Soft tissue /connective tissue tumors lecture 20 printed

Question 1

List 5 types of soft tissues and connective tissue

Answer 1

Fibrous connective tissue 
Adipose tissue 
muscle (smooth and skeletal 
vessels 
nerves

Question 2

What is the etiology of soft and connective tissue tumors?

Answer 2

• 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

Question 3

Discuss the presentation of soft tissue tumors

• sex
• age
• sizes
• Benign / malignant
• common sites

Answer 3

>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

Question 4

List the 4 ways soft tissue tumors are diagnosed

Answer 4

> 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

Question 5

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

Answer 5

t

Question 6

What are the common histological features we look out for?

Answer 6

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

Question 7

What other diagnostic tools are used to diagnose tumors

Answer 7

1. immunohistochemistry
2. cytogenesis
3. molecular analysis
4. electron microscope

Question 8

Prognosis depends on :

4 things

Answer 8

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

Question 9

How can tumors be treated

Answer 9

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

Question 10

Give an example of superficial fibromatosis

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

Answer 10

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

it is benign

Question 11

Give 3 subtypes of deep-seated fibromatosis and common sites

Answer 11

• 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

Question 12

Discuss the frequency, sites, and appearance of fibrosarcoma

Answer 12

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

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

Question 13

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

Answer 13

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

Question 14

Discuss the lipoma

Answer 14

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

Question 15

Discuss the liposarcoma

Answer 15

• 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

Question 16

Discuss the rhabdomyosarcoma

Answer 16

• 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

Question 17

What are the 3 types of rhabdomyosarcoma

Answer 17

1. Embryonal
2. alveolar
3. pleomorphic

Question 18

Discuss the embryonal rhabdomyosarcoma and list their subtype

Answer 18

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

Question 19

Discuss the alveolar rhabdomyosarcoma

Answer 19

• 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

Question 20

PLeomorphic rhabdomyosarcoma

Answer 20

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

Question 21

Tumors of the smooth muscles

Answer 21

leiomyoma

leiomyosarcoma

Question 22

Leiomyoma :

common site and effect

Answer 22

> 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

Question 23

Leiomyosarcoma

Answer 23

• 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

Question 24

Vascular tumors

Answer 24

haemangioma
kaposi sarcoma
angiosarcoma

Question 25

Discuss the haemangioma

Answer 25

* 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

Question 26

t/f: Haemangioma are greatly capsulated

Answer 26

false ,hey are poorly capsulated

Question 27

Discuss the Kaposi sarcoma rare type of tumor

Answer 27

* intermediate malignancy * initially presents as a skin lesion * may disseminate to involve viscera: lungs and git *Caused by HHV8

Question 28

List the 4 forms of Kaposi sarcoma

Answer 28

* 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

Question 29

Angiosarcoma

Answer 29

* 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

Question 30

Peripheral nerve sheath tumors

Answer 30

>Neurofibroma >Schwannoma >Granular cell tumor >Malignant peripheral nerve sheath tumor

Question 31

Neurofibroma

Answer 31

>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

Question 32

Discuss the schwannoma

Answer 32

* solitary tumors (arise to one side of nerve root ) * Can rise anywhere Histologically *palisading

Question 33

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

Answer 33

true

Question 34

Malignant peripheral nerve sheath tumors

Answer 34

* affects adults and children * 50% associated with neurofibroma * Bulky deep-seated tumors arise from major nerves

Question 35

summary

Answer 35

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