Practice questions answers renal neoplasms
Practice questions answers
Renal neoplasms
© Jun Wang, MD, PhD
1. A. A benign tumor
with three components of thick-walled distorted vessel, muscular stroma and
adipose tissue is consistent with angiomyolipoma.
The muscular differentiation of the spindle cells is supported by positive
reactivity to desmin,
a muscular marker. Collecting
duct renal cell carcinoma is a high grade tumor composed of solid cords or
nests of markedly atypical epithelioid cells, with or without tubular
architecture. Renal
papillary adenoma is characterized by papillary growth lined by benign
epithelial cells. Rhabdomyoma
is characterized by skeletal muscle differentiation, but does not have vascular
and adipose components. Atypical
lipomatous tumour/well differentiated liposarcoma is a tumor with adipose
tissue differentiation. It contains lipoblasts, but usually not thick-walled
vessels and muscular components.
2. B. Angiomyolipoma
express HMB45, a melanocytic marker. CK7 is a cytokeratin commonly expressed by
many types of renal
cell carcinoma. P63 is positive in squamous cell carcinoma and urothelial
carcinoma. TFE-3 is positive for Xp11
translocation renal cell carcinoma. WT-1 is positive in Wilms
tumor.
3. C. Angiomyolipoma
may be associated with tuberous sclerosis, a condition of hamartomatous growth
involving multiple system. Patients usually have cardiac, neurological and/or dermatological
presentations, as seen in this patient. It is associated with mutations of TSC1
or TSC2 genes. MET mutation is seen in papillary
type renal cell carcinoma. PAX6 and WT-1 abnormalities are associated with WAGR
syndrome/Wilms tumor. VHL mutation is seen in von Hippel-Lindau syndrome.
4. D.
Well-circumscribed renal mass composed of tumor cells with eosinophilic
granular cytoplasm can be oncocytoma
or chromophobe
type renal cell carcinoma. Lack of cytological atypia and diffuse
reactivity to CK7, as well as negative colloid iron stain are all consistent
with oncocytoma.
Adult Wilms
tumor has similar triphasic histological findings as seen in pediatric
tumors. Adenocarcinoma of colon is usually positive for CK20. Urothelial
carcinoma of renal pelvis are composed of tumor cells with urothelial
differentiation, and usually do not have granular cytoplasm.
5. A. Nuclear
pleomorphism, clear cytoplasm, and fine vasculatures are highly suggestive of clear
cell type renal
cell carcinoma. Co-expression of both cytokeratin and vimentin is
relatively specific for clear
cell type renal
cell carcinoma. Chromophobe
type renal cell carcinoma is characterized by eosinophilic granular
cytoplasm with perinuclear halo. Negative reactivity to CD56 and chromogranin
is not compatible with neuroendocrine tumor. Oncocytoma
has eosinophilic granular cytoplasm, but lack nuclear pleomorphism, perinuclear
halo, and are negative for colloidal iron stain. Urothelial
carcinoma of renal pelvis are composed of tumor cells with urothelial
differentiation, and usually do not have clear cytoplasm and fine vasculatures.
6. D. This patient
has history of pancreas neuroendocrine tumor. With current finding of clear
cell type renal
cell carcinoma, she likely has von Hippel-Lindau syndrome, a
condition associated with VHL mutation. MET mutation is seen in papillary
type renal cell carcinoma. PAX6 and WT-1 abnormalities are associated with WAGR
syndrome/Wilms tumor. TSC2 mutation is seen in tuberous sclerosis.
7. D. Clear
cell type renal
cell carcinoma is associated with von Hippel-Lindau syndrome. Birt-Hogg-Dubé
syndrome is associated with oncocytoma
or chromophobe
type renal cell carcinoma. Hereditary papillary renal carcinoma is
characterized by in papillary
type renal cell carcinoma. Tuberous sclerosis is associated with angiomyolipoma.
WAGR syndrome is characterized by Wilms
tumor, aniridia, genitourinary malformations, and mental retardation.
8. C. The tumor
morphology is most consistent with metastatic clear
cell type renal
cell carcinoma. Metastasis of renal
cell carcinoma can occur ANY TIME, ANYWHERE, although the most common sites
include lung and bone. Follicular adenoma of thyroid and multinodular goiter
have follicular differentiation. Papillary carcinoma of thyroid has nuclear
grooves and inclusions. All these three are positive for thyroiglobulin.
Medullary thyroid carcinoma express calcitonin.
9. B. The most
commonly seen malignant tumor of lung is metastatic malignancy. The tumor cell
morphology of eosinophilic granular cytoplasm, slightly pleomorphic nuclei with
perinuclear halo is highly suggestive of a metastatic chromophobe
type renal cell carcinoma. With the presentation, the major problem for
this patient is metastatic tumor, not infection. She has no sign of HIV
infection. EGFR mutation is seen in adenocarcinoma
of lung.
10. B. The tumor
cell morphology of eosinophilic granular cytoplasm, slightly pleomorphic nuclei
with perinuclear halo is highly suggestive of a metastatic chromophobe
type renal cell carcinoma. Adenocarcinoma
of lung is positive for CK7 and TTF-1. Adenocarcinoma of colon is usually
positive for CK20. Squamous
cell carcinoma has squamous differentiation, characterized by squamous
pearls in well differentatiated tumors, and always has intercellular bridges.
11. B. The tumor
cell morphology of eosinophilic granular cytoplasm, slightly pleomorphic nuclei
with perinuclear halo is highly suggestive of chromophobe
type renal cell carcinoma. Clear
cell type renal
cell carcinoma is characterized by clear cytoplasm, nuclear pleomorphism,
and fine vasculatures. Adenocarcinoma
of lungs can be seen kidney, but they are usually positive for CK7 and
TTF-1. Oncocytoma
has eosinophilic granular cytoplasm, but lack nuclear pleomorphism, perinuclear
halo, and are negative for colloidal iron stain. Urothelial
carcinoma of renal pelvis are composed of tumor cells with urothelial
differentiation, and usually do not have granular cytoplasm.
12. A. BHD mutation
is seen in oncocytoma
or chromophobe
type renal cell carcinoma. MET mutation is seen in papillary
type renal cell carcinoma. Xp11
translocation renal cell carcinoma usually has papillary and nested growth,
with eosinophilic and clear cells. PAX6 and WT-1 abnormalities are associated
with WAGR
syndrome/Wilms tumor. VHL mutation is seen in von Hippel-Lindau syndrome and clear
cell type renal
cell carcinoma.
13. D. Papillary
growth with atypical cells is supportive of a papillary
type renal cell carcinoma. Chromophobe
type renal cell carcinoma is characterized by eosinophilic granular
cytoplasm with perinuclear halo. Papillary carcinoma of thyroid has nuclear
grooves and inclusions, and is positive for thyroiglobulin. Renal
papillary adenoma does not have cytological atypia, and invasion. Urothelial
carcinoma of renal pelvis are composed of tumor cells with urothelial
differentiation.
14. B. See
discussion for question 12.
15. C. High grade
tumor composed of solid cords or nests of markedly atypical epithelioid cells,
with or without tubular architecture is most likely of collecting
duct renal cell carcinoma. Angiomyolipoma
is a benign tumor with three components: thick-walled distorted vessel,
muscular stroma and adipose tissue. Chromophobe
type renal cell carcinoma is characterized by eosinophilic granular
cytoplasm with perinuclear halo. Papillary carcinoma of thyroid has nuclear
grooves and inclusions, and is positive for thyroiglobulin. Papillary
type renal cell carcinoma is characterized by papillary growth covered by
atypical cells, as well as clusters of foamy histiocytes and foci of
calcification. Urothelial
carcinoma of renal pelvis are composed of tumor cells with urothelial
differentiation.
16. E. Urothelial
carcinoma of renal pelvis are composed of tumor cells with urothelial
differentiation, morphologically similar to squamous cells. Clear
cell type renal
cell carcinoma is characterized by clear cytoplasm, nuclear pleomorphism,
and fine vasculatures. Collecting
duct renal cell carcinoma is a high grade tumor composed of solid cords or
nests of markedly atypical epithelioid cells, with or without tubular
architecture. Papillary
type renal cell carcinoma is characterized by papillary growth covered by
atypical cells, as well as clusters of foamy histiocytes and foci of
calcification.
17. A. UroVysion is
useful in detecting aneuploidy of chromosomes 3, 7, 17, and loss of the 9p21 in
high grade urothelial carcinoma. BHD mutation is seen in oncocytoma
or chromophobe
type renal cell carcinoma. MET mutation is seen in papillary
type renal cell carcinoma. Xp11
translocation renal cell carcinoma usually has papillary and nested growth,
with eosinophilic and clear cells. VHL mutation is seen in von Hippel-Lindau syndrome and clear
cell type renal
cell carcinoma.
18. E. Triphasic
tumor with epithelial elements (tubular), blastemal elements (nests of
undifferentiated epithelioid cells) and fibrotic stroma with positive reactivity
to WT-1 is most likely Wilms
tumor. Collecting
duct renal cell carcinoma is a high grade tumor composed of solid cords or
nests of markedly atypical epithelioid cells, with or without tubular
architecture. Neuroblastoma is positive for S-100. Xp11
translocation renal cell carcinoma usually has papillary and nested growth,
with eosinophilic and clear cells. Urothelial
carcinoma has urothelial differentiation, morphologically similar to
squamous cells. These four tumors are negative of WT-1.
19. E. WT-1
abnormalities are associated with Wilms
tumor. MET mutation is seen in papillary
type renal cell carcinoma. TSC2 mutation is seen in tuberous sclerosis. BHD
mutation is seen in oncocytoma
or chromophobe
type renal cell carcinoma. VHL mutation is seen in von Hippel-Lindau syndrome and clear
cell type renal
cell carcinoma.
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