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80 Cards in this Set
- Front
- Back
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Why are some malignancies classified as rare tumors in the pediatric oncology setting?
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- they originate from epithelial tissues (the source of most adult cancer) rather than embryonal tissues (source of most childhood cancers)
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What age group is more commonly diagnosed with a rare pediatric cancer?
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15 to 19 year-old age group
[see Figure 2-6; page 55] |
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Why do children & families who are affected by a rare cancer require additional support?
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- often little information available
- the child & family often feel isolated |
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What rare pediatric tumor is second to rhabdomyosarcoma, involves the upper respiratory tract, and is associated with EBV?
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Nasopharyngeal carcinoma
(3% of all occur in kids <19yrs) |
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How can one use EBV as a way to monitor tumor markers?
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Monitoring EBV titers
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What causes scientists to strongly believe that there is an environmental and genetic cause in the pathology of a nasopharyngeal carcinoma tumors?
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There is a high-incidence of EBV-associated tumors in Asia
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What the often the first and only presenting symptom of nasopharyngeal carcinoma?
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Lymphadenopathy
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Why is lymphadenopathy a presenting symptom of nasopharyngeal carcinoma?
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It develops in pharyngeal recesses and spreads to cervical lymph nodes
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What are a few other presenting symptoms of nasopharyngeal carcinoma?
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1. epistaxis
2. nasal obstruction 3. trismus 4. hearing loss 5. ear pain 6. Headache 7. Chronic otitis media |
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What do 50% of nasopharyngeal carcinoma patients have?
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Cranial nerve involvement
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Where does nasopharyngeal carcinoma typically metastasize to?
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locally to lymph nodes, lungs, vertebrae, long bones, or liver
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Why does radiation treatment for nasopharyngeal carcinoma often have long-term effects?
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Radiation is to extended fields (6,000 -7,000 cGy)
- may cause xerostomia & fibrosis of neck muscles |
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Can radiation alone be used as treatment for nasopharyngeal carcinoma?
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Yes, in low-stage tumors
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What tumors are typically treated with adjuvant chemotherapy?
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Advanced tumors using
- cisplatin (Platinol) - fluorouracil (5-FU) - MTX (Mexate) - Bleomycin (Blenoxane) - VCR, Cytoxan, Doxo, VP-16 |
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What is the overall survival rate of nasopharyngeal carcinoma?
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78%
small tumors = favorable prognosis vs tumor extension outside nasopharynx |
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What is one of the most common soft-tissue sarcomas (non-RMS) in children & adolescents?
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Fibrosarcoma = malignancy of fibrous tissue
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T or F: In children younger than 1 year fibrosarcoma is the most common soft-tissue sarcoma
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True
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When do the 2 peak incidences of fibrosarcoma occur in children?
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#1 = kids <5yrs of age (congenital/infant)
#2 = kids 10-15yrs of age (adult form) |
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What is true about the fibrosarcoma tumors usually found in infants?
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They are usually benign
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What has the adult form of fibrosarcoma been associated with?
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Previous radiation exposure
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What is the most common site for a fibrosarcoma?
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Extremity, especially distal segments
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Can fibrosarcoma occur anywhere else?
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Yes, trunk or head & neck
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How is infant fibrosarcoma usually treated?
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Wide surgical excision
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Does a late local recurrence of infant fibrosarcoma affect overall survival?
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No, and can occur in 17-43% of cases
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sexually transmitted disease
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la enfermedad venérea, la enfermedad de transmisión sexual
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What is the 5-year survival rate for congenital fibrosarcoma? for an older child with fibrosarcoma?
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congenital = 95% 5-yr survival
older children = 60% 5-yr survival |
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What is the most common site for metastasis with a diagnosis of fibrosarcoma?
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Lung
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What are most malignant hepatic tumors?
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- hepatoblastoma carcinoma
- hepatocellular carcinoma |
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What percentage of hepatic tumors are malignant?
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50%
-non-malignant tumors = hemangiomas, hamartomas |
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What is the median age of a patient with hepatoblastoma?
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1 year
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What is the median age of a patient diagnosed with hepatocellular carcinoma?
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12 years
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What constitutional syndromes is hepatoblastoma associated with?
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- familial adenomatous polyposis
- Beckwith-Wiedemann syndrome - Li-Fraumini syndrome |
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What is hepatocelluar carcinoma associated with in patients younger than 15yrs of age?
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Hepatitis B virus
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What hepatic tumor is associated with prolonged use of anabolic steroids and cirrhosis?
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Heptocellular carcinoma
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How does heptoblastoma most often present?
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asymptomatic abdominal mass
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What do many patients present with in hepatoblastoma that will regress once the tumor has been resected?
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Osteopenia
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What liver malignancy often presents with abdominal distention and mass in the pt's RUQ?
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Hepatocellular carcinoma
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What presenting signs accompany mass in RUQ and abd. distention?
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Abdominal pain
nausea vomiting |
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What blood level may be elevated in hepatocellular carcinoma?
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Alpha-fetoprotein
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Are the cure rates high for hepatoblastoma that has been completely resected?
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Yes, 75% overall survival for those w/complete resection of primary tumor
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How is complete resection made possible in hepatoblastoma?
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preoperative chemotherapy with commonly 5-FU (fluorouraci), VCR, Doxo, Cisplatin,
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What is correlated with poor prognosis in hepatoblastoma?
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- both lobes of liver involved w/tumor
- multifocal, disseminated liver mets - distant mets - vascular invasion - embryonal differentiation - serum alpha-fetoprotein (<100,000 or >100,000 |
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Where does recurrent disease of hepatoblastoma occur?
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Liver or lungs
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Is cure possible for hepatoblastoma metastatic disease?
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Yes, only if metastases are surgically resected
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What is the survival rate of hepatocellular carcinoma?
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10-20%
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What variant form of hepatocellular carcinoma is associated with high rate of surgical resectability and improved overall survival?
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Fibrolamellar
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Is hepatocellular carcinoma easy to perform a complete resection on?
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No, it is difficult to resect and to cure; even if complete resection is possible
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Is radiation or liver transplant an effective form of treatment for hepatocellular carcinoma?
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Radiation = little benefit
Liver transplant = being investigated |
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Where do germ cell tumors arise from?
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Primitive germ cells or embryonal cells
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Where can germ cell tumors occur?
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- gonads
- sites along migratory path of germ-cells (from yolk sac to gonads) |
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Is it common for germ cells to include more than one cell type?
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Yes; morphology depends on site of tumor and patient's age
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What are some germ cell tumor classifications?
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- teratomas
- germinomas - endodermal sinus tumors - choriocarcinomas - embryonal carcinomas |
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Germ cell tumors account for 1% of all childhood tumors, and are associated with what abnormalities?
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- abnormal sex chromosomes
- CNS abnormalities - genitourinary abnormalities - malformations of lower spine |
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What population does sacrococcygeal teratomas & ovarian tumors occur in?
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Sacrococcygeal = girls
Ovarian = postmenarchal girls |
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What 2 groups of males have a higher incidence of testicular tumors?
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Peak in Infancy & postpubertal
- incr'd incidence in boys with history of cryptorchidism |
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What common sites do germ-cell tumors often metastasize to?
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lungs, liver, regional nodes, & CNS
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Do germ cell often spread to bone marrow?
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NO
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What blood levels are often elevated when a patient has a germ-cell?
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1. alpha-fetoprotein levels
2. Beta HCG levels |
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What is the most common germ-cell tumor?
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Teratoma
- in infants appears w/large external masses (often sacrococcygeal) |
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How do ovarian germ-cell tumors often present?
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- abdominal pain & swelling
- palpable mass - nausea/vomiting - constipation - genitourinary symptoms |
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How do most testicular germ-cell tumors present?
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- painless irregular scrotal masses
often w/hydroceles or inguinal hernias |
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What is unique about the treatment of germ-cell tumors?
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Treatment is individualized d/t the wide variety of germ-cell tumors.
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When is surgical resection the treatment of choice in terms of germ-cell tumor treatment?
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benign germ-cell tumors like Teratoma
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When is surgical debulking or tumor biopsy the surgical treatment for germ-cell tumors?
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When malignant tumors cannot be resected without sacrificing vital structures or organs
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Why is chemotherapy given for treatment of germ-cell tumors?
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Chemo is giving initially to debulk
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What type of chemo is recommended for malignant lesions w/microscopic residual lymph-node disease or metastatic disease?
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Platinum-based chemotherapy
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QWhat are the 3 standard chemo drugs used to treat malignant germ-cell tumors?
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Cisplatin
Etoposide Bleomycin |
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What diagnostic characteristics have prognostic significance in a malignant germ-cell tumor?
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1. Site
2. Stage 3. Alpha-fetoprotein level |
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What is the percentage of current survival of germ cell tumor stages?
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Stages I & II gonadal sites= 100%
Stages III & IV gonadal sites = 95% Stages I & II extragonadal sites = 90% Stages III & IV extragonadal sites = 75% |
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What location of primary tumor sites for germ-cell tumors have been associated with improved prognosis?
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Retroperitoneal & testicular primary sites of disease
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What tumor sites have a poorer prognosis with germ-cell tumors?
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Sacrococcygeal & mediastinal tumors
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What is the origin a synovial sarcoma tumor?
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Fibrous and epithelial origins
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What population of patients are synovial sarcoma tumors most commonly seen?
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- more common in older children & young adults
- 31% of cases in pts <20yrs old |
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Where are synovial sarcomas most commonly located?
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Lower extremity - especially knee & thigh
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Where is the most common site of metastatic disease in synovial sarcoma?
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the lung
- can spread to regional lymph nodes |
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What is related to poor prognosis in synovial sarcoma?
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- presence of metastases
- tumor >5cm - tumor invasiveness - primary site other than hand, foot, or knee - older age at diagnosis - bone or neurovascular invasion - poor histologic differentiation |
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What rare pediatric tumor is most often seen in the orbit and in the head & neck, slow-growing, asymptomatic mass that doesn't produce symptoms?
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Alveolar Soft-Part Sarcoma
- a soft-tissue sarcoma of unclear histogenesis (15 to 35yrs - usual population) |
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Where is the most common site of metastases in alveolar soft-part sarcoma?
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#1: lung
then brain, bone, & lymph nodes |
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Why is it that most patients die of alveolar soft-part sarcoma?
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It grows slowly & recurrent disease is common & difficult to treat
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What is the common patient outcome when alveolar soft-part sarcoma has been diagnosed?
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80% of patients are alive at 2yrs post diagnosis;
but most die of the disease sometimes as long as 20yrs later |
