In this manuscript, we summarize the main features of angiomyolipoma highlighting the recognition of epithelioid angiomyolipoma and the discovery of immunohistochemical expression of HMB45 in a group of tumors that now are referred to as as PEComas. In this scenario, Dr. Rosai believed in our intuition, demonstrating his intellectual honesty and motivated us with his experience ("") and enthusiasm for the new entities (""). He really pushed the improvement of the knowledge in this field.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8720413PMC
http://dx.doi.org/10.32074/1591-951X-335DOI Listing

Publication Analysis

Top Keywords

epithelioid angiomyolipoma
8
angiomyolipoma pathological
4
pathological entity
4
entity discovered
4
discovered verona
4
verona endorsement
4
endorsement doctor
4
doctor rosai
4
rosai manuscript
4
manuscript summarize
4

Similar Publications

Hepatic epithelioid angiomyolipoma is a rare mesenchymal liver tumor within the perivascular epithelioid cell tumor family, known for its uncertain malignancy and potential for aggressive behavior. Clinical presentation, laboratory findings, and imaging results lack specificity, making pathological examination critical for definitive diagnosis. This case report presents a 61-year-old male patient with a single tumor in the right liver lobe, measuring approximately 101 mm × 99 mm.

View Article and Find Full Text PDF

Gamna-Gandy (GG) bodies are sclerosiderotic nodules composed of iron pigment and calcium, that have been described predominantly in the spleens of patients with sickle cell disease. Their formal depiction in the kidney is mainly limited to case reports and small series. We aimed to investigate the incidence of GG bodies and associated clinicopathologic features in consecutive nephrectomies performed for renal tumors.

View Article and Find Full Text PDF

Background: A right adrenal gland may present in the form of adreno-hepatic fusion (AHF), in which the adrenal cells are interspersed among the hepatocytes without septation. This rare, naturally-occurring phenomenon may be associated with preoperative misdiagnosis. We present two cases of adrenal tumor in patients with AHF that were misdiagnosed, despite thorough preoperative work-ups.

View Article and Find Full Text PDF

Background: Perivascular epithelioid cell tumors (PEComas) rarely appear in the head and neck region. This case report describes two transcription factor E3 (TFE3)-rearranged PEComa cases, consisting of one in the orbit and one in the nasal cavity.

Case Presentation: Both cases demonstrated sheet-like or focal nested architecture and comprised epithelioid cells with abundant clear to eosinophilic cytoplasm and vascular stroma.

View Article and Find Full Text PDF

A 55-year-old man with tuberous sclerosis complex (TSC) was diagnosed with left renal angiomyolipoma (AML), a group of perivascular epithelioid cell tumors called PEComas. He had received the mTOR inhibitor everolimus, which resulted in a complete response. However, a left renal mass relapsed in two years, followed by the occurrence of a hepatic mass five months later.

View Article and Find Full Text PDF

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!