osteopaenia, osteoporosis, and increase the risk of osteogenic sarcoma.
What are the potential long-term effects of cancer therapy on the chest, GIT, and urinary tract?
Long-term effects include cardiac toxicity (e.g., from adriamycin), lung fibrosis (e.g., from radiotherapy or bleomycin), radiation or chemotherapy-induced enteritis, fibrosis, renal toxicity, and cystitis.
How can cancer therapy affect the endocrine system?
Therapy can lead to stunted growth and, less frequently, thyroid cancer.
How does cancer therapy impact reproductive function?
It can lead to infertility.
What psycho-social effects can childhood cancer therapy have?
Significant long-term psycho-social abnormalities can occur in childhood cancer survivors.
What are some examples of solid tumours in childhood?
Examples of solid tumours in childhood include Wilms tumour (nephroblastoma), neuroblastoma, rhabdomyosarcoma, teratomas, soft tissue sarcomas, hepatoblastoma, testicular tumours, brain tumours, bone tumours (osteogenic sarcoma, Ewing’s sarcoma), and retinoblastoma.
What are common haematological malignancies in children?
Common haematological malignancies in children include leukaemia and lymphoma (Hodgkin, non-Hodgkin, including Burkitt lymphoma).
What is Wilms tumour?
Wilms tumour (nephroblastoma) is a kidney cancer that commonly affects children.
What is neuroblastoma?
Neuroblastoma is a cancer that develops from nerve tissue, often affecting the adrenal glands.
What is rhabdomyosarcoma?
Rhabdomyosarcoma is a cancer that forms in the soft tissues, typically involving muscles.
What are teratomas?
Teratomas are tumours composed of different types of tissue, such as hair, muscle, and bone, and can occur in various organs, including the ovaries or testes.
What are soft tissue sarcomas in childhood?
Soft tissue sarcomas are cancers that develop in the connective tissues, such as muscles, tendons, and fat.
What is hepatoblastoma?
Hepatoblastoma is a rare liver cancer typically found in young children.
What are testicular tumours in childhood?
Testicular tumours are cancers that develop in the testes, often seen in adolescent or young children.
What are common brain tumours in children?
Brain tumours in children include gliomas, medulloblastomas, and other types depending on the tumour location.
What are bone tumours in childhood?
Bone tumours include osteogenic sarcoma and Ewing’s sarcoma, both of which typically affect the long bones.
What is retinoblastoma?
Retinoblastoma is a rare eye cancer that typically affects young children and involves the retina.
What is leukaemia in children?
Leukaemia is a cancer of the blood and bone marrow, and it is the most common type of childhood cancer.
What types of lymphoma are seen in childhood?
The types of lymphoma in childhood include Hodgkin lymphoma, non-Hodgkin lymphoma, and Burkitt lymphoma.
What is neuroblastoma?
Neuroblastoma is a common intra-abdominal tumour of neural crest origin, usually found in the adrenal medulla or sympathetic chain. It often presents before the age of two years.
What is the incidence of neuroblastoma?
The incidence of neuroblastoma is approximately 1:10,000.
Can neuroblastoma spontaneously regress?
Yes, neuroblastoma is one of the few human malignancies known to demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular appearance.
How is neuroblastoma stratified in terms of risk?
Neuroblastoma is stratified into three risk categories: low, intermediate, and high risk.
What is the prognosis for low-risk neuroblastoma?
Low-risk neuroblastoma is most common in infants, and good outcomes are often seen with observation alone or surgery.
What is the prognosis for high-risk neuroblastoma?
High-risk neuroblastoma is difficult to treat successfully, even with intensive multi-modal therapies.
Where can neuroblastoma occur?
Neuroblastoma can occur anywhere along the sympathetic nervous system, from the neck to the pelvis, and in the cerebral medulla.
How does neuroblastoma histologically vary?
Neuroblastoma can range from a primitive, highly malignant form to a more benign ganglioneuroma. Maturation of the tumour can occur either spontaneously or after chemotherapy.
How do most neuroblastoma cases present?
More than 50% of neuroblastoma cases present with an abdominal mass. Associated symptoms may include pain, fever, anaemia, weight loss, failure to thrive (FTT), flushing, sweating, or irritability.
What percentage of neuroblastoma patients have hypertension at diagnosis?
25% of neuroblastoma patients have hypertension at diagnosis.
How do more advanced neuroblastomas present?
More advanced neuroblastomas may present with paraplegia due to cord compression, proptosis, or CNS signs.
What is the typical stage of neuroblastoma at diagnosis?
The majority of children with neuroblastoma have advanced or disseminated disease at presentation.
What do neuroblastomas secrete, and how is it measured?
Neuroblastomas secrete catecholamines, which are best measured by spot urine homovanillic acid (HVA).
What biopsy method is preferred for diagnosing neuroblastoma?
Needle (Tru-cut) biopsy is preferred over fine needle aspiration biopsy (FNAB) when neuroblastoma is suspected.
What imaging and diagnostic investigations are used for neuroblastoma?
Investigations include AXR (for calcification), ultrasound (US), CT, or MRI to assess the mass, and a metastatic workup including nuclear medicine bone isotope scans for bone metastases and bone marrow biopsy.
What characterizes Stage I of neuroblastoma?
Stage I involves a tumour confined to the organ or structure of origin.
What is the definition of Stage II neuroblastoma?
Stage II tumours extend beyond the organ or structure of origin but do not cross the midline. Regional lymph nodes on the ipsilateral side may be involved.
What is the criteria for Stage III neuroblastoma?
Stage III tumours extend in continuity and involve both sides of the body (bilaterally).
What defines Stage IV neuroblastoma?
Stage IV involves remote disease, including skeleton, organs, soft tissue, or distant lymph node groups.
What is Stage IVS of neuroblastoma?
Stage IVS involves patients who would otherwise be Stage I or II but have remote disease confined to one or more of the following sites: liver, skin, or bone marrow (without radiographic evidence of bone metastases on a complete skeletal survey).
What is the survival rate for children with Stage I + II neuroblastoma?
Children with Stage I + II neuroblastoma have a ±90% survival rate if the tumour is completely removed.
What is the survival rate for Stage III neuroblastoma?
Stage III neuroblastoma has a 60% survival rate, requiring chemotherapy, surgical excision, and radiotherapy to areas of known residual tumour.
What is the prognosis for Stage IV neuroblastoma?
Stage IV neuroblastoma has an ominous prognosis, with only 15% of patients surviving.
What is unique about Stage IVS neuroblastoma?
Stage IVS neuroblastoma is rare, often occurring in children less than 1 year of age, with a better prognosis than Stage IV. It may even undergo spontaneous regression.
What age at diagnosis is associated with a poorer outcome in neuroblastoma?
Age >15 months at diagnosis is associated with a poorer outcome in neuroblastoma.
What tumour characteristics are indicators of a poorer outcome?
Indicators of a poorer outcome include advanced stage, the extent and site of the tumour (especially adrenal primary), and histological characteristics such as anaplasia.
How does N Myc amplification affect the prognosis of neuroblastoma?
N Myc amplification is an indicator of a poorer outcome in neuroblastoma.
How does ploidy affect the prognosis of neuroblastoma?
Hyperploidy DNA (as opposed to diploid DNA) is associated with a poorer outcome in neuroblastoma.
What is the incidence of Wilms tumour in children?
The incidence of Wilms tumour is 8-10 per 100,000 (1:10,000), with a peak at 3 years of age.
Are males or females more commonly affected by Wilms tumour?
Males and females are equally affected by Wilms tumour.
What genetic factors are associated with Wilms tumour?
Wilms tumour is associated with genetic factors such as mutations in the WT1 and WT2 genes.
What pre-malignant anomaly of renal tissue is linked to Wilms tumour?
Nephrogenic rests or nephroblastomatosis, a pre-malignant anomaly of renal tissue, is linked to Wilms tumour.
What are some clinical associations of Wilms tumour?
Clinical associations include Beckwith-Weideman syndrome, hemihypertrophy, aniridia, familial occurrence, WAGR syndrome (WT1 gene), and Denys-Drash syndrome (point mutations).
What is the most common presentation of Wilms tumour?
An abdominal mass, often found after minor abdominal trauma.
What are some other features of Wilms tumour at presentation?
Haematuria (20%), hypertension (25%), and weight loss.
What imaging modalities are used to diagnose Wilms tumour?
Ultrasound (US) and CT scanning are commonly used.
Why is it important to assess the opposite kidney in Wilms tumour?
To evaluate kidney function and check for bilateral involvement (Stage V disease).
What additional investigations might be done to assess the extent of the tumour?
Abdominal and chest X-ray, CT chest, MRI abdomen, and urine HVAs (if neuroblastoma is a consideration).
What biopsy method is adequate for histology in Wilms tumour?
Fine needle aspiration biopsy (FNAB).
What are the differential diagnoses of Wilms tumour?
• Neuroblastoma
• Hydronephrotic kidney
• Multicystic kidney
• Nephroblastomatosis
• Other renal and retroperitoneal tumours
What is Stage I of Wilms tumour according to the US Children’s Oncology Group?
Tumour limited to the kidney and completely resected.
What characterizes Stage II of Wilms tumour?
Tumour extends beyond the kidney but is completely resected.
What defines Stage III of Wilms tumour?
Residual non-hematogenous tumour confined to the abdomen, including tumour rupture, peritoneal implants, and lymph node involvement.
What is Stage IV of Wilms tumour?
Haematogenous metastases (lung/liver).
What is Stage V of Wilms tumour?
Bilateral renal involvement.
Why is it important to differentiate between favourable and unfavourable histology in Wilms tumour?
Histological type influences prognosis and treatment planning.
What are the two main approaches to the treatment of Wilms tumour in Europe and North America?
• European approach: Neoadjuvant chemotherapy prior to surgery.
• North American approach: Primary surgery when feasible, followed by chemotherapy.
What is the therapeutic approach at our institution for Wilms tumour?
Initial resection followed by chemotherapy. Irresectable tumours are treated with chemotherapy first, followed by resection after tumour shrinkage.
When is radiotherapy (RT) indicated in Wilms tumour treatment?
• Residual tumour after surgical resection.
• Unfavourable histologic findings.
• Pulmonary metastases.
What is the 5-year survival rate for Stage I/II Wilms tumour?
95%
What is the 5-year survival rate for Stage III Wilms tumour?
85%
What is the 5-year survival rate for Stage IV Wilms tumour?
55%
What is the 5-year survival rate for Wilms tumour with unfavourable histology?
50%
What is the age distribution of rhabdomyosarcoma?
Bimodal distribution with peaks at 2-5 years and 15-19 years of age.
What are the common primary sites for rhabdomyosarcoma?
• Head and neck (35%)
• Genitourinary system (26%) (prostate, bladder, vagina)
• Trunk and extremities (19%)
• Biliary tract
• Retroperitoneal areas
What factors affect the prognosis of rhabdomyosarcoma?
Site, histology (embryonal has a good prognosis, alveolar a poor prognosis), and stage.
How is rhabdomyosarcoma diagnosed?
Biopsy of the tumour mass (no tumour markers).
What are the stages of rhabdomyosarcoma according to surgical groups?
• Stage I: Localized disease, completely resected, no lymph node involvement.
• Stage II: Localized or regional disease with total gross resection.
• Stage III: Incomplete resection or biopsy, with residual unresected disease.
• Stage IV: Distant metastatic disease present at diagnosis.
What is the main treatment approach for rhabdomyosarcoma?
• Surgical excision followed by chemotherapy.
• Local irradiation if microscopic residual disease or lymph node involvement.
• For unresectable disease: chemotherapy and RT followed by surgical resection of residual disease.
What age at diagnosis is associated with a poorer outcome in neuroblastoma?
Age >15 months at diagnosis is associated with a poorer outcome in neuroblastoma.
What tumour characteristics are indicators of a poorer outcome?
Indicators of a poorer outcome include advanced stage, the extent and site of the tumour (especially adrenal primary), and histological characteristics such as anaplasia.
How does N Myc amplification affect the prognosis of neuroblastoma?
N Myc amplification is an indicator of a poorer outcome in neuroblastoma.
How does ploidy affect the prognosis of neuroblastoma?
Hyperploidy DNA (as opposed to diploid DNA) is associated with a poorer outcome in neuroblastoma.
What is the incidence of Wilms tumour in children?
The incidence of Wilms tumour is 8-10 per 100,000 (1:10,000), with a peak at 3 years of age.
Are males or females more commonly affected by Wilms tumour?
Males and females are equally affected by Wilms tumour.
What genetic factors are associated with Wilms tumour?
Wilms tumour is associated with genetic factors such as mutations in the WT1 and WT2 genes.
What pre-malignant anomaly of renal tissue is linked to Wilms tumour?
Nephrogenic rests or nephroblastomatosis, a pre-malignant anomaly of renal tissue, is linked to Wilms tumour.
What are some clinical associations of Wilms tumour?
Clinical associations include Beckwith-Weideman syndrome, hemihypertrophy, aniridia, familial occurrence, WAGR syndrome (WT1 gene), and Denys-Drash syndrome (point mutations).
What is the most common presentation of Wilms tumour?
An abdominal mass, often found after minor abdominal trauma.
What are some other features of Wilms tumour at presentation?
Haematuria (20%), hypertension (25%), and weight loss.
What imaging modalities are used to diagnose Wilms tumour?
Ultrasound (US) and CT scanning are commonly used.
Why is it important to assess the opposite kidney in Wilms tumour?
To evaluate kidney function and check for bilateral involvement (Stage V disease)
What additional investigations might be done to assess the extent of the tumour?
Abdominal and chest X-ray, CT chest, MRI abdomen, and urine HVAs (if neuroblastoma is a consideration).
What biopsy method is adequate for histology in Wilms tumour?
Fine needle aspiration biopsy (FNAB)
What are the differential diagnoses of Wilms tumour?
• Neuroblastoma
• Hydronephrotic kidney
• Multicystic kidney
• Nephroblastomatosis
• Other renal and retroperitoneal tumours
What is Stage I of Wilms tumour according to the US Children’s Oncology Group?
Tumour limited to the kidney and completely resected.
What characterizes Stage II of Wilms tumour?
Tumour extends beyond the kidney but is completely resected.
What defines Stage III of Wilms tumour?
Residual non-hematogenous tumour confined to the abdomen, including tumour rupture, peritoneal implants, and lymph node involvement.
What is Stage IV of Wilms tumour?
Haematogenous metastases (lung/liver).
What is Stage V of Wilms tumour?
Bilateral renal involvement
Why is it important to differentiate between favourable and unfavourable histology in Wilms tumour?
Histological type influences prognosis and treatment planning.
What are the two main approaches to the treatment of Wilms tumour in Europe and North America?
• European approach: Neoadjuvant chemotherapy prior to surgery.
• North American approach: Primary surgery when feasible, followed by chemotherapy.
What is the therapeutic approach at our institution for Wilms tumour?
Initial resection followed by chemotherapy. Irresectable tumours are treated with chemotherapy first, followed by resection after tumour shrinkage.
When is radiotherapy (RT) indicated in Wilms tumour treatment?
• Residual tumour after surgical resection.
• Unfavourable histologic findings.
• Pulmonary metastases.
What is the 5-year survival rate for Stage I/II Wilms tumour?
95%
What is the 5-year survival rate for Stage III Wilms tumour?
85%
What is the 5-year survival rate for Stage IV Wilms tumour?
55%
What is the 5-year survival rate for Wilms tumour with unfavourable histology?
50%
What is the age distribution of rhabdomyosarcoma?
Bimodal distribution with peaks at 2-5 years and 15-19 years of age.
What are the common primary sites for rhabdomyosarcoma?
• Head and neck (35%)
• Genitourinary system (26%) (prostate, bladder, vagina)
• Trunk and extremities (19%)
• Biliary tract
• Retroperitoneal areas
What factors affect the prognosis of rhabdomyosarcoma?
Site, histology (embryonal has a good prognosis, alveolar a poor prognosis), and stage.
How is rhabdomyosarcoma diagnosed?
Biopsy of the tumour mass (no tumour markers).
What are the stages of rhabdomyosarcoma according to surgical groups?
• Stage I: Localized disease, completely resected, no lymph node involvement.
• Stage II: Localized or regional disease with total gross resection.
• Stage III: Incomplete resection or biopsy, with residual unresected disease.
• Stage IV: Distant metastatic disease present at diagnosis.
What is the main treatment approach for rhabdomyosarcoma?
• Surgical excision followed by chemotherapy.
• Local irradiation if microscopic residual disease or lymph node involvement.
• For unresectable disease: chemotherapy and RT followed by surgical resection of residual disease.
Head and neck tumours can be managed without surgical ablation.
Head and neck tumours can be managed without surgical ablation.
Sacrococcygeus, ovary, testis, retroperitoneum, and other possible sites.
How do sacrococcygeal teratomas typically present?
As a mass in the pre-sacral area at birth, with potential presacral extension or as an abdominal mass.
What is the gender distribution of sacrococcygeal teratomas?
75% occur in girls.
Why is early excision of sacrococcygeal teratomas important
They are premalignant if not resected early.
What is a good tumour marker for teratomas?
Serum alfa-feto protein (αFP).
What is the treatment for sacrococcygeal teratoma?
Complete excision along with the coccyx
What proportion of childhood hepatic malignancies does hepatoblastoma represent?
Approximately 2/3 of hepatic malignancies in childhood.
At what age does hepatoblastoma most commonly occur?
60% occur under the age of 2
What are the risk factors for hepatoblastoma?
Beckwith-Wiedemann syndrome, hemihypertrophy, and the FAP gene.
What is the role of chemotherapy in hepatoblastoma management?
To reduce tumour size prior to surgical excision.
When is liver transplantation indicated in hepatoblastoma?
When all liver sectors are involved and the tumour is chemo-sensitive.
What are the four most common childhood brain tumors?
Medulloblastoma, Cerebellar astrocytoma, Brainstem glioma, and Ependymoma.
Where are most childhood brain tumors located compared to adults?
In children, 60% are located below the tentorium cerebri.
What are the two main ways brain tumors present clinically?
- Raised intracranial pressure (headaches, vomiting, seizures, papilloedema).
- Localizing signs (ataxia, incoordination of limb movements, 6th cranial nerve palsy).
How long are symptoms typically present before a brain tumor diagnosis is made?
Often for 6 months
What characteristics make lymphadenopathy abnormal in children?
• Presence in neonates
• Supraclavicular or mediastinal position
• Firm and non-tender
• Matted together
• Fixed to skin or underlying tissue
• Enlarging
• More than 2.5 cm in diameter
• Not responding to antibiotics
When should an excisional biopsy be performed in a child with lymphadenopathy?
If there is uncertainty about the gland’s size, localization, or character, or if there is rapid expansion (e.g., suspecting Burkitt lymphoma).
What percentage of childhood cancer does lymphoma account for?
10%
What are the common types of non-Hodgkin lymphoma (NHL) in children?
Burkitt lymphoma, Lymphoblastic lymphoma, and Anaplastic large cell lymphoma.
At what ages do childhood Hodgkin lymphoma and non-Hodgkin lymphoma typically peak?
NHL peaks between 5 and 15 years, while Hodgkin lymphoma peaks in late childhood and early adolescence.
How do we distinguish between stage IV lymphoma and leukemia based on bone marrow blast count?
• Stage IV lymphoma: Bone marrow blast count < 25%
• Leukemia: Bone marrow blast count > 25%
How have survival rates for childhood lymphomas improved with chemotherapy?
• NHL: From 30% in the 1960s to over 80% in the 2000s
• Hodgkin lymphoma (HL): Over 90% survival rates achieved
What percentage of childhood malignancies are neonatal tumors?
2%
What is the most common type of neonatal tumor?
Teratomas and mixed germ cell tumors (>80%).
What are other types of neonatal tumors besides teratomas and germ cell tumors?
• Soft tissue sarcomas (e.g., fibrosarcoma, rhabdomyosarcoma)
• Neuroblastoma
• Renal tumors
• Hepatoblastoma
What is the mainstay of treatment for most neonatal tumors?
Surgery
When is chemotherapy indicated in neonatal tumors?
For size reduction prior to surgery, using reduced doses to minimize toxic side effects.
Why is radiation therapy avoided in neonatal tumors?
Due to risks of bone growth disruption and carcinogenesis.
