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      Ectopic pituitary adenoma with empty sella: A case report

      case-report

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          Abstract

          Ectopic pituitary adenoma is a condition characterized by the presence of an adenoma originating from extra-sellar pituitary cell residue from the migration of Rathke’s pouch. First described in 1909, ectopic pituitary adenoma is a very rare, largely unknown condition with a challenging diagnosis, that requires proving the absence of communication between the extra-sellar mass and the normal pituitary gland (or any of the intra-sellar contents). Here, we describe the case of a 38-year-old female who presented with symptomatic hyperprolactinemia in whom magnetic resonance imaging revealed a space-occupying mass in the sphenoid sinus with an empty sella turcica. Clinical information coupled with magnetic resonance imaging findings raised suspicion for the diagnosis, which was later confirmed through surgery (showing the absence of a breach in the sellar floor dura), and histology. Given the rarity of such cases, management is not standardized; however, most patients reported in the literature, as in our case, have undergone surgical treatment. This case report aims to provide an additional example to the existing literature on this rare condition, especially when it is associated with an empty sella turcica.

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          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 2-84 years (mean, 57.1 years), 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 3.4 cm. 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 (0.8 months). 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.
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            Sphenoid sinus ectopic pituitary adenomas: CT and MRI findings.

            Ectopic pituitary adenomas (EPAs) are rare lesions. The purpose of this study was to describe the CT and MRI features of sphenoid sinus EPAs. Eight patients with histology-proven EPAs in the sphenoid sinus, all of whom underwent CT and MRI, were reviewed retrospectively. The following imaging features were analysed: (i) size, (ii) margin, (iii) CT attenuation characteristics and (iv) MRI signal intensity. In addition, the involvement of adjacent structures and the time-intensity curve (TIC) of dynamic contrast-enhanced (DCE) MRI were analysed. All EPAs had well-defined margins and showed no relationship to the intrasellar pituitary gland. The mean size was 28 mm (range, 20-46 mm). On non-enhanced CT, the lesions appeared isodense to grey matter in 7 (88%) patients and hypodense in 1 (12%) patient. Only two patients underwent post-contrast CT, and they showed moderate enhancement. On T1 weighted images, EPAs appeared isointense in 6 (75%) patients and hypointense in 2 (25%). On T2 weighted images, the lesions appeared hyperintense in 2 (25%) patients and isointense in 6 (75%). EPAs showed mild to moderate heterogeneous contrast enhancement and exhibited a cribriform-like appearance. Two patients underwent DCE MRI; the TIC showed a rapidly enhancing and slow washout pattern. The following features were also seen: an empty sella, bone changes and involvement of the cavernous sinus (5 patients; 62.5%). In conclusion, a high index of suspicion for EPA and a familiarity with the imaging findings may help to diagnose this rare entity accurately.
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              Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella.

              We present a case of growth hormone (GH)-secreting ectopic pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. It has been well known that acromegaly is sometimes associated with an empty sella. However, such a case usually has a macroadenoma and an empty sella that is large. The authors considered the possible mechanisms of the association between a normal-sized empty sella and an ectopic pituitary adenoma in the sphenoid sinus as the following. Primary empty sella existed originally, and the pituitary adenoma developed later. The adenoma extended into the sphenoid sinus because of the pulsatile intracranial cerebrospinal fluid pressure. Copyright 2002, Elsevier Science Ltd. All rights reserved.
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                Author and article information

                Journal
                SAGE Open Med Case Rep
                SAGE Open Med Case Rep
                SCO
                spsco
                SAGE Open Medical Case Reports
                SAGE Publications (Sage UK: London, England )
                2050-313X
                30 December 2023
                2024
                : 12
                : 2050313X231223465
                Affiliations
                [1-2050313X231223465]Department of Emergency Radiology, Ibn Sina University Hospital of Rabat, Morocco
                Author notes
                [*]Bourekba Iliass, Department of Emergency Radiology, Ibn Sina University Hospital of Rabat, Imm 9 appt 8 Med Hansali Qebibat, Rabat 10170, Morocco. Email: iliass.bourekba@ 123456gmail.com
                Author information
                https://orcid.org/0000-0002-6336-3613
                https://orcid.org/0000-0003-0662-2647
                Article
                10.1177_2050313X231223465
                10.1177/2050313X231223465
                10757430
                a4d96e6e-c20a-491c-b5ac-73d40ed513dd
                © The Author(s) 2024

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 10 October 2023
                : 11 December 2023
                Categories
                Case Report
                Custom metadata
                January-December 2024
                ts1

                ectopic pituitary gland,ectopic pituitary adenoma,pituitary adenoma,empty sella

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