Pediatric Hematology/Oncology: Tumors

solid, death, astrocytoma, posterior, location, headache, nausea, balance

Brain Tumors: Background

-Most common ______ tumor and second most common overall malignancy in children

-Leading cause of cancer _______ in children

-Etiology → unknown, genetic, environmental, and immune factors

-Most common types → medulloblastoma, ependymoma, ____________, brainstem glioma, craniopharyngioma, optic nerve glioma

• 2/3rds of all pediatric brain tumors are located in the __________ fossa

-Symptoms → depends on ________, cell type, and rate of growth

• ICP symptoms → _________ (worse in AM), vomiting (worse in AM, without _______), lethargy, irritability

• Cerebellum involvement → impaired coordination and _________ (ataxia, gait difficulties)

astrocytes, cerebellum, slow, unilateral, ataxia, vermis, CSF, fast, ependymal, lateral

Brain Tumors: Types

-Astrocytomas (20% cerebellar, 8% cerebral)

• Arise from __________ in the __________ or cerebral hemispheres

• _____ growing, often very large before diagnosed

• Cerebellar astrocytomas often cause _______ symptoms such as head tilt, limb _______, and nystagmus

-Medulloblastoma (18%)

• Arises in the _____ of the cerebellum and can extend into the 4th ventricle, subarachnoid space, and ____

• _____ growing

-Ependyoma

• Arises from ___________ cells that line the ventricular system

• Most often 4th and _______ ventricles

pons, weakness, unilateral, contralateral, optic chiasm, slow, neurofibromatosis, visual, pituitary, slow, ICP, puberty

Brain Tumors: Types

-Brainstem Gliomas

• Arise from the _____ or medulla

• Can compress CN V-X

• Can cause facial __________, limitation of horizontal eye movement, ataxia, and corticospinal tract dysfunction

• Common presentation → _______ paralysis of CNS with _________ paralysis of the arm and leg, hyperreflexia, and extensor plantar responses

-Optic Nerve Gliomas

• Arise from _____ _______ or optic nerve

• Low grade astrocytoma

• ____ growing

• Often associated with _____________ type I

• Blindness or other ______ disturbances

-Craniopharyngioma

• Arises near the _______ gland, optic chiasm, and hypothalamus

• _____ growing

• Can compress pituitary gland, hypothalamus, or optic chiasm and cause ___

MRI, vascularity, CSF, resection, chemotherapy

Brain Tumors: Diagnosis and Treatment

-Diagnosis

• Neurologic, developmental, and ophthalmic exams

• ____ is the imaging of choice

• MRA to assess __________ of tumor and relationship to other major vessels

• MRI of spine

• LP to examine ____ for tumor cells

• Histochemical and molecular genetic analysis

-Treatment

• Surgical _________ unless contraindicated

• Radiation and _____________

soft tissue, 10, rhabdomyoblasts, striated, anywhere, head, GU

Rhabdomyosarcoma: Background

-Most common _____ ________ sarcoma of childhood, accounting for > 50% of soft tissue tumors

-Overall rare

-Epidemiology

• Most diagnosed by __ years old (peak 2-6 y/o and 15-19 y/o)

• Male > Female

-Pathogenesis

• Arises from embryonal _____________ that normally differentiate into _______ muscle

• Can occur _______ there is striated muscle

• Primary locations of _____ and neck, trunk, extremities, and __ tract

located, ptosis, urinary, mass, biopsy, blue cell

Rhabdomyosarcoma: Symptoms and Diagnosis

-Symptoms

• Depends on where the tumor is _________

• _____, dysphagia, facial nerve palsy, _______ obstruction, hematuria, abdominal pain

• Painless, palpable or visible _____

-Diagnosis

• ________ of small, round mass is confirmatory

• _____ _____ tumors

• Serology

• Imaging

resection, radiation, metastatic, widespread

Rhabdomyosarcoma: Treatment

-Surgical _________

-Chemotherapy

-_________

-20% have _________ disease at diagnosis

-Prognosis

• Localized disease = 70% overall survival rate

• ________ disease = 20% survival rate

bone, 10-18, mesenchymal, metaphyses, long, femur, tibia, pain, redness, lung, fracture

Osteosarcoma: Background

-Most common malignant _____ tumor in childhood

-Epidemiology → ___-___ years old

-Pathogenesis → Arises from bone producing ___________ cells

• Occurs mainly in the __________ of ____ bones

• Distal end of _____ or proximal end of _____ (MC)

• Can extend beyond the bone into the soft tissues

-Symptoms → _____, swelling/warmth/_________ over the area, cough/SOB/chest pain if _____ mets, limp, pathologic __________

MRI, primary, CT, biopsy, osteoid, resection

Osteosarcoma: Diagnosis and Treatment

-Diagnosis

• ___ to evaluate extent of the _________ tumor

• Metastatic evaluation with bone scan and chest __

• Tissue _______ (confirms), shows presence of _______

-Treatment

• Chemotherapy

• Surgical ___________

bone, soft, lethal, 10-20, bone marrow, diaphysis, flat, pelvis, early

Ewing Sarcoma: Background

-Malignant tumor of _____ and _____ tissue

-Second most common but most _______ malignant bone tumor in childhood

-Epidemiology

• Peaks between ___-___ years old

• Male > female

• Most common in Caucasians

-Pathogenesis

• Neural crest cell origin

• Arises from _____ ________ and can break through cortex to form soft tissue mass

• Most often occurs in the _________ of long bones or ____ bones (femur, _____, tibia most common)

-Mets occur _______

pain, mass, MRI, biopsy, round, chemotherapy, resection

Ewing Sarcoma: Symptoms, Diagnosis, Treatment

-Symptoms

• ______ is most common

• Soft tissue _____

• Fever, malaise, anorexia

-Diagnosis

• CT and ____ to evaluate extent of tumor

• Met evaluation with bone scan, chest CT, bone marrow biopsy

• _______ (confirms) → small, _______, blue cell tumors

-Treatment

• _____________, radiation, surgical __________, or all of the above

• Resection is a must

adrenal medulla, sympathetic, solid, 5, 17

Neuroblastoma: Background

-Derived from neural crest cells that form the _______ ________ and the _________ nervous system

-Epidemiology

• Most common extra-cranial _____ tumor in infants <1 y/o

• 75% are found before age _ (median age __ months)

• Rare after 10 y/o

-Etiology is unknown

mass, bowel, Horner, loss, sweating

Neuroblastoma: Symptoms

-Retroperitoneal region/adrenal medulla

• Abdominal pain/_____, anorexia, _______ or bladder issues, spinal cord compression

-Mediastinum

• Shortness of breath, infection, airway obstruction

-Cervical sympathetic ganglion

• _______ syndrome

-Systemic symptoms

• Weight _____, irritability, fatigue, fever

-Paraneoplastic syndromes

• Secretory diarrhea, profuse ________, and opsomyoclonus

catecholamines, biopsy, resection, chemotherapy, radiation, metastatic, die

Neuroblastoma: Diagnosis and Treatment

-Diagnosis

• Urine ____________

• CT

• Bone scan

• Bone marrow aspiration/biopsy

• Tissue ________ (makes the diagnosis)

-Treatment

• Low risk → surgical __________

• Immediate risk → surgery then ____________

• High risk → chemotherapy, resection, stem cell rescue, _________, sometimes immunotherapy

-70% have ___________ disease at diagnosis

-50% of children with high risk disease will ____ despite treatment

bilateral, 1st, 2-3, retinal, vitreous, CNS, choroid

Retinoblastoma: Background

-Etiology

• Inherited → more likely to be ________

• Acquired

-Epidemiology

• Inherited → diagnosed within the ____ year of life

• Acquired → diagnosed around __-__ years old

-Pathogenesis

• Arise from ______ cells and extend into _________ humor

• Can invade the optic nerve

• Can gain access to subarachnoid space and ____

• Metastasis possible if invade the ________

leukocoria, painful, CT, aspiration, MRI

Retinoblastoma: Symptoms and Diagnosis

-Symptoms

• Unilateral or bilateral

• _________ (white reflex) → most common finding

• Strabismus, ________ eye, limited vision

-Diagnosis

• Full ophthalmologic exam

• __ or orbits

• Metastatic studies of bone marrow _________, LP, bone scan, ____

chemotherapy, glaucoma, inheritable

Retinoblastoma: Treatment

-External beam irradiation

-___________ (replacing irradiation), decreased need for enucleation

-Enucleation

• Indicated if no vision, neovascular __________, inability to examine the treated eye, inability to control tumor growth with conservative therapy

-Monitor patients with _________ form for development of other tumors like osteosarcoma

1-5, 2-3, kidney, stem cells

Nephroblastoma/Wilms Tumor: Background

-Etiology

• Mostly unknown

• Sporadic vs inherited

-Epidemiology

• Most diagnosed between __-__ years old with peak between __-__ years old

• Female > male

• AA > Caucasian > Asian

-Pathogenesis

• Embryonal tumor of the _______ arising from abnormal proliferation of renal _____ _______ (metanephric blastema)

asymptomatic, mass, smooth, fever, CBC, CT, biopsy

Nephroblastoma/Wilms Tumor: Symptoms and Diagnosis

-Symptoms

• _____________ abdominal _____ (MC) → firm, nontender, _______

• Abdominal pain, ______, HTN, hematuria possible

-Diagnosis

• ____, UA, LFTs, BUN/creatinine

• Abdominal US or __ shows solid intrarenal mass

• CT of chest and abdomen/pelvis to assess for metastasis

• Tumor ______ confirms diagnosis

resection, radiation, chemotherapy, nephrectomy

Nephroblastoma/Wilms Tumor: Treatment

-Surgical exploration and __________ followed by treatment based on histology

• Chemotherapy with or without __________

-___________ followed by surgical resection

-Some low risk patients with unilateral disease can be cured with ___________ only