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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Tuberous sclerosis can cause angiomyolipomas. It carries an increased risk of renal cell carcinoma and is associated with intracranial tumours such as subependymal giant cell astrocytomas as well as other parenchymal brain abnormalities. However, the other features in the question are not typical. Pulmonary cysts, sclerotic bone lesions and cardiac rhabdomyomas are also encountered.
Tuberous Sclerosis Complex
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Joana Jesus Ribeiro, Filipe Palavra, Flávio Reis
Renal angiomyolipomas are benign tumors composed of abnormal vessels, immature smooth-muscle cells, and fat cells (Figure 99.2c and d) [28]. Fat-containing angiomyolipomas are observed in 80% of TSC patients, and fat-poor lesions are also common in these individuals but occur in less than 0.1% of the general population [2]. Renal angiomyolipomas are bilateral, multiple, and tend to grow slowly [1,28]. However, sometimes they increase rapidly within a short period of time [73]. Although the majority of angiomyolipomas behave in a benign manner, there is a small subgroup which may behave more aggressively and become locally invasive [83], being more often symptomatic in women [1]. They appear by 10.5 years of age and increase in size and number in the late teens and early 20s, not regressing spontaneously [1,73]. Spontaneous hemorrhage can be the presenting sign in 50%–60% of the patients, as these tumors have abnormal vasculature and often contain aneurysms, leading to hemorrhagic shock in one-third of patients [28,83]. Although the size of an angiomyolipoma roughly correlates with the risk of hemorrhage, the size of the aneurysm appears to be proportional to the risk of bleeding, with aneurysms larger than 5 mm presenting the highest risk [73].
Kidneys and ureters
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
Angiomyolipoma is an unusual tumour of the kidney, often but not always associated with tuberous sclerosis. Its high fat content has a typical appearance on CT. Malignant elements are present in about one-quarter and may metastasise.
Fetal Tuberous Sclerosis: Sirolimus for the Treatment of Fetal rhabdomyoma
Published in Fetal and Pediatric Pathology, 2022
Ana Dagge, Luísa Andrade Silva, Sofia Jorge, Estela Nogueira, Mónica Rebelo, Luísa Pinto
There are no studies evaluating what the best gestational age and route of delivery for fetuses with rhabdomyomas are. Specifically when sirolimus was used prenatally, in only one case was the delivery mode reported [9]. In this case, an induction of labor was carried out at 36 weeks because of the development of preeclampsia and fetal growth restriction and a vaginal delivery ocurred. In our case, the multidisciplinary team decided for an elective cesarean given the risk of rupture of the large maternal renal angiomyolipomas and because of the impossibility of an adequate intrapartum fetal cardiotocographic monitoring due to the presence of intermittent arrythmia. The evidence regarding the behavior of angiomyolipomas is based on clinical reports. Pregnancy is a risk factor for tumor growth and invasiveness with a consequent increased risk of rupture, with massive retroperitoneal hemorrhage, although this is extremely rare [15]. When rupture occurs, it is usually a spontaneous event that occurs more frequently around the 27th week of gestation [16]. There is no evidence regarding the safest mode of delivery in pregnant women with renal angiomyolipomas.
Spontaneous Page kidney from ruptured renal angiomyolipoma
Published in Baylor University Medical Center Proceedings, 2021
Niharika Neela, Hula Taha, Jaya Kala
The first case was a 55-year-old hypertensive, diabetic woman who was admitted with 1 week of abdominal pain, cough, fever, dyspnea, and elevated blood pressure. Ultrasound of the abdomen revealed previously seen benign right kidney angiomyolipomas (largest mass of 0.9 cm). She was diagnosed with acute respiratory failure from COVID-19 pneumonia and treated with remdesivir, dexamethasone, and enoxaparin. She continued to experience abdominal pain. On hospital day 14, her hemoglobin dropped to 2 g/dL, prompting computed tomography (CT) of the abdomen, which revealed a large right perinephric expanding hematoma that compressed the right kidney (Figure 1a, 1b), for which the main and accessory renal arteries were embolized. Hemodialysis was initiated, and she died from complications. Autopsy revealed necrosis due to embolization at the site of renal hematoma corresponding to prior larger angiomyolipomas (Figure 1c).
Recent advances in PET probes for hepatocellular carcinoma characterization
Published in Expert Review of Medical Devices, 2019
Luca Filippi, Orazio Schillaci, Oreste Bagni
As concerns the potential usefulness of this tracer as a metabolic probe for the characterization of HCC, Ho et al. evaluated the role of dual-tracer PET with FDG/11C-acetate in 57 patients with hepatic lesions [38]. In subjects with biopsy-confirmed HCC, PET with 11C-acetated presented a sensitivity of 87.3% whereas FDG had a sensitivity of 47.3%. Of note, some lesions (34.5%) presented positivity for both tracers even with a complementary pattern (different tracer uptake in different parts of the same tumor). It has to be pointed out that patients with exclusive FDG uptake were affected by advanced-stage, moderately to poorly differentiated forms. As concerns specificity of 11C-acetate PET for characterizing hepatic lesions, a false-positive case was firstly reported by Lhommel and colleagues: a 64-year-old male patient with a suspected 7-cm lesion on MRI, without any history of liver disease and with normal level of AFP, presented highly increased 11C-acetate and no FDG uptake within the lesion, consistent with a diagnosis of well-differentiated HCC [39]. In such a case, histology revealed a benign angiomyolipoma with high density of smooth muscle cells. As 11C-acetate represents a substrate for the oxidative process typical of the muscle cells, angiomyolipoma showed an avid accumulation of the tracer thus mimicking a well-differentiated HCC.