Alterations in Cellular Growth

Neoplasm Benign Malignant Neoplasm-An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Neoplasms may be benign (not cancer), or malignant (cancer). Also called tumor. Benign-Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant. Malignant- Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.

Variable Etiology. Alterations in cellular growth Variable Etiology Alterations in cellular growth Single or Combination of factors External stimuli Innate immune system & gene abnormalities Chromosomal abnormalities

A protooncogene (normal gene that has potential to transform itself into oncogene) normally regulates cellular growth and development. When altered by a virus or other external cause, it can change to an oncogene (A gene that is a mutated (changed) form of a gene involved in normal cell growth. Oncogenes may cause the growth of cancer cells. Mutations in genes that become oncogenes can be inherited or caused by being exposed to substances in the environment that cause cancer. Tumor-suppressor genes regulate the effects of oncogenes to decrease wildly proliferating cellular growth. A type of gene that makes a protein called a tumor suppressor protein that helps control cell growth. Mutations (changes in DNA) in tumor suppressor genes may lead to cancer.

Incidence of Childhood Cancer 2012 Statistics Estimated 12, 060 new cases in children under the age of 14 Estimated 1, 340 cancer deaths to occur in children under age 14 (1/3 of those deaths from leukemia) Cancer Facts & Figures 2012 American Cancer Society Cancer is the second leading cause of death in children. Who knows what the leading cause of death is? Accidents

Peak incidence of tumors vary by age-here is a breakdown by ages-Under the age of 14- leukemia most prevalent

Prevention. Smoking Prevention. Limit exposure to sun Prevention Smoking Prevention Limit exposure to sun Testicular self exams Breast self exams Pap smears Really no know ways to prevent childhood cancers but we can teach children and parents about prevention for adult type cancers Lung CA leading cause of death from cancer in adults Malignant melanoma leading cause from diseases of the skin Who is at higher risk for skin cancer? Children with light colored eyes, fair complexion, those with freckles associated with sunburn, those who sunburn easily, living by equator

Cardinal Symptoms of Cancer in Children. Unusual mass or swelling Cardinal Symptoms of Cancer in Children Unusual mass or swelling Unexplained paleness & loss of energy Sudden tendency to bruise Persistent, localized pain or limping Prolonged, unexplained fever/illness Frequent headaches, often with vomiting Sudden eye or vision changes Excessive, rapid weight loss Fever and pain are common symptoms seen in pediatrics-so it’s understandable how diagnosis could be delayed if these signs/symptoms are brushed off as minor ailments

Laboratory Tests. Complete blood count (CBC). Serum chemistries Laboratory Tests Complete blood count (CBC) Serum chemistries Liver function tests Coagulation studies Urinalysis What types of results may you expect to see in patients with cancer? Leukemia-low hemoglobin, low platelet count, low, normal or high white blood counts.

Diagnostic Tests. Lumbar puncture. Bone marrow aspiration Diagnostic Tests Lumbar puncture Bone marrow aspiration Bone marrow biopsy Radiographic examinations CT MRI Ultrasound Biopsy of tumor LP- looking for infection, cancer cells Bone marrow aspiration-examine the bone marrow to determine type of leukemia, biopsy Intrathecal-Describes the fluid-filled space between the thin layers of tissue that cover the brain and spinal cord. Drugs can be injected into the fluid or a sample of the fluid can be removed for testing.

Treatment Modalities. Surgery. Chemotherapy. Radiotherapy. Biotherapy Treatment Modalities Surgery Chemotherapy Radiotherapy Biotherapy Hematopoietic stem cell transplant Palliative Care Treatments may be used alone or in combination Surgery-to obtain biopsy and remove tumor Chemo-causes cell destruction to kill cancer cells-via various mechanism-mainly by interfering with function or production of nucleic acids, DNA & RNA Radiation-curative or palliative-to shrink size of tumor Biotherapy-Treatment to boost or restore the ability of the immune system to fight cancer, Also used to lessen certain side effects that may be caused by some cancer treatments. Agents used in biotherapy include monoclonal antibodies. These agents may also have a direct antitumor effect. Also called biological response modifier therapy, biological therapy, BRM therapy, and immunotherapy. HSCT- prior to transplant administer high dose chemotherapy/often with radiotherapy to kill all cancer cells. Once the body is free of cancer cells & immunosuppressed- the stem cells are transfused by IV infusion. The newly transfused stem cells begin to produce non-cancerous blood cells. Palliative Care-Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual.

Common Forms of Childhood Cancer. Leukemia. Lymphoma Common Forms of Childhood Cancer Leukemia Lymphoma Brain & CNS tumors Bone Solid tumors Soft tissue tumors Luekemia- Acute Lymphoctyic Leukemia & Acute Myelogenous Leukemia (Acute nonlymphoblastic leukemia) Lymphoma- Non-Hodgkin & Hodgkin Brain & CNS Tumors- gliomas, medulloblastomas, cerebellar astocyoma, ependymoma Bone- Osteosarcoma & Ewing sarcoma Soft tissue sarcomas-A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body- like Rhabdomyocarcoma Solid tumors are tumors that do no contain cysts or liquids like Wilms tumor

Leukemia. Most common form of childhood. cancer Leukemia Most common form of childhood cancer Peak age is 4 with more boys than girls affected Classified by type of WBC that becomes neoplastic & immaturity of neoplastic cell Leukemia is broad term given to cancers of the bone marrow and lymphatic system

Normally, the bone marrow makes blood stem cells (immature cells) that become mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells: Red blood cells that carry oxygen and other substances to all tissues of the body. Platelets that form blood clots to stop bleeding. White blood cells that fight infection and disease. A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells): B lymphocytes that make antibodies to help fight infection. T lymphocytes that help B lymphocytes make the antibodies that help fight infection. Natural killer cells that attack cancer cells and viruses.

Leukemia Unrestricted proliferation of immature white blood cells Leukemia Unrestricted proliferation of immature white blood cells Infiltration & replacement of body tissues with nonfunctioning leukemic cells-most effected are bone marrow, spleen, liver CNS at risk-can have leukemic infiltration of the meninges Etiology unknown-sometimes genetics can be implicated Trisomy 21 (Down Syndrome) Meninges-3 membranes covering the brain and spinal cord (dura mater, arachnoid & pia mater) In leukemia there is an overproduction of WBC-in acute phase there is increased immature cells-these cells depress bone marrow production of formed elements by competing for and stealing nutrients need for metabolism- So think about this- what types of signs/symptoms would we see if this is what is going on in the body Bone marrow is depressed- anemia form decreased red blood cells, infection form neutropenia, bleeding from decreased platelets. fever, pallor, fatigue, anorexia, hemorrhage(usually petechiae) Invasion of bone marrow-increased pressure- weakens bone- bone & joint pain, fractures Other organs-liver, spleen, lymph glands- enlarged-hepatosplenomegaly, lymphadenopathy If CNS involvement- most common effect is increased ICP-headache, vomiting, irritability, lethargy, coma

Acute Lymphoctyic Leukemia Most common form of cancer Better Prognosis-current survival rates about 80 % In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes.

Acute Myelogenous Leukemia Poorer prognosis Current cure rates approximately 40-50%

Diagnostic Evaluation. CBC. Bone Marrow Aspiration or Biopsy Diagnostic Evaluation CBC Bone Marrow Aspiration or Biopsy (definitive test) Lumbar puncture What types of results would we expect to see on the CBC in Leukemia- low platelets, low hb/hct, neutropenia, blasts Normal bone marrow < 5% blasts Leukemia marrow 60-100% Why would they do a LP-one dx confirmed-determine if there is any CNS involvement

Treatment. Chemotherapy-IV & Intrathecal. Radiation –Cranial Treatment Chemotherapy-IV & Intrathecal Radiation –Cranial Bone marrow transplant Because there is a risk of CNS invasion, all children receive CNS prophylactic with intrathecal chemo Cranial therapy is sometimes used for resistant CNS disease Bone marrow transplant may be recommended for some pt with ALL & AML

Phases of Chemotherapy Phases of Chemotherapy Induction: achieves complete remission or disappearance of leukemic cells Intensification: further decreases number of leukemic cells Maintenance: preserve remission & reduce number of leukemic cells Reinduction after relapse

Nursing Care Priorities. Manage symptoms Nursing Care Priorities Manage symptoms Prevent/ treat side effects chemo Neutropenic precautions Protect from injury/trauma due to anemia & low platelet count Nutrition Communication Manage symptoms like pain, fever Side effects- nausea/vomiting, mucosal irritation (What to avoid-lemon glycerin swabs/acidic foods/frequent rinses saline Neutropenic precautions- isolation, transmission precautions, careful handwashing, avoidance of sick family Nutrition- high protein, high calorie foods monitor for weight loss Communication-accurate, age appropriate be careful with your words

Lymphomas Hodgkin disease Originates in lymphoid system-primarily involves lymph nodes Can metastasize to spleen, liver, bone marrow, lungs, mediastinum Excellent prognosis for localized disease Mostly adolescents affected Lymphomas re divided according to tissue type and extent of disease(staging) Mediastinum-mass of tissues & organs separating the lungs, including the heart and its vessels, trachea/esophagus, thymus and lymph nodes 90% 10 year survival rate

Signs & Symptoms Painless enlargement of lymph nodes (most commonly in supraclavicular or cervical area) Mediastinal lymph nodes-persistent dry cough Systematic symptoms-fevers, night sweats, anorexia, nausea, weight loss, pruritus

Diagnostic Evaluation Blood work-CBC, Uric acid levels, Liver function tests, C-reactive protein, alkaline phosphatase, UA CXR CT of neck, chest, abdomen, pelvis Gallium scan Bone scan (looking for metastasis) Lymph node biopsy

Treatment Chemotherapy Radiation

Lymphoma. Non-Hodgkin. Diffuse disease. Dissemination occurs earlier & Lymphoma Non-Hodgkin Diffuse disease Dissemination occurs earlier & more rapidly Mediastinal & invasion of meninges are typical

Diagnostic Evaluation. Surgical biopsy. Bone Marrow Aspiration. CXR Diagnostic Evaluation Surgical biopsy Bone Marrow Aspiration CXR CT- lungs, abdomen Lumbar puncture Blood work Why would they do an LP

Treatment. Treatment tailored to staging. Chemotherapy. Radiation Treatment Treatment tailored to staging Chemotherapy Radiation CNS prophylaxis with intrathecal chemo

Brain Tumors About 1500 children under 15 are diagnosed annually as having brain/CNS tumors. Most common brain tumors in children are Medulloblastoma, cerebral astrocytoma, ependymoma and brainstem glioma Most common CA in children next to leukemia Infratentorial-occur in posterior part of brain mainly the cerebellum or brainstem Supratentorial-lie within the midbrain structures

Signs & Symptoms. Increased signs ICP. Personality changes. Seizures Signs & Symptoms Increased signs ICP Personality changes Seizures Ataxia Visual disturbances Delayed or precocious puberty & growth failures

Diagnostic Evaluation MRI CT Biopsy done during surgery

Treatment Surgery Radiation Chemotherapy

Nursing Care Priorities. Nutrition. Frequent assessment of mental Nursing Care Priorities Nutrition Frequent assessment of mental status I & O/ Fluid regulation Head positioning post op Monitor surgical site Prepare family Promote return to optimum functioning

Neuroblastoma Tumors originate in cells that normally give rise to the adrenal medulla and sympathetic nervous system Primary site is within abdomen but also could include head/neck, chest, and pelvis Median age of diagnosis is 19 months. Slightly more prevalent in males.

Signs & Symptoms. Depend on stage/location Signs & Symptoms Depend on stage/location Abdominal tumors-firm mass in abdomen-crosses midline Urinary frequency/retention Widespread metastasis- pallor, weakness, irritability, anorexia, weight loss

Diagnostic Evaluation. CT of abdomen, pelvis, chest Diagnostic Evaluation CT of abdomen, pelvis, chest Bone scan /MIBG scan Bone marrow aspirate & biopsy Urinalysis to evaluate for excretion of catecholamines MIBG-metaiodobenzylguanidine

Treatments Surgery Chemotherapy Radiation HSCT

Bone Tumors Osteosarcoma. Most common bone cancer in Bone Tumors Osteosarcoma Most common bone cancer in children affects metaphysis Ewing Sarcoma Tumor originates in shaft of bones (pelvis, femur, tibia, fibia, humerus, ulna, vertebra, scapula, ribs, skull) MetaphysisThe metaphysis is the wider portion of a long bone adjacent to the epiphyseal plate.[1] This is this part of the bone that grows during childhood; as it grows, it ossifies near the diaphysis and the epiphyses. At roughly 18 to 25 years of age, the metaphysis stops growing altogether and completely ossifies into solid bone. Treatment includes surgery and chemo Surgery consists of surgical biopsy & either limb salvage or amputation in osteosarcoma Treatment for Ewing sarcoma- for majority is radiation/chemo

Wilms Tumor (Nephroblastoma). Most common kidney tumor of. childhood Wilms Tumor (Nephroblastoma) Most common kidney tumor of childhood 80% diagnosed under age 5 Peak incidence 3-4 years

Signs & Symptoms. Painless swelling/mass in Signs & Symptoms Painless swelling/mass in abdomen-confined to one side Hematuria Anemia Hypertension

Diagnostic Evaluation. Abdominal ultrasound. CT/MRI abdomen Diagnostic Evaluation Abdominal ultrasound CT/MRI abdomen CT chest to look mets to lung Blood work-CBC, Chemistries UA NO ABDOMINAL PALPATIONS Great care is taken to keep tumor intact-if rupture can seed cancer throughout abdomen, lymph channel and bloodstream

Treatment Treatment based on staging Surgery Chemotherapy Radiation Children with localized tumor (stage 1 & II) 90% survial rate

Rhabdomyosacroma. Most common soft tissue sarcoma. in children Rhabdomyosacroma Most common soft tissue sarcoma in children Skeletal muscle is most everywhere so tumors can occur throughout body Most common site head/neck especially the orbit

Signs & Symptoms Initial signs/symptoms related to site of tumor and compression of organs Many signs & symptoms vague frequently passes off as common childhood illness- Primary tumor site rarely identified

Here is a child with rhabdmyosacroma of the orbit

Diagnostic Evaluation. Careful history and physical. CT/MRI. Bone scan Diagnostic Evaluation Careful history and physical CT/MRI Bone scan Bone marrow aspirate & biopsy Lumbar puncture Biopsy or surgical resection of tumor Tumor staging is done

Treatment Complete removal of tumor if possible Chemotherapy Radiation

Retinoblastoma. Arises from retina Retinoblastoma Arises from retina Hereditary & Nonhereditary Signs/Symptoms White pupil-Leukocoria Treatment Radiation, Chemotherapy, Removal of eye in advanced disease The book has a nice picture with an infant with prosthetic eye

Cancer Survival. Long-term effects of chemotherapy, Cancer Survival Long-term effects of chemotherapy, radiation, and surgery Neurocognitive impairment Endocrinopathy Second malignancy Organ dysfunction-kidney/liver failure Frequent follow up=physical, psychological, developmental and cognitive