Answer: FIBRINOGEN STORAGE DISEASE (fibrinogen gene mutation, incl. FGG). The globular inclusions are present throughout (panlobular); while, A1AT deficiency is more pronounced in zone 1 hepatocytes. EM shows tubular inclusions in a fingerprint pattern.

Answer: VCL-ALK renal cell carcinoma This subtype of RCC is associated with younger patients, sickle cell trait, renal pelvis involvement, and intact INI1. (PMIDs: 24698962, 26773439) H&E: discohesive, polygonal cells with cytoplasmic vacuoles, ALK IHC: focal membranous staining

Answer: chronic granulomatous disease. Along with granulomas, pigmented macrophages are a feature of CGD. The pigment represents a by-product ineffective digestion of organisms, membranes, and lipid. Other features: eosinophilia, activity, and chronic changes (PMID: 23887163).

The H&Es show spider cells characteristic of CARDIAC RHABDOMYOMA. It is considered a hamartoma of developing cardiomyocytes and associated with tuberous sclerosis (TSC1/2 mutations). Others: Cardiac fibroma - Gorlin syndrome (PTCH1) Cardiac myxoma - Carney complex (PRKAR1A)

Note how the nest is composed of spindle cells in the periphery and round cells in the center. The cells are positive for WT1 (N-terminus). The lesion is associated with CTNNB1 and TERT promoter alterations (PMID: 33994539). Ddx/ hepatoblastoma and DSRCT ( for WT1 C-terminus).