Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with characteristic tumors involving multiple organ systems. Whereas renal angiomyolipoma (AML) is common in TSC, renal cell carcinoma (RCC) is rarely reported. Fifty-seven RCCs from 13 female and 5 male TSC patients were reviewed. Age at surgery ranged from 7 to 65 years (mean: 42 y). Nine patients (50%) had multiple synchronous and/or metachronous RCCs (range of 2 to 20 RCCs) and 5 had bilateral RCCs (28%). Seventeen patients (94%) had histologically confirmed concurrent renal AMLs, including 15 with multiple AMLs (88%) and 9 (50%) with AMLs with epithelial cysts. None of the 15 patients with available clinical follow-up information had evidence of distant metastatic disease from 6 to 198 months after their initial surgery (mean: 52 mo). The 57 RCCs exhibited 3 major distinct morphologies: (1) 17 RCCs (30%) had features similar to tumors previously described as "renal angiomyoadenomatous tumor" or "RCC with smooth muscle stroma"; (2) 34 RCCs (59%) showed features similar to chromophobe RCC; and (3) 6 RCCs (11%) showed a granular eosinophilic-macrocystic morphology. Distinct histologic changes were also commonly present in the background kidney parenchyma and included cysts or renal tubules lined by epithelial cells with prominent eosinophilic cytoplasm, nucleomegaly, and nucleoli. Immunohistochemically, all RCCs tested showed strong nuclear reactivity for PAX8 and HMB45 negativity. Compared with sporadic RCCs, TSC-associated RCCs have unique clinicopathologic features including female predominance, younger age at diagnosis, multiplicity, association with AMLs, 3 recurring histologic patterns, and an indolent clinical course. Awareness of the morphologic and clinicopathologic spectrum of RCC in this setting will allow surgical pathologists to better recognize clinically unsuspected TSC patients.

Liver shear wave speed is abnormally increased in neonates and infants with biliary atresia.

- Multiple histologic patterns of fetal vascular malperfusion have been described including thrombosis, avascular villi, villous stromal-vascular karyorrhexis, intramural fibrin thrombi, and stem villous vascular obliteration. Various underlying etiologies can be involved in fetal vascular malperfusion. Cord lesions including abnormal insertion, length, and coiling are important causes. Maternal vascular malperfusion such as preeclampsia, hypercoagulable states, lupus anticoagulant, and sometimes diabetes have been associated with this condition. Fetal cardiac dysfunction/malformations and severe fetal inflammatory response in the setting of ascending intrauterine infection have also been attributed to this important finding. Fetal vascular malperfusion has been implicated in several significant and sometimes devastating clinical associations; these include intrauterine growth restriction, poor perinatal outcome, fetal demise, and neurodevelopmental sequelae. A diagnostic challenge may be encountered in cases with prior intrauterine fetal death, since degenerative changes post demise result in a similar histomorphologic picture. The diffuse versus the focal nature of the lesions may help in the distinction.

Fetal ultrasound remains an important tool in the detection and evaluation of congenital lung malformations. However, it does not correctly predict histology in approximately 25% of prenatally detected CLMs and remains limited by relatively poor sensitivity for systemic feeding vessels pathognomic for a bronchopulmonary sequestration. These data suggest the importance of obtaining additional cross-sectional imaging, preferably a postnatal CT scan, in all patients to help counsel families and to guide in the optimal management of these lesions.