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World Journal of Oncology Jun 2024Perivascular epithelioid cell neoplasms (PEComas) are a rare category of mesenchymal tissue tumors, manifesting across various tissues and organs such as the kidneys,... (Review)
Review
Perivascular epithelioid cell neoplasms (PEComas) are a rare category of mesenchymal tissue tumors, manifesting across various tissues and organs such as the kidneys, liver, lungs, pancreas, uterus, ovaries, and gastrointestinal tract. They predominantly affect females more than males. PEComas characteristically express both melanocytic and smooth muscle markers, making immunohistochemistry vital for their diagnosis. Renal angiomyolipoma (AML) represents a common variant of PEComas, typically marked by favorable prognoses. Nonetheless, only a small fraction of subtypes, especially epithelioid AML, possess the capacity to be malignant. Renal PEComas usually appear as asymptomatic masses accompanied by vague imaging characteristics. The main methods for diagnosis are histopathological analysis and the application of immunohistochemical stains. Presently, a uniform treatment plan for renal PEComas is absent. Strategies for management include active surveillance, selective arterial embolization, surgical procedures, and drug-based treatments. The focus of this review is on renal PEComas, shedding light on their pathogenesis, pathological characteristics, clinical presentations, diagnosis, and treatment modalities, and incorporating a clinical case study.
PubMed: 38751707
DOI: 10.14740/wjon1794 -
Frontiers in Surgery 2024A limiting factor in expanding the kidney donor pool is donor kidneys with renal tumors or cysts. Partial nephrectomy (PN) to remove these lesions prior to...
BACKGROUND
A limiting factor in expanding the kidney donor pool is donor kidneys with renal tumors or cysts. Partial nephrectomy (PN) to remove these lesions prior to transplantation may help optimize organ usage without recurrence of malignancy or increased risk of complications.
METHODS
We retrospectively analyzed all recipients of a living or deceased donor graft between February 2009 and October 2022 in which a PN was performed prior to transplant due to the presence of one or more concerning growths. Donor and recipient demographics, perioperative data, donor allograft pathology, and recipient outcomes were obtained.
RESULTS
Thirty-six recipients received a graft in which a PN was performed to remove suspicious masses or cysts prior to transplant. Majority of pathologies turned out to be a simple renal cyst (65%), followed by renal cell carcinoma (15%), benign multilocular cystic renal neoplasm (7.5%), angiomyolipoma (5%), benign renal tissue (5%), and papillary adenoma (2.5%). No renal malignancy recurrences were observed during the study period (median follow-up: 67.2 months). Fourteen complications occurred among 11 patients (30.6% overall) during the first 6mo post-transplant. Mean eGFR (± standard error) at 36 months post-transplant was 51.9 ± 4.2 ml/min/1.73 m ( = 23). Three death-censored graft losses and four deaths with a functioning graft and were observed.
CONCLUSION
PN of renal grafts with suspicious looking masses or cysts is a safe option to optimize organ usage and decrease the kidney non-use rate, with no observed recurrence of malignancy or increased risk of complications.
PubMed: 38726469
DOI: 10.3389/fsurg.2024.1391971 -
Malaysian Family Physician : the... 2024Benign renal lesions are relatively rare. Angiomyolipoma (AML) is the most commonly encountered benign renal lesion. One of the complications of AML is rupture, which...
Benign renal lesions are relatively rare. Angiomyolipoma (AML) is the most commonly encountered benign renal lesion. One of the complications of AML is rupture, which results in retroperitoneal haemorrhage with a mortality rate of up to 20%. Pregnancy poses a major risk for the rupture of AML. This is attributed to its hormonal effect, which causes the tumour to grow rapidly during pregnancy. The possibility of AML rupture should be considered when encountering pregnant patients with hypovolemic shock but with normal initial obstetric ultrasound findings. We present a case of a pregnant patient who was admitted with hypovolemic shock and CT scan confirmed rupture of AML.
PubMed: 38725613
DOI: 10.51866/cr.399 -
Saudi Journal of Kidney Diseases and... Nov 2023Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem...
Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem disorder. Renal AMLs can lead to life-threatening complications like hemorrhage and cause progressive renal failure requiring dialysis and kidney transplant. mTOR inhibitors showed promising results in TS patients with renal AMLs, LAM, and subependymal giant cell astrocytomas. This case report is a follow-up of a patient we reported in 2010 with giant AMLs and LAM who required an emergency nephrectomy for massive hemorrhage from giant left-sided AMLs.
Topics: Humans; Kidney Neoplasms; Angiomyolipoma; Lymphangioleiomyomatosis; Lung Neoplasms; Female; Nephrectomy; Tuberous Sclerosis; Adult; Treatment Outcome; Hemorrhage; Time Factors; Tumor Burden; Tomography, X-Ray Computed
PubMed: 38725217
DOI: 10.4103/sjkdt.sjkdt_324_21 -
International Journal of Critical... 2024Wunderlich syndrome is characterized by the presence of abdominal pain, hematuria, and hypovolemic shock. We report a rare case of a 25-year-old pregnant woman, who came...
Wunderlich syndrome is characterized by the presence of abdominal pain, hematuria, and hypovolemic shock. We report a rare case of a 25-year-old pregnant woman, who came to the emergency department due to the sudden onset of low back pain and diaphoresis. The patient, during medical evaluation, experienced an altered state of consciousness. Diagnosed with hypovolemic shock, she was admitted to the operating room, where examination of the abdominal cavity revealed a left retroperitoneal hematoma. Damage control surgery was performed, but given the postoperative clinical deterioration, computerized tomography angiography of the abdomen was performed, showing a mass-like lesion arising from the upper pole of the left kidney, consistent with Wunderlich syndrome. Left nephrectomy was the definitive treatment for the 10-cm renal angiomyolipoma. Since Wunderlich syndrome is a potentially lethal entity, CT is usually the preferred diagnostic approach, and supra-selective vascular embolization is the first-line treatment.
PubMed: 38715755
DOI: 10.4103/ijciis.ijciis_45_23 -
BMC Urology May 2024Renal sinus angiomyolipoma (RSAML) is a rare and typically complex renal tumor. The objective is to present our single-center experience with a modified technique of...
BACKGROUND
Renal sinus angiomyolipoma (RSAML) is a rare and typically complex renal tumor. The objective is to present our single-center experience with a modified technique of robotic nephron-sparing surgery (NSS) for treating RSAML.
METHODS
We retrospectively evaluated 15 patients with RSAMLs who were treated with robotic NSS at the Department of Urology of Tongji hospital, ranging from November 2018 to September 2022. Renal vessels and ureter were dissected. The outer part of RSAML was resected. The rest of tumor was removed by bluntly grasp, curettage and suction. Absorbable gelatin sponges were filled in the renal sinus. The preoperative parameters, operative measures and postoperative outcomes were all collected. Follow-up was performed by ultrasonography and estimated glomerular filtration rate (eGFR).
RESULTS
Robotic NSS was successfully performed in all the patients, without any conversion to open surgery or nephrectomy. The mean operation time was 134.13 ± 40.56 min. The mean warm ischemia time was 25.73 ± 3.28 min. The median estimated blood loss was 100 [50, 270] ml and 1 patient required blood transfusion. The mean drainage duration was 5.77 ± 1.98 days. The median postoperative hospital stay was 6.90 [5.80, 8.70] days. Two patients experienced postoperative urinary tract infection (Clavien-Dindo Grade II). During the median follow-up of 25.53 ± 15.28 months, patients received 91.18% renal function preservation. No local recurrence occurred in all the patients.
CONCLUSIONS
Robotic NSS for RSAML is a complicated procedure that demands technical expertise and a well-designed strategy is critical in the operation. Treating RSAML with modified robotic NSS is safe, effective and feasible.
Topics: Humans; Robotic Surgical Procedures; Kidney Neoplasms; Female; Retrospective Studies; Adult; Male; Middle Aged; Organ Sparing Treatments; Angiomyolipoma; Nephrons; Nephrectomy
PubMed: 38715034
DOI: 10.1186/s12894-024-01492-x -
Case Reports in Neurological Medicine 2024Tuberous sclerosis (TS) is a rare multisystem autosomal dominant genetic disorder with characteristic pathognomonic genetic mutations involving the TSC (tuberous...
Tuberous sclerosis (TS) is a rare multisystem autosomal dominant genetic disorder with characteristic pathognomonic genetic mutations involving the TSC (tuberous sclerosis complex) group of genes. Ocular signs are fairly common and include an achromic patch and retinal astrocytic hamartomas, which usually have a maximum size of between 0.5 and 5 mm. The incidence of tuberous sclerosis is estimated to be 1 in 5000-10,000 individuals, with both familial and sporadic cases reported. The diagnostic criteria for tuberous sclerosis include the presence of major and/or minor clinical features as well as genetic mutations. We present the case of a 15-year-old girl, presented with a history of seizures and blurred vision. Physical examination revealed angiofibroma on the face. Further evaluation, including contrast-enhanced MRI of the brain and ophthalmological consultation, led to the diagnosis of tuberous sclerosis. Additional imaging studies confirmed the presence of subependymal giant cell astrocytoma, retinal astrocytoma, lymphangioleiomyomatosis in the lungs, and renal angiomyolipoma. This case highlights the importance of considering tuberous sclerosis in patients presenting with seizures and ocular symptoms. This case sheds light on early diagnosis and appropriate management which are crucial in preventing complications and improving patient outcomes.
PubMed: 38694182
DOI: 10.1155/2024/5559615 -
World Journal of Gastroenterology Apr 2024Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors (PEComas) because PEComas are mainly benign tumors and may... (Review)
Review
Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors (PEComas) because PEComas are mainly benign tumors and may not require surgical intervention. By analyzing the causes, properties and clinical manifestations of PEComas, we summarize the challenges and solutions in the diagnosis of PEComas.
Topics: Humans; Perivascular Epithelioid Cell Neoplasms; Liver Neoplasms; Hepatectomy; Preoperative Care; Biomarkers, Tumor; Diagnosis, Differential; Liver
PubMed: 38659487
DOI: 10.3748/wjg.v30.i13.1926 -
The American Journal of Case Reports Apr 2024BACKGROUND Wünderlich syndrome (WS) is a rare diagnosis of nontraumatic spontaneous renal hemorrhage into the subcapsular, perirenal, or pararenal spaces. Prompt and...
BACKGROUND Wünderlich syndrome (WS) is a rare diagnosis of nontraumatic spontaneous renal hemorrhage into the subcapsular, perirenal, or pararenal spaces. Prompt and effective intervention is necessary for an accurate pathological diagnosis and preservation of life. In the current literature, open surgery is the primary option when conservative treatment fails, but there can be serious trauma and corresponding consequences. Herein, we present 3 cases of Wünderlich syndrome managed by robot-assisted laparoscopic nephrectomy via a retroperitoneal approach. CASE REPORT Patient 1 was a 44-year-old woman with right flank pain for 6 h. Patient 2 was a 53-year-old woman with a history of diabetes who had pain in her right flank pain and nausea for 1 day. Patient 3 was a 45-year-old man with left flank pain for 1 day. All cases of WS were confirmed by CT. All 3 patients were treated with retroperitoneal robot-assisted nephrectomy after conservative treatment failed. Pathological examination confirmed that patient 1 had angiomyolipoma, and patients 2 and 3 had renal clear cell carcinoma. At the 9-month follow-up, renal function was good and no evidence of recurrence or metastasis has been detected. CONCLUSIONS These cases have highlighted the importance of the clinical history and imaging findings in the diagnosis of Wünderlich syndrome, and show that rapid management can be achieved using robot-assisted laparoscopic nephrectomy. However, it is crucial to have a skilled surgical team and adequate preoperative preparation.
Topics: Humans; Nephrectomy; Middle Aged; Robotic Surgical Procedures; Male; Laparoscopy; Female; Adult; Syndrome; Kidney Diseases; Hemorrhage; Kidney Neoplasms; Angiomyolipoma; Carcinoma, Renal Cell
PubMed: 38659203
DOI: 10.12659/AJCR.942826