LAUSR

The term “craniopharyngioma” was coined by Frazier and Alpers in 1931 to describe the developmental tumor that arises from the cell rests of the hypophyseal duct that fails to involute during the first trimester.1 The sellar and suprasellar region is a site of intense and precise actions in the first trimester, wherein the formation of adenohypophysis and neurohypophysis occurs in a definite sequence. Some of the cells rests can be found in the sella, suprasellar region, pituitary stalk, third ventricle, and posterior pharyngeal wall. Craniopharyngioma can develop at any of the sites and, depending on its growth, can manifest with visual loss, optic atrophy, growth disturbances, feeding and endocrinologic problems, disturbances in sexual function, and multiple cranial nerve palsy. Craniopharyngiomas are slow-growing benign tumors, and the adjacent brain tissue reacts with intense gliosis and formation of Rosenthal fibers.2 Kawamata et al3 described the craniopharyngioma-brain interface that has a bearing on resectability of the tumor in contact with the hypothalamus. As per this description, type I tumors are characterized by encapsulated tumor with inflammation, facing a gliotic brain tissue. Type II shows a clear cleavage, and type III has interdigitating tumor margin into hypothalamus. Brain tissue layer adjoining the tumor is characterized with piloid gliosis and Rosenthal fibers. Their anatomical location with their proximity to visual apparatus, hypothalamus, and cranial nerves can result in severe visual, endocrine and cognitive deficits, and these deficits can occur due to surgical intervention too. Surgical removal of craniopharyngiomas remains a challenge, even in this second decade of 21st century, more than 100 years after their removal was attempted. Cushing descr ibed craniopharyngiomas as “the kaleidoscopic tumors, solid and cystic, which take their origin from epithelial cell rests ascribable to an imperfect closure of hypophyseal or craniopharyngeal duct...whose management is one of the most baffling problems to the neurosurgeon.”4 Hoffman described quite succinctly, the needed surgical attitude for their management, when he stated, “...perhaps the most important factor governing operative management of craniopharyngioma is the surgeon’s attitude towards the tumor. If he or she regards the tumor as unresectable, it will be treated as such and no attempt will be made to remove it. If on the other hand, the surgeon feels the tumor that the craniopharyngioma can be totally removed, its management is likely to be successful.”5 The first attempt to remove these tumors is likely to be the best, whereas attempts to remove a previously operated and/or irradiated tumor will be difficult due to adherence of the tumor to the blood vessels, nerves, and hypothalamus.6 Thus, every attempt should be made to remove these tumors completely in the first surgery. The approach, a combination of frontal interhemispheric and trans-lamina terminal approaches gives an excellent view of the entire anatomy and pathology.7 Imaging displayed in the OR is studied (►Fig. 1), and the visible tumor anatomy is correlated with the imaging appearances. All components and lobulations of the tumor are identified and kept in mind as the dissection commences and planes get defined further. Subfrontal, subchiasmatic, and parasellar extensions are seen, and the extent of tumor growth and proposed excision is appreciated. No attempt should be made to coagulate the tumor surface. Following structures are displayed and will be repeatedly used as reference points as the dissection progresses: