HISTIOCYTOSIS

a general term for a group of syndromes that involve an abnormal accumulation and infiltration of histiocytes (monocytes, dendritic cells & macrophages)

ASSESSMENT Sign and Symptoms

Children: Abdominal pain Bone pain (possibly) Delayed puberty Ear drainage that continues long-term Eyes that appear t stick out (protrude) more and more Irritability Failure to thrive Frequent urination Headache, dizziness, fever Jaundice Mental deterioration Rash (petechiae or purpura) Swollen lymph glands Thirst, vomiting, weight loss Adult: Bone pain Chest pain Cough Fever General discomfort, uneasiness, malaise Irritability Increased urine output Rash Shortness of breath Thirst Weight loss

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Medical Management
Predisposing Factors: Age- 1-15 y/o, peaks at 1-3 y/o Gender- male (most common) Precipitating Factors: Viral infection Cellular & Immune dysfunction (lymphocytes & cytokines) Neoplastic mechanism & genetic factors Cellular adhesion molecules Chemotherapy Cyclophosphamide Etoposide Methotrexate Vinblastine Radiotherapy Antibiotics & Corticosteroids Breathing support (eg. mechanical ventilation) Hormone replacement theory Physical Therapy Special shampoos for scalp problem Supportive care

Stimulate differentiation of group of specialized cells

Dendritic cells

Langerhans cell cells Responsible for B & T cell activation Creation of permissive immunosurveillance system

Nursing Management
Maintain a link between patients, families, and members of the multidisciplinary team. Be able to communicate and provide written document to specialists regarding appropriate pathologic diagnosis, clinical laboratory and radiographic studies. Provide psychosocial support to children and their families on coping with the diagnosis of Histiocytosis. Collaborate with specialist in a multidisciplinary setting to reduce the need for the family for multiple clinic visits. Promote patient and family education. Emphasize the need for long-term care by multidisciplinary team especially those with extensive multisystem disease or treated with systemic chemotherapy.

Enhance antigen presenting capacity to B & T cells

Phagocytize antigen (immature state)

Diagnostic Exams
Induction of immune response Tests in children: 1. Bone XRay Reveals a punched out look of the skull Find out how many bones are affected 2. Bone Marrow & Skin Biopsy Presence of Langerhans cells 3. CBC ct.Hgb, WBC, Other tests: platelet ct Tests in adult: 1. Bronchoscopy with Biopsy Reveals presence of pulmonary histiocytosis 2. Chest XRay Ruke out infection and presence of nodular infection 3. Pulmonary function test

Failure of immune response to recognize tumor

Increased expression of inhibitory proteins capable of inhibiting death receptor-mediated apoptosis

Increased tumor growth proliferation

Move into tissues (epidermal layer)

Prognosis
Increased proliferation of dendritic cells Upregulates expression of MHC & co-stimulatory receptors Results in granulomatous lesions 80% of children who develop LCH will recover from it. A small number of children may develop side effects such as reduced growth impairment, infertility, cardiac and pulmonary abnormalities and secondary malignancies many years later because the treatment they have received.

Other tests: CT & MRI- show detailed, anatomic pattern of involvement and can help in staging the disease

B & T cell activation, increased secretion of cytokines

THYMOMA

A neoplasm of thymic epithelial cells

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Chest pain Dyspnea Dysphagia Cough Bleeding Muscle weakness Paresthesia Fever and malaise secondary to infection Predisposing Factors: Age- 40 y/o above Gender- both men & women are at risk Precipitating Factors: Presence of paraneoplastic syndromes Myasthenia Gravis Lambert-Eathon Myasthenic Syndrome Subacute sensory neuropathy Red cell aplasia Immunodeficiency

Medical Management
Surgery Thymectomy Radiotherapy Chemotherapy Cisplatin, Epirubicin, Etoposide- 3 courses repeated q3wk before and after surgery Cisplatin, Doxorubicin, Cyclophosphamide- 2-4 cycles q3wk ff by radiation Corticosteroid Prednisone (Deltasone) Immunoglobulin(Ig) therapy Prophylactic antibiotics

Dysregulation of lymphocyte negative & positive selection process

Diagnostic Exams
1. Lab Studies CBC ct.Hgb, WBC, platelet ct Quantitative Immunoglobulins (Igs)- reveals panhypogammaglobulinemia Immunophenotypic analysis of peripheral blood lymphocytes- shows absent or very low B cell ct & absolute CD4+ T-cell no. 2. Imaging studies Chest Radiography- mediastinal widening on posteroanterior (PA)views or retrosternal opacification on lateral views Chest CT scan or MRI- reveals the morphology of the mass and detect fat invasion, cysts or necrosis 3. Biopsy Fine-needle aspiration or core biopsy 4. Histologic findings characterized by mixture of epithelial and lymphoid tissue and usually encapsulated

Abnormal proliferation of thymic epithelial cells

Autoimmunity

Immunodeficiency

Nursing Management
Formation of autoantibodies to synaptic receptors at the neuromuscular junction and various neuromuscular antigens Failure of the T lymphocytes to mature Provide supportive care such as administering prophylactic antibiotics, corticosteroid and IVIG as prescribed. Promote patient and family education regarding treatment procedures, diagnostic results and prognosis of the disease. Provide psychosocial support to patient and his/her family on coping with the diagnosis of Thymoma. Collaborate with specialist in a multidisciplinary setting. Emphasize that recurrence can occur after resection and a long-term monitoring for complications such as compression syndrome.

Encapsulated tumor spreads locally

Hypogammaglobulinemia/ Agammaglobulinemia

May invade surrounding fatty tissue, mediastinal pleura, pericardium, great vessels, lungs and spread to lymph nodes & blood

Skeletal muscle weakness (Myasthenia Gravis), hyperactivity of peripheral motor nerves, muscle twitching, and muscle cramps (Neuromyotonia)

Immunosupression

Prognosis
Patients with invasive metastatic tumor, tracheal or vascular compression, age younger than 30 years, epithelial or mixed histology, and tumor size of more than 8 cm have poor prognosis. Recurrence after resection is possible. Presence of Myasthenia Gravis is thought to have a favorable prognosis.

GERM CELL NEOPLASM

A malignant or non-malignant (benign) neoplasm that are comprised mostly of germ cells that arise to the formation of male & female reproductive organs

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Ovarian tumor Abdominal swelling Testicular tumor Presence of mass usually associated with pain Mediastnal tumor Chest pain, breathing problems, cough & fever Presacral tumor Mass in the lower abdomen or buttocks Difficulty in passing urine Difficulty in bowel movement Pineal gland tumor Headache, N/V, memory loss, lethargy, difficulty walking, inability to look upward, uncontrolled eye movements or double vision and puberty at an abnormally young age Sacrococcygeal tumor Constipation, leg weakness, incontinence

Medical Management
Predisposing Factors: Age- 20 y/o below Gender- both men & women are at risk Precipitating Factors: Congenital defects CNS and GU tract malformation Males with cryptorchidism Structural chromosome abnormalities (chromosome #12) Extra or missing sex chromosomes Klinefelters syndrome (in males) Surgery Gross total resection of tumor Radiotherapy Chemotherapy Cisplatin, Etoposide and Bleomycin BMT Hormonal replacement (if necessary) Supportive care (for the effects of treatment) Prophylactic antibiotics

Abnormal migration of germ cells during embryogenesis

Widespread distribution of germ cells to multiple sites

Uncontrolled cell growth and tumor formation

Nursing Management
Provide supportive care such as administering prophylactic antibiotics, nutritional supplements or feeding via enteral tube or parenteral. Promote patient and family education regarding treatment procedures, diagnostic results and prognosis of the disease. Provide psychosocial support to patient and his/her family on coping with the disease. Emphasize the possibility of impotence in older clients and address issues about sexual identity. Monitor all patients with sacrococcygeal teratoma with serial rectal exams and serum markers q3mos for the first 3 years to detect signs of recurrence.

Diagnostic Exams
1. Lab Studies Alpha-Feto Protein (AFP) level- elevated Human Chorionic Gonadotropin (HCG) level- elevated Lactate Dehydrogenase level- elevated 2. Imaging studies Chest Radiography- used to detect metastasis Abdominal and Pelvic CT scan & MRI- essential for staging abdominal and pelvic tumors Bone scan- detect bone metastasis 3. Biopsy

Misplacement of germ cells to other location in the body (eg. mediastinum, pelvis, head, neck)

Incomplete or abnormal development of reproductive system

Extragonadal germ cell tumor

Germ cells fail to follow midline path through the body

Prognosis
Prognosis improves over time and when diagnosis and treatment is done early. Generally, the younger the patient is, the better their chances of survival.

Failure of ovarian cells to descend into the pelvis and testicular cells into the scrotal sac

Attaches to surface of adjacent organs

COLON CANCER
a malignancy in the cells lining the bowel wall or develop as adenomatous polyps in the colon or rectum

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Change in bowel habits Passage of blood in stool Unexplained anemia, anorexia, weight loss and fatigue Abnormal stools Ascending colon tumor: diarrhea Descending colon tumor: constipation or some diarrhea, flat ribbon-like stool caused by partial obstruction Rectal tumor: alternating constipation and diarrhea Guarding or abdominal distention, abdominal mass (late sign) Cachexia (late sign) Predisposing Factors: Age- 50 y/o above Race- African Americans (more at risk than whites) Gender- both men & women are at risk Family history of colon cancer or polyps (FAP) Precipitating Factors: Previous colon cancer or adenomatous polyps History of inflammatory bowel disease (IBD) High-fat, High protein (high intake of beef), low fiber diet Genital cancer (endometrial, ovarian or breast cancer)

Medical Management
Surgery Colostomy Ileostomy Radiotherapy Chemotherapy 5-fluorouracil and levamisole regimen IV fluids and nasogastric suction- for signs of intestinal obstruction Blood component therapy- for active bleeding

Mutation of APC (Adenomatous Polyposis gene)

Abnormal DNA methylation

Deficient DNA mismatch repair system

Diagnostic Exams
1. Lab Studies 4. Fecal occult blood test- shows presence of blood in the stool 2. Imaging studies Colonoscopy with biopsy- reveals presence of mass in the colon or rectum with elevation of carcinoembryonic antigen (CEA) on cytologic findings Barium enema Proctosigmoidoscopy Activation of oncogene (C-myc, KRAS) Deactivation of tumor suppressor genes (p53)

Nursing Management
Monitor for signs of complication which include bowel perforation with peritonitis, abscess or fistula formation, hemorrhage (signs of shock), and complete intestinal obstruction. Monitor for signs of bowel perforation which include low blood pressure, rapid and weak pulse, distended abdomen and elevated temperature. Monitor for signs of intestinal obstruction which include vomiting (may be fecal contents), pain, constipation, and abdominal distention. Provide comfort measures. Auscultate bowel sounds. Note that in intestinal obstruction, a hyperactive bowel sound may be heard first (early sign) then hypoactive bowel sounds as obstruction progresses. Prepare patient for radiation preoperatively and postoperatively. Prepare patient for chemotherapy postoperatively.

Proliferation of cancer cells to the epithelial lining of the intestine

Prognosis
Patients who were diagnosed early and undergo prompt treatment have 5-year survival rate of 90%. Survival rates after late diagnosis are very low.

May invade and extend to the surrounding tissues

Metastasis (most often to the liver)

ACUTE MYELOID LEUKEMIA (AML)

A malignant disease of the bone marrow in which the hematopoietic precursors are arrested in an early stage of development

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Weakness, fatigue Hypotension, tachycardia, tachypnea Fever Ecchymoses, petechiae Hepatosplenomegaly Bone pain Abdominal pain Predisposing Factors: Age- 60 y/o above Gender- more common in men Race- more common in whites Family history Precipitating Factors: Exposure to radiation and certain chemicals (benzene and alkylating) Genetic abnormalities Congenital disorders- Down syndrome, Fanconi anemia

Medical Management
Induction Therapy High dose of Cytarabine (CYtosar), Daunorobucin (Cerubidine), Mitoxantrone (Novantrone) or Idarubicin (Idamycin) Consolidation therapy One cycle of treatment of chemo agents at lower dosage BMT or PBSCT Supportive care PRBCs and platelets Antimicrobial therapy (antibacterial or antifungal)Amphotericin, ciprofloxacin, Fluconezole, Acyclovir Allopurinol (Zyloprim, Aloprim) Granulocytic stimulating growth factors (G-CSF)

Diagnostic Exams
Blood Studies 5. CBC ct.Hgb, , ct. 6. Coagulation studies-

Somatic mutation in the DNA platelet ct, high or normal WBC PTT, Fibrinogen

Abnormal hematopoiesis

Impaired cell proliferation control

Myeloblast abnormality

7. Blood chemistry profileuric acid, lactate dehydrogenase (LDH) Imaging studies UTZ- shows enlargement of liver and spleen Biopsy Bone marrow aspiration Histologic findings Presence of 20% blasts

Uncontrolled growth of immature clone of cells

Arrest cell differentiation

Nursing Management
Freeze cell maturation Initiate neutropenic precautions. Initiate bleeding precautions. Provide optimal nutrition by giving high-caloric foods and performing oral care regularly. Provide comfort measures to relieve pain and discomfort. Advise patient to limit physical exertion to prevent fatigue. Maintain fluid and electrolyte balance by monitoring electrolyte and ABG values as well as fluid status. Provide psychosocial support to the patient and family. Promote clients self-care by providing him/her with teachings about certain procedures. Encourage spiritual well-being.

Accumulation in the bone marrow

Decreased production of normal blood cells

Prognosis
Patients who are older or have more undifferentiated form of AML have poor prognosis. Patients having leukemia stemming from preexisting MDS have poor prognosis. Patients who previously received alkylating agents for cancer survive an average of <1 yr. Patients who are younger may survive for 5 years or more after diagnosis. Patients receiving supportive care usually surve ,1 yr, dying of infection and bleeding Spillage of abnormal cells in the bloodstream

Organ infiltration (spleen, liver, CNS)

CHRONIC MYELOID LEUKEMIA (CML)

A myeloproliferative disorder characterized by increased proliferation of the granulocytic cell line without the loss of their capacity to differentiate

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Sign and Symptoms

Shortness of breath Confusion Hepatosplenomegaly Abdominal pain Malaise, anorexia, weight loss Bleeding tendencies

Medical Management Causes/Risk Factors

Tyrosine Kinase Inhibitor Imatinib mesylate (Gleevec) daily Interferon-alfa (Referon-A) & cytosine Oral chemotherapy agents Hydroxyurea (Hydrea) Busulfan (Meleran) Leukapharesis BMT or PBSCT Induction therapy

Predisposing Factors: Age- 40- 60 y/o & above

Precipitating Factors: Chromosomal translocation Exposure to radiation or chemicals

Diagnostic Exams
1. Lab Studies CBC ct.RBC, WBC, platelet ct 2. Imaging studies UTZ- shows enlargement of the spleen and liver 3. Biopsy

Translocation of BCR gene on chromosome 22 (Ph1) to ABL gene on chromosome 9

Fusion of these two genes (BCR-ABL gene)

Nursing Management
Initiate neutropenic precautions. Initiate bleeding precautions. Provide optimal nutrition by giving high-caloric foods and performing oral care regularly. Provide comfort measures to relieve pain and discomfort. Advise patient to limit physical exertion to prevent fatigue. Maintain fluid and electrolyte balance by monitoring electrolyte and ABG values as well as fluid status. Provide psychosocial support to the patient and family. Promote clients self-care by providing him/her with teachings about certain procedures. Encourage spiritual well-being.

Release of tyrosine kinase protein

Rapid division and proliferation of leukocytes

Prognosis
Patients diagnosed with CML in chronic phase may have 3-5 years survival. Patients whose diagnosis transforms to acute phase may only have few months survival.

ACUTE LYMPHOCYTIC LEUKEMIA (ALL) A form of leukemia or cancer of the blood characterized by increased lymphoblasts

ASSESSMENT Sign and Symptoms

Weakness, fatigue Hypotension, tachycardia, tachypnea Fever Ecchymoses, petechiae Hepatosplenomegaly Bone pain Abdominal pain Headache Vomiting

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Predisposing Factors: Age- 4 y/o & below Gender- more common in boys

Precipitating Factors: HTLV-1 virus Exposure to radiation or chemicals Family history of leukemia Genetic abnormalities Chromosomal translocation Somatic mutation in the DNA

Medical Management
Prophylactic cranial irradiation or intrathecal chemotherapy Methotrexate Induction therapy Corticosteroids & vinca alkaloids Tyrosine Kinase Inhibitor Imatinib mesylate (Gleevec) Monoclonal antibody Alemtuzumab (Campath) BMT or PBSCT

Diagnostic Exams
1. Lab Studies CBC ct.RBC ct, or WBC ct, platelet ct 2. Imaging studies UTZ- shows enlargement of the spleen and liver 3. Bone marrow biopsy

Activate oncogen/ deactivate tumor suppressor gene

Nursing Management
Malignant transformation of lymphoid stem cells Initiate neutropenic precautions. Initiate bleeding precautions. Provide optimal nutrition by giving high-caloric foods and performing oral care regularly. Provide comfort measures to relieve pain and discomfort. Advise patient to limit physical exertion to prevent fatigue. Maintain fluid and electrolyte balance by monitoring electrolyte and ABG values as well as fluid status. Provide psychosocial support to the patient and family. Promote clients self-care by providing him/her with teachings about certain procedures. Encourage spiritual well-being.

Prognosis
Age. Younger patients (especially those younger than age 50) have a better prognosis than older patients. Initial white blood cell (WBC) count. People diagnosed with a WBC count below 50,000 tend to do better than people with higher WBC counts. ALL subtype. The subtype of T cell or B cell affects prognosis. For example, patients with T-cell ALL tend to have a better prognosis than those with mature B-cell ALL (Burkitt leukemia.) Chromosome translocations. People who have Philadelphia chromosome-positive ALL tend to have a poorer prognosis, although new treatments are helping many of these patients achieve remission. Response to chemotherapy. Patients who achieve complete remission (disappearance of signs and symptoms of cancer) within 4 - 5 weeks of starting treatment tend to have a better prognosis than those who take longer. Patients who do not achieve remission at any time have a poor prognosis. Evidence of minimal residual disease (presence of leukemia cells in the bone marrow) may also affect prognosis. Other factors, such as central nervous system involvement or recurrence, may also indicate a poorer prognosis.

Uncontrolled proliferation of lymphoblast in the bone marrow

Lymphoblasts replace the normal marrow elements

Decreased production of normal blood cells

Spillage of lymphoblast into the bloodstream

Organ infiltration

OSTEOGENIC SARCOMA (OSTEOSARCOMA)

A malignant connective tissue tumor whose neoplastic cells present osteoblastic differentiation and form tumoral bone

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Sign and Symptoms

Pathologic fracture Bone pain Limited ROM Weight loss Palpable , tender & fixed bony mass Edema, warmth and venous distention over the mass

Causes/Risk Factors

Medical Management
Surgery Limb-sparing (salvage) surgery when possible or amputation in some cases Radiotherapy Combined Chemo Palliative care Analgesics

Predisposing Factors: Age- 10-25 y/o above Gender- most frequent in males Heredity

Precipitating Factors: Older people with Pagets disease Exposure to carcinogens

Adjacent normal bone alters normal pattern of remodeling

Primary tumors cause bone destruction

Nursing Management
Administer prescribed IV or epidural analgesics during early postoperative period. Support and handle the affected extremities gently during nursing care. Teach patient how to use assistive devices safely and how to strengthen unaffected extremities. Explain all diagnostic procedures, treatments and expected results. Monitor and manage potential complication such as delayed wound healing, osteomyelitis, wound infection, inadequate nutrition. Assist patient in dealing with changes in body which may be due to surgery and possible amputation. Encourage the patient and family to verbalize their fears, concerns and feelings.

Diagnostic Exams
Lab Studies Serum alkaline phosphatase elevated Serum calcium level- elevated Imaging studies XRay, CT Scan, MRI- shows presence of pathologic fracture and site of bone tumor Chest XRay- determine presence of lung metastasis Surgical bone biopsy- determine histologic characteristics of the tumor Bone enlargement near tumor area

Weakness the structure of the bone

Characteristic pathologic fracture

Prognosis
Prognosis is worse if patient seeks health care when the tumor has metastasized to the lungs.

LUNG CANCER

A malignant tumor of the bronchi and peripheral lung tissue

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Sign and Symptoms

Dry, persistent cough Dyspnea Hemoptysis Chest or shoulder pain Recurring fever

Causes/Risk Factors

Medical Management
Surgery Resection of the tumor, lobectomy or pneumonectomy Radiotherapy Chemotherapy Platinum analogues- Cisplatin & Carboplatin Non-platinum containing agents- Taxanes (Paclitex, Docetaxel) Vinca alkaloids- Vinblastine, Vindesine Others- Doxorubicin, Gemcitabine, Vinorelbine, Irinotecan (CDT-11), Etoposide (VP-16), Pemetrexed (Alimta) Tyrosine kinase inhibitor (in oral form) Gefitinib (Iressa), Erlotinib (Tarceva) Immunoglobulin(Ig) therapy Prophylactic antibiotics

Predisposing Factors: Familial history

S/S of metastasis Chest pain & tightness Hoarseness Dysphagia Head & neck edema Pleural/Pericardial effusion

Precipitating Factors: Cigarette smoking or exposure to secondhand smoke Exposure to carcinogens (eg. Radon gas, asbestos, arsenic)

Certain genetic expression alters the cellular DNA

Entry of carcinogens to trachea and bronchial airways by inhalation

Diagnostic Exams
Imaging studies Chest Radiography- shows a solitary pulmonary nodule (coin lesion), areas of atelectasis and infection Chest CT scan- shows small nodules not easily detected on CXR; examine areas for lymphadenopathy Endoscopy with esophageal UTZ- used to obtain a transesophageal biopsy of enlarged subcarinal lymph nodes Fiberoptic bronchoscopy- provides detailed study of tracheobronchial tree and allows brushings and biopsies of suspicious areas Biopsy Transthoracic fine-needle aspiration used to aspirate tumor cells from a suspicious area Sputum studies Positive cytological study for cancer cells

Carcinogen binds to and damages the epithelial cells DNA

Nursing Management
Encourage the patient to assume positions that promote lung expansion. Instruct patient how to perform deep breathing and coughing exercise. Perform chest physiotherapy and suctioning per physicians order to promote airway clearance. Administer bronchodilator medications and supplemental oxygen as ordered. Educate the patient about energy conservation techniques to reduce fatigue. Instruct the patient and family about the potential side effects of specific treatment and strategies to manage them.

DNA further undergoes changes and become unstable

Pulmonary epithelium undergoes malignant transformation

Prognosis
In approximately 70% of of patients with lung cancer, the disease has spread to regional lymphatics and other sites by the time of diagnosis. As a result, long term survival rate is low.

BREAST CANCER
A malignancy in the tissue surrounding the mammary duct which tends to grow in an irregular pattern

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Sign and Symptoms

Mass usually felt in the upper outer quadrant Fixed, typically non-tender mass (except in late stages) Skin dimpling Nipple retraction or elevation Assymetry (affected breast appears higher) Bloody or clear nipple discharge Skin edema or peau d orange skin S/S of metastasis Axillary lymphadenopathy Lymphedema of affected arm S/S of lung & bone metastasis

Medical Management Causes/Risk Factors

Breast surgery Lumpectomy, Simple mastectomy or Modified Radical Mastectomy Radiotherapy Brachytherapy, External beam radiation therapy, Intraoperative Radiation Therapy (IORT) Adjuvant Chemotherapy Cyclophosphamide, Methotrexate & Fluorouracil (CMF) regimen Cyclophosphamide, Doxorubicin (Adriamycin), Fluorouracil (CAF) regimen Doxorubicin & cyclophosphamide (AC) regimen Doxorubicin, Cyclophosphamide, Paclitaxel (Taxol) (ACT) regimen Hormonal therapy Selective Estrogen Receptor Modulators (SERMs)Tamoxifen Aromatase inhibitors- Anastrazole (Arimidex), Letrozole (Femara) & Exemestane (Aromasin) Targeted Therapy Trastuzumab (Herceptin)

Predisposing Factors: Age- 50 y/o & above Gender- women are more at risk Genetic make-up (BRCA-1 & BRCA-2 mutation) Hormonal factors (early menarche, late menopause, nulliparity, having first child after 30 y/o, hormone therapy)

Precipitating Factors: Obesity High-dose radiation exposure to chest Increase alcohol intake High-fat diet

Growth of malignant tumor in the ductal-lobular epithelial cells of the breast

Diagnostic Exams
Breast Self Examination (BSE) presence of a lump or mass upon palpation Imaging studies Mammogarphy- shows presence of lesion Biopsy- confirms malignancy of cells Stereotactic needle-guided biopsy- identify nonpalpable lesions in the breast which is previously detected with mammography Excisional biopsy

Spread via lymph system to the axillary lymph nodes

Metastasis to distant regions of the body (lungs, liver, bone & brain)

Nursing Management
Promote patient and family education regarding treatment procedures, diagnostic results and prognosis of the disease. Provide relief measures after surgery such as encouraging the patient to take the prescribed home analgesic and take warm showers or use distraction methods. Reassure the patient that the experience of variety of sensations in the operative site is part of normal healing and these are not indicative of a problem. Promote patients positive body image. Monitor and manage occurrence of potential complications such as lymphedema, hematoma or seroma formation and infection.

Prognosis
The smaller the tumor, the better the prognosis. The further the spread of cancer (advanced stages), the worse the prognosis.

STOMACH (GASTRIC) CANCER

A malignant neoplasm in the stomach, usually adenocarcinoma and lymphomas

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Sign and Symptoms

Pain relieved by antacids (early disease) Presence of palpable mass (Sister Mary Josephs nodule) around the umbilicus Ascites & hepatomegaly Bone pain Dysphagia Indigestion Early satiety Anorexia Abdominal pain (just above umbilicus) Bloating after meals N/V

Medical Management Causes/Risk Factors

Surgery Total gastrectomy Radical total gastrectomy (Billroth I & II) Proximal subtotal gastrectomy Gastroenterostomy- palliative procedure to reduce N/V Radiotherapy Chemotherapy (either single or in combination) 5-Fluorouracil (5-FU), MItomycin C, Doxorubicin (Adriamycin), Etoposide Oral Imatinib mesylate (Gleevec)

Predisposing Factors: Heredity

Precipitating Factors: Helicobacter pylori infection Diet high in smoked, salted or pickled foods Chronic inflammation of the stomach & gastric ulcers Smoking Achlorydia Previous subtotal gastrectomy

Diagnostic Exams
Lab Studies CBC ct.Hgb Gastric juice aspiration- presence of lactic acid & increased level of lactic dehydrogenase (LDH) Imaging studies Upper GI XRay & Esophagogastroduodenoscopy (EGD)- confirmatory Endoscopic UTZ- assess tumor depth and any lymph node involvement CT scan- identify extent of metastasis to other organs

Malignant cells arise from the mucous lining of the stomach (usually in pyloric and antral regions) 1. Spread via hematogenous infiltration

Nursing Management
Provide optimal nutrition. Monitor IV therapy, nutritional status, I& O and daily weight. Assess daily results of lab studies to note any metabolic abnormality. Encourage patient to eat small, frequent portions of non- irritating foods. Administer TPN and antiemetics as prescribed. 2. Provide measures to relieve pain. 3. Provide measure to reduce anxiety. 4. Provide psychosocial support. Encourage patient to express fears, concerns and grief about the disease and treatment.

Spread via lymphatic channels

Spread by penetration causing ulceration

Metastasize to pancreas and peritoneal cavity

Metastasize to liver, lungs and bones

Metastasize to adjacent tissue structure (esophagus & duodenum)

Prognosis
Generally poor because diagnosis is usually made late because patients are asymptomatic at early stages.

HEPATOBLASTOMA
A primary malignancy in the liver commonly affecting the pediatric group

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Enlarged abdominal mass (usually arising from right the lobe) Abdominal pain Jaundice Severe anemia Anorexia Weight loss Predisposing Factors: Age- 3 y/o & below Gender- males are more at risk Race- whites are 5x more frequently affected Inherited disorder Beckwith-Wiedenmann Syndrome (BWS) Familial Adenomatous Polyposis (FAP) Hemihypertrophy Precipitating Factors: Exposure to Hepa B infection at an early age

Medical Management
Surgery Lobectomy (resectable tumors) Liver transplant (non-resectable tumor) Thoracotomy & pulmonary resection of metastasis Radiotherapy Chemotherapy Combination of Cisplatin (Platinol), Vincristine (Oncovin), 5-Fluorouracil or Doxorubicin (Adriamycin)

Diagnostic Exams
Lab Studies CBC ct.Hgb, platelet ct Liver enzymes- elevated AFP test- elevated (100,000-300,000 mcg/ml) Imaging studies Abdominal XRay- reveal RUQ abd mass UTZ- allows assessment for tumor size CT scan & MRI- identifies involvement of nearby structures Radionuclide scan- evaluate bone metastasis PET Scan- used for ff. up evaluation of hepatoblastoma Biopsy Open biopsy or surgical resection

Germline mutations in APC tumor suppressor gene

Intracellualar accumulation & mutation of the proto-oncogene Beta-catenin

Promote carcinogenesis

Alteration in the Wnt signaling pathway

Nursing Management
Assist in the insertion of a central line for the administration of multiple parenteral medications. Instruct patients family on the diagnosis and assist them in choosing among the therapeutic care options. Monitor patient periodically in the clinic after each course of treatment to assess for complication sand response to therapy. Monitor lab results with platelet and Hgb ct. Provide supportive care such as administering blood products and antibiotics as prescribed. Children with hemihypertrophy or BWS should be instructed to be screened regularly for AFP levels. Emphasize the need for long-term follow-up surveillance- monitoring of AFP levels and physical exam.

Oncogene activation of embryonic fetal hepatocytes

APC tumor suppressor inactivation

Prognosis
Complete surgical resection of the tumor at diagnosis, followed by adjuvant chemotherapy is associated with 100% survival rates but he outlook remains poor in children with residual disease after initial resection, even if they receive aggressive adjuvant therapy.

Uncontrolled proliferation of cancer cells

Encapsulated tumor formation in the liver

NEUROBLASTOMA
A tumor that arise from the embryonic neural crest cells and the most common extracranial solid tumor in children

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Sign and Symptoms

Abdominal pain Vomiting Weight loss Anorexia Fatigue Bone pain S/S of hypertension Neck or facial swelling Bruising above the eyes Periorbital edema (metastasis to skull bones) Bruising of the skin (bone marrow metastasis) Lymphadenopathy Firm, nontender abdominal mass

Causes/Risk Factors

Medical Management
Surgical resection or Debulking Radiotherapy Chemotherapy (in combination) Cyclophosphamide, Doxorubicin, Carboplatin, Etoposide BMT

Predisposing Factors: Age- 1-2 y/o & above Gender- males are more at risk Race- whites are more at risk Familial history Germline mutation in PHOX2B & MYCN

Precipitating Factors: Others- medications, hormones, birth characteristics, congenital anomalies, previous spontaneous abortion, fetal death, alcohol, tobacco use and paternal occupational exposures

Genetic mutation

Nursing Management
Once diagnosis is established, instruct the patient and family on the diagnosis and therapeutic options. Provide detailed instructions for home care with outpatient follow-up after completion of chemotherapy cycle. Monitor for CBC ct. as often as twice a week after discharge. Administer blood product if signs of bleeding are present. Periodically monitor urinary cathecholamines, physical exam and diagnostic imaging.

Diagnostic Exams
Lab Studies CBC ct.Hgb, WBC, platelet ct Urine test- presence of HVA (Homovanillic acid) and VMA (Vanillylmadelic acid) Imaging studies Chest and abdominal CT Scan & MRI- determine site of tumor and evidence of metastasis Chest XRay & Bone Scan- identify metastasis Fluorescent in situ hybridization (FISH) Detect MYCN amplification

Amplification of distal arm of chromosome 2

Allelic losses of chromosome 11q, 14q, & 17q

Deletion of the short arm of chromosome 1

Overexpression of MYCN (an oncogene)

Absence or decrease in tumor suppressor genes

Neural crest rapid tumor progression

Prognosis
Patients with localized disease has survival rate of 70% while those having metastatic disease have a longterm survival rate of <25%.

Migrate & invaginate the sympathetic ganglia, adrenal medulla and other sites

Health Teaching
Educate the patient and family about the importance of treatment and adverse effects of medications used. Emphasize the need to recognize and identify signs and symptoms of complications that require urgent medical care.

EWING SARCOMA
A highly malignant primary bone tumor that is derived from neural crest cells

ASSESSMENT

PATHOPHYSIOLOGY

MANAGEMENT

Causes/Risk Factors Sign and Symptoms

Back pain Palpable mass Fever, weight loss Lesions of long bones & pathologic fractures Bleeding & infection- bone marrow metastasis Numbness, weakness & pain in the extremities Predisposing Factors: Age- late teenage years Gender- males are more at risk Race- whites males are more at risk Precipitating Factors: Chromosomal translocation

Medical Management
Surgery Removal of fibula, limb salvage of extensive margins Radiotherapy Chemotherapy- 6-9 mos of alternating courses of 2 chemo regimens Doxorubicin, Cyclophosphamide & Vincristine Ifosfamide & Etoposide

Reciprocal translocation between chromosome 11 & 22 [t (11; 22)]

EWS-FLI1 fusion generate 68 kDA protein

Nursing Management
Promote patient and family education regarding treatment procedures, diagnostic results and prognosis of the disease. Collaborate with specialists such as an orthopedic oncologist, neurologist and pathologist in a multidisciplinary setting. Initiate neutropenic and bleeding precautions. Administer blood products as ordered. Obtain full physical exam before each cycle of chemotherapy. Provide teachings about expected complications particularly fever and its management.

Diagnostic Exams
Cytogenetic & Molecular studies Presence of t(11;22) Imaging studies CT Scan & XRay- delineate bony involvement Chest CT scan, Radioisotope Bone Scanning & MRI - used for evaluation of metastasis Biopsy For definitive diagnosis Histologic findings Staining with MIC2 (12E7) antigen (CD99)

Abnormal gene transcription factor

Transforms fibroblasts

Tumor growth usually deriving from neural crest cells

Prognosis
At this time, the only significant factor that determines the prognosis is the presence of or absence of metastatic disease.

RHABDOMYOSARCOMA
Most common tissue sarcoma (cancer of connective tissues) in children in which cancer cells are thought to arise from skeletal muscle progenitors. Has 2 common forms: Embryonal RMS & Alveolar RMS

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Orbit- proptosis or dysconjugate gaze Paratesticular- painless scrotal mass Prostate- bladder or bowel difficulties Uterus, cervix, bladdermenorrhagia or metrorrahagia Vagina- protruding polypoid mass Extremity- painless mass Parameningeal- upper respiratory symptoms or pain

Medical Management
S/S of metastasis Bone pain Respiratory difficulty Anemia, Thrombocytopenia, neutropenia Predisposing Factors: Age- 1-5 y/o, 15-19 y/o (rare) Genetic syndromes Neurofibromatosis Li- Fraumeni syndrome Rubinstein-Taybi syndrome Beckwith- Weidenmann syndrome Costello syndrome Precipitating Factors: Parental use of marijuana & cocaine Intrauterine exposure to XRAY Previous exposure or use of alkylating agents Surgical resection For primary and feasible tumor Radiotherapy Chemotherapy Etoposide, Cyclophosphamide, Dactinomycin, Vincristine, Ifosfamide, irinotecan

Reciprocal chromosomal translocation t(2;13) or t(1;13)

Nursing Management
PAX3-FOXO1a or PAX7-FOXO1a (potent transcription activator) fusion Provide supportive care such as administering feeding via enteral tube or parenteral if indicated especially those having primary tumor in the head or neck or who may have mucositis after chemo. Promote patient and family education regarding treatment procedures, diagnostic results and prognosis of the disease. Provide psychosocial support to patient and his/her family on coping with the disease. Initiate neutropenic precautions and continue to assess patient for having fever indicative of infection. Emphasize the need for long-term follow up care and to recognize and identify signs and symptoms of complications that require urgent medical care.

Diagnostic Exams
Lab Studies CBC ct.Hgb, WBC, platelet ct Urinalysis- hematuria (involvement of GU tract) Imaging studies Chest Radiography- determine presence of calcification CT scan, MRI, UTZ (lungs, chest, bone, liver)- assess extent of metastases Biopsy Open biopsy or core needle biopsy Procedures Cytogenetics/ Fluorescent in situ hybridization (FISH)determine translocations Reverse transcriptase testing (RT-PCR)- assess translocation assoc. with ARMS Histologic findings Immunohistochemical marker test- (+) myoD1 and myogenin proteins or myoglobin, actin, desmin Activate N-Ras & K-Ras oncogene Repress t53

Formation of rhabdomyeblasts in the head & neck, extremities, GU tract, trunk, orbit or retroperitoneum & mucosal cavities

Prognosis
Metastases (lungs, bone marrow, bony lymph nodes, breast & brain) Patients with localized disease has survival rate of 80% while those having metastatic disease have a long-term survival rate of <30%.

LYMPHANGIOMA
An uncommon congenital malformation of the lymphatic system that involve the skin and subcutaneous tissues

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Lymphangioma circumscriptum Small clusters of vesicles (2-4 mm) that vary from pink to black color secondary to hemorrhage Can have a warty appearance Cavernous lymphangioma A subcutaneous rubbery nodule with no skin changes Cystic hygroma Deep, subcutaneous swelling of the axilla, neck, groin (larger than cavernous lymphangioma)

Medical Management
Predisposing Factors: Age- newborn Genetic disorder Noonan syndrome Trisomies 13, 18, 21 Turner syndrome Down syndrome Precipitating Factors: Maternal alcohol use Viral infections during pregnancy Surgery Surgical excision of tumors, hypertonic saline sclerotherapy, cryotherapy, LASER, cautery Intralesional OK 432 (Picibanil)- for macrocystic lesions only Postop vacuum assisted closure device Decreases risk of recurrence & infection Propranolol

Failure of the primitive lymph sac to connect with the rest of the lymph system during embryogenesis

Nursing Management
Institute infection precaution. Instruct the patients family not to expose the child to any source of radiation to prevent progression of disease to lymphangiosarcoma (complication). Monitor the patient for occurrence and possible recurrence of cellulitis. Regularly perform skin examination. Provide reassurance to the family and stress out the risk of recurrence of the disease. Provide supportive measures such as performing tracheostomy care, monitoring respiratory problems, administering enteral feeding secondary to dysphagia.

Alignment of a thick coat of muscle fibers to the primary sac

Diagnostic Exams
Imaging studies MRI- help define the degree of involvement and entire anatomy of the lymphangioma lesion Immunohistochemical studies Factor VIII-related antigen test- differentiate hemangioma from lymphangioma (negative or weakly positive in lymphangioma) Dermoscopic Findings Aid in the diagnosis of lymphangioma circumscriptum Histologic findings Rhythmic contraction of muscle fiber increases intramural pressure

Collection of lymphatic cisterns in the deep subcutaneous plane or dermis (loose connective tissue)

Protrusion of dilated lymph channels from the walls of the cisterns toward the skin

Cavernous lymphangioma

Cystic hygroma

Formation of characteristic vesicles (in lymphangioma circumscriptum) as outpouchings of the dilated lymph channels

Prognosis
Lymphangiomas are benign hamartomatous malformations instead of true neoplasms. The prognosis for lymphangioma is excellent.

HEMANGIOMA
A benign, and usually a self-involuting tumor (swelling or growth) of the endothelial cells that line blood vessels and is characterized by increased number of normal or abnormal blood vessels filled with blood.

ASSESSMENT

PATHOPHYSIOLOGY Causes/Risk Factors

MANAGEMENT

Sign and Symptoms

Early signs Blanching of involved skin followed by fine telangiectasias and a red or crimson macule Birth-12 months (periods of active proliferation) Lesions may be dome-shaped, bosselated or plague-like Color-can be bright red or crimson, purple-blue or flesh-colored Size- size of pinhead to >20cm in diameter Telangiectases & large superficial veins radiating from the hemangioma is evident Consistency- firm, rubbery, tense & expands with increased intravascular pressure (eg.crying) Tender to palpation Birth or as late as 2-3 y/o (periods of involution) Superficial lesions become less red, from duskier maroon to purple color to regaining normal flesh tones or graying Softer, more compressible with decreased tenderness, decreased expansion Late involution (5-7 y/o) Skin may return to normal 50-60% leaves permanent skin changes

Medical Management
Predisposing Factors: Age- at birth or several weeks of life Race- more common in whites Gender- females are more at risk Precipitating Factors: Fetal hypoxia Increased VEGF releaseplacental response to angiogenesis during pregnancy Missense genetic encoding for VEGFR2 Surgery Surgical excision LASER surgery Beta blockers- Propranolol (Inderal) Oral and topical corticosteroid- Prednisolone Interferons Biologic immune response modifiersImiquimod (Aldara cream)

Increased angiogenetic peptides (beta- fibroblast growth factor, VEGF, proliferating cell nuclear
antigen)

Nursing Management
Educate parents about the variable natural history, prognosis, risks and benefits of potential treatment and possible complication. Provide emotional support to parents of children with severe or complicated hemangioma. Refer patients with significant complications such as visual and airway obstruction to specialized pediatric physician. Prevent infection and severe bleeding from ulcerated hemangioma.

Diagnostic Exams
Lab Studies Presence of serum VEGF Presence of urinary beta-fibroblast growth factor, VEGF and matrix metalloproteinases (MMPs) Imaging studies MRI w/ or w/o IV gadolinium- delineate the extent of both cutaneous and extracutaneous hemangiomas, differentiate other high-flow vascular lesions UTZ- differentiate hemangioma from other deep dermal or subcutaneous lesions such as cysts or lymph nodes Plain radiographs- evaluate hemangiomas that impede on the airway Biopsy Skin biopsy- distinguish unusual or atypical hemangioma from other vascular lesions

Immature endothelial cells coexist with immature pericytes during the 3rd trimester of pregnancy

Induce proliferation of immature endothelial cells

Increased proliferative capacity of endothelial cells

Influx of mast cells, myeloid cells and tissue inhibitors of metalloproteinases (TIMPs) occurs

Prognosis
Causes termination of endothelial cell proliferation Patients with uncomplicated hemangioma have good prognosis but may have residual skin changes or scar formation. Hemangiomas that are take time to involute and exists in the lip, nasal tip, eyelid and ears have increased incidence of permanent cutaneous residua.

Passive induction of involution by senescence of endothelial cells