L Hermitte - Duclos Disease - Intraoperative Cytological Findings of a Rare Entity

Abstract

OBJECTIVE: A rare case of LDD confirmed by biopsy is being reported. Review of the published cases shows that the disease usually manifests by the signs of increased intracranial pressure with inconstant cerebellar symptoms. MRI is the imaging modality of choice by now. As such, clinical problems after gross total or complete removal have not been reported so far, but herein we stress the importance of intraopeartive diagnosis of lesions that have no distinct border between the lesion and normal tissue. METHODS: After subtotal resection of lesion, small representative tissue was sent to pathology department intraoperatively in saline for squash smear cytology and postoperatively, whole tissue was sent in formalin for histopathology. The squash smears were stained with MGG, H&E, and PAP stain and report was rendered within 30 minutes. The morphological details as observed in the smears were correlated with Histopathology findings. RESULTS: Squash smears showed a fibrillary background in most of the smears with scattered ganglion cells having vesicular nuclei and prominent nucleoli. Some of the smears showed small dark granular cells representing internal granular cell layer. The corresponding histopathological findings showed widening of the molecular layer with abnormal myelination that is occupied by abnormal ganglion cells, absence of the Purkinje cell layer and hypertrophy of the granular cell layer. CONCLUSION: Squash smear preparation is a fairly accurate, simple and reliable tool for rapid intraoperative diagnosis of CNS lesions. It is helpful in defining the limits of resection in cases like LDD where margins are not well defined.

Cytopathology of glioneuronal and neuronal tumours with histological correlations

“…The smear findings are similar to those of gangliocytoma, 47 showing granular cell layer remnants can be seen intermixed with the ganglion cell component. Also, like gangliocytoma, DCG lacks a neoplastic glial cell component (Figure 4D).…”