V Malignant fibrous histiocytoma

(MFH), a type of sarcoma, is a

malignant neoplasm of uncertain
origin that arises both in soft tissue
and bone.
V It was first introduced in 1961 by
Kauffman and Stout .
V No true cell of origin has ever been
identified.
V In 2002, the World Health
Organization (WHO) renamed it as
an Y
 

 
   

  
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NOS .
V Storiform-pleomorphic :
Storiform-
Most common type, accounting
for up to 70% of cases.
V Myxoid :
Second most common accounting
for approximately 20% of cases. A
low grade neoplasm and Have a
indolent course.

V Giant cell : Rare

V Inflammatory : Aggressive and

usually occur in the
retroperitoneum..
retroperitoneum
V ½ypically presents approximately at 50 to
70 years of age
V Very rare below 20 years.
V Slight male predominance.
V Most common in the lower extremity,
especially the thigh.
V Other locations include the upper
extremity, retro peritoneum and
posterior mediastinum.
V Patient present with a mass or lump that
has arisen over a period of time ranging
from weeks to months.
V Usually painless unless it is compressing a
nearby nerve.
V Weight loss, easy fatigability and
anorexia are features of advanced
disease.
V Gonfirmation of the diagnosis

u FNAG

u Incisional biopsy

u Excisional biopsy (for tumors' size <3cm)

V Staging Investigations :
èÿ-ray when limb is affected, is
the first imaging test.
èMRI is the most powerful tool
irrespective of location. It
provides vital information
about
èExact size
èInfiltration to surrounding
è½umour necrosis
èProximity to neurovascular
structures.
è For limb mass,
compartmental involvement
è GÿR & G½ Scan of the chest to
detect metastasis to the lungs.
V ^ungs (90%).

V Extra-pulmonary sites
Extra-
è^ymph nodes (10%)
èBone (8%)
è ^iver (1%)
½he American Joint Gommittee on Gancer (AJGG)
Staging System for Soft ½issue Sarcoma,
6th Edition
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Requires a multimodality
approach.

½ypes of treatment :
ü Surgery
ü Radiation
ü Ghemotherapy
V ½he goal of surgery is to acquire
local control of the lesion
V Surgical options for extremity
sarcomas
è limb
limb--sparing surgery
è Amputation
V ^imb sparing surgery mandates
ècomplete removal of the tumour
ègood function of the remaining
limb
V Reconstruction following tumor
resection is sometimes necessary
V ½he purpose of radiation is to improve
local tumor control by killing residual
microscopic disease.

V ½ypes of radiation:
I. External beam radiation commonly
used.
ii. Brachytherapy

V May be given
è pre
pre--operatively
è intra
intra--operatively
è post
post--operatively
è in some combination.
V ½he role of chemotherapy in the
treatment of MFH is usually
palliative
V Ghemotherapy is given to patients
è½o down stage the lesion
èhave metastatic disease
èat the highest risk for developing
metastatic disease (unfavorable
prognostic factors)

V Extremity MFH responds better

than that of the retroperitoneal
ones
ü Favorable prognostic factors
è^ow grade
èSuperficial
è^ess than 5 cm
èAbsence of metastatic disease
è^ess than 60 years
èMyxoid subtype

ü 5 year survivality depend on stage of

the disease at diagnosis

ü A series reported 34% to 70% rate for

stage III tumors
V MFH is a curable disease.
V ½he mainstays of treatment for MFH are
complete surgical excision most often
supplemented with adjuvant radiation
therapy.
V Ghemotherapy is reserved for patients at
the highest risk of disease recurrence or
patients that already have recurrence.
V Patients with recurrent MFH can still be
cured.
V Favourable prognostic factors that
correspond to superior survival include
small tumour size, low grade, extremity
location, superficial location, and
localized disease.