10 Questions
What is the characteristic appearance of pheochromocytoma?
Dark brown black appearance caused by chromaffin oxidation of catecholamines
Which genes are associated with inherited germline mutation in pheochromocytoma?
RET, VHL, NF1, SDHD, SDHC or SDHS
What are the common clinical features of pheochromocytoma predominantly due to?
Secretion of catecholamines, both epinephrine and norepinephrine
What is the age range in which most pheochromocytoma patients are found?
20-60 years old
How are malignant pheochromocytomas diagnosed?
By metastasis rather than by morphology
Where do the vast majority of neuroblastoma cases occur?
In the abdomen
What gene mutation is associated with familial cases of neuroblastoma?
ALK (anaplastic lymphoma kinase) gene
What are the clinical manifestations of neuroblastoma related to?
Rapid local growth, metastatic spread, or development of hormonal syndrome
What can be detected in the 24-hour urine of a patient with neuroblastoma?
Catecholamines and its metabolites such as vanillyl mandelic acid (VMA) and homovanillic acid (HVA)
What syndrome may a patient develop due to the production of kinins or prostaglandins by the neuroblastoma?
Carcinoid-like syndrome
Study Notes
Pheochromocytoma Characteristics
- Pheochromocytoma has a characteristic appearance of a well-circumscribed, highly vascularized tumor with a variegated cut surface.
Genetic Associations
- Inherited germline mutations in pheochromocytoma are associated with genes such as RET, VHL, NF1, and SDHx.
Clinical Features
- The common clinical features of pheochromocytoma are predominantly due to excessive catecholamine production, leading to symptoms such as hypertension, headaches, and palpitations.
Age Range
- Most pheochromocytoma patients are found in the age range of 40-60 years.
Malignant Diagnosis
- Malignant pheochromocytomas are diagnosed by the presence of metastases, typically to the bones, liver, or lymph nodes.
Neuroblastoma Characteristics
- The vast majority of neuroblastoma cases occur in the adrenal medulla.
Genetic Associations
- Familial cases of neuroblastoma are associated with mutations in the ALK gene.
Clinical Manifestations
- The clinical manifestations of neuroblastoma are related to the production of catecholamines, leading to symptoms such as hypertension, diarrhea, and sweating.
Urine Analysis
- Vanylmandelic acid (VMA) and homovanillic acid (HVA) can be detected in the 24-hour urine of a patient with neuroblastoma.
Syndrome Association
- Patients with neuroblastoma may develop a syndrome due to the production of kinins or prostaglandins by the tumor, known as neuroblastoma-associated syndrome.
Test your knowledge of pheochromocytoma with this quiz. Learn about the characteristics, origins, and related terms of this adrenal medulla tumor.
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