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      Laparoscopic radical nephroureterectomy in the oblique supine lithotomy integrative position for rare renal malignant perivascular epithelioid cell tumor with renal vein cancerous thrombosis: A case report and literature review

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          Rationale:

          Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor that arises from perivascular epithelioid cells and can differentiate into melanocytes and smooth muscle cells. Malignant renal perivascular epithelioid cell tumor is extremely rare. Due to the lack of specific clinical manifestations and imaging features, diagnosing PEComa depends on postoperative pathology and immunohistochemistry. Surgery is the primary treatment for malignant PEComa because the efficacy of radiotherapy and chemotherapy is uncertain. There is still a lack of unified diagnostic criteria and treatment guidelines for renal malignant PEComa, especially with vascular invasion. Hence, the treatment experience depends on a small number of cases reported worldwide.

          Patient concerns:

          A 68-year-old woman was admitted to our hospital due to intermittent hematuria for over 8 months. The color Doppler ultrasound and computed tomography scan revealed a mass in the lower middle part of the left kidney.

          Diagnosis:

          Rare renal malignant perivascular epithelioid cell tumor with renal vein cancerous thrombosis.

          Interventions:

          A laparoscopic radical left nephroureterectomy in the oblique supine lithotomy position was performed.

          Outcomes:

          The operation process went smoothly, and no pulmonary embolism occurred after the operation. The final pathological diagnosis was a renal malignant perivascular epithelioid cell tumor. After a 12-month follow-up, no recurrence or metastasis was found.

          Lessons:

          Renal malignant PEComa is an extremely rare mesenchymal tumor diagnosed mainly based on pathology. Surgery is currently the effective treatment for malignant PEComa. For the surgical treatment of malignant renal PEComa with vascular invasion, laparoscopic radical nephroureterectomy in the oblique supine lithotomy integrative position has many benefits, as exemplified by our current case.

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          Most cited references22

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          Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases.

          Oluwole Fadare (2008)
          The uterus and retroperitoneum have emerged as the most frequently reported anatomic sites of origin of perivascular epithelioid cell tumors (PEComas), a poorly defined neoplasm that is characterized by varying amounts of spindle and epithelioid cells with clear to eosinophilic cytoplasm that display immunoreactivity for melanocytic markers, most frequently HMB-45. Published reports on 41 previously reported uterine PEComas are reviewed in this report. Of these 41 cases, 31 originating in the corpus and for which there was adequate follow-up information (or clinical malignancy) were categorized into 2 groups: (1) a malignant group that was comprised of cases associated with patient death of disease and/or clinical malignancy as evidenced by local and/or distant extension outside of the uterus (n=13, group 1) and (2) a "nonmalignant" group of cases in which neither of the above features were present (n=18, group 2). Groups 1 and 2 did not significantly differ regarding duration of follow-up (25 mo vs. 24.3 mo, respectively, P=0.9) or patient age (45.61 y vs. 43.46 y, respectively, P=0.7). Five of the group 1 patients experienced distant (extra-abdominal) metastases. The group 1 tumors were significantly larger than the group 2 tumors (averages 9.6 cm vs. 4.67 cm respectively, P=0.04); however, there were no size thresholds that, in of themselves, reliably classified 75% or more of the cases in both groups. Coagulative necrosis was highly associated with group 1, being present in 82% of cases as compared with only 11.8% of group 2 cases (P=0.0002). Eighty-eight percent of the group 2 cases had a mitotic rate of 1/10 HPF and/or coagulative necrosis are features that, if present, raise the definite potential for aggressive behavior.
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            Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature

            Henry Armah, Anil V Parwani (2007)
            Background Perivascular epithelioid cell tumor (PEComa), other than angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangioleiomyomatosis (LAM), is a very rare mesenchymal tumor with an unpredictable natural history. The uterus is the most prevalent reported site of involvement of PEComa-not otherwise specified (PEComa-NOS). To the best of our knowledge, about 100 PEComa-NOS have been reported in the English Language medical literature, of which 38 were uterine PEComa-NOS. These reported cases of uterine PEComa-NOS have usually shown clinically benign behavior, but 13 tumors, three of them associated with tuberous sclerosis complex (TSC), exhibited local aggressive behavior and four of them showed distant metastases. Case presentation We report the case of a 59-year-old woman, who presented with renal and pulmonary lesions seven years after the initial diagnosis of uterine leiomyosarcoma. Left nephrectomy and right middle lobe wedge resection were performed. Histological and immunohistochemical analysis of the renal and pulmonary lesions, in addition to retrospective re-evaluation of the previous uterine tumor, led to the final diagnosis of malignant uterine PEComa with late renal and pulmonary metastases. All three lesions had the typical histological appearance of PEComa-NOS showing a biphasic growth pattern with continuous transition between spindle cells and epithelioid cells, often arranged around vascular spaces. Immunohistochemically, the tumor cells of both phenotypes in all three lesions stained for melanocytic (HMB-45 and Melan-A/MART-1) and myoid (desmin, smooth muscle actin, and muscle-specific actin/all muscle actin/HHF-35) markers. Conclusion The findings indicate that despite the small number of reported cases, PEComas-NOS should be considered tumors of uncertain malignant potential, and metastases to other organs might become evident even several years after the primary diagnosis.
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              Perivascular epithelioid cell neoplasms (PEComas): four malignant cases expanding the histopathological spectrum and a description of a unique finding.

              E Latta, David JC Howarth, Danny Ghazarian … (2007)
              Four cases of malignant PEComas were stained with smooth muscle actin, muscle specific actin, desmin, human melanoma black-45, melan-A, microphthalmia transcription factor, S100 and cyclin D1. One case was studied by electron microscopy (EM). Tumour locations were the thigh, elbow, retroperitoneum and bladder in association with a urachal cyst. There were two men and two women; the average age was 51.3 years, and the size ranged from 5.0-23.0 cm. In three cases, 50-95% of the tumour was composed of pleomorphic sarcomatous areas. All cases had at least focal clear-cell areas. One case showed a continuous single layer of perivascular clear cells remote from the tumour, transitioning to invasive nests and to PEComa. EM demonstrated these cells in apposition to and in direct contact with the abluminal surface of the basal lamina of the capillaries. We suggest the term "pecosis" for these areas. All cases were positive for two or more melanocytic markers and for at least one actin. S100 and desmin were focally positive in one case. Cyclin D1 was positive in 3:4 cases. Four cases of malignant PEComa are described with the existence of a unique lesion (pecosis) in one case. These tumours may manifest largely as sarcomas appearing to be undifferentiated and should be considered in their differential diagnosis.
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                Author and article information

                Contributors
                Canbin Lin
                Shulin Liang
                Yongxing Wang
                Aidi Liang
                Weiting Qin
                Jiapeng Huang
                Hao Meng
                Hong Liu
                Journal
                Journal ID (nlm-ta): Medicine (Baltimore)
                Journal ID (iso-abbrev): Medicine (Baltimore)
                Journal ID (publisher-id): MD
                Title: Medicine
                Publisher: Lippincott Williams & Wilkins (Hagerstown, MD )
                ISSN (Print): 0025-7974
                ISSN (Electronic): 1536-5964
                Publication date Collection: 23 September 2022
                Publication date (Electronic): 23 September 2022
                Volume: 101
                Issue: 38
                Electronic Location Identifier: e30653
                Affiliations
                [a ] Department of Urology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, Guangdong, PR China
                [b ] The First Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou, Guangdong, PR China
                [c ] Department of Traditional Chinese Medicine, Jinan University, Guangzhou, Guangdong, PR China.
                Author notes
                *Correspondence: Ming Chen or Lei Meng, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, No. 16 Yard, Airport Road, Baiyun, Guangzhou, Guangdong 510405, PR China (e-mail: chenming0953@ 123456163.com ; 2861448648@ 123456qq.com ).
                Author information
                https://orcid.org/0000-0003-1203-6273
                Article
                Accession ID: 00054
                DOI: 10.1097/MD.0000000000030653
                PMC ID: 9509118
                SO-VID: 991f5842-fd38-4832-8151-46c2d5e68956
                Copyright © Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.
                License:

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 17 August 2022
                Date received : 18 August 2022
                Date accepted : 19 August 2022
                Categories
                Subject: Research Article
                Subject: Clinical Case Report
                Custom metadata
                OPEN-ACCESS TRUE

                Keywords: laparoscopic radical nephroureterectomy,malignant,oblique supine lithotomy position,perivascular epithelioid cell tumor,rare
                Data availability:
                Keywords: laparoscopic radical nephroureterectomy, malignant, oblique supine lithotomy position, perivascular epithelioid cell tumor, rare

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