Neoplasms Flashcards by Jeddi Delmendo

What is the most significant risk factor for developing renal cell carcinoma

Smoking

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Mutation in clear cell renal cell carcinoma? Cell of origin?

Deletion of 3p region harboring VHL, somatic mutation or hypermethylation-induced inactivation —> unopposed HIF-1–> VEGF, IGF-1
Proximal tubules

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Mutations in papillary carcinoma? Cell of origin?

Sporadic: trisomies 7 and 17, loss of Y in male patients
Familial: trisomy 7
Chromosome 7 contains MET (TKR for hepatocyte growth factor or scatter factor) -> abnormal growth, cell mobility, invasion, and morphogenetic differentiation
Distal convoluted tubules

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Chromophobe carcinoma prognosis and cell of origin

Excellent prognosis
Also from intercalated cells of collecting ducts (like oncocytoma)

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Xp11 translocation carcinoma: which gene is translocated

TFE3

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Collecting duct (Bellini duct) carcinoma location

CD in MEDULLA

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Which renal cell carcinoma can be multifocal and bilateral?

Papillary carcinoma
Clear cell RCC if with VHL syndrome

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A 50 year old man came in due to vague abdominal pain and hematuria. CT scan showed a multilocular cystic mass on the left kidney. Resection shows a multilocular cystic tumor. The cysts are lined by clear cells with conspicuous nucleoli at 400x only. A focal area of solid growth measuring 0.5 mm was seen. There were no papillary projections, necrosis, atypical
Mitosis or rhabdoid features.
IHC: (+) PAX8, CAIX (diffuse and membranous), CD10
(-) CK7, AMACR, CD117
Dx? Criteria? Pathogenesis? Prognosis?

Multilocular cystic renal neoplasm of low malignant potential (MCRNLMP)
- solid growth should not exceed 1 mm (20x field)
VHL gene alterations
Excellent prognosis regardless of stage

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Resection of a kidney showed a 2 cm mass with variegated hemorrhagic, necrotic and friable cut surfaces. Microsections show papillary and tubule-papillary architecture lined by low grade cuboidal cells with abundant eosinophilc to basophilic to clear cytoplasm, some with hemosiderin. Foamy macrophages are seen at papillary cores and few scattered psammoma bodies.
IHC:
(+)PAX8, CD10, AMACR, CK7 (but negative in eosinophilic tumors), vimentin, FH
(-) CAIX (some focal positivity)
Dx? Criteria? Predisposing factors?

Papillary RCC
Must not be <1.5 cm (papillary adenoma)
There shouldn’t be too much architectural pattern heterogeneity
More common in CKD or acquired cystic disease in adults

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A 42 year old man came in due to hematuria. On imaging, a renal mass was seen with a stellate scar.
Resection was done revealing a mahogany brown to a yellow tan well-circumscribed tumor with a central stellate scar. Microsections show cells in tublues and solid nests with abundant eosinophilic granular cystoplasm and round eccentric nuclei with prominent nucleoli.
IHC: (+) CD117, S100A, CK7 (scattered single cells or negative)
(-) CAIX
Dx? Assoc with what syndrome? Cell of origin?

Oncocytoma
Birt-Hogg-Dube syndrome (hybrid tumor with chromophobe)
Intercalated cells of distal tubules

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A 31 year old man came in due to flank pain. CT scan showed a right renal mass. Resection shows a gray to light-tan tumor with a central scar. Microsections show solid sheets and neats of pale cells with clear to eosinophilic cytoplasm with prominent cell membrane, hyperchromatic raisinoid/wrinkled nuclei with characteristic perinuclear halo. Stroma is vascular and hyalinized.
IHC: (+) CK7 (diffuse), CD117, hale’s colloidal iron
(-) CD10, CAIX, vimentin
Dx? Assoc syndrome?

Chromophobe RCC
Birth-Hogg-Dube syndrome (in hybrid oncocytoma- chromophobe tumors)

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A 57 year old male came in due to hematuria and flank pain. CT scan showed an ill-defined medullary renal mass. Microsections show predominantly infiltrating high grade cells with tubular architecture surrounded by desmoplastic stroma in the medulla. Necrosis is seen in areas.
IHC: (+) HMWCK, CK7, PAX8, SMARCB1, FH
(-) GATA3
Dx? Ddx?

Collecting duct carcinoma
Ddx: exclude other RCCs, urothelial CA, or metastasis

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A 70 year old man with ESRD came in due to hematuria. CT scan showed a left kidney mass. Resection was done which showed an encapsulated cystic 3 cm kidney mass. Microsections show tubules, cysts and papillae lined by cells with grade 1-2 nuclei located on the apical pole with clear cytoplasm creating a piano key appearance.
IHC: (+) CK7 (diffuse), CAIX (cup-shaped), GATA3, PAC8, HMWCK (34BE12)
(-) AMACR, CD10
Dx? Pathogenesis?

Clear cell papillary renal cell tumor (CCPRCT)
Unknown (no 3p loss or vhl gene alterations)

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A 50 year old woman came in due to hematuria. A left kidney mass was seen on CT. Resection shows a solid gray-white to yellow mass. Microsections show tightly packed, anastomosing tubules lined by low-grade cuboidal cells merged with spindled cells and myxoid matrix.
IHC:
(+) PAX8, CK7, AMACR
Dx? Prognosis? Ddx?

Mucinous tubular and spindle cell carcinoma (MTSCC)
High grade transformation occurs in a subset
Ddx: papillary RCC

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A 40 year old male came in due to left flank pain. CT scan showed a left renal mass. Resection shows a mass with cystic, bubble wrap appearance. Microsections show hobnail to flattened cells lining tubulocystic structures with fibrotic stroma.
Dx? Pathogenesis?

Tubulocystic RCC
Gain of chromosome 17, losses of 9 and Y

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A 60 year old man with ESRD on long-term dialysis came in due to hematuria. CT showed bilateral cystic masses on kidneys. Transplant was done and microsections the kidney shows clear to eosinophilic tumor cells in sieve-like, microcystic pattern. Tumor cells have intracytoplasmic calcium oxalate crystals.
IHC: (+) pax8, CD10, AMACR
(-) ck7, cd117, gata3
Dx? Pathogenesis?

Acquired cystic disease-associated RCC
oxalate accumulation

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A 20 year old woman with a history of epilepsy came in due to flank pain. Ct showed a right renal mass. Resection was done and microsections show solid and cystic, and tubular architecture lined by oncocytic cells with abundant eosinophilic cytoplasm with basophilic cytoplasmic STIPPLING (like leishmaniasis) vacuolation and multinucleation.
IHC: (+) PAX8, CK20 (diffuse or focal), ck7 (focal), cathepsin
Dx? Assoc syndrome?

Eosinophilic solid cystic (ESC) RCC
TSC1 or TSC2
Upregulation of mTOR pathway

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A 5 year old boy undergoing chemotherapy for leukemia came in due to hematuria. CT showed a left renal mass. Resection was done and microsections show mixed papillary architecture with psammoma bodies and epithelioid cells with clear to eosinophilic cytoplasm.
IHC: (+) PAX8, melanocytic markers (HMB45, MelanA)
(-) CK, EMA
Dx? Pathogenesis?

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TFE3 rearranged RCC/Xp11 translocation RCC
Exposure to chemotherapy
TFE3 gene (X11p) rearrangements with multiple partners like ASPSCR1, PRCC and others

A 31 year old man came in due to flank pain. A renal mass was seen on CT and was resected. Microsections show a biphasic tumor with nests of larger epithelioid cells and smaller cells surrounding BM material.
IHC: (+) PAX8 and melanocytic markers (HMB45, melanA)
Dx? Pathogenesis?

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TFEB altered RCC t(6;11)
TFEB gene fusion or amplification t(6,11)

A 44 year old female came in due to hematuria. She has a hx of bizarre leiomyomas. CT scan showed a right renal mass. Resection was done and microsections show mixed papillary, solid and tubulocystic patterns with cells having eosinophilc macronucleoli with SURROUNDING PERI-NUCLEOLAR HALOS
Dx? Pathogenesis? IHC?

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Fumarate hydratase (FH) deficient RCC
Germline or somatic mutations of the FH gene
Hereditary leiomyomatosis and RCC (HLRCC) syndrome associated RCC - hx of skin and uterune leiomyomas
IHC: (+) pax8, 2SC
(-) FH

A 50 year old male came in due to hematuria. Bilateral kidney masses were seen. Microsections show solid sheets of eosinophilic cells with occasional flocculent cytoplasmic inclusions (cytoplasm not granular).
IHC: (+) PAX 8, FH
DX? Pathogenesis?

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SDH-deficient RCC
Inactivation of any one if SDH genes due to germline mutation
Bilateral in 30%
IHC (-) SDH

A 65 year old African man came in due to hematuria. A renal mass was seen and resected. Microsections show infiltrating sheets, cords, nests and tubules with high grade rhabdoid cells. Stroma is myxoid and desmoplastic. RBCs in vessels are sickle-shaped.
IHC: (+) pax8, ck, ema, vimentin, ck7, hmwck, cea, oct3/4
(-) INI1
Dx? Pathogenesis? Prognosis?

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SMARCB1-deficient renal medullary carcinoma
Associated with sickle cell trait>disease
Sickle-cell mediated hypoxic injury to the medulla
SMARCB1 gene inactivation at chromosome 11 by deletion or translocation
Poor prognosis

A 50 year old woman came in due to hematuria. A renal mass was seen and excised. Microsections show small
Primitive tubules without lumens, minimal stroma and no mitosis. Tumor is basophilic and well-circumscribed on low magnification.
IHC: (+) PAX8, WT1, CD57
Dx? Pathogenesis? Prognosis?

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Metanephric adenoma
BRAF mutation in 90% of cases
Good prognosis

A 45 year old woman came in for executive check up. Ct scan showed a renal mass which was resected. Microsections show solid-cystic tumor with epithelial elements in a variably cellular stroma that resembles OVARIAN STROMA.
IHC: glands (+) pax8, stromal cells (+) sma, desmin, cd10, ER, PR, inhibin, calretinin
Dx? Location? Prognosis?

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Mixed epithelial and stromal tumor of the kidney (MEST)
Medulla
Good prognosis
Cystic nephroma is part of the spectrum (cystic only)- not associated with DICER1 mutations unlike pediatric cystic nephrona

A 40 year old woman came in due to hematuria. On PE, patient had hypomelanotic macules and shagreen patches. A mass on right renal area was seen and resected. Microsections show myoid spindle cells, mature adipose tissue and dysmorphic thick-walled blood vessels, (-) elastic stain. IHC: (+) SMA, calponin, HMB45, melanA, er, pr, s100 Dx? Pathogenesis?

Classic angiomyolipoma/PEComa of kidney Proliferation of perivascular epithelioid cells, hence the name TSC1 and TSC2 inactivation

A 35 year old female came in due to hematuria and flank pain. Patient has a history of lympangioleiomyomatosis (LAM). A kidney fat-containing tumor was seen on CT which was resected. Microsections show nests of large eosinophilic ganglion-like cells and sheets of epithelioid and spindle cells. Renal vein was involved and one LN was positive. IHC: (+) HMB45, MelanA, MITF, variable expression of SM markers (-) CK, PAX8 Dx? Criteria? Pathogenesis? Behavior?

Epithelioid angiomyolipoma/ epithelioid pecoma of kidney Contains at least 80% epithelioid cells From PEC, TSC2 mutation in sporadic cases Rare cases of malignant transformation (criteria: >70% atypical, mitosis >/= 2/10hpfs, atypical mitotic figures, necrosis)

A 25 year old female came in the ER due to weakness and dizziness. Patient had BP of 180/100. Lab test showed decreased K levels. Work up was done which showed a renal mass. Mass was resected which showed a well-circumscribed tumor with tan cut surfaces. Microsections show solid sheets and lobules of round to polygonal to spindle cells with prominent vasculatyre, variable cytologic atypia and hyalinized vessels. Stains: (+) CD34, PAS Dx? Prognosis? Your co resident wanted to see it on EM. Why do you think?

Juxtaglomerular cell tumor Good prognosis EM: characteristic rhomboid crystals and renin granules

A 23 year old male came in due to paresthesias. Work up was done which revealed a kidney mass. Resection was done which showed a well-circumscribed mass with hemorrhage. Microsections show an anastomosing proliferation of capillary-sized vessels, reminiscent of splenic sinusoids within a framework of non-endothelial supporting cells and these are lined by hobnail endothelial cells with fibrin thrombi. Extramedullary hematopoiesis and mature fat is also seen. IHC: (+) CD31, CD34, ERG, SMA (supporting cells) Dx? Pathogenesis? Ddx?

Anastomosing hemangioma GNA14 and GNAQ mutations Angiosarcoma

A woman gave birth to a stillborn baby. Baby had occipital encephalocoele, polydactyly and cystic kidneys. On autopsy kidneys were asymmetric with varisized cysts. Dx? Pathogenesis?

Multicystic renal dysplasia Associated with Meckel-Gruber syndrome Usually unilateral but if bilateral, asymmetric and will have oligohydramnios and its complications

A 4 year old girl complains of abdominal pain. A right renal mass was seen and resected. Microsections show primitive epithelial tubules, sheets of small high N:C ratio cells and stroma. Dx? Ihc?

Wilm’s tumor (Nephroblastoma) (+) WT-1 on all components, CK on epithelium, desmin on blastema

A 2 year old boy presented with abdominal pain. A renal Mass was seen and resected. Microsections show well-defined multilocular cysts lined by flattened cuboidal epithelium with seta containing blastema, embryonal stroma and epithelial elements. Dx? Prognosis?

Cystic partially differentiated nephroblastoma Benign

A 22 year old girl came indue to hematuria. A renal mass was seen and was resected. Microsections show monotonous ovoid to spindle cells, conspicuous nucleoli and clear cytoplasm arranged in nests and fascicles separated by collagen. Also seen are MNGCs with pwripherally located nuclei. You also see brown pigments. IHC: (+) HMB45, MelanA Dx? Molecular? Characteristic cells?

Clear cell sarcoma EWSR1-ATF1 t(12;22) Wreath cells (the MNGCs)

A newborn was palapted to have an abdominal mass which was a renal mass. Resection done. Microsections show an unencapsulated mass with finger-like projections and composed of intermingling fascicles of spindle cells with low mitotic activity. Also seen are entrapped nephrons and dilated thin walled vessels. IHC: (+) CD34 Dx? Types?

Mesoblastic nephroma (congenital mesoblastic nephroma) - looks like leiomyoma Classic, cellular (MC) and mixed

A 6 year girl came in due to a palpable abdominal mass. A kidney mass was seen and resected. Microsections show a WC and unencapsulated tumor composed of spindle cells with a nodular appearance and onion skin cuffing around entrapped tubules IHC: (+) vimentin, focal CD34 (-) WT-1, desmin, SMA Dx? Molecular? Prognosis?

Metanephric stromal tumor BRAFV600E mutation Benign, no further tx

A 3 year old boy came in due to a renal mass which was then resected. Microsections show poorly cohesive infiltrative sheets of neoplastic cells with pink cytoplasm with eccentric nuclei due to intracytoplasmic inclusions of eosinophilic hyaline globules IHC: (+) CK, vimentin (-) INI1 Dx? Molecular? Prognosis?

Rhabdoid tumor of kidney Mutations in SNF5/INI1 Very aggressive

A 4 year old boy was noted to have a renal cyst. Microsections of the cyst show a multicystic tumor with variably sized simple cysts lacking immature nephrogenic elements, solid areas and cytologic atypia. The cysts are lined by flattened to cuboidal Epithelium with frequent hobnailing and no epithelial complexity. Dx? Prognosis?

Cystic nephroma Benign

These are precursors of nephroblastoma and are associated with certain syndromes carrying a risk for it

Nephrogenic rests - abnormally persistent foci of embryonal cells in a normal kidney

Microsections of a kidney tumor show a tumor with hypo and hypercellular areas with a loose myxoid stroma. These areas are composed of sheets of small, stellate, spindled or round cells with scant or deeply eosinophilic cytoplasm and eccentric, small oval nuclei, with other cells having elongated tails of cytoplasm. IHC: (+) desmin, MyoD1, myogenin Dx? Molecular?

Embryonal rhabdomyosarcoma PAX3-FOXO1, PAX7-FOXO1 translocations Alveolar - larger more round cells in alveolar pattern with hobnailing of cells Botryoid - with cambium layer

A 9 year old boy came in due to right leg pain and abdominal pain. Xray showed a moth eaten femur. Also noted was a 7 cm renal mass which was then resected. Microsections show uniform round blue cells with finely Granular chromatin in sheets and lobular pattern (+) CD99 and vimentin Dx? Molecular? Prognosis?

Ewing sarcoma EWSR-FLI1 Favorable prognosis if age <10 and tumor size < 8 cm

A 7 year old boy was found to have a renal mass. Microsections show primitive round blue cells in sheets, nests and trabeculae with a background of desmoplastic storma and rosette like structures. (+) cam5.2, CK, desmin (dot-like), WT-1, NSE DX? Molecular? Prognosis?

Desmoplastic small round cell tumor EWSR1-WT1 Poor prognosis

CK7 negative tumors

Clear cell RCC MCRNLMP Acquired cystic disease assoc RCC

MIT family translocation RCC with nested and acinar-like Arias of cells with clear or granular acidophilic cytoplasm with round nuclei. Some cases show a biphasic pattern with a population of smaller cells surrounding eosinophilic basement membrane like material

TFEB-altered RCC

A 45 year old woman came in to the clinic due to a kidney mass. Patient has a history of paraganglioma. kidney mass resection was done. Microsections show neoplastuc plastic cells arranged in solid nested to be there architecture. These cells have interest, cytoplasmic, vacuoles, or inclusion that contains is in the physic material. + PAX8 Rarely CK7 and CD117 DX molecular

SDH-deficient RCC Germline mutation of SDH gene

Neoplasms Flashcards

(43 cards)