Renal Epithelioid Angiomyolipoma in a Rural Indian Setting: A Case Series Emphasizing Morphological Diagnosis and Malignant Potential Amidst Resource Constraints

Abstract

Epithelioid angiomyolipoma (EAML), a rare kinship within the perivascular epithelioid cell tumor (PEComa) family, presents a diagnostic conundrum. Unlike its classical counterpart, EAML often lacks the tell-tale fatty signature on imaging and harbors a significant propensity for malignant behavior, frequently masquerading as renal cell carcinoma (RCC). This diagnostic obscurity poses a considerable clinical hurdle, particularly within the constraints of resource-limited healthcare environments Objective: To highlight the diagnostic difficulty of EAML and emphasize the critical role of histopathology in its identification, particularly in the absence of immunohistochemistry. The study also underlines the tumor’s malignant potential and the need for appropriate management. Our study focuses on morphological histopathological findings of EAML in a resource-limiting setting, specifically in a tertiary care center of rural background in North India. Methods: We report three cases of EAML in patients presenting with flank pain, hematuria, or abdominal mass at a rural tertiary center in Uttar Pradesh, India. Contrast-enhanced CT showed solid renal masses without visible fat, leading to a provisional diagnosis of malignancy. One case involved bilateral kidneys. All patients underwent surgical excision due to lesion size and suspicious imaging features. Results: Histopathological examination confirmed EAML in all cases based solely on morphology, in the absence of immunohistochemical facilities. Distinct cellular features and growth patterns were diagnostic. Surgical resection was curative, with no recurrence or metastasis during follow-up. Conclusion: EAML, though rare, must be considered in the differential diagnosis of fat-poor renal masses. Its malignant potential and ability to radiologically mimic RCC necessitate high clinical suspicion. In settings lacking immunohistochemistry, careful histopathological evaluation is essential. Surgery remains the mainstay of treatment, serving both diagnostic and therapeutic roles.