4 - peds oncology Flashcards

Question

wilms tumor staging called

Answer 1

NTWS (nitwit's)

Answer 2

stage 1: confined, total resection. Stage 2: outside capsule, total resection. Stage 3: incomplete resecton or biopsy, any spill, + LN. Stage 4: hematogenous spread. Stage 5: bilateral tumors

Answer 3

1. transperitoneal nx, 2. don’t need to explore contralateral kidney (ct's are better now), 3. selective LN sampling, not RPLND

Answer 4

tumor spillage, unfavorable histology, incomplete resection, absence of LN sampling

Answer 5

no open bx, just do upfront chemo for tumor shrinkage and pnx.

Answer 6

if response - do pnx. If NO response - bilateral open biopsy. At 2nd look PNX if 2/3 kidney can be preseerved. do NX if unfavorable histology or chemo failure

Answer 7

bilateral, vascular invasion, unresectable tumor, solitary kidney

Answer 8

stage 1 or 2 favorable or unfavorbale histology

Answer 9

infertility, hypogonadism, 2nd malignancy, CHF (doxorubicin)

Answer 10

mesoblastic nephroma

Answer 11

nx is curative

Answer 12

3% kids renal tumors, **bone mets common**, multimodal tx needed

Answer 13

doxorubicin

Answer 14

2% kids renal tumors, ** brain mets common**, very bad

Answer 15

chemo resistant, advanced stage, high mortality

Answer 16

most commin 2nd decade, papillary most common, 5% kids

Answer 17

most comm extracranial solid tumor in kids,

Answer 18

yes - 50% with mets

Answer 19

presents anywhere along symp chain (75% retroperitoneum)

Answer 20

neural crest, like pheo

Answer 21

n-myc amplification in 20% and poor prognostic marker

Answer 22

autosomal dominant

Answer 23

racoon eyes (periorbital mass), sick appearing, anemia (bm mets), blueberry muffin spots on trunk, Opsoclonus-Myoclonus

Answer 24

urine catecholamine (VMA, HVA) in upto 90%, BM bx

Answer 25

BETTER prognosis: age < 1 (best prognosis), nonadrenal origin, tumor stage.

Answer 26

WORSE: N-MYC amplification, elevated serum ferritin

Answer 27

1 - localized tumor, 2 - unilteral tumor w gross total resection and neg LN. 2b - stage 2 w/ ipsilateral +LN only. 3 - tumor crosses midline OR + contralat LN. 4 - distant LN + or mets to BM, bone, or liver

Answer 28

adrenal tumor origin and those < 1 yo

Answer 29

mets to liver, skin, or BM, but NOT cortical bone

Answer 30

90% survival, ** may spontaneously regress**

Answer 31

stage 1, 2, or 4s

Answer 32

N-MYC neg, no diploidy, favorable histology

Answer 33

only surg, 95% survival

Answer 34

stage 3 and 4, or 4s if symptomatic AND unfavorable histology

Answer 35

N-MYC neg, < 18 mo old

Answer 36

surg + chemo

Answer 37

all stages if N-MYC +

Answer 38

chemo + surg +/- rad. Survival - 20-40%

Answer 39

most comm soft tissue sarcoma < 15 yo, 25% GU tract

Answer 40

mesenchyma

Answer 41

2q37 locus

Answer 42

bimodal - < 10yo and late adolescence

Answer 43

neurofibromatosis

Answer 44

embryonal and botryoid (grapes) - younger pts and better prognosis. alveolar

Answer 45

surgery is diagnostic. Chemo is mainstay. Radiation is controversial b/c long term effects

Answer 46

organ preservation

Answer 47

VAC - vincristine, actinomycin D, cyclophosphamide

Answer 48

do not place SPT (seeding). Foley until tumor shrinkage w chemo

Answer 49

PET scan differentiates fibrosis from tumor. Residual disease is RF for local recurrence.

Answer 50

all get orchiectomy (as opposed to just bx) then chemo. +/- RPLND

Answer 51

kids > 10 yo w neg LN on CT should get ipsilateral RPLND prior to chemo. If + LN - chemo +/- rplnd

Answer 52

bimodal - < 2 yo and young adulthood

Answer 53

ct CHEST, abdomen and pelvis

Answer 54

AFP only, no HCG as no pediatric tumors make this

Answer 55

embryonal, choriocarcinoma, seminoma

Answer 56

elevated - normal by 6 months

Answer 57

90% yolk sack

Answer 58

> 6 mo and elev AFP

Answer 59

most pre-pubertal.

Answer 60

Clamp vessels, frozen section of bx. Remove tumor alone if benign

Answer 61

CIS present in most POST-pubertal, rare in PRE-pubertal (1 case).

Answer 62

stage 1 - observation. Mets/rec - chemo. RPLND if postchemo mass

Answer 63

remains elevated for 6-9 months postpartum

Answer 64

precocious puberty, testis mass, elevated serum testosterone and urinary 17-ketosteroids

Answer 65

high testosterone, low-nl gonadotropins

Answer 66

testis sparing sx if possible

Answer 67

usu hormonally inactive, but can see gynecomastia

Answer 68

orchiectomy. r/o mets if aggressive histology. Benign tumor

Answer 69

1/3 syndromic - putz-jeghers, carney's synd

Answer 70

benign - orchiectomy

Answer 71

1st yr of life

Answer 72

y chr abnormalities

Answer 73

benign, rare, testis sparing

Answer 74

dysgenetic gonad WITH y-chromosome

Answer 75

streak, dysgenetic, indeterminate

Answer 76

have germ cell + stromal elements

Answer 77

10% malig after puberty. Remove early while benign

Answer 78

germ cell elements outgrow stromal components after puberty --> dysgerminoma develops

Answer 79

dysgerminoma (seminoma)

Answer 80

4-6x increased risk

Answer 81

higher up, the higher the likelyhood of malignancy

Answer 82

seminoma before ox, NSGCT after ox

Answer 83

orchidopexy BEFORE puberty decreases ca risk

Answer 84

A) Palpable abdominal mass Explanation: The most common symptom of pediatric adrenal tumors is a palpable abdominal mass. Other symptoms may include pain or focal symptoms from metastatic disease, incidental finding from imaging, hypertension, and symptoms of catecholamine excess.

Answer 85

C) Urinary levels of metabolites of catecholamines Explanation: Urinary levels of metabolites of catecholamines, vanillylmandelic acid (VMA) and homovanillic acid (HVA), are important for the diagnosis of neuroblastoma.

Answer 86

B) Abdominal ultrasound Explanation: Abdominal ultrasound is generally done to guide additional cross-sectional imaging after an abdominal mass is found in a pediatric patient.

Answer 87

A) Presence of calcifications Explanation: The classic finding for distinguishing a pediatric abdominal mass as neuroblastoma or nephroblastoma is whether it crosses midline or if it has calcifications. Classically, but not always, neuroblastoma crosses the midline and may have calcifications while nephroblastoma does not generally cross midline or have calcifications.

Answer 88

A) >95% 5-year overall survival Explanation: The prognosis for neuroblastoma patients is highly dependent on risk status, which combines pathologic features, stage, and patient age. Low risk has a >95% 5-year overall survival (OS).

Answer 89

What are the symptoms of pediatric adrenal tumors and how is it diagnosed? Pediatric adrenal tumors may present with a variety of symptoms, including a palpable abdominal mass, pain or focal symptoms from metastatic disease, incidental finding from imaging, hypertension, symptoms of catecholamine excess (tachycardia, anxiety, headaches, seizures), opsoclonus-myoclonus, and urinary retention. To diagnose pediatric adrenal tumors, urinary levels of metabolites of catecholamines, vanillylmandelic acid (VMA) and homovanillic acid (HVA), and plasma free metanephrines are important. A complete metabolic profile and a complete blood count (CBC) should also be checked. Abdominal ultrasound can guide additional cross-sectional imaging. If an abdominal mass is found, the next step is generally a computed tomography (CT) of the chest, abdomen, and pelvis.

Answer 90

Neuroblastoma is a type of pediatric urologic oncology that requires multimodal treatment with surgery, chemotherapy, and radiation. The first step in patient management is stabilization, as infants with widely metastatic neuroblastoma may be very ill. In general, the next step for a suspected neuroblastoma is biopsy. However, this should only be done after a multidisciplinary discussion with pediatric oncology. Subsequent steps with chemotherapy or surgical resection may require a nuanced analysis of patient and tumor factors. Symptoms of neuroblastoma may include a palpable abdominal mass, pain or focal symptoms from metastatic disease, incidental finding from imaging, hypertension, and symptoms of catecholamine excess (tachycardia, anxiety, headaches, seizures), as well as opsoclonus-myoclonus and urinary retention. To diagnose neuroblastoma, urinary levels of metabolites of catecholamines, vanillylmandelic acid (VMA) and homovanillic acid (HVA), are important. Abdominal ultrasound can guide additional cross-sectional imaging. If an abdominal mass is found, the next step is generally a computed tomography (CT) of the chest, abdomen, and pelvis. Further specialized imaging such as metaiodobenzylguanidine (MIBG) or positron emission tomography (PET) may be warranted. The classic finding for distinguishing a pediatric abdominal mass as neuroblastoma or nephroblastoma is whether it crosses midline or if it has calcifications.

Answer 91

Neuroblastoma generally requires multimodal treatment with surgery, chemotherapy, and radiation. The first step in patient management is stabilization, as infants with widely metastatic neuroblastoma may be very ill. In general, the next step for a suspected neuroblastoma is biopsy. However, this should only be done after a multidisciplinary discussion with pediatric oncology. Subsequent steps with chemotherapy or surgical resection may require a nuanced analysis of patient and tumor factors. The prognosis for neuroblastoma patients is highly dependent on risk status, which combines pathologic features, stage, and patient age. Low risk has a >95% 5-year overall survival (OS). Intermediate risk has a 70%–90% 5-year OS, and high risk has a 20%–40% 5-year OS.

Answer 92

During the history and examination for a pediatric patient with an abdominal mass, the patient/family should be asked about the duration and acuity of symptoms. Was there any preceding event? The age of the patient is important in prognosis. Thorough examination should assess for any abdominal or pelvic mass as well as any localized areas of tenderness or neurologic deficit. Check for periorbital ecchymosis and any signs of opsoclonus-myoclonus. It is also important to assess whether the child appears overall well or ill appearing, as children with neuroblastoma may be ill appearing or have unstable vital signs (tachycardic, hypotensive, tachypneic) at the time of diagnosis.

Answer 93

Neuroblastoma and nephroblastoma are both types of pediatric urologic oncology that can present with a palpable abdominal mass in children, but they are different types of tumors. Neuroblastoma arises from the neural crest cells, which are embryonic cells that form the sympathetic nervous system, while nephroblastoma (also known as Wilms tumor) arises from the embryonic kidney. The classic finding for distinguishing a pediatric abdominal mass as neuroblastoma or nephroblastoma is whether it crosses midline or if it has calcifications. Classically, but not always, neuroblastoma crosses the midline and may have calcifications while nephroblastoma does not generally cross midline or have calcifications. In addition, the treatment and prognosis for neuroblastoma and nephroblastoma are different. Neuroblastoma generally requires multimodal treatment with surgery, chemotherapy, and radiation. The prognosis for neuroblastoma patients is highly dependent on risk status, which combines pathologic features, stage, and patient age. Low risk has a >95% 5-year overall survival (OS), intermediate risk has a 70%–90% 5-year OS, and high risk has a 20%–40% 5-year OS. Nephroblastoma is generally treated with a combination of surgery and chemotherapy. The prognosis for nephroblastoma patients is also dependent on factors such as age and tumor stage, but the overall survival rate is high, with over 90% of patients surviving at least 5 years.

Answer 94

B. Tachycardia Explanation: Patients with pheochromocytoma typically present with hypertension, attacks of hypertension/anxiety, and symptoms of catecholamine excess such as tachycardia, anxiety, headaches, seizures, pallor, tremor, and perspiration.

Answer 95

C. Alpha blockade Explanation: Prior to surgery, management with endocrinology for catecholamine blockade is necessary. Alpha blockade (phenoxybenzamine or prazosin) should be done first. Beta blockade is only indicated if persistent arrhythmia or tachycardia or hypertension.

Answer 96

C. Laparoscopic resection Explanation: After a 10- to 14-day blockade, surgery can be done with laparoscopic resection preferred for tumors <8 cm. Early ligation of the adrenal vein will aid in patient stability. Close collaboration with anesthesia is necessary as patients can be very hemodynamically labile during surgery.

Answer 97

Patients with pheochromocytoma typically present with hypertension, attacks of hypertension/anxiety, and symptoms of catecholamine excess such as tachycardia, anxiety, headaches, seizures, pallor, tremor, and perspiration. A personal or family history of genetic predispositions such as von Hippel Lindau, multiple endocrine neoplasia, neurofibromatosis, or succinate dehydrogenase mutations is important in the diagnosis of this condition because these conditions are associated with a higher risk of developing pheochromocytoma.

Answer 98

The recommended diagnostic workup for pheochromocytoma includes a thorough investigation of symptoms and a personal or family history of genetic predispositions, as well as vital signs (heart rate, blood pressure), a complete metabolic profile, and a CBC. If plasma free metanephrines are elevated, the next step is generally a CT or MRI of the abdomen and pelvis. Further specialized imaging such as nuclear medicine imaging (MIBG, Dotatate, or PET) may be warranted. Plasma free metanephrines are critical for diagnosis as they are highly sensitive and specific for pheochromocytoma.

Answer 99

Prior to surgery, management with endocrinology for catecholamine blockade is necessary. Alpha blockade (phenoxybenzamine or prazosin) should be done first. Beta blockade is only indicated if persistent arrhythmia or tachycardia or hypertension. Increased fluid

Answer 100

C. Palpable abdominal mass Explanation: Wilms tumor often presents as a palpable abdominal mass, which can be accompanied by other symptoms such as hematuria, fever, anorexia, weight loss, and constipation.

Answer 101

D. Coagulation profile Explanation: Wilms tumor can cause an acquired von Willebrand factor deficiency in 2% of patients, so it is important to assess the coagulation profile in addition to other laboratory tests such as a complete metabolic profile and CBC.

Answer 102

D. Radical nephrectomy and lymph node sampling Explanation: Surgical intervention is generally the next step for patients with a unilateral, nonsyndromic Wilms tumor. The treatment of choice is radical nephrectomy and lymph node sampling, and biopsy is generally not indicated except in extenuating circumstances.

Answer 103

Wilms tumor, also known as nephroblastoma, is a type of kidney cancer that primarily affects children. The most common symptom of Wilms tumor is a palpable abdominal mass, which may be accompanied by other symptoms such as hematuria (blood in the urine), fever, anorexia (loss of appetite), weight loss, and constipation.

Answer 104

In addition to assessing the duration and acuity of symptoms, the patient/family should be asked about personal or family history of genetic predispositions, as some conditions such as hemihypertrophy and aniridia are associated with an increased risk of developing Wilms tumor. During examination, the child's overall appearance should be assessed to determine whether they are well or ill appearing. Thorough examination should assess for any abdominal or pelvic mass and for signs of hereditary predisposition, such as genitourinary anomalies (hypospadias, undescended testis, ambiguous genitalia).

Answer 105

Von Willebrand disease is a genetic bleeding disorder that affects the body's ability to form blood clots. It is caused by a deficiency or dysfunction of von Willebrand factor, a protein that is important for platelet function and blood clotting. Symptoms of von Willebrand disease can include easy bruising, nosebleeds, prolonged bleeding after injury or surgery, and heavy menstrual bleeding.

Answer 106

Factor V Leiden is also a genetic disorder that affects blood clotting. It is caused by a mutation in the Factor V gene, which results in a form of Factor V that is resistant to inactivation by a protein called activated protein C. This can lead to an increased risk of blood clots, particularly in the veins. Symptoms of Factor V Leiden can include deep vein thrombosis (DVT), pulmonary embolism, and recurrent miscarriage.

Answer 107

Hemophilia A and B are bleeding disorders caused by deficiencies in clotting factors VIII and IX, respectively. These factors are important for the formation of blood clots, so deficiencies can result in prolonged bleeding after injury or surgery, as well as spontaneous bleeding in severe cases. Hemophilia A and B are both genetic disorders, with hemophilia A being more common than hemophilia B. Von Willebrand disease, on the other hand, is caused by a deficiency or dysfunction of von Willebrand factor, a protein that is important for platelet function and blood clotting. While von Willebrand disease can result in similar symptoms as hemophilia A and B, such as easy bruising and prolonged bleeding after injury, the bleeding tendencies in von Willebrand disease are generally milder and more variable. In terms of treatment, hemophilia A and B are typically managed with clotting factor replacement therapy, while von Willebrand disease may be treated with desmopressin or von Willebrand factor replacement therapy. Hemophilia A and B are also more commonly associated with joint damage and other complications due to the frequent bleeding episodes.

Answer 108

C Explanation: The common symptoms of renal cell carcinoma in children include palpable abdominal mass and hematuria, among others.

Answer 109

B Explanation: Abdominal ultrasound can help guide additional cross-sectional imaging. If an abdominal mass is found, the next step is generally a CT of the chest, abdomen, and pelvis.

Answer 110

C Explanation: Radical nephrectomy with lymph node sampling is the treatment of choice in most patients with a unilateral, non-syndromic tumor. Biopsy is generally not indicated outside of extenuating circumstances.

Answer 111

The common presenting symptoms of renal cell carcinoma in children include a palpable abdominal mass, hematuria, fever, anorexia, weight loss, and constipation. The patient/family should be asked about duration and acuity of symptoms, and about known personal or family history of genetic predispositions. Thorough examination should assess for any abdominal or pelvic mass, and assess for signs of hereditary predisposition. A complete metabolic profile and a CBC should be done. Abdominal ultrasound can help guide additional cross-sectional imaging. If an abdominal mass is found, the next step is generally a CT of the chest, abdomen, and pelvis.

Answer 112

Radical nephrectomy with lymph node sampling is the treatment of choice in most patients with a unilateral, non-syndromic tumor. Biopsy is generally not indicated outside of extenuating circumstances. Those with bilateral renal tumors or a syndrome-associated unilateral tumor concerning for renal cell carcinoma should be treated with attempted partial nephrectomy. Complete surgical resection is the treatment of choice, and there is no role for adjuvant therapy in nonmetastatic disease. Prognosis is dependent on stage. The majority of renal cell carcinoma in children is translocation type RCC. These generally involve chromosomal translocations in Xp11 (TFE gene). Five-year OS by stage: stages I and II, >90%; stage III, 80%; and stage IV, <25%.

Answer 113

C. Hematuria Explanation: Hematuria, or blood in the urine, is a common symptom of bladder or prostate tumors in children.

Answer 114

B. Biopsy of the primary tumor Explanation: The next step in evaluation if a pelvic mass is found in a child is to perform a biopsy of the primary tumor, ideally achieved endoscopically.

Answer 115

C. Organ preservation Explanation: A major goal of treatment for pediatric urologic oncology is organ preservation and avoiding the morbidity of complete urinary diversion.

Answer 116

Children with bladder or prostate tumors may present with symptoms such as urinary frequency/urgency, stranguria, hematuria, constipation, palpable abdominal mass, fever, anorexia, and weight loss. These symptoms are significant because they can indicate the presence of a serious condition and should prompt further evaluation and management.

Answer 117

The recommended approach to evaluating a child with a pelvic mass includes assessing for abdominal or pelvic mass, performing a urethral meatus exam, and evaluating for urinary retention. In addition, a complete metabolic profile and a CBC should be obtained, and imaging such as abdominal US and CT of the chest, abdomen, and pelvis may be necessary. This approach is important because it helps to identify the underlying cause of the pelvic mass and guide appropriate treatment.

Answer 118

Treatment options for pediatric urologic oncology may include surgical excision, chemotherapy, and radiation. The choice of treatment is determined based on the ability to fully resect the tumor at diagnosis, the stage and location of the tumor, and the goals of treatment such as organ preservation and avoidance of morbidity. A multidisciplinary discussion with pediatric oncology and radiation oncology is necessary to determine the best course of treatment for each individual patient.

Answer 119

c. Vaginal bleeding Explanation: Vaginal bleeding is a common symptom of gynecologic rhabdomyosarcoma. Other symptoms include urinary frequency/urgency, stranguria, hematuria, constipation, palpable abdominal mass, fever, anorexia, and weight loss.

Answer 120

c. The abdominal and pelvic area Explanation: During the examination, a thorough assessment should be made for any abdominal or pelvic mass. An exam of the introitus may visualize a prolapsing vaginal mass. Urinary retention should also be evaluated.

Answer 121

c. Both A and B Explanation: If a pelvic mass is found, the next step is generally a CT of the chest, abdomen, and pelvis. MRI of the pelvis may also be helpful for fine anatomic details.

Answer 122

b. Organ preservation and avoiding morbidity Explanation: The major goals of treatment are organ preservation and avoiding the morbidity of complete pelvic exenteration. Many patients are managed with radiation to preserve the vagina/uterus.

Answer 123

: c. >80% Explanation: The 5-year overall survival rate for gynecologic rhabdomyosarcoma is >80%. It is >90% in those younger than 10 years old and 70% in those older than 10 years old.

Answer 124

The common symptoms of gynecologic rhabdomyosarcoma include vaginal bleeding, urinary frequency/urgency, stranguria, hematuria, constipation, palpable abdominal mass, fever, anorexia, and weight loss. During the history taking process, it is important to ask about personal or family history of genetic predispositions (such as Li-Fraumeni and neurofibromatosis) because some genetic conditions increase the risk for developing certain cancers, including gynecologic rhabdomyosarcoma.

Answer 125

During the examination, a thorough assessment should be made for any abdominal or pelvic mass. An exam of the introitus may visualize a prolapsing vaginal mass. Urinary retention should also be evaluated. Labs that are recommended include a complete metabolic profile and a CBC. Abdominal US can help guide additional cross-sectional imaging. If a pelvic mass is found,

Answer 126

Symptoms: Palpable scrotal/testicular mass or swelling, scrotal/testicular pain, dysuria, or focal symptoms from metastatic disease (cough, bone pain, neurologic deficits) History: Duration and acuity of symptoms, history of cryptorchidism, family history of testicular cancer and genetic syndromes (Klinefelter syndrome, DICER-1, Peutz-Jeghers syndrome, Carney complex, congenital adrenal hyperplasia) Examination: Evaluate the scrotum, testes, and paratesticular structures and presence or absence of varicocele. Perform an abdominal and pelvic exam to assess for any abdominal masses. Assess for any signs of precocious puberty or gynecomastia.

Answer 127

Serum tumor markers should be checked, which include alpha fetoprotein (AFP), beta-human chorionic gonadotropin (bHCG), and lactate dehydrogenase (LDH). Elevated levels of these markers can indicate the presence of a tumor, and can also be used to monitor treatment response.

Answer 128

Scrotal US can localize the tumor to the testis or paratesticular structures. CT of the chest, abdomen, and pelvis can be used to stage the tumor. Germ cell tumors may be associated with concomitant microlithiasis

Answer 129

Symptoms of paratesticular rhabdomyosarcoma include a palpable scrotal/testicular mass or swelling, scrotal/testicular pain, dysuria, or focal symptoms from metastatic disease (cough, bone pain, neurologic deficits).

Answer 130

During a physical examination, the scrotum, testes, and paratesticular structures should be evaluated, as well as the varicocele. An abdominal and pelvic exam should also be performed to assess for any abdominal masses. Signs of precocious puberty or gynecomastia should also be evaluated.

Answer 131

If there is concern for a testicular stromal tumor, serum tumor markers such as inhibin-B, testosterone, and estradiol should be drawn. To help distinguish from a germ cell tumor, AFP, bHCG, and LDH are recommended. A complete metabolic profile and a CBC should also be checked.

Answer 132

Scrotal US can localize the tumor to the testis or paratesticular structures and may also evaluate the contralateral testis. If pathologic is concerning for malignancy, the next step in imaging is full staging with CT of the chest, abdomen, and pelvis. Staging for rhabdomyosarcoma will also include a PET scan.

Answer 133

Paratesticular rhabdomyosarcoma is initially treated with a radical, inguinal orchiectomy. All patients receive adjuvant chemotherapy. Boys younger than 10 years old with no metastatic disease on imaging do not need a staging retroperitoneal lymph node dissection prior to adjuvant chemotherapy. However, those who are 10 years of age or