|Year : 2022 | Volume
| Issue : 2 | Page : 152-154
A rare case of primary renal leiomyosarcoma with peritoneal sarcomatosis – A case report
Maruthi D Karri, Kaumudi Konkay, Vijaya S Mandava, Geetha V Panchakarla, Padmavathi D Chaganti
Department of Pathology, Guntur Medical College, Guntur, Andhra Pradesh, India
|Date of Submission||20-Nov-2021|
|Date of Acceptance||06-Feb-2022|
|Date of Web Publication||3-Aug-2022|
Dr. Kaumudi Konkay
Assistant Professor of Pathology, Guntur Medical College, Guntur, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Primary renal leiomyosarcoma is an extremely rare renal malignancy. This case report presents clinicopathological and immunophenotypic features of a case of primary renal leiomyosarcoma with peritoneal sarcomatosis.
Keywords: Immunohistochemistry, peritoneal metastasis, renal sarcoma
|How to cite this article:|
Karri MD, Konkay K, Mandava VS, Panchakarla GV, Chaganti PD. A rare case of primary renal leiomyosarcoma with peritoneal sarcomatosis – A case report. J NTR Univ Health Sci 2022;11:152-4
|How to cite this URL:|
Karri MD, Konkay K, Mandava VS, Panchakarla GV, Chaganti PD. A rare case of primary renal leiomyosarcoma with peritoneal sarcomatosis – A case report. J NTR Univ Health Sci [serial online] 2022 [cited 2022 Dec 10];11:152-4. Available from: https://www.jdrntruhs.org/text.asp?2022/11/2/152/353214
| Introduction|| |
Renal sarcomas are rare but highly aggressive neoplasms, accounting for 1 to 2% of all malignant renal tumors. Primary renal leiomyosarcoma is an extremely rare entity accounting for 0.12% of renal malignancies.
| Case Summary|| |
A 48-year-old female presented with intermittent flank pain in the past six months. The pain was gradual in onset, intermittent type, and was unrelated to diet. There were no associated symptoms like nausea, vomiting, diarrhea, and tarry stool. On examination, all her vitals were stable, and there were no peritoneal signs. Physical examination revealed a firm nodular, mildly tender swelling in the hypochondriac region extending up to the lumbar region. The complete blood picture revealed microcytic hypochromic anemia with neutrophilic leukocytosis. The renal function tests were normal-blood urea levels were 23 mg/dl, serum creatinine level was 0.9 mg/dl. Liver function tests and serum electrolytes were within normal limits. Ultrasound of the abdomen revealed a mass lesion of the right kidney. CT Abdomen was reported as a mass lesion in the mid pole of the right kidney (5.6 cm × 5.2 cm). CECT of the whole abdomen and CT of the kidney urinary bladder revealed 57 × 47 × 43 mm heterogeneously enhancing exophytic mass arising from the upper pole parenchyma [Figure 1]a and [Figure 1]b. There was no evidence of calcifications. The diagnosis was likely right renal neoplastic mass. Based on these findings, right radical nephrectomy was performed. Intraoperatively, a mass of size 7 × 5 cm was noted arising from the mid-upper pole, which was found to be adherent to the transverse colon, ascending colon, liver, and diaphragm. The excised specimen was sent for histopathological examination. The patient's hospital stay was uneventful, and she was discharged on the seventh postoperative day. The specimen measured 13 × 9 × 5 cm, and the cut section showed a solid, grey-white to yellowish tumor in the perinephric fat [Figure 2]a and peritoneal seedings [Figure 2]b were also solid grey-white on cut section. The specimen was processed routinely, and hematoxylin and eosin-stained sections were prepared. The sections studied showed a sarcomatoid tumor composed of round to oval to spindle-shaped cells arranged in fascicular pattern infiltrating renal parenchyma [Figure 2]c. Individual cells showed scant cytoplasm and cellular pleomorphism with increased mitotic activity of 15/10 HPF. Tumor giant cells were also observed [Figure 2]d. Adjacent renal parenchyma showed chronic pyelonephritis changes.
|Figure 1: (a and b) CECT of the whole abdomen and CT-KUB revealed 57 × 47 × 43 mm enhancing heterogeneously exophytic mass is seen arising from the upper pole parenchyma|
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|Figure 2: (a) Radical Nephrectomy specimen cut section shows a solid, grey-white to yellowish tumor in the perinephric fat (b) Peritoneal seedings (c) Hematoxylin and eosin (H and E) 40× tumor composed of spindle cells in fascicles infiltrating adjacent renal parenchyma (d) H and E 100× tumor giant cells and necrosis (e) immunohistochemistry (IHC) with smooth muscle actin (SMA), tumor cells show cytoplasmic positivity (f) IHC with desmin, tumor cells show cytoplasmic positivity|
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Peritoneal nodules showed evidence of secondary deposits. Immunohistochemistry profile showed tumor cells to be strongly positive for smooth muscle actin [Figure 2]e, desmin [Figure 2]f, and negative for cytokeratin. So, the final diagnosis was given as renal leiomyosarcoma with peritoneal sarcomatosis.
| Discussion|| |
Leiomyosarcoma is a malignant tumor that arises from the smooth muscle components of the soft tissue and commonly occurs in the retroperitoneum. It forms a significant percentage of retroperitoneal sarcomas and is the predominant sarcoma arising from larger blood vessels, like inferior vena cava and large veins of the lower extremity. Leiomyosarcomas involving non-retroperitoneal soft tissue sites are found in the lower extremity but may develop elsewhere in intramuscular and subcutaneous locations in equal proportions. Primary renal leiomyosarcoma is rare and can occur in the renal capsule, pelvis, parenchyma, and renal vessels. There is a predominance in females, with most cases diagnosed in the fourth to sixth decades of life. Renal leiomyosarcoma must be differentiated from sarcomatoid renal cell carcinoma on histological examination. Immunohistochemistry with SMA and Desmin helps in differentiating. The positivity for two of these markers is more supportive of leiomyosarcoma than positivity for one alone. Cytokeratin, S100, and HMB 45 are negative in leiomyosarcoma Leiomyosarcoma can metastasize to distant sites by the hematogenous route. There are case reports of leiomyosarcoma of the retroperitoneum and uterus metastasizing to unusual sites like skeletal muscle and pancreas. There are documented cases of uterine leiomyosarcoma metastasizing to the peritoneum. The term “peritoneal sarcomatosis” can be applied to such an entity. Perhaps our case is the first documented case of renal leiomyosarcoma with peritoneal metastasis. As high as 40 percent of leiomyosarcoma cases can present with peritoneal metastasis at primary presentation, and liver and lung are the most common sites. The prognosis is poor and depends on the completeness of surgical excision.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Demir A, Yazici CM, Eren F, Türkeri L. Case report: Good prognosis in leiomyosarcoma of the kidney. Int Urol Nephrol 2007;39:7-10.
Srivastava P, Prasad R, Khanna G. Primary leiomyosarcoma of kidney: A rare case report. Muller J Med Sci Res 2015;6:157-9. [Full text]
Angel CA, Gant LL, Parham DM, Rao BN, Douglass EC, Lobe T. Leiomyosarcomas in children: Clinical and pathologic characteristics. Pediatr Surg Int 1992;7:116-20.
Venkatesh K, Lamba Saini M, Niveditha SR, Krishnagiri C, Babu S. Primary leiomyosarcoma of the kidney. Patholog Res Int 2010;2010:652398.
Lal H, Neyaz Z, Kapoor VK, Pottakkat B, Gupta P. Local recurrence and multi-organ metastasis of primary retroperitoneal leiomyosarcoma in unusual locations after surgical resection. J Radiol Case Rep 2011;5:1-8.
Oei TN, Jagannathan JP, Ramaiya N, Ros PR. Peritoneal sarcomatosis versus peritoneal carcinomatosis: Imaging findings at MDCT. AJR Am J Roentgenol 2010;195:W229-35.
Beardo P, José Ledo M, Jose Luis RC. Renal leiomyosarcoma. Rare Tumors 2013;5:e42.
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