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Spindle cell oncocytomas (SCOs) are rare, benign, non-functioning tumours of the pituitary gland that were included in the World Health Organisation’s (WHO) classification of central nervous system tumours in 2007. Due to their radiological appearance, they often are mistaken for pituitary adenomas prior to histopathological confirmation. Due to their rarity, there are no known risk factors or correlations in the literature between SCOs and other primary central nervous system tumours.This is a case report of a 71 year old gentleman who underwent transsphenoidal resection of a symptomatic and histologically-confirmed growth-hormone secreting pituitary adenoma. Due to persistently elevated IGF-1 levels, he underwent repeat surgery which led to biochemical remission. The histology from the second surgery returned a diagnosis of SCO. We hypothesise that our patient had concomitant pathologies of a GH-secreting adenoma and a SCO. This is the first described case of a synchronous functional pituitary macroadenoma and a spindle cell oncocytoma.

Pituicytoma (PITUI), granular cell tumor (GCT), and spindle cell oncocytoma (SCO) are rare tumors of the posterior pituitary. Histologically, they may be challenging to distinguish and have been proposed to represent a histological spectrum of a single entity. We performed targeted next-generation sequencing, DNA methylation profiling, and copy number analysis on 47 tumors (14 PITUI; 12 GCT; 21 SCO) to investigate molecular features and explore possibilities of clinically meaningful tumor subclassification. We detected two main epigenomic subgroups by unsupervised clustering of DNA methylation data, though the overall methylation differences were subtle. The largest group (n = 23) contained most PITUIs and a subset of SCOs and was enriched for pathogenic mutations within genes in the MAPK/PI3K pathways (12/17 [71%] of sequenced tumors: FGFR1 (3), HRAS (3), BRAF (2), NF1 (2), CBL (1), MAP2K2 (1), PTEN (1)) and two with accompanying TERT promoter mutation. The second group (n = 16) contained most GCTs and a subset of SCOs, all of which mostly lacked identifiable genetic drivers. Outcome analysis demonstrated that the presence of chromosomal imbalances was significantly associated with reduced progression-free survival especially within the combined PITUI and SCO group (p = 0.031). In summary, we observed only subtle DNA methylation differences between posterior pituitary tumors, indicating that these tumors may be best classified as subtypes of a single entity. Nevertheless, our data indicate differences in mutation patterns and clinical outcome. For a clinically meaningful subclassification, we propose a combined histo-molecular approach into three subtypes: one subtype is defined by granular cell histology, scarcity of identifiable oncogenic mutations, and favorable outcome. The other two subtypes have either SCO or PITUI histology but are segregated by chromosomal copy number profile into a favorable group (no copy number changes) and a less favorable group (copy number imbalances present). Both of the latter groups have recurrent MAPK/PI3K genetic alterations that represent potential therapeutic targets.

We report the case of a 61-year-old male with spindle cell oncocytoma of the hypophysis. On presentation to the Department of Neurosurgery at the German Armed Forces Hospital of Ulm, the patient reported a history of several years of left sixth nerve palsy, right ptosis, increased sensitivity to light, and a bilateral retrobulbar pressure sensation. Pituitary function was normal. A chromophobe non-functioning pituitary adenoma was initially suspected. The diagnosis was established on the basis of examination at a histopathology reference laboratory using immunohistochemistry to identify cell surface markers. During two years of follow-up, there were two clinical recurrences requiring surgery. To our knowledge, this is the 35th documented case of spindle cell oncocytoma of the pituitary gland and the first that was immunohistochemically negative for epithelial membrane antigen (EMA) and S100; and the first that displayed haematogenous metastasis to the right sphenoparietal sinus. The three surgical procedures were associated with massive intraoperative bleeding and thus resulted in subtotal tumor resection. Following surgery for the recurrences, the patient underwent radiotherapy. [ABSTRACT FROM AUTHOR]

Background Tumors arising from the posterior pituitary gland are rare and closely resemble pituitary adenoma in presentation and imaging. Most of them come as a histopathologic surprise. We have analyzed the posterior pituitary tumors managed in our institute and have discussed the dilemmas in imaging, challenges in intraoperative squash cytology, and surgical management. Methods We retrospectively reviewed our operative database of pituitary tumors over the past 10 years, which included five posterior pituitary tumors (three granular cell tumors [GCTs] and two spindle cell oncocytomas [SCOs]). Clinical, imaging, and endocrine characteristics; intraoperative details; histopathologic features; and postoperative outcomes were collected and analyzed. Results The mean age of the patients was 47 years. All patients presented with varying degrees of vision loss. Radiology revealed a sellar / suprasellar lesion with the pituitary gland seen separately in two of three GCTs, whereas a separate pituitary gland could not be identified in both the SCOs. Pituitary adenoma was a radiologic diagnosis in only two of five cases. Three patients underwent a transsphenoidal surgery, whereas two underwent surgery by the transcranial approach. Intraoperative cytology was challenging, though a possibility of posterior pituitary tumor was considered in three of four cases, whereas one was considered meningioma. All the tumors were very vascular and influenced the extent of resection. Conclusions GCTs and SCOs are relatively uncommon tumors that are difficult to diagnose on preoperative imaging. Intraoperative squash cytology too can pose challenges. A preoperative suspicion can prepare the surgeon for surgery of these hypervascular tumors. The transcranial approach may be necessary in cases of uncertainty in imaging. [ABSTRACT FROM AUTHOR]

Between 1996 and 2020, 12,565 cases were enrolled in the German Registry of Pituitary Tumors including 10,084 PitNETs (10,067 adenomas and 19 carcinomas obtained surgically and 193 adenomas diagnosed at autopsy) as well as 69 spindle cell tumors of the neurohypophysis (64 surgical specimens and 5 autopsies). In six patients (1 post mortem and 5 surgical specimens), PitNETs as well as posterior lobe tumors were found in the specimens. Two of the PitNETs were sparsely granulated prolactin-producing tumors, combined in one case with a granular cell tumor and in one case with a pituicytoma. One of the PitNETs revealed that autopsy was a sparsely granulated GH tumor combined with a neurohypophyseal granular cell tumor. Two PitNETs were null cell adenomas combined with a pituicytoma and a spindle cell oncocytoma, respectively. Further, one Crooke cell tumor was combined with a spindle cell oncocytoma. In five cases, the PitNETs were larger than the posterior lobe tumors and accounted for the clinical symptoms. Previously, four cases of co-existing pituitary anterior and posterior lobe tumors were described in the literature, comprising two ACTH PitNETs, one gonadotrophic PitNET and one null cell PitNET, each in combination with a pituicytoma. PitNETs and concomitant granular cell tumor or spindle cell oncocytoma, as observed in our cohort, have not been reported before. [ABSTRACT FROM AUTHOR]

Introduction: Spindle cell oncocytoma (SCO) is an extremely rare sellar neoplasm. No observational studies have been reported so far to investigate the prognostic factors of this tumor entity. This systematic review aimed to elucidate the risk factors for tumor recurrence/progression of SCO. Methods: We searched for relevant articles in PubMed and Web of Science. Studies providing individual patient data with follow-up information of SCO cases were included. Pearson's Chi square and Fisher's exact test were used for categorical variables while t test or Mann–Whitney tests were applied for continuous variables, if applicable. We used the Cox regression model to assess the effects of suspected variables on progression-free survival (PFS). Results: A total of 38 case reports and case series comprising of 67 SCOs were included for final analyses. Recurrent/progressive tumors were noted in 38.8% of cases. Among the clinicopathological factors, only the extent of surgery was a significant risk factor for tumor recurrence/progression. SCO patients with a subtotal resection had a significantly higher risk for tumor relapse in comparison with complete removal (HR 7.51; 95% CI 1.75–32.31; p = 0.007). Conclusion: Our study demonstrated the characteristic clinicopathological features of SCOs with a high recurrence/progression rate and outlined the predictor for tumor relapse. Failure to achieve gross total resection is the only risk factor for tumor recurrence/progression. [ABSTRACT FROM AUTHOR]

Pituitary spindle cell oncocytomas represent a rare pathologic entity within the differential diagnosis for pituitary tumors. As part of a class of tumors that includes low grade non-endocrine pituitary tumors such as pituicytomas and granular cell tumors (GCT), these can have similar characteristics to pituitary adenomas, and are occasionally misdiagnosed as such. We present a case of a 74-year-old woman who presented for work up of persistent headaches and vertigo. MRI brain demonstrated a heterogeneously enhancing soft tissue mass at the left lateral aspect of the sella. Patient underwent a biopsy and resection of the lesion. Histopathological examination is reviewed which revealed a spindle cell oncocytoma. Follow up MRI and examination at 6 months revealed partial resection. Additionally, we present an overview of the literature on clinical characteristics of this tumor and considerations for treatment and follow up.

To discuss optimal treatment strategy for spindle cell oncocytoma (SCO) of the pituitary gland. 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. 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). 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. [ABSTRACT FROM AUTHOR]

The rare clinical entity of primary posterior pituitary tumors (PPTs) includes pituicytomas, granular cell tumors, spine cell oncocytomas, and sellar ependymomas. The recent World Health Organization classification of PPTs based on thyroid transcription factor 1 positivity has led to more investigations into the epidemiology, clinical presentation, nature history, histologic features, and operative characteristics of these tumors. The aim of this review is to summarize the characteristics of primary PPTs. Our summary involved an in-depth review of the literature on PPTs. Our systematic review was carried out using the PubMed database and PRISMA guidelines. An initial search identified 282 publications. After strict application of the inclusion criteria, we found 16 articles for case series of patients with primary PPT (N > 5), which were included in our table for literature review. An additional 10 articles were review articles on PPTs published in the last 20 years and were used as resource for our systematic review. An extensive analysis was then performed to extract relevant clinical data with respect to the clinical radiologic histopathologic profile of primary PPTs and their treatment outcome. Primary PPTs are a rare group of pituicyte-derived low-grade nonneuroendocrine neoplasms that arise from the sellar region. The nondescript radiographic findings and subtle endocrine abnormalities also veil their accurate diagnostic prediction. As shown through the narrative as well as the literature review, there is still a lot to be understood about PPTs. A prospective multicenter registry of these rare tumors would benefit both the neurosurgical as well as the endocrinologic knowledge base. [ABSTRACT FROM AUTHOR]

To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy for these rare tumors and add to the limited existing literature. Retrospective review of 8 cases (5 pituicytomas, 2 spindle cell oncocytomas, and 1 granular cell tumor) managed at our institution between 2004 and 2019. The patients' clinical course, histologic features, and radiologic findings were reviewed. Their management and long-term follow-up is presented and compared with the literature. Long-term follow-up ranged from 1 to 9 years. There was 1 recurrence in a patient with spindle cell oncocytoma, and this was treated with radiotherapy. The endoscopically managed cases resulted in complete tumor excision with no recurrence. Epidemiologic data on primary tumors of the neurohypophysis is limited because of the rarity of these tumors. This study adds to the literature that these tumors behave as World Health Organization grade I tumors, although close follow-up is recommended as a few cases have shown recurrence. The endoscopic approach resulted in better gross total tumor resection rate in this series. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) is a rare tumor of adenohypophysis, arising from the sellar region. So far, about 35 cases of SCO in the sellar region have been reported. In this report, we present the first case of pediatric SCO and review the literature concerning the tumor origin, clinical presentations, radiological features, and treatment modalities. An 8-year-old male was referred to our clinic with progressive visual loss in the left eye and headache over the past 6 months. Cranial magnetic resonance imaging revealed a solid adenohypophysis mass with suprasellar extension, as well as compression and displacement of the optic chiasm. The patient underwent endoscopic trans-sphenoidal resection of the tumor. The tumor was diagnosed as SCO based on the histological study. He did not receive radiation therapy. The patient's condition remained stable, with no radiological recurrence in the past follow-up 2 years after the surgery. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytomas (SCOs) are rare neuroendocrine tumors of the posterior pituitary that are often misdiagnosed as nonfunctional pituitary tumors. Fewer than 50 cases of SCOs have been described in the literature, and many of these reports have documented the tumors to be hypervascular on imaging or histology. We present the first cerebral angiography imaging findings of an SCO before primary resection. The discovery of a prominent tumor blush, enlarged meningohypophyseal feeders bilaterally, and prominent tumor draining veins aided in preoperative planning and subsequent successful endoscopic transsphenoidal surgical resection. Despite being a rare entity, SCOs should be included in the differential diagnosis when working up a hypervascular sellar tumor. Flow voids may be present on initial magnetic resonance imaging evaluation. Subsequent digital subtraction angiography can be used to further investigate abnormal vasculature and aid in surgical planning. [ABSTRACT FROM AUTHOR]

Granular cell tumors (GCTs), pituicytomas, and spindle cell oncocytomas are rare, nonfunctioning pituitary tumors sharing positive staining of thyroid transcription factor 1. We present our series, the first single-institutional report with long-term surgical follow-up of all 3 tumor types. Our institutional pathology database was queried for these 3 pathologic diagnoses. Clinical records were assessed for clinical presentation, preoperative and postoperative endocrine status, tumor location on imaging, surgical characteristics, pathology results, and tumor recurrence. Data were analyzed for 4 patients with GCTs, 4 with pituicytomas, and 3 with spindle cell oncocytomas. The most common symptoms at presentation were vision changes (64%), headache (55%), endocrine abnormalities (55%), and fatigue (46%). GCTs were the only subtype to present exclusively in the infundibulum and the only subtype in our series to be treated with a transcranial transsylvian approach to resection (n = 2). In our study, in contrast to other reports, estimated blood loss was less than 300 mL in all patients. Imaging confirmed gross total resection in all 11 cases with no known recurrences at a mean (standard deviation) follow-up of 4.7 (3.7) years. We present a single-institution series of rare thyroid transcription factor 1–staining posterior pituitary tumors of the sellar region. Key novel findings include gross total resection with no tumor recurrence at nearly 5 years of mean follow-up and no cases of excess or uncontrolled blood loss. Our findings reinforce the observation that GCTs present in the suprasellar space. [ABSTRACT FROM AUTHOR]

In 2017, the World Health Organization established that pituicytoma, granular cell tumor (GCT), spindle cell oncocytoma (SCO) and sellar ependymomas (SE) are posterior pituitary tumors (PPT). They probably arise from the pituicytes and may constitute a unique histopathological entity. We carried out a systematic review using PubMed's database. A total of 266 patients with pathological diagnosis of PPT (135 pituicytomas, 69 GCT, 47 SCO, 8 SE and 7 mixed histology tumors) were analyzed. Gender distribution was identical and median age at diagnosis was 48 ± 21.8 years. Main presentation symptoms were visual disorders (n = 142; 58.1%), headache (n = 99; 40.5%), hypopituitarism (n = 84; 34.4%), hypercortisolism (n = 10; 4.1%), polyuriapolydipsia (n = 6; 2.4%) and acromegaly features (n = 5; 2.0%). On MRI, 122 (47.6%) patients showed sellar with suprasellar extension masses, 67 (23.1%) were suprasellar and 63 (24.6%) exclusively sellar. Median tumor size was 22.0 ± 14.2 mm. Two hundred sixty four patients underwent surgery, transphenoidal access was selected in 132 (64.4%) and craniotomy in 58 (28.3%). Complications were hypopituitarism (n = 70; 42.1%), diabetes insipidus (n = 55; 33.1%) and hemorrhage (n = 50; 30.1%). Tumor persisted in 93 patients (45.6%) and recurred in 13 (6.4%). Regarding comparison between main types of PPT, SCO patients were diagnosed later (60.0 vs 47.0 vs 47.0 years, p = 0.023), the tumor was larger 25.0 mm [10.8] vs 20.0 mm [14.2] vs 2.0 mm [15.0] and they were frequently sellar with suprasellar extension tumors (71.7% vs 46.2% vs 32.8%, p = 0.003) compared to pituicytoma and GCT. In conclusion, PPT are rare tumors and have been misdiagnosed mainly as non-functioning pituitary adenomas. Different types of PPT share similar epidemiology, clinical manifestations and surgical outcomes. Surgery is the only curative option but complications and subtotal resection are common. [ABSTRACT FROM AUTHOR]

Purpose: Spindle cell oncocytomas (SCOs) are very rare tumors of the posterior pituitary with potential for locally aggressive behaviour. Their treatment includes surgery and possibly radiotherapy, however other options are lacking. Somatostatin receptors (SSTs) are a possible therapeutic target for somatostatin analogues and their expression has been demonstrated recently in closely related pituicytomas, but there are no data about their presence in SCOs.Methods: We collected five cases of SCO from four patients including one recurrent case. Immunohistochemical detection of TTF1, GFAP, CD68, SST1, SST2, SST3, SST5 and D2 dopamine receptor (D2DR) was performed. Intensity, percentage of positive cells and pattern of expression was evaluated in semiquantitative fashion. Protein expression of SST1−5 and D2DR was further evaluated by western blot.Results: Mean patient age was 61.8 years (range 47-71 years) with male to female ratio 1:1. In one patient, samples from the original tumor and its recurrence 16 years later were assessed. TTF1 was positive in all five cases, no expression of GFAP and CD68 was seen. Immunohistochemical expression of SST1 was noted in 1/5 cases, SST2 in 2/5 cases, including recurrent case but not the original case. SST3 was expressed in 3/5 tumors and D2 dopamine receptor in 4/5 cases. Western blot was successfully performed in four samples. SST2, SST3 and D2DR expression was identified in all the samples, including two cases originally negative for SST2 and one case negative for SST3 by immunohistochemistry. The number of positive cells and level of expression varied among different areas of the same tumors. No expression of SST5 was observed. In the patient with the recurrent tumor, intensity of SST2, SST3 and D2DR expression varied between original tumor and its recurrence.Conclusions: We demonstrated presence of different SST subtypes and D2DR in spindle cell oncocytomas. The most commonly expressed subtype was SST2 and SST3, while no expression of SST5 was observed. Expression showed spatial heterogeneity and temporal changes as seen in the recurrent case. The biological meaning of SSTs expression in SCOs is unclear as well as whether it may be exploited in treatment of selected cases. [ABSTRACT FROM AUTHOR]

Purpose: In 2017, the WHO established that pituicytoma, granular cell tumour (GCT) and spindle cell oncocytoma (SCO) are posterior pituitary tumours (PPT). Recent data suggests that these tumours probably arise from the pituicytes and may constitute a spectrum of a unique histopathological entity. Our aim is to report the clinical findings and surgical outcomes of 16 patients with PPT. We also evaluated the tissue specimens available in light of current knowledge.Method: Cross-sectional study with retrospective data.Results: PPT were 7 pituicytomas, 3 GCT and 6 SCO. Patients mean age was 55 years old and 75% were female. Basal hormonal study showed hyperprolactinemia (43.7%) and hypopituitarism (37.5%). There was no case of diabetes insipidus (DI). MRI showed sellar/suprasellar masses with mean size of 19.7mm. PPT was not suspected in any patient. Fifteen patients underwent surgery and complications were common: 20% had perioperative bleeding (one patient died because of a massive haemorrhage), 57.1% hypopituitarism, 35.7% permanent DI and 21.4% underwent a second surgery. Pathological findings shown positivity for thyroid transcription factor 1, vimentin and negativity for cytokeratin and chromogranin A in all specimens evaluated. S100 protein was positive in 88.8% of tumours. Ki67 was ≥ 3% in 66.6% and ranged from 4-7% in SCO.Conclusion: PPT have similar histology, clinical features and are frequently misdiagnosed as nonfunctioning pituitary tumours. However, post-surgical complications including haemorrhage are common. A high clinical suspicion is needed to presume the diagnosis prior surgery and diminish the high morbidity of these tumours. [ABSTRACT FROM AUTHOR]

Background Posterior pituitary tumor is rare, and its optimal surgical strategy is undetermined. Surgical removal of posterior pituitary tumors is often complicated by their hypervascularity. Prevention and preparation for excessive hemorrhage is crucial for safe resection. Case Description A 69-year-old woman presented with bitemporal hemianopsia and was found to have a sellar tumor with suprasellar extension, resembling a pituitary adenoma. Microscopic transsphenoidal resection was attempted but was interrupted by a massive intraoperative hemorrhage. Pathologic diagnosis was a spindle cell oncocytoma, and she was referred to our institution. She underwent preoperative embolization to decrease tumor vascularity, and resection was performed using a combined endoscopic endonasal and video-microscopic transcranial approach, using a 4K three-dimensional (3D) video microscope. The final diagnosis was a posterior pituitary tumor with immunopositivity for thyroid transcription factor-1, with preferred interpretation as a pituicytoma. Conclusions A combined endonasal and transcranial approach with preoperative embolization is a useful strategy for hypervascular posterior pituitary tumors. A 4K 3D video microscope is compact, and it provides high-resolution images, contributing larger surgical space and facilitating a multi-team surgery. Highlights • A hypervascular posterior pituitary tumor was resected with combined endonasal and transcranial approach. • Branches of external carotid arteries were embolized to reduce the vascularity of the tumor. • A 4K 3D video microscope contributed to larger surgical space and facilitating a multi-team surgery. [ABSTRACT FROM AUTHOR]

Purpose: Pituicytomas and spindle cell oncocytomas (SCOs) are two rare neoplasms of the sellar and suprasellar region, known to be challenging as they are extremely vascular and almost always misdiagnosed, altering our surgical planning and the patients’ outcomes. Also we argue that recent update concerning the pathology findings of these tumors should be more widely generalized to our practice.Methods: This is a retrospective multicenter study, reporting the clinical manifestations, radiological characteristics, histopathological features, treatment strategies and long-term outcomes of patients who have been treated for a Pituicytoma at various institutions in Paris, France over the past 10 years. In addition, we compared our results to the world literature in order to identify similarities concerning the radiographic diagnosis and the treatment strategies of these tumors.Results: Eight patients were operated on in four different hospitals. Misdiagnosis was constant before surgery, pituitary adenoma or craniopharyngioma being suspected. During surgery (transsphenoidal approach: six cases, transcranial approach: two cases) unusual tumors were noted, with important bleeding in most cases. Complete resection could be obtained in five patients. Pathological diagnosis was confirmed in all cases. During the follow up two recurrences occurred. One was subsequently treated with radiotherapy, the other underwent a second surgery.Conclusion: Recent updates concerning the histological diagnosis of pituicytomas should be generalized to our practice in order to provide a better understanding of this rare pathology and its natural course. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) is a newly described rare entity simulating clinicoradiological features of a nonfunctional pituitary adenoma and is corresponding to the category of World Health Organization grade I tumor. However, because of the reported incidence of recurrence and invasive presentation in some cases, its categorization as a low grade tumor is questionable. Earlier, it was thought to arise from the folliculostellate cells of adenohypophysis. Recently, few reports have described expression of thyroid transcription factor-1 [TTF-1], which is a specific marker for pituicytes of neurohypophysis, suggesting this tumor to be a variant of pituicytoma. We describe a case of SCO in a 28-year-old young female patient with TTF-1 immunopositivity, and ultra-structurally showing abundant mitochondria along with few neurosecretory granules. [ABSTRACT FROM AUTHOR]

The fourth edition of the World Health Organization classification of endocrine tumors (EN-WHO2017) was released in 2017. In this new edition, changes in the classification of non-neuroendocrine tumors are proposed particularly in tumors arising in the posterior pituitary. These tumors are a distinct group of low-grade neoplasms of the sellar region that express thyroid transcription factor-1, and include pituicytoma, granular cell tumor of the sellar region, spindle cell oncocytoma, and sellar ependymoma. This short review focuses on the classification of posterior pituitary tumors newly proposed in EN-WHO2017, and controversies in their pathological differential diagnosis are discussed based on recent cases. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) of the adenohypophysis is an extremely rare sellar-region tumor that creates clinicopathologically relevant problems in the differential diagnosis and difficulties in patient management due to the limited data available regarding its biological behavior. A sellar/suprasellar mass was detected and surgery was performed in a 61-year-old male admitted for impaired vision. The patient was re-operated on twice due to persistence of symptoms and an increase in tumor size at the third and sixth postoperative month. The histopathological examination revealed tumor cells with oncocytic cytoplasm and spindleepithelioid morphology fascicles/layers/pseudoacinar structures. Neoplastic cells showed a positive immunoreactivity with vimentin, epithelial membrane antigen, galectin 3, thyroid transcription factor-1, and anti-mitochondrial antibodies, and a negative immunoreactivity with epithelial markers, pituitary hormones, and neuroendocrine markers. Compared with the first biopsy sample, the material obtained from recurrent lesions was histologically characterized by increased pleomorphism, atypia, and mitotic activity. Although SCO is defined as Grade I according to the current World Health Organization classification, the considerable risk of early recurrence should be taken into account, especially in cases with atypiapleomorphism and increased mytotic activity. [ABSTRACT FROM AUTHOR]

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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 14 years, 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. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) is a rare pituitary tumor, classified as a WHO grade I neoplasm. Due to its rarity, SCO is often preoperatively misdiagnosed as a pituitary macroadenoma. In the present study we report two recent cases of SCO, a 61-year-old male and a 65-year-old female presenting at Treviso General Hospital between March 2014 and April 2015. Tumor resection was achieved by endoscopic transsphenoidal approach but massive hemorrhagic events hampered surgery, endangering the patient's life in both cases. Both tumors featured fascicles of spindle cells with eosiniphilic cytoplasm expressing vimentin, S-100 and thyroid transcription factor-1 (TTF-1). The diagnosis of SCO was confirmed by second opinion in both cases. Extensive review of available literature, about 30 cases from 2002 to 2015, provided valuable clinical data for preoperative diagnosis and surgical removal of SCO tumors. [ABSTRACT FROM AUTHOR]

Purpose: To analyse the antigen expression profiles of 27 cases of pituicytoma, spindle cell oncocytoma, and granular cell tumour of the sellar region concerning a common pituicytic origin of neoplastic cells. Methods: Material from 12 female and 15 male patients (13 granular cell tumours of the sellar region, 10 pituicytomas, four spindle cell oncocytomas) collected in the German Registry of Pituitary Tumours between 1993 and 2015 was re-evaluated according to the current WHO classification of tumours of the central nervous system and supplementary immunohistochemistry including S100-protein, CD56, CD68, thyroid transcription factor-1 (TTF-1), and Ki-67 was performed. Results: S100-protein was detected in all 27 tumours and TTF-1 in all 16 tumours that were assessed. Vimentin was expressed in all 13 cases investigated whereas broad spectrum cytokeratin was not detected in any of 14 evaluated cases. GFAP was observed in nine out of 21 cases. 15 out of 17 investigated lesions showed some CD68 expression and five out of 14 cases were labelled with CD56 antibodies. Proliferative activity did not differ significantly between the three tumour subgroups although one primary and one recurrent pituicytoma showed exceptionally high Ki-67-proliferation indices of 15.3 and 12.7 %, respectively (means: granular cell tumour of the sellar region 2.0 %, pituicytoma 2.8 %, spindle cell oncocytoma 2.7 %). Conclusions: The study confirms and expands earlier data and is in line with the notion that the three tumour types are variants of pituicytoma. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) of the pituitary gland is very difficult to differentiate from other pituitary neoplasms and is often misdiagnosed based on imaging procedure features. We report a rare case of SCO arising from the neurohypophysis and suggest a useful diagnostic criterion for accurate diagnosis and surgical pitfalls. A 53-year-old man was admitted to our hospital with slight headache and diplopia. Neuroimaging revealed pituitary tumour in the suprasellar and sellar regions with speckled gadolinium enhancement on T1-weighted magnetic resonance imaging, as a so-called blooming artefact. The enhanced anterior pituitary gland was located anteriorly. Computed tomography (CT)-scan demonstrated an isodense mass without calcification showing strong contrast enhancement with iodine contrast medium. Laboratory findings showed no abnormalities. Subtotal resection of the tumour was achieved by an endoscopic endonasal transsphenoidal approach. Histological examinations showed spindle-shaped to epithelioid tumour cells featuring eosinophilic and granular cytoplasm staining strongly for anti-mitochondrial antibody and thyroid transcription factor 1. The tumour was therefore diagnosed as SCO, belonging to tumours of the posterior pituitary. Headache and diplopia were disappeared immediately postoperatively, and follow-up at 12 months demonstrated no signs of recurrence. SCO of the pituitary gland is a rare tumour that originates from the neurohypophysis and is difficult to diagnose on routine neuroimaging procedure. Accurate diagnosis requires careful identification of clinical signs, neuroimaging features including contrast-enhanced CT, and analysis of combined results from morphological and immunohistochemical evaluation of tumour tissue. • Spindle cell oncocytoma of the pituitary gland is rare tumour of sellar and suprasellar regions that originates from the neurohypophysis. • This tumour is very difficult to differentiate from other pituitary neoplasms and is often misdiagnosed based on imaging studies. • Accurate diagnosis requires careful identification of clinical signs, neuroimaging and analysis of immunohistochemical studies. [ABSTRACT FROM AUTHOR]

We present a case of spindle cell oncocytoma (SCO) of the adenohypophysis in a 70-year-old Vietnamese male. The patient was admitted to Cho Ray Hospital after suffering from headache and visual disturbance for 6 months. Clinicians detected a 60 × 55 × 45 mm 3 mass located in the suprasellar-sellar region. Histopathologically, the resected tumor was composed of spindle cells with oncocytic appearance. Immunohistochemical examination revealed expression of anti-mitochondria antibody (AMA), vimentin, thyroid transcription factor 1 (TTF-1), epithelial membrane antigen (EMA) and galectin-3. These histologic and immunohistochemical findings are suggestive of SCO. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) of the adenohypophysis is a rare tumor in the sellar region. Due to its rarity, little information is available regarding SCO. It is often misdiagnosed as another type of sellar tumor. In the present study, two cases of SCO were reported. One patient was a 35-year-old female presenting with decreased visual acuity, amenorrhea and lactation. The other patient was a 62-year-old female with no clear symptoms or signs. Cranial magnetic resonance imaging (MRI) revealed a suprasellar mass with marked homogeneous enhancement in the two cases. A craniotomy was performed to completely resect the tumors. The tumors were immunopositive for vimentin, epithelial membrane antigen (EMA), S-100 and thyroid transcription factor-1 (TTF-1). The tumors were pathologically diagnosed as SCO. No recurrence occurred during the follow-up period of 15-21 months. In the present study, the literature was reviewed and the clinical data, imaging features, intraoperative findings and recurrence of 24 cases were analyzed in the literature as well as the present two cases. The average age of the SCO patients was 58.5 years and no gender preference was observed for the disease. The tumor exhibited homogeneous enhancement on the MRI. The intraoperative assessment revealed that the tumor had a rich blood supply and the SCO tumors were immunopositive for vimentin, S-100, EMA and TTF-1. These findings provided valuable clinical data for the preoperative diagnosis and surgical removal of SCO tumors. [ABSTRACT FROM AUTHOR]

We describe herein the unique case of a 70-year-old male with a TTF-1-positive non-adenomatous sellar tumor that has unusual morphological and immunohistochemical features. MRI examination detected a 2-cm sellar mass that was enhanced heterogeneously. By histology, the tumor was composed of epithelioid and oncocytic cells arranged in a trabecular pattern with occasional luminal structures. The lesion was diffusely immunopositive for thyroid transcription factor-1 (TTF-1) and vimentin but negative for S100 protein and GFAP. Immunoreactivity for epithelial membrane antigen, low molecular weight cytokeratin (CAM 5.2), and neuronal markers was also observed in the tumor cells. By electron microscopy, the tumor cells were filled with abundant mitochondria and extended microvillous projections into small extracellular and intracellular lumens. TTF-1 is considered to be an excellent marker of pituicytes, specialized glia of the neurohypophysis. This case can be regarded as a variant of pituicytoma, showing both ependymal differentiation and oncocytic changes. However, the immunoprofile was not completely consistent with a pituicyte lineage; the epithelial features suggested a possibility of folliculostellate cell origin. TTF-1-positive sellar neoplasms might therefore have variable morphological and immunohistochemical profiles. For suitable classification of TTF-1 positive sellar neoplasms, their histological features should be carefully re-evaluated. [ABSTRACT FROM AUTHOR]

Purpose: Pituicytomas and spindle cell oncocytomas (SCOs) are extremely rare neoplasms of the sellar and suprasellar region that can often mimic pituitary adenomas. To date, there are relatively few cases of pituicytomas and SCOs reported; and most of these are small case series. Methods: In this paper, we provide a retrospective review of the treatment, imaging characteristics, post-operative course, and histopathology of five cases of pituicytomas and two SCOs treated at the University of California, San Francisco (UCSF) over a 10-year period from 2003 to 2013. Results: We find that pituicytomas and SCOs present similarly to pituitary adenomas, and look identical on CT or MR imaging. We histopathologically confirmed all pituicytomas with a combination of hematoxylin and eosin morphology and immunohistochemical positivity for vimentin and S100; SCOs stain for anti-mitochondrial antigen and endothelial membrane antigen. We observe positive thyroid transcription factor 1 (TTF1) immunohistochemistry in both cases of SCO, as well as in both of the cases of pituicytoma in which TTF1 staining was available. Conclusions: This represents the largest single-institution case series of pituicytomas and SCOs to date, and also includes the first description of the management of a pregnant female with SCO. Our findings are consistent with the idea of common histogenesis for pituicytomas and SCOs, and also raise the possibility of more aggressive growth in SCOs as compared to pituicytomas. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma of the adenohypophysis is a rare tumor classified by the World Health Organization as benign with no malignant features. Recent publications have questioned its identity as a benign tumor, as six postsurgical recurrences of spindle cell oncocytoma (SCO) have been reported. This case is the first to illustrate the natural history of the disease due to delayed intervention, as the tumor showed virtually no growth. This is an important illustrative case to highlight that urgent surgery may not be required and conservative close serial surveillance MRI may be a valid management option. [ABSTRACT FROM AUTHOR]

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This article reviews recent literature on sellar region masses that most closely mimic nonsecretory pituitary adenomas: hypophysitis, pituicytoma, spindle cell oncocytoma, and granular cell tumor of neurohypophysis. Even today, these four entities often cannot be confidently distinguished from each other clinically or by preoperative neuroimaging features. Thus, they often come to biopsy/surgical resection and require tissue confirmation of diagnosis. Causes of secondary and primary hypophysitis will be discussed, including two newly described types, IgG4 plasma cell hypophysitis and hypophysitis caused by anti-cytotoxic T-lymphocyte antigen 4 antibody therapy for cancer. For the neoplastic conditions, emphasis will be placed on literature that has emerged since these entities were first codified in the 2007 World Health Organization fascicle. The finding that immunohistochemical staining for thyroid transcription factor-1 is shared by pituicytoma, spindle cell oncocytoma, and granular cell tumor of neurohypophysis suggests common lineage and explains why histological overlap can be encountered. We incorporate our own experiences over the last 30 years from two referral institutions with specialty practices in pituitary region masses. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) is a rare non-functioning tumour of the pituitary which has just been formally recognized as a distinct entity by the 2007 WHO classification of brain tumours. We report a case of SCO who presented with symptoms of visual blurring, weight loss, intermittent vomiting and excessive tiredness of several months duration. Investigations revealed a bitemporal visual field defect, a panhypopituitary hormonal profile and a large pituitary tumour with suprasellar extension. He underwent a successful trans-sphenoidal resection of the pituitary tumour but it subsequently recurred twice at 9 months interval which required further two debulking procedures. A diagnosis of SCO was made based on its unique histologic and staining properties. To date there are only ten reported cases of SCO in total with only two of these cases being recurrent. Our case displayed the most aggressive clinical course despite having a low Ki-67 index contrary to the previously reported cases of recurrent SCO. [ABSTRACT FROM AUTHOR]

Spindle cell oncocytoma (SCO) is a rare, non-adenomatous tumor originating from the anterior pituitary gland. Composed of fusiform, mitochondrion-rich cells sharing several immunophenotypic and ultrastructural properties with folliculo-stellate cells (FSC), SCO has been proposed to represent a neoplastic counterpart of the latter. To date, however, SCO has failed to meet one criterion commonly used in histological-based taxonomy and diagnostics; that of recapitulating any of FSCs' morphologically defined developmental or physiological states. We describe a unique example of SCO wherein a conventional fascicular texture was seen coexisting with and organically merging into follicle-like arrangements. The sellar tumor of 2.7 × 2.6 × 2.5 cm was transphenoidally resected from a 55-year old female. Preoperative magnetic resonance imaging indicated an isointense, contrast enhancing mass with suprasellar extension. Histology showed multiple rudimentary to well-formed, follicle-like cavities on a classical spindle cell background; while all the participating cells exhibited an SCO immunophenotype, including positivity for S100 protein, vimentin, EMA, Bcl-2, and TTF-1, as well as staining with the antimitochondrial antibody 113-1. Conversely no expression of GFAP, follicular-epithelial cytokeratin, carcinoembryonic antigen, or anterior pituitary hormones was detected. Ultrastructurally, tumor cells facing follicular lumina displayed organelles of epithelial specialization, in particular surface microvilli and apical tight junctions. This constellation is felt to be reminiscent of FSCs' metaplastic transition to follicular epithelium, as observed during embryonic development and physiological renewal of the hormone-secreting parenchyma. Such finding is apt to being read as a supporting argument for SCO's descent from the FSC lineage. [ABSTRACT FROM AUTHOR]

Background: Spindle cell oncocytoma (SCO) and pituicytoma are rare nonfunctioning tumors of the pituitary. Both tumors are low grade and macroscopically indistinguishable from a nonfunctioning pituitary adenoma. We report one case of SCO and one case of pituicytoma and review the previous literature. Case Description: One patient was a 39-year-old man who presented with progressive headache, visual blurring, and polyuria of 3-year duration. He underwent partial resection (30% of the tumor) and postoperative adjuvant radiation therapy. Histopathology revealed SCO. However, after 9 months, the residual tumor grew and partial resection (70% of the tumor) was performed again. Four months after the second surgery, the tumor recurred again and he underwent transsphenoidal resection of the tumor with stable residual tumor to date. The other patient was a 59-year-old man who presented with a 3-month history of visual decline, fatigue, difficulty in writing, and polyuria. He underwent transsphenoidal resection (total) of the tumor. Histopathology revealed pituicytoma. He has been stable without evidence of recurrence for 1 year and 4 months. Conclusion: To date, there are 15 reported cases of SCO and 45 reported cases of pituicytoma including our cases. An incomplete resection of the tumor was a significant risk factor for recurrence in both SCO and pituicytoma (P = 0.0014 and P = 0.019, respectively). These tumors have a tendency to be hypervascular, which may hamper total resection. Epithelial membrane antigen (EMA) and mitochondria positivity is characteristic to SCO and they are considered to be important immunomarkers to distinguish these tumors. [ABSTRACT FROM AUTHOR]