Ectopic Pituitary Adenoma Associated with an Empty Sella: A Case Report and Review of the Literature

A case of ectopic pituitary adenoma in the sphenoid sinus associated with an empty sella is reported. The coexistence of an ectopic pituitary adenoma and an empty sella is quite rare. The diagnosis was made with an intraoperative finding of the intact dura mater of the sellar floor. In the present case, the hypointense line that coated the pituitary gland was clearly demonstrated on 3‐tesla T2‐weighted magnetic resonance imaging. The hypointense line is considered to be the pituitary capsule and was critical in diagnosing this rare entity.     

Coexistence of ectopic pituitary adenoma and empty sella in a patient with acromegaly : a case report and review of literature

Ectopic pituitary adenoma with an empty sella is extremely rare. We report an unusual patient with an ectopic growth hormone-secreting pituitary adenoma in the sphenoid sinus with an empty sella. The association is related to a development disorder of the anterior pituitary tissues. Tumor in the sphenoid sinus was completely removed by endoscopic endonasal transsphenoidal approach. During the follow-up, the patient met the criteria for endocrinological cure.     

Progressive visual loss because of a suprasellar pneumatocele after trans-sphenoidal resection of a pituitary adenoma.

A 63-year-old man who underwent uneventful trans-sphenoidal resection of a pituitary adenoma with fat packing complained postoperatively of progressive binocular visual acuity loss. Neuroimaging showed a suprasellar pneumatocele compressing the optic chiasm and a communication between the sphenoid sinus and the sella. After a second trans-sphenoidal procedure to remove the air and fully pack the sphenoid sinus, visual acuity recovered dramatically. A rare complication of trans-sphenoidal surgery for pituitary adenoma, suprasellar pneumatocele probably forms through a ball-valve mechanism that results from incomplete packing of the sellar floor. This case highlights the need for effective sphenoid sinus packing and for ophthalmic monitoring after trans-sphenoidal surgery.     

Internal carotid artery aneurysm in the sella turcica area simulating pituitary tumor

A giant aneurysm of the internal artery is described which was situated in the sella and simulated hormonally inactive hypophyseal adenoma. Axial computer tomography demonstrated a hypophyseal tumour. During operation from intranasal approach through sphenoid sinus massive haemorrhage developed but was controlled with fibrin sponge. Postoperative angiography demonstrated a giant aneurysm of the internal artery situated in the sella. Although this location of aneurysm is exceptionally rare, each case of inactive hormonally pituitary adenoma should be subjected before the operation to cerebral angiography.     

Adult hydrocephalus and the empty sella

Abstract– A 51-year-old man presented with a pituitary apoplexy with symptoms and signs predominantly of meningitis. Later on, hydrocephalus and an empty sella were disclosed. It is likely that the empty sella and the hydrocephalus were both caused by the adenoma necrosis. Based on this case and cases reported in the literature, it is suggested that concomitant presence of an empty sella and hydrocephalus results from necrosis in a pre-existing pituitary adenoma.     

内窥镜经蝶窦垂体腺瘤切除术

目的 探讨应用内窥镜经蝶窦入路切除垂体腺瘤的方法及优缺点。方法 自2000年6月～2001年2月对12例垂体腺瘤患者施行内窥镜辅助经蝶窦腺瘤切除手术。经单侧鼻腔鼻中隔入路,在0°角内窥镜下分离鼻粘膜,暴露蝶窦前壁,以中鼻甲下缘和蝶窦开口为界切开蝶窦前壁,打开鞍底,切开硬膜,在内窥镜下探查肿瘤和正常腺体的分界,在手术显微镜下切除垂体腺瘤。结果 术后所肩异常升高的激素水平明显下降,无一例发生严重并发症。结论 内窥镜辅助经蝶窦垂体腺瘤切除术能提供良好的照明和手术视野,是一种安全、可行的手术方法。     

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy

We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis.     

侵袭性垂体腺瘤侵袭性的综合判断及其与临床预后的关系

目的分析经侵袭性垂体腺瘤侵袭性的判断标准及其与临床预后的关系。方法回顾性分析我科自2000年1月至2003年5月行经蝶手术切除的具有完整影像学、病理学和随访资料的侵袭性垂体腺瘤82例,随访时间5～30个月。结果侵袭性垂体腺瘤MR影像学特点为鞍底受侵下陷,部分肿瘤突入蝶窦;颈内动脉包绕;海绵窦受累,鞍隔突破等。病理检查以鞍底硬膜浸润的发生率最高。PRL腺瘤的全切率明显低于无功能腺瘤(P<0.01),无功能腺瘤、GH腺瘤与ACTH腺瘤之间相差不明显。PRL腺瘤的复发率较无功能腺瘤和GH腺瘤高(P<0.05)。结论垂体腺瘤的侵袭性生物学行为导致手术全切几率下降,是术后复发的主要原因。     

Ectopic pituitary adenoma. Case report and review of literature

Ectopic pituitary adenomas are very rare neoplasms. They are usually located in the sphenoid sinus or nasopharynx. Ectopic tumors derive from a residual pituitary fragment in the craniopharyngeal duct. They are similar in morphology, immunohistochemistry and hormonal activity to the typical pituitary adenoma. One third ectopic pituitary tumors reported in the literature are endocrine inactive, while the remaining ones indicate a hormonal activity and usually secrete ACTH, causing the Cushing disease. About 50% of pituitary adenomas are located at the skull base and present as aggressive neoplasms. The computed axial tomography (CAT) and magnetic resonance imaging (MRI) are very useful in revealing the presence of these tumors. Preoperative diagnosis of ectopic pituitary adenomas located at the skull base is difficult. Diagnostic procedures usually suggest another type of neoplasm, e.g. chordoma, nasopharyngeal carcinoma, or a metastatic tumor. Pathological examination including immunohistochemical studies of neuroendocrine markers is important in establishing the diagnosis. A unique case of ectopic, nonfunctioning pituitary adenoma involving the sphenoid sinus and nasopharynx and causing palsy of cranial nerves is presented. MRI examination revealed the presence of a large invasive tumor. The neoplasm was partially resected by transnasal approach. The diagnosis was confirmed by a pathological examination including immunohistochemical smears.     

Need for intrasellar packing in sellar reconstruction of transsphenoidal surgery: Less is more?

BACKGROUND: This study investigated patients with pituitary macroadenomas who received transsphenoidal surgery using two different sellar reconstruction methods, to determine the need for intrasellar packing. METHODS: The authors reviewed 53 patients with pituitary macroadenomas at a single institution between January 1999 and November 2002. On tumour removal, the group 1 patients underwent traditional sellar reconstruction (intrasellar and sphenoid sinus packing), while the group 2 patients did not undergo intrasellar packing. RESULTS: The two groups did not differ significantly in terms of complications. There was no mortality; notably, no delayed empty sella syndrome occurred in the group 2 patients. CONCLUSIONS: Simplified reconstruction of the sella floor using only sphenoid bone without intrasellar packing is thought to be safe and effective. This technique obviates the need for a second surgical incision, risk of overpacking, complications associated with packing material and interference on postoperative magnetic resonance imaging.     

发生脑脊液鼻漏的垂体腺瘤经蝶手术治疗

目的　介绍发生脑脊液鼻漏的垂体腺瘤的临床特征和经蝶手术治疗方法。方法　分析１９９１ 年至１９９７ 年北京协和医院经蝶手术治疗的５ 例发生脑脊液鼻漏的垂体腺瘤, 并结合文献加以讨论。结果　５ 例中４ 例为巨大型泌乳素腺瘤, １ 例为部分空泡蝶鞍合并垂体无功能腺瘤, ２ 例以脑脊液鼻漏为首发症状。术后随访半年至６ 年, ３ 例肿瘤全切除, ２ 例次全切除, 脑脊液鼻漏均消失。结论垂体腺瘤自发性脑脊液鼻漏可见于巨大型垂体腺瘤; 也可见于合并部分空泡蝶鞍的垂体腺瘤; 放疗、服溴隐亭治疗可促进垂体腺瘤脑脊液鼻漏的发生。经蝶入路是最适手术入路; 肿瘤切除后严密填塞蝶窦是补漏的主要措施; 术后去枕平卧和腰蛛网膜下腔引流有助于所修补漏口的愈合。     

Gamma knife radiosurgery for GH-secreting microadenoma with empty sella.

We report a case of a growth hormone secreting microadenoma associated with empty sella treated by gamma knife radiosurgery and we evaluate the effectiveness of radiosurgery for this lesion. A 58-year-old female complained of an acromegalic appearance for 10 years. Magnetic resonance imaging revealed an enlarged sella floor with a compressed, but slightly enlarged pituitary gland. Serum GH values were 8.52 ng/ml. From these results, we diagnosed the presence of a GH-secreting pituitary microadenoma with empty sella. Stereotactic gamma knife radiosurgery was performed to treat the adenoma. The treatment dose was 30 Gy for the tumour margin (50% isodose). The patient was monitored for 5 years without any reported problem. Serum GH values were gradually decreased to 4.1 and 3.5 ng/ml at 1.6 and 3 years. Serum GH values at 5 years after radiosurgery were 1.8 ng/ml within 2 h after glucose load and serum IGF-1 was normalized. We conclude that gamma knife radiosurgery can be an useful alternative treatment modality for GH-secreting adenoma with empty sella in the selected patients when surgery is contraindicated as the primary treatment.     

Spontaneous pneumoventriculogram following radiation of a pituitary adenoma.

Three years after radiation therapy for an intrasellar tumor, a 42-year-old housewife presented with headache, lethargy, and remarkable plain skull roentgenograms, in which dilated lateral and third ventricles were filled with air. Air apparently had entered the cranium through the sphenoid sinus and eroded sellar floor, extending directly through intrasellar remnants of the chromophobe adenoma and into the floor of the third ventricle. Frontal exploration showed an empty sella turcica and no residual tumor. She made an excellent recovery and has done well for 5 years after operative closure of the defect.     

Primary pituitary abscess: case report

Pituitary abscesses are potentially life-threatening lesions if not appropriately diagnosed and treated. The authors have operated on more than five hundred cases of pituitary tumors and only one represented a case of pituitary abscess. A 35-year-old woman was investigated for chronic frontal headache. CT scan showed a cystic sellar lesion with ring enhancement after contrast injection leading to an initial diagnosis of pituitary adenoma. She underwent a sublabial transsphenoidal approach to the pituitary gland. After dural opening, purulent material was obtained and no tumor or other associated lesion was detected. There was no evidence of current or previous septicemic illness, meningitis, cavernous sinus thrombosis or sinus infection. Cultures were negative. She was put on antibiotics and discharged after 4 weeks. Nowadays, 10 years after treatment, she is doing well, with no anterior pituitary hormone deficit. MRI shows a partially empty sella without residual lesion and the pituitary stalck is in the midline. The early diagnosis and adequate treatment of this life-threatening lesion may result in excellent prognosis.     

The empty sella and pituitary adenomas

In 44 consecutive patients with sellar volume larger than 1100 mm³, computer tomography showed that 20 had an empty or partly empty sella. None had radiological evidence of a suprasellar tumor. 10 of the 20 patients had experienced episodes with acute neurological symptoms presumably reflecting a pituitary apoplexy. It is suggested that an unknown proportion of intrasellar adenomas may disappear as a result of an infarction, which may comprise the entire adenoma or part of it - leaving an empty or partly empty sella as diagnosed by computer tomography. Air encephalography will demonstrate the empty sella only if the diaphragmatic aperture is large enough to allow cisternal herniation. The infarction may present clinically with no, slight or severe acute neurological symptoms. Late consequences of a pituitary adenoma infarction may be rhinorrhea or hydrocephalus.     

Cerebrospinal fluid fistula as the presenting manifestation of pituitary adenoma: case report with a 4-year follow-up

We report the case of a young woman who presented with cerebrospinal fluid (CSF) rhinorrhea due to an undiagnosed and untreated pituitary adenoma. The tumor had extended well beyond sella turcica and caused bony erosion. The patient initially refused surgery and was treated with bromocriptine and a radiation therapy. CSF leakage did not improved and she was submitted to surgery by the transsphenoidal approach with removal of a tumor mass located in sphenoid sinus and sellar region. Origin of the leak was localized and repaired with fascia lata and a lumbar subarachnoid drain was left in place for 5 days. After 4 years she has normal serum PRL levels and no rhinorrhea. The management, complications and mechanisms involved in this rare condition are discussed.     

Ectopic pituitary fossa thyroid tissue.

Developmental anomalies of the thyroid gland may result in ectopic glandular tissue. We report a unique case of ectopic thyroid tissue within the pituitary fossa and sphenoid sinus, associated with a lingual thyroid gland. The aetiology of this ectopic tissue is postulated to be ascent of primitive thyroid endoderm at the time of formation of Rathke's pouch.     

Pseudotumor cerebri. A theory on etiology and pathogenesis

Ten patients with pseudotumor cerebri were studied. The diagnosis was based on bilateral papilledema and absence of intracranial masses and hydrocephalus. Based on the radiological findings, three groups of patients emerged: (A) Five patients with an empty sella. Four had an enlarged sella; (B) Two patients with a pituitary adenoma; and (C) Three patients without sellar or pituitary pathology. The onset of symptoms was acute in four patients, two from group A and one each from groups B and C. One patient from group A had a lesion of the chiasma. Two patients from group B and two from group C had symptoms and signs suggesting engagement of structures in the cavernous sinus. It is suggested that pseudotumor cerebri may be caused by bilateral compression of the cavernous sinuses because of acute necrotic swelling of a preexisting pituitary adenoma.     

MRI in the diagnosis of so-called pituitary apoplexy: seven cases

The authors describe seven cases of pituitary adenoma with acute neurological symptoms (the so-called pituitary apoplexy), which were examined by magnetic resonance imaging (MRI). Four patients underwent surgery and the diagnosis of pituitary adenoma was histologically confirmed. In 3 patients, conservative treatment was decided and serial MRI examinations showed progressive decrease in size of the tumour, leading in one case to an empty sella. Computerized tomography (CT) was positive in all 7 cases, but less contributive than T1-weighted MRI images both for tumoral volume determination and detection of intratumoral haemorrhage. Although theoretical problems may arise concerning the diagnosis of acute intratumoral bleeding at an early stage, the authors emphasize the value of MRI in showing the extension of the tumor into the retroclival cistern and the cavernous sinus, especially when surgery is not performed in the acute period.