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Pathological genetics > Soft tissue /connective tissue tumors lecture 20 printed > Flashcards

Soft tissue /connective tissue tumors lecture 20 printed Flashcards

1
Q

List 5 types of soft tissues and connective tissue

A 
Fibrous connective tissue 
Adipose tissue 
muscle (smooth and skeletal 
vessels 
nerves
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2
Q

What is the etiology of soft and connective tissue tumors?

A
  • Vause is unknown and sporadic
  • Some are associated with genetic syndromes eg neurofibromatosis type 1
  • Some show specific genetic derangements/mutations and chromosomal abnormalities eg synovial sarcoma
  • Some are associated with radiation therapy, chemicals, thermal burns and trauma, viruses
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3
Q

Discuss the presentation of soft tissue tumors

  • sex
  • age
  • sizes
  • Benign / malignant
  • common sites
A 
>Occurs more in Males 
>Incidence increase with age but 15% occur in children and 4th common malignancy in pediatrics
>Mass can grow to an enormous size causing disfigurement and may be painful 
>BT more common than MT
>Common sites 
*40% lower limb
*30% trunk 
*20%upper limb 
*20% head and neck
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4
Q

List the 4 ways soft tissue tumors are diagnosed

A

> Fine needle aspiration

  • Good for recurrence /metastases
  • sitological assessment
  • Not for primary diagnosis

Incisional biopsy
*Preferred when core biopsy is not representative

> Core biopsy

  • First line for most tumors
  • May not be fully represented in the case of sarcoma

> Excisional biopsy
*Only is small (<3-5 cm ) and superficial

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5
Q

T/F; Histological diagnosis provides valuable information for treatment and prognosis

A

t

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6
Q

What are the common histological features we look out for?

A
  1. Growth pattern and architecture (storiform, palisading, and herringbone )
  2. Mitotic activity
  3. Pleomorphism
  4. Necrosis
  5. Behaviour
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7
Q

What other diagnostic tools are used to diagnose tumors

A
  1. immunohistochemistry
  2. cytogenesis
  3. molecular analysis
  4. electron microscope
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8
Q

Prognosis depends on :

4 things

A
  1. Accurate histological classification of the tumor
  2. Histological grading
    a. Tumor differentiation
    b. Percentage of tumor necrosis
    c. Mitoses
  3. Stage
  4. location: superficial tumors have a better prognosis than deep tissue tumors because they are easy to discover and excisie
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9
Q

How can tumors be treated

A
  1. Surgery
    * enucleation for BT only
  2. Chemotherapy
  3. Radiation
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10
Q

Give an example of superficial fibromatosis

Fibromatosis of superficial tissue includes palmar, plantar, and penile (Peyronies disease )

A

Dupuytren’s contracture: overgrowth of fibrous tissue around tendon resulting in flexion deformity.

it is benign

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11
Q

Give 3 subtypes of deep-seated fibromatosis and common sites

A
  • Extra-abdominal: shoulders, chest wall, back, and thighs
  • Abdominal: Desmoid tumors (anterior abdominal wall )
  • Intra-abdominal (mesenteric, pelvic wall )

Tend to recur but do not metastasize

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12
Q

Discuss the frequency, sites, and appearance of fibrosarcoma

A
  • Rare
  • Retroperitoneum ,thigh ,knee and distal extremities
  • necrosis ,hemorrhage

Histologically: spindle cells growing in a herringbone pattern, mitoses, pleomorphic and necrosis

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13
Q

T/F: Fibrohistolytic tumors are deep-seated in tissue

A

False: Usually superficial and range from benign to locally aggressive malignant

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14
Q

Discuss the lipoma

A
  • benign
  • common in adults
  • can grow to a large size
  • soft mobile and painless except for for angiolipoma
  • cured by local excision
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15
Q

Discuss the liposarcoma

A
  • Malignant
  • arise in deep soft tissues of the proximal extremities and retroperitoneum
  • Arise de novo and not from malignant transformation of lipoma

> has 4 types

  1. well differentiated
  2. myxoid
  3. pleomorphic
  4. dedifferentiated
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16
Q

Discuss the rhabdomyosarcoma

A
  • MT of the skeletal muscle
  • Common soft tissue sarcoma in child(<20)
  • common sites(head ,neck and genitourinary tract )
  • The diagnostic cell is a rhabdomyoblast
17
Q

What are the 3 types of rhabdomyosarcoma

A
  1. Embryonal
  2. alveolar
  3. pleomorphic
18
Q

Discuss the embryonal rhabdomyosarcoma and list their subtype

A

The ERS is common in children <10 yrs and occurs in nasal cavity ,orbital ,prostate ,middle ear

> Subtype : Sarcoma botryoides

  • Resembles a branch of grapes
  • Grows in the wall of hallow mucous lined structures eg bladder ,vagina and nasopharynx
19
Q

Discuss the alveolar rhabdomyosarcoma

A
  • Commonly arise in the deep muscles of the extremities
  • Accounts for 10% RMS
  • network of fibrous septae divides cells into clusters similar to alveolae ; cells in the center are discohesive
20
Q

PLeomorphic rhabdomyosarcoma

A
  • Least common
  • occurs more in adults
  • arises in deep soft tissue
  • Characterised by large ,multinucleated ,bazarre eosinophilic cells
21
Q

Tumors of the smooth muscles

A

leiomyoma

leiomyosarcoma

22
Q

Leiomyoma :

common site and effect

A

> Arises commonly in the uterus, it can be multiplied and grow to enormous sizes
it is associated with dysfunctional uterine bleeding and infertility

> c: Erector pili muscle in skin, labia, and scrotum

e: Less frequent in deep soft tissue and wall of the gut

23
Q

Leiomyosarcoma

A
  • not common in soft tissue sarcoma
  • occur in adults
  • F>M
  • c: skin tumors ,deep tissue of the extremities ad retroperitoneum
  • painless and firm
  • with/ without abdominal symptoms
24
Q

Vascular tumors

A

haemangioma
kaposi sarcoma
angiosarcoma

25
Q

Discuss the haemangioma

A
  • BT
  • present from birth and exapand with growth of child
  • 7% occur in infancy and childhood
  • C: head and neck ,some internal and others liver

Main variants’ :
capillary hemangioma
Cavernous hemangioma

26
Q

t/f: Haemangioma are greatly capsulated

A

false ,hey are poorly capsulated

27
Q

Discuss the Kaposi sarcoma

rare type of tumor

A
  • intermediate malignancy
  • initially presents as a skin lesion
  • may disseminate to involve viscera: lungs and git

*Caused by HHV8

28
Q

List the 4 forms of Kaposi sarcoma

A
  • Classic ( associated with immunosuppression but not HIV)
  • African(endemic; associated with immunosuppression but not HIV)
  • transplant-associated
  • AIDS-associated

Histology
*haemosiderin deposition and plasma cells

29
Q

Angiosarcoma

A
  • C: May occur anywhere especially liver and breast
  • Highly malignant tumors ,locally aggressive and readily metastasize

*histologically (anastomosing channels in other words blood lakes and proliferation of endothelial cells

30
Q

Peripheral nerve sheath tumors

A

> Neurofibroma
Schwannoma
Granular cell tumor
Malignant peripheral nerve sheath tumor

31
Q

Neurofibroma

A

> It is associated with neurofibromatosis type 1
*it is neurofibroma , patches of skin pigmentation and lish nodules

Histologically :
*mast cells and shredded carrot collage and un-encapsulated proliferation of spindle cells

32
Q

Discuss the schwannoma

A
  • solitary tumors (arise to one side of nerve root )
  • Can rise anywhere

Histologically
*palisading

33
Q

t/f: Schwannoma are problematic when in relation to the sheath of the auditory nerve intracranially for they can compromise hearing

A

true

34
Q

Malignant peripheral nerve sheath tumors

A
  • affects adults and children
  • 50% associated with neurofibroma
  • Bulky deep-seated tumors arise from major nerves
35
Q

summary

A

Soft tissue tumours are diverse
Commonly present as masses of the extremities, retroperitoneum, trunk or head and neck region
Clinically, difficult to determine whether benign or malignant
Definitive diagnosis requires histology  core or incisional biopsy
Treatment depends on the nature of disease (whether benign or malignant), grade and stage
Prognosis depends on multiple factors, but is generally poor for malignant lesions

Pathological genetics (13 decks)

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