Renal Mass Flashcards
Clinical Vignette:
A 35-year-old male patient comes in for a follow-up regarding multiple clear cell renal cell carcinomas (RCC) as part of his von Hippel-Lindau (VHL) disease. During the clinic visit, you also discuss other associated tumors he may experience. His most recent renal imaging showed one renal mass of 2.5 cm.
Multiple-choice options:
A) Recommend immediate surgical excision
B) Advise active surveillance for the renal mass
C) Start the patient on medications targeting the HIF-VEGF pathway
D) Refer to a neurosurgeon for possible central nervous system hemangioblastomas
Correct Answer:
B) Advise active surveillance for the renal mass
In-depth explanation:
A: Incorrect. Surgical excision is generally recommended for masses ≥3 cm in VHL disease.
B: Correct. Active surveillance is the recommended management for renal tumors <3 cm in VHL disease.
C: Incorrect. While HIF-VEGF is a potential therapeutic target, medication is not the standard approach for a renal mass <3 cm.
D: Incorrect. The question is focused on the management of the renal mass, not other manifestations of VHL.
Memory Tool:
For VHL and renal masses, remember “3 cm Cut-Off” for deciding between surveillance and surgical intervention.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Understanding the management guidelines for renal masses in VHL disease is essential for appropriate patient care.
Clinical Vignette:
A 40-year-old female presents with cutaneous leiomyomas and is diagnosed with Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC). She has a 2 cm renal mass on imaging.
Multiple-choice options:
A) Start the patient on antioxidant medications
B) Perform surgical excision
C) Recommend active surveillance for the renal mass
D) Refer her to a dermatologist for skin lesion management
Correct Answer:
B) Perform surgical excision
In-depth explanation:
A: Incorrect. Although the antioxidant response pathway is a therapeutic target, medications are not the primary management for small renal masses in HLRCC.
B: Correct. In HLRCC, surgical excision is recommended, even for smaller masses.
C: Incorrect. Active surveillance is not advised for renal masses in HLRCC.
D: Incorrect. While skin lesions are a manifestation, the renal mass needs immediate attention.
Memory Tool:
HLRCC – ‘S’urgical ‘S’olution for ‘S’mall masses. All S’s align.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Management of renal tumors in HLRCC differs from other hereditary RCC syndromes, and recognizing this is crucial for optimal care.
Clinical Vignette:
A 50-year-old male, known to have Birt-Hogg-Dube (BHD) syndrome, comes in for his regular follow-up. He has multiple lung cysts and a new renal mass of 3.5 cm on recent imaging.
Multiple-choice options:
A) Recommend active surveillance for the renal mass
B) Immediate surgical excision
C) Refer for pulmonary function tests
D) Start treatment targeting the mTOR pathway
Correct Answer:
B) Immediate surgical excision
In-depth explanation:
A: Incorrect. Active surveillance is only advised for masses <3 cm in BHD syndrome.
B: Correct. Surgical excision is recommended for renal masses ≥3 cm in BHD syndrome.
C: Incorrect. Although lung cysts are a manifestation, the renal mass needs immediate attention.
D: Incorrect. mTOR pathway is a potential therapeutic target but is not recommended as the first-line approach for a 3.5 cm mass.
Memory Tool:
“BHD 3 cm Boundary” – surgical excision for ≥3 cm renal masses.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Correctly identifying the cutoff for surgical intervention in BHD syndrome will influence patient management and prognosis.
Clinical Vignette:
A 25-year-old woman presents with palpitations and shortness of breath. Further evaluation reveals malignant paragangliomas, and she is ultimately diagnosed with Succinate Dehydrogenase-deficient RCC (SDH-RCC). She has a 2 cm renal mass on imaging.
Multiple-choice options:
A) Start the patient on medications targeting the HIF-VEGF pathway
B) Advise active surveillance for the renal mass
C) Recommend surgical excision
D) Refer to an endocrinologist for further evaluation
Correct Answer:
C) Recommend surgical excision
In-depth explanation:
A: Incorrect. While HIF-VEGF pathway is a therapeutic target, medications are not the primary treatment for a renal mass in SDH-RCC.
B: Incorrect. Active surveillance is not recommended for SDH-RCC.
C: Correct. Surgical excision with wide margins is recommended in SDH-RCC, irrespective of the size.
D: Incorrect. Although an endocrinologist might manage paragangliomas, the renal mass requires immediate surgical attention.
Memory Tool:
SDH-RCC – ‘S’urgical ‘D’ecision for all ‘H’istologies and sizes.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Understanding the surgical necessity for SDH-RCC helps with immediate and effective management of the disease.
Clinical Vignette:
A 42-year-old woman with known PTEN hamartoma tumor syndrome presents for her annual follow-up. She has had benign breast tumors in the past. Recent imaging reveals a 1.5 cm renal mass.
Multiple-choice options:
A) Recommend a breast biopsy
B) Recommend active surveillance for the renal mass
C) Recommend surgical excision of the renal mass
D) Start the patient on mTOR pathway-targeting medications
Correct Answer:
B) Recommend active surveillance for the renal mass
In-depth explanation:
A: Incorrect. Breast tumors are an associated manifestation but not the focus here.
B: Correct. Active surveillance is recommended for renal masses <3 cm in PTEN hamartoma tumor syndrome.
C: Incorrect. Surgical excision is generally advised for masses ≥3 cm.
D: Incorrect. Although the mTOR pathway is a therapeutic target, it’s not the primary treatment for a renal mass <3 cm in this syndrome.
Memory Tool:
For PTEN, ‘P’atiently ‘T’rack < 3 cm renal masses.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
The nuances in management of renal masses in PTEN hamartoma tumor syndrome should be understood for patient-specific care.
Clinical Vignette:
A 30-year-old male with a history of tuberous sclerosis complex presents with seizures and skin lesions. Imaging shows multiple renal angiomyolipomas, the largest being 4 cm.
Multiple-choice options:
A) Start everolimus therapy
B) Consider embolization or surgical excision
C) Continue active surveillance
D) Refer to a dermatologist for skin lesion management
Correct Answer:
B) Consider embolization or surgical excision
In-depth explanation:
A: Incorrect. Everolimus is considered for angiomyolipomas between 3–5 cm, but for ≥5 cm, other interventions like embolization or surgical excision are preferred.
B: Correct. Embolization or surgical excision is recommended for angiomyolipomas ≥5 cm.
C: Incorrect. Active surveillance is advised for <3 cm masses.
D: Incorrect. While skin lesions are associated, the 4 cm angiomyolipoma takes precedence.
Memory Tool:
In TSC, “5 cm Shifts Gears” from medication to intervention.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Recognizing the thresholds for intervention in TSC is crucial to avoid complications such as hemorrhage.
Clinical Vignette:
A 38-year-old man presents with recurrent episodes of spontaneous pneumothorax. On further evaluation, multiple chromophobe RCCs and facial fibrofolliculomas are noted. The largest renal mass is 2.5 cm.
Multiple-choice options:
A) Refer the patient for immediate surgical excision of the renal mass
B) Advise active surveillance for the renal mass
C) Start targeted therapy for the mTOR pathway
D) Refer the patient for lung transplantation
Correct Answer:
B) Advise active surveillance for the renal mass
In-depth explanation:
A: Incorrect. Surgical excision is recommended for renal masses ≥3 cm in BHD.
B: Correct. Active surveillance is recommended for renal masses <3 cm.
C: Incorrect. mTOR pathway is a potential therapeutic target, but not the primary treatment for a renal mass of this size in BHD.
D: Incorrect. While lung cysts and pneumothorax are associated, they’re not the focus for this renal mass.
Memory Tool:
In BHD, “Below 3 cm, Be Happy to Delay” surgical intervention.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Understanding the subtleties in renal mass management in BHD can prevent unnecessary surgical intervention.
Clinical Vignette:
A 45-year-old man with a known genetic mutation of BAP1 presents with a 3.5 cm high-grade clear cell RCC.
Multiple-choice options:
A) Recommend active surveillance
B) Recommend immediate surgical excision
C) Refer the patient to genetic counseling
D) Start the patient on an experimental therapy
Correct Answer:
B) Recommend immediate surgical excision
In-depth explanation:
A: Incorrect. Active surveillance is not recommended for high-grade or larger masses in BAP1 syndrome.
B: Correct. Surgical excision with a preference for nephron-sparing approaches is recommended.
C: Incorrect. While genetic counseling is useful, it’s not the primary treatment modality for this clinical scenario.
D: Incorrect. Experimental therapies aren’t the recommended first-line treatment.
Memory Tool:
In BAP1, “Big And Problematic (≥ 3 cm and high-grade), Act Promptly” with surgical excision.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Knowing when surgical intervention is necessary in BAP1 tumor predisposition syndrome can significantly influence the prognosis.
Correct Answer:
B) Recommend immediate surgical excision
In-depth explanation:
A: Incorrect. Active surveillance is not recommended for high-grade or larger masses in BAP1 syndrome.
B: Correct. Surgical excision with a preference for nephron-sparing approaches is recommended.
C: Incorrect. While genetic counseling is useful, it’s not the primary treatment modality for this clinical scenario.
D: Incorrect. Experimental therapies aren’t the recommended first-line treatment.
Memory Tool:
In BAP1, “Big And Problematic (≥ 3 cm and high-grade), Act Promptly” with surgical excision.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Knowing when surgical intervention is necessary in BAP1 tumor predisposition syndrome can significantly influence the prognosis.
Correct Answer:
C) Refer the patient to genetic counseling
In-depth explanation:
A: Incorrect. Since MiTF is not well-defined in terms of histology, a biopsy may not be diagnostic.
B: Incorrect. The therapeutic targets for MiTF are yet to be determined.
C: Correct. Given the unknown nature of MiTF-associated cancer syndrome, genetic counseling is essential.
D: Incorrect. Surgical intervention isn’t recommended without a confirmed diagnosis.
Memory Tool:
For MiTF, “Mysteries in The Field” require genetic counseling.
Reference Citation:
Paragraph 1, TABLE 97.4
Rationale:
Understanding that MiTF-associated cancer syndrome is still not well-defined guides clinicians to adopt a conservative approach with genetic counseling.
Vignette: A 45-year-old male is diagnosed with renal cell carcinoma (RCC). Biopsy reports indicate a clear cell subtype. You are considering targeted molecular therapy and immunotherapy.
Options:
A) Clear cell RCC rarely responds to targeted molecular therapy and immunotherapy.
B) Clear cell RCC often responds to targeted molecular therapy and immunotherapy.
C) Clear cell RCC primarily originates from the collecting duct.
D) Clear cell RCC is hypovascular.
Correct Answer: B) Clear cell RCC often responds to targeted molecular therapy and immunotherapy.
Explanation:
A: Incorrect. Clear cell RCC often responds to targeted molecular therapy and immunotherapy.
B: Correct. Clear cell RCC often responds to targeted therapies, making them a viable option in treatment.
C: Incorrect. Clear cell RCC originates from the proximal tubule, not the collecting duct.
D: Incorrect. Clear cell RCC is described as hypervascular, not hypovascular.
Memory Tool: “Clear choice, Clear therapy” to remember that clear cell RCC responds well to targeted molecular therapy and immunotherapy.
Reference Citation: Table 97.9, WHO classification of tumours: pathology and genetics of tumours of the urinary system and male genital organs, Lyon, France, 2004, IARC Press.
Rationale: Clear cell RCC’s responsiveness to targeted therapies is clinically relevant for treatment planning.
Vignette: A patient with a familial history of renal carcinoma is found to have a papillary type 1 RCC on imaging. Genetic testing is being considered.
Options:
A) Fumarate hydratase (FH) gene mutation is commonly associated.
B) Altered MET proto-oncogene status is common in sporadic cases.
C) Loss of multiple chromosomes is a characteristic feature.
D) HLRCC is the most common familial form.
Correct Answer: B) Altered MET proto-oncogene status is common in sporadic cases.
Explanation:
A: Incorrect. Fumarate hydratase (FH) gene mutation is associated with HLRCC, not type 1 papillary RCC.
B: Correct. Altered MET proto-oncogene status is present in 81% of sporadic cases of type 1 papillary RCC.
C: Incorrect. Loss of multiple chromosomes is a feature of chromophobe RCC.
D: Incorrect. HPRC is more commonly associated with type 1 papillary RCC, not HLRCC.
Memory Tool: “Meet MET in Pap Type 1” to remember that MET proto-oncogene alteration is common in type 1 papillary RCC.
Reference Citation: Table 97.9, WHO classification of tumours: pathology and genetics of tumours of the urinary system and male genital organs, Lyon, France, 2004, IARC Press.
Rationale: Knowledge of genetic markers like MET can be crucial in both diagnosis and management strategies for RCC.
Clinical Vignette: A 45-year-old man comes to the clinic with complaints of flank pain. Imaging reveals a renal tumor. After a biopsy, the histology shows nests or sheets of clear cells with a delicate vascular network.
Multiple-choice options
A. Clear cell RCC
B. Papillary RCC Type 1
C. Papillary RCC Type 2
D. Chromophobe RCC
Correct Answer: A. Clear cell RCC
In-depth Explanation:
A: Correct. Clear cell RCC histology often presents with nests or sheets of clear cells with a delicate vascular network.
B: Incorrect. Papillary RCC Type 1 is characterized by papillary structures with a single layer of cells around fibrovascular cores and basophilic cells with low-grade nuclei.
C: Incorrect. Papillary RCC Type 2 also has papillary structures but with eosinophilic cells with high-grade nuclei.
D: Incorrect. Chromophobe RCC is characterized by “plant cells” with pale cytoplasm, perinuclear clearing, or “halo.”
Memory Tool: Think “Clear as Day” to remember clear cell RCC shows clear cells in nests or sheets.
Reference Citation: Paragraph 1, TABLE 97.9. Modified from Eble JN, et al.
Rationale for the Question: The question aims to test the understanding of histological features associated with different types of RCC, which is crucial for diagnosis.
Clinical Vignette: A 30-year-old woman with a family history of kidney tumors comes for genetic counseling. She is concerned about her risk of developing renal cell carcinoma. Her family has a known mutation in the VHL gene.
Multiple-choice options
A. Chromophobe RCC
B. Clear cell RCC
C. Papillary RCC Type 1
D. Unclassified RCC
Correct Answer: B. Clear cell RCC
In-depth Explanation:
A: Incorrect. Chromophobe RCC is commonly associated with Birt-Hogg-Dube syndrome, not VHL disease.
B: Correct. Clear cell RCC is commonly associated with VHL disease.
C: Incorrect. Papillary RCC Type 1 is associated with HPRC, not VHL.
D: Incorrect. Unclassified RCC has no known genetic factors at this time.
Memory Tool: Remember “VHL is Clearly a Bigger deal” to connect VHL with clear cell RCC.
Reference Citation: Paragraph 2, TABLE 97.9. Modified from Eble JN, et al.
Rationale for the Question: This question helps assess knowledge of the genetic factors linked with different RCC subtypes, critical for familial risk assessments.
Clinical Vignette: A pathologist reviews a kidney biopsy, which shows papillary structures with eosinophilic cells and high-grade nuclei. What can be said about the prognosis of this type of renal cell carcinoma?
Multiple-choice options
A. Good prognosis
B. Worse than type 1 but better than clear cell RCC
C. Worse than both type 1 and clear cell RCC
D. Generally good prognosis unless it’s a sarcomatoid variant
Correct Answer: C. Worse than both type 1 and clear cell RCC
In-depth Explanation:
A: Incorrect. This type (Papillary RCC Type 2) has a worse prognosis than Type 1 and clear cell RCC.
B: Incorrect. It has a worse prognosis when compared to both.
C: Correct. Papillary RCC Type 2 has a worse prognosis than both type 1 and clear cell RCC.
D: Incorrect. This statement is more appropriate for Chromophobe RCC.
Memory Tool: “Eosinophils Elevate Risk” to remember eosinophilic cells in Type 2 means worse prognosis.
Reference Citation: Paragraph 5, TABLE 97.9. Modified from Eble JN, et al.
Rationale for the Question: Assessing the understanding of prognosis related to histological features is essential for both diagnosis and patient counseling.
Question 4: Treatment of Metastatic RCC
Clinical Vignette: A 55-year-old man presents with metastatic renal cell carcinoma, and no contraindications for systemic therapy. What is the first-line treatment of choice?
Multiple-choice options
A. Pazopanib
B. Axitinib
C. Nivolumab
D. Sunitinib
Correct Answer: A. Pazopanib
In-depth Explanation:
A: Correct. Pazopanib is a first-line option for patients with metastatic RCC with no contraindications for systemic therapy.
B: Incorrect. Axitinib is generally used for refractory cases.
C: Incorrect. Nivolumab is typically used in later lines of treatment.
D: Incorrect. Sunitinib could also be a first-line treatment, but is not as favored due to its side-effect profile.
Memory Tool: Think “Pazo the First” to remember that Pazopanib is often a first-line treatment.
Reference Citation: Paragraph 3, TABLE 97.9. Modified from Eble JN, et al.
Rationale for the Question: This question gauges understanding of current first-line treatments for metastatic RCC, which is crucial for effective patient management.