Ectopic Pituitary Tumors Research Articles

Ectopic Pituitary Neuroendocrine Tumors/Adenomas Around the Sella Turcica

Functional or non-secretory ectopic pituitary neuroendocrine tumors (PitNET) can form around the sella turcica during the development of the adenohypophysis by differentiating and detaching from the pharyngeal roof. These tumors usually appear in the sphenoid sinus, clivus, cavernous sinus, infundibulum, and suprasellar cistern. Ectopic PitNETs typically display the characteristic magnetic resonance imaging findings of pituitary adenomas. However, preoperative diagnosis of PitNETs is usually challenging because of the variety of clinical and imaging presentations, locations, and sizes. Ectopic suprasellar PitNETs resemble mass lesions in the pituitary stalk. Ectopic cavernous sinus of PitNETs are typically microadenomas in the medial wall. Ectopic sphenoclival tumors are characterized by more aggressive tumor activity than the other ectopic PitNETs. Although ectopic PitNETs are exceedingly rare, they should be considered as a differential diagnosis for masses around the sella turcica. Treatment of the disease should be individualized and may include medical care, surgical resection, gamma-knife radiosurgery, and radiotherapy.

Comparative epigenomics indicate a common origin of ectopic and intrasellar corticotroph pituitary neuroendocrine tumors/adenomas: a case report.

Ectopic pituitary neuroendocrine tumors (PitNET)/adenomas are rare and diagnostically challenging extra-sellar tumors. Previous studies have demonstrated the impact of epigenomic analyses in the diagnostics of sellar neoplasms and characterized the close relationship of epigenomic signatures and cellular origins of PitNET/adenomas. As of today, little is known about the pathogenesis of ectopic PitNET/adenomas, and epigenomic analyses have not been performed in these rare tumors. We report on the clinical course of an 81-year-old patient with sphenoid ectopic sparsely granulated corticotroph PitNET/adenoma and deploy genome-wide DNA methylation analysis to compare its methylation profile to a reference cohort of sellar neoplasms. Genome-wide methylation analysis revealed an epigenomic profile analogous to reference sellar corticotroph PitNET/adenomas, and the copy number variation profile showed loss of chromosomes 18 and 22. The methylation profile shows concordance with sellar corticotroph PitNET/adenomas suggesting a common cellular origin and confirming the reliability of methylation analyses as a diagnostic method in these rare tumors. This is the first data suggesting that epigenetic profiles of ectopic PitNET/adenoma do not differ from their sellar counterparts.

Ectopic Pituitary Neuroendocrine Tumor: A Case Report Written With the Help of ChatGPT

Ectopic Pituitary Neuroendocrine Tumor: A Case Report Written With the Help of ChatGPT

Persistent Hypokalemia Induced by an Intracavernous Ectopic Pituitary Neuroendocrine Tumor.

Persistent Hypokalemia Induced by an Intracavernous Ectopic Pituitary Neuroendocrine Tumor.

Top 10 Nested Pattern Head and Neck Lesions to Notice.

Nested is defined as "cellular clusters arranged in small groupings with intervening vascular or stromal networks, lacking lumens or glandular formation." Using this definition, multiple neoplastic and non-neoplastic lesions of the head and neck come into the differential. We have broadly organized the differential diagnosis of "nested" tumors into entities with neuroendocrine differentiation, squamous differentiation, thyroid follicular cell differentiation, and other lesions. Review. Many different entities have a nested appearance and the morphologic, immunohistochemical, clinical, and radiographic features contribute to the differential diagnosis. The different tumors covered in this review include neuroendocrine neoplasms, paraganglioma, middle ear neuroendocrine tumor (formerly known as middle ear adenoma), medullary thyroid carcinoma, poorly differentiated thyroid carcinoma, olfactory neuroblastoma, ectopic pituitary neuroendocrine tumor, hyalinizing trabecular tumor, solid subtype of papillary thyroid carcinoma, solid cell nests/C-cell hyperplasia, necrotizing sialometaplasia, and meningioma. In this review, we discuss the morphologic and immunohistochemical features of the covered entities as a guide to differential diagnosis when nested-patterned head and neck lesions are encountered.

Ectopic pituitary adenoma: a rare and unexpected diagnosis, clinical presentation and review of the literature

<p>Nasal obstruction can be caused by many causes. Most common cause of nasal obstruction is deviated nasal septum followed by nasal masses. Nasal masses can be polyps, tumors or foreign bodies. We are presenting a case of ectopic pituitary tumor as a as cause of nasal obstruction along. Ectopioc pituitary tumors are rare tumors and can present in nasal cavity causing nasal symptoms. They can cause unilateral or even bilateral nasal obstruction depending on their spread in nasopharynx and nose and should be kept in mind while treating a case of nasal masses.</p>

Pituitary tumors: epidemiology and clinical presentation spectrum

Pituitary tumors (PTs) are a heterogeneous group of lesions of the central nervous system that are usually benign. Most of them occur sporadically, but 5% can do so within family syndromes, usually at a young age. There are differences by sex, age, race, and genetic factors in the prevalence of different tumor cell types and clinical presentation. Functioning-PTs (FPTs) are usually diagnosed earlier than non-functioning PTs (NFPTs). However, this depends on the PT type. Headaches and visual disturbances are the most frequent mass-effect symptoms, but seizures or hydrocephalus may also occur. Pituitary apoplexy is another possible mode of presentation, and it requires special attention because of its potential severity. PTs in pregnancy, childhood, and old age present a series of clinical peculiarities that must be taken into account when evaluating these patients. Ectopic PTs (EPTs) are uncommon and share the same clinical-epidemiological data as eutopic PTs, but, depending on their location, other types of clinical manifestations may appear. Silent PTs are often detected as an incidentaloma or due to neurologic symptoms related to mass-effect. Aggressive PTs and pituitary carcinomas (PCs), which are very rare, are characterized by multiple local recurrences and metastases, respectively. This review addresses the epidemiology and clinical presentation of PTs, from the classical hormonal and mass-effect symptoms to the different rare presentations, such as pituitary apoplexy, hydrocephalus, or diabetes insipidus. Moreover, special situations of the presentation of PTs are discussed, namely, PTs in pregnancy, childhood, and the elderly, EPTs, silent and aggressive PTs, and PCs.

In the nose, not the sella: Case report of an ectopic pituitary adenoma

Abstract Background and importance Ectopic pituitary adenomas presenting in the nasal and paranasal sinuses are very rare. We report a patient with a large ectopic nonfunctioning pituitary macroadenoma involving the nasal passageways, ethmoid sinus, and extending through the cribriform plate extradurally to the anterior fossa with no involvement of the sella turcica. The patient presented with epistaxis and denied any visual disturbances or other associated clinical presentations that would demonstrate extensive cranial nerve involvement. Ectopic pituitary adenomas should be considered in the differential diagnosis of a nasal mass involving the nasal sinuses, even if there is no communication with the sella. Clinical presentation The patient is a 46-year-old male who presented with complete nasal obstruction. MRI revealed a large bilateral nasal mass extending into the anterior fossa through the cribriform plate. On nasal endoscopy, the patient was found to have a large mass occluding the entire nasal cavity. Subsequently, an endoscopic endonasal resection was performed. Conclusion This case represents a rare presentation of an ectopic pituitary adenoma. The extensive tumor involvement of the nasal passageways, ethmoid sinuses, and the anterior fossa with the absence of cranial nerve involvement are notable. Histopathologic confirmation of the ectopic pituitary tumor was made after complete endoscopic endonasal removal.

Ectopic prolactinomas and somatotropinomas

Introduction Ectopic pituitary tumors (EPT) are very rare comparing to eutopic ones. Their mechanism is still debated. Our aim was to report 10 cases observed in our practice. Cases series Six prolactinomas and 4 somatotropinomas were collected. They were located in the suprasellar area for 3: two prolactinomas and one somatotropinoma, in the clivus for 3: 2 prolactinomas and one somatotropinoma, or in the sphenoid sinus n = 4: 2 prolactinomas and 2 somatotropinomas. For the infrasellar lesions, the diagnosis was relatively easy as the tumors were visible in infrasellar position and were associated to an empty sellar suggesting an inhibition of the pituitary gland by an ectopic hormonal secretion. Regarding suprasellar lesions, the 3 tumors invaded the pituitary area. The diagnosis was made in retrospect after tumor shrinkage due to medical treatment. Actually, MRI done after dopamine agonists or somatostatin analogues showed a quick disappearance of the intrasellar part of the tumor first, and then it was the suprasellar part turn. After the last control, only a very small residual tumor was visible in the suprasellar area. On the therapeutic point, we remarked that inhibition of prolactin and growth hormone secretion was similar in both positions; however tumor shrinkage was better in suprasellar tumors. Conclusion Those 10 unusual cases should be known for their rarity, their unknown physiopathology and their response to medical treatment.

Immunohistochemical survey of migration of human anterior pituitary cells in developmental, pathological, and clinical aspects: a review

Developmentally pathological conditions of the anterior pituitary cells include failed separation of the primary pituitary gland into sellar and pharyngeal ones, ectopic migration into the subarachnoid space, and basophil invasion into the posterior lobe although the last is a physiological phenomenon with pathological potentiality in certain circumstances. Pituitary primordium appears at about 4 weeks of gestation. One of the causes of the pituitary gland agenesis may be a formation of the primary hypothalamic ganglionic hamartoma just at the time of occurrence of the pituitary primordium, as analyzed in cases of Pallister-Hall syndrome. A double pituitary in a single individual is a rare malformation. Its pathogenesis is considered as a result of notochordal anomaly. In the 8th gestational week, the primary pituitary gland separates into sellar and pharyngeal parts. The disturbance of this histogenesis results in a rare pituitary malformation, a “pharyngosellar pituitary.” Despite the failed separation in this case, differentiation of the pituitary cells proceeds and the hormone production of this malformed pituitary gland can be displayed immunohistochemically. In this case, the distribution of the different hormone producing cells was atypical, particularly in those of gonadotropic hormones and ACTH. Life-long existence of the pharyngeal pituitary is a normal anatomical state in humans. Cell differentiation (hormone production) in the pharyngeal pituitary occurs about 4–10 weeks later than in the sellar pituitary. In pharyngeal pituitary, all kinds of adenohypophyseal hormones are produced. Extracranial pituitary adenomas (with intact sellar pituitary), exclusively found in the nasopharynx, sphenoid sinus, and clivus, may occur from the pharyngeal pituitary while another tumorigenesis can develop from the residual tissue fragment in the craniopharyngeal canal. The “overshoot” of the adenohypophyseal cell migration in the distal part of the sellar pituitary is frequently observed in the leptomeninges of the peri-infundibular or peri-hypothalamic region as ectopic pituitary cell clusters that are apparently independent of the pars tuberalis. It is suggested that these cells, frequently found in “normal” individuals, may be one of the possible origins of the intracranial ectopic pituitary adenomas. However, the reason why a majority of the reported intracranial ectopic pituitary tumors are ACTH-adenomas remains unexplained, since the ectopic cells, found in “normal” individuals, consist of fairly different hormone-producing cells. A further enigmatic phenomenon is a “basophil invasion.” ACTH-positive cells invade from the pars intermedia into the posterior lobe of the pituitary. This invasion increases in intensity and frequency according to increase in age. However, the invasion of ACTH cells is observed as early as in the fetal life. The invasive cells display occasionally cell atypia as well as mitotic activity. The origin of extremely rare pituitary adenomas inside the posterior lobe can be explained by the existence and proliferative activity of basophil invasion. Microsc. Res. Tech. 46:59–68, 1999. © 1999 Wiley-Liss, Inc.

Immunohistochemical survey of migration of human anterior pituitary cells in developmental, pathological, and clinical aspects: a review.

Developmentally pathological conditions of the anterior pituitary cells include failed separation of the primary pituitary gland into sellar and pharyngeal ones, ectopic migration into the subarachnoid space, and basophil invasion into the posterior lobe although the last is a physiological phenomenon with pathological potentiality in certain circumstances. Pituitary primordium appears at about 4 weeks of gestation. One of the causes of the pituitary gland agenesis may be a formation of the primary hypothalamic ganglionic hamartoma just at the time of occurrence of the pituitary primordium, as analyzed in cases of Pallister-Hall syndrome. A double pituitary in a single individual is a rare malformation. Its pathogenesis is considered as a result of notochordal anomaly. In the 8th gestational week, the primary pituitary gland separates into sellar and pharyngeal parts. The disturbance of this histogenesis results in a rare pituitary malformation, a "pharyngosellar pituitary." Despite the failed separation in this case, differentiation of the pituitary cells proceeds and the hormone production of this malformed pituitary gland can be displayed immunohistochemically. In this case, the distribution of the different hormone producing cells was atypical, particularly in those of gonadotropic hormones and ACTH. Life-long existence of the pharyngeal pituitary is a normal anatomical state in humans. Cell differentiation (hormone production) in the pharyngeal pituitary occurs about 4-10 weeks later than in the sellar pituitary. In pharyngeal pituitary, all kinds of adenohypophyseal hormones are produced. Extracranial pituitary adenomas (with intact sellar pituitary), exclusively found in the nasopharynx, sphenoid sinus, and clivus, may occur from the pharyngeal pituitary while another tumorigenesis can develop from the residual tissue fragment in the craniopharyngeal canal. The "overshoot" of the adenohypophyseal cell migration in the distal part of the sellar pituitary is frequently observed in the leptomeninges of the peri-infundibular or peri-hypothalamic region as ectopic pituitary cell clusters that are apparently independent of the pars tuberalis. It is suggested that these cells, frequently found in "normal" individuals, may be one of the possible origins of the intracranial ectopic pituitary adenomas. However, the reason why a majority of the reported intracranial ectopic pituitary tumors are ACTH-adenomas remains unexplained, since the ectopic cells, found in "normal" individuals, consist of fairly different hormone-producing cells. A further enigmatic phenomenon is a "basophil invasion." ACTH-positive cells invade from the pars intermedia into the posterior lobe of the pituitary. This invasion increases in intensity and frequency according to increase in age. However, the invasion of ACTH cells is observed as early as in the fetal life. The invasive cells display occasionally cell atypia as well as mitotic activity. The origin of extremely rare pituitary adenomas inside the posterior lobe can be explained by the existence and proliferative activity of basophil invasion.

Adjuvant γ-Knife Radiosurgery for a Resected Suprasellar Pituitary Adenoma with Atypical Immunohistochemistry: Case Report

A resected suprasellar mass was found to be an ectopic pituitary adenoma on light microscopy, but immunohistochemistry revealed unusual staining patterns. Residual disease was demonstrated on postoperative studies and γ-knife radiosurgery was used for treatment. The literature on ectopic pituitary tumors was reviewed. We concluded that postoperative stereotactic radiosurgery (SRS) for ectopic, suprasellar pituitary adenomas (and variants thereof) is an appropriate management option for persistent disease.

Pituitary gland involvement of the sinonasal tract.

Involvement of the upper aerodigestive tract by a pituitary adenoma is due either to downward extension of an intracranial tumor or to a tumor arising from ectopic (Rathke's pouch) remnants. Sites of predilection for the ectopic adenoma are headed by the sphenoid bone and sinus, followed by the soft tissue of the dorsal pharynx and clivus. Both invasive sellar and ectopic adenomas can be destructive, with the extracranial extension of pituitary adenomas being more locally aggressive. There have been no examples of metastasizing ectopic pituitary tumors.

Pituitary Apoplexy in an Ectopic Pituitary Tumour

A case report of a patient with unilateral visual loss, due to infarction in an ectopic pituitary tumour, is presented. Good return of vision followed surgical removal of the tumour.

Intracranial ectopic pituitary tumour

Pituitary tumours originating primarily from sites other than the sella turcica are rare. A case of an ectopic pituitary tumour in the region of the lesser wing of the sphenoid is reported. The patient presented with signs and symptoms of a progressive increase in intracranial pressure. CT scan appearances resembled those of a sphenoid wing meningioma. The vascular lesion was partially excised. Histology showed it to be a pituitary tumour.

Modulation (feminization) of hepatic enzymes by an ectopic pituitary tumor.

The ontogeny and endocrine regulation of sex-differentiated hepatic metabolism is mediated via the hypothalamic-pituitary axis. Using in vitro-in vivo systems, we demonstrate alterations in activity levels of six sex-differentiated enzyme systems in male rats bearing ectopic pituitary tumors after the injection of a pituitary cell line, C811RAP. Activity levels of hepatic glutathione S-transferase, UDP-glucuronyltransferase, and aryl hydrocarbon hydroxylase are reduced to activity levels of control females, while histidase, 5 alpha-reductase, and serum cholinesterase levels are increased to levels of control females, i.e. feminization of all of these enzymes. RIAs of testosterone, estrogen, FSH, and PRL are similar in tumor-bearing and control animals, but GH levels are significantly higher in tumor-bearing animals than in the controls. It is suggested that GH may be the pituitary factor responsible for the expression of sex-differentiated hepatic metabolism.

Development of Hyperlipidemia Associated with Increased Lipolytic Response of Isolated Adipose Tissue Cells Following Prolonged Stimulation by an Ectopic Pituitary Tumor

Implantation of MtT-F4 tumor, a mammotropic tumor that secretes large quantities of ACTH, GH and prolactin, into male Fisher rats induced the development of hyperlipidemia. Free fatty acid, triglyceride and cholesterol levels in the plasma were significantly increased at 31 days after tumor implantation. Blood glucose and glycerol levels remained normal, while uric acid concentration in the blood was significantly decreased. The lipolytic response of isolated adipose tissue cells to ACTH was significantly higher in cells derived from rats bearing an MtT-F4 tumor for 31 days than from their corresponding controls. However, the activity of adenylate cyclase in fat cells stimulated with ACTH was not significantly higher in cells derived from tumor bearing rats than in cells from control rats.

Plasma steroids in rats with experimental ectopic pituitary tumor and arterial hypertension.

Peripheral plasma concentrations of progesterone, 11-deoxycorticosterone (DOC) and corticosterone in rats bearing transplanted mammotropic tumors (MtT/F4) were measured simultaneously by means of a new competitive protein-binding method. Increased plasma levels of DOC (25-fold), as well as progesterone (6-fold) and corticosterone (3.5-fold) in rats bearing the tumors were observed when compared to control rats. Plasma aldosterone was decreased. The adrenal glands in tumor-bearing rats being 8-fold larger than in control rats, the ratio of the steroid concentration in plasma per milligram adrenal gland weight showed an increased DOC production, while no change in progesterone and decreased corticosterone production was observed. These findings suggest that the increased DOC production plays a role in the pathogenesis of hypertension in these rats and that the disproportionate change in steroid production is due to reduced llβ-hydroxylating activity in the adrenal cortex possibly related to the excessive am...