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GSSE > Important cancers > Flashcards

Important cancers Flashcards

1
Q

Inheritance pattern of MEN

A

autosomal dominant

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2
Q

What glands are implicated in MEN and what adaptation do they undergo

A

Hyperplasia of several endocrine glands

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3
Q

List the 3 variants of MEN

A

Men I
Men II or IIa
Men IIb or III

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4
Q

What glands are implicated in MEN I

A

Parathyroid
Pancreatic
Pituitary

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5
Q

What glands are implicated in MEN II

A

Pheo/ andreal medullary hyperplasia
Parathyroid hyperplasia
Thyroid - Medullar cancer and C - cell hyperplasia

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6
Q

What are the metabolic problems associated with MEN (5)

A

Zollinger-Ellison syndrome, hyperinsulinism, pheochromocytoma, Cushing’s syndrome,
hyperparathyroidism

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7
Q

What glands are implicated in MEN IIb or III

A

Pheo
Medullay thyroid hyperplasia
Medullar Ca and C cell hyperplasia of the thyroid
Mucosal neruomas
Marafanoid features

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8
Q

What life threatening cancers are associated with MEN

A

Medullary thyroid
Pancreatic

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9
Q

2 life threatening sequelae of MEN

A
  • Hypertension
  • Relentless peptic ulcer syndrome
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10
Q

Testing for MEN (what to use and what not to use

A

To use
- Urinary catecholamine estimation
- Plasma calcitonin

Not
- Plasma calcium estimation
- Not serum ionized calcium/phosphate ratio

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11
Q

What cells is medullary carcinoma of the thyroid derived from, what do they secrete

A

Parafollicular cells or C cells
Secrete calcitonin
- may also secrete serotonin and prostaglandin
- sometimes carcinoid, rarely ACTH

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12
Q

Are MEN genetic

A

arise sporadically 80% of cases, the other 20% from genetic associations (MEN IIa & b)

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13
Q

What gene is implicated in medullary thyroid carcinoma (when due to MEN)

A

germ line mutations in the RET proto-oncogenes

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14
Q

What is the stroma of medullar carcinoma of the thyroid rich in

A

Amyloid

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15
Q

Medullary thyroid carcinoma slow or fast growing

A

slow

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16
Q

Biochemically what will people with medullary thyroid carcinoma have or not have

A

Will have high circulating calcitonin
WONT have
- Hypocalcemia
- Hypercalcemia
- Hypothyroidism

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17
Q

What is the most common thyroid cancer in adults and children

A

Papillary carcinoma of the thyroid

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18
Q

What is papillary thyroid carcinoma strongly assocaited with

A

Radiation - especially in childhood

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19
Q

How do people with papillary thyroid cancer often present

A

With nodal metases (50% at time of diagnosis)

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20
Q

What is the prognosis of papillary thyroid cancer

A

90% in 20 yrs > the best survival with thyroid neoplasia

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21
Q

What dystrophic calcification is papillary thyroid cancer a/w

A

pasmmoma bodies

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22
Q

What is the growth pattern of papillary thyroid cancer

A

papillary (often plus follicular) growth pattern > but co-existing with follicular DOES NOT worsen prognosis

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23
Q

What hormone is papillary thyroid cancer dependent on and what implications does this have

A

TSH dependent > implications for therapy, relapse during pregnancy, etc…

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24
Q

Where do carcinoid tumours arise from (structurally and what type of cell)

A

GIT (including pancreas),
Also bronchi
Thymus
Occasionally ovary

Neuroendocrine cells

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25
Q

When are carcinoid tumours often found

A

Incidentally at surgery or autopsy

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26
Q

What type of carcinoid tumours rarely metastaise

A

appendiceal, rectal carcinoids infrequently metastasize

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27
Q

What carcinoid tumours are frequently aggressive

A

ileal, gastric and colonic carcinoids are frequently aggressive

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28
Q

What is the main hormone released in carcinoid syndrome and what cancers is it associated with

A

Serotonin
- Hepatic mets

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29
Q

What are the 4 vasoactive substances that can be released in carcinoid syndrome + other rare hormones

A
  1. serotonin
  2. bradykinin & kallikreins
  3. prostaglandin
  4. histamine

ACTH or gastrin

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30
Q

Symptoms of carcinoid syndrome

A
  1. cramping abdominal pains
  2. facial flushes
  3. tachycardia
  4. pulmonary stenosis
  5. tricuspid regurgitation
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31
Q

When is gastric cancer defined as early

A

When it is confined to the mucosa and submucosa

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32
Q

Post resection survival of gastric cancer

A

90-95% if confined to mucosa/sub mucosa

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33
Q

Risk factors for gastric cancer

A

diet
smoking
H. pylori infection
autoimmune gastritis
partial gastrectomy

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34
Q

Final common pathway for gastric cancer

A

dysplasia of the gastric mucosa

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35
Q

Locations of gastric cancer and their frequencies

A

50-60% at pylorus & antrum 25% cardia
15-25% body and fundus

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36
Q

Name the cancer that is gastric in origin and mets to the ovaries and the cell associated

A

Krukenberg (signet ring cell)

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37
Q

What type of cancer causes rigid thickened stomach wall

A

Linitis plastica

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38
Q

What type of polyps are benign

A

hyperplastic
juvenile
inflammatory
Peutz-Jeghers polyps

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39
Q

What types of polyps are neoplastic

A

adenomas
(tubular, villous and mixed tubulovillous)

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40
Q

Chance of an adenoma <1cm progressing to invasive cancer

A

1%

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41
Q

Chance of an adenoma 1-2cm progressing to invasive cancer

A

10%

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42
Q

Chance of an adenoma >2cm progressing to invasive cancer

A

45%

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43
Q

What 3 factors are implicated in the association between polyp and cancer

A
  • Polyp size
  • Relative proportion of villous component
  • Degree of cell atypia (dysplasia) in the neoplastic cells
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44
Q

Peak incidence of colorecal carcinoma

A

60-70

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45
Q

Proportion of colorectal cancers located in rectosigmoid

A

<25%

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46
Q

Do most colorectal carcinomas occur in people with FAP or UC

A

No most occur in their absence

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47
Q

Is carcinoma insitu considered a malignant lesion and why/why not

A
  • No
  • Has not yet acquired attributes that will cause mets
  • because lymphatic channels are largely absent from colonic mucosa, intra-mucosal carcinoma is regarded as having little or no metastatic potential
  • invasion into submucosa indicates adenocarinoma which has now acquired metastatic potential > real in all case
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48
Q

What histological evidence suggests benign colonic epithelial neoplasia

A
  1. adenoma with carcinoma in situ
  2. adenoma showing severe epithelial dysplasia with focal intramucosal carcinoma
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49
Q

Why is Gardner’s syndrome is a clinically more sinister variant of FPC

A

Aggressive neoplasms, other than colonic carcinoma, may determine mortality in Gardner’s syndrome

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50
Q

What is Gardners syndrome (what other things does it exhibit)

A
  • An autosomal dominant familial polyposis variant
  • It is an inherited disease that is characterised by gastrointestinal polyps, multiple osteomas (benign bone tumours), and various skin and soft tissue tumours.
  • epidermal cysts, fibromatosis, abnormal dentition, higher frequency of duodenal and thyroid cancer
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51
Q

What is Gardners syndrome characterised by

A

lots of adenomatous polyps (minimu 100) in colon & elsewhere in the gut ! risk of becoming adenocarinoma 100%

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52
Q

How do you mitigate colon cancer risk in Gardners syndrome

A

Prophylactic colectomy

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53
Q

Average age of onset of Gardners syndrome and when does progression to cancer occur without surgery

A
  • Teens to 20’s
  • in 10-15 years
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54
Q

Tumour marker for Gardeners syndrome

A

CEA

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55
Q

What side of the colon does Gardners cause cancer

A

Right

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56
Q

What is Turcots associated with

A

FPC and CNS tumours

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57
Q

What cancers/ gastric disorders are implicated by dietary factors

A

FPC
UC

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58
Q

What Genes are associated with Gardeners and which isnt

A

HNPCC

  • NOT APC, DNA, K-ras, not DCC, or p53
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59
Q

Most common form of renal cancer

A

RCC

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60
Q

Most important risk factor for RCC

A

Smoking

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61
Q

When does RCC present

A

50-60

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62
Q

Is RCC more common in men or women

A

Men

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63
Q

Is RCC genetic

A

Most sporadic
4% can be rare autosomal dominant forms

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64
Q

Does RCC present early or late

A

Normally presents late with mets, sometimes they are solitary and stay so

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65
Q

Clinical manifestations/complications of RCC

A
  1. amyloidosis
  2. polycythemia
  3. hypercalcemia
  4. fever & cachexia
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66
Q

Paraneoplastic syndrome associated with RCC (6)

A
  • polycythemia (EPO pure RBC blood volume increase)
  • hypercalcemia
  • hypertension
  • masculinisation or feminization
  • Cushing’ s syndrome
  • Amyloidosis - AA type
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67
Q

Common site of spread for RCC

A

renal vein extension very common > haematogenous metastases are common

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68
Q

Describe the gross appearance of RCC, and what type of cell does it have lots of

A
  • basic yellow-grey color, but variegated with haemorrhage, necrosis, cystic change, etc.
  • High lipid content
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69
Q

Describe the gross appearance of Wilms tumour

A

Wilm’s tuour is usually of very uniform cut surface in contrast to the
variegated colours and consistency of RCC

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70
Q

Peak age of cervical cancer

A

40-45

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71
Q

Risk factors for cervical cancer

A

early of first intercourse
multiple sex partners
high risk male sex partners
married women

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72
Q

Main HPV types associated with cervical cancer

A

16, 18, 31, 33

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73
Q

Peak incidence of CIN III

A

~ 30 years

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74
Q

% of cervical cancer associated with HPV

A

85%

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75
Q

2 Main groups of testicular cancer

A
  • germ cell tumours (95% of cases)
  • nongerminal tumours (stromal or sex cord tumours)
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76
Q

Give 2 examples of germ cell testicular tumours

A

seminoma & teratoma are both germ cell tumours

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77
Q

Which is the most commone testicular germ cell cancer

A

Seminoma

78
Q

Peak incidence of testicular seminoma, and which presents first, seminoma or teratoma

A

30 (older onset than teratoma)

79
Q

What is the least aggressive of testicular germ cell cancer

A

seminoma

80
Q

Compare seminoma vs NSGT with respect to radiosensitivity

A

Seminoma - Highly radiosenitive
NSGT - Radioresistance

81
Q

Is hcg prognostic in testicular seminoma

A
  • No
  • Significance remains unknown
82
Q

What are the most clinically important testicular tumours

A

seminoma and non-seminomatous germ cell tumours (NSGT)

83
Q

Compare the growth and metastasis pattern of NSGT and seminomas

A
  • seminomas remain confined to the testis longer and typically metastasise to LNs with blood spread occurring (when it does) as
    a latera phenomenon
  • NSGCT metastasize earlier and more frequently by blood stream
84
Q

NSGT management post orchidectomy (if confined to organ)

A

Intensive surveillance without additional therapy

85
Q

NSGT management for lesions out of the testes

A

Surgical resection

86
Q

What are testicular teratomas made of and what behaviour do they exhibit

A
  • Often occur as combined growth with seminoma or embryonal carcinoma
  • complex mixtures usually have the behaviour of the worse component
  • highly malignant
87
Q

Where can extra-testicular teratomas occur

A

midline region from head to sacrococcygeal area

88
Q

Karyotype associated with ovarian teratomas

A

46xx

89
Q

Risk factors for prostate cancer

A

increasing age, race, hormonal influences, genetic factors, ?diet

90
Q

How many patients gave advance cancer on diagnosis with prostate cancer

A

> 75%

91
Q

Describe the relationship between PSA and prostate cancer

A
  • Correlates well with total tumour volume
  • also raised in hyperplasia and in early & localized cancer, so can’t be used alone for reliable early detection of cancer
92
Q

What else causes a high PSA

A

levels may be elevated in BPH, prostatitis or prostatic infarct
so ~70-80% of men with raised PSA usually have one of these conditions rather than cancer

93
Q

What is PSA

A

is a glycoprotein secreted by prostate cells into the ejaculate

94
Q

How is prostate cancer best diagnosed

A
  • DRE
  • PSA serum level
  • Transrectal U/S of prostate and biopsy
95
Q

What biochemical marker is associated with metastatic prostate cancer

A

prostatic acid phosphatase

96
Q

Where does prostate cancer start and where does hyperplasia occur

A

Prostate cancer starts in the peripheral zone (hence why cant biopsy)
BPH occurs in peri-urethral zone

97
Q

Where does prostate cancer spread

A
  • when hematogenous spread occurs > mainly to axial skeleton and produces predominantly ‘osteoblastic’ behaviour
  • osteoblastic metastases virtually diagnostic of prostatic cancer in males (mets to bone)
98
Q

Describe the relationship between cell differentiation and prostate cancer prognosis

A

there is good correlation between tumour differentiation and prognosis

99
Q

Is hypercalcemia a feature of prostate cancer

A

No

100
Q

What structure is commonly involved in local extension of prostate cancer

A

Seminal vesicles

101
Q

What is prostate cancer commonly associated with (biochemically and common site of met)

A
  • Raised serum acid phosphatase > indicate extra-prostatic invasiona/metastasis
  • androgen dependency
  • osteoblastic (osteosclerotic) bony metastases
102
Q

Common occurences in advanced prostate cancer (3)

A
  • spinal cord compression
  • haematuria
  • renal failure from bilateral ureteric obstruction
103
Q

Follow up of localized prostate cancer following definitive therapy

A
  • DRE
  • PSA
104
Q

Treatment choices for prostate cancer (5)

A
  1. no initial treatment
  2. external beam radiotherapy alone
  3. brachytherapy alone
  4. external beam radiotherapy + brachytherapy
  5. surgery (radical prostatectomy)
105
Q

What factors may influence treatment choice for prostate cancer (4)

A
  1. age of patient >70 yrs with co-morbidity is likely to die from co-morbid diseases, rather than cancer
  2. co-morbid illnesses
  3. patient preference > as treatment affects continence, rectal irritation, impotence, etc…
  4. grade of cancer
    - well differentiated > excellent prognosis
    - mod-severe differentiated > definite benefits with treatment
106
Q

Is oral anti-andogren therapy alone sufficient for treatment of prostate cancer/ what is its role

A

No
Only tend to eradicate additional 5-10% of male androgens (which are adrenal derived as opposed to testes)

107
Q

What is the mainstay of prostate cancer treatment

A

testosterone deprivation therapy
80% are responsive to this
- Surgical (bilateral orchidectomy)
- Medical (LHRH agonist)
Dont need both

108
Q

Side effects of testosterone deprivation therapy (5)

A

hot flushes
impotence
weight gain
gynaecomastia
mood disturbances

109
Q

What is a neuroblastoma/ where does it occur

A

Childhood cancer (not a CNS tumour)
- occurs in the adrenal, but arises in primitive cells (neuroblasts) of neural tube origin
- may mature to ganglioneuroma or regress

110
Q

Growth pattern of neuroblastomas

A

Highly aggressive > have often metastasized at the time of diagnosis

111
Q

What do neuroblastomas secrete

A

90% of them secrete catecholamines, but HTN is rate

112
Q

Hisotological feature of medulloblastomas and gross appearance

A

Rosette formation
often well circumscribed, gray, friable, usually extrememly cellular

113
Q

Where does a medulloblastoma form (cell type and location)

A
  • Primitive neurons (ot adrenal medulla)
  • Midline in children, more lateral in adults
  • Often found near the 4th ventricle
114
Q

Growth pattern of medulloblastomas and where do they spread to

A

Grow rapidly, highly malignant, poor prognosis if untreated
Near 4th ventricle so spread to CSF and associated with hydrocephalus

115
Q

Proportion of childhood brain tumours that occur exclusively in the cerebellum

A

20%

116
Q

Treatment and prognosis for medulloblastoma

A

exclusively radiosensitive
total excision + radiation 75% survival, 5 yrs

117
Q

Inheritance genetics of familial retinoblastoma

A

######

118
Q

When do wilms tumours present

A

2-5

119
Q

Are wilms tumours malignant or benign and what is their prognosis

A

Malignant but good survival >90%

120
Q

Are Wilms tumours genetic or sporadic

A

Some sporadic but most are genetic and associated with abnormalities in chromosome 11p.

121
Q

3 Malformation syndromes associated with WIlms

A
  • WAGR (Wilms tumour, aniridia, genital anomalies, mental retardation)
    > patient with this syndrome have 33% chance developing Wilm’s tumour
    > Due to deletions on chromosome 11p band 13 of the Wilms tumour 1 (WT-1) gene
  • Denys-Drash syndrome
  • Beckwith-Wiedemann syndrome
122
Q

What is hepatoblastoma

A
  • a rare form of primary liver cancer
  • a tumour of young childhood, usually fatal within a few years if not resected
123
Q

What are the two anatomic variants of hepatoblastoma

A
  • epithelial (fetal, well differentiated, embryonal, macrotrabecular) > form acini tubules etc vaguely recapitulating liver development
  • mixed epithelial & mesenchymal type, which contains foci of mesenchymal differentiation > may consist of primitive mesenchyme, osteoid, cartilage, or striated muscle
124
Q

Prognosis of non gestational choriocarcinoma

A

Terrible prognosis

125
Q

How common is Hydatidform mole

A

1 in 2000 pregnancies

126
Q

How do you monitor therapy and progress for gestational trophoblastic neoplasia

A

Plasma HCG

127
Q

How do you treat gestational trophoblastic neoplasia

A

Highly responsive to chemo

128
Q

Growth pattern of choriocarcinoma of gestational origin

A

Mets early and widely

129
Q

What happens if you dont treat choriocarcinoma of gestational origin

A

if untreated, parallels the behaviour of primary ovarian choriocarcinoma

130
Q

What are the clinical manifestations of acute leukaemia (6)

A
  • Anaemia with pallor & fatigue
  • Infection with fever
  • Thrombocytopenic bleeding > petechii/ecchymoses, epitaxis, gingival
  • ALL&raquo_space; AML for lymphadenopathy, hepatosplenomegaly > leukaemic infiltration
  • Bone infiltration (medullary, subperiosteal) > with resorption & pain + hypercalcemia common
  • CNS involvement common + mediastinum (ALL); chloromas (AML)
131
Q

Side effect of successful treatment of acute leukemia

A

successful therapy may precipitate acute gout > because successful therapy > rapid tumour lysis > hyperuricemia > induce symptomatic gout

132
Q

What chromosomal abnormalities are associated with ALL and which have a better prognosis

A
  • hyperdiploidy common - better prognosis
  • All translocations are a/w a poor prognosis
133
Q

Cell types associated with ALL and which have a better prognosis

A
  • Most are B cell lineage
  • T cell type more commonly has mediastinal mass > poor prognosis
134
Q

Growth profile of ALL, prognosis (comparing kids and adults)

A
  • highly aggressive, but in children responds to therapy
  • 80% remission, 60% 5yr ‘cure’ (in kids)
  • adult response generally poor
135
Q

What is the most common acute leukemia

A

ALL (85%)

136
Q

Who gets AML

A

young adults

137
Q

Important marker for AML

A

terminal deoxynucleotidyl transferase (TdT) – DNA polymerase is an important marker

138
Q

Prognosis of AML

A
  • remission is common
  • relapse is the usual long-term outcome <20% ‘cure’
139
Q

Chronic myeloid leukemia

A

Myeloproliferative disorder

140
Q

Who gets CLL

A

Elderly - indolent course

141
Q

Genetic associations of CLL

A

.Deletions as opposed to translocations like most lymphoid malignancies.

142
Q

What cell type predominates in PRV

A
  • erythroid precursors dominate with absolute increase in RBC mass + WBC + platelets
143
Q

Clinical feature of PRV

A

increased blood viscosity with vascular stasis & thromboses

144
Q

What occurs with prolonged survival in those with PRV

A
  • may develop ‘spent phase’ > develop a myeloid metaplasia/myelofibrosis picture
  • treated patients (alkylating agents) > may develop terminal AML
145
Q

What is myelofibrosis

A

Clonal proliferation of stem cells (mainly megakaryocytes) that secrete cytokines such as platelet derived growth factor. This results in a reactive fibrosis (not clonal) of the bone marrow. This leads to impaired haematopoesis, cytopenis and extensive extra medullary haematopoesis.

146
Q

Cells seen in peripheral blood of patient with myelofibrosis

A
  1. normoblasts
  2. myeloblasts
  3. myelocytes
  4. NOT megaloblasts

(see tear drop red cells
poikilocytosis and a leukoerythroblastic cell i.e immature cells, like nucleated red blood cells)

147
Q

What is multiple myeloma

A

− Cancer of plasma cells, should only make up 1% of bone marrow.
− Plasma cells produce different types of antibodies (immunoglobulins) to fight a variety of infections.
− Malignant plasma cells only release one type of immunoglobulin-paraprotein.
(Most common is igG, followed by igA
- Immunoglobulins can be complete or INCOMPLETE (i.e light chains)

148
Q

Clinical features of multiple myeloma (including main causes of death)

A

Clinical features stem from
1. effects of plasma cell growth in tissues.
2. Production of excessive igG which have abnormal properties.
3. Suppression of normal humoral immunity

  • Bone resorption: Fractures, pain hypercalcemia.
  • Hyperviscosity, esp. IgA > polymers (from excess Ig production)
  • Amyloidosis (Associated with delta light chains in particular)

Main causes of death
- Reduced igG results in increased susceptibility to bacterial/ pyogenic infections. Essentially - T cell functions, well preserved, therefore, viral infection not a problem.
- Renal failure: Multifactorial, but most importantly Bence jones proteins (light chains that are toxic to tubules) and amyloidosis

149
Q

What contributes to renal failure in multiple myeloma (5)

A
  1. amyloidosis (often complicated MM)
  2. renal tubular protein casts
  3. protein deposition - Bence Jones protein (blocks renal tubules)
  4. pyelonephritis (increased susceptibility to pyogenic organisms)
  5. hypercalcemia (due to skeletal demineralization)
150
Q

What is Waldenstorms macroglobulinaemia

A

IgM production by a lymphoma-like malignancy + hyperviscosity & cryoglobulinaemia

151
Q

Pathology of Hodgkins lymphoma (cell type histologically etc)

A

T cell
- Reed-sternberg cells > malignant large mononuclear cells
- plus large numbers of reacting lymphocytes, plasma cells, and others
-. giving characteristics pleomorphic microscopic appearance

152
Q

5 subtypes of Hodgkins lymphoma

A
  1. Nodular sclerosis
  2. Mixed cellularity
  3. Lymphocyte rich
  4. Lymphocyte depletion
  5. Nodular lymphocyte predominance
153
Q

Prognosis and side effects associated with treatment of Hodgkins lymphoma

A
  • excellent outlook; tumour burden correlates inversely with prognosis
  • But therapy is followed by risk of ‘second cancer’ – leukaemia, lung cancer, & others
154
Q

Definition of MGUS

A

Asymptomatic patients with serum M protein <3 g/dL
i.e no CRAB
1% progress to a plasma neoplasm (usually myeloma)
Exhibit the same mutations as multiple myeloma
Progression to myeloma is unpredictable

155
Q

What is smouldering myeloma

A

Middle ground between MGUS and multiple myeloma
- Serum M protein >3 but patients are asymptomatic
75% of people progress to multiple myeloma

156
Q

What 2 haematological conditions are closely associated with massive splenomegaly

A

CML
Myelofibrosis

157
Q

What tyrosine kinases are implicated in myeloproliferative disorders

A

JAK2 (PV, ET, MF)
BCR-ABL (CML)

158
Q

What is ET

A
  • platelets due to megakaryocte proliferation
  • Clinically: recurrent haemorrhage and
    thrombosis. splenomegaly
  • Laboratory: numerous large platelets with
    hypercellular marrow.
159
Q

Treatment for PRV and ET

A

Venesection, aspirin +/- cytoreduction
(usually hydroxycarbamide AKA
hydroxyurea)

160
Q

Compare and contrast Hodgkins lymphoma with NHL with respect to
- Location
- Spread
- Node involvement
- Extra-nodal presentation

A

Hodgkins
- Localized to a single axial group of nodes (cervical, mediastinal, para-aortic)
- Spread by contiguity
- Mesenteric nodes and Waldeyer ring rarely involved
- Extranodal presentation rare

NHL
- More frequent involvement of multiple peripheral nodes
- Non contiguous spread
- Extra-nodal presentation common

161
Q

Cells in AML (microscopic)

A

Auer rods

162
Q

What do Reed-sterburg cells do

A

Release factors that induce accumulation of reactive lymphocytes, macrophages and granulocytes (make up 90% if tumor cellularity)

163
Q

How is Hodgkins lymphoma staged

A
  1. Single lymph node region or single extra-lymphatic origin or site
  2. 2 or more regions on same side of diaphragm or localized involvement of an extra-lymphatic organ
  3. Both sides of diaphragm with or without involvement fo extra-lymphatic organ or site
  4. Diffuse involvement of one or mote extra-lymphatic organs with or without lymphatic involvement
164
Q

What subtypes of HL tend to have what stage

A

Nodular sclerosis - I or II, tend to be free of systemic manifestations
Mixed cellularity or lymphocyte depletion - III and IV, more likely to have systemic symptoms

165
Q

How does HL tend to spread

A

Nodal disease
Splenic disease
Hepatic
Marrow
Other tissues

166
Q

What is MDS

A

Clonal stem cell disorders characterised by maturation defects that result in ineffective haematopoiesis and high risk transformation to AML.

I.e there is a clonal progeny that is able to differentiate but it does it ineffectively

167
Q

What are Myeloproliferative disorders

A

Mutated tyrosine kinase (or other signalling pathways) that leads to growth factor independent proliferation and survival of marrow progenitors. Differentiation is not impaired so tend to get mature blood elements.
Results in
- Increased proliferative drive of marrow
- Homing of neoplastic stem cells to secondary haemoatopoteic organs
- Variable transformation to spent phase (marrow fibrosis and cytopenias)
- Variable transformation to acute leukemia.

168
Q

What gene/ chromosome is implicated in CML

A
  • BCR gene on chromosome 22 an - - ABL gene on chromosome 9
  • Most cases of CML (more than 90%) are caused by the Philadelphia chromosome. Translocation between 22 and 9 (molecular weight of 210)
  • majority at least, of the remainder, show the same EFFECTIVE genetic transfer by way of fusion of abl oncogen with bcr
    gene on chromosome 22
169
Q

Marrow of CML

A

very hypercellular with every type of myeloid cel increased with a relative emphasis on the granulocyte lineage rather than the red cell lineage. Platelets and megakaryocytes are also usually
increased.

Sea blue histocytes are characteristic (macrophages)

170
Q

Blood film of CML

A

greatly increased WBC, often in the 100s, and predominantly
neutrophils and myelocytes.

171
Q

Cell appearance MDS

A

Dysplastic differentiation!
- Ringed sideroblasts
- Eythroblasts with iron laden mitochondria
- Megaloblasts
- Dhole bodies
- Pseudo-pelger-huet

172
Q

What is CML

A

The myeloproliferative disorder where there are too many granulocytes and their precursors.
- Unlike acute myeloid leukaemia, we see predominantly mature granulocytes and not an increased number of immature myeloblasts.

173
Q

Is CML pluripotent and if so how do we know

A

Yes, because you can get lymphoid blast crises or myeloid blast crises.

174
Q

Marrow of PCV

A

Hypercellular
increase in red cell progentiors is subtle and accompanied by increase in granulocytic and megakaryocytes as well

175
Q

Marrow of ET

A

Cellularity only mildly increased but you see megakaryocytes

176
Q

What is pathopneumonic for CLL and what other cells are present

A

Proliferation centres
Smudge cells
lymphocytosis, sometimes as high as several hundred. The malignant lymphocytes are small and rather nondescript, resembling ordinary lymphocytes. The nuclear chromatin pattern is “condensed” suggesting the cell is not dividing very rapidly.

177
Q

Marrow of CLL

A

Lymphocytes are increased in the marrow, usually massively. In this example we see a hypercellular, diffuse infiltrate. There may be other cytopenias as a result, e.g. anaemia, thrombocytopenia and absolute neutropenia.

178
Q

What is the only difference between CLL and SLL

A

Degree of peripheral blood lymphocytosis
i.e major overlap of clinical & pathological features with small lymphocytic lymphoma

179
Q

Immunophenotype of CLL/ SLL

A
  • pan B cell markers (CD19, CD20),
  • CD5
  • surface Ig (usualy igM or igM and igG)
  • no TdT or CD10 - i.e. ‘early’ B cell markers are lost
  • express kappa or delta, light chains, but NOT both
180
Q

is CLL T or B cell

A

95% are B cell neoplasms > T cell CLL is rare

181
Q

Clinical presentation of CLL

A

Normally asymptomatic
When symptoms appears - non specific, fatigue, weight loss

182
Q

Complications of CLL

A
  • Disrupts normal immune function through uncertain mechanisms.
  • Hypogammaglobunlinemia is common and contributes to increased susceptibility to infection
  • Haemolytic anemia
  • Thrombocytopenia
  • Transformation into more aggressive cancer - DLBCL (Ritcher syndrome)
  • Transformation to blast cell crisis is rare
183
Q

Poor prognostic features associated with CLL

A

11q and 17p deletions
ZAP-70 protein expression

184
Q

How do you differentiate CML from a leukemoid reaction

A

Total lack of leukocyte alkaline phosphatase

185
Q

Exposure to what 2 substances increases your risk of bladder cancer and 2 that dont

A
  1. cigarette smoke
  2. 2-naphthylamine
  3. NOT ! asbestos or lead
186
Q

5 core biopsy results that need excisional biopsy to exclude or diagnose associated cancer

A
  1. a papillary lesion
  2. atypical ductal hyperplasia
  3. intraduct papilloma
  4. radial scar
  5. ductal carcinoma in situ
187
Q

2 things that increase your risk of breast cancer and 2 that dont

A
  1. positive family history for breast cancer
  2. obesity
    NOT ! early first pregnancy, or late menarche
188
Q

4 facts about gallbladder cancers

A
  1. are adenocarcinomas
  2. clinical presentation is typically insidious and indistinguishable from the symptoms and signs of benign gallbladder disease !
    palpable enlargement of the GB is distinctly unusual
  3. have invaded the liver at the time of operation
  4. are NOT always a/w presence of gallstones
189
Q

Lymph node biopsy from patient with NHL will show/ not show what 4 things

A
  1. Will NOT show Reed Sternberg cells
  2. is unlikely to be composed of malignant T cells than B cells
  3. will contain reactive lymphocytes of the same lineage (i.e. T or B) as the neoplastic cells
  4. will contain a monoclonal population of tumour cells
190
Q
A

GSSE (16 decks)

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