Cellular fetal rhabdomyomas are composed of bland, primitive spindled cells. The spindle cells are haphazardly arranged primitive, elongated skeletal muscle cells. The cells are set within a well-developed fibromyxoid stroma. A different pattern (intermediate type) is predominantly composed of cells with better differentiation towards skeletal muscle. There are often large ganglion cell-like rhabdomyoblasts showing prominent nucleoli within nuclei that show vesicular chromatin distribution. Another population includes strap-like rhabdomyoblasts with darkly staining pink cytoplasm. Nearly all tumors show short to more sweeping fascicles of spindled rhabdomyoblasts. The tumor cells may infiltrate into adjacent skeletal muscle or fat. It is not uncommon to see peri-neural association, although not perineural infiltration. Diagnosis
These tumors may be detected prenatally by ultrasound and MRI. Additionally, preoperative fine needle aspiration can be used to diagnose the tumor. Ancillary testing
A phosphotungstic acid hematoxylin stain may be used to highlight cross striations in the cytoplasm of the tumor cells. PAS with diastase will highlight the presence of glycogen in the tumor cells cytoplasm. Immunohistochemistry will yield a positive reaction with a variety of myoid markers, including desmin, myoglobin, myogenin, MYOD1 and muscle specific actin. They may also be positive with vimentin, smooth muscle actin, and Leu-7. However, the tumor cells are almost always negative with glial fibrillary acidic protein, S100 protein, cytokeratin, epithelial membrane antigen, CD68, FLI1, CD99 and CD56. Although not used as frequently now, electron microscopy will show thick and thin myofilaments, Z-bands: these are features of sarcometric differentiation.
Ligneous conjunctivitis is a rare form of chronic conjunctivitis characterized by recurrent, fibrin-rich pseudomembranous lesions of wood-like consistency that develop mainly on the underside of the eyelid (tarsal conjunctiva). It is generally a systemic disease which may involve the periodontal tissue, the upper and lower respiratory tract, kidneys, middle ear, and female genitalia. It can be sight-threatening, and death can occasionally occur from pulmonary involvement.It has been speculated hola ligneous conjunctivitis may be a manifestation of IgG4-related disease (IgG4-RD) involving the conjunctiva. Pathogenesis
Histopathological findings from affected humans indicate that wound healing is impaired due to a deficiency in plasmin-mediated extracellular fibrinolysis. Episodes may be triggered by minor trauma, eye surgery, or by systemic events such as infections or antifibrinolytic therapy. Histology shows amorphous subepithelial deposits of eosinophilic material consisting predominantly of fibrin. Diagnosis
Treatment
Ligneous conjunctivitis may be managed by topical treatments of plasminogen, topical and subconjunctival fresh frozen plasma, and fibrinolytic therapy. References


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