Infrasellar craniopharyngioma: case report

Abstract

-We report a case of infrasellar craniopharyngioma in a 34 year-old woman who presented with progressive headache and diplopia. Computed tomographic and magnetic resonance images showed a heterogeneous tumor originating from the sphenoid bone with ethmoid sinus and sella turcica extension. A sublabial rhinoseptal transsphenoidal surgery was performed. Craniopharyngiomas with infrasellar development are very rare. Infrasellar craniopharyngioma is uncommon, thirty-five cases has been reported in literature. The embryology, clinical features and radiographic investigation of these tumors are discussed.KEY WORDS: craniopharyngioma, sphenoid sinus, skull base tumor.Craniofaringioma infra-selar: relato de caso RESUMO -Relatamos um caso de craniofaringioma infra-selar em uma paciente de 34 anos com sintomas de cefaléia e diplopia. A investigação radiológica com tomografia computadorizada e ressonância magnética de encéfalo demonstrou um tumor heterogêneo localizado no osso esfenoidal e com extensão para o seio etmoidal e sela turcica. Realizada cirurgia pela via transesfenoidal. A ocorrência de craniofaringioma com topografia infra-selar é incomum, havendo relato na literatura de 35 casos. A literatura é revisada, sendo discutidas a embriologia, a apresentação clínica e características radiológicas do tumor. . Occasionally, the tumor can occur without sellar involvement. These tumors rarely extend below the sellar floor into the sphenoid sinus or invade the pharynx and the nasal cavities . The infrasellar craniopharyngioma may then originate anywhere along the tract of the obliterated craniopharyngeal duct, which would include the sphenoid bone, vomer, and nasopharynx 35,36 . In this study we report a case of infrasellar craniopharyngioma with the epicenter situated in the sphenoid sinus and review the 35 other reported cases in the literature of this unusual localization since 1924. PALAVRAS-CHAVE CASEA 34 years-old woman presented with frontal headache with progression of 7 months and diplopia that appeared in the last month. Clinical evaluation revealed left abducens nerve palsy. No signs and symptoms of pituitary dysfunction were evident. The laboratory studies, including hypophyseal function were normal.Coronal computed tomographic (CT) scans after intravenous contrast showed a large sphenoid sinus heterogeneous mass with destruction of the ethmoid sinus and sella turcica (Fig 1). T1-weighted (plain and gadoliniumenhanced 600/11/2 [repetion-time/echo time/excitations]) and T2-weighted (3000/108/1) magnetic resonance showed isointense image (Fig 2). Post-contrast the brightly enhancing, heterogeneous mass was clearly visible in the sphenoid bone (Fig 3). There was extension of the tumor to ethmoid sinus and to the sella turcica. Carotid and vertebral angiography revealed that the tumor was avascular.Sublabial rhinoseptal transsphenoidal surgery was performed. After removal of the anterior wall of the sphenoid sinus, a solid firm mass was filled the sphenous sinus. The circumferential wall of the tumor was...

Craniopharyngioma with infrasellar extension in an eight-year boy: An unusual entity

“…Such craniopharyngioma may have completely different presentation from typical endocrinopathy and visual deficit and instead it may present with conglomeration of variable symptoms, which depends on extent of involvement and obstruction of ostia of the paranasal sinuses causing difficulty in breathing, nasal obstruction and sinus headache. These craniopharyngioma with sellar component can also encroach and the sella floor and cause direct compression of pituitary gland or secondary to vascular compromise leads endocrinopathy [10][11][12][13][14][15][16][17]19,21,25,27,28,32]. So, majority of infrasellar reported craniopharyngioma usually had an intrasellar extension.…”

“…About eleven cases of infrasellar craniopharyngioma are reported in the literature, whose either majority of the chief epicenter or wholly tumour is located in the infrasellar region. Craniopharyngioma with such locations have termed with many confusing terminology including these are ectopic basisphenoidal infrasellar [21,28,[31][32][33][34][35][36][37] , pharyngeal [16], nasopharyngeal [17,29,34,38], purely pharyngeal [16], and purely nasopharyngeal located [38]. Our case is unusual in having extensive extension into nasal cavity, nasopharynx, and ethmoid sinus with intracranial component lying above sella causing indenting floor of third ventricle.…”

Isolated craniopharyngioma in the nasal cavity

“…Calcifications are uncommon in the papillary squamous subtype, and prognosis is generally better compared to adamantinomatous tumors. [8,9] Although usual suprasellar craniopharyngioma is not considered to be a malignant neoplasm, [1] aggressive and complete surgical excision is recommended. Recurrence rates are reported to be up to 50% with incomplete tumor excision.…”

Surgical Approaches

“…While craniopharyngiomas can occur anywhere along the craniopharyngeal duct, 90% occur in the sellar and suprasellar region (Falavigna and Kraemer, 2001). Large craniopharyngiomas that arise from this region and invade the third ventricle make the transcortical/ transcallosal-transventricular approach inadequate when gross total resection is the goal.…”

“…The relationship of these tumors to critical neurovascular structures (namely the circle of Willis, optic pathways, and the hypothalamus) results in potential significant morbidity and mortality associated with surgical access (Adamson et al, 1990;. Additionally, even though 90% of tumors arise in the sellar and parasellar regions, they can originate anywhere along the obliterated craniopharyngeal duct, including the nasopharynx, sphenoid bone, third ventricle, pineal region, infrasellar region, and cerebellopontine angle (Falavigna and Kraemer, 2001;Ragel et al, 2007). As understanding of the anatomic variability of this tumor has grown through the use of advanced microsurgical, skull base, and endoscopic techniques, so too has the surgical armamentarium.…”