ODP347 Rare case of intrasellar hemangiomas associated with growth hormone and gonadotropin deficiency
Authors: Sibai Zakaria , Pansare Rahul , Williams Brain , Mokshagundam Sriprakash 
Published: November 2022

Background

Cavernous sinus hemangiomas are rare extra-cerebral vascular lesions and account for 2-3% of all benign lesions in the cavernous sinus. They have a tendency to extend into the intrasellar and suprasellar area but in very rare cases hemangiomas can arise from the sellar region and can be misdiagnosed as pituitary adenoma. Pituitary hormone deficiencies associated with intrasellar hemangiomas has only been reported in 3 cases in the literature. We present a unique case of intrasellar hemangiomas associated with growth hormone and gonadotropin deficiency.

Case presentation

71 year old female with history of primary hypothyroidism and breast cancer, presented with syncopal episode, preceded by facial muscle twitching and followed by urinary incontinence and was hospitalized for seizure evaluation. MRI of brain with and without contrast showed 3.7×2.5×2.6 cm sellar and right parasellar mass occupying the sella turcica, abutting and displacing the internal carotid arteries and causing superior displacement of the optic chiasm. Pituitary axis evaluation: TSH, Free T4, cortisol, ACTH and Prolactin levels within normal range. FSH and LH were low for a post-menopausal woman (1.11 mIU/mL, &lt;0.20 mIU/mL respectively), with undetectable IGF-1 &lt;10ng/mL) and growth hormone &lt;0.1ng/mL. Due to proximity of mass to the carotid artery, patient underwent subtotal mass resection by Endonasal Endoscopic Approach with uneventful post-operative course. Surgical pathology was consistent with Hemangioma. Patient was referred to radiation-neuro-oncology for CyberKnife stereotactic radiation therapy.

Discussion

Cavernous hemangiomas are benign and vascular lesions found in the CNS, within the intra and extra cerebral parenchyma, with incidence rate of 0.5% in the general population. Extra-cerebral hemangiomas are mostly located in the middle cranial fossa with tendency to extend into the cavernous sinus and sellar region. They are less common, have heterogenous clinical presentations, and are more difficult to remove compared to intra-cerebral hemangiomas. Moreover, extra-cerebral hemangiomas have a strong female predominance, attributed to estrogen influence. We reviewed 23 case reports and 8 case series of cavernous sinus/sellar hemangiomas. This included a total of 161 patients, 116(72%) of whom were females. In very rare cases, extra-cerebral hemangiomas arise from intrasellar region and are misdiagnosed as pituitary adenoma. Few cases of intrasellar hemangiomas reported with and without parasellar involvement, presented with headache, vision changes, facial numbness/pain, extraocular muscle palsy and rarely seizures like our patient. Pituitary hormone deficiency was reported in only 3 patients with intrasellar hemangiomas, two with central hypothyroidism and one with central hypothyroidism/hypercortisolism but none associated with growth hormone deficiency and Hypogonadotropic hypogonadism. While radiosurgery alone has shown to reduce tumor size, the most common current approach is surgical resection followed by stereotactic radiosurgery.

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