대한근골격영상의학회

Title : Case 524

Age / Sex : 56 / M Chief complaint: Growing soft tissue mass for 10 years What is your impression? Two weeks later, you can see the final diagnosis with a brief discussion of this case. (Please submit only one answer) Courtesy: Chang Ho Kang, Korea University Anam Hospital Diagnosis: Angioleiomyoma Discussion Findings: Radiography) Soft tissue mass at 1^st web space of right hand. Sonography) Well-defined, heterogeneously hypoechoic mass with multiple anechoic foci within the mass. MRI) T1-weighted MR images showed isointense signal intensities compared with the skeletal muscles. T2-weighted MR images showed mixed areas of hyper- and isointense to adjacent muscles. Hyperintense areas within the mass showed strong enhancement, whereas isointense areas on T2-weighted MR images did not show enhancement after the IV administration of contrast media. Signal voids representing vascular flow are present within the mass. Differential Diagnosis: Soft tissue sarcomas with hypervascularity, giant cell tumor, neurogenic tumor Diagnosis: Angioleiomyoma Discussion: Angioleiomyoma is a benign smooth muscle tumor that originates in the tunica media of the veins. It can occur anywhere in the body but is most often seen in the extremities, particularly the lower leg. It has a predilection of middle-aged women. It can be located in the dermis, the subcutaneous fat, or the superficial fasciae of the extremities. Approximately 89% occur in the extremities with 67% occurring in lower extremity and they are typically <2 cm in size. The most common complaint is pain that is often paroxysmal and initiated by the slightest touch or exposure of the tumor to environmental stimuli. The development of multiple lesions is thought to be hereditary and transmitted as an autosomal dominant trait. Treatment usually consists of marginal excision of the mass. Based on the presence of tortuous vascular channels surrounded by smooth muscle bundles and areas of myxoid change, angioleiomyoma should be considered a possible diagnosis when a well-demarcated subcutaneous mass of isointense signal on T1-weighted images, heterogeneous high signal intensity on T2-weighted images with homogeneous strong enhancement, and an adjacent or intralesional tortuous vascular structure is seen in the extremities. References: Hye jin Yoo et al., Angioleiomyoma in Soft Tissue of Extremities: MRI Findings, AJR 2009; 192:W291–W294 Ashley D Willoughby et al., Angioleiomyoma in a Rare Location: A Case Report, Hawaii Journal of Medicine and Public Health, 2013 Feb; 72(2): 45–48 Ramesh P et al., Angioleiomyoma: a clinical, pathological and radiological review, International journal of clinical practice, 2004 Jun; 58(6):587-91

Title : Case 524

Age / Sex : 56 / M

Chief complaint: Growing soft tissue mass for 10 years

What is your impression?

Two weeks later, you can see the final diagnosis with a brief discussion of this case. (Please submit only one answer)

Courtesy: Chang Ho Kang, Korea University Anam Hospital

Diagnosis:

Angioleiomyoma

Discussion

Findings:

Radiography) Soft tissue mass at 1^st web space of right hand.

Sonography) Well-defined, heterogeneously hypoechoic mass with multiple anechoic foci within the mass.

MRI) T1-weighted MR images showed isointense signal intensities compared with the skeletal muscles. T2-weighted MR images showed mixed areas of hyper- and isointense to adjacent muscles. Hyperintense areas within the mass showed strong enhancement, whereas isointense areas on T2-weighted MR images did not show enhancement after the IV administration of contrast media. Signal voids representing vascular flow are present within the mass.

Differential Diagnosis:

Soft tissue sarcomas with hypervascularity, giant cell tumor, neurogenic tumor

Diagnosis: Angioleiomyoma

Discussion:

Angioleiomyoma is a benign smooth muscle tumor that originates in the tunica media of the veins. It can occur anywhere in the body but is most often seen in the extremities, particularly the lower leg. It has a predilection of middle-aged women. It can be located in the dermis, the subcutaneous fat, or the superficial fasciae of the extremities.

Approximately 89% occur in the extremities with 67% occurring in lower extremity and they are typically <2 cm in size. The most common complaint is pain that is often paroxysmal and initiated by the slightest touch or exposure of the tumor to environmental stimuli. The development of multiple lesions is thought to be hereditary and transmitted as an autosomal dominant trait. Treatment usually consists of marginal excision of the mass.

Based on the presence of tortuous vascular channels surrounded by smooth muscle bundles and areas of myxoid change, angioleiomyoma should be considered a possible diagnosis when a well-demarcated subcutaneous mass of isointense signal on T1-weighted images, heterogeneous high signal intensity on T2-weighted images with homogeneous strong enhancement, and an adjacent or intralesional tortuous vascular structure is seen in the extremities.

References:

Hye jin Yoo et al., Angioleiomyoma in Soft Tissue of Extremities: MRI Findings, AJR 2009; 192:W291–W294

Ashley D Willoughby et al., Angioleiomyoma in a Rare Location: A Case Report, Hawaii Journal of Medicine and Public Health, 2013 Feb; 72(2): 45–48

Ramesh P et al., Angioleiomyoma: a clinical, pathological and radiological review, International journal of clinical practice, 2004 Jun; 58(6):587-91