Sellar chondroma in a case of Ollier's disease

Abstract

Chondromas are rare intracranial tumours. Sometimes they occur as a manifestation of a 'generalised chondromatosis'. The authors present a case of sellar chondroma in a patient with Ollier's disease. The tumour showed a mainly suprasellar extension resulting in a chiasmatic syndrome. The pathogenesis of intracranial chondromas, their clinical features, and CT- and MRI-findings are discussed.

Giant Chondroma of the Saddle Area: Case Report and Literature Review*

“…5,15 Embryonic cartilaginous rests from the base of the skull might be misplaced in the developmental stage. 5,13,18 In addition, it is tempting to speculate that basic fibroblast growth factor (b-FGF) secretion, which in turn is stimulated by pituitary tumourtransforming gene (PTTG), may have contributed to the development and progression of the chondroma through a paracrine effect. 4,24 Chondromas are usually very large when diagnosed, so they have varying degrees of invasion into adjacent structures such as the optic nerves, optic chiasm, internal carotid artery, or other cranial nerves.…”

“…5,10,18,20,23,28,30,31 CT and MR imaging findings that contribute to the diagnosis of chondroma are as follows: calcification from granular to massive of the sellar turcica, resulting from varying degrees of degeneration and necrosis; bone destruction of the sellar turcica; marked delayed contrast enhancement on CT scan; hyperintensity on T2-weighted images reflecting the chondroid matrix; and markedly low signal intensity similar to that of cerebrospinal fluid (CSF) on infrared (IR) images. 5,8,10,17,18,25,28,[33][34][35][36][37][38] According to Lacerte et al, intradural chondromas have two distinct types of CT images. 39 One is more common and shows mixed density with minimal or moderate enhancement, the other has a central hypodense area that is composed of cystic degeneration or of a very loosetexture connective tissue without necrosis in pathological assessment.…”

“…5,8,9,10,13,15,18,20,23,[25][26][27][28][29][30][31] The common clinical characteristics of intrasellar chondromas 8,10,13,15,17,18,32 include chiasmatic syndrome associated with irregular bitemporal hemianopsia or inferior quadrantanopsia, decline of visual acuity, and hypopituitarism; the signs of parasellar or clival chondromas are characterized by involvement of the third, fourth, fifth, and sixth cranial nerves, occasionally with incongruous homonymous hemianopsia when the optic tract is invaded. 5,10,18,20,23,28,30,31 CT and MR imaging findings that contribute to the diagnosis of chondroma are as follows: calcification from granular to massive of the sellar turcica, resulting from varying degrees of degeneration and necrosis; bone destruction of the sellar turcica; marked delayed contrast enhancement on CT scan; hyperintensity on T2-weighted images reflecting the chondroid matrix; and markedly low signal intensity similar to that of cerebrospinal fluid (CSF) on infrared (IR) images. 5,8,10,17,18,25,28,[33][34][35][36]…”

“…[4][5][6][7][8][9] According to Falconer et al, skull base chondromas can be divided into three groups: sellar chondromas, parasellar chondromas, and clival chondromas, 10 but even among basal chondromas, sellar chondromas are rare, as no more than 20 cases have been reported to date. 5,[8][9][10][11][12][13][14][15][16][17][18][19] The origin of skull base chondromas is not clear, many theories have been suggested. 2 De Divitiis et al 13 first discussed the origin of intrasellar chondromas in 1979 and they stated that it should be the same as that of intracranial chondromas in general.…”

“…2,37 Coronal CT of bone windows shows multiple small circular and curvilinear calcifications; 60% of intracranial chondromas have mottled shapes and 50% have bone destruction. 2,5,18,20,25,30,31,32,[40][41][42] MR imaging of the chondroma mass depends on the hyaline cartilaginous or ossification content. 5 Vasopressin storage, bone marrow, and adenohypophysial hyperactivity are the three main factors for high T1 signal intensity in normal conditions.…”