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Leiomyosarcoma of the renal vein: analysis of outcome and prognostic factors in the world case series of 67 patients


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Flow chart of identification, screening, eligibility and inclusion of studies.
Flow chart of identification, screening, eligibility and inclusion of studies.

Enhanced computed tomography showing retroperitoneal tumour, interposed between the aorta and the left kidney, axial (A) and coronal plane (B). Separately removed satellite node in coronal plane, arrow (C).
Enhanced computed tomography showing retroperitoneal tumour, interposed between the aorta and the left kidney, axial (A) and coronal plane (B). Separately removed satellite node in coronal plane, arrow (C).

(A) A gross specimen of renal vein leiomyosarcoma. The tumour is well-circumscribed, is lying in the renal hilum, without infiltration of the renal parencyma. (B) Hematoxylin & Eosin stain section. Showing the vascular lumen (L) and the tumour (TU) growing from the wall of the renal vein (WV). Immunohistochemical stains for SMA (C) and desmin (D) showing strong positivity.
(A) A gross specimen of renal vein leiomyosarcoma. The tumour is well-circumscribed, is lying in the renal hilum, without infiltration of the renal parencyma. (B) Hematoxylin & Eosin stain section. Showing the vascular lumen (L) and the tumour (TU) growing from the wall of the renal vein (WV). Immunohistochemical stains for SMA (C) and desmin (D) showing strong positivity.

Clinical presentation of leiomyosarcoma of the renal vein cases.
Clinical presentation of leiomyosarcoma of the renal vein cases.

Age distribution of leiomyosarcoma of the renal vein patients.
Age distribution of leiomyosarcoma of the renal vein patients.

Kaplan-Meier curve of overall freesurvival.
Kaplan-Meier curve of overall freesurvival.

Kaplan-Meier curve of local recurrence free survival.
Kaplan-Meier curve of local recurrence free survival.

Kaplan-Meier curve of distant metastases free survival.
Kaplan-Meier curve of distant metastases free survival.

Univariate analysis of overall, local recurrence free and distant metastases free survival (log-rank)

CharacteristicSubgroupOVERALL SURVIVALLOCAL RECURRENCE FREE SURVIVAL
Alive (n = 39)Dead (n = 17)p-valueNo LR (n = 45)LR (n = 11)p-value
Age≤ 501350.2851530.519
502612308
GenderF32150.8123890.993
M7272
SideL2990.4483170.987
R108144
Weight lossY560.414920.734
N3411369
Palpable massY960.5811240.562
N3011349
OperationNephrectomy35150.5434190.562
Tumorectomy22(nephrectomy or tumorectomy vs. compartment)22
Compartment2020
Intracaval luminal extensionY530.340440.016
N3414417
Tumour size≤ 10 cm23100.4852850.219
> 10 cm113(≤ 10 cm vs. > 10 cm)113(≤ 10 cm vs. > 10 cm)
Unknown5463
Grade1420.265 (1 vs. 2 or 3)600.05 (1 vs 2 or 3)
2 or 3136127
Unknown229274
MarginsNegative1410.014 (neg. vs. pos.)1230.096 (neg. vs. pos.)
Positive1102
Unknown2415336
Mitoses/10hpf<10820.782 (<10 vs. ≥10)820.244 (<10 vs. ≥10)
≥102112
Unknown2914367
Neoadjuvant/adjuvant chemotherapyY830.987740.337
N3114387
Neoadjuvant/adjuvant raditherapyY640.165820.975
N3313379

Patients data and histologic variables, treatment modalities and disease progression

CharacteristicSubgroupValue (n = 67)%
Age at diagnosis (year)Mean56.6
Range27–93
GenderFemale5176.1
Male1623.9
SideLeft4668.7
Right2131.3
Size (cm)Mean8.9
Range3.5–25
Tumour thrombus extensionIVC913.4
Preoperative biopsyHistology913.4
Fine needle aspiration23.0
No biopsy5683.6
Tumour gradeG169.0
G2811.9
G31420.9
Unknown3958.2
Surgical marginsNegative1522.4
Positive34.5
Unknown4973.1
OperationNephrectomy6089.6
Tumorectomy46.0
Compartment resection23.0
No operation11.5
Preoperative treatmentEmbolization34.5
ChT11.5
RT + ChT23.0
Intraoperative treatmentRT11.5
Postoperative treatmentRT710.4
ChT913.4
ChT + RT11.5
Immunotherapy11.5
Disease progressionLR34.5
M2029.9
LR + M1014.9
Total3349.3
Site of disseminationLiver1725.4
Lungs1623.9
Bone811.9
Soft tissue46.0

j.raon-2016-0051.tab.003.w2aab3b8c70b1b7b1ab1b4b5Aa

CharacteristicSubgroupDISTAN1 METASTASES FREE SURVIVAL
No DM (n = 31)DM (n = 25)p-value
Age≤ 50990.805
> 502216
GenderF27200.221
M45
SideL19190.138
R126
Weight lossY290.087
N2916
Palpable massY5100.277
N2615
OperationNephrectomy27230.336
Tumorectomy31(nephrectomy or tumorectomy vs. compartment)
Compartment11
Intracaval luminal extensionY440.284
N2721
Tumour size≤ 10 cm21120.210
> 10 cm77(≤ 10 cm vs. > 10 cm)
Unknown36
Grade1420.131 (1 vs. 2 or 3)
2 or 3910
Unknown1813
MarginsNegative1050.815 (neg. vs. pos.)
Positive11
Unknown2019
Mitoses/10hpf<10910.266 (<10 vs. ≥10)
≥1012
Unknown2122
Neoadjuvant/adjuvant chemotherapyY560.683
N2619
Neoadjuvant/adjuvant radiotherapyY550.087
N2620
eISSN:
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Sprache:
Englisch
Zeitrahmen der Veröffentlichung:
4 Hefte pro Jahr
Fachgebiete der Zeitschrift:
Medizin, Klinische Medizin, Allgemeinmedizin, Innere Medizin, Hämatologie, Onkologie, Radiologie