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  • 1 Regional Hospital of High Specialty of Oaxaca, Mexico
  • | 2 Oaxaca Regional High Specialty Hospital, Mexico
  • | 3 Regional Hospital of High Specialty of Oaxaca, Mexico
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Introduction

Renal leiomyosarcoma is a rare entity in the world and its understanding is based on reports and various cases; however, the prognosis is bleak for its malignant potential and an average survival of 18 months.

Clinical case

A 54-year-old woman with a clinical picture of 6 months of pain in the right flank and a tomographic image of a bilateral renal lesion underwent right radical nephrectomy and left conservative surgery. The definitive histopathological study reported right primary renal leiomyosarcoma with left metastasis.

Conclusion

Renal leiomyosarcoma is an entity of low incidence and high mortality; however, our case represents the minority of patients with contralateral kidney metastasis reported in the literature.

Abstract

Introduction

Renal leiomyosarcoma is a rare entity in the world and its understanding is based on reports and various cases; however, the prognosis is bleak for its malignant potential and an average survival of 18 months.

Clinical case

A 54-year-old woman with a clinical picture of 6 months of pain in the right flank and a tomographic image of a bilateral renal lesion underwent right radical nephrectomy and left conservative surgery. The definitive histopathological study reported right primary renal leiomyosarcoma with left metastasis.

Conclusion

Renal leiomyosarcoma is an entity of low incidence and high mortality; however, our case represents the minority of patients with contralateral kidney metastasis reported in the literature.

Introduction

Primary renal leiomyosarcoma is a rare tumor in the adult population, in the western hemisphere, which represents 0.1% [1] of all malignant tumors of the kidney and in the eastern, 0.6%–2.7% [2].

The most common effects appear in women among 50 years old or above. The prognosis is bleak, for example, the average survival is 18 months, with a survival rate of 29%–36% at 5 years. Metastases occur most frequently in specific zones, for example, the liver, bones, and lungs [3].

This is one of the few cases reported in the literature with metastatic activity related to the contralateral kidney. The focus is on the clinical picture, diagnostic imaging, immunohistochemical characteristics, surgical treatment, and follow-up at 6 months [4].

Clinical Case

A 59-year-old woman with oppressive pain in the right flank of 6 months of evolution, who came to our unit with abdominal tomography. Initially, the diagnosis of bilateral renal angiomyolipoma was concluded by the tomography. The findings indicate a 10-cm right kidney tumor with heterogeneous and irregular borders around it. A left renal tumor was also observed in the upper pole and a 1-cm paracaval lymph node, without evidence of other lesions (Fig. 1).

Fig. 1.
Fig. 1.

Tomographic findings of the right primary tumor and contralateral metastasis

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

Based on the tomographic findings, she suffered midline laparotomy and a right radical nephrectomy plus retroperitoneal lymphadenectomy was performed on her. A sample was taken and sent to a transoperative study to confirm the tumor etiology. For this reason, a left partial nephrectomy was performed and also a transoperative study that revealed negative surgical margins (Figs 2 and 3). The patient had a good evolution and was discharged 5 days after the surgery.

Fig. 2.
Fig. 2.

Surgical sample of the primary tumor

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

Fig. 3.
Fig. 3.

Product of the contralateral metastasectomy of the kidney

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

The definitive histopathological study of the right and the left tumors reported cell necrosis, capsule and renal fat infiltrations, and metastasis in five lymph nodes. The immunohistochemical analysis was positive for actin, vimentin, calponin, and desmin and negative for cytokeratin AE2/AE3, CD68, BCL2, and WT1 (Figs 47). With these findings, the diagnosis of high-grade primary renal leiomyosarcoma with metastasis to the left kidney was concluded.

Fig. 4.
Fig. 4.

Positive histological sections at S100

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

Fig. 5.
Fig. 5.

Positive histological sections to actin

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

Fig. 6.
Fig. 6.

Positive histological sections to vimentin

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

Fig. 7.
Fig. 7.

Positive histological sections to calponin

Citation: Interventional Medicine and Applied Science IMAS 10, 2; 10.1556/1646.10.2018.12

Discussion

Histological types of renal sarcoma include leiomyosarcoma, liposarcoma, fibrosarcoma, rhabdomyosarcoma, and malignant fibrous histiocytoma. Leiomyosarcoma is the most common type, representing 50%; however, it is still a rare neoplasm originating in the intrarenal blood vessels or smooth muscle fibers of the renal pelvis [5]. The cause of the predominance in women is unknown; however, some studies suggest association with the X chromosome [6].

The symptoms are indistinct with respect to the carcinoma; however, in the literature, it is reported as the incidental finding of abdominal tumor, abdominal pain, lumbar pain, and hematuria and in isolated cases as fever [7]. In our patient, the form of presentation was an abdominal tumor associated with pain in the right flank, which motivated her to seek medical assistance.

Tumors, such as leiomyosarcoma or leiomyoma, contain a dense group of fusiform muscle cells and a variable amount of fibrous connective tissue, the latter produce a low-intensity sequence in T1, in magnetic resonance [8]. In computed tomography, areas of fibrous connective tissue produce a pattern of low reinforcement in the early phase and a greater reinforcement in the late phase. This form of behavior in imaging studies is characteristic of non-epithelial tumors, but it is not exclusive of these neoplasms [8].

The distinctive histological characteristics are the presence of fusiform cells with abundant necrosis, nuclear polymorphism, and high mitotic activity [9]. The immunohistochemical analysis expresses positivity to actin, calponin, desmin, and h-caldesmin [10]. The differential diagnosis of this neoplasm includes renal carcinoma with sarcomatoid differentiation, leiomyoma, and classic carcinoma, which are distinguishable only in the histopathological study.

The treatment of choice is a total radical nephrectomy [11]. Deyrup et al. [12] reported the nephron-sparing surgery in a patient with a 4-cm tumor as an option. In our patient, we chose for a conservative surgery in the left kidney because it is a bilateral tumor, with a negative intraoperative pathological margin of 1 and 0.5 cm. Considering the general aggressiveness of sarcomas and their high capacity for local and distant recurrences, treatment with adjuvant chemotherapy through mesna, adriamycin, ifosfamide, and dacarbazine and radiation therapy at a dose of 44 Gy to the renal fossa and adjacent lymphatic area is justified [13]. Especially, when having any of the following high risk factors, such as higher than 5-cm tumor, high histologic grade, retroperitoneum, and presence of necrosis. In the case of neoadjuvant therapy with chemotherapy or radiotherapy, more studies are needed to demonstrate its effectiveness in 5-year survival.

Conclusions

Renal leiomyosarcoma occurs in less than 0.1%, its clinical evolution is similar to other renal neoplasms, although its tomographic image may suggest the diagnosis in some cases. Its biological behavior is aggressive with an overall survival of 27%–36% at 5 years. Our case is one of the few reported with contralateral renal metastasis, treated with radical surgery for the primary tumor and conservative surgery in the metastasis, with survival until this time of 6 months.

Conflict of interest

The authors declare no conflict of interest.

References

  • 1.

    Dhawan S , Chopra P , Dhawan S : Primary renal leiomyosarcoma: A diagnostic challenge. Urol Ann 4, 4850 (2012)

  • 2.

    Ozturk H : High-grade primary renal leiomyosarcoma. Int Braz J Urol 41, 304311 (2015)

  • 3.

    Niceta P , Lavengood RW Jr , Fernandes M , Tozzo PJ : Leiomyosarcoma of kidney. Review of the literature. Urology 3, 270277 (1974)

  • 4.

    Ellouze S , Abid N , Kossentini M , Gouiaa N , Charfi S , Mhiri N , Boudawara T : Leiomyosarcoma of the kidney. Clin Genitourin Cancer 9, 6869 (2011)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5.

    Miller JS , Zhou M , Brimo F , Guo CC , Epstein JI : Primary leiomyosarcoma of the kidney: A clinicopathologic study of 27 cases. Am J Surg Pathol 4, 238242 (2010)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 6.

    Brown CJ , Greally JM : A stain upon the silence: Genes escaping X inactivation. Trends Genet 19, 432438 (2003)

  • 7.

    Kwon YS , Salmasi A , Han CS : Renal leiomyosarcoma: Case report and review of the literature. World J Nephrol Urol 4, 213217 (2015)

  • 8.

    Ochiai K , Onitsuka H , Honda H , Kawamoto K , Uozumi J , Kumazawa J , Masuda K : Leiomyosarcoma of the kidney: CT and MR appearance. J Comput Assist Tomogr 17, 656658 (1993)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 9.

    Venkatesh K , Lamba Saini M , Niveditha SR , Krishnagiri C , Babu S : Primary leiomyosarcoma of the kidney. Patholog Res Int 2010, 562398 (2010)

    • Search Google Scholar
    • Export Citation
  • 10.

    Pong YH , Tsai VFS , Wang SM : Primary leiomyosarcoma of the kidney. Formos J Surg 45, 124126 (2012)

  • 11.

    Hemmi A , Komiyama A , Ohno S , Fujii Y , Katoh R , Yokoyama A , Kawaoi A : Poorly differentiated desmin-negative and vimentin-positive leiomyosarcoma of the stomach examined by the immunohistochemical and quick-freezing and deep-etching methods. Virchows Arch 432, 377383 (1998)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 12.

    Deyrup AT , Montgomery E , Fisher C : Leiomyosarcoma of the kidney: A clinicopathologic study. Am J Surg Pathol 28, 178182 (2004)

  • 13.

    Sharma D , Pradhan S , Aryya NC , Shukla VK : Leiomyosarcoma of kidney: A case report with long term result after radiotherapy and chemotherapy. Int Urol Nephrol 39, 397400 (2007)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 1.

    Dhawan S , Chopra P , Dhawan S : Primary renal leiomyosarcoma: A diagnostic challenge. Urol Ann 4, 4850 (2012)

  • 2.

    Ozturk H : High-grade primary renal leiomyosarcoma. Int Braz J Urol 41, 304311 (2015)

  • 3.

    Niceta P , Lavengood RW Jr , Fernandes M , Tozzo PJ : Leiomyosarcoma of kidney. Review of the literature. Urology 3, 270277 (1974)

  • 4.

    Ellouze S , Abid N , Kossentini M , Gouiaa N , Charfi S , Mhiri N , Boudawara T : Leiomyosarcoma of the kidney. Clin Genitourin Cancer 9, 6869 (2011)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5.

    Miller JS , Zhou M , Brimo F , Guo CC , Epstein JI : Primary leiomyosarcoma of the kidney: A clinicopathologic study of 27 cases. Am J Surg Pathol 4, 238242 (2010)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 6.

    Brown CJ , Greally JM : A stain upon the silence: Genes escaping X inactivation. Trends Genet 19, 432438 (2003)

  • 7.

    Kwon YS , Salmasi A , Han CS : Renal leiomyosarcoma: Case report and review of the literature. World J Nephrol Urol 4, 213217 (2015)

  • 8.

    Ochiai K , Onitsuka H , Honda H , Kawamoto K , Uozumi J , Kumazawa J , Masuda K : Leiomyosarcoma of the kidney: CT and MR appearance. J Comput Assist Tomogr 17, 656658 (1993)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 9.

    Venkatesh K , Lamba Saini M , Niveditha SR , Krishnagiri C , Babu S : Primary leiomyosarcoma of the kidney. Patholog Res Int 2010, 562398 (2010)

    • Search Google Scholar
    • Export Citation
  • 10.

    Pong YH , Tsai VFS , Wang SM : Primary leiomyosarcoma of the kidney. Formos J Surg 45, 124126 (2012)

  • 11.

    Hemmi A , Komiyama A , Ohno S , Fujii Y , Katoh R , Yokoyama A , Kawaoi A : Poorly differentiated desmin-negative and vimentin-positive leiomyosarcoma of the stomach examined by the immunohistochemical and quick-freezing and deep-etching methods. Virchows Arch 432, 377383 (1998)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 12.

    Deyrup AT , Montgomery E , Fisher C : Leiomyosarcoma of the kidney: A clinicopathologic study. Am J Surg Pathol 28, 178182 (2004)

  • 13.

    Sharma D , Pradhan S , Aryya NC , Shukla VK : Leiomyosarcoma of kidney: A case report with long term result after radiotherapy and chemotherapy. Int Urol Nephrol 39, 397400 (2007)

    • Crossref
    • Search Google Scholar
    • Export Citation

2019  
Scimago
H-index
11
Scimago
Journal Rank
0,220
Scimago
Quartile Score
Medicine (miscellaneous) Q3
Scopus
Cite Score
155/133=1,2
Scopus
Cite Score Rank
General Medicine 199/529 (Q2)
Scopus
SNIP
0,343
Scopus
Cites
206
Scopus
Documents
23

 

Interventional Medicine and Applied Science
Language English
Size  
Year of
Foundation
2009
Publication
Programme
changed title
Volumes
per Year
 
Issues
per Year
 
Founder Akadémiai Kiadó
Founder's
Address
H-1117 Budapest, Hungary 1516 Budapest, PO Box 245.
Publisher Akadémiai Kiadó
Publisher's
Address
H-1117 Budapest, Hungary 1516 Budapest, PO Box 245.
Responsible
Publisher
Chief Executive Officer, Akadémiai Kiadó
ISSN 2061-1617 (Print)
ISSN 2061-5094 (Online)

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