Introduction/Background:
Renal sarcoma constitute about 0.8-2.7% of all primary malignant renal tumor [1]. Of all the different types, Renal Leiomyosarcoma is the most common type (50-60%) which originates from the smooth muscle fibers of the renal pelvis, renal capsule or renal vessel [2].
Case Report:
65-year-old smoker male was seen in the clinic with for progressive right upper quadrant and right flank abdominal from the last 4-5 months, worse with eating (Spicy food) and associated with 30 pounds unintentional weight loss in the last 6 months. Patient denied nausea, vomiting, changes in bowel habits, melena, hematochezia and hematuria. There was an ill-defined firmness in the right upper quadrant on examination and laboratory work up was negative. Patient had an abdominal ultrasound as initial work up to rule out gallbladder pathology that showed right lower quadrant mass attached to the right kidney 13.5x11.9x8.9 cm, later confirmed on Computed Tomography (CT) of abdomen that revealed 14.7 x 10.5 cm fungating multilobular mass attached to the inferior pole of right kidney with extracapsular extension and retroperitoneal lymphadenopathy. Further, metastatic work include CT scan of the chest showed multiple small bilateral pulmonary nodules suggestive of metastatic disease. There was an increase in size of renal mass with SUV 10.8 and no bone metastasis was seen during follow up on positron emission tomography scan. CT guided needle core biopsy of renal mass showed spindle cells arranged in fascicles, increased nuclear polymorphisms and mitotic rates which were positive for desmin, smooth muscles myosin/actin immunostains and negative for CD 34, CD117 and HMB-45. Patient had an elective right radical nephrectomy and resection of leiomyosarcoma.
Discussion:
Primary renal leiomyosarcoma is very rare, more common in females age 50-60 and involves right kidney [3]. The clinical presentation is similar to renal cell carcinoma include abdominal pain or mass, and hematuria. The most common metastatic sites include liver, lungs, bone, and soft tissue. CT scan can be helpful in diagnosis but cannot differentiate from renal cell carcinoma. Biopsy with histopathology and immunohistochemistry is required to confirm the diagnosis. Management includes surgical approach, radiation and chemotherapy. Radical Nephrectomy is the treatment of choice. Most favorable prognostic factors includes tumor less than 4 cm, low grade, absence of nodal metastasis, and radical surgical treatment.
Conclusion:
Leiomyosarcoma is a rare aggressive tumor of kidney with higher chances of recurrence and metastasis. Only few case reports have been published so far.
Reference:
1: Vogelzang NJ, Fremgen AM, Guinan PD, et al. Primary renal sarcoma in adults: A natural history and management study by the American Cancer Society, Illinois division. Cancer 1993; 71:804-10. 10.1002/1097-0142(19930201)71:33.0.CO; 2-A
2: Kavantzas N, Pavlopoulos PM, Karaitianos I, et al. Renal leiomyosarcoma: Report of three cases and review of the literature. Arch Ital Urol Androl 1999; 71:307-11
3: J. S. Miller, M. Zhou, F. Brimo, C. C. Guo, and J. I. Epstein, “Primary leiomyosarcoma of the kidney: a clinicopathologic study of 27 cases,” The American Journal of Surgical Pathology , vol. 34, no. 2, pp. 238–242, 2010
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1318 |
| Date | 12 April 2019 |
| Creators | Khalid, Muhammad Faisal, Velilla, Rowena, Jain, Vinay, Qayum, Salman |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Source | Appalachian Student Research Forum |
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