Introduction: Renal angiomyolipoma (also called renal hamartoma) is a benign Tumor, composed of adipose tissue, smooth muscle tissue, and blood vessels. Tumors more than 10 cm (referred to as "giant" AMLs) are infrequent and a very small number of reported cases were measured more than 20 cm. Epithelioid angiomyolipoma (EAML), is a rare variant of angiomyolipoma with malignant potential. There are less than 10 cases of huge EAML reported in English literature, that were all associated with recurrence and complications.
Case Presentation: Here we report a rare case of renal EAML in a 48-year-old male patient that was not associated with tuberous sclerosis. He was admitted to our hospital with a complaint of severe abdominal pain. Computer Tomography Scan (CT-scan), revealed two masses attached to the left kidney with a size of 32 × 22 × 8 cm and 20 × 10 × 3 cm. The patient underwent a radical left nephrectomy. Postoperative histopathological examination confirmed the diagnosis of an EAML. The two-year follow-up CT-scan showed no recurrence.
Conclusion: Malignant EAML, although rare, does exist. Thus, besides evaluations required for ruling out the diagnosis of renal sarcomas, surgeons must follow patients after total nephrectomy for possible recurrence. Benign cases can be completely cured by surgery and do not show recurrence at the end of the follow-up period.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6965190 | PMC |
| http://dx.doi.org/10.1016/j.ijscr.2019.12.032 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Risk prediction criteria for the primary hepatic perivascular epithelioid cell tumour family, including angiomyolipoma: analysis of 132 cases with a literature review.
Histopathology
December 2024
Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Aims: The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors.
Methods And Results: In total, 132 patients were included.
Renal PEComa in a young male: A case report and insights from the literature.
Urol Case Rep
January 2025
Department of the Emergency Medicine, State Security Service Military Hospital, Baku, Azerbaijan.
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, commonly found in the uterus and retroperitoneum. Renal PEComas are exceedingly rare, often posing diagnostic challenges due to their resemblance to renal cell carcinoma (RCC) on imaging. We present the case of an 18-year-old male who presented with non-specific symptoms of fever, chills, and shivering.
View Article and Find Full Text PDFAdrenal PEComa Treated by Surgical Resection and Postoperative Radiotherapy: A Case Report.
Am J Case Rep
December 2024
Department of Pathology, Eskişehir City Hospital, Eskişehir, Turkey.
BACKGROUND Perivascular epitheloid cell neoplasm (PEComa) is a rare mesenchymal tumor that is evaluated in the same tumor family as angiomyolipoma, sugar cell tumor of the lung, and lymphangioleiomyomatosis. Immunohistochemically, the disease can express melanocytic and myogenic markers, such as HMB45, HMSA1, MelanA/Mart1, and actin. The disease can be seen in almost every organ, especially the uterus and retroperitoneum.
View Article and Find Full Text PDFCeus features of liver pecoma: a case report and literature review.
J Ultrasound
November 2024
Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy.
Perivascular epithelioid cell neoplasms (PEComas) and epithelioid angiomyolipomas (EAMLs) are two different denominations for the same "mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells". Hepatic PEComa/EAML is a very rare neoplasm, and only 29 case reports of hepatic PEComa and 25 of hepatic EAML have been reported in the current literature. A clear female predominance with a mean age at diagnosis of 42.
View Article and Find Full Text PDFAngiomyolipomatous Lesions of the Nasal Cavity (Sinonasal Angioleiomyoma with Adipocytic Differentiation): A Multi-Institutional Immunohistochemical and Molecular Study.
Head Neck Pathol
October 2024
Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH, USA.
Article Synopsis
- Mesenchymal neoplasms in the nasal cavity, such as angioleiomyoma (AL) and angiomyolipoma (AML), are rare tumors that involve vascular, smooth muscle, and adipocytic components, with unclear classifications as hamartomas or true neoplasms due to limited genetic research.
- A multi-institutional retrospective study collected data on 15 nasal cavity lesions, primarily affecting older males with obstructive symptoms, and analyzed tissue samples for genetic features.
- The study discovered that most cases had a high proportion of smooth muscle, with some showing additional components like blood vessels and fatty tissue, while all lesions were positive for smooth muscle markers, indicating their muscular origin.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!