Ectopic Pituitary Adenoma Research Articles

Ectopic pituitary null cell adenoma arising from the infundibulum in the third ventricle: A successful endonasal transsphenoidal resection after long-term follow-up MR imaging – A technical note

Abstract Background Since the origin and growth pattern of third ventricle ectopic pituitary adenoma (ectPA) remain unclear, its optimal surgical approach is debatable. Clinical presentation We present a rare case of null cell pituitary adenoma arising from the pituitary infundibulum with long-term preoperative follow-up images. The tumor was resected gross-totally via an extended transsphenoidal approach. Conclusion To our best knowledge, this is the first case with long-term preoperative follow-up images, which can bridge the knowledge gap in operations of third ventricle ectPA as following: (1) Truly third ventricle ectPA can exist, (2) the third ventricle ectPA extended into the sella turcica along the pituitary stalk, (3) this ectPA can arise from the suprasellar peri-infundibular ectopic pituitary cells or the pars tuberalis of the adenohypophysis, and therefore adhere to the optic chiasm, (4) thus neurosurgeons should take great care in resection of ectPA arising from the infundibulum, and (5) it can be resected through an endoscopic extended transsphenoidal approach.

‘Ectopic’ suprasellar type IIa PRL-secreting pituitary adenoma

Ectopic pituitary adenomas (EPAs) are rare, and the suprasellar cistern seems to be the most common location. At this time, no detailed original classification, diagnosis, or treatment protocols for suprasellar pituitary adenomas (SPAs) have been described. A 19-year-old man showed visual disturbances and lack of libido for 3years, he suffered a sharp decline in vision with only light perception in the last week. Magnetic resonance imaging scans revealed a large suprasellar cystic lesion with a normal pituitary in the sella turcica. Endocrinological findings showed an extremely high prolactin level of 1250ng/mL. Because of the sharp decline in vision, the patient underwent total removal of the suprasellar lesion using a transfrontal interhemispheric approach. The tumor pedicle originated in the lower pituitary stalk without any connection to the anterior pituitary gland in the sella turcica, while the diaphragma sellae was incomplete. Clinical and endocrinological cure criteria were fulfilled and postoperative pathology confirmed a prolactin-secreting pituitary adenoma. Ectopic suprasellar pituitary adenomas (ESPAs) are extremely rare intracranial extracerebral tumors. SPAs can be classified into three types according to their origin and their relationship with surrounding tissue. Only type III is theoretically a true ectopic, based on previous reports. Thus, ESPAs are uncommon compared to other EPAs. Our case is the first reported case of a type IIa 'E'SPA and the first description of this subtype classification until now. The pars tuberalis may be different from the pars distalis, and each subtype of adenohypophyseal cells may have different migration characteristics, which leads to different proportions of each hormone-secreting subtype in SPAs and EPAs. Transsphenoidal surgery is minimally invasive, but transcranial surgery may remain a universal option for the treatment of suprasellar lesions.

Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: An Underestimated Entity.

The diagnosis of Cushing disease is based on endocrinological pa-rameters, with no single test being specific. In some patients, dynamic thin-slice sellar magnetic resonance imaging fails to detect a pituitary tumor. The purpose of this study is to investigate the role of ectopic pituitary adenoma in this situation. In a retrospective chart review, 5 patients (6%) with ectopic adenomas were identified in 83 consecutive patients undergoing transsphenoidal surgery for adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas by 1 surgeon. In all 5 patients (all female, 32-41years of age), an exclusively extrasellar ACTH-secreting adenoma was excised. Three adenomas were located in the cavernous sinus, 1 in the sphenoid sinus, and 1 in the ethmoidal cells. Histologically, none of the tumors showed signs of aggressiveness. Three of the 5 adenomas specifically expressed somatostatin receptor 5. In 4 patients with Cushing disease, postoperative remission was obtained, with 1 recurrence after 14months. In the patient with Nelson syndrome, ACTH decreased from >800 to <80pg/mL. Three patients underwent previous surgery elsewhere, including 1 hypophysectomy. In this case, the ectopic adenoma (positive for somatostatin receptor 5) in the ethmoidal cells turned out to be positive on gallium 68 DOTATATE positron emission tomography/computed tomography. The incidence of primarily ectopic ACTH-secreting adenomas in this series was 6%. In cases of negative MRI findings, an ectopic ACTH-secreting adenoma should be taken into account. 68 Ga DOTATATE positron emission tomography/computed tomography may identify ectopic pituitary adenomas. Hypophysectomy should always be avoided in primary surgery for CD.

Ectopic Pituitary Adenoma: Case Report with Review of Literature

AbstractEctopic pituitary adenoma (EPA) is a rare presentation first described by Erdheim. Most of the EPAs described in literature occur in the sphenoid sinus or suprasellar region. We describe a case of an EPA originating from the choana presenting as a case of nasal mass along with detailed literature review.

Ectopic Pituitary Adenomas Presenting as Sphenoid or Clival Lesions: Case Series and Management Recommendations.

Background An ectopic pituitary adenoma presenting as a clival or sphenoid mass is a rare clinical occurrence that may mislead the clinician and result in unnecessary interventions or potential medicolegal consequences. Here, we present one of the largest multi-institutional case series and review the literature with an emphasis on radiological findings and critical preoperative workup. Methods Retrospective chart review. Results Nine patients were identified with ectopic pituitary adenomas of the sphenoid or clivus. There were four females and five males. Median age was 60 years old (range, 36-73 years). The most common presenting symptom was headache (56%). Five (56%) patients presented with a mass arising from the clivus while four (44%) presented with a mass in the sphenoid. Six (67%) patients demonstrated biochemical evidence of hypersecretion on full endocrinology panel. All masses showed evidence of enhancement with gadolinium with a propensity for adjacent bone involvement. Lesions also had a predilection for growth toward the cavernous sinus, carotid artery, or sellar floor. Surgical intervention was performed in eight patients (89%). In eight patients (89%), tumors demonstrated immunoreactivity to prolactin. Conclusions Pituitary adenomas can rarely present as an isolated sphenoid or clival mass. Lesions displayed similar magnetic resonance imaging findings with an erosive growth pattern toward the sellar floor, cavernous sinus, or adjacent carotid artery. Patients with clival or parasellar lesions with comparable features should have a preoperative workup which includes prolactin level and alert the physician to consider an ectopic pituitary adenoma in the differential to prevent unnecessary surgery and potential complications.

Abstract #921: Ectopic Growth Hormone Secreting Pituitary Adenoma Presenting as Chronic Sinusitis

Abstract #921: Ectopic Growth Hormone Secreting Pituitary Adenoma Presenting as Chronic Sinusitis

Ectopic prolactinoma in the clivus: a case report.

We report the case of an ectopic pituitary adenoma in a 65-year-old man with an empty sella who initially presented with right ptosis and eyelid edema and headache. Neuroimaging studies revealed a large tumoral process at the height of the clivus, with partial destruction of surrounding bone structure. He underwent transphenoidal surgery and histopathologic examination, including immunohistochemical studies, revealed a prolactin-producing pituitary adenoma. A careful review of the literature was done.

Ectopic Functional Adenoma in the Cavernous Sinus: A Diagnostic and Surgical Challenge

Object: Ectopic pituitary adenomas are extremely rare and mostly arise in the proximity of the pituitary gland, e.g., the cavernous sinus (CS), the pituitary stalk or the suprasellar space. Their location may be a common cause of failed diagnosis and unsuccessful transsphenoidal surgery. We present a case of mixed ACTH- and PRL-secreting adenoma located in the left CS.

Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature.

Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury) and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20). Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment.

Acromegaly With an Ectopic Clival Pituitary Adenoma

Ectopic pituitary adenomas usually occur within the sphenoid sinus or nasopharynx and seldom within the clivus. In this report, we describe the fourth case of ectopic growth hormone (GH)-secreting adenoma in the clivus. A 49-year-old female patient was admitted with acromegaly and blurred vision. Biochemical evaluation revealed a notable elevation in the GH level at >40 ng/mL (normal range, 0.0 to 5.0 ng/mL). A magnetic resonance imaging scan of the pituitary showed a mass below the sellar turcica. The patient underwent an endoscopic transnasal transsphenoidal approach to the sella, and the tumor in the clivus was completely removed. The tumor was separated from the intrasellar contents by intact bone of the sella. Histologic examinations of the resected tumor showed a chromophobe pituitary adenoma, and immunocytochemistry was positive for GH adenoma. Including our case, only 16 reported ectopic adenomas have occurred in the clivus. Relevant literature is briefly reviewed.

The Endoscopic Endonasal Transtubercular Route to the Suprasellar Cistern: Surgical Nuances and Rationale for Periinfundibular Tumors

Introduction: Suprasellar lesions surrounding the pituitary stalk such as ectopic pituitary adenoma and craniopharyngiomas represent challenging pathologies in terms of surgical access. The classical intracranial routes (pterional, subfrontal) require brain retraction and dissection of neurovascular structures (optic nerves, carotid arteries, and their branches). We detail the transtubercular supradiaphragmatic approach to the periinfundibular region in a minimally invasive manner. Methods: We present two illustrative cases among a series of more than 1,000 endoscopic endonasal procedures, one supradiaphragmatic ectopic adenoma, and one craniopharyngioma, totally resected through this approach sparing the pituitary gland and stalk. Surgical nuances, tips and tricks are detailed. Conclusion: The endonasal endoscopic presellar transtubercular approach provides an excellent route to the supradiaphragmatic area (pituitary stalk, inferior aspect of optic chiasm, anterior communicating artery complex) without manipulating the neurovascular surrounding structures. A multilayer rigorous closure prevents CSF leakage.

Ectopic ACTH-secreting pituitary adenomas within the sphenoid sinus.

Ectopic pituitary adenomas are defined by the presence of adenomatous pituitary tissue outside the sella and distinctly separate from the pituitary gland. Ectopic ACTH-secreting pituitary adenomas (EAPAs) are a rare cause of Cushing's syndrome. Detecting these radiologically can prove difficult, in part, due to their typically small size and unpredictable anatomical location. In ACTH-dependent Cushing's syndrome, if, despite comprehensive testing, the source of excess ACTH remains occult (including negative work up for ectopic ACTH syndrome) thought should be given to the possibility of the patient harboring an EAPA. In most cases, ectopic ACTH pituitary adenomas within the sphenoid sinus will manifest with symptoms of hormonal excess, have an obvious sphenoid sinus mass on pre-operative imaging and will demonstrate resolution of hypercortisolism after surgical excision if located and removed. Twenty cases of EAPAs have been reported in the literature to date. This paper will review the current literature on all previously reported EAPAs within the sphenoid sinus in addition to the current case.

Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: case report with literature review

Ectopic pituitary adenomas are uncommon entities that may pose substantial diagnostic challenges. In the majority of these cases, patients present with endocrine and/or nasal obstruction symptoms. We report the case of an ectopic pituitary adenoma in a 76-year-old man with an empty sella who initially presented with right-sided hearing loss progressing to bilateral hearing loss over the next 4 years. Neuroimaging studies revealed a large, expansile central skull base mass replacing the clivus and sphenoid sinus, and invading the internal auditory canals and inner ear bilaterally. The tumor also involved the floor of the middle cranial fossae and bilateral medial temporal and occipital bones. Histopathologic examination, including immunohistochemical studies, revealed a sparsely granulated lactotroph adenoma. Hearing loss in a patient with ectopic pituitary adenoma constitutes an extremely unusual presentation. This case was further complicated by the presence of an empty sella and the absence of symptoms related to hyperprolactinemia.

Cushing-type ectopic pituitary adenoma with unusual pathologic features

Ectopic pituitary adenomas comprise, by varying reports, approximately 1-2% of all pituitary adenomas. They are often located in the nasopharyngeal region associated with the pharyngeal pituitary. The location and pathologic features of these masses make them atypical when compared with intrasellar pituitary adenomas. A 54-year-old man presented with vertebral compression fracture and physical stigmata of Cushing’s disease. Biochemical testing confirmed hypercortisolemia responsive to high dose dexamethasone suppression. MRI of the head demonstrated an enhancing mass in the posterior aspect of the sphenoid sinus not involving the sella turcica. Endoscopic biopsy followed by resection confirmed this mass to be a pituitary adenoma with unusual pathologic features. Most notably, the tumor cells demonstrated large, eosinophilic, vacuolated cytoplasm. Immunohistochemical profile of the tumor was typical of an ACTH secreting tumor, notably with positivity for ACTH. The patient did well from his surgery. Post-operatively his serum cortisol level normalized and he remains in chemical remission one year after surgery. Ectopic pituitary adenomas are an unusual manifestation of hormonally active pituitary neoplastic disease. Their atypical clinical presentations, location, and pathologic features can make them a diagnostic challenge. Clinicians should be aware of these entities, especially when considering differential diagnosis for a mass in the sphenoid sinus and nasopharyngeal region. • Ectopic pituitary adenomas frequently arise in the nasopharyngeal region. • These tumors may be associated with the pharyngeal pituitary. • The pharyngeal pituitary is a normal anatomical variant arising in the nasopharynx that may undergo neoplastic transformation. • Ectopic pituitary adenomas frequently have atypical pathologic features, including vacuolar change that may cause them to be mistaken for another, more aggressive tumor type. • In the case presented, electron microscopy was a useful adjunct to identify and diagnose the tumor as an ectopic pituitary adenoma.

Ectopic ACTH-secreting pituitary adenomas

The diagnosis of Cushing's disease (CD) is based on several endocrinological parameters, with no single test being specific. In a certain amount of patients MRI fails to detect a pituitary tumor. Extensive pituitary exploration may be necessary. Sometimes hypophysectomy is advocated, ignoring the possibility of ectopic pituitary adenoma.

Nonfunctioning Ectopic Pituitary Adenoma Presenting as Epistaxis: A Report of Two Cases

ABSTRACTEctopic pituitary adenomas are uncommon lesions and are found along the migratory pathway of the Rathke's pouch. Sites reported include suprasellar region, clivus, sphenoid sinus, nasopharynx, third ventricle, petrous temporal bone, hypo thalamus, etc. Compared to intrasellar adenomas, a higher proportion of the ectopic examples are functional and most commonly produce adenocorticotropic hormone (ACTH). The authors report two cases of ectopic pituitary adenoma in the sphenoid sinus in two male patients 36 and 40 years old, presenting with epistaxis. Both the patients did not have any endocrine abnor malities. The clinical and imaging findings were suggestive of sinonasal malignancy. The final diagnosis was made after histopathological examination and immunohistochemistry for cytokeratin, chromogranin and pituitary hormones. The diagnosis of ectopic pituitary adenomas is difficult especially in those tumors that are nonfunctioning. After extensive literature search, we could find only six cases of nonfunctioning adenomas reported in the sphenoid sinus and in all these cases the correct diagnosis could be made only by histopathology.How to cite this articleGovindan A, Sasi P, Radhakrishnan S, Alapatt JP, Aravindan KP. Nonfunctioning Ectopic Pituitary Adenoma Presenting as Epistaxis: A Report of Two Cases. Int J Otorhinolaryngol Clin 2014;6(3):130133.

3 cases of ectopic ACTH pituitary adenoma and literature review

Objective To evaluate the clinical and laboratory features of 3 cases of ectopic ACTH pituitary adenoma (EAPA). Methods 3 cases of EAPA confirmed by surgery and pathological,examination have been retrospectively investigated in Peking Union Medical College Hospital since 1994.There were 2 female and 1 male patients with age ranging between 17 ～ 22y. The relevant literatures of EAPA were reviewed.Results The level of plasma ACTH was 126 ～ 207 pg/ml.The result of high dose dexamethasone suppression test was not suppressed in 1 case. The localization of EAPA tumor was suprasellar in 2 cases and left cavernous sinus in lcase. The results of inferior petrosal sinus sampling (IPSS) showed the ratio of inferior petrosal sinus (IPS):Peripheral (P) of plasma ACTH was 10.26 for 1 case with suprasellar EAPA and ＜ 2 for the other 1 case with left cavernous sinus EAPA.The pathological examination confirmed the diagnosis of EAPA with positive immunohistochemical staining of ACTH for all 3 cases after operation.The level of plasma ACTH and urine free cortisol decreased obviously after operation.Conclusions EAPA should be considered in the differential diagnosis for all patients with Cushing 's syndrome.The diagnosis and treatment of EAPA tumor are both difficult to carry out.IPSS may be helpful for the diagnosis of EAPA. Key words: Cushing syndrome; Cushing disease; ACTH syndrome, ectopic; Petrosal sinus sampling

Ectopic growth hormone-secreting pituitary adenoma involving the clivus treated with octreotide: role of magnetic resonance imaging in the diagnosis and clinical follow-up

Ectopic pituitary adenomas (EPAs) are rare neoplasms, mainly described in the sphenoid sinus (SS) and suprasellar region; hormone-secreting EPAs with clival involvement have been rarely reported. We describe a case of a pharmacologically treated GH-secreting EPA involving the clivus and SS, with satisfactory clinical outcome and tumor size reduction. As transsphenoidal resection is the standard approach, post-treatment imaging findings in the nonsurgical management of this entity, to our knowledge, have not been reported before. Ectopic growth hormone-secreting pituitary adenoma involving [...]

Ectopic pituitary adenoma with empty sella in the setting of MEN-1 syndrome: detection with 68Ga-DOTANOC PET/CT

Patients with multiple endocrine neoplasia type-1 syndromes are known to have neuroendocrine tumors (NETs) involving the pituitary and gastroenteropancreatic region, in addition to the presence of parathyroid abnormalities. In rare instances adenomas in the ectopic pituitary gland have been reported. As pituitary gland and pituitary adenomas are known to express SSTRs, somatostatin receptor scintigraphy can be used for imaging. Somatostatin receptor-based PET/CT imaging using 68Ga-DOTANOC has become a popular noninvasive imaging modality for evaluation of patients with NETs. The application of 68Ga-DOTANOC PET/CT can be extended to multiple endocrine neoplasia type 1 patients, as is evident from this case study.

Ectopic Sphenoid Sinus Pituitary Adenoma (ESSPA) with Normal Anterior Pituitary Gland: A Clinicopathologic and Immunophenotypic Study of 32 Cases with a Comprehensive Review of the English Literature

Ectopic sphenoid sinus pituitary adenoma (ESSPA) may arise from a remnant of Rathke's pouch. These tumors are frequently misdiagnosed as other neuroendocrine or epithelial neoplasms which may develop in this site (olfactory neuroblastoma, neuroendocrine carcinoma, sinonasal undifferentiated carcinoma, paraganglioma, melanoma). Thirty-two patients with ESSPA identified in patients with normal pituitary glands (intact sella turcica) were retrospectively retrieved from the consultation files of the authors' institutions. Clinical records were reviewed with follow-up obtained. An immunohistochemical panel was performed on available material. Sixteen males and 16 females, aged 2-84years (mean, 57.1years), presented with chronic sinusitis, headache, obstructive symptoms, and visual field defects, although several were asymptomatic (n=6). By definition, the tumors were centered within the sphenoid sinus and demonstrated, by imaging studies or intraoperative examination, a normal sella turcica without a concurrent pituitary adenoma. A subset of tumors showed extension into the nasal cavity (n=5) or nasopharynx (n=9). Mean tumor size was 3.4cm. The majority of tumors were beneath an intact respiratory epithelium (n=22), arranged in many different patterns (solid, packets, organoid, pseudorosette-rosette, pseudopapillary, single file, glandular, trabecular, insular). Bone involvement was frequently seen (n=21). Secretions were present (n=16). Necrosis was noted in 8 tumors. The tumors showed a variable cellularity, with polygonal, plasmacytoid, granular, and oncocytic tumor cells. Severe pleomorphism was uncommon (n=5). A delicate, salt-and-pepper chromatin distribution was seen. In addition, there were intranuclear cytoplasmic inclusions (n=25) and multinucleated tumor cells (n=18). Mitotic figures were infrequent, with a mean of 1 per 10 HPFs and a <1% proliferation index (Ki-67). There was a vascularized to sclerotic or calcified stroma. Immunohistochemistry highlighted the endocrine nature of the tumors, with synaptophysin (97%), CD56 (91%), NSE (76%) and chromogranin (71%); while pan-cytokeratin was positive in 79%, frequently with a dot-like Golgi accentuation (50%). Reactivity with pituitary hormones included 48% reactive for 2 or more hormones (plurihormonal), and 33% reactive for a single hormone, with prolactin seen most frequently (59%); 19% of cases were non-reactive. The principle differential diagnosis includes olfactory neuroblastoma, neuroendocrine carcinoma, melanoma, and meningioma. All patients were treated with surgery. No patients died from disease, although one patient died with persistent disease (0.8months). Surgery is curative in the majority of cases, although recurrence/persistence was seen in 4 patients (13.8%). In conclusion, ESSPAs are rare, affecting middle aged patients with non-specific symptoms, showing characteristic light microscopy and immunohistochemical features of their intrasellar counterparts. When encountering a tumor within the sphenoid sinus, ectopic pituitary adenoma must be considered, and pertinent imaging, clinical, and immunohistochemical evaluation undertaken to exclude tumors within the differential diagnosis. This will result in accurate classification, helping to prevent the potentially untoward side effects or complications of incorrect therapy.