. Author manuscript; available in PMC: 2023 Jul 1.

Published in final edited form as: Surg Oncol Clin N Am. 2022 May 31;31(3):321–340. doi: 10.1016/j.soc.2022.03.001

Table 4.

Clinico-pathologic features helpful in the distinction of myxofibrosarcoma and undifferentiated pleomorphic sarcoma.

Differential Diagnosis
Myxofibrosarcoma Mostly limbs and limb girdle (lower>upper extremities) Adults (usually 50–70 years), M≥F Multinodular architecture, infiltrative margins Myxoid stroma, pleomorphism, characteristic curvilinear blood vessel, increased cellularity in higher-risk cases Highly complex karyotypes Local/repeated recurrences in 30–50% of cases; metastases in up to 35% of cases	Undifferentiated pleomorphic sarcoma Wide anatomic distribution (extremities>trunk) Mostly adults (50–70 years) Diagnosis of exclusion, i.e., absence of specific morphologic, immunohistochemical or molecular genetic features Pleomorphic morphology with frequent bizarre multinucleated giant cells, often patternless Highly complex karyotypes Local recurrence/distant metastases (limited data)

Differential Diagnosis

Myxofibrosarcoma

Mostly limbs and limb girdle (lower>upper extremities)
Adults (usually 50–70 years), M≥F
Multinodular architecture, infiltrative margins
Myxoid stroma, pleomorphism, characteristic curvilinear blood vessel, increased cellularity in higher-risk cases
Highly complex karyotypes
Local/repeated recurrences in 30–50% of cases; metastases in up to 35% of cases

Undifferentiated pleomorphic sarcoma

Wide anatomic distribution (extremities>trunk)
Mostly adults (50–70 years)
Diagnosis of exclusion, i.e., absence of specific morphologic, immunohistochemical or molecular genetic features
Pleomorphic morphology with frequent bizarre multinucleated giant cells, often patternless
Highly complex karyotypes
Local recurrence/distant metastases (limited data)