Well differentiated-differentiated liposarcomas (WDLPS) or atypical lipomatous tumours (ALT) are locally aggressive adipocytic soft tissue neoplasms and are the most common form of liposarcomas.

Terminology

Atypical lipomatous tumours/well differentiated-differentiated liposarcomas (ALT/WDLPS) are also termed ‘atypical lipomas’, but this term has been discouraged ¹.

Epidemiology

Atypical lipomatous tumours or well differentiated-differentiated liposarcomas account for 40-45% of liopsarcomasliposarcomas and constitute the largest subgroup of adipocyte malignancies. They occur mostly in adults with a peak incidence in the 30s and 40s. In childhood, they are extremely rare. Inguinal lesions are more common in men otherwise there is no gender predominance however ^1-3.

Associations

Atypical lipomatous tumours/well differentiated-differentiated liposarcomas may be associated with Li-Fraumeni syndrome ¹.

Clinical presentation

The most common presentation is that of a painless mass. Deep seated-seated atypical lipomatous tumours/well differentiated-differentiated liposarcomas are most often found incidentally ¹.

Complications

If left untreated atypical lipomatous tumours/well differentiated-differentiated liposarcomas can dediffrientiatededifferentiate into higher-grade malignancies such as dedifferentiated liposarcoma or undifferentiated pleomorphic sarcoma ^1-4.

Pathology

Atypical lipomatous tumours/well differentiated-differentiated liposarcomas are adipocytic neoplasms characterized by a proliferation of pleomorphic mature adipocytes of different patterns featuring atypical hyperchromatic stromal cells. They are intersected by fibrous septa, might have myxoid or fibrous components and areas of fat necrosis ^1-4.

Location

Atypical lipomatous tumours/well differentiated-differentiated liposarcomas commonly involve the deep soft tissues in particular of the proximal extremities and the trunk ^1-5.

Common locations include the following ¹:

• thigh and buttocks
• shoulder and back
• retroperitoneum
• paratesticular area

Rarer locations of involvement include the following ¹:

• head and neck
• mediastinum
• distal extremities
• skin

Classification

Atypical lipomatous tumours/well differentiated-differentiated liposarcomas includesinclude the following subtypes ^1-4,6:

• lipoma-like liposarcoma
• sclerosing liposarcoma
• inflammatory liposarcoma

Macroscopic appearance

Macroscopically atypical lipomatous tumours/well differentiated-differentiated liposarcomas usually present as well-circumscribed, large lobulated masses of variable consistency and white to yellowish colour. They might display foci of necrosis or small punctate haemorrhages ¹.

Microscopic appearance

The microscopic appearance of atypical lipomatous tumours/well differentiated-differentiated liposarcomas includes the following features. Larger tumours and especially retroperitoneal tumours commonly show more than one morphologic pattern within the same lesion^1,4:

• presence of atypical hyperchromatic stromal cells
• variable number of lipoblasts
• possibly variations in adipocyte size with nuclear atypia (lipoma-like subtype)
• possibly copious inflammatory infiltrates (inflammatory subtype)
• possibly bizarre stromal cells within a fibrillary sclerotic, collagenous background (sclerosing subtype)

Immunohistochemistry

Immunohistochemistry stains are usually positive MDM2 and/or CDK4 ¹.

Genetics

The pathogenesis of atypical lipomatous tumours/well differentiated-differentiated liposarcomas involves MDM2 and/or CDK4 nuclear gene amplification ^1,2.

Radiographic features

Imaging features of atypical lipomatous tumours/well differentiated-differentiated liposarcomas resemble that of benign lipomas as discussed in the article lipoma vs well-differentiated liposarcoma and include ^3-5:

• size > 5 cm
• thick or nodular septae (>2 mm) with possible enhancement
• multinodular tumour margins
• focal nodular patchy non-fatty tissue components
• more than ¾ of fatty tissue

They might displace other organs or tissue.

Ultrasound

Usually appears as multilobulated well defined-defined mass sometimes with hyperechoic foci ³.

CT

CT usually shows a fat tissue density mass with thick or nodular enhancing septae. Calcifications might be rarely found ⁴.   

MRI

On MRI atypical lipomatous tumours/well differentiated-differentiated liposarcomas will display a mass of fat isointense signal in all sequences. In addition, atypical lipomatous tumours/well differentiated-differentiated liposarcomas will show thick septae or nodular non-lipomatous areas with contrast enhancement ^3-5.

• T1: hyperintense
• T2: hyperintense possibly with prominent high signal foci
• T2FS/PDFS: hypointense
• T1 C+ (Gd): moderate to markedenhancement of septae

Radiology report

The radiological report should include a description of the following ⁵:

• form, location and size
• tumour margins
• thick septae and septal enhancement
• amount of non-adipose tissue
• distance from the muscular fascia

Treatment and prognosis

The management of choice in atypical lipomatous tumours/well differentiated-differentiated liposarcomas is resection, which is curative if complete ¹. The tumours are not sensitive to radiotherapy or chemotherapy ². Tumour prognosis is therefore mostly dependent on how amenable the location is to surgical excision. The tumour does not cause distant metastasis, unless there is dedifferentiation. Therefore tumours of the mediastinum retroperitoneum or spermatic cord have a worse prognosis, due to their location they are more prone to local recurrence ^1,2.

Differential diagnosis

Conditions or tumours which can mimic the presentation and/or the appearance of atypical lipomatous tumours/well differentiated-differentiated liposarcomas include ^1-5:

• lipoma e.g. intermuscular/intramuscular lipoma
• lipomatosis
• myxoid liposarcoma

  pleomomyxoid

• pleomorphic liposarcoma
• dedifferentiated liposarcoma
• myolipoma
• angiomyolipoma

  fibrolipoma

• fibrolipomatosis
• spindle cell/pleomorphic lipoma
• hibernoma
• fat necrosis

See also

• lipoma vs well-differentiated liposarcoma
• liposarcoma