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Radiology Case Reports Aug 2024Renal angiomyolipoma (AML) is a rare benign tumor of the kidney, often detected incidentally on radiological images as the presence of macroscopic fat characterizes...
Renal angiomyolipoma (AML) is a rare benign tumor of the kidney, often detected incidentally on radiological images as the presence of macroscopic fat characterizes them. In the majority of the cases, they are usually sporadic. Despite their benign nature, venous invasion, a rare occurrence in renal AMLs, poses management challenges. We present a case of bilateral renal AML in a 52-year-old female with a right renal vein and hepatic inferior vena cava invasion.
PubMed: 38800084
DOI: 10.1016/j.radcr.2024.04.006 -
Cureus Mar 2024Renal angiomyolipoma (AML) is a rare benign tumor of the kidney that can occur as a sporadic lesion or a part of tuberous sclerosis. A 77-year-old female patient with a...
Renal angiomyolipoma (AML) is a rare benign tumor of the kidney that can occur as a sporadic lesion or a part of tuberous sclerosis. A 77-year-old female patient with a history of hypertension, hyperlipidemia, and an unclear history of left nephrectomy in 1999 presented with progressive shortness of breath and palpitations. Her vital signs showed elevated blood pressure, and the examination was benign and non-focal. A work-up showed multiple lesions in her lungs and right kidney, representing lymphangioleiomyomatosis. The patient was diagnosed with tuberous sclerosis and was followed up by pulmonology and nephrology. She underwent embolization of the renal AML, after which her blood pressure (BP) was more controlled, and she reported feeling well and symptom-free. Renal AML, as a part of tuberous sclerosis, is a rare cause of secondary hypertension. Embolization of AML is effective in controlling BP.
PubMed: 38567223
DOI: 10.7759/cureus.55410 -
Asian Journal of Surgery Nov 2023
Topics: Humans; Kidney Neoplasms; Angiomyolipoma; Tuberous Sclerosis; Nephrectomy; Hamartoma
PubMed: 37537037
DOI: 10.1016/j.asjsur.2023.07.057 -
Oncology Letters Nov 2023Metanephric adenoma (MA) is a rare type of benign renal epithelial tumor that can develop at any age. Nonetheless, MA is extremely rare in children and only a few cases...
Metanephric adenoma (MA) is a rare type of benign renal epithelial tumor that can develop at any age. Nonetheless, MA is extremely rare in children and only a few cases have been reported to date. The present study aimed to report the case of a 5-year-old female found to have a mass in the right kidney during a routine pre-enrollment physical examination. Computed tomography (CT) images revealed multiple high-density calcifications in the mass, and contrast-enhanced CT and magnetic resonance imaging demonstrated that the mass was significantly enhanced in the cortical phase and decreased in the medullary phase. Based on these findings, the mass was initially diagnosed as angiomyolipoma before surgery; however, postoperative pathology confirmed the mass to be a MA. MAs are typically a type of soft tissue mass with relatively uniform density or signal, showing delayed enhancement in contrast-enhanced scanning. However, the mass found in the present study presented diffused high-density calcification, which was obvious in the early phase of contrast-enhanced scanning but weakened in the delayed enhancement phase. In conclusion, the present case study demonstrated that MA should be considered as one of the imaging differential diagnoses of fat-poor angiomyolipoma, renal carcinoma and oncocytoma.
PubMed: 37818137
DOI: 10.3892/ol.2023.14073 -
Frontiers in Oncology 2023Epithelioid angiomyolipoma (EAML) is a rare type of mesenchymal angiomyolipoma with potential malignancy in the kidney that can cause lymph node metastases, local...
Epithelioid angiomyolipoma (EAML) is a rare type of mesenchymal angiomyolipoma with potential malignancy in the kidney that can cause lymph node metastases, local recurrence, and distant metastases. Herein, we describe a case of EAML in the right kidney of a 51-year-old man who was admitted to the hospital with a right abdominal mass. Computed tomography revealed a heterogeneously enhanced mass with blurred margins, which was considered a malignant tumor. A radical nephrectomy was then performed. Two years later, the patient developed liver metastases from EAML and was administered sintilimab combined with bevacizumab. The patient survived after 6 months of follow-up. Histologically, the tumors showed clear boundaries and no obvious capsules. The tumor tissue mainly consisted of epithelioid tumor cells, thick-walled blood vessels, and a small amount of adipose tissue. Tumor cells with lipid vacuoles and acinar areas were large, round, polygonal, eosinophilic, or transparent in the cytoplasm. The enlarged and hyperchromatic nuclei were accompanied by distinct nucleoli and pathological mitosis. These histopathological findings resembled those of renal cell carcinoma, and immunohistochemical analysis was performed. The tumor cells were diffusely positive for HMB45, Melan-A, CK20, vimentin antibodies, and TFE3, suggesting that the tumor originated from perivascular epithelioid cells, excluding renal cell carcinoma. The Ki-67 index was 10%. These histopathological features were observed in liver mass puncture tissues. We also summarized 46 cases of EAML with distant metastasis and explored the clinicopathological features of EAML to improve the treatment of the disease. EAML is often ignored in the clinical setting, leading to metastasis and recurrence. Therefore, EAMLs require long-term follow-up, and timely detection of recurrent disease can improve the prognosis.
PubMed: 37675231
DOI: 10.3389/fonc.2023.1207536 -
Cureus Oct 2023Hemangioblastoma, also known as capillary hemangioblastoma, is a rare benign mesenchymal tumor commonly found in the central nervous system (CNS). It can also manifest...
Hemangioblastoma, also known as capillary hemangioblastoma, is a rare benign mesenchymal tumor commonly found in the central nervous system (CNS). It can also manifest in various organs, including the kidney. Renal hemangioblastoma (RH) is often associated with Von Hippel-Lindau (VHL) disease, but sporadic occurrences are observed infrequently. While RH is usually asymptomatic, it can also cause abdominal pain and hematuria. In this study, we present a case of an elderly patient without history of VHL but complaining of abdominal pain for three days. Serological evaluations were unremarkable, and a CT scan identified a 2.4 cm mixed solid-cystic mass lesion on the left kidney's superior aspect. The patient subsequently underwent a biopsy followed by lesion ablation. Microscopic analysis revealed sheets of eosinophilic cells with ovoid nuclei, showing focal rhabdoid and spindle cell features, with an intricate capillary network. Focal nuclear atypia without necrosis or mitosis was noted. Immunohistochemistry (IHC) demonstrated positive staining for inhibin, S100, PAX8, and vimentin, along with patchy positivity for CD10 and RCC. Negative staining was observed for cytokeratin AE1/AE3, CK7, EMA, CK8/18, desmin, and HMB-45. The overall morphological characteristics and distinct IHC markers were consistent with RH. Although its pathogenesis remains unclear because of its rarity, distinguishing RH from renal cell carcinoma is crucial. IHC markers facilitate differentiation among lesions. The preferred treatment involves ablation or partial nephrectomy. Further assessment for possible VHL syndrome is essential, considering the distinct management approaches for sporadic and VHL-linked RH.
PubMed: 38022288
DOI: 10.7759/cureus.47102 -
Sultan Qaboos University Medical Journal Dec 2023Angiomyolipoma is a common benign solid tumour that accounts for up to 3% of all renal tumours; most of the cases are sporadic. However, it can be part of other...
Angiomyolipoma is a common benign solid tumour that accounts for up to 3% of all renal tumours; most of the cases are sporadic. However, it can be part of other diseases. Angiomyolipomas are usually found incidentally through unrelated clinically indicated images but also, they can be diagnosed after complications have occurred. We report the case of retroperitoneal haemorrhage following the rupture of renal angiomyolipoma post-fibrinolysis, we are highlighting such a rare condition, the management options and the follow-up plan. The management of angiomyolipomas ranges from conservative treatment to surgical intervention depending on the patient's condition and the tumour's radiological features. Following-up on patients with angiomyolipomas depends on the symptoms and the tumour size. Till date, there is no reported cases of renal angiomyolipoma rupture post-fibrinolysis therapy as a treatment for myocardial infarction in non-percutaneous intervention capable facility.
Topics: Humans; Kidney Neoplasms; Angiomyolipoma; Fibrinolysis; Hamartoma; Thrombolytic Therapy
PubMed: 38161759
DOI: 10.18295/squmj.12.2023.074 -
Journal of Cancer Research and... Aug 2023Transcatheter-arterial chemoembolization (TACE) is a well-established interventional technique for various tumor treatments, whereas its application in renal...
OBJECTIVE
Transcatheter-arterial chemoembolization (TACE) is a well-established interventional technique for various tumor treatments, whereas its application in renal angiomyolipoma (RAML) is seldom reported. Conventional TACE (cTACE) with bleomycin-lipiodol emulsion is effective and tolerable for RAML treatment. In this study, we aimed to further explore the efficacy and safety between bleomycin-loaded CalliSpheres microsphere TACE (CSM-TACE) and cTACE in treating RAML patients.
METHODS
We retrospectively analyzed the data of 54 RAML patients treated by CSM-TACE (n = 17) or cTACE (n = 37). Data on tumor size, tumor volume reduction ratio, patient percentage with tumor size reduction, white blood cells (WBCs), creatinine (Cre) after treatment, complications, and adverse events were retrieved.
RESULTS
Tumor size (88.66 vs. 81.19 cm, P = 0.970), patient percentage with tumor size reduction (12 [70.59%] vs. 30 [81.08%], P = 0.486) after treatment, WBCs (P = 0.114), Cre (P = 0.659), and change in Cre after treatment (P = 0.947) were not significantly different between groups, whereas tumor volume reduction ratio was slightly lower in the CSM-TACE group than in the cTACE group (12 ± 34% vs. 32 ± 31%, P = 0.047). The most common postoperative complication was a post-embolization syndrome, including fever, nausea, and abdominal pain, which occurred in 9 (52.94%) and 14 (37.84%) patients from the CSM-TACE and cTACE groups, respectively (P = 0.347).
CONCLUSION
CSM-TACE is effective in and well tolerated by RAML patients, implying its potential as an alternative therapy.
Topics: Humans; Kidney Neoplasms; Angiomyolipoma; Microspheres; Retrospective Studies; Hamartoma; Bleomycin; Creatinine
PubMed: 37675719
DOI: 10.4103/jcrt.jcrt_2135_22 -
Ear, Nose, & Throat Journal Jan 2024Angiomyolipoma is a benign, mesenchymal, hamartomatous lesion often described in the kidney and may be associated with tuberous sclerosis. Nasal angiomyolipoma is an...
Angiomyolipoma is a benign, mesenchymal, hamartomatous lesion often described in the kidney and may be associated with tuberous sclerosis. Nasal angiomyolipoma is an extremely rare tumor with fewer than 20 cases reported in the literature. We report a case of angiomyolipoma arising in the right nasal vestibule in a 68-year-old male with mild, recurrent right-sided epistaxis, and nasal obstruction. With the exception of arterial hypertension, the patient did not have any comorbidities. Gross examination showed a well-circumscribed, lobulated mass. On the microscopic level, it was composed of mature smooth muscle cells, thick-walled blood vessels of varying sizes, and islands of mature adipose tissue. Angiomyolipoma should be considered as a differential diagnosis for unilateral nasal masses, especially those situated in the nasal vestibule.
Topics: Male; Humans; Aged; Angiomyolipoma; Nose Neoplasms; Nasal Cavity; Kidney Neoplasms; Epistaxis; Hamartoma
PubMed: 34328822
DOI: 10.1177/01455613211036241 -
International Journal of Surgery Case... Dec 2023Renal angiomyolipoma (AML) are benign tumors, often incidentally diagnosed with rupture being the commonest complication and cause of mortality. These tumors are rare...
INTRODUCTION AND IMPORTANCE
Renal angiomyolipoma (AML) are benign tumors, often incidentally diagnosed with rupture being the commonest complication and cause of mortality. These tumors are rare with a higher prevalence among patients with tuberous sclerosis and female predominance. Management is dependent on tumor size and whether or not the tumor has ruptured.
CASE PRESENTATION
32-year-old female presenting with sudden right flank pain with shock without history of prior trauma or surgeries. Underwent imaging revealing a suspected ruptured AML thus underwent emergent nephrectomy with admission to intensive care.
CLINICAL DISCUSSION
Wunderlich syndrome manifests as the Lenk triad, which includes acute flank pain, flank mass, and hypovolemic shock with signs of internal bleeding such as hematuria. It is a rare manifestation signifying spontaneous renal hemorrhage. Due to the instability of the patient had to undergo emergency laparotomy and nephrectomy.
CONCLUSION
Renal AML occur as a rare benign phenomenon which when ruptured are associated with high mortality rate if not treated promptly in a setting with specialized treatment and intensive unit care. We hope that through our experience patients presenting with Lenk's triad are identified early for adequate intervention.
PubMed: 38006740
DOI: 10.1016/j.ijscr.2023.109073