Quiz-summary
0 of 1 questions completed
Questions:
- 1
Information
Presented by Dr. Andres Matoso and prepared by Dr. Yembur Ahmad
This case talks about an adult man with a kidney mass.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
- Kidney 0%
- 1
- Answered
- Review
-
Question 1 of 1
1. Question
An adult male presents with a kidney mass.
What is the diagnosis?
Correct
Correct: D
Histology: The tumor is well circumscribed and surrounded by a thick fibrous capsule. Tumor cells have a primitive appearance and are columnar in areas of tubule formation. Mitoses are frequent.
Discussion: Wilms tumor, also known as nephroblastoma, is the most common kidney malignancy in children, occurring mainly in the first 5 years of life. Wilms tumor is very rare in adults with an incidence of about 0.2 per million per year. Histologically, the Wilms tumors are comprised of blastemal, stromal and epithelial elements, but cases can be monophasic or biphasic. The blastemal elements can be present in nodular fashion or have a syncytial pattern. Spindle cells are the most common form of stromal component but some tumors can show heterologous elements including skeletal muscle, smooth muscle, adipose tissue, cartilage, osteoid/bone, glial tissue, and squamous, ciliated, and glandular epithelium (teratoid Wilms tumors). Classical triphasic WT rarely presents diagnostic difficulty for pathologists, but when only one component is present, especially in a small biopsy specimen, the differential diagnosis may include renal cell carcinoma, metanephric adenoma and hyperplastic nephrogenic rest for epithelial elements, and clear cell sarcoma of the kidney, mesoblastic nephroma and synovial sarcoma for stromal elements. Pure blastemal-type WT may be difficult to distinguish from other embryonal small round blue cell tumors, including neuroblastoma, Ewing sarcoma, desmoplastic small round cell tumor and lymphoma. The presence of anaplasia defined as nuclear sized 3× tumor cells and tripolar mitosis is associated with high risk. All the three components, though usually blastema, can become anaplastic, leading to the diagnosis of either focal or diffuse anaplasia. WT with diffuse anaplasia and WT with blastemal predominance (after preoperative chemotherapy) are regarded as high-risk tumors and require more aggressive treatment. By immunohistochemistry, tumor cells are positive for WT1, PAX8 and CD56 and negative for CK7.
References:
Nat Rev Urol 2018 Nov;15(11):693-701
J Kidney Cancer VHL. 2021 Jul 20;8(2):40-48.
Pediatr Surg 2019 Mar;54(3):390-397.Incorrect
Correct: D
Histology: The tumor is well circumscribed and surrounded by a thick fibrous capsule. Tumor cells have a primitive appearance and are columnar in areas of tubule formation. Mitoses are frequent.
Discussion: Wilms tumor, also known as nephroblastoma, is the most common kidney malignancy in children, occurring mainly in the first 5 years of life. Wilms tumor is very rare in adults with an incidence of about 0.2 per million per year. Histologically, the Wilms tumors are comprised of blastemal, stromal and epithelial elements, but cases can be monophasic or biphasic. The blastemal elements can be present in nodular fashion or have a syncytial pattern. Spindle cells are the most common form of stromal component but some tumors can show heterologous elements including skeletal muscle, smooth muscle, adipose tissue, cartilage, osteoid/bone, glial tissue, and squamous, ciliated, and glandular epithelium (teratoid Wilms tumors). Classical triphasic WT rarely presents diagnostic difficulty for pathologists, but when only one component is present, especially in a small biopsy specimen, the differential diagnosis may include renal cell carcinoma, metanephric adenoma and hyperplastic nephrogenic rest for epithelial elements, and clear cell sarcoma of the kidney, mesoblastic nephroma and synovial sarcoma for stromal elements. Pure blastemal-type WT may be difficult to distinguish from other embryonal small round blue cell tumors, including neuroblastoma, Ewing sarcoma, desmoplastic small round cell tumor and lymphoma. The presence of anaplasia defined as nuclear sized 3× tumor cells and tripolar mitosis is associated with high risk. All the three components, though usually blastema, can become anaplastic, leading to the diagnosis of either focal or diffuse anaplasia. WT with diffuse anaplasia and WT with blastemal predominance (after preoperative chemotherapy) are regarded as high-risk tumors and require more aggressive treatment. By immunohistochemistry, tumor cells are positive for WT1, PAX8 and CD56 and negative for CK7.
References:
Nat Rev Urol 2018 Nov;15(11):693-701
J Kidney Cancer VHL. 2021 Jul 20;8(2):40-48.
Pediatr Surg 2019 Mar;54(3):390-397.
