Malignant fibrous histiocytoma (MFH), became Undifferntiated pleomorphic sarcoma UPS after 2002 WHO classification of tumors

1. By
Ahmed Nabeel Mohamed

2. Overview:
Definition.
Incidence.
Presentation.
Diagnosis.
Treatment.

3. Definition:
The most common soft tissue sarcoma of adult life.
 Malignant neoplasm of uncertain origin that arises
both in soft tissue and bone.
The precise origin of MFH cells has been disputed.
In 2002, the World Health Organization (WHO)
renamed it as an undifferentiated pleomorphic
sarcoma.

4. Epidemiology:
 Rarely occurs in the bone.
 Males are affected more often than females (9:4).
 MFH occurs most commonly in the extremities
(70-75%) with lower extremities accounting for 60% of
cases), followed by the retroperitoneum.
 Commonly in the metaphysis of long bones such as
femur and tibia.
 Accounts for 10% to 20 % of all soft tissue malignant
neoplasms.

5. CLASSIFICATION
Five subtypes of MFH are present, These are:
(1) Storiform pleomorphic.
(2) Myxoid.
(3) Inflammatory.
(4) Giant cell.
(5) Angiomatoid.

6. Gross picture:
• MFH is a lobulated, fleshy, gray
white mass.
• There may be yellow areas of
lipid or darker areas of
hemorrhage.
• The mass may be all soft tissue
or have intra-osseous extension.
• The margins of the tumor are
normally ill defined and
destructive.

7. Microscopic picture:
• In all forms, there are fibroblast and
histiocyte elements in some ratio.
• Calcifications are formed by reactive
periosteal cells and are not produced by
tumor cells, which help to differentiate the
tumor from fibrosarcoma.

8. Inflammatory
malignant fibrous
histiocytoma
5% of cases
Myxoid malignant
fibrous histiocytoma
10%-20% of cases
The storiform-
pleomorphic MFH
60-70% of cases

9. Nomenclature and Categorization of Malignant Fibrous Histiocytoma
(MFH) Subtypes (2002 World Health Organization Classification)
Old Nomenclature of
MFH Subtype
Current Nomenclature of
MFH Subtype
Tumor Category
Storiform-pleomorphic
MFH
Undifferentiated high-
grade pleomorphic
sarcoma
Fibrohistiocytic
Myxoid MFH Myxofibrosarcoma Myofibroblastic
Giant cell MFH
Undifferentiated
pleomorphic sarcoma
with giant cells
Fibrohistiocytic
Inflammatory MFH
Undifferentiated
pleomorphic sarcoma
with prominent
inflammation
Fibrohistiocytic
Angiomatoid MFH
Angiomatoid fibrous
histiocytoma
Tumors of uncertain
differentiation

10. Symptoms and Presentation:
 Patients often complain of
a rapidly enlarging mass or
lump .
 Usually painless unless it is
compressing a nearby
nerve.
 weight loss and fatigue are
not typical .

11. Imaging:
 As with other soft-tissue tumors, MRI is the imaging
method of choice.
 CT scanning is also useful for evaluation of
calcifications.
 MFH usually presents with a soft tissue mass with or
without cortical erosion.
 There is no periosteal reaction.

12. x-ray :
 Plays a key role in establishing the initial diagnosis of bone
tumors.
 Advantages: helps to detect
1. Margins.
2. Cortical Expansion.
3. Periosteal Reaction.
4. Matrix and Tumor Mineralization.

14.  Computed Tomography:
CT may be used to evaluate potential internal matrix
and/or cortical erosion.

15.  Magnetic Resonance Imaging:
As with other soft-tissue tumors, MRI is the imaging
method of choice.

16. Many other techniques can helps the diagnosis as:
 Biopsy.
 Immunohistochemistry.
 Electron Microscopy.
 Molecular Techniques.
 Cytology.
 Nuclear Imaging.
 Angiography.

17. Biopsy
 The definitive diagnostic test for tumors.
Fine needle aspiration biopsy.
Core needle biopsy.

18. Open biopsy:
Incisional biopsies.
Excisional biopsies.

19. The recommendations of succesful biopsy:
 It is not a simple procedure.
 Pay as close attention to asepsis, skin preparation,
haemostasis, wound closure and so on as with any
other operation.
 Avoid transverse incisions.
 CT-guided biopsy offers an additional advantage as
it has an overall accuracy of 80%.

20. Differential diagnosis:
 Synovial sarcoma.
 Aggressive fibromatosis.
 Lytic metastasis.
 Soft tissue metastases.
 Myositis ossificans.
 Benign fibrous histiocytoma.

22. There are essentially three main types of
treatment that will need to be coordinated to
treat the MFH:
1. Surgery.
2. Radiation.
3. Chemotherapy.

23.  Staging:
 The American Joint Committee on Cancer (AJCC)
Staging System for Soft Tissue Sarcoma, 6th Edition
Stage Size Depth Grade Metastases
I Any Any Low No
II
< 5cm, any
depth OR >
5cm
Superficial High No
III > 5cm Deep High No
IV Any Any Any Yes

24. Surgery:
 Surgery is the cornerstone of treatment for all soft
tissue sarcomas.
 Historically, soft tissue sarcomas were treated with
amputation.
 Currently at least 90% of tumors are now removed
using limb-sparing surgery .

25.  Classification of surgical resections for the treatment
of sarcoma .
Intralesional Partial removal of the tumor
Marginal
Through the reactive zone; may leave
residual microscopic disease
Wide
Entire tumor removed with a cuff of
normal tissue
Radical
Entire compartment containing the
tumor removed

26. Intraoperative image demonstrates a posterior approach to
the calf for removal of a malignant fibrous histiocytoma.
Neurovascular dissection and wide excision of the mass.

29. Radiation therapy :
 The purpose of it is to improve local tumor control
by killing residual microscopic disease.
 Pre-operative radiation can potentially shrink the
tumor, making limb-sparing surgery possible or
easier but not commonly used.
 Post-operative radiation is probably the most
commonly used modality.

30. The advantages and disadvantages of the timing of
radiation therapy:
Delivery Method Advantages Disadvantages
Pre-operative
1. Potentially shrinks
tumor
2. Smaller volume required
1. Increase in wound
complications
2. Delay in definitive surgery
Intra-operative
1. Can concentrate very
high doses to close
margins
2. Minimal injury to
normal tissue
1. Requires a special
operating room with
exposure to O.R. staff
2. Wound complications
Post-operative
1. Fewer wound
complications
2. Immediate surgery
1. Larger volume required
secondary to operative
contamination.
2. Second sarcoma .

31. Chemotherapy:
 Is often incorporated into the treatment of patients
with distant recurrence.
 One of the major limitations of chemotherapy is the
associated toxicities with the doses necessary to
have a significant impact on disease-specific
survival.
 The role of chemotherapy in the treatment of MFH is
not entirely clear.

32. PROGNOSIS:
 Tumor grade, size, and presence of distant
metastases, are the most important prognostic factors.
 The 5 year survival estimates range from 34% to 60%.
 Local recurrence (LR), recurrence of the tumor in the
same location, will occur in approximately 20-30% of all
patients with soft tissue sarcomas.

33.  MFH is a curable disease if early diagnosed.
 "Malignant Fibrous Histiocytoma" has been changed
by the WHO to Undifferentiated Pleomorphic
Sarcoma “UPS”.
 surgical excision most often supplemented with
adjuvant radiation therapy.