Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

PATHOLOGY > Pediatric Tumors > Flashcards

Pediatric Tumors Flashcards

1
Q

under the SIOP protocol, a history of wedge biopsy prior to chemotherapy or surgery with upstage the post-chemotherapy pediatric renal tumor to Stage__

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

In the SIOP protocol, history of these biopsies does not upstage post-chemotherapy pediatric renal tumor but the size of the needle gauge should be mentioned to the pathologist.

A
  • Fine needle aspiration biopsy; or

- percutaneous core needle (“tru-cut”) biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

In the SIOP protocol, what tumor histologic types needs to subtyped (4)?

A
  • Wilms Tumor (nephroblastoma)
  • Mesoblastic nephroma
  • Clear cell sarcoma of the Kidney
  • Rhabdoid tumor of the Kidney
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

SIOP protocol

Categorize the risk (Low, Intermediate, or High) of these tumor subtypes:

  • Mesoblastic nephroma
  • Cystic partially differentiated nephroblastoma
  • Nephroblastoma-completely necrotic
A

Low-risk

MCN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

SIOP protocol

Categorize the risk (Low, Intermediate, or High) of these tumor subtypes:

  • Nephroblastoma-blastemal type
  • Nephroblastoma-diffuse anaplasia type
  • Clear Cell Sarcoma of the kidney
  • Rhabdoid tumor of the kidney
A

High-risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

SIOP protocol

Categorize the risk (Low, Intermediate, or High) of these tumor subtypes:

  • Nephroblastoma-epithelial type
  • Nephroblastoma-stromal type
  • Nephroblastoma-mixed type
  • Nephroblastoma-regressive type
  • Nephroblastoma-focal anaplasia type
A

Intermediate-risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

SIOP protocol

Only Nephroblastoma subtype that is Low-risk

A

Nephroblastoma-completely necrotic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

SIOP protocol

Two Nephroblastoma subtypes that are high-risk

A
  • Nephroblastoma-blastemal type

- Nephroblastoma-diffuse anaplasia type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

SIOP protocol

Nephroblastoma-anaplasia type that has Intermediate-risk

A

-Nephroblastoma-focal anaplasia type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

SIOP protocol

Nephroblastoma-anaplasia type that has High-risk

A

-Nephroblastoma-diffuse anaplasia type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

SIOP protocol

Criteria for anaplasia in a RESECTION specimen (3):

A
  1. atypical (tri/multipolar) mitotic figures
  2. marked nuclear enlargement (defined as diameters 3X of adjacent cells)
  3. hyperchromatic tumor cell nuclei.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

SIOP protocol

In a RESECTION specimen, Anaplasia is defined as presence of _ three of these criteria.

A

ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

SIOP protocol

For BIOPSY specimens, anaplasia can be mentioned if the tumor meets at least _ of these criteria.

A

ONE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

SIOP protocol

Criteria for anaplasia in a BIOPSY specimen (2):

A

(1) atypical (tri/multipolar) mitotic figures; OR

2) marked nuclear enlargement (defined as diameters 3X of adjacent cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

SIOP protocol

__ do NOT qualify as anaplasia and should not be included in the assessment of nuclear enlargement.

A

rhabdoymyoblastic foci

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

SIOP protocol

defined as anaplasia involving a clearly defined focus within the primary intrarenal tumor

A

Focal anaplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

SIOP protocol

The criteria for focal anaplasia must meet _ of the following criteria

A

ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

SIOP protocol

Criteria for focal anaplasia (5):

A
  1. anaplasia present in less than five foci in the intrarenal tumor;
  2. absence of anaplasia in the renal vessels;
  3. absence of anaplasia outside the kidney,
  4. anaplastic focus should be completely surrounded by non-anaplastic tissue; AND
  5. the rest of the non-anaplastic tumor must NOT show severe nuclear unrest
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

SIOP protocol

In focal anaplasia, this is defined as enlarged nuclei with no atypical mitosis

A

Severe nuclear unrest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

SIOP protocol

TRUE / FALSE:
For multifocal tumors, specify dimension of each additional tumor in the gross, but not necessarily in the final diagnosis

A

TRUE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

SIOP protocol

Lymph nodes with “involvement by tumor” includes (3):

A
  • viable tumor cells
  • nonviable tumor cells
  • chemotherapy-induced changes in the lymph nodes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

SIOP protocol

STAGE:
The tumor is limited to kidney or surrounded with a fibrous (pseudo)capsule if outside of the normal contours of the kidney. The renal capsule or pseudocapsule may be infiltrated by the tumor but it does not reach the outer surface.

A

Stage I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

SIOP protocol

STAGE:
The tumor may be protruding (“bulging”) into the pelvic system and “dipping” into the ureter
but it is not infiltrating their walls.

A

Stage I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

SIOP protocol

STAGE:
The vessels or the soft tissues of the renal sinus are not involved.

A

Stage I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

SIOP protocol

STAGE:

Intrarenal vessel involvement may be present.

A

Stage I

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

SIOP protocol

STAGE:

Viable tumor penetrates through the renal capsule and/or fibrous pseudocapsule into perirenal fat but is completely resected (resection margins “clear”).

A

Stage II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

SIOP protocol

STAGE:

Viable tumor infiltrates the soft tissues of the renal sinus.

A

Stage II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

SIOP protocol

STAGE:

Viable tumor infiltrates blood and lymphatic vessels of the renal sinus or in the perirenal
tissue but it is completely resected.

A

Stage II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

SIOP protocol

STAGE:

Viable tumor infiltrates the renal pelvic or ureter’s wall.

A

Stage II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

SIOP protocol

STAGE:

Viable tumor infiltrates adjacent organs or vena cava but is completely resected.

A

Stage II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

SIOP protocol

STAGE:
Viable or non-viable tumor extends beyond resection margins.

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

SIOP protocol

STAGE:
Any abdominal lymph nodes are involved.

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

SIOP protocol

STAGE:
Tumor rupture before or intraoperatively (irrespective of other criteria for staging).

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

SIOP protocol

STAGE:
The tumor has penetrated through the peritoneal surface.

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

SIOP protocol

STAGE:
Tumor implants are found on the peritoneal surface.

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

SIOP protocol

STAGE:
The tumor thrombi present at resection margins of vessels or ureter, are transsected or
removed piecemeal by surgeon.

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

SIOP protocol

STAGE:
The tumor has been surgically biopsied (wedge biopsy) prior to preoperative chemotherapy or surgery.

A

Stage III

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

SIOP protocol

STAGE:
Hematogenous metastases (lung, liver, bone, brain, etc) or lymph node metastases outside the abdomino-pelvic region.
A

Stage IV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

SIOP protocol

STAGE:
Bilateral renal tumors at diagnosis. Each side should be sub-staged according to the above criteria.

A

Stage V

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Typical demographics of Lymphoma

A

Children and Adolescents

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Typical demographics of Ewing Sarcoma

A
  • 5yo to 30s

- Caucasians

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Typical demographics of Alveolar Rhabdomyosarcoma

A

Adolescents

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Typical demographics of Neuroblastoma

A

Birth to 5yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Typical demographics of Synovial Sarcoma

A

Teens to 30s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Typical demographics of Wilms Tumor

A

Infants to 6yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Typical demographics of Rhabdoid Tumor

A

Birth to 3yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Typical demographics of MPNST

A
  • Teens to 50s

- 50% also NF1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Typical demographics of Melanoma

A

increasing incidence with age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Typical demographics of Desmoplastic Small Round Cell Tumor

A
  • Teens to 30s

- Males

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Typical Locations of Lymphoma (3)

A
  • Bone Marrow
  • Nodes
  • Thymus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Typical Locations of Ewing Sarcoma (2)

A
  • Diaphyseal bone

- Soft tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Typical Locations of Alveolar Rhabdomyosarcoma (3)

A
  • Head and Neck
  • Extremities
  • Genitourinary
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Typical Locations of Neuroblastoma (2)

A
  • Adrenal gland

- Paraspinal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Typical Locations of Synovial Sarcoma (2)

A
  • Extremities

- Head and Neck

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Typical Location of Wilms Tumor

A

Kidney

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Typical Locations of Rhabdoid tumor (2)

A
  • Kidney

- Soft tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Typical Locations of of MPNST (2)

A
  • Proximal limbs

- Trunk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Typical Locations of Melanoma (2)

A
  • Skin

- Metastatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Typical Locations of DSRCT

A

-Diffuse abdominal disease “Carcinomatosis”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Key histologic features of Lymphoma (2)

A
  • Discohesive

- Lymphoglandular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Key histologic feature of Ewing Sarcoma

A

-Foamy cytoplasm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Key histologic features of Alveolar Rhabdomyosarcoma

A
  • Discohesive

- liner “picket fence” of cells at edges of nests

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Key histologic features of Neuroblastoma (2)

A
  • Neuropil

- Neural differentiation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Key histologic features of Synovial sarcoma (2)

A
  • HPC-vessels

- Variable features

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Key histologic features of Wilms Tumor (2)

A
  • Triphasic

- Glomeruloid differentiation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Key histologic feature of Rhabdoid tumor

A

-Prominent rhabdoid cytology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Key histologic features of MPNST (2)

A
  • Spindled

- relative pleomorphism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

Key histologic feature of Melanoma

A

-Variable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Key histologic features of DSRCT (2):

A
  • Desmoplastic stroma

- Ewing-like cytology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Key Test for Lymphoma

A

CBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Key Tests for Ewing Sarcoma

A
  • CD99+
  • Nkx2.2+
  • EWSR1 fusions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Key Tests for Alveolar Rhabdomyosarcoma

A
  • Myogenin
  • MyoD1
  • Desmin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Key Test for Neuroblastoma

A

PHOX2B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Key Test for Synovial Sarcoma

A

SS18 rearranged

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Key Tests for Wilms Tumor

A
  • WT1 (N-term+, C-term+)
  • Desmin +/-
  • Keratin +/-
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Key Test for Rhabdoid Tumor

A

INI-1 negative

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Key Tests for MPNST

A
  • focal S100

- SOX9/SOX10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Key Tests for Melanoma

A
  • S100

- MART-1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

Key Tests for DSRCT

A
  • Desmin
  • Keratin
  • EWSR1-WT1 translocation
  • WT1 (N-term-, C-term+)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Key Molecular Feature of Lymphoblastic Lymphoma

A

Hyperdiploid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Favorable / Unfavorable:

The hyperdiploid molecular feature in Lymphoblastic Lymphoma

A

Favorable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Key Molecular Features of Burkitt Lymphoma (3):

A
  • t(8;14) - MYC-IgH
  • t(2;8) - Kappa-MYC
  • t(8;22) - MYC-Lambda
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

Key Molecular Features of Ewing Sarcoma (2):

A
  • t(11;22) - EWSR1-FLI1

- t(21;22) - EWSR1-ERG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Key Molecular Features of Alveolar Rhabdomyosarcoma (2)

A
  • t(1;13) - PAX7-FOXO1

- t(2;13) - PAX3-FOXO1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

Key Molecular Feature of Neuroblastoma:

A

MYCN amplification (high risk)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

Key Molecular Features of Synovial Sarcoma

A

t(X;18) SS18-SSX1, 2, 4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

Key Molecular Feature of DSRCT:

A

t(11;22) EWSR1-WT1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

Key Molecular Feature of Rhabdoid Tumor

A

INI-1 (SMARCB1) deletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

SIOP protocol

Three tumor interfaces needed to block:

A
  • Tumor-kidney interface
  • Tumor-capsule interface
  • Tumor-renal sinus interface
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

SIOP protocol

In subtyping of post-treated Wilms Tumor, percentage of WHAT needs to be assessed? (2)

A
  • Necrosis

- Different components of the tumor (blastemal, epithelial, stromal)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

SIOP protocol

TRUE/FALSE:
Tumors with focal anaplasia should still be subtyped on the basis of other components

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed Necrosis = 100%

A

Completely Necrotic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed Necrosis >66%

A

Regressive type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed Necrosis <66%

A

-Assess Blastemal Component in Viable tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed Blastemal component >66%

A

Blastemal type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed Blastemal component 10%-66%

A

Mixed type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed Blastemal component <10%

A

-Assess other components in Viable tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed other components (Epithelial >66%)

A

Epithelial type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed other components (Stromal >66%)

A

Stromal type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

SIOP protocol

post-treated Wilms Tumor:

Assessed other components (No predominant)

A

Mixed type

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

SIOP protocol

Renal sinus vascular involvement is easy to confirm when (2):

A
  • Tumor fills the lumen; OR

- Tumor invades the vascular wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

SIOP protocol

Displacement artifact is also readily identified when (3)

A
  • present in arterial lumina
  • accompanied by abundant displacement artifact elsewhere
  • ink is present within the aggregates
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

The protocol for pediatric extragonadal germ cell tumors is only applied to tumors located in the: (5)

A
  • Mediastinum
  • Sacrococcygeal area
  • Retroperitoneum
  • Neck
  • Intracranial sites
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

Pediatric EGGCT protocol

Congenital/neonatal age group

A

Birth to 6 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

Pediatric EGGCT protocol

Childhood/prepubertal age group

A

7 months to 11 years old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

Pediatric EGGCT protocol

Postpubertal/adult

A

greater than or equal to 12 years old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

Pediatric EGGCT protocol

Head and Neck region tumor site INCLUDES

A

-Thyroid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

Pediatric EGGCT protocol

Head and Neck region tumor site EXCLUDES

A

-Intracranial

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
109
Q

Pediatric EGGCT protocol

Mediastinum tumor site includes (4):

A
  • Pericardium
  • Heart
  • Thymus
  • Lung
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
110
Q

Pediatric EGGCT protocol

Histologic (Norris) Grade:
Neoplasms with some immaturity (of any cell type), but with immature neuroepithelium absent or limited to collectively occupying no more than one 4x objective, on a single slide, ≤1 LPF.

A

Grade 1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
111
Q

Pediatric EGGCT protocol

Histologic (Norris) Grade:
Neoplasms with more immaturity and primitive neuroepithelium greater than 1 and not exceeding 3 low-power (4x objective) fields per slide, between 1-3 LPF’s.

A

Grade 2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
112
Q

Pediatric EGGCT protocol

Histologic (Norris) Grade:
Neoplasms in which immaturity and primitive neuroepithelium are prominent, primitive neuroepithelium present in >3 low-power (4x objective) fields per slide, >3 LPFs.

A

Grade 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
113
Q

Pediatric EGGCT protocol

Which type of resection specimen does Microscopic Tumor Extension needs to be reported?

A

Sacrococcygeal resections ONLY

114
Q

Pediatric EGGCT protocol

Which of the elements that needs to be reported is optional but still needed to be indicated if present? (2)

A
  • LVI

- PNI

115
Q

Pediatric EGGCT protocol

This staging for any Malignant EGGCT is based on the pretreatment tumor characteristics

A

Children’s Oncology Group (COG) staging

116
Q

Pediatric EGGCT protocol

COG stage:

  • Complete resection at any site;
  • coccygectomy for sacrococcygeal site;
  • negative tumor margins
A

Stage I

117
Q

Pediatric EGGCT protocol

COG stage:

  • Microscopic residual;
  • lymph nodes negative
A

Stage II

118
Q

Pediatric EGGCT protocol

COG stage:
-Lymph nodes involved by metastatic disease.
-Gross residual or biopsy only, retroperitoneal
nodes negative or positive

A

Stage III

119
Q

Pediatric EGGCT protocol

COG stage:
-Distant metastases, including liver

A

Stage IV

120
Q

Pediatric EGGCT protocol

What serologic markers are needed?

A
  • alpha-fetoprotein (AFP)

- human chorionic gonadotropin (HCG)

121
Q

Pediatric EGGCT protocol

What are needed to be identified when grossing sacrococcygeal tumors?

A
  • Coccyx

- Soft tissue margins

122
Q

Pediatric EGGCT protocol

At least _ section per centimeter of the tumor’s greatest dimension.

A

one

123
Q

Pediatric EGGCT protocol

Within the pediatric age range, prognosis is worse with _ age.

A

increasing

124
Q

Pediatric EGGCT protocol

For _ age group, immaturity is not predictive of malignant behavior

A

congenital/neonatal

125
Q

Pediatric EGGCT protocol

For congenital/neonatal age group, completeness of resection is more associated with recurrences of a _ tumor

A

pure yolk sac tumor

126
Q

Pediatric EGGCT protocol

For prepubertal/child age group, grade 2 to 3 immaturity are more frequently associated with _ tumor

A

admixed yolk sac tumor

127
Q

Pediatric EGGCT protocol

For the _ age group, no grading schema for extragonadal immature teratomas is used at present, however, it is reasonable to report the percentage of immature elements

A

postpubertal

128
Q

Pediatric EGGCT protocol

In specimens treated with chemotherapy prior resection, the tissue usually shows (4):

A
  • Necrosis
  • Fibrosis
  • Mixed inflammatory infiltrates
  • Xanthogranulomatous inflammation
129
Q

Pediatric EGGCT protocol

Less than _% viable tumor cells are a good prognostic factor as defined by the International Germ Cell Consensus Classification Group (IGCCCG)

A

10%

130
Q

RB protocol

All measurements are in _

A

Millimeters

131
Q

RB protocol

Histologic Grade:

Tumor with areas of retinocytoma (fleurettes or neuronal differentiation)

A

G1

132
Q

RB protocol

Histologic Grade:

Tumor with many rosettes (Flexner-Wintersteiner or Homer Wright)

A

G2

133
Q

RB protocol

Histologic Grade:

Tumor with occasional rosettes (Flexner-Wintersteiner or Homer Wright)

A

G3

134
Q

RB protocol

Histologic Grade:

Tumor with poorly differentiated cells without rosettes and/or with extensive areas (more
than half of tumor) of anaplasia

A

G4

135
Q

RB protocol

Histologic Grade:

Grade cannot be assessed

A

GX

136
Q

RB protocol

Choroid extension of solid tumor is less than 3 mm in maximum diameter (width or thickness)

A

Minimal

137
Q

RB protocol

Choroid extension of solid tumor is more than 3 mm in maximum diameter (width or thickness)

A

Massive

138
Q

RB protocol

What part of the eye should be mentioned if the tumor involves less than or more than two-thirds?

A

Sclera

139
Q

RB protocol

The depth of the tumor invasion into the optic nerve should be measured from the (3):

A
  1. Limiting membrane of the optic disc
  2. Exit of the large central vessels
  3. level of the Bruch membrane
    * in decreasing order
140
Q

RB protocol

Primary Tumor (pT):

Unknown evidence of intraocular tumor

A

pTX

141
Q

RB protocol

Primary Tumor (pT):

No evidence of intraocular tumor

A

pT0

142
Q

RB protocol

Primary Tumor (pT):

Intraocular tumor(s) without any local invasion, focal choroidal invasion, or pre- or
intralaminar involvement of the optic nerve head
A

pT1

143
Q

RB protocol

Primary Tumor (pT):

Intraocular tumor(s) with local invasion

A

pT2

144
Q

RB protocol

Primary Tumor (pT):

Concomitant focal choroidal invasion and pre- or intralaminar involvement of the optic nerve head

A

pT2a

145
Q

RB protocol

Primary Tumor (pT):

Tumor invasion of stroma of iris and/or trabecular meshwork and/or Schlemm’s canal

A

pT2b

146
Q

RB protocol

Primary Tumor (pT):

Intraocular tumor(s) with significant local invasion

A

pT3

147
Q

RB protocol

Primary Tumor (pT):

Massive choroidal invasion (>3 mm in largest diameter, or multiple foci of focal choroidal involvement totaling >3 mm, or any full-thickness choroidal involvement)

A

pT3a

148
Q

RB protocol

Primary Tumor (pT):

Retrolaminar invasion of the optic nerve head, not involving the transected end of the optic nerve

A

pT3b

149
Q

RB protocol

Primary Tumor (pT):

Any partial-thickness involvement of the sclera within the inner two thirds

A

pT3c

150
Q

RB protocol

Primary Tumor (pT):

Full-thickness invasion into the outer third of the sclera and/or invasion into or around emissary channels

A

pT3d

151
Q

RB protocol

Primary Tumor (pT):

Evidence of extraocular tumor:

  • tumor at the transected end of the optic nerve
  • tumor in the meningeal spaces around the optic nerve
  • full-thickness invasion of the sclera with invasion of the episclera, adjacent adipose tissue, extraocular muscle, bone, conjunctiva, or eyelids
A

pT4

152
Q

RB protocol

Regional Lymph Nodes (pN):

Regional lymph nodes cannot be assessed

A

pNX

153
Q

RB protocol

Regional Lymph Nodes (pN):

No regional lymph node involvement

A

pN0

154
Q

RB protocol

Regional Lymph Nodes (pN):

Regional lymph node involvement

A

pN1

155
Q

RB protocol

Distant Metastasis (pM) (required only if confirmed pathologically in this case):

Distant metastasis with histopathologic confirmation

A

pM1

156
Q

RB protocol

Distant Metastasis (pM) (required only if confirmed pathologically in this case):

Histopathologic confirmation of tumor at any distant site (eg. bone marrow, liver, or
other)

A

pM1a

157
Q

RB protocol

Distant Metastasis (pM) (required only if confirmed pathologically in this case):

Histopathologic confirmation of tumor in the cerebrospinal fluid or CNS parenchyma

A

pM1b

158
Q

RB protocol

Definition of Heritable Trait (H):

Unknown or insufficient evidence of a constitutional RB1 gene mutation.

A

HX

159
Q

RB protocol

Definition of Heritable Trait (H):
Normal RB1 alleles in blood tested with demonstrated high-sensitivity assays

A

H0

160
Q

RB protocol

Definition of Heritable Trait (H):
Bilateral retinoblastoma, any retinoblastoma with an intracranial primitive neuroectodermal tumor (ie, trilateral retinoblastoma), patient with family history of retinoblastoma, or molecular definition of a constitutional RB1 gene mutation

A

H1

161
Q

RB protocol

Pathologic stage group:
pT - pT1, pT2, pT3
pN - pN0
M - cM0
pH - any
A

I

162
Q

RB protocol

Pathologic stage group:
pT - pT4
pN - pN0
M - cM0 
pH - any
A

II

163
Q

RB protocol

Pathologic stage group:
pT - any
pN - pN1
M - cM0
pH - any
A

III

164
Q

RB protocol

Pathologic stage group:
pT - any
pN - any
M - cM1 or pM1
pH - any
A

IV

165
Q

RB protocol

What should be sampled first if fresh tumor samples are needed for genetic studies?

A

Optic nerve margin

166
Q

RB protocol

Fix the specimen for _ hours in buffered formalin in a _ ratio

A
  • 48 hours

- 10:1

167
Q

RB protocol

The cornea is:

A

Wider than taller

168
Q

RB protocol

The optic nerve attachment is:

A

skewed nasally (medially)

169
Q

RB protocol

Superior oblique muscle insertion is at the:

A

-UO quadrant (superotemporal) behind superior rectus insertion

170
Q

RB protocol

The tendon and muscle fibers of superior oblique muscle run towards the:

A

superonasal aspect

171
Q

RB protocol

Inferior oblique muscle wide insertion is at the:

A

inferotemporal (inferolateral) quadrant of sclera

172
Q

RB protocol

Inferior oblique muscle wide insertion is _ to the optic nerve

A

temporal (lateral)

173
Q

RB protocol

What structure marks the equatorial (horizontal) plane?

A

Long posterior ciliary arteries

174
Q

RB protocol

What structure exits at 45 degrees from the sagittal (vertical) and equatorial planes?

A

Vortex veins

175
Q

RB protocol

This invasion is identified when there are groups of tumor cells present in the open spaces between intraocular structures, extraocular tissues and/or subarachnoid space.

A

Artifactual invasion

176
Q

RB protocol

This invasion is defined as 1 or more solid nests of tumor cells that fills or replaces the choroid and has pushing borders

A

True invasion

177
Q

RB protocol

Invasion of this is not a form of choroidal invasion

A

sub-retinal pigment epithelium (RPE) space

-where tumor cells are present under the RPE (but not beyond Bruch’s membrane into the choroid)

178
Q

RB protocol

This invasion is defined as a solid nest of tumor that measures less than 3 mm in maximum diameter (width or thickness)

A

Focal choroidal invasion

179
Q

RB protocol

This invasion is defined as a solid tumor nest 3 mm or more in maximum diameter (width or thickness) in contact with the underlying sclera.

A

Massive choroidal invasion

180
Q

Histologic features:

  • No schwannian stroma / No Neuropil
  • Requires adjunctive diagnostic tests

Diagnosis?

A

Neuroblastoma, undifferentiated

181
Q

Histologic features:

  • Schwannian stroma poor
  • Presence of neuropil allows for H&E diagnosis
  • Less than 5% differentiating neuroblasts
A

Neuroblastoma, Poorly differentiated

182
Q

Histologic features:

  • Schwannian stroma poor
  • More abundant neuropil
  • > 5% differentiating neuroblasts
A

Neuroblastoma, Differentiating

183
Q

Histologic features:

  • Schwannian stroma comprises >50% of the tumor
  • Microscopic foci of neuropil contain neuroblastic cells at varying stages of maturation
  • No macroscopic nodules present
A

Ganglioneuroblastoma, intermixed

184
Q

Histologic features:

  • Schwannian stroma is the predominant element of the tumor
  • Scattered ganglion cells are mature or maturing
  • No neuropil is present
A

Ganglioneuroma, maturing

185
Q

Histologic features:

  • Schwannian stroma is the predominant element of the tumor
  • Scattered ganglion cells are mature with surrounding satellite cells
  • No neuropil is present
A

Ganglioneuroma, mature

186
Q

Histologic features:

  • Composite stroma rich/dominant and stroma poor
  • Grossly visible nodule consists of any type of stroma poor neuroblastoma
  • Background of ganglioneuroblastoma or ganglioneuroma
  • Nodular component should also be classified
A

Ganglioneuroblastoma, Nodular

187
Q

INPC:

Any age with Favorable Histology (2)

A
  • Ganglioneuroblastoma, intermixed

- Ganglioneuroma, mature or maturing

188
Q

INPC:

<18 months with Favorable Histology (2)

A
  • Neuroblastoma, poorly differentiated, with low or intermediate MKI
  • Neuroblastoma, differentiating, with low or intermediate MKI
189
Q

INPC:

18-60 months with Favorable Histology (1)

A

Neuroblastoma, differentiating, with low MKI

190
Q

INPC:

Any age with Unfavorable histology (2)

A
  • Neuroblastoma, undifferentiated

- Neuroblastoma, of any subtype, with high MKI

191
Q

INPC:

18-60 months with Unfavorable histology (2):

A
  • Neuroblastoma, poorly differentiated

- Neuroblastoma, differentiating, with intermediate MKI

192
Q

INPC:

> 60 months with Unfavorable histology

A

Neuroblastoma of any subtype

193
Q

INPC

Count of all mitotic and karyorrhectic figures present per 5000 neuroblasts

A

Mitosis-karyorrhexis index (MKI)

194
Q

INPC

Category of MKI:

  • <100/5000
  • 2%
A

Low

195
Q

INPC

Category of MKI:

  • 100-200/5000
  • 2%-4%
A

Intermediate

196
Q

INPC

Category of MKI:

> 200/5000
->4%

A

High

197
Q

Tumors of the neuroblastoma group are defined as embryonal tumors of the sympathetic nervous system derived from the __

A

Neural crest

198
Q

Tumors of the neuroblastoma group arise in the (3):

A
  • Adrenal medulla
  • Paravertebral sympathetic ganglia
  • Sympathetic paraganglia, such as the Organ of Zuckerkandl
199
Q

Most common solid neoplasm in childhood other than CNS tumors accounting for approximately 15% of all neoplasms encountered in the first 4 years of life

A

Neuroblastic tumors

200
Q

The neural crest is known to give rise to (6):

A
  • cells of the future Adrenal medulla
  • neuronal cells of the autonomic NS
  • Schwannian cells
  • Melanocytes
  • some types of NEC
  • mesenchymal-type tissue in the H and N region
201
Q

Types of unique biologic behaviors of Neuroblastic tumors (3)

A
  • Involution / Spontaneous regression
  • Maturation
  • Aggressive proliferation
202
Q

Type of Biologic behavior of NTs:

characterized by massive cellular death of still-immature neuroblasts before complete differentiation.

A

Involution / Spontaneous regression

203
Q

Aggregates of immature neural crest cells

A

Neuroblastic nodules

204
Q

Two main cell populations of Neuroblastic tumors:

A
  • Neuroblastic / Ganglionic cells

- Schwann cells

205
Q

Microscopically, these are composed of neuroblastic cells that form groups or nests separated by delicate, often in- complete stromal septa without or with limited Schwannian proliferation

A

Schwannian stroma-poor tumors

206
Q

characterized by a ganglioneuromatous appearance with mature and/or maturing ganglion cells individually scattered in a background of highly developed Schwannian stroma (2)

A
  • Schwannian stroma-rich tumors

- Schwannian stroma-dominant tumors

207
Q

The INPC has restricted the term ganglioneuroblastoma to those tumors with two distinctive components:

A

1) a mature Schwannian stromal component with individually scattered mature and/or maturing ganglion cells (i.e., ganglioneuromatous tissue); AND
2) a neuroblastic component

208
Q

This term refers to the ganglioneuromatous component of the tumor

A

Ganglio-

209
Q

The neuroblastic component can be seen (2):

A

1) as multiple microscopic foci (ganglioneuroblastoma, intermixed subtype); or
2) in distinct macroscopic and commonly hemorrhagic nodule(s) (ganglioneuroblastoma, nodular subtype)

210
Q

In neuroblastic tumors, this is defined as the number of tumor cells in mitosis and in the process of karyorrhexis

A

Mitosis karyorrhexis index (MKI)

211
Q

in MKI, high cellularity is

A

700-900 cells per HPF

212
Q

in MKI, moderate cellularity is

A

400-600 tumor cells

213
Q

in MKI, low cellularity with extensive neuropil

A

100-300 cells per HPF

214
Q

In highly cellular tumors, the MKI can be determined in:

A

6-8 HPFs (x400)

215
Q

in tumors with a low cellularity with a prominent neuropil, MKI can be determined in:

A

20 or more HPFs

216
Q

in MKI, these are characterized by more or less rod-shaped condensations of chromatin with spiked projections and the absence of a nuclear membrane

A

Mitotic figures

217
Q

in MKI, these show condensed and fragmented nuclear material usually accompanied by condensed eosinophilic cytoplasm

A

Karyorrhectic cells

218
Q

This is determined by counting of 10 contiguous HPFs at x400 magnification

A

Mitotic rate (MR)

219
Q

Low MR class

A

less than or equal to 10 mitoses in 10 HPFs

220
Q

a characteristic feature of embryonal tissues and some solid neoplasms of infancy, without the connotation that the latter are overtly malignant or aggressive tumors

A

Higher mitotic rate

221
Q

in NTs, this appears as dense basophilic clumps or amorphous granular material in areas of viable or necrotic tumor

A

Calcification

222
Q

defines tumors that show an unequivocal categorization, although the subtype cannot be determined be- cause of poor quality of the sections, extensive hemorrhage, cystic degeneration, necrosis, crush artifact, and/or diffuse calcification

A

Neuroblastoma (Schwannian stroma-poor), NOS

223
Q

refers to the rare, but observed, tumor with a stroma-rich appearance containing area(s) of extensive calcification that may obscure a stroma-poor neuroblastic nodule.

A

Ganglioneuroblastoma, NOS

224
Q

INSS Stage:

Localized tumor with complete gross excision

A

Stage I

225
Q

INSS Stage:

Ipsilateral “non-adherent” lymph node negative
microscopically

A

Stage I

226
Q

INSS Stage:

May include positive margins (microscopic residual disease)

A

Stage I

227
Q

INSS Stage:

May include positive lymph node if adherent to
resected tumor

A

Stage I

228
Q

INSS Stage:

Localized tumor with incomplete gross excision

A

Stage II

229
Q

INSS Stage:

Ipsilateral “non-adherent” lymph node negative
microscopically

A

Stage IIA

230
Q

INSS Stage:

Ipsilateral “non-adherent” lymph node positive
microscopically

A

Stage IIB

231
Q

INSS Stage:

Any enlarged contralateral lymph node
confirmed microscopically negative

A

Stage IIB

232
Q

INSS Stage:

Unresectable unilateral tumor crossing vertebral column with or without regional lymph node involvement

A

Stage III

233
Q

INSS Stage:

Localized unilateral tumor with contralateral lymph node involvement

A

Stage III

234
Q

INSS Stage:

Unresectable midline tumor with bilateral tumor infiltration or lymph node involvement

A

Stage III

235
Q

INSS Stage:

Any primary tumor with distant metastasis to lymph node, bone, bone marrow, liver, skin or other organs not defined as stage IVS

A

Stage IV

236
Q

INSS Stage:

Infant younger than 12 months

A

Stage IVS

237
Q

INSS Stage:

Localized primary tumor (stage I, IIA, or IIB) with dissemination limited to skin, liver, and/or bone marrow

A

Stage IVS

238
Q

INSS Stage:

Bone marrow involvement must have <10% cellularity

A

Stage IVS

239
Q

INSS Stage:

Bone marrow must be metaiodobenzyl guanidine scan negative

A

Stage IVS

240
Q

Most common neoplasm of childhood, typically presenting as a leukemia

A

Lymphoblastic leukemia/lymphoma

241
Q

Lymphoblastic leukemia/lymphoma are most often

A

B-cell (>80%)

242
Q

40% of all childhood lymphomas

A

Burkitt lymphoma

243
Q

Histologic features of Burkitt Lymphoma:

A
  • Cytoplasmic vacuoles

- Starry sky of tingible body macrophages

244
Q

IHC of lymphoblastic lymphoma targets:

A

Immature lymphocyte, most often B-cell

245
Q

POSITIVE/NEGATIVE IHCs for
Lymphoblastic Lymphoma:

  • CD20
  • CD19
  • CD10
  • TdT
  • CD34
  • CD99
A

Positive

-CD10 (+/-)

246
Q

POSITIVE/NEGATIVE IHCs for Lymphoblastic Lymphoma:

  • surface Ig
  • myeloid markers
A

Negative

247
Q

IHC for Burkitt Lymphoma targets:

A

Mature germinal center B-cell

248
Q

POSITIVE/NEGATIVE IHCs for Burkitt Lymphoma:

  • CD20
  • CD19
  • CD10
  • BCL-6
  • Ki-67
  • surface Ig
A

Positive

-Ki-67 (>90%)

249
Q

POSITIVE/NEGATIVE IHCs for Burkitt Lymphoma:

  • TdT
  • BCL2
  • CD34
A

Negative

250
Q

ENDEMIC / SPORADIC
Burkitt Lymphoma:

  • Males
  • Africa and Papua New Guinea
A

Endemic

251
Q

ENDEMIC / SPORADIC
Burkitt Lymphoma:

-Jaw or facial bones

A

Endemic

252
Q

ENDEMIC / SPORADIC
Burkitt Lymphoma:

-EBV+ (98%)

A

Endemic

253
Q

ENDEMIC / SPORADIC
Burkitt Lymphoma:

  • Males
  • Caucasians
A

Sporadic

254
Q

ENDEMIC / SPORADIC
Burkitt Lymphoma:

-Abdominal mass

A

Sporadic

255
Q

ENDEMIC / SPORADIC
Burkitt Lymphoma:

-Less often EBV+ (20-30%)

A

Sporadic

256
Q

B- or T- LL:

Young child with anterior mediastinal mass

A

T-Lymphoblastic Lymphoma

257
Q

can present as “blueberry muffin” babies

A

Neuroblastoma

258
Q

Classify:

  • At least 50% schwannian stroma
  • Gross nodule that is microscopically neuroblastoma
A

Ganglioneuroblastoma, nodular

259
Q

Classify:

  • At least 50% schwannian stroma
  • No nodule identified
A

Assess location of Neuroblasts

260
Q

Classify:

  • At least 50% schwannian stroma
  • No nodule identified
  • Neuroblasts all in Schwannian stroma
A

Ganglioneuroma

261
Q

Classify:

  • At least 50% schwannian stroma
  • No nodule identified
  • Neuroblasts any in Neuropil
A

Ganglioneuroblastoma, intermixed

262
Q

Classify:

  • Less than 50% Schwannian stroma
  • No neuropil
A

Neuroblastoma, undifferentiated

263
Q

Classify:

  • Less than 50% Schwannian stroma
  • with neuropil
A

-Assess degree of differentiation

264
Q

Classify:

  • Less than 50% Schwannian stroma
  • with neuropil
  • at least 5% degree of differentiation
A

Neuroblastoma, differentiating

265
Q

Classify:

  • Less than 50% Schwannian stroma
  • with neuropil
  • less than 5% degree of differentiation
A

Neuroblastoma, poorly differentiated

266
Q

INRG Pretreatment risk group:

  • <18 months
  • NA MYCN
  • with Hyperdiploidy
A

Low risk

267
Q

INRG Pretreatment risk group:

  • <12 months
  • NA MYCN
  • with diploidy
A

Intermediate risk

268
Q

INRG Pretreatment risk group:

  • 12 to <18 months
  • NA MYCN
  • with diploidy
A

Intermediate risk

269
Q

Type of Synovial Sarcoma:

Histology - round/oval cells, short fascicles

A

Poorly differentiated

270
Q

Type of Synovial Sarcoma:

Histology - spindle cells with long fascicles

A

Monophasic

271
Q

Type of Synovial Sarcoma:

Histology - spindle cells with epithelial elements

A

Biphasic

272
Q 
IHC:
-TLE1+
-CD99+
CK7+
-decreased INI-1

Diagnosis?

A

Synovial Sarcoma

273
Q

associated with WAGR and Beckwith-Wiedemann syndrome

A

Wilms Tumor

274
Q

Renal tumor to be expected in <1 yo (3)

A
  • Congenital mesoblastic nephroma
  • Wilms Tumor
  • Rhabdoid tumor
275
Q

Renal tumor to be expected in 1-4 yo (3)

A
  • Wilms tumor
  • Rhabdoid tumor of kidney
  • Clear cell sarcoma
276
Q

Renal tumor to be expected in >4-5 yo

A

Wilms tumor

277
Q

Renal tumor to be expected in >5-10 yo (3)

A
  • Wilms tumor
  • Clear cell sarcoma
  • Renal cell carcinoma
278
Q

Renal tumor to be expected in >10-19 yo (2)

A
  • Renal cell carcinoma

- PNET/Ewing sarcoma

279
Q

INI-1 loss tumors (9):

A
  • Malignant rhabdoid tumor
  • ATRT (CNS)
  • Renal medullary carcinoma
  • Epithelioid sarcoma
  • Rhabdoid GI carcinomas
  • PD Sinonasal carcinomas
  • Epithelioid MPNST (subset)
  • Myoepithelial carcinoma (subset)
  • Extraskeletal myxoid chondrosarcoma (subset)
280
Q

Predisposition for germline heterozygous loss

A

Malignant Rhabdoid tumor

281
Q

Has prominent eosinophilic cytoplasmic inclusions

A

Malignant Rhabdoid tumor

PATHOLOGY (26 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions