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Oncological disorders in children
- 1. ONCOLOGICAL DISORDERS IN CHILDREN •LEUKEMIA • NEUROBLASTOMA • LYMPHOMA • RHABDOMYOSARCOMA
- 2. INTRODUCTION Cancer isa group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body These contrast with benign tumors, which do not spread to other parts of the body. In humans, cancer may be caused by genetics and environmental exposures.
- 3. 1. LEUKEMIA Theterm leukemia refers to cancers of the white blood cells, which are also referred to as leukocytes or WBCs. Leukemia is often described as being either acute (growing quickly) or chronic (growing slowly).
- 4. ACUTE LEUKEMIAS Acutelymphocytic leukemia -This leukemia starts from the lymphoid cells in the bone marrow. Acute myelogenous leukemia -This leukemia starts from the cells that form white blood cells (other than lymphocytes), red blood cells, or platelets. Hybrid or mixed lineage leukemias - The cells have features of both ALL and AML. They are generally treated like ALL and respond to treatment like ALL.
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- 6. CHRONIC LEUKEMIAS JUVENILEMYELOMONOCYTIC LEUKEMIA (JMML) This rare type of leukemia is neither chronic nor acute. It begins from myeloid cells. It occurs most often in young children (under age 4). Symptoms can include pale skin, fever, cough, trouble breathing (due to too many white blood cells in the lungs), and an enlarged spleen and lymph nodes.
- 7. ACUTE LYMPHOBLASTIC LEUKEMIA(ALL) Acute lymphoblastic leukemia (ALL) is a cancer of the white blood cells. ALL affects lymphoid cells. symptoms of ALL include: • Fever; • Fatigue; • Frequent infections; • Swollen or tender lymph nodes, liver, or spleen; • Paleness or pallor; • Easy bleeding or bruising; • Tiny red spots (called petechiae) under the skin; and/or • Bone or joint pain.
- 8. Risk for ChildhoodLeukemia commonly occurs in younger children ages 2 to 8, with a peak incidence at age 4. But it can affect all age groups. Children have a 20% to 25% chance of developing ALL or AML if they have an identical twin who was diagnosed with the illness before age 6. In general, nonidentical twins and other siblings of children with leukemia have two to four times the average risk of developing this illness.
- 9. Diagnosis of Leukemia bone marrow biopsy and aspiration lymph node biopsy lumbar puncture
- 10. Treatment of childrenwith Leukemia chemotherapy phases A. Induction B. consolidation or intensification C. maintenance
- 11. CHEMOTHERAPY vincristine, L-asparaginase,anthracyclines (doxorubicin, daunorubicin), cyclophosphamide, cytarabine (ara-C), and epipodophyllotoxins (etoposide, teniposide). child will also receive a steroid (prednisone or dexamethasone) and vincristine unless he or she is known to be resistant to these medications. Intrathecal chemotherapy will also be given.
- 12. 2. NEUROBLASTOMA Neuroblastomais a tumor arises from primitive neural crest cells that form the adrenal medulla and sympathetic nervous system. - It occurs 1 in 10,000 live births - About 50% of cases occur in the first two years and three-fourths occur by 5 years of age. - It is slightly more common in males than in females
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- 14. Risk factors The exactcause is unknown Some cases of Neuroblastoma have familial incidence autosomal dominant inheritance
- 15. Common sites ofNeuroblastoma Neuroblastoma may find in any site where neural crest cells are located. One-half of these tumors arise in the abdomen, largely in the adrenal gland. Other areas include the thorax, cervical region, nostrils, liver or an intracranial site. Neuroblastoma can spread directly to local tissues, to the regional lymph nodes via the lymphatic, and to the liver, bone marrow and skeleton via the blood circulation
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- 17. CLINICAL FEATURES Abdominalswelling Hepatic enlargement Hemorrhage may due enlarging tumor leads to Anemia Urinary retention or frequency increased ICP Periorbital swelling, ecchymoses and proptosis Respiratory obstruction leads to dyspnea and stridor Spinal cord compression Paralysis fever, lethargy, anorexia, chronic diarrhea & weight loss An infant may manifest sweating &hypertension due to increased catecholamine excretion
- 18. DIAGNOSTIC EVALUATION AbdominalUltrasound and CT abdomen Bone survey or bone scan Bone marrow aspiration and biopsy to rule out infiltration. Intravenous pyelogram. X- ray examination Vanillylmandelic Acid (VMA) and Homovanillic Acid (HVA) to find out elevated catecholamine levels in urine Biopsy and excision of tumor provide the final diagnosis
- 19. PROGNOSIS Prognosis isbased on stage and the disease, location of the tumor, degree of maturation and age at presentation. Children less than one year of age and or with stages 1 and 2 or 4S (do bony involvement) do well as compared to older children or advanced stage disease with primary tumor in retro peritoneum. Good nutrition, normal serum ferritin and neuron-specific enolase are associated with good prognosis.
- 20. MANAGEMENT If thetumor is stage I or stage II, curative resection is performed Surgery after initial chemotherapy may be attempted to improve survival and reduce morbidity Surgery on children whose tumors are stages III and IV is generally limited to biopsy, because there is no improvement in prognosis if the tumor is removed. Chemotherapy or irradiation is used to shrink the mass.
- 21. Chemotherapy Drugs mostcommonly used in children Allopurinol L- Asparaginase and vincristine Bleomycin Cisplatin Cyclophosphamide Cytosine arabinoside Dactinomycin Doxorubicin 6- Mercaptopurine
- 22. 3. LYMPHOMA Cancersthat develop in the lymphatic system are called lymphomas. There are two main types: HODGKIN Lymphoma NON-HODGKIN Lymphoma.
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- 24. HODGKIN LYMPHOMA Hodgkin lymphoma,also known as Hodgkin's disease, most commonly affects children aged 15 and older. Children with this disease typically have abnormal cells called Reed-Sternberg cells in the cancerous lymph node. These cells may develop from a type of lymphocytes known as B cells.
- 25. Incidence About 60children develop Hodgkin lymphoma in each year. It is rare in children under five years and is more common in teenagers and young adults. Boys and girls are equally affected. The exact cause of Hodgkin lymphoma is unknown. it more often in children with immune problems.
- 26. Signs and symptoms fever night sweats weight loss tiredness or lethargy itching cough or breathlessness. painless swelling of one gland or a group of glands, usually in the neck.
- 27. Tests / investigations Bloodtests Chest X-ray Ultrasound scan CT scan Biopsy
- 28. STAGING Stage I:One group of lymph glands is affected, and the lymphoma is only on one side of the diaphragm. Stage II: Two or more groups of lymph glands are affected, and the lymphoma is only on one side of the diaphragm. Stage III: Lymph nodes are affected above and below the diaphragm. The spleen may also be affected. Stage IV: The lymphoma has spread beyond the lymph glands, for example to the lungs, liver or bone marrow.
- 29. TREATMENT OF HODGKINLYMPHOMA Chemotherapy radiotherapy combination of both.
- 30. General side effectsof chemotherapy Bone marrow suppression (myelosuppression) Nausea and vomiting Hair loss
- 31. NON-HODGKIN LYMPHOMA Non-Hodgkinlymphoma (NHL) is a group of blood cancers that includes all types of lymphoma except Hodgkin's lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss and tiredness. About 80 children develop NHL in the UK each year. It can develop at any age and is slightly more common in boys. It is more common in children with weak immune systems.
- 32. Signs and symptom painless swelling of a group of lymph glands. cough breathlessness swelling of the face abdominal (tummy) swelling tiredness or lethargy weight loss poor appetite fever.
- 33. TESTS AND INVESTIGATIONS blood tests chest X-ray ultrasound scan CT scan biopsy bone marrow aspiration lumbar
- 34. STAGING Stage I:one group of lymph glands is affected or there is a single tumour outside of the lymph glands (extra nodal) Stage II: two or more groups of lymph glands are affected or there are two extras nodal tumours, but only on one side of the diaphragm Stage III: lymphoma is present on both sides of the diaphragm, either in two or more groups of lymph glands or two single extra nodal tumours Stage IV: the lymphoma has spread beyond the lymph glands to other organs such as the bone marrow or nervous system.
- 35. Treatment of non-Hodgkinlymphoma Chemotherapy Radiotherapy Surgery
- 36. 4. RHABDOMYOSARCOMA Rhabdomyosarcomais a type of sarcoma. Sarcoma is cancer of soft tissue (such as muscle), connective tissue (such as tendon or cartilage), or bone. Rhabdomyosarcoma usually begins in muscles that are attached to bones and that help the body move. It can arise from muscles in many different areas of the body, most commonly the head and neck, bladder, prostate gland, arms, legs, and vagina.
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- 38. Epidemiology The mostcommon pediatric soft tissue sarcoma, rhabdomyosarcoma, accounts for approximately 3.5% of childhood cancers. Extremity lesions are more likely to occur in older children and to have alveolar histology. Rhabdomyosarcoma occurs with increased frequency in patients with neurofibromatosis and has been associated with maternal breast cancer in the Li-Fraumeni syndrome, suggesting a genetic influence.
- 39. Pathogenesis Rhabdomyosarcoma isthought to arise from the same embryonic mesenchyme as striated skeletal muscle. Based on light microscopic appearance, it belongs to the general category of small round cell tumors that includes Ewing sarcoma, neuroblastoma, and non-Hodgkin lymphoma. Determination of the specific histologic subtype is important in treatment planning and assessment of prognosis.
- 40. SUBTYPES The embryonaltype accounts for about 60% of all cases and has an intermediate prognosis. The botryoid type, a variant of the embryonal form in which tumor cells and an edematous stroma project into a body cavity like a bunch of grapes, is found most often in the vagina, uterus, bladder, nasopharynx, and middle ear.
- 41. Cont. The alveolar typeaccounts for about 25-40% of cases and often is characterized by 2;13 or 1;13 chromosomal translocations. The tumor cells tend to grow in nests that often have cleft like spaces resembling alveoli.
- 42. Clinical Manifestations Amass that may or may not be painful. Symptoms are caused by displacement or obstruction of normal structures. Origin in the nasopharynx may be associated with nasal congestion, mouth breathing, epistaxis, and difficulty with swallowing and chewing. Regional extension into the cranium can produce cranial nerve paralysis, blindness, and signs of increased intracranial pressure with headache and vomiting. When the tumor develops in the face or cheek, there may be swelling, pain, trismus, and, as extension occurs, paralysis of cranial nerves.
- 43. Diagnosis Definitive diagnosisis established by biopsy, microscopic appearance, and results of immunohistochemical stains.. CT or MRI is necessary for evaluation of the primary tumor site. A radionuclide bone scan, chest CT, and bilateral bone marrow aspiration and biopsy. The most critical element of the diagnostic work-up is examination of tumor tissue, which includes the use of special histochemical stains and immunostains. Cytogenetics and molecular genetics Lymph nodes also should be sampled for presence of disease spread.
- 44. Cont. Tumors inthe neck can produce progressive swelling with neurologic symptoms after regional extension. Orbital primary tumors are usually diagnosed early in their course because of associated proptosis, periorbital edema, ptosis, change in visual acuity, and local pain. When the tumor arises in the middle ear, the most common early signs are pain, hearing loss, chronic otorrhea, or a mass in the ear canal; extensions of tumor produce cranial nerve paralysis and signs of an intracranial mass on the involved side. An unremitting croupy cough and progressive stridor can accompany rhabdomyosarcoma of the larynx. Because most of these signs and symptoms also are associated with common childhood conditions, clinicians must be alert to the possibility of tumor
- 45. Treatment Group Itumors are treated with complete local excision followed by chemotherapy to reduce the likelihood of subsequent metastases. Group II tumors (microscopic residual tumor) are treated with surgery followed by local irradiation and systemic chemotherapy. Group III tumors (gross residual tumor) are treated with systemic chemotherapy, irradiation, and possibly surgery. Group IV rhabdomyosarcoma (metastatic) is treated principally with systemic chemotherapy and irradiation.
- 46. Cont. Standard chemotherapeuticagents include vincristine, dactinomycin, and cyclophosphamide (VAC). Radiation therapy. More intensive approaches utilizing multi-agent chemotherapy in conjunction with biologic agents such as insulin-like growth factor inhibitors and other agents are being evaluated in clinical trials for the most high-risk disease.
- 47. Prognosis Prognostic factorsinclude age, stage, histology, and primary site. About 65-70% of patients with incompletely resected tumor also achieve long-term disease- free survival. Patients with disseminated disease have a poor prognosis; only about 50% achieve remission, and fewer than 50% of these are cured. Older children have a poorer prognosis than younger children.
- 48. SUMMARY Type ofcancer that occur most often children are different from those seen in adults. Childhood cancer is uncommon and can manifest with symptoms seen with benign illnesses. Treatment of children with cancer is one of the most complex endeavors in pediatrics. It begins with an absolute requirement for the correct diagnosis.