11: Pediatric urologic oncology Flashcards
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FIG. 11.1 Magnetic resonance imaging (MRI) before and after chemotherapy showing marked reduction in size of right suprarenal neuroblastoma. (A) Before chemotherapy. (B) After chemotherapy.
What is the most common symptom of pediatric adrenal tumors?
A) Palpable abdominal mass
B) Chest pain
C) Headache
D) Joint pain
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.
What lab test is important for diagnosing neuroblastoma?
A) Complete metabolic profile
B) Complete blood count
C) Urinary levels of metabolites of catecholamines
D) Liver function tests
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.
What imaging is generally done after an abdominal mass is found in a pediatric patient?
A) Chest x-ray
B) Abdominal ultrasound
C) Magnetic resonance imaging (MRI)
D) Bone scan
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.
What is the classic finding for distinguishing a pediatric abdominal mass as neuroblastoma or nephroblastoma?
A) Presence of calcifications
B) Location of the mass
C) Size of the mass
D) Shape of the mass
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.
What is the prognosis for low-risk neuroblastoma patients?
A) >95% 5-year overall survival
B) 70%–90% 5-year overall survival
C) 20%–40% 5-year overall survival
D) <5% 5-year overall survival
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).
What are the symptoms of pediatric adrenal tumors and how is it diagnosed?
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.
What is neuroblastoma and how is it diagnosed?
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.
What is the treatment for neuroblastoma and what is the prognosis?
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.
What should be considered during the history and examination for a pediatric patient with an abdominal mass?
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.
What is the difference between neuroblastoma and nephroblastoma?
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.
Table 11.1
Hereditary Syndromes Associated with Pheochromocytoma
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FIG. 11.2 Magnetic resonance imaging (MRI) T2 of left adrenal pheochromocytoma: axial (A) and coronal (B).
Which of the following is a common symptom of pheochromocytoma?
A. Hypotension
B. Tachycardia
C. Pallor
D. Fatigue
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.
What is the recommended first step in the management of pheochromocytoma prior to surgery?
A. Beta blockade
B. Laparoscopic resection
C. Alpha blockade
D. Biopsy
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.
What is the recommended surgical approach for tumors <8 cm in pheochromocytoma?
A. Open resection
B. Robotic resection
C. Laparoscopic resection
D. Endoscopic resection
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.
What are the common symptoms of pheochromocytoma, and why is a personal or family history of genetic predispositions important in the diagnosis of this condition?
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.
What is the recommended diagnostic workup for pheochromocytoma, and what is the role of plasma free metanephrines in the diagnosis of this condition?
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.
What is the recommended management of pheochromocytoma prior to surgery, and what is the preferred surgical approach for tumors <8 cm?
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
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FIG. 11.3 Unilateral pediatric renal mass algorithm. AWT, Anaplastic Wilms Tumor; CAP, Chest, Abdomen, Pelvis; CMN, Congenital Mesoblastic Nephroma; CT, Computed Tomography; FH, Favorable Histology; LN, Lymph Node; RCC, Renal Cell Carcinoma; VLR, Very Low Risk; WT, Wilms Tumor; XRT, Radiation Therapy.
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FIG. 11.4 Bilateral pediatric renal mass algorithm. AP, Abdomen Pelvis; C, Chest; CAP, Chest, Abdomen, Pelvis; CT, Computed Tomography; LN, Lymph Node; MRI, Magnetic Resonance Imaging; NSS, Nephron Sparing Surgery; RN, Radical Nephrectomy US; Ultrasound; VAD, Vincristine, Actinomycin, Doxorubicin; WT, Wilms Tumor.
Table 11.2
Pediatric Renal Tumor Overview
Table 11.3
Hereditary Syndromes Associated with Wilms Tumor
FIG. 11.5 (A) Computed tomography of a Wilms tumor that was pretreated with chemotherapy. (B) After 6 weeks of chemotherapy, the tumor is much smaller in size.
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FIG. 11.6 Patient with bilateral tumors who was treated with chemotherapy. (A) Computed tomography (CT) before treatment. (B) CT after 12 weeks of chemotherapy, revealing only minimal decrease in the size of the tumors. Bilateral partial nephrectomies were performed, revealing mature tumor elements with rhabdomyoblastic differentiation.
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FIG. 11.7 Postoperative image from patient shown in Fig. 11.6. Demonstrates that the kidneys have pretty near normal volume after resection of the bilateral tumors.
What is the most common presenting symptom of Wilms tumor in children?
A. Headache
B. Vomiting
C. Palpable abdominal mass
D. Joint pain
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.
What laboratory test should be done for a child suspected of having Wilms tumor?
A. Complete blood count
B. Urine analysis
C. Blood glucose test
D. Coagulation profile
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.
What is the treatment of choice for most patients with a unilateral, nonsyndromic Wilms tumor?
A. Biopsy
B. Chemotherapy
C. Radiation therapy
D. Radical nephrectomy and lymph node sampling
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.
What is Wilms tumor and what are its symptoms?
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.
What should be included in the history and examination of a child suspected of having Wilms tumor?
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).
What is von Willebrand disease?
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.
Table 11.4
Hereditary Syndromes Associated with Renal Cell Carcinoma
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FIG. 11.8 Magnetic resonance imaging of abdomen and pelvis in boy with bladder-prostate rhabdomyosarcoma.
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FIG. 11.9 Botryoid vaginal rhabdomyosarcoma emanating from the introitus of a young girl.
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FIG. 11.10 Testicular ultrasound of a pure embryonal carcinoma in a 14-year-old male patient.
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FIG. 11.11 Testicular ultrasound of a Leydig cell tumor in a 15-year-old male patient.
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FIG. 11.12 (A) Leydig cell tumor demonstrating characteristic brown appearance related to abundant lipofuscin pigmentation. (B) Reinke crystals. Source: (A, Courtesy Fernando Ferrer, MD; B, from http://www.webpathology.com.)
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FIG. 11.13 Testicular ultrasound of a paratesticular rhabdomyosarcoma in a 2-year-old male patient. (Yellow star indicates the large paratesticular mass. Red arrow indicates the normal testicle adjacent to the mass.
