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DMD3 > Soft tissue (mesenchymal) tumours > Flashcards

Soft tissue (mesenchymal) tumours Flashcards

1
Q

What is a neurofibroma?

A

A nerve sheath tumour caused by growth of perineural fibroblasts, schwann cells.

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2
Q

How does a neurofibroma grow?

A

Slow-growing

Soft, painless lesion

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3
Q

What is neurofibromatosis I?

A

An autosomally dominant genetic condition caused by mutations in the NF1 gene.

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4
Q

What is NF1?

A

A tumour supressor gene that encodes for the protein neurofibromin which regulates the ras-mediated growth pathway

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5
Q

What are the potential outcomes of neurofibromatosis?

A

5% of cases have malignancies:

A malignant schwannoma can form

A malignant neurofibrosarcoma can also form.

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6
Q

What are the histopathological features of a neurofibroma?

A

Proliferation of all elements of peripheral nerves.

Cells have wavy nuclei.

Fibromyxoid background.

Mast cells

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7
Q

How is a neurofibroma treated?

A

Excision of the lesion

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8
Q

What is a neurilemmoma?

A

AKA schwannoma. A benign peripheral nerve sheath tumour composed of schwann cells.

Schwann cells normally produce the insulating myelin sheath covering peripheral nerves

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9
Q

How do neurilemmomas arise?

A

Can be sporadic or genetically acquired.

Loss of function of merlin, either by direct genetic change involving NF2 gene or secondarily to merlin inactivation

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10
Q

What are the histopathologic features of a neurilemmoma?

A

Well-circumscribed lesion in the Connective tissue.

The tumour is composed of different areas of different cellular densities. Shows Antoni A and B pattern

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11
Q

What are the features of the antoni B pattern?

A 
Loose, less cellular areas are
composed of a loose
oedematous and mucinous
stroma with fibrillar
collagen.
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12
Q

What are the features of the antoni A pattern?

A

MORE CELLULAR AREAS ARE COMPOSED OF A HAPHAZARD
ARRANGEMENT OF BLAND
CELLS WITH SPINDLED AND
OVAL NUCLEI

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13
Q

How is a neurilemmoma/schwannoma treated?

A

Excision of the lesion

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14
Q

What is a lipoma?

A

Benign tumour of adipose tissue more common in obese people

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15
Q

What are the clinical features of a lipoma?

A

Mobile, painless submucosal lesion. Yellow tinge seen if it is superficial enough.

More common in obese people but metabolism is completely independent of body fat.

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16
Q

What are the histopathological features of a lipoma?

A

Lesion composed of abundant mature adipocytes arranged in lobules separated by fibrous tissue septa.

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17
Q

How are lipomas treated?

A

Surgical excision

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18
Q

What are haemangiomas?

A

Tumours identified by rapid endothelial cell proliferation in early infancy, commonly followed by involution
over time.

A proliferating mass of
endothelial cells and
pericytes.

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19
Q

What causes haemangiomas?

A 
One hypothesis postulates
that placental cells, such
as the trophoblast, may be
the cell of origin for
haemangiomas.
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20
Q

What are the clinical features of haemangiomas?

A

Soft mass, smooth or lobulated, sessile or
pedunculated.

A few millimeters to several centimeters.

Pink to red / purple and tumour blanches on the
application of pressure,

Hemorrhage may occur either spontaneously or
after minor trauma.

Generally painless.

21
Q

How are haemangiomas diagnosed?

A

Doppler ultrasonography

MRI

MRI angiogram

CT angiogram

22
Q 
What does haemangioma look like on Coronal contrast-enhanced T1-
weighted image (MRI T1 weighted)?
A 
Coronal contrast-enhanced T1-
weighted image.
A well-rounded homogeneously
enhancing cervical mass is
associated with high-flow vessels
(signal voids) in and around the
mass.
23
Q

What do early haemangiomas look like on histology?

A

Numerous plump endothelial cells

Indistinct vascular spaces

24
Q

What do mature haemangiomas look like on histology?

A

Endothelial cells flatten,

Vascular spaces become more evident

25
Q

What do haemangiomas look like on histology during involution?

A

The vascular spaces become less prominent

Replaced by fibrous connective tissue.

26
Q

How are haemangiomas treated?

A

Monitoring and Education- most haemangiomas of infancy undergo involution - 90% at age 9 yrs and normal skin is restored in 80% of patients. up to 40% of patients will show permanent changes such as atrophy, scarring, wrinkling and telengiectasis

If intervention is required, oral corticosteroids are used, intralesional injection of fibrosing agents, INFα-2b, radiation, electrocoagulation,
cryosurgery, laser therapy,
embolisation and surgical excision

27
Q

What are the potential adverse outcomes of haemangiomas following involution?

A

Up to 40% of patients will show permanent changes such as atrophy, scarring, wrinkling and telengiectasis

28
Q

What is a fibrosarcoma?

A

Malignant tumour of

fibroblasts

29
Q

Where is a fibrosarcoma typically seen?

A

Male = female

Most common between 30 - 60 years of age

Uncommon in the head and neck

30
Q

What is the cause of a fibrosarcoma?

A

Not entirely understood. Several thoughts about how it arises:

Idiopathic

Genetically predisposing

Radiation exposure

Viral-induced

Chemical induced.

Genetic alterations?

31
Q

What are the clinical symptoms of a fibrosarcoma?

A

Pain

Swelling

Tooth losening

Paraesthesia

32
Q

How is a fibrosarcoma diagnosed?

A

Imaging:

Combined MRI and CT scans are recommended.

CT scan of the chest is important to assess metastatic lesions to the
lungs.

Positron-emission
tomography (PET) using
fluorodeoxyglucose (FDG) is a functional technique that evaluates glucose utilization by the tumour.

Immunohistochemistry: +ve for vimentin

Fluorescence in situ hybridization (FISH)

33
Q

What are the histopathologic features of a fibrosarcoma?

A

Proliferation of fibroblasts with variable amounts of collagen and reticulin fiber formation

34
Q

How are fibrosarcomas graded?

A

Low to high;

Low = spindle cells arranged in fascicles, mild nuclear pleomorphism

High = atypical mitoses, intense nuclear polymorphism.

35
Q

How is a fibrosarcoma treated?

A

Surgery is the treatment of choice

Radiotherapy indicated for: High grade sarcomas, positive surgical margins, lesions larger than 5 cm, and recurrent lesions

Chemotherapy if unresectable or a highly aggressive lesion

36
Q

What is a kaposi sarcoma?

A

Vascular lesion of endothelial origin that develops from cells that line the blood and lymph vessels.

37
Q

What causes kaposi sarcoma?

A

Human Herpes Virus8 which reprograms the host’s blood
endothelial cells to:

•Resemble lymphatic endothelium,
upregulating several lymphatic- associated genes

•Upregulates lymphatic vessel endothelial receptor 1, podoplanin, and
vascular endothelial growth
factor receptor 3.

Relies on some degree of immune dysfunction.

HHV8 remains latent within B lymphocytes (developed a variety of mechanisms to evade the host immune system)

38
Q

What are the types of kaposi sarcoma?

A

Classic

Endemic

Iatrogenic immunosuppression associated (recipients of organ transplants. Affects 0.8% of renal transplant patients)

AIDS - related

An interplay of HHV-8 with immunologic, genetic, and
environmental factors determine which type.

39
Q

Who commonly gets classic kaposi sarcoma?

A

Primarily a disease of late adult life,

70% to 90% of cases occur in men.

Mostly affects individuals of Italian, Jewish, or Slavic ancestry.

40
Q

How is kaposi’s sarcoma treated?

A

Depends on the clinical subtype and stage of the disease.

Treating the cause of the immune system dysfunction can slow or stop the progression of kaposi’s sarcoma.

AIDS-associated Kaposi sarcoma (Lesions may shrink with HAART)

People with a few local lesions can often be treated with local measures such as:
§ radiation therapy
§ Cryosurgery
§ injection of chemotherapeutic agents

Systemic chemotherapy:
§ vinblastine

41
Q

What is rhabdomyosarcoma?

A

Malignant neoplasm characterized by skeletal muscle differentiation. Much more common in young children.

42
Q

Who most commonly gets rhabdomyosarcoma?

A

60% of soft tissue tumours of childhood are rhabdomyosarcomas

43
Q

What causes rhabdomyosarcoma?

A

Genetic translocation producing new protein that interrupts skeletal muscle differentiation

44
Q

What are the clinical symptoms of a rhabdomyosarcoma?

A

Infiltrative mass that grows rapidly

may cause:

Pain

Paraesthesia

Facial swelling

Trismus

Nasal discharge

45
Q

What are the subtypes of rhabdomyosarcoma?

A

International Classification of Rhabdomyosarcoma:
Superior prognosis
§ Botryoid
§ Spindle cell

Intermediate prognosis
§ Embryonal

Poor prognosis
§ Alveolar
§ Undifferentiated sarcoma

46
Q

How can rhabdomyosarcoma be diagnosed?

A

Imaging: CT and MRI scans.

47
Q

What are the histopathologic features of rhabdomyosarcoma?

A

Rhabdomyoblasts

48
Q

How is rhabdomyosarcoma treated?

A

Local surgical excision then:

Multi-agent chemo (vincristine, actinomycin D, and cyclophosphamide)

Postop radiotherapy (Except for localised tumors that have been completely resected at initial surgery)

DMD3 (53 decks)

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