GU Flashcards

Question

Diagnosis? Stains?

Answer 1

POSTOPERATIVE SPINDLE CELL NODULE/PSEUDOSARCOMATOUS FIBROMYXOID TUMOR * Many are immunoreactive for ALK-1 – inflammatory myofibroblastic tumor * Can locally recur * Variably reactive for pankeratin; actin stains accentuate the cytoplasmic membranes – tram track pattern

Answer 2

UROTHELIAL CARCINOMA * Strong association with smoking * more commonly seen in men * Variants: micropapillary, nested, sarcomatoid, squamous and glandular differentiation * IHC markers: CK7, CK20, CK903, p63, GATA3

Answer 3

Mucinous tubular and spindle cell carcinoma * Histology: * Well-circumscribed with partial surrounding rim of compressed fibrous tissue * tubular/cordlike * uniform, low cuboidal cells with eosinophilic, focally vacuolated cytoplasm * spindling * low grade nuclei * Stroma is myxoid and bubbly, abundant extracellular mucin, foamy macrophages * Can be mucin poor (highlighted by Alcian blue), well formed papillae, clear cells, necrosis * Positive: EMA, AMACR, AE1-AE3, CK7, CK 8/18, CK19, PAS (highlights basal lamina around tubules), Alcian blue highlights mucin; also neuron specific enolase and either chromogranin or synaptophysin * Loss of multiple chromosomes * Negative for trisomy 7 and 17

Answer 4

Translocation carcinoma (Xp11 ) * TFEE on Xp11.2 * t(X;17)(p11.2;q25) --\> TFE3-ASPL fusion gene (low grade) * t(X;1)(p11.2;p34) --\> TFE3-PSF fusion gene * t(X;1)(p11.2;q21) --\> TFE3-PRCC fusion gene (high grade) * proximal tubule * F \> M * kids \> adults * Gross: similar to RCC * Histology: nests of discohesive cells with clear/eosinophilic cytoplasm, +/- papillary * Neg: CK * TFE3+ IHC, FISH better

Answer 5

Translocation carcinoma (6p21 ) * TFEB on 6p21 * worse than classic RCC * proximal tubule * F \> M * kids \> adults * Gross: similar to RCC * Histology: * solid/alveolar, clear/eosinophilic cytoplasm * pink basement membrane material * Fuhrman nuclear grade 3 nuclei * CK neg * TFEB+ IHC, FISH better

Answer 6

**Fuhrman Grading System** * Grade X Cannot be assessed * Grade 1 * Nuclei round, uniform, approximately 10 µm in diameter; nucleoli inconspicuous or absent * Grade 2 * Nuclei slightly irregular, approximately 15 µm in diameter; nucleoli evident (20-40x) * Grade 3 * Nuclei very irregular, approximately 20 µm in diameter; nucleoli large and prominent (10x) * Grade 4 * Nuclei bizarre and multilobated, 20 µm or greater in diameter, nucleoli prominent, chromatin clumped

Answer 7

Kidney pTNM Staging Primary Tumor (pT) * pTX: Primary tumor cannot be assessed * pT0: No evidence of primary tumor * pT1: Tumor \<= 7 cm, limited to the kidney * pT1a: \<= 4 cm, limited to the kidney * pT1b: 4 - 7 cm, limited to the kidney * pT2: \> 7 cm, limited to the kidney * pT2a: 7 - 10 cm, limited to the kidney * pT2b: \> 10 cm, limited to the kidney * pT3: Tumor extends into major veins or perinephric tissues but not into the ipsilateral adrenal gland and not beyond Gerota’s fascia * pT3a: Tumor grossly extends into the renal vein or its segmental (muscle containing) branches, or tumor invades perirenal and/or renal sinus fat but not beyond Gerota’s fascia * pT3b: Tumor grossly extends into the vena cava below the diaphragm * pT3c: Tumor grossly extends into vena cava above diaphragm or invades the wall of the vena cava * pT4: Tumor invades beyond Gerota’s fascia (including contiguous extension into the ipsilateral adrenal gland)

Answer 8

Regional Lymph Nodes (pN) * pNX: Regional lymph nodes cannot be assessed * pN0: No regional lymph node metastasis * pN1: Metastasis in regional lymph node(s) Distant Metastasis (pM) * pM1: Distant metastasis

Answer 9

Kidney Stage Grouping

Answer 10

Bladder Primary Tumor (pT) * pTX: Primary tumor cannot be assessed * pT0: No evidence of primary tumor * pTa: Noninvasive papillary carcinoma * pTis: Carcinoma in situ: “flat tumor” * pT1: Tumor invades subepithelial connective tissue (lamina propria) * pT2: Tumor invades muscularis propria (detrusor muscle) * pT2a: Tumor invades superficial muscularis propria (inner half) * pT2b: Tumor invades deep muscularis propria (outer half) * pT3: Tumor invades perivesical tissue * pT3a: Microscopically * pT3b: Macroscopically (extravesicular mass) * pT4: Tumor invades any of the following: prostatic stroma, seminal vesicles, uterus, vagina, pelvic wall, abdominal wall * pT4a: Tumor invades prostatic stroma or uterus or vagina * pT4b: Tumor invades pelvic wall or abdominal wall

Answer 11

Bladder Regional Lymph Nodes (pN) * pNX: Lymph nodes cannot be assessed * pN0: No lymph node metastasis * pN1: Single regional lymph node metastasis in the true pelvis (hypogastric, obturator, external iliac or presacral lymph node) * pN2: Multiple regional lymph node metastasis in the true pelvis (hypogastric, obrutrator, external iliac or presacral lymph node metastasis) * pN3: Lymph node metastasis to the common iliac lymph nodes Distant Metastasis (pM) * Not applicable * pM1: Distant metastasis

Answer 12

Bladder Stage Grouping

Answer 13

Bladder pT

Answer 14

Prostate Primary Tumor (T): Clinical Classification * TX Primary tumor cannot be assessed * T0 No evidence of primary tumor * T1 Clinically inapparent tumor neither palpable nor visible by imaging * T1a Tumor incidental histologic finding in 5% or less of tissue resected * T1b Tumor incidental histologic finding in more than 5% of tissue resected * T1c Tumor identified by needle biopsy (eg, because of elevated prostate specific antigen [PSA]) * pT2 Confined to prostate * pT2a Unilateral, 1/2 of 1 side or less * pT2b Unilateral, \> 1/2 of 1 side * pT2c Bilateral * pT3 Extraprostatic extension * pT3a Extraprostatic extension or microscopic bladder neck invasion * pT3b Seminal vesicle invasion * pT4 Invasion of rectum, levator muscles and/or pelvic wall

Answer 15

Prostate Regional Lymph Nodes (pN) * pNX: Cannot be assessed * pN0: No regional lymph node metastasis * pN1: Metastasis in regional lymph node or nodes Distant Metastasis (pM) * Not applicable * pM1: Distant metastasis * pM1a: Nonregional lymph nodes(s) * pM1b: Bone(s) * pM1c: Other site(s) with or without bone disease

Answer 16

Prostate Stage Grouping

Answer 17

Label Prostate pT

Answer 18

Gleason Grading: * Needle bx * Predominant + Worst * TURP * Predominant + Worst * Radical prostatectomy * Predominant + Secondary * Mention worst in Dx

Answer 19

SEMINOMA * Most common of the pure germ cell tumor of testis * slightly older age group compared to the non-seminomatous germ cell tumor * Tumor markers generally negative; mild elevation of bHCG levels in cases with isolated syncytial trophoblasts * Positive: SALL4, OCT3/4, CD117, Podoplanin, SOX17 * Negative: Glypican3, CD30, AE1/AE3, SOX2

Answer 20

SPERMATOCYTIC SEMINOMA * older men * indolent in behavior unless there is a sarcomatous component * Solid growth pattern, often interrupted by lakes of edema * Filamentous spireme chromatin, uniform rounded nuclear contours, increased mitotic activity * Negative for IGCNU (intratubular germ cell neoplasia unclassified); Can have intratubular growth * Positive: SALL4 * Negative: OCT3/4, AE1/AE3, CD30, Glypican3

Answer 21

Seminoma with STGCs * 10-20% have recognizable STCs * HCG positive cells up to 25% * May be surrounded by hemorrhage: Don’t confuse with choriocarcinoma * Associated with elevated serum HCG level * Not have a poorer prognosis * Other giant cells: Langhans GC associated with inflammatory reaction * Intratubular trophoblasts- 5 of 29 seminomas

Answer 22

YOLK SAC TUMOR * Most common testicular germ cell of childhood * Variety of growth patterns: microcystic/reticular, festoon/SchillerDuvall, polyvesicular vitelline, enteric, hepatoid, parietal * Rare cases of sarcomatous component * Positve: Glypican 3, SALL4, AE1/AE3 * Negative: OCT3/4, CD30

Answer 23

Germ Cell Tumor IHC

Answer 24

TERATOMA * Second most common testicular germ cell tumor of childhood * Regarded as somatic differentiation of a non-teratomatous germ cell * Rare cases of associated somatic malignancy – carcinoma, sarcoma

Answer 25

SERTOLI CELL TUMOR * Rare in childhood * Invariably unilateral except in the setting of Peutz-Jeghers or Carney Complex * Histologic variants: sclerosing sertoli cell tumor, large cell calcifying sertoli cell tumor * Immunoreactive for keratin, variably immunoreactive for inhibin/calretinin

Answer 26

LEYDIG CELL TUMOR * Often presents with gynecomastia * Gross: solid yellow nodule * Histology: crystals of Reinke and/or lipofuschin may be seen * Immunoreactive for inhibin and calretinin

Answer 27

Renal Medullary Fibroma * Benign, usually incidental * Multiple a/w systemic hypertension * Gross: Small (\< 1-5 cm), well-circumscribed, tan-white nodule in the renal medulla / pyramids * Histology: * Stellate fibroblast-like cells * background of loose or dense collagenous tissue * entrapped tubules at periphery * +/- heterotopic bone with marrow * Positive: Oil Red O, Sudan Black B * Negative: CD34

Answer 28

Renal tumors F \> M * Mucinous tubular and spindle cell tumor * Mixed epithelial and stromal tumor * Metanephric adenoma

Answer 29

Mixed epithelial and stromal tumor * Benign * Perimenopausal women, increased hormones * Gross: well circumscribed, unencapsulated, in renal pelvis, mean 6 cm, cystic, yellow-tan * Histology: * Cystic and solid, mesenchymal and epithelial * ovarian-type stroma * embedded epithelial structures (tubules and cysts) * Cysts often have hobnailed epithelium * Epithelium+: keratin, EMA, CEA and vimentin * Stroma+: alpha smooth muscle actin, desmin, vimentin, ER and PR

Answer 30

Metanephric adenoma * F \> M * BRAF V600E * No trisomy 7 and 17 * Gross: * Single, well circumscribed, non encapsulated, tan-gray-white-yellow, solid / lobulated, mean 5 cm (range 0.3 to 15 cm) * Large tumors may have secondary cystic or hemorrhagic changes * Histology: * Small, uniform, closely packed tubules or papillae in loose stroma * small cells with minimal cytoplasm, bland nuclei that may overlap, uniform chromatin, glomeruloid bodies and rare mitoses * +/- hemorrhage, necrosis, calcifications (psammoma bodies) or cysts * No atypia, no blastema, no / rare mitotic figures, no infiltrative growth and no vascular invasion * Positive: WT1 (strong / diffuse), CD57 (strong / diffuse), CK7 (focal), AE1 (focal) and vimentin (solid areas), S100 * Negative: AMACR, glycogen, CD56, desmin, NCAM and EMA

Answer 31

Acquired cystic disease associated RCC * Dialysis * M \> F * More aggressive than other ESRD related RCC * No trisomy 7 and 17 * Histology: * Microcystic sieve-like, abundant eosinophilic cytoplasm * grade 3 nuclei * intratumoral oxalate crystals

Answer 32

Angiomyolipoma with epithelial cysts * Histology: * cystic space lined by cuboidal cells (PAX8+) * cambium layer under epithelium (ER+) * thick exterior wall of smooth muscle * +/- TSC * Smooth muscle predominant angiomyolipoma * Same prognosis

Answer 33

Enteric adenocarcinoma in the bladder - bladder vs. colon primary * Bladder * CK7+ * Beta-catenin+ membranous * Colon * CK7- * Beta-catenin+ nuclear

Answer 34

DDx for Prostate Cancer * Ejaculatory duct * monster cells, brown pigment, intranuclear inclusions * Cowpers gland * Veramontanum hyperplasia * Prostatic atrophy * central dilated duct with branches * dark nuclei * Clear cell atrophy * small glands * clear cells * cuboidal * small dark nuclei * Atypical small acinar proliferation (ASAP) * \< 3 atypical glands

Answer 35

Cowper's gland

Answer 36

Prostate clear cell atrophy, cribriform

Answer 37

Prostate Patterns of atrophy. (a) Simple lobular; (b) Sclerotic; (c) Cystic; (d) Linear or streaming.

Answer 38

Seminal vesicle

Answer 39

Prostate basal cell hyperplasia

Answer 40

Sclerosing adenosis of prostate. (a) H&E appearance showing small glands with thick basement membranes embedded in cellular fibrous stroma. (b) High molecular weight keratin staining (34E12) demonstrating positive basal cells. (c) HHF35 (muscle-specific actin) staining in basal cells indicating myoepithelial metaplasia. (d) S100 positivity in basal cells.

Answer 41

Verumontanum mucosal gland hyperplasia

Answer 42

Hyperplasia of mesonephric glands. (a) Low power. Note prostatic glands on right side and mesonephric glands on left. (b) High-power photomicrograph. Note small acini, some of which are cystically dilated and some containing colloid-like secretions.

Answer 43

Michaelis–Gutmann bodies * 2 to 10 μm in diameter * partially digested bacteria accumulate in monocytes or macrophages and lead to the deposition of calcium and iron on residual bacterial glycolipid * pathognomonic feature of malakoplakia Malakoplakia * immunocompromise * mostly GU * results from the inadequate killing of bacteria by macrophages or monocytes that exhibit defective phagolysosomal activity * the Michaelis-Gutmann body, is considered pathognomonic for malakoplakia

Answer 44

Germ cell tumors: seminoma vs non-seminoma •In seminoma - **tumor size and rete testis invasion** - possible LVI •In NSGCTs - **% of embryonal carcinoma component** - **vascular invasion;** possible rete invasion * Spermatic cord status and margin * Germ cell neoplasia in situ (former ITGCN) * Other findings (presence of STGCs) * Tumor staging (TNM)

Answer 45

Germ cell tumor * GCN in situ (GCNIS) * Classic seminoma * Embryonal ca * Yolk sac tumor * Teratoma (M/IM) * Choriocarcinoma * Mixed GCT

Answer 46

GCT, without GCNIS * Spermatocytic tumor * Prepub T, YST, mixed

Answer 47

Sex-cord Stromal Tumors * Leydig cell * Sertoli cell * Granulosa cell * Fibroma/thecoma group * Mixed/unclassified * TAGS

Answer 48

Mixed GCT & SCST * Gonadoblastoma * Unclassified

Answer 49

Three types of GCTs Type I * No precursor; \< 6 yrs * Prepubetal teratoma, YST and mixed T+YST Type II * GCNIS, median age, 35 for S; 25 for non-S * Isochromosome p12 Type III * Spermatocytic tumor * No precursor; \>50 yrs * Only seen in testis

Answer 50

Germ Cell Neoplasia In Situ (GCNIS) * Precursor lesion of invasive GCT * 50% of pts with GCNIS progress to invasive GCT within 5 yrs or longer if orchiectomy is not performed * Seen in almost all cases of invasive GCT except for spermatocytic tumor and prepubertal teratoma and yolk sac tumor * Histology: * Tubules show decreased diameter and thickened tubular walls; microlithiasis * Atypical cells (2x normal germ cell) with prominent nucleoli and abundant clear cytoplasm scattered along periphery of tubules * May replace entire tubules (seminoma in-situ): seen in equal frequency adjacent to seminomas & NSGCT, not an intratubular spread, advanced form of GCNIS

Answer 51

Germ Cell Neoplasia In Situ (GCNIS) * Positive: * PAS with and without diastase * PLAP, D2-40, **OCT4**, NANOG * AP2 gamma, Mab-M2A, -43-9F, c-Kit (CD117) * Negative: * TRA-1-60: + in tubules adjacent to NSGCT and – in seminoma * keratin, AFP, hCG * **Do not use C-Kit, SOX17, or SALL4**

Answer 52

Seminoma vs. Non-seminoma Seminoma * 46-53% of GCT * bilaterality (2%) * 35-45 years of age * hemorrhage & necrosis--infrequent * cord invasion (5-8%) * uniform growth * distinct cell border * no nuclear overlapping * uniform cells * FV septa, lymphocyte, granuloma -- constant * CK - or focal + Non-Seminoma * 47-54% of GCT * bilaterality (\<2%) * 10 years younger * hemorrhage & necrosis--frequent * cord invasion (20%) * various patterns * indistinct cell border * nuclear overlapping * pleomorphic cells * FV septa, lymphocyte, granuloma -- inconstant * CK + (Ki-1 & AFP/Gly3)

Answer 53

Choriocarcinoma

Answer 54

Spermatocytic Tumor * FGFR3, HRAS mutations or ampl of 9p (DMRT1) * Derived from post-pubertal type germ cells: resemble spermatogonia or 1st spermatocytes * GCN unique to testis. 1-2% of all testis tumors * Almost always seen in pure form. 3-5 cm size * Frequent in men over 50 yrs (m: 52-59; 19-92 yrs). * Bilaterality is higher (9%) than seminoma (2%). * Painless testicular enlargement. * Indolent clinical behavior. * Not associated with GCNIS * Positive: c-kit, VASA, NY-ESO-1, SAGE1, DMRT1, OCT2, Chk2, MAGE-A4, UTF1, SALL4 * Negative: OCT4, PLAP and other GCT markers

Answer 55

Transcription Factors in S and EC Transcription factor: OCT3/4, NANOG, SOX2, 17 * Seminoma: OCT3/4+, NANOG+, SOX2-, SOX17+, (MAGEC2+) * EC: OCT3/4+, NANOG+, SOX2+, SOX17-, (MAGEC2-)

Answer 56

LIN28 in GCTs * RNA binding protein involved in maintaining the pluripotency of embryonic stem cells * Cytoplasmic stain * Similar results with SALL4 * In YST, OCT3/4-; LIN28+

Answer 57

Useful markers for yolk sac tumor * AFP, Glypican 3, SALL4, GDF3

Answer 58

Useful markers for choriocarcinoma * h-CG, SALL4

Answer 59

Useful markers for teratoma * HE, GDF3, SALL4, AP-2gamma

Answer 60

GCTs unrelated to GCNIS * Spermatocytic tumor * Teratoma, prepubertal * Dermoid cyst * Epidermoid cyst * Well diff NE tumor (monodermal teratoma) * Mixed teratoma and YST, prepubertal * Prepubertal teratoma c AFP elevation \> normal for age: considered mixed T and YST, missed small foci * YST, prepubertal * No GCNIS * No 12p amplification * Seen in prepubertal testis * Extremely rare: * YST: 2-3 cases per 1 million/yr (most common) * mixed YST/T: 2-3 per 10 mil/yr

Answer 61

Sex cord-Stromal tumors * 2-5% of testicular neoplasms in adults; 25% in children: ~5% malignant * Leydig cell tumor * Sertoli cell tumors * Large cell calcifying Sertoli cell tumor * Intratubular large cell hyalinizing Sertoli cell tumor * Granulosa cell tumor (adult and juvenile GCT) * Tumors in fibroma-thecoma group * Mixed and unclassified sex cord-stromal tumor * Tumors containing both germ cell and sex cord- stromal elements * Gonadoblastoma

Answer 62

Sex Cord-Stromal Tumors * Non-functioning * Subset associated with clinical syndromes * Leimyomatosis RCC syndrome in Leydig tumor * Familial adenomatosis polyps with SCTtumor * Carney complex with large cell calcifying SCT * Intratubular LCCSCT in Peutz-Jeghers syndrome * LCCSCT vs. intratubular LCCSCT: gynecomastia in intratubular form; PRKARIA in LCCSCT vs. STK11 in intratubular LCCSCT * Myoid gonadal stromal tumor-emerging entity * \>5cm, infiltrative border, cytologic atypia, \> 5 mitoses, vascular invasion, necrosis, extratesticular growth

Answer 63

Testes: Hematolymphoid Tumors * 1-2% of all lymphomas * 4% of extranodal lymphomas * 5% of testicular neoplasms * Most common testicular neoplasm in \>50 yr * 80-90% DLBCL * Follicular, NK/T cell nasal-type, Burkitt, plasmablastic, B and T lymphoblastic, peripheral T cell, and anaplastic large cell lymphoma, myeloid sarcoma rarely occur

Answer 64

Membranous Nephropathy

Answer 65

Choriocarcinoma * presents as vaginal bleeding or mets * Gross: hemorrhagic tumor mass, extensive necrosis * Histology: * hallmark: presence of all three trophoblast cell types * cytotrophoblasts * intermediate trophoblasts * syncytiotrophoblasts * Chorionic villi are absent * Hemorrhage and necrosis * Mitotic activity is brisk * does not make its own blood vessels or tumor stroma --\> extensive necrosis * Chemo most effective in chorio arising from complete hydatidiform moles, best prognosis * Intraplacental choriocarcinomas are usually small and are most commonly identified on gross examination as a blood clot or an area that appears to surround an infarct * Stains * trophoblasts: CK+ * Intermediate trophoblasts: hPL+(strong), β-hCG+(weak) * Syncytiotrophoblasts: hPL+(weak), β-hCG+(strong) * absence of nuclear β-catenin (normal POC+)

Answer 66

Pauciimmune Crescentic Glomerulonephritis

Answer 67

Pauciimmune Crescentic Glomerulonephritis * At least 80% of patients with pauciimmune crescentic glomerulonephritis test seropositive for antineutrophilic cytoplasmic antibody (ANCA). * ANCA has specificity for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) on enzyme-linked immunosorbent assay. * By definition, in pauciimmune crescentic glomerulonephritis, immunofluorescence reveals minimal to absent positivity for immunoglobulins and complement. * The pathogenesis of pauciimmune crescentic glomerulonephritis involves ANCA-mediated neutrophil activation, causing neutrophil degranulation on endothelial surfaces and leading to vasculitis and crescentic glomerulonephritis.

Answer 68

Pauciimmune Crescentic Glomerulonephritis • Alternative causes of crescentic glomerulonephritis include * anti-glomerular basement membrane (GBM) disease, which shows linearstaining for IgG, kappa, and lambda along the GBM * immune complex–mediated forms of crescentic glomerulonephritis (e.g., lupus nephritis (LN) and IgA nephropathy), which show granular deposits of immunoglobulins and complement on immunofluorescence and electron microscopy.

Answer 69

Adenomatoid tumor situated on the testicular surface * benign neoplasm, curable by local resection * mesothelial cell origin * can present in any age group from adolescence to senescence * most common tumor of the epididymis and can also occur in the spermatic cord and paratestes * Grossly they are often firm white well-circumscribed nodules * Histology can vary * some lesions containing spaces resembling vascular channels, and others having more glandular or tubular structures suggesting an epithelial neoplasm * sometimes, signet ringlike cells are present, simulated by the presence of cytoplasmic vacuoles * cells have abundant eosinophilic cytoplasm and the nuclei are small with occasional inconspicuous nucleoli * can be infiltrative microscopically and are present in a fibrotic background * some are associated with abundant smooth muscle; these are known as adenomatoid leiomyomas * muscle cells will be positive for smooth muscle actin (SMA), but not the cells lining the channels and spaces * Positive: calretinin and epithelial markers, such as AE1AE3, epithelial membrane antigen (EMA), Cam5.2, CK5/6, and CK7 * Negative: CD31 and CD34 * In difficult cases, in which the differential diagnosis is metastatic adenocarcinoma, a panel to include markers that are positive in carcinoma and not in mesothelial proliferation may include carcinoembryonic antigen (CEA), factor VIII-related antigen, HBME-1,MOC31, BER-EP4, B72.3, and CD15.

Answer 70

Mesoblastic nephroma * Congenital mesoblastic nephroma is the most common renal tumor in newborns. * Microscopically, it is divided into three subtypes: (1) Classic: less common, resembles infantile fibromatosis or leiomyoma with fascicles and whorls of bland spindled myofibroblasts and thin collagen fibers; tumor surrounds tubules and glomeruli, has irregular borders; chondroid metaplasia / dysplasia of the entrapped tubules is common; mitoses are rare; necrosis / desmoplasia are not present (2) Cellular: resembles infantile fibrosarcoma with a sheet-like proliferation of plump, atypical spindle cells with abundant cytoplasm, vesicular nuclei and nucleoli; frequent mitotic figures (25-30 / 10 HPF) and necrosis; the tumor has a pushing border * t(12;15), which results in ETV6-NTRK3 gene fusion, which is similar to the translocation seen in infantile fibromatosis (3) the mixed subtype, which is a combination of both types • Most cases, including the cellular variant, are cured by surgical excision.

Answer 71

Ileal urine * These degenerated cells with pyknotic nuclei resemble histiocytes, but are degenerated ileal glandular epithelial cells in ileal urine. They usually lack a columnar appearance because epithelial cells tend to “round up” in fluid, and they frequently resemble macrophages. * Ileal urine commonly has a granular background containing bacteria, cellular debris, and inflammatory cells. This background debris may make it difficult to identify neoplastic cells from a recurrent or metachronous tumor; however, the malignant cells actually may be better preserved than the degenerated intestinal cells, and will show cytologic features characteristic of malignant urothelial cells. * Cytologic changes due to radiation of urothelium are similar to its effects on other types of epithelial cells; features include marked cell enlargement (cytomegaly; enlargement of both the nucleus and the cell itself), vacuolization of the cytoplasmic and nucleus, “smudged” nuclei, and polychromatic or two-tone cytoplasmic staining. * Radiation changes may persist for years following treatment. Examination of voided urine is not a primary method of diagnosing prostatic diseases. In fact, it is very uncommon to find normal prostatic cells or evidence of benign disorders of the prostate in voided urine. * Neutrophils, lymphocytes, and histiocytes almost always are present in ileal urine specimens and do not necessarily indicate an infectious process.

Answer 72

Thin Basement Membrane Nephropathy * The clinical presentation of microscopic hematuria, in the absence of significant proteinuria or renal insufficiency, is most commonly encountered in the following three renal conditions: thin basement membrane nephropathy (TBMN), hereditary nephritis (i.e., Alport syndrome), and IgA nephropathy. * Before a patient with microscopic hematuria undergoes renal biopsy, possible urologic causes for hematuria must be excluded. * The glomeruli in TBMN usually appear unremarkable on light microscopy. The normal glomerular basement membrane (GBM) thickness averages approximately 320 nm in women and 350 nm in men. Diagnostic criteria for TBMN are diffuse GBM thinning, with mean thickness less than 225 nm in women and less than 250 nm in men. * The main clinical finding in TBMN is isolated microscopic hematuria. Many patients have a family history of isolated hematuria with autosomal dominant transmission. * TBMN results from a heterozygous mutation in either COL4A3 or COL4A4; these genes encode the alpha-3 (COL4A3) and alpha-4 (COL4A4) chains of collagen IV. * TBMN is a nonprogressive condition that is often referred to clinically as benign essential hematuria.

Answer 73

Hyaline membrane disease * Hyaline membrane disease (HMD) is most commonly seen in white male infants weighing less than 2500 grams. * Besides prematurity, other predisposing conditions include maternal diabetes, twin gestation, and caesarean section without trial of labor. * Hyaline membranes form in about 2 to 3 hours after onset of respiratory distress (won’t be seen in stillborns or in infants who died within an hour of birth). * HMD is caused by deficiency in surfactant protein B, caused either by prematurity or genetic mutation. * Bronchopulmonary dysplasia (chronic lung disease of infancy) is a complication of the treatment of HMD. * Exogenous surfactant administration significantly improves morbidity and mortality

Answer 74

Secondary tumors involving the penis * In spite of the rich vascularity of the penis, it is a rare site for metastatic carcinoma. * All of the carcinomas listed have been reported to metastasize to the penis; however, genitourinary tumors, particularly of **prostatic and urinary bladder origin, are the most common primary site for tumors metastasizing to the penis**. * In the penis, most metastases are within the vascular channels of the erectile tissues of the penis. This is usually manifested as multiple painless subcutaneous nodules. Rarely, malignant priapism can occur. * The distinction between metastatic urothelial carcinoma of the urinary bladder and primary penile urethral carcinoma can be difficult. Demonstration of in situ urothelial carcinoma at the site in the penile urethra can be a helpful clue that it is a primary carcinoma.

Answer 75

Dysproteinemic Renal Disease * The particular amino acid sequence of a monoclonal light chain dictates its biochemical properties, which determine the propensity of the light chain to form light chain amyloidosis, light chain deposition disease (LCDD), or light chain cast nephropathy (i.e., myeloma cast nephropathy). * The lambda light chain isotype is seen in most cases of primary amyloidosis. In contrast, the kappa isotype predominates in myeloma cast nephropathy and LCDD. * In approximately 90% of cases of LCDD, the deposits stain solely for kappa light chain. A monoclonal lambda light chain is responsible for the remaining 10%. By definition, Congo red staining for amyloid is negative. * In approximately 75% of cases of light chain amyloidosis, the deposits stain for lambda light chain. A monoclonal kappa light chain is responsible for the remaining 25%. By definition, Congo red staining reveals apple-green birefringence when viewed under polarized light. * Myeloma cast nephropathy or light chain cast nephropathy is the most common pattern of renal involvement in patients with multiple myeloma, and the usual manifestation is acute renal failure. The light microscopy findings in myeloma cast nephropathy include distinctive tubular casts and widespread tubular injury. Immunofluorescence microscopy demonstrates intense staining of the casts for either kappa or lambda light chain; staining for the reciprocal light chain is typically weak or negative. Kappa staining is seen in most cases.

Answer 76

Autosomal Dominant Polycystic Kidney Disease (ADPKD) * one of the most common human heritable diseases, affecting approximately 1:500 to 1:1000 individuals * bilateral renal cyst formation, leading to massively enlarged kidneys * expanded renal parenchyma comprises a massive aggregate of cysts lined by flattened-to-cuboidal epithelium * presentation * 20-40 yrs * renal insufficiency, hypertension, hematuria, and renal colic * slowly progressive, most develop ESRD (8-10% of all patients with ESRD in the US) * most common extrarenal manifestations * hepatic cysts, 50% of patients, may cause pain, not a/w functional impairmen * complications that reflect the expression of polycystin in vascular smooth muscle * Cardiac valve abnormalities, mitral valve prolapse, 25% of patients * Intracranial berry aneurysms, 5% to 8% of patients * 85% mutation in the gene PKD1 (chromosome 16) * 13% mutation in the gene PKD2 (chromosome 4) * gene products, polycystin-1 and polycystin-2, are proteins that regulate renal epithelial cell proliferation and differentiation * mechanism of cyst formation requires an inherited (autosomal dominant) mutation of one allele and the development of a somatic mutation (second hit) in the second allele

Answer 77

Nephrotic Syndrome * The most common cause of nephrotic syndrome varies depending on age and ethnic group. * The most common cause of primary nephrotic syndrome in children is minimal change disease. * Different sources differ with respect to whether the most common cause of nephrotic syndrome in nondiabetic adults is membranous nephropathy or focal segmental glomerulosclerosis. * Focal segmental glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome in African American adults. * Patients with IgA nephropathy typically present with hematuria and subnephrotic proteinuria; full nephrotic syndrome is unusual in IgA nephropathy.

GU Flashcards

(107 cards)