Intrasellar chordoma masquerading as a pituitary neuroendocrine tumor: Illustrative case.

Background: Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and intracranial chordomas exhibit proclivity for the spheno-occipital region. However, purely intrasellar occurrences are extremely rare. We report a case of intrasellar chordoma, which masqueraded as a pituitary neuroendocrine tumor. Case Description: An 87-year-old female presented with an acutely altered mental state after a few-week course of headaches and decreased left vision. Adrenal insufficiency was evident, and magnetic resonance imaging revealed an intrasellar lesion with heterogeneous contrast enhancement and marked T2 hyperintensity. Central adrenal insufficiency due to an intrasellar lesion was suspected. Cortisol replacement was initiated, and transsphenoidal surgery was performed. Anterosuperior displacement of the normal pituitary gland and the absence of the bony dorsum sellae were notable during the procedure. Histological examination led to a diagnosis of conventional chordoma, and upfront adjuvant stereotactic radiosurgery was executed. She has been free from tumor progression for 12 months. Conclusion: This case and literature review suggested that the pathognomonic features of intrasellar chordoma were heterogeneous contrast enhancement, marked T2 hyperintensity, osteolytic destruction of the dorsum sellae, and anterosuperior displacement of the pituitary gland. Clinical outcomes seemed slightly worse than those of all skull base chordomas, which were the rationale for upfront radiosurgery in our case. Neurosurgeons should include intrasellar chordomas in the differential diagnosis of intrasellar lesions, carefully dissect them from the adjacent critical anatomical structures, and consider upfront radiosurgery to achieve optimal patient outcomes.

Sellar hemangiopericytoma masquerading as pituitary adenoma: an overlooked intriguing case study unveiling a rare surgical conundrum.

Hemangiopericytoma (HPC) constitutes less than 1% of all primary central nervous system tumors. It is a vascular neoplasm with potential malignancy that, in rare instances, manifests as a primary lesion within the brain. Typically, it originates from the meninges. Here, we describe an exceptionally uncommon sellar region solitary fibrous tumor/hemangiopericytoma (SFT/HPC) that mimicked a nonfunctional pituitary adenoma. Case presentation: A 54-year-old male was referred to our hospital due to progressive blurred vision in the left eye over the past year. A homogeneous iso-dense extra-axial intrasellar round mass with extension into the suprasellar region, mainly on the left side, along with bony erosion and osteolysis around the sellar region, was observed on a brain computed tomography (CT) scan. Brain magnetic resonance imaging (MRI) revealed a well-defined 251,713 mm mass with iso-signal on T1-weighted images and hypersignal on T2-weighted images, originating from the pituitary gland within the sella turcica. The mass avidly enhanced following Gadolinium injection and adhered to both carotid arteries without vascular compression or invasion. It extended to the suprasellar cistern and compressed the optic chiasm. The diagnosis was nonfunctional pituitary macroadenoma, leading to the decision for Endoscopic Trans-Sphenoidal Surgery (ETSS). A non-sustainable, soft, grayish mass was grossly and totally resected during the operation. Subsequently, there was a significant improvement in visual acuity during the early postoperative period. Histopathologic examination confirmed hemangiopericytoma (WHO grade II). Conclusion: Due to its malignant nature, hemangiopericytoma should be included in the differential diagnosis of a sellar mass, both from a clinical and morphological perspective.

Chronic lymphocytic leukemia/small lymphocytic lymphoma arising in the pituitary gland: illustrative case.

BACKGROUND: The authors describe a 60-year-old female who underwent a correlative examination for an accidental scalp injury, revealing a sellar mass, which was surgically excised and pathologically confirmed to be a non-Hodgkin's small B-cell lymphoma. These findings in combination with the immunophenotype led to a final diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma. Previous studies have shown that hematological solid tumors occurring in the pituitary gland are extremely rare, and there are only approximately three other cases of living patients with similarities to this case, all of which had ambiguous expression of subsequent hematological treatment. OBSERVATIONS: In this case, the authors used an endoscopic approach to completely excise the tumor. Follow-up of the patient was continued after surgery, and the patient is currently receiving standardized treatment with zanubrutinib. LESSONS: This patient did not have any previous history of tumor, had a good postoperative recovery with a normal quality of life, and still receives hormone replacement and zanubrutinib on a standardized basis. This is a complete case that has not been previously reported and reveals the diagnostic and therapeutic process of rare diseases in the sellar area.

Pituicytoma: A rare tumor of the sella. A case report and review of literature for diagnosis and management.

Background: Pituicytomas are rare tumors of the pituitary gland derived from the ependymal cells and line the pituitary stalk and posterior lobe. These tumors are located in the vulnerable regions of the brain: Either in the sellar or suprasellar area. The location marks the difference in the clinical features of the tumor. Here, we report a case of histopathologically diagnosed pituicytoma of the sellar region. Literature is also reviewed and discussed to gain a better understanding of this rare disease. Case Description: A 24-year-old female presented to the outpatient department with complaints of headache, diplopia, dizziness, and decreased vision in the right eye for 6 months. Computed tomography scan brain without contrast showed a well-defined hyperdense lesion in the sella without associated bony erosion. Her magnetic resonance imaging showed well defined rounded lesion in the pituitary fossa which was isointense on T1-weighted image and hyperintense on T2-weighted images. A presumptive diagnosis of pituitary adenoma was made. She underwent endoscopic endonasal transsphenoidal resection of pituitary mass. Intraoperatively, normal pituitary gland was visualized and there was a grayish-green-colored, jelly like tumor which was pulled gently. On 9th postoperative day, she presented with cerebrospinal fluid (CSF) rhinorrhea. She underwent endoscopic CSF leak repair. Her histopathology was concluded to be Pituicytoma. Conclusion: Pituicytoma is an uncommon diagnosis. The surgical aim is to completely excise the tumor which results in complete cure, but incomplete resection may be performed due to high vascularity of this tumor. In case of incomplete excision, recurrence is common and adjuvant radiotherapy may be administered.

Sellar B lymphoblastic lymphoma mimics pituitary apoplexy with newly discovered gene mutations in TP53 and PAX5: A case report.

Lymphoblastic lymphoma (LBL) is a rare tumor that accounts for approximately 2-4% of all non-Hodgkin lymphomas, and less than 20% of LBLs are derived from B cells. B- Lymphoblastic lymphoma (B-LBL) often presents as bone marrow and peripheral blood lesions, and is very rare to present as a seller mass. We report a case of sellar B lymphoblastic lymphoma mimicking pituitary apoplexy, and review its diagnosis and treatment process, combined with the literature to deepen the understanding of sellar tumors.

Intrasellar Chordoma Mimicking Pituitary Macroadenoma with Hyperprolactinemia and Hypopituitarism: Clinical Images with a Surgical Video.

A purely intrasellar chordoma is rare among skull base chordomas and is recognized as originating from ectopic embryological notochord located in the sella turcica. In view of its rarity and nonspecific symptoms, clinicians may misdiagnose intrasellar chordoma as pituitary adenoma based on preoperative radiographic images. In this report, we present an intrasellar chordoma that clinically mimicked pituitary macroadenoma with hyperprolactinemia and hypopituitarism and was successfully resected by endoscopic endonasal transsphenoidal surgery. This case demonstrated radiographic features that chordoma should be suspected in sellar lesions. The enlarged sellar with thinned remodeled bone without clival destruction was firstly reminiscent of pituitary adenoma, whereas the very high signal on T2-weighted images and heterogeneous enhancement characteristically suggested chordoma. This rare diagnosis must be considered in the preoperative evaluation of sellar lesions because it can affect how the neurosurgeon prepares for surgery and the surgical goals.

Serum copeptin levels at day two after pituitary surgery and ratio to baseline predict postoperative central diabetes insipidus.

PURPOSE: Central diabetes insipidus is a complication that may occur after pituitary surgery and has been difficult to predict. This study aimed to identify the cutoff levels of serum copeptin and its optimal timing for predicting the occurrence of central diabetes insipidus in patients who underwent transsphenoidal surgery. METHODS: This was a prospective observational study of patients who underwent transsphenoidal surgery for pituitary gland or stalk lesions. Copeptin levels were measured before surgery, 1 h after extubation, and on postoperative days 1, 2, 7, and 90. RESULTS: Among 73 patients, 14 (19.2%) and 13 (17.8%) patients developed transient and permanent central diabetes insipidus, respectively. There was no significant difference in copeptin levels before surgery and 1 h after extubation; copeptin levels on postoperative days 1, 2, 7, and 90 were significantly lower in patients with permanent central diabetes insipidus than in those without central diabetes insipidus. Copeptin measurement on postoperative day 2 exhibited the highest performance for predicting permanent central diabetes insipidus among postoperative days 1, 2, and 7 (area under the curve [95% confidence interval] = 0.754 [0.632-0.876]). Serum copeptin level at postoperative day 2(< 3.1 pmol/L) showed a sensitivity of 92.3% and a negative predictive value of 97.1%. The ratio of copeptin at postoperative day 2 to baseline (< 0.94) presented a sensitivity of 84.6% and a negative predictive value of 94.9%. The copeptin levels > 3.4 and 7.5 pmol/L at postoperative day 2 and 7 may have ruled out the occurrence of CDI with a negative predictive value of 100%. CONCLUSION: The copeptin level at postoperative day 2 and its ratio to baseline can predict the occurrence of permanent central diabetes insipidus after pituitary surgery.

A Rare Case of Adamantinomatous Craniopharyngioma in an Adult.

Craniopharyngiomas represent a rare group of intracranial tumors that often arise in the sellar/suprasellar region of the brain. Adamantinomatous craniopharyngioma is significantly more common than papillary craniopharyngioma. The former most often arises in children whereas the papillary craniopharyngioma is mainly limited to adults. We present the case of a 34-year-old female with visual disturbances and other vague complaints who was found to have a large lobulated sellar mass on neuroimaging studies. She was subsequently diagnosed with an adamantinomatous craniopharyngioma after undergoing transsphenoidal resection. We discuss the patient's clinical, radiological, and pathological findings in correlation with the current literature and recommendations regarding this type of tumor. Given that adamantinomatous craniopharyngioma rarely presents in adulthood, especially in middle-aged adults, this case is considered rare, and we hope to increase awareness to include adamantinomatous craniopharyngioma in the differential diagnosis for sellar lesions in this age group.

Supratentorial Pediatric Midline Tumors and Tumor-like Lesions: Clinical Spectrum, Natural History and Treatment Options.

Childhood Central Nervous System tumors account for 25% of all pediatric tumors. Large availability and broadening of indications to imaging has made incidental findings more common. Among these, midline lesions have different clinical relevance depending on their intrinsic pattern of behaviour and on their specific location. In this narrative review we describe the natural history and treatment options of midline lesions in children.

Subarachnoid Hemorrhage From Rupture of an Undiagnosed Posterior Circulation Aneurysm During Sellar Tumor Surgery.

Association between cerebral aneurysms and sellar tumors has been previously reported. Rupture of anterior circulation aneurysms during a transsphenoidal surgery causing massive subarachnoid hemorrhage (SAH) is uncommon, but rupture of a posterior circulation aneurysm is an infrequent event. We present three cases of SAH secondary to rupture of an undetected posterior circulation aneurysm during transsphenoidal surgery to treat a sellar tumor. The common factor in these cases was the adverse outcome despite treatment.  The fatal outcome seen in all these cases questions whether to include a (magnetic resonance) MR angiography or (computed tomography) CT angiography during preoperative evaluation for sellar tumors in order to identify inadvertently associated aneurysms.

Application of anterior clinoid grinding in sellar region tumors and ophthalmic/superior clinoid process aneurysms of internal carotid artery / 中国医师进修杂志

Objective:To evaluate the clinical effect of anterior clinoid process grinding in the treatment of ophthalmic / superior clinoid process aneurysms and sellar tumors.Methods:The clinical data of 16 patients who underwent anterior clinoid process grinding in Sanbo Brain Hospital, Capital Medical University from January 2015 to July 2021 were analyzed retrospectively. There were 1 patient with recurrent craniopharyngioma, 1 patient with recurrent pituitary adenoma, 13 patients with aneurysms, and 1 patient with suprasellar granulosa cell tumor combined with ophthalmic aneurysm of right internal carotid artery. The Modified Rankin Scale (mRS) score was used to evaluate the situation at discharge and in the medium-and-long term.Results:Sixteen patients underwent anterior clinoidprocess grinding. At discharge and the latest follow-up, the mRS scores of the patients were 0-2. A total of 15 aneurysms were treated, and there were no symptoms of visual loss or visual field defect after operation. No cerebrospinal fluid leakage occurred in all patients.Conclusions:The grinding of anterior clinoid process can effectively and fully stretch the optic nerve and internal carotid artery, and can observe the tumor neck at the lower end of pituitary stalk and the ocular segment/superior clinoid process of internal carotid artery under direct vision. It is one of the important auxiliary methods for the treatment of sellar lesions.

A Comprehensive Study of Spindle Cell Oncocytoma of the Pituitary Gland: Series of 6 Cases and Meta-Analysis of 85 Cases.

OBJECTIVE: To discuss optimal treatment strategy for spindle cell oncocytoma (SCO) of the pituitary gland. METHODS: Institutional cases were retrospectively reviewed. A systematic literature search and subsequent quantitative synthesis were performed for further analysis. The detailed features were summarized and the tumor control rate (TCR) was calculated. RESULTS: Eighty-five patients (6 institutional and 79 literature) were included. The annual incidence was approximately 0.01-0.03/100,000. The mean age was 56 years. Vision loss was present in 60%. Seventy-three percent showed hormonal abnormalities. On magnetic resonance imaging, tumor was avidly enhancing, and the normal gland was commonly displaced anterosuperiorly. Evidence of hypervascularity was seen in 77%. Gross total resection (GTR) was achieved in only 24% because of its hypervascular, fibrous, and adhesive nature. The mean postoperative follow-up was 3.3 years for institutional cases and 2.3 years for the integrated cohort. The TCR was significantly better after GTR (5-year TCR, 75%; P = 0.012) and marginally better after non-GTR + upfront radiotherapy (5-year TCR, 76%; P = 0.103) than after non-GTR alone (5-year TCR, 24%). The TCRs for those with low Ki-67 index (≤5%) were marginally better than those with higher Ki-67 index (5-year rate, 57% vs. 23%; P = 0.110). CONCLUSIONS: Frequent endocrine-related symptoms, hypervascular signs, and anterosuperior displacement of the gland support preoperative diagnosis of SCO. GTR seems to have better long-term tumor control, whereas the fibrous, hypervascular, and adhesive nature of SCO makes it difficult to achieve GTR. In patients with non-GTR, radiotherapy may help decrease tumor progression.

Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature.

BACKGROUND: Solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a rare tumor which originates from the walls of capillaries and has historically been thought to be able to occur anywhere in the body that blood vessels are found. It is rarely found in the sellar region. CASE DESCRIPTION: InS this report, we present the first case of this tumor occurring in the sellar region of a pediatric patient. This 12-year-old male presented with progressive vision loss which prompted surgical resection after a sellar lesion was discovered on imaging. The initial transsphenoidal approach resulted in subtotal resection and the patient experienced reoccurrence within 3 months. He underwent an orbitozygomatic craniotomy to achieve gross total tumor resection. CONCLUSION: We conducted a literature review of intracranial SFT/HPC in the pediatric population and found it to be an extremely rare occurrence, with <30 cases reported. The incidence of SFT/HPC occurring in the sellar region for any age group was also found to be a rare entity. Treatment recommendations for this tumor are also scarce, based on retrospective chart reviews from the adult population. The role for adjuvant radiation has mixed results.

A Systematic Review of Sellar and Parasellar Brown Tumors: An Analysis of Clinical, Diagnostic, and Management Profiles.

OBJECTIVE: To systematically review and analyze clinical, diagnostic, and management trends in sellar and parasellar brown tumors reported in existing literature. METHODS: In this systematic review, PubMed, Ovid MEDLINE, Scopus, and Google Scholar databases were searched for reported cases of sellar/parasellar brown tumors. Relevant titles and abstracts were screened in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol. Articles meeting inclusion criteria were subjected to data extraction, summarization, and analysis. A rare case of parasellar brown tumor was also presented. RESULTS: Eight reports (including the current report) were eligible for inclusion. Mean patient age was 42.75 years. Reported symptoms included visual disturbances (n = 6), headache (n = 5), fatigue (n = 3), nausea/vomiting (n = 2), chest pain (n = 1), neck pain (n = 1), and dysphagia (n = 1). In cases where computed tomography findings were provided (n = 6), lesions were noted to be expansile and lytic. Lesions were hyperintense on T2-weighted magnetic resonance imaging (66.7%) and demonstrated contrast enhancement (83.3%). Histology unanimously showed multinucleated giant cells in a fibrovascular connective tissue stroma. Dramatic symptom resolution was noted in all patients who underwent resection of the sellar/parasellar brown tumor (n = 4; 50%). CONCLUSIONS: Sellar/parasellar brown tumors are a rare, tertiary manifestation of hyperparathyroidism and can be elusive to diagnose. Diagnosis requires a high index of clinical suspicion in addition to comprehensive biochemical testing, imaging, and histopathologic analysis. Surgical extirpation is favored in cases where the lesion is causing compressive symptoms, or if it is unresponsive to management of hyperparathyroidism.

Mixed gangliocytoma-pituitary adenoma in the sellar region: a large-scale single-center experience.

BACKGROUND: Mixed gangliocytoma-pituitary adenoma is an extremely rare tumor occurring in the sellar region, histologically composed of both gangliocytic and pituitary adenomatous architectures. The histogenesis of these tumors remains unknown, and the diagnosis, treatment, and prognosis are yet to be fully understood. METHODS: We retrospectively reviewed the clinical, radiological, and histopathological profiles from a series of 20 patients with sellar mixed gangliocytoma-pituitary adenomas. All patients underwent surgical tumorectomy via an endoscopic or microscopic transsphenoidal approach. Perioperative magnetic resonance imaging (MRI) and computed tomography (CT) data were reviewed. Immunohistochemical stains and electron microscopy examination were performed. Follow-up outcomes were presented. RESULTS: This case series consisted of 13 females and seven males (age range, 20-59 years; mean age, 42.3 ± 11.2 years). Preoperative endocrine examination showed elevated growth hormone (GH) in nine patients and hyperprolactinemia in eight patients. The tumors were positive for GH in 15 cases, prolactin in 13 cases, adrenocorticotropic hormone in three cases, and thyroid-stimulating hormone in one case. Gross total resection was achieved in 15 patients, and subtotal resection in five patients. During an average follow-up period of 42.5 ± 29.0 months, no recurrence was noted. CONCLUSIONS: The clinical and neuroimaging features of sellar mixed gangliocytoma-pituitary adenomas are non-specific, and invasion into the cavernous sinus is common. Surgical resection via a transsphenoidal approach is the preferred treatment, and the surgical outcomes are favorable. Moreover, our histopathological findings are more likely to support the theory that mixed gangliocytoma-pituitary adenoma originates from the neuronal transdifferentiation of adenomatous cells.

[Microsurgical resection of sellar tumors via lateral supraorbital approach: clinical analysis of 20 cases].

Objective: To research the application of lateral supraorbital (LOS) approach for resecting sellar tumors. Methods: Clinical symptoms of 20 patients with surgery via LOS approach were analyzed retrospectively.According to the neuroradiological findings and the improvement of symptoms, and some score scale, the efficacy and safety of LOS were assessed for removing sellar tumors.There were tuberculum sellae meningioma (n=11), craniopharyngioma (n=4), epidermoid cysts (n=4) and pituitary adenoma (n=1). Results: Complete resection was achieved in seventeen patients(85%). The mean operating time was 144.0±54 minutes and the mean intraoperative blood loss was 96.5±51.1 ml.Four patients had post-operative fever, four had endocrine disorders, and one had seizure.No other complications were noted.Fifteen patients had visual function impairment before operation, seven of which were improved while one was aggravated after surgery.The median Karnofsky score (3-46 mon) was 95(0-100) and the Glasgow outcome scale (GOS) at follow-up (3-46 mon) was 4.7(0-5). In general, the outcomes were improved in 18 patients and the mortality was 2. Conclusion: The resection of tumors at sellar region via lateral supraorbital approach is effecient, simple and minimally invasive, which can make the removal of the tumors reliably and safely.It is worthy to be popularized clinically.

Endoscopic Trans-Sphenoidal surgery; Efficacy and response in Pituitary Adenoma.

OBJECTIVES: The purpose of the study was to access the efficacy and response of the endoscopic transsphenoidal surgery in pituitary adenomas. METHODS: It was descriptive case study, conducted at Neurosurgery Department in collaboration with the Endocrine Unit (Medical Unit-II) Of Jinnah Post Graduate Medical Center Karachi from January 2015 to July 2017. Patients with sellar, supra sellar and para sellar tumors were enrolled in the study. Patients with prolactinoma and recurrent pituitary tumors were excluded. Data was analyzed using SPPS 17. RESULTS: Sixty three patients were included in the study with mean age of 42±8.34 years. There were 40(63.5%) male patients and 23(36.5%) female patients with pituitary adenoma. Headache and visual impairment were the main presentation 55(87.3%) and 56 (88.8%) respectively. Out of all these patients the pituitary adenomas, 51(81%) patients had non secretory and 12 (19%) patients had secretory tumor. Out of these pituitary adenomas 53(84.1%) were macroadenomas and 10(15.9%) were microadenoma. Post operatively marked improvement in the headache was in all 100% patients and vision improved in 54 (96.4%). The most common post operative complication was cerebrospinal fluid (CSF) leak in 10 (15.9%) with 44 (69.8%) having no post complications at all. Mortality was reported to be just 1.6% i-e one patient. CONCLUSION: The endoscopic transsphenoidal approach for pituitary adenoma is the safest procedure with marked improvement in complications and reduction in patient's hospital stay.

Complete endoscopic resection of a pituitary stalk epidermoid cyst using a combined infrasellar interpituitary and suprasellar endonasal approach: case report.

Intracranial epidermoid cysts are benign lesions of epithelial origin that most frequently present with symptoms of mass effect. Although they are often associated with a high rate of residual tumor and recurrence, maximal safe resection usually leads to good outcomes. The authors report a complete resection of an uncommon pituitary stalk epidermoid cyst with intrasellar extension using a combined suprasellar and infrasellar interpituitary, endoscopic endonasal transsphenoidal approach. The patient, a 54-year-old woman, presented with headache, visual disturbance, and diabetes insipidus. Postoperatively, she reported improvement in her visual symptoms and well-controlled diabetes insipidus using 0.1 mg of desmopressin at bedtime and normal anterior pituitary gland function. One year later, she continues to receive the same dosage of desmopressin and is also taking 50 mcg of levothyroxine daily after developing primary hypothyroidism unrelated to the surgical procedure. A combined infrasellar interpituitary and suprasellar approach to this rare location for an epidermoid cyst can lead to a safe and complete resection with good clinical outcomes.

[Clinical analysis of endoscopic endonasal approach for removal of sellar tumors].

Objective:To investigate the safety and efficacy of endoscopic endonasal approach for removal of sellar tumors. Method: The clinical data of 18 patients with sellar tumors undergoing endoscopic endonasal surgery were analyzed retrospectively. This series consisted of ten pituitary adenomas, five craniopharyngiomas, three meningiomas. The headache, visual improvement, endocrine, extent of resection and surgical complications were evaluated. Result:Total removal was achieved in 14 patients (77.8%), almost total removal was achieved in one patient of pituitary adenoma with two craniopharyngiomas (16.7%), and one patient with the subtotal removal of craniopharyngioma (5.6%) received gamma knife therapy. Overall, 18 patients with headache got improved obviously; 15 patients presented with visual and view impairment, All of them, vision and view were improved or even recovered to normal following surgery; There were 2 new cases of postoperative diabetes insipidu; 5(27.8%) patients had hyposmia after operation; 1 patient (5.6%) with adenoma suffered cerebrospinal fluid rhinorrhea, the second operation was successful. The follow-up lasted 12-84 months, no tumor recurrence was identified by MRI examination, and no patient died. Conclusion:The endoscopic endonasal approach is feasible and safe for the treatment of tumors in the sellar region. With advantages of clear vision, better exposure, without brain retraction, and preservation of vascular and neurological function. Skilled endoscopic technique and reliable skull base reconstruction are important to ensure success of surgery.

Multiple recurrences require long-term follow-up in patients diagnosed with spindle cell oncocytoma of the sella turcica.

Spindle cell oncocytoma (SCO) of the sella turcica is characterized as a WHO grade I non-endocrine neoplasm of the sella turcica. Histologically, these tumors contain spindled and variably oncocytic cellular processes. Although SCOs lack immunoreactivity for neuroendocrine markers and pituitary hormones, they are clinically indistinguishable from non-functioning pituitary adenomas. In contrast to the initially described benign clinical course, several reports have subsequently illustrated cases with multiple recurrences with or without histological features of anaplasia in the form of nuclear pleomorphism, frequent mitoses, high Ki-67 index, and/or necrosis. With a follow-up of 14years, we report a case of SCO with multiple recurrences along with an exhaustive clinico-pathological review of all 41 cases of SCO reported in the literature, of which recurrence has been described in 11 cases. Collectively, this report highlights the importance of long-term follow-up and the possible need for adjuvant radiotherapy in patients diagnosed with a sellar SCO and provides a comprehensive review of this rare nonadenomatous sellar tumor.