8 Lypmhomas, brain tumours, embryonal tumours and teratomas Flashcards
Define a lymphoma
Lymphomas are neoplastic proliferations of lymphoid cells of various types
Malignant process of mature cells - lymphoma
(T, B cell lymphomas)
Define leukaemia’s
Leukaemia’s are neoplastic proliferations of the cells of the haemopoietic bone marrow
(mainly blood, white cells and their precursors)
Tumour from blast phase (progenitors)
List the two main groups of lymphomas
- Hodgkin’s disease: and the rest (grouped under same name)
- Non-hodgkin’s lymphoma - most common and important of these are lymphocytic lymphomas
Give the clinical features and behaviour of lymphomas
- Most present clinically with lymphadenopathy - lymph node enlargement, localised or generalised
- Some may also infiltrate (Hepatomegaly), spleen (Splenomegaly) or Bone Marrow (marrow replacement, with haematological consequences)
- Bad prognosis types may diffusely infiltrate other organs
List the main types of Hodgkin’s disease
- Lymphocyte rich Hodgkin’s
- Mixed cellularity Hodgkin’s
- Nodular sclerosing Hodgkin’s
- Lypmhocyte-depleted Hodgkin’s
What gives rise to the different types of Hodgkin’s disease
The different types of Hodgkin’s disease are based on the differing proportions of:
- Reed-Sternberg cells
- Lymphocytes
What is the neoplastic cell found in Hodgkin’s lymphomas?
Reed-Sternberg cells
Describe Reed-Sternberg cells
Main neoplastic cell found in Hodgkin’s Disease
- They are often multi nucleated
- Prominent nuclei
- Large cell
Describe the different types of Hodgkin’s disease based on the
Lymphocyte rich Hodgkin’s
- Few R-S cells, lots of lymphocytes
Lymphocyte depleted Hodgkin’s
- Lots of R-S cells, few lymphocytes
Mixed-cellularity Hodgkin’s
- Roughly equal proportions of R-S cells and lymphocytes
Nodular sclerosing Hodgkin’s
- As mixed cellularity, but different architecture
What is the relationship between the number of Reed-Sternberg cells and the prognosis of the type of Hodgkin’s disease
The more the number of R-S cells, the worse the prognosis
Describe a myeloma
A myeloma is a tumour of mature plasma cells (later stage of differentiation of B cell lymphocytes
Describe what myelomas can present with
It presents with bone tumours, osteolytic, painful, but with interesting systemic effects
- Anaemia, renal/cardiac failure, infections, hypercalcaemia - lytic bone lesions)
Pronounced cytoplasm in plasma cells (function is to release antibodies) - so immune system is seen to be compromised (suppression) - they can accumulate other immunoglobulins and can form amyloids, which can deposit themselves on organs (renal/cardiac failure)
What are most primary tumours of the brain derived from?
The most primary tumours of the brain are derived from the support cells called:
- Glial cells
Name some different glial cells:
- astrocytes
- Oligodendrocytes
- ependymal cells
List some different tumours that can arise from glial cells
Gliomas:
- Astrocytomas (most common)
- Oligodendrocytoma
- Ependymoma
There can be combined tumours - oligodendroastrocytomas
Do astrocytomas show malignant traits (if so, which ones/which ones not?)
All astrocytes behave in a malignant manner, but DO NOT metastasise
How are astrocytomas graded?
On a 1-4 grading system
- 1 is good
- 4 is bad
4 is worst prognosis - glioblastoma
Give some features of grade 2 astrocytomas
- Well differentiated
- No metastasis
> grow locally into skull (not expandable) - so even benign tumour can show symptoms - There is a shift of median line, because of the growth of the tumour, and will cause compression in the ventricles, where there is CSF
So, increase in pressure in CSF = cause symptoms of cranial hypertension
- Symptoms depend on area of brain involved
Surgery must be done - to remove the tumour
Give some features of grade 4 astrocytomas
- Poorly differentiated
- This is a glioblastoma - astrocytoma grade 4
- Huge infiltration
- Complete compression of the ventricles
- So, this tumour is treated with surgery - problem is it is difficult to eradicate tumour
- On periphery of this tumour, there are cells which infiltrate normal parenchyma
- So, surgeon will not be able to completely excise the tumour, due to normal parenchyma, causing additional symptoms
- External radiotherapy followed to treat remaining tumour cells
Surgery, followed by radiotherapy and chemotherapy
Describe embryonal tumours
Embryonal tumours are derived from embryonic remnants of primitive ‘blast’ tissues
The tumour arises at the stage of the pluripotent embryonic stem cells:
- they will be very undifferentiated
List the main characteristics of embryonal tumours
- Mainly occurs in young children
- Highly malignant (proliferative)
- Spread early and widely by lymphatics and veins
- Sensitive to chemotherapy (due to high proliferation)
- Formerly rapidly fatal, chemotherapy has revolutionised prognosis
Give some examples of embryonal tumours
- Nephroblastoma (Wilm’s tumour) - in kidney, most common
- Neuroblastoma - mostly in adrenal gland, derived from primitive adrenal medullary precursors (neuroblasts); next most common
Much rarer are:
- Retinoblastoma - retina; often bilateral; genetic basis
- Medulloblastoma - cerebellum
- Hepatoblastoma - liver
Give an example os a neuroblastoma
- Arise from adrenal gland
- Very advanced local invasion with lymph node spread
- Also, extensive blood-borne spread (lung, liver, and bones)
- This is renal vein - completely infiltrated
Give the histology of a neuroblastoma
- Small, dark staining undifferentiated cells
- Radically, proliferating cells
- Mitosis can be seen
- Multi nucleated
Give possible management options of a patient with neuroblastoma
Treat this patient with chemotherapy (responds well - due to high proliferative nature
- sometimes surgery needed if possible, to eradicate disease
Describe teratomas
Teratomas are tumours derived from the primordial germ cells - retain the capacity to differentiate along all 3 primitive embryological lines
Hence, teratomas should contain representatives of ectoderm, mesoderm and endoderm
- Since they are of germ cell origin, they mainly occur in the ovary and testis
Describe a teratoma of the ovary (key facts)
- They occur in young women
- Occur as a benign tumour
- They are invariably cystic (benign cyst of ovary)
- Contain many tissues: Skin, hair, bronchial, and gut epithelium, thyroid, neuroglia, bone, cartilage
- GOOD PROGNOSIS
Describe a benign cystic teratoma of the ovary
- Thin-walled cyst filled with keratin and hairs etc.
- Epidermal tissue, fat
Describe the histology of benign cystic teratoma walls
- Note - epidermis, hair follicles, sebaceous glands (fat), nerve tissue with ganglion cells
Describe a teratoma of the testis
- Occur in young women
- Painless welling of testis (clinical presentation)
- Malignant tumour
- Malignancy varies according to type
- Spreads early via blood stream ( -> lung + liver etc.)
- Chemotherapy has revolutionised prognosis (responds between in immature form) - they are very undifferentiated, could differentiate in other parts of body - pluripotent stem cell features