Answer for BIR CoW 14 Nov 2021
Leiomyosarcoma of Right External iliac vein
Findings
Dumbbell shaped hetergenously contrast enhancing lesion seen extending from the right side pelvis into the right superficial inguinal region . The lesion is seen arising from the Right external iliac vein . MRI shows areas of restricted diffusion on DW images , low signal intensity on T1-weighted images and mixed signal intensity on T2-weighted images with areas of necrosis seen within.
Discussion
Primary tumors of the peripheral veins are rare lesions, and they are generally malignant. LMS is the most common pathologic variant. Venous LMS is an aggressive tumor with a high rate of early hematogenous metastasis and local recurrence. LMS originating from the external iliac vein are especially rare, Venous LMS has clinical signs that may be confused with those of DVT, thus causing delay in the correct diagnosis and timely intervention. The clinical manifestation of a venous LMS is nonspecific. It primarily depends on the growth pattern, size, site, growth rate of the tumor, and relationship with the surrounding tissues. The growth pattern of venous LMS vary from intraluminal (5%) to extraluminal (62%) to mixed forms (33%). Intraluminal venous LMS presents early with vague symptoms of impaired venous circulation such as thrombus, edema, pain or Budd-Chiari syndrome. However, extraluminal venous LMS accounts for the majority of these tumors, and such symptoms do not occur until the tumor grows to a large size. The laboratory results and serological markers were within normal ranges, and thus, the diagnosis is usually neglected. On contrast-enhanced CT, if the tumor has an intraluminal component, it appears as a heterogeneously enhancing filling defect in the inferior vena cava .For tumors with an extraluminal component, CT-guided core needle biopsy is typically employed to obtain tissue and establish the diagnosis. On MRI,Signal characteristics depend on the degree of necrosis, which appears low intensity on T1-weighted images and high intensity on T2-weighted images .In contrast to bland thrombus, intraluminal leiomyosarcoma tumor thrombus appears iso- to hyperintense on T2-weighted images, enhances on T1-weighted postcontrast images, and expands the lumen.
References:
1]. Roland CL, Boland GM, Demicco EG, et al. Primary vascular leiomyosarcoma: clinical observations and molecular variables. JAMA Surg 2016;151:347–54. [2]. Reix T, Sevestre H, Sevestri-Pietri MA, et al. Primary malignant tumors of the venous system in the lower extremities. Ann Vasc Surg 1998;12:586–96. [3]. Sahu R, Aggarwal R, Kumar P, et al. Primary external iliac vein leiomyosarcoma. Ann Vasc Surg 2019;57:274.e5– e9. [4]. Fukuda W, Taniguchi S, Fukuda I. Leiomyosarcoma of the external iliac vein. Vasc 2012;20:178–80.
Findings
Dumbbell shaped hetergenously contrast enhancing lesion seen extending from the right side pelvis into the right superficial inguinal region . The lesion is seen arising from the Right external iliac vein . MRI shows areas of restricted diffusion on DW images , low signal intensity on T1-weighted images and mixed signal intensity on T2-weighted images with areas of necrosis seen within.
Discussion
Primary tumors of the peripheral veins are rare lesions, and they are generally malignant. LMS is the most common pathologic variant. Venous LMS is an aggressive tumor with a high rate of early hematogenous metastasis and local recurrence. LMS originating from the external iliac vein are especially rare, Venous LMS has clinical signs that may be confused with those of DVT, thus causing delay in the correct diagnosis and timely intervention. The clinical manifestation of a venous LMS is nonspecific. It primarily depends on the growth pattern, size, site, growth rate of the tumor, and relationship with the surrounding tissues. The growth pattern of venous LMS vary from intraluminal (5%) to extraluminal (62%) to mixed forms (33%). Intraluminal venous LMS presents early with vague symptoms of impaired venous circulation such as thrombus, edema, pain or Budd-Chiari syndrome. However, extraluminal venous LMS accounts for the majority of these tumors, and such symptoms do not occur until the tumor grows to a large size. The laboratory results and serological markers were within normal ranges, and thus, the diagnosis is usually neglected. On contrast-enhanced CT, if the tumor has an intraluminal component, it appears as a heterogeneously enhancing filling defect in the inferior vena cava .For tumors with an extraluminal component, CT-guided core needle biopsy is typically employed to obtain tissue and establish the diagnosis. On MRI,Signal characteristics depend on the degree of necrosis, which appears low intensity on T1-weighted images and high intensity on T2-weighted images .In contrast to bland thrombus, intraluminal leiomyosarcoma tumor thrombus appears iso- to hyperintense on T2-weighted images, enhances on T1-weighted postcontrast images, and expands the lumen.
References:
1]. Roland CL, Boland GM, Demicco EG, et al. Primary vascular leiomyosarcoma: clinical observations and molecular variables. JAMA Surg 2016;151:347–54. [2]. Reix T, Sevestre H, Sevestri-Pietri MA, et al. Primary malignant tumors of the venous system in the lower extremities. Ann Vasc Surg 1998;12:586–96. [3]. Sahu R, Aggarwal R, Kumar P, et al. Primary external iliac vein leiomyosarcoma. Ann Vasc Surg 2019;57:274.e5– e9. [4]. Fukuda W, Taniguchi S, Fukuda I. Leiomyosarcoma of the external iliac vein. Vasc 2012;20:178–80.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!