Malignant Fibrous Histiocytoma (MFH)

8/6/2019 Malignant Fibrous Histiocytoma (MFH)

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Malignant fibrous histiocytomaMalignant fibrous histiocytoma(MFH), a type of sarcoma, is a(MFH), a type of sarcoma, is amalignant neoplasm of uncertainmalignant neoplasm of uncertainorigin that arises both in soft tissueorigin that arises both in soft tissueand bone.and bone.

It was first introduced in 1961 byIt was first introduced in 1961 byKauffman and Stout .Kauffman and Stout .

No true cell of origin has ever beenNo true cell of origin has ever beenidentified.identified.

In 2002, the World HealthIn 2002, the World HealthOrganization (WHO) renamed it asOrganization (WHO) renamed it as

diff i d l hidiff i d l hi


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StoriformStoriform--pleomorphicpleomorphic ::Most common type, accountingMost common type, accounting

for up to 70% offor up to 70% of cases.cases. Myxoid :Myxoid :

Second most common accountingSecond most common accounting

for approximately 20% of cases. Afor approximately 20% of cases. Alow grade neoplasm and Have alow grade neoplasm and Have aindolent course.indolent course.

Giant cell : RareGiant cell : Rare

I fl A i dI fl A i d


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Typically presents approximately at 50 to70 years of age

Very rare below 20 years. Slight male predominance. Most common in the lower extremity,

especially the thigh.

Other locations include the upperextremity, retro peritoneum andposterior mediastinum.

Patient present with a mass or lump that

has arisen over a period of time rangingfrom weeks to months. Usually painless unless it is compressing a


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Confirmation of the diagnosis

FNACFNAC

IncisionalIncisional biopsybiopsy

ExcisionalExcisional biopsybiopsy (for tumors' size


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Staging Investigations :

X-ray when limb is affected, is

the first imaging test.MRI is the most powerful tool

irrespective of location. Itprovides vital information

aboutExact size

Infiltration to surroundingTumour necrosis

Proximity to neurovascularstructures.


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Lungs (90%).Lungs (90%).

ExtraExtra--pulmonary sitespulmonary sites

Lymph nodes (10%)Lymph nodes (10%)

Bone (8%)Bone (8%)

Liver (1%)L

iver (1%)


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The American Joint Committee onCancer (AJCC)Staging System for SoftTissue Sarcoma,

6th Edition

Stage Size Depth Grade Met.

I Any Any Low No

II < 5 cm,

anydepth

Or > 5

cm

Superfi

cial

High No

III > 5 cm Deep High No


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Requires a multimodalityapproach.

T

ypes of treatment : Surgery

Radiation

Chemotherapy


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The goal of surgery is to acquireThe goal of surgery is to acquirelocal control of the lesionlocal control of the lesion

Surgical options for extremitySurgical options for extremity

sarcomassarcomas

limblimb--sparing surgerysparing surgery AmputationAmputation

Limb sparing surgery mandatesLimb sparing surgery mandates

complete removal of the tumourcomplete removal of the tumour

good function of the remaininggood function of the remainingli bli b


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The purpose of radiation is to improveThe purpose of radiation is to improvelocal tumor control by killing residuallocal tumor control by killing residual

microscopic disease.microscopic disease.

Types of radiation:Types of radiation:I. External beam radiation commonlyI. External beam radiation commonly

used.used.ii. Brachytherapyii. Brachytherapy

May be givenMay be given

prepre--operativelyoperatively

intraintra--operativelyoperatively


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The role of chemotherapy in theThe role of chemotherapy in thetreatment of MFH is usuallytreatment of MFH is usually

palliativepalliative

Chemotherapy is given to patientsChemotherapy is given to patients

To down stage the lesionT

o down stage the lesionhave metastatic diseasehave metastatic disease

at the highest risk for developingat the highest risk for developing

metastatic disease (unfavorablemetastatic disease (unfavorableprognostic factors)prognostic factors)


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Favorable prognostic factors

Low gradeLow grade

SuperficialSuperficial

Less than 5 cmLess than 5 cm

Absence of metastatic diseaseAbsence of metastatic disease

Less than 60 yearsLess than 60 years

Myxoid subtypeMyxoid subtype

5 year survivality depend on stage of5 year survivality depend on stage ofthe disease at diagnosisthe disease at diagnosis

A series reported 34% to 70% rate forA series reported 34% to 70% rate for


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MFH is a curable disease.MFH is a curable disease.

The mainstays of treatment for MFH areThe mainstays of treatment for MFH are

complete surgical excision most oftencomplete surgical excision most oftensupplemented with adjuvant radiationsupplemented with adjuvant radiationtherapy.therapy.

Chemotherapy is reserved for patients atChemotherapy is reserved for patients at

the highest risk of disease recurrence orthe highest risk of disease recurrence orpatients that already have recurrence.patients that already have recurrence.

Patients with recurrent MFH can still bePatients with recurrent MFH can still becured.cured.

Favourable prognostic factors thatFavourable prognostic factors thatcorrespond to superior survival includecorrespond to superior survival include


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Malignant Fibrous Histiocytoma (MFH)

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