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Urol Case Rep
Urol Case Rep
Urology Case Reports
2214-4420
Elsevier

S2214-4420(24)00189-X
10.1016/j.eucr.2024.102835
102835
Oncology
A rare case of renal leiomyosarcoma treated with partial nephrectomy
Cherry Amy cherryal@main.erlanger.org
a⁎
Talluri Shriharsha a
Hessler Richard b
Waldorf Benjamin a
a Erlanger Department of Urology, College of Medicine, The University of Tennessee-Chattanooga, Chattanooga, TN, USA
b Erlanger Health System, Department of Pathology, Chattanooga, TN, USA
⁎ Corresponding author. cherryal@main.erlanger.org
28 8 2024
9 2024
28 8 2024
56 10283519 8 2024
27 8 2024
© 2024 Published by Elsevier Inc.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Renal Leiomyosarcoma (LMS) is an exceptionally rare and aggressive form of RCC (renal cell carcinoma), comprising of 0.5–1% of all adult RCC. This disease constitutes a 5-year 25 % overall survival. We present a rare case of renal leiomyosarcoma in a 58 year-old Caucasian female with final pathologic staging of pT2 who underwent robotic-assisted right partial nephrectomy. Despite negative margins, given her aggressive histology, adjunctive treatment is discussed.
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pmc1 Introduction

Primary renal leiomyosarcomas are an exceedingly rare and aggressive form of renal sarcomas, constituting 0.5–1% of all renal tumors in adults.1 As such, treatment recommendations and information pertaining to clinical outcomes are limited, but diagnosis is associated with overall poor prognosis.1 The median overall survival is estimated between 9 and 24 months.2,3 Based on a SEER database analysis, 5-year overall survival for LMS is 25 %.4 The recommended treatment for primary sarcomas of the kidney is radical nephrectomy, which has demonstrated curable efficacy in roughly one-third of cases.2 However, given the low prevalence of leiomyosarcoma in the general populace, no definitive data exists to define the role of neoadjuvant vs adjuvant chemotherapy or radiation therapy.3 Here, we present a case of renal leiomyosarcoma in a 58-year-old with final pathological staging of pT2.

2 Case presentation

A 58-year-old Caucasian woman with a history notable for asthma, tobacco use, gastroesophageal reflux, chronic renal disease, nephrolithiasis presented to left-sided flank pain. CT abdomen and pelvis without contrast obtained on the day of initial presentation revealed a soft tissue density mass projecting anteriorly from the right kidney measuring 6.2 × 4.5 × 3.9 cm as well as left ureteral stones (Fig. 1) The patient was treated with laser lithotripsy for left-sided stone burden. The patient elected for a robotic-assisted right partial nephrectomy 3 weeks following initial presentation. Postoperative course was uneventful and the patient discharged home on postoperative day one. CT revealed no evidence of metastatic disease. Pathologic review of the renal mass revealed leiomyosarcoma approximately 6.3 cm in largest dimension with negative margins (Fig. 2), with pathologic stage pT2. Histological evaluation revealed a mitotic rate of 15 per high-powered field (HPF) (Fig. 3). Immunohistochemistry positive for desmin, calponin, and smooth muscle actin and negative for MelanA, HMB45, and CD34.Fig. 1 CT abdomen and pelvis without contrast showing a 6.2 × 4.5 × 3.9 cm lesion in upper pole of right kidney.

Fig. 1

Fig. 2 En bloc and cross-sectional gross pathology of mass measuring 6.3 × 4.5 × 3.9 cm with well-demarcated borders and myxoid-appearing changes with 40 % hemorrhagic changes.

Fig. 2

Fig. 3 Histology revealed spindle-shaped cells organized in intersecting fascicles with 15 mitoses per HPF.

Fig. 3

3 Discussion

This case serves as an addition to the sparse literature regarding renal leiomyosarcoma. This patient fits many of the characteristics of the typical patient population with renal leiomyosarcoma: female sex and presentation between 4th and 6th decade of life.3,5, 6, 7

Renal leiomyosarcoma is thought to arise from smooth muscle of blood vessels within kidney or renal pelvis.3,6 There is no consensus for sidedness of tumor.3,7 There are several chromosomal changes believed to be responsible for development of LMS which pertain to tumor suppressor genes, specifically RB1 and PTEN: losses in chromosome 10q11 to 21.2 and 13q14.3 to q21.1 as well as gains at 17p11 to p12.8 The role of estrogen in development of LMS has not been fully evaluated.7,8 LMS is associated with high risk of local recurrence and disseminates via hematogenous spread with high risk of distant metastasis to lung, liver, and bone.3,6,7

There are no distinguishing factors of presenting illness to differentiate LMS from other renal malignancies. LMS typically presents with hematuria, abdominal pain, and/or abdominal mass.3 There are also no significant radiological findings specific to LMS as compared to other primary renal carcinomas; one study recommends CT-guided core needle biopsy for histopathological diagnosis to guide appropriate treatment regimen, as there is low risk of micrometastasis and/or seeding.3,7 Radiologically-defined borders can distinguish LMS from retroperitoneal-derived LMS.3

Given variable prognosis and treatment outcomes, it is crucial to differentiate renal leiomyosarcoma from similar pathology.2 There are several characteristic histopathological markers that distinguish LMS from other carcinomas: alternating fascicles of eosinophilic spindle cells with blunt-edged, non-tapering, monomorphic nuclei.6 LMS is also associated with either focal or extensive cellular pleomorphism and a cellular necrosis rate of roughly 21 %, which can distinguish LMS from benign leiomyoma.1,6

There are several epithelioid immunohistochemical markers which distinguish LMS from similar histopathology: LMS expresses cytokeratin, EMA, desmin, calponin, h-caldesmon, vimentin, and do not express actin, S100 protein, HMB-45, CD117.1,3,5, 6, 7, 8, 9 Sarcomatoid carcinomas, which may exhibit a malignant epithelial component, express cytokeratin, actin, and EMA, but do not typically express desmin.1,3 LMS can is differentiated from angiomyolipoma (AML) due to AML's typical presentation with organized smooth muscle fascicles, adipose cells, and presence of blood vessels, as well as fact that AML is typically HMB-45-positive.1,3

LMS is associated with poor prognosis in part due to high risk of local recurrence; as such, radical nephrectomy is the treatment of choice.3,7 Outcomes are largely dependent on tumor grade, with grade 1 disease associated with significantly improved survivability as compared to grade 3.1,7 Specifically, grade of tumor as well as extension into the inferior vena cava are associated with rate of local recurrence, and risk of metastasis.3,7 One study following 9 patients after nephrectomy for LMS found one patient with grade 1 disease alive at 5 years with no evidence of disease recurrence whereas all 3 patients with grade 3 disease had died of disease.5 Another study found that 90 % of patients who underwent radical nephrectomy for LMS developed distant metastatic disease and that 75 % of patients eventually died from LMS.6 One study by Novak et al. found overall survival of 76 % at 24 months, but with 49.3 % of subjects with local recurrence and/or dissemination of disease after treatment.7 According to analysis using SEER criteria, 5-year survivability is 25 %.4 90 % of LMS is found to be moderate-to high-grade.8 Tumor grade and margin status are independent prognosticators of overall survival and disease-free survival rate.7 One study by Miller et al. found 55 % of cases of LMS with extracapsular extension and 26 % of cases with lymphovascular invasion at time of nephrectomy.6

There is limited data on the role of partial nephrectomy in treatment of leiomyosarcoma, but it may be considered an appropriate treatment for renal masses less than 5 cm.10 A case reported by Cocuzza et al. identified a 52 year old female who survived 2 years following partial nephrectomy for LMS of 1.7cm, lending to the idea that nephron-sparing treatment should be considered on per-case basis.10 One study by Ozturk had one of 7 patients with LMS treated with partial nephrectomy due to size of tumor at 5 cm with post-treatment survival of 44 months, higher than expected.9

Due to low prevalence, there is currently no consensus on the role of neoadjuvant vs adjuvant chemoradiation in the treatment of LMS.3 However, several studies have promoted the use of adjacent chemotherapy if positive margins are identified with pathologic review and/or unresectable or metastatic disease burden.3 Specifically, anthracycline is the preferred chemotherapeutic agent; ifosfamide is second line due to toxicity profile.3,11

4 Conclusion

We present an exceptionally rare care of renal leiomyosarcoma in a 58-year old Caucasian female treated with partial nephrectomy.

CRediT authorship contribution statement

Amy Cherry: Writing – original draft, Writing – review & editing. Shriharsha Talluri: Writing – review & editing. Richard Hessler: Project administration. Benjamin Waldorf: Conceptualization, Project administration.

Declaration of competing interest

None.
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References

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