This document provides information about soft tissue tumors. It discusses the epidemiology, classification, etiology, diagnosis and treatment of both benign and malignant soft tissue tumors. Some key points include: - Benign soft tissue tumors are more common than sarcomas. Common benign tumors include lipomas, schwannomas and giant cell tumors of the tendon sheath. - Risk factors for soft tissue sarcoma include exposure to herbicides/pesticides, radiation exposure, genetic conditions and viral infections. - MRI is usually the best imaging modality for evaluating soft tissue tumors. Biopsy is needed for diagnosis. - Treatment depends on whether the tumor is benign or malignant. Benign tumors may

1. SOFT TISSUE TUMOR
DR MANOJ DAS
Ortho resident
Institute Of Medicine , TUTH , Nepal

2. Intoduction
• Neoplastic condition arising from extra-skeletal mesoderm
-Fibrous tissue
-Smooth muscle
-Skeletal muscle
-Adipose tissue
-Blood and lymphatics vessels
-Peripheral nerves

3. Epidemology
-The annual clinical incidence (number of new patients
consulting a doctor) of benign soft tissue tumours has been
estimated as up to 3000/million population
-The annual incidence of soft tissue sarcoma is around
30/million i.e. less than 1 percent of all malignant tumours
-Benign mesenchymal tumours outnumber sarcomas by a
factor of at least 100.

4. Classification

5. Classification…

6. Classification…

7. Aetiology
• Unknown
• In rare cases, genetic and environmental factors,
irradiation, viral infections and immune deficiency
have been found associated with the development of
usually malignant soft tissue tumours

8. Aetiology
• Chemical carcinogens
-Several studies, many of them from Sweden, have reported an increased
incidence of soft tissue sarcoma after exposure to phenoxyacetic
herbicides,chlorophenols, and their contaminants(dioxin) in agricultural or
forestry work
{607,608}.
-Other studies have not found this association.
-One explanation for different findings may be the use of herbicides with
different dioxin contaminations {4,2333}.

9. Aetiology…
Radiation
-incidence of post-irradiation sarcoma ranges from some few per
thousand to nearly one percent.
-incidence estimates are based on breast cancer patients treated
with radiation as adjuvant therapy. The risk increases with dose;
most patients have received 50 Gy or more and the median
time between exposure and tumour diagnosis is about 10 years.
-Patients with a germline mutation in the retinoblastomas gene
(RB1) have a significantly elevated risk of developing post-
irradiation sarcomas, usually osteosarcoma

10. Aetiology…
- Viral infection and immunodeficiency Human herpes virus 8 plays a
key role in the development of Kaposi sarcoma and the clinical
course is dependent on the immune status of the patient {2232}.
-Epstein-Barr virus is associated with smooth muscle tumours in
patients with immunodeficiency {1368}.
-StewartTreves syndrome, development of angiosarcoma in chronic
lymphoedema, particularly after radical mastectomy, has by some
authors been attributed to regional acquired immunodeficiency

11. Aetiology…
Genetic susceptibility
hereditary multiple lipomas (often angiolipomas)
Desmoid tumours occur in patients with the familial Gardner syndrome (including
adenomatous polyposis, osteomas and epidermal cysts)
Neurofibromatosis (types 1and 2) is associated with multiple benign nerve tumours (and
sometimes also nonneural tumours). In around 2% of the patients with
neurofibromatosis type1 malignant peripheral nerve sheath tumours develop in a
benign nerve sheath tumour
Li-Fraumeni syndrome {954} is a rare autosomal dominant disease caused by germline
mutations in the TP53 tumour suppressor gene, which seems to be of importance for
sarcomagenesis. Half of the patients have already developed malignant tumours at age
30, among them, in more than 30% of cases, soft tissue and bone sarcomas
.

12. Approach to diagnosis
History
- Age
- Symptoms
Mass
Pain
Numbness
- Duration
- Trauma
- Family History
- Constitution Symptoms

13. Approach to diagnosis…
Local Examination
-site
-Size
-Depth
-Consistency
-Mobility
-Pulsation
-Tinel’s Sign
-Neurovascular Status

14. • General Physical
examination
- café-lauit spot
- Freckles
• Lymph Node
Examination

15. X -Ray
• Provide little
information
• Fat density
radiolucencies
• Phleboliths
• calcification

16. CT Scan
• Replaced by MRI in most institutions due to
the superior soft tissue contrast achieved by
MRI
• still be useful for imaging soft tissues near the
chest wall that are subject to motion artifact
with MRI, and in cases where MRI is not an
option

17. MRI
- modality of choice for detecting, characterizing,
and staging soft tissue tumours
- distinguish tumour tissue from adjacent muscle
and fat
- define relationships to key neurovascular
bundles

22. PET Scan
• assess metabolic function and can be used
to diagnose, stage, and monitor treatment
response for many cancers, and some soft
tissue tumors.
• One specific application is in the evaluation
of multifocal desmoid tumors, and more
specifically, response to treatment.
“ Kasper et al. demonstrated that PET imaging
might complement CT and improve the
assessment of patients with desmoid
tumors. Imatinib, a tyrosine kinase inhibitor,
has been shown to successfully stabilize
desmoid tumors, and PET imaging has been
used to monitor response to imatinib
treatment in patients with multifocal
desmoids [9]”

23. Biopsy
• open (incisional or excisional) or closed (needle or trephine)
Closed Biopsy
Advantage
- less invasive,
-require smaller doses of anesthetic and analgesic agents
-lower risk of tumor contamination, hemorrhage, and
infection
Disadvantage
-Smaller amount obtained for pathologic review

24. Some rules for for Biopsy
“One should always approach tissue biopsy with the assumption
that there is a possibility for malignancy, using a well-planned biopsy site”
- Perform the biopsy through a longitudinal incision.
-In the operative dissection, do not expose or contaminate important vascular or neural
structures.
- Do not violate anatomic compartments other than those containing the tumor.
- Take special care around joints to prevent intraarticular contamination with tumor cells.
- Biopsies of soft tissue should not involve major tendons (e.g., the patellar tendon) or their
insertion
-In a biopsy, do not use intramuscular or intranervous planes, as in more standard orthopaedic
approaches. Instead, use a direct approach with the smallest possible incision directly
through skin, subcutaneous tissue, fascia, and muscle down to tumor

25. Staging of soft tissue tumor

26. Staging of soft tissue sarcoma
American joint committee on cancer for staging of soft
tissue sarcoma

27. Treatment of Benign Soft tissue tumor
Observation
- Asymptomatic
- <5cm
- Superficial to fascia
-Reevaluation every 6-12 weeks followed by every
3-6 months for 1 yr to document lack of growth
Biopsy and Surgical Excision
- not following natural history
-Symptomatic

28. Treatment of Soft tissue Sarcoma
Goal of Treatment
-minimizing local recurrence
-maximizing function
-improving patient survival
Surgery- Mainstay of treatment

29. Limb Salvage Surgery VS Amputation
• Amputation rarely done nowadays
• Limb sparing procedure are performed in 90-95% of cases
“Rosenberg et al published a landmark article in 1982, a prospective
randomized trial to evaluate the issue of amputation versus limb-
sparing surgery plus radiation therapy in soft tissue sarcomas of the
extremity. They found that although local recurrence was greater in
the limb-sparing group, disease-free survival was no different”

31. Role of Radiotherapy in soft tissue
sarcoma
• enhance local control
• preserve function
• achieve acceptable cosmesis by contributing to tissue preservation
“Dagan et al. performed a retrospective review to evaluate the local control
and amputation-free survival in patients who received preoperative
radiation therapy prior to undergoing a marginal resection for a soft tissue
sarcoma of the extremity. The authors concluded that patients can expect
excellent rates of local control and limb preservation regardless of whether
they have a marginal, wide, or radical resection according to the classic
Enneking margin definitions [37].”

32. Role of Chemotherapy in soft tissue
Sarcoma
• Controversial and still investigated
• palliative and used to treat unresectable or metastatic disease to try and slow
progression
“ In 1997, the Sarcoma Meta-Analysis Collaboration published their results of
doxorubicin-based chemotherapy regimens combined with surgery for local
control. Disease-free survival improved from 45 % for control patients to 55 % for
chemotherapy patients, an advantage of only 10 %. The authors were also unable
to show a statistically significant benefit to improved survival at 10 years, with
survival of patients undergoing chemotherapy at 54 %, compared to the control at
50 %”
Favorable response to chemotherapy,
-synovial sarcoma
-Liposarcoma
- pediatric rhabdomyosarcom

33. Surveillance Following Definitive
Treatment
• For potential development of local recurrence or metastatic
disease
• 40–60 % of patients will develop local or distant recurrence, and most
recurrences occur within 2 years of treatment of the primary tumor
• Current recommendations for surveillance include:-
- clinical exam and chest radiograph or CT scan every 3 months for
the first 2 years, every 4 months for the third year, every 6 months for
the following 2 years, and annually thereafter
• Local recurrence monitored with physical exams of the surgical site at
routine intervals
-If the patient is felt to be at high risk for local recurrence or there
is concern on physical exam findings, then an ultrasound or MRI with and
without gadolinium should be obtained.

34. Some important soft tissue tumor

35. LIPOMA
• the most common soft tissue lesions
• AGE:- 5th to 7th decades of life without a clear gender predilectioN
• prevalence:- 2.1 per 100 people
• Site:-upper back, neck, proximal extremities, and
abdomen
• Clinical Presentation
-Painless mass
-can be a/w local pain, limitation of range of motion, and nerve
compression in approximately 25 % of patients
-Usually Solitary
• On physical Examination
Mobile
Doughy in consistency
usually S/C
Investgations
X-ray - Soft tissue radiolucency
MRI – Investigation of choice
Isointense to s/c fat

37. LIPOMA…
Tissue Diagnosis
Gross
-yellow to orange
color with a greasy
appearance
Microscopic
-mature adipocytes with
uniform eccentric nuclei

38. Lipoma…
Treatment
 Observation
 Excisional biopsy
-increasing size
-Symptomatic
- Cosmetically undesirsble

39. Benign Peripheral Nerve Sheath Tumor
Include
-Schwannoma
-Neurofiroma
Age:- 20-50yr Schwannoma
40-50 yr Neurofibroma
A/W Neurofibromatosis Type 2
Clinical Presentation
- Painless mas
- Neurological symptoms
On Physical Examination
-Firm
- Mobile
-Tender to touch and pressure ( Schannoma)

40. Benign Peripheral Nerve Sheath
Tumor..
X-Ray
- Soft tissue mass
MRI
-Fusiform mass, often
displacing
neurovascular bundle

42. Benign Peripheral Nerve Sheath
Tumor…
Tissue Diagnosis
Gross
-cut surface of a schwannoma has a
yellow-gray
color, sometimes with cystic regions
Microscopic
- Antoni A
- Antoni B
Neurofibromas-
- not encapsulated
- Histologically spindle-shaped cells in
a myxoid stroma, with some collagen
fibers.

43. Benign Peripheral Nerve Sheath
Tumor…
Treatment
Observation with Serial Radiograph
Surgical Excision
Risk for neurological deficit after surgical excision???
- secondary to iatrogenic fascicular injury
“Kim et al. evaluated postoperative neurological deficits and potential
risk factors associated with this complication. They found an
immediate neurological deficit in 76.7 % of their 30
patients, and at final followup, residual deficits persisted in 36.7 % of
patients. Therisk for deficits was highest in patients with larger
tumors.”

44. Giant Cell Tumor of Tendon Sheath
• the most common benign neoplasm of the hand, often found on the fingers
• fourth to fifth decade of life
• On physical examination
-firm
-minimally mobile mass that runs along tendon
-usually nontender
Radiographs
-nonspecific
-cortical erosion
Ultrasound
-To distinguish this solid tumor from a ganglion cyst
MRI
- circumscribed soft tissue mass adjacent to a tendon, and is isointense with
muscle on T1-weighted images

45. Giant Cell Tumor of Tendon Sheath…
• On gross examination
-dense capsule
- C/S gray-white appearance with
variegated pink, brown, or yellow
discoloration
• Histologically
the predominant cells are
mononuclear cells, epithelioid
histiocyte like cells, giant cells, and
xanthomatous cells, hemosiderin-
laden macrophages
Treatment
-Excision Biopsy
Recurrence Rate 10-20 %

46. Hemangioma
• benign vascular lesions that histologically resemble normal blood vessels
• Common tumor in 1st decade of life
• Sites
- Skin
- Subcutaneus tissue
- Intramuscular
-Intraarticular
-Bone
Types
- Capillary
- Cavernous
-Venous
-Arteriovenous Malformation

47. Intramuscular Hemangioma
• More often larger than cutaneous hemangioma
• Composed of morphologically benign vascular channels occuring within
muscle
• Age – Adolescents and adult
• Sites –Lower limb m/c, Head neck, upper limb
• Clinical presentation
- Slowly enlarging mass
- painful
-pain worse after exercise
-Changes size according to position of limb
X-Ray
-Phlebolith
-Pressure erosion of bone or extension in to bone( Channel like
radiolucencies )

48. Intramuscular Hemangioma…
CT Scan
- poorly defined lesion attenuation
similar to muscle
-Marked contrast enhancement of
serpentine vascular channels
-Subtle phlebolith
MRI
- hypointense, or isointense to
muscle on T1-weighted sequences
-hyperintense on T2-weighted
sequences due to the
vascular channels

49. Intramuscular Hemangioma…
Gross
- large, poorly demarcated,
yelowish
- Vascular and hemorhagic areas
Microscopic
- Mixed vessel type
- Lined by normal appearing
epithelial cells
- Diffusely infiltrate muscle and
entrap muscle cells
- Prominent adipose component

50. Intramuscular Hemangioma…
Treatment
-Observation
-Surgical excision
-Embolization
-Injection with sclerosing agents

51. Desmoid Tumor
• Benign , non metastasizing infiltrative fibrofroliferative neoplasm
• Composed of fibrocytes, fibroblasts, and myofibroblasts within a
collagenenous to myxoid stroma
• Infiltration to muscle occur routinely
• High propensity for local recurrence
• A/W Familial Adenomatous polyposis

52. Desmoid Tumor…
• Age 15-40
• F>M
• Sites –
-shoulder girdle/upper arm(mc)
-Buttock
-Trunk
-Head and Neck
Clinical Presentation
- painless/painful mass
-When in close proximity to a joint, range of motion may be limited
-Neurological symptoms if the tumor compresses or invades peripheral
motor or sensory nerves.

53. Desmoid Tumor…
X-ray
-Soft tissue mass
-Cortical erosion
CT scan
- Similar accentuation to
muscle
MRI
-low signal intensity on both T1
and T2 Sequence
- Frequently enhance after i/v
contrast

54. Desmoid Tumor…
Gross
- Large tumor
- Glistening with fibrous tissue
- Infiltrates skeletal muscle at
periphery
Microscopic
- Normal appearing sparse
fibroblastic cells within dense
fibrous stroma
- Macrophage, lymphocyte, giant
cells

55. Desmoid Tumor…
Treatment
- Observation for asymptomatic and stable
- Aggressive treatment for symptomatic lesion
Surgical excision+ Adjuvant Radiotherapy

56. Glomus Tumor
• Benign lesion arising from glomus bodies
• 2 % of soft tissue tumors
• Sites- hand especially within the subungual skin, palm, wrist, forearm, or
foot
• Age – adult
• Clinical presentation: Triad of
-hypersensitivity to cold
- paroxysmal pain
-pinpoint pain
• Love’ s Pin test- 100% sensitive
• Hildreth’s test- 100% specific and & 79% sensitive

57. MRI
-increased signal intensity on T2-
weighted images, and strong
contrast enhancement
Gross- small blue red nodules
Microscopic- glomus cells , smooth
cells, blood vessels
Treatment
-Excision biopsy

58. Synovial chondromatosis
• disorder in which cartilaginous nodules are formed within a synovial joint
• The nodules then detach from the synovium and may undergo
calcification
• Age- third and fifth decades
• M:F 2:1
• Site- Usually monoarticular with the knee being the most common joint
affected, but it can involve any synovial joint
• Clinical presentation
-pain, swelling, stiffness, and sometimes locking of the involved joint.
On physical examination
- effusion, tenderness, and decreased ROM.

59. Synovial chondromatosis…
Radiograph
-multiple intra-articular densities
when calcified
- ‘‘ring-and-arc’’ chondroid
mineralization pattern
MRI
-visualizing radiolucent nodules where
they have low signal on T1-weighted
images, and increased signal on T2-
weighted images
-erosive changes of the
articular cartilage
-in late stages, there is joint space
narrowing, osteophytes, and
sclerosis much like an osteoarthritic
joint

60. Synovial
chondromatosis…
On gross inspection
-the synovial nodules, which are composed of
hyaline
cartilage, typically have a cobblestone
appearance
histologic appearance
-benign hyaline cartilage, with low to
moderate cellularity, and a synovial tissue
lining outside
Treatment
-Nodule removal and Synovectomy
-Open or arthroscopic approach

61. Liposarcoma
• 20 % of all soft tissue sarcomas in adults
• Ages 50–65
• Site-thigh and retroperitoneum
• 1) Well-differentiated liposarcomas (atypical lipomatous tumor)
2)Dedifferentiated liposarcomas
• 3)Myxoid or Round cell liposarcomas
• 4)pleomorphic

62. Rhabdomyosarcoma
• most common soft tissue sarcoma of infants and children, although they
can occur in adults
• Site – in areas lacking skeletal muscle- head, neck and genitourinary tract
• Embryonal rhabdomyosarcoma
–most common type
-head , neck and genitourinary tract
• Alveolar rhabdomyosarcoma
- Aggressive type
-arm, leg and trunk
Pleomorphic rhabdomyosarcoma
-common in adults
- poor prognosis
Clinical presentation
depends on location

64. Treatment
Surgery+ Chemotherapy+Radiotherpy

65. In summary…

66. Thank you