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Primary renal vein leiomyosarcoma: a case report
Cardiovascular Pathology 24 (2015) 332–333
Contents lists available at ScienceDirect
Cardiovascular Pathology
Images in Cardiovascular Pathology
Primary renal vein leiomyosarcoma: a case report Abhijit Chougule a, Amanjit Bal a,⁎, Arup Kumar Mandal b a b
Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, Sector-12, India Department of Urology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, Sector-12, India
a r t i c l e
i n f o
Article history: Received 11 April 2015 Received in revised form 13 May 2015 Accepted 14 May 2015 Keywords: Leiomyosarcoma Renal vein
a b s t r a c t Primary renal vein leiomyosarcoma (LMS) is a rare tumor with only a few cases reported in the literature. Clinical diagnosis of renal vein LMS can be difficult because of nonspecific symptoms and nonpathognomonic radiological features. The primary treatment modality is radical nephrectomy followed by chemotherapy and/or radiotherapy. There is scarcity of literature regarding prognosis because of rarity of tumor; however, tumor size (N 3 cm) determines the risk of local recurrence and distant metastasis. Overall prognosis of renal vein LMS is poor. Here, we describe clinical and histopathological features of a 50-year-old female patient with LMS of right renal vein. © 2015 Elsevier Inc. All rights reserved.
A 50-year-old female presented with complaints of right flank pain of 1-month duration, which was intermittent, radiating to back, and it gradually became constant in nature. Computerized tomography scan showed heterogeneous lobulated mass in the region of right renal vein measuring 6.4×4.3×3.5 cm. The mass was draping the renal vein and indenting the inferior vena cava (IVC). Magnetic resonance imaging showed a lobulated mass extending from renal hilum along right renal vein with luminal narrowing up to the IVC. Color Doppler ultrasonography showed luminal narrowing near the confluence with IVC with absent flow in distal portion. The other laboratory findings were within normal limits. Based on the history and radiological features, en bloc resection of tumor with right nephrectomy was done. The resected specimen had a tumor measuring 6×4×3.5 cm, arising from the right renal vein (Fig. 1A). The tumor was well encapsulated with lobulated outer surface, and the cut surface was firm, gray white in color. The tumor was indenting the renal hilum; however, infiltration into renal parenchyma or perinephric fat was not seen. Microscopic examination showed interlacing fascicles of spindle-shaped cells with elongated blunt-ended nuclei (Fig. 1B), moderate nuclear pleomorphism, coarse chromatin, and moderate cytoplasm (Fig. 1C). The mitotic count was 4–5/10 high power fields, and areas of coagulative necrosis were seen (Fig. 1D). The tumor cells showed diffuse positivity for smooth muscle actin (Fig. 1E) and were negative for S100, desmin, estrogen receptor (ER), and progesterone receptor (PR). The renal vein resection margin was free of tumor. Vascular leiomyosarcoma (LMS) is the commonest malignant vascular tumor which arises from smooth muscles of tunica media. The most Funding: No funding was received from any organization for the preparation of the manuscript. Financial Disclosure: The authors have no funding, financial relationships, or conflicts of interest to disclose. ⁎ Corresponding author at: Department of Histopathology, PGIMER, Chandigarh160012, India. E-mail address: [email protected] (A. Bal). http://dx.doi.org/10.1016/j.carpath.2015.05.002 1054-8807/© 2015 Elsevier Inc. All rights reserved.
common site is IVC, which accounts for around 70% of all vascular LMS [1]. Primary renal vein LMS is rare with around 30 cases reported in the English literature mostly as single cases attesting its exceeding rarity [2]. It occurs predominantly in women, with a peak incidence in fifth and sixth decades of life and commonly involves the left renal vein which may be related to its longer length [2,3]. Preoperative clinical diagnosis can be difficult because of nonspecific symptoms and nonpathognomonic radiological features which mimics retroperitoneal tumors and renal cell carcinoma with vascular invasion [4]. The primary treatment modality is radical nephrectomy followed by chemotherapy and/or radiotherapy. Local recurrence rate of around 40% has been reported with distant metastasis commonly occurring to lungs, liver, bone, skin, and subcutaneous tissue. Little is known about prognostic factors because of lack of large systematic case series; but tumor size (N3 cm) determines the risk of local recurrence and distant metastasis [2]. Accumulated evidence based on review of previously published reports indicate overall poor prognosis [3]. A subset of extrauterine LMS has been shown to express ER and PR, which can be treated with hormonal therapy that might lead to improvement in survival outcome [5]. But in the present case, the tumor cells were negative for hormone receptors. The index patient is free of recurrence or distant metastasis, 15 months postsurgery.
References [1] Butany J, Singh G, Henry J, Leong SW, Nair V, Thangaroopan M, et al. Vascular smooth muscle tumors: 13 cases and a review of the literature. Int Angiol 2006;15:43–50. [2] Aguilar IC, Benavente VA, Pow-Sang MR, Morante CM, Meza L, Destefano V, et al. Leiomyosarcoma of the renal vein: case report and review of the literature. Urol Oncol 2005;23:22–6. [3] Brandes SB, Chelsky MJ, Petersen RO, Greenberg RE. Leiomyosarcoma of the renal vein. J Surg Oncol 1996;63:195–200. [4] Kaushik S, Neifeld JP. Leiomyosarcoma of the renal vein: imaging and surgical reconstruction. AJR Am J Roentgenol 2002;179:276–7. [5] Kelley TW, Borden EC, Goldblum JR. Estrogen and progesterone receptor expression in uterine and extrauterine leiomyosarcomas an immunohistochemical study. Appl Immunohistochem Mol Morphol 2004;12:338–41.
A. Chougule et al. / Cardiovascular Pathology 24 (2015) 332–333
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Fig. 1. (A) Gross photograph showing lobulated tumor arising from renal vein; (B) tumor composed of fascicles of spindle-shaped cells; (C) moderate nuclear atypia with few bizarre nuclei; (D) coagulative tumor cell necrosis; (E) strong diffuse SMA positivity in tumor cells.
Contents lists available at ScienceDirect
Cardiovascular Pathology
Images in Cardiovascular Pathology
Primary renal vein leiomyosarcoma: a case report Abhijit Chougule a, Amanjit Bal a,⁎, Arup Kumar Mandal b a b
Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, Sector-12, India Department of Urology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, Sector-12, India
a r t i c l e
i n f o
Article history: Received 11 April 2015 Received in revised form 13 May 2015 Accepted 14 May 2015 Keywords: Leiomyosarcoma Renal vein
a b s t r a c t Primary renal vein leiomyosarcoma (LMS) is a rare tumor with only a few cases reported in the literature. Clinical diagnosis of renal vein LMS can be difficult because of nonspecific symptoms and nonpathognomonic radiological features. The primary treatment modality is radical nephrectomy followed by chemotherapy and/or radiotherapy. There is scarcity of literature regarding prognosis because of rarity of tumor; however, tumor size (N 3 cm) determines the risk of local recurrence and distant metastasis. Overall prognosis of renal vein LMS is poor. Here, we describe clinical and histopathological features of a 50-year-old female patient with LMS of right renal vein. © 2015 Elsevier Inc. All rights reserved.
A 50-year-old female presented with complaints of right flank pain of 1-month duration, which was intermittent, radiating to back, and it gradually became constant in nature. Computerized tomography scan showed heterogeneous lobulated mass in the region of right renal vein measuring 6.4×4.3×3.5 cm. The mass was draping the renal vein and indenting the inferior vena cava (IVC). Magnetic resonance imaging showed a lobulated mass extending from renal hilum along right renal vein with luminal narrowing up to the IVC. Color Doppler ultrasonography showed luminal narrowing near the confluence with IVC with absent flow in distal portion. The other laboratory findings were within normal limits. Based on the history and radiological features, en bloc resection of tumor with right nephrectomy was done. The resected specimen had a tumor measuring 6×4×3.5 cm, arising from the right renal vein (Fig. 1A). The tumor was well encapsulated with lobulated outer surface, and the cut surface was firm, gray white in color. The tumor was indenting the renal hilum; however, infiltration into renal parenchyma or perinephric fat was not seen. Microscopic examination showed interlacing fascicles of spindle-shaped cells with elongated blunt-ended nuclei (Fig. 1B), moderate nuclear pleomorphism, coarse chromatin, and moderate cytoplasm (Fig. 1C). The mitotic count was 4–5/10 high power fields, and areas of coagulative necrosis were seen (Fig. 1D). The tumor cells showed diffuse positivity for smooth muscle actin (Fig. 1E) and were negative for S100, desmin, estrogen receptor (ER), and progesterone receptor (PR). The renal vein resection margin was free of tumor. Vascular leiomyosarcoma (LMS) is the commonest malignant vascular tumor which arises from smooth muscles of tunica media. The most Funding: No funding was received from any organization for the preparation of the manuscript. Financial Disclosure: The authors have no funding, financial relationships, or conflicts of interest to disclose. ⁎ Corresponding author at: Department of Histopathology, PGIMER, Chandigarh160012, India. E-mail address: [email protected] (A. Bal). http://dx.doi.org/10.1016/j.carpath.2015.05.002 1054-8807/© 2015 Elsevier Inc. All rights reserved.
common site is IVC, which accounts for around 70% of all vascular LMS [1]. Primary renal vein LMS is rare with around 30 cases reported in the English literature mostly as single cases attesting its exceeding rarity [2]. It occurs predominantly in women, with a peak incidence in fifth and sixth decades of life and commonly involves the left renal vein which may be related to its longer length [2,3]. Preoperative clinical diagnosis can be difficult because of nonspecific symptoms and nonpathognomonic radiological features which mimics retroperitoneal tumors and renal cell carcinoma with vascular invasion [4]. The primary treatment modality is radical nephrectomy followed by chemotherapy and/or radiotherapy. Local recurrence rate of around 40% has been reported with distant metastasis commonly occurring to lungs, liver, bone, skin, and subcutaneous tissue. Little is known about prognostic factors because of lack of large systematic case series; but tumor size (N3 cm) determines the risk of local recurrence and distant metastasis [2]. Accumulated evidence based on review of previously published reports indicate overall poor prognosis [3]. A subset of extrauterine LMS has been shown to express ER and PR, which can be treated with hormonal therapy that might lead to improvement in survival outcome [5]. But in the present case, the tumor cells were negative for hormone receptors. The index patient is free of recurrence or distant metastasis, 15 months postsurgery.
References [1] Butany J, Singh G, Henry J, Leong SW, Nair V, Thangaroopan M, et al. Vascular smooth muscle tumors: 13 cases and a review of the literature. Int Angiol 2006;15:43–50. [2] Aguilar IC, Benavente VA, Pow-Sang MR, Morante CM, Meza L, Destefano V, et al. Leiomyosarcoma of the renal vein: case report and review of the literature. Urol Oncol 2005;23:22–6. [3] Brandes SB, Chelsky MJ, Petersen RO, Greenberg RE. Leiomyosarcoma of the renal vein. J Surg Oncol 1996;63:195–200. [4] Kaushik S, Neifeld JP. Leiomyosarcoma of the renal vein: imaging and surgical reconstruction. AJR Am J Roentgenol 2002;179:276–7. [5] Kelley TW, Borden EC, Goldblum JR. Estrogen and progesterone receptor expression in uterine and extrauterine leiomyosarcomas an immunohistochemical study. Appl Immunohistochem Mol Morphol 2004;12:338–41.
A. Chougule et al. / Cardiovascular Pathology 24 (2015) 332–333
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Fig. 1. (A) Gross photograph showing lobulated tumor arising from renal vein; (B) tumor composed of fascicles of spindle-shaped cells; (C) moderate nuclear atypia with few bizarre nuclei; (D) coagulative tumor cell necrosis; (E) strong diffuse SMA positivity in tumor cells.