Oncology 1 Section 6 Other

cutaneous lymphomas (includes, definition, treatment)

• includes Mycosis Fungoides

• a neoplastic disease of the T-cells, manifesting on the skin surface creating eczema-like or fungus-like patches at usually several different areas of the skin

• total skin irradiation (TSI) using Stanford technique-6 angles

• can also be treated with topical nitrogen mustard for early stages only

Kaposi’s sarcoma (epidemiology, etiology, appearance, progression, treatment for solitary vs multiple lesions)

• rare

• AIDS patients (aggressive form)

• North African and Mediterranean men

• thought to arise from vascular tissue

• hard, raised purplish patches anywhere on the body

• progression is slow but fatal

• solitary lesions = surgery

• multiple lesions = RTT or sometimes chemo

melanoma (AKA, cases per year, develop from)

• AKA “The Killer Tan”

• about 54,000 new cases per year

• develop from melanocytes

epidemiology of melanoma (vs other skin cancers, deaths, peak age of incidence, predominant gender, incidence rate, common in age/gender, latent period, 5 year survival)

• less common but more lethal than other skin cancers

• 70% of all skin cancer deaths while only making up 5% of all skin cancers

• peak age of incidence: 40s or 5th decade

• male predominant

• incidence is increasing dramatically - doubling every 15 years and about 7% per year increase

• most common cancer in men and 2nd most common in women 30-40 years old

• most common cancer in people between 25-29 years old

• latent period = 10-20 years

• 70-80% 5-year survival

etiology of melanoma

• same as other skin cancers

• pre-existing nevi

  • 70% due to nevi

• heredity

  • people with family history of melanoma have 8x higher risk

    • familial atypical mole and melanoma (FAM-M) syndrome AKA dysplastic nevus syndrome (DNS)

ABCD’s of melanoma

• A = asymmetry

• B = border (irregular, notched, uneven)

• C = color change

• D = diameter change

general info of melanoma (screening, symptoms)

• monthly skin exam using a mirror recommended

• may cause itching or bleeding that doesn’t seem to heal

diagnosis of melanoma

• biopsy

• epiluminescence microscopy (ELM) - uses a dermatoscope to differentiate between benign and malignant lesions and images can be digitized

pattern of growth and metastatic spread of melanoma

• tend to grow down into tissue rather than grow across the skin surface like other skin cancers

• most commonly mets to lungs followed by brain

treatment principles of melanoma

• surgery

  • surgical excision is treatment of choice (100% cure for early lesions using a wide margin (2 cm or greater)

  • possible nodal dissection during procedure

• RTT

  • limited to palliative treatment for advanced cases or mets due to radioresistance

  • doses are lower than typical - 30Gy in 10 fractions is common

• chemotherapy

  • Dacarbazine (DTIC) or Interferon (immunotherapy) used for treatment of advanced systemic disease

  • not curative but can produce remission in some cases

conjunctiva

mucous membrane that lines the inside of the eyelids

retina

innermost layer of the eye that receives images formed by the lens

lacrimal gland

tear duct

proptosis

downward displacement of the eye

diplopia

double vision

exophthalmos

protrusion of the eyeball

uveitis

inflammation of the uvea, which is the middle layer of the eye and includes the iris, ciliary body, and choroid

aniridia

congenital loss of the iris

treatment of tumors of the eyelid

• surgery

• KV x-ray w/ eye shielding

pterygium (definition, treatment)

• benign thickening of the conjunctiva

• Strontium-90 (Sr90) beta plaque

retinoblastoma (age of incidence, etiology and risk of other cancers, typical presentation, diagnosis, staging system, mets, prognosis)

• usually occurs before age 2 and nearly always before age 4

• spontaneous regression in 1% of patients (highest regression rate of all true malignancies)

• related to chromosome 13 - tumor suppressing gene

  • susceptible to other cancers, notably sarcomas, later in life due to gene

• cat’s eye reflex - white calcification/tumor in the retina

• diagnosed with:

  • ophthalmoscopy

  • CT

• Reese-Ellsworth system

• spreads directly along optic nerve to cranial cavity

• can also go through blood to various organs but rare

• 80% 5-year survival, better in younger patients

treatment of retinoblastoma

• surgery

  • enucleation has great cure rate but other modalities may be used since sacrificing the globe is not desirable

• RTT

  • definitive treatment

  • always used if positive margins at the optic nerve or if tumor is bilateral

  • high risk of developing late malignancy

  • radiosensitive compared to most eye tumors

  • 40Gy total dose

  • various oblique fields to avoid the lens

  • lens tolerance = 1000 cGy

  • lacrimal glands tolerance = 3000 cGy

  • IMRT advisable

  • radioactive plaques used with high cure rates

• chemotherapy

  • good response rate

intraocular melanoma (epidemiology, etiology, presentation)

• most common intraocular cancer

• more common in adults

• 2nd leading cause of enucleation of the eye following trauma

• etiology is unknown

• loss of peripheral vision

• floaters - sensation of floating spots

treatment of intraocular melanoma

• surgery - enucleation is definitive treatment

• RTT - rarely used because of radioresistance

• chemotherapy - not effective

tumors of/on the orbit

• rhabdomyosarcoma - aggressive sarcoma that can occur in many areas including the eye and muscles around the eye

• hemangioma - benign tumor of dilated blood vessels

  • most common orbital tumor

  • 90% spontaneous regression rate

pediatric cancers cases and deaths per year in the US

• 9000 diagnosed each year

• 1500 deaths

pneumonitis

inflammation of the lungs

encephalopathies

any dysfunctions or diseases of the brain

enteritis

inflammation the small intestines

alveolar

holly honey-comb shaped

pleomorphic

many shapes

malaise

massive fatigue or lethargy

epidemiology of pediatric cancers (cases, deaths, common sites, most common, incidence males vs females, treatment norm and location)

• 2nd leading cause of death in children after trauma/accidents

• 9000 cases per year

• 1500 deaths per year

• 6000 cases per year with 1/3 dying (solid tumors)

• usually involve either the hematopoietic, nervous, or connective tissues (generally epithelial tissues and environmentally induced for adults)

• most common: leukemia (80%)

• 2nd most common: CNS/brain

• higher incidence in males

• multi-disciplinary treatment is the norm because of concerns for late effects

• usually treated in specialized clinics rather than local hospitals

etiology of pediatric cancers

• genetic/hereditary

• virus

  • Epstein Barr Virus (EBV)

  • AIDS

    • Kaposi’s sarcoma

    • lymphoma

RTT late effects on children

• late carcinogenesis

• retardation of bone/cartilage growth, e.g., scoliosis, stunted growth

• intellectual impairments

• sterilization (if gonads are in the field)

• other organ specific effects

chemo late effects on children

• same as RTT

• renal atrophy

• cardiomyopathy

• encephalitis

• enteritis

psychosocial aspects due to late effects on pediatric cancers

• stigmas from peers

• helicopter parenting

Wilms tumor (AKA, first to, clinical presentation)

• AKA nephroblastoma or embryonal kidney tumor

• 1st cancer treated with multi-modal approach

• palpable abdominal mass

  • usually painless

  • pain is a sign of advanced disease

• can be bilateral

• hard initially to differntiate with neuroblastoma

epidemiology/etiology of Wilms tumor (cases per year, correlation, gene/chromosome, average age, risk factors, arises in, 5 year survival)

• about 500 cases per year

• 1/3 of all patients that develop associated sporadic aniridia develop Wilms tumor

• Wilms tumor gene discovered that represents a deletion on chromosome 11p13

• average age = 3, almost all occur before age 7

• risk factor: genetics

  • multiple genitourinary abnormalities increases risk

• arises in embryonal kidney cells

• 80% 5-year survival rate, younger is better

diagnosis, staging, grading, lymphatics, and mets of Wilms tumor

• abdominal ultrasound or CT scan

• staging: National Wilms Tumor Study system (NWTS)

  • stages I-V

  • stage V = bilateral kidney involvement

• grading

  • low grade = well differentiated

  • high grade = poorly differentiated

• lymphatics commonly positive at diagnosis

  • renal hilar nodes

  • para-aortic nodes

• often mets to lungs

treatment of Wilms tumor (surgery, RTT, chemo, treatment per stage)

• surgery

  • definitive

  • nephrectomy

  • 2nd look surgery - check response or convert partial response into a better response, but no longer common

• RTT

  • previously used in all stages but now only used in late stages (3 or 4)

  • treat the tumor bed and cover the entire width of the vertebral body plus 1-2 cm and including para-aortic nodes

  • parallel opposed fields to avoid the contralateral kidney

  • use low doses due to radiosensitivity

    • 1080-2000 cGy

    • 180 cGy starting on day 9 w/ surgery on day 0

• chemotherapy

  • VACA

    • vincristine

    • adriamycin (doxorubicin)

    • cytoxan (cyclophosphamide)

    • actinomycin-D

  • 70% cure rate even with mets

• Stage I: nephrectomy

• Stage II or II: nephrectomy + VACA

• Stage IV or V: nephrectomy + VACA + RTT

neuroblastoma (originates from, most common, median age, site, etiology, prognosis)

• originates from neural crest cells that normally develop into the adrenal medulla

• 2nd most common pediatric solid tumor

• median age: 2 years old

• rare over 7 years old

• usually occurs on adrenal glands but can occur anywhere along hte sympathetic nerve chain

• etiology

  • heredity

  • possibly present in most at birth but spontaneously regresses

    • 1-3% incidence at autopsy of unsuspected cases in infants

  • may be linked to abnormal maturation of fetal neural crest cells

• age is most important prognostic factor

  • > 18 months = 70% chance of mets

  • < age 1 = 70% 5-year survival

  • > age 1 = 30% 5-year survival

adrenal glands

triangular shaped body that covers the superior surface of each kidney

symptoms and diagnosis of neuroblastoma

• symptoms

  • abdominal mass

  • pain

  • malaise

  • Horner’s syndrome

• urinalysis from diapers can detect by-products of excess hormone production

• hypertension check

treatment of neuroblastoma

• surgery - best hope for cure

• RTT

  • only useful for late stage (3 or 4)

  • adjunct treatment: 20-30 Gy to tumor bed

  • palliative treatment: 10 Gy

• chemo

  • multi-agent is a common adjuvant for all stages

rhabdomyosarcoma (most common, etiology, detection/sign, pathology, survival)

• most common pediatric soft tissue sarcoma

• sarcomas are more common in children and teens than in infants

• can occur anywhere but most commonly in H&N

• etiology unknown but associated with fetal alcohol syndrome

• presents as a painful mass

• pathology - pleomorphic and several other subtypes

• 5 year survival rates vary considerably depending on primary site and cell type/behavior

treatment of rhabdomyosarcoma

• surgery - excision preferred but chemo/RTT have better cosmetic results

• RTT

  • fields include tumor volume with at least a 2cm margin

  • 180 cGy/day for 5040 total

  • 110 cGy x 2/day for 5940 total

  • good local control, especially if combined with chemo

• chemotherapy - effective adjuvant/adjunct

Langerhans cell histiocytosis (AKA, involves, survival)

• AKA Histiocytoses X

• involves several different diseases involving abnormal reproduction of the histiocytic immune system’s Langerhan’s cells

• 30-40% of patients survive without treatment

Hodgkins disease and non-Hodgkin's lymphomas (age of incidence, curable, worst prognosis)

• more common among adolescents and young adults than in children or infants

• HD is fairly curable but NHL less so

• Burkitt’s lymphoma = poorest prognosis of all lymphomas

osteogenic sarcoma AKA osteosarcoma (most common, age, site, treatment)

• most common primary bone cancer

• usually occurs in teen years

• usually occurs in the bones around the knee

• surgery - amputation, not commonly done anymore

• RTT - rarely used

• chemotherapy - used more and more, helps minimize need for amputation

Ewings sarcoma (definition, peak incidence, most common site, treatment, 5-year survival)

• non-osseous tumor that usually arises form soft tissue near bone (not an actual bone tumor)

• peak incidence: teen years

• most common site: on the femur

• surgery - usually not used

• RTT - used often since most radionsensitive in bone/soft-tissue sarcoma category

• chemo - common adjunct to RTT

• 50% 5-year survival

germ cell tumors (definition, example/type, common sites, etiology)

• rare tumors that arise from ovum or sperm cell but arise in a variety of sites

• ex. teratoma

  • most often found in the ovary or testis

  • “monstrous tumor” that can contain tissue or organ components of all three germ layers

  • thought to be present at birth but often not diagnosed until much later in life

• etiology unclear but may represent an abortive attempt at twinning

treatment of keloids/fibromas if repeated resections and steroid injections are not successful

resection followed by immediate low-dose radiation of 900-1200 cGy in 3 fractions