8850/3 Fibroliposarcoma


Related terms

Liposarcoma, NOS

Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)




Breast
ICD-O-3 topography code: C50
ICD10: C50
           

A malignant soft-tissue tumour showing pure adipocytic differentiation.



Thymus
ICD-O-3 topography code: C37
ICD10: C37
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)




Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)




Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)




Mediastinum
ICD-O-3 topography code: C38.1-C38.3
ICD10: C38.1-C38.3
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)




Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)




Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrangements, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)
In the vulva, liposarcomas are extremely rare. Reported cases included atypical lipomatous tumours (well differentiated liposarcomas) and myxoid liposarcomas 3
Click to access Pubmed
Nucci MR, Fletcher CD (1998)
Liposarcoma (atypical lipomatous tumors) of the vulva: a clinicopathologic study of six cases.
Int J Gynecol Pathol 17: 17-23



4
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

Liposarcoma is a malignant tumor composed entirely or in part of neoplastic adipocytes. There are five variants that differ in morphology, clinical manifestation, genetic profile and prognosis:
Variant/synonymFraction of liposarcomasAgeLocationMetastasisGenetics
Well differentiated liposarcoma / Atypical lipomatous tumour40-50%Adults; peak 6th decadeLimbs (thigh), retroperitoneum, paratesticular area, mediastinumOnly after dedifferentiation (<10% of cases)Supernumerary ring and giant marker chromosomes, MDM2 amplification
Dedifferentiated liposarcoma<10%AdultsRetroperitoneum, limbsYes, in 15-20% of casesSupernumerary ring and giant marker chromosomes; multiple abnormal clones
Myxoid liposarcoma/Round cell liposarcoma30-35%Peak 4th-5th decadeMusculature of the thigh; deep soft tissues (limbs)Yes, in approx. 1/3 of casesTranslocation t(12;16)(q13;p11) in 90% of cases
Pleomorphic liposarcomApprox. 5%Adults > 50 yearsLower limbs > upper limbsYes, in 30-50% of casesComplex rearrange-ments, with ring, giant marker and double minute chromosomes
Mixed-type liposarcomaVery rare> 60 yearsRetroperitoneum, abdomenYesRing and giant marker chromosomes, 12q14-15 amplification (MDM2)