ArticleFrontiers in oncology2026
Renal malignant perivascular epithelioid cell tumor: a case report and literature review.
Article in Frontiers in oncology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
0 citing papers in PubMed.
No citing paper in PubMed yet.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by melanocytic and smooth muscle differentiation. Although the kidney is a relatively common site, malignant renal PEComa is exceedingly rare, particularly in patients with tuberous sclerosis complex (TSC). Overlapping radiologic features between angiomyolipoma and malignant PEComa often result in diagnostic challenges. Case presentation: We report an 18-year-old female who presented with right flank pain. Contrast-enhanced CT revealed a large heterogeneous mass in the right kidney (127 × 81 mm) with intratumoral hemorrhage, initially interpreted as angiomyolipoma associated with TSC. Physical examination and family history were suggestive of TSC, and genetic testing confirmed a pathogenic TSC2 frameshift mutation. The patient underwent open right partial nephrectomy. Histopathology demonstrated a malignant PEComa composed of epithelioid cells with focal coagulative necrosis. Immunohistochemistry showed diffuse positivity for Melan-A and smooth muscle actin, focal HMB-45 expression, and a Ki-67 index of approximately 5%. The patient recovered postoperatively; however, CT imaging at one-month follow-up revealed a suspected enhancing mass in the lateral mid-portion of the right kidney, requiring continued close surveillance. Based on the suspected enhancing mass identified at the one-month postoperative CT follow-up and the confirmed malignant potential, the patient has been advised to initiate adjuvant mTOR inhibitor therapy (Everolimus). A rigorous 3-month interval follow-up protocol has been established to evaluate treatment efficacy and prognosis. Conclusion: This case highlights a rare occurrence of malignant renal PEComa in a young patient with genetically confirmed TSC. Large tumor size, necrosis, and mitotic activity are important indicators of malignant potential. In young patients with TSC and atypical renal masses, malignant PEComa should be considered in the differential diagnosis. Multidisciplinary evaluation, genetic testing, and awareness of emerging mTOR-targeted therapies are essential for optimal management.
Indexed as
Identifiers
What Socratic holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.