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Ultrastructure of SCO. The neoplastic cells of spindle morphology with accumulation of swollen mitochondria exhibiting disrupted lamellar cristae. Intermediate junctions are seen. Original magn. × 2850.
Source publication
Spindle cell oncocytoma (SCO) of the pituitary gland is a relatively recently established, very rare subtype of adenohypophysis tumours that was introduced as a distinct clinicopathological entity in the fourth edition of WHO classification of the central nervous system tumours (2007). It is non-endocrine neoplasm of the anterior pituitary that occ...
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Citations
... These tumor cells do not express GFAP, cytokeratins CAM2.5, CD34, CD68, synaptophysin, chromogranin, BCL-2, or smooth muscle actin (1,3). Similar expression of vimentin, S-100 protein, EMA, and galectin-3 together with the presence of desmosomes and intermediate junctions suggests their possible derivation from folliculostellate cells of the adenohypophysis, which are capable of divergent differentiation as a type of stem cell (4,5). ...
Introduction
Spindle cell oncocytoma (SCO) of the pituitary gland is increasingly established with improvements in histological and immunohistochemical examination. However, the diagnosis was often mistaken based on imaging studies and nonspecific clinical manifestations.
Purpose
This case is presented to provide an overview of the characteristics of the rare tumor as well as to demonstrate the difficulties in diagnosis and current treatments.
Clinical discussion
The pathogenesis of SCO remains unclear, and a possible origin was described. Further research is needed to optimize pre-operative diagnosis and surgical strategy.
Conclusion
SCO should be considered when images indicate some features. Gross total resection (GTR) after surgery seems to have better long-term tumor control, and radiotherapy may help decrease tumor progression in patients with non-GTR. Regular follow-up is advised because of the higher recurrence rate.
... 3,6 Ultrastructurally, SCOs appear in a spindled shape with eosinophilic cytoplasm full of mitochondria rich in lamellar cristae. 6,7 SCO commonly presents in adults with slow but progressive symptoms. Our literature review of 81 cases (Table 1) from 2002 to 2021 determined that the most common presenting symptoms are visual deficits (67.9%), hypopituitarism (37.0%), headache (33.3%), and nausea (11.1%). ...
BACKGROUND
Spindle cell oncocytoma (SCO) of the pituitary gland is an extremely rare nonfunctional World Health Organization grade I tumor. SCOs are often misdiagnosed as nonfunctional pituitary adenomas on the basis of preoperative imaging. They are often hypervascular and locally adherent, which increases hemorrhage risk and limits resection, leading to increased risk of recurrence. The authors report a case of SCO treated at their institution and provide a review of the current literature.
OBSERVATIONS
SCO of the pituitary gland can be a rare cause of progressively growing pituitary tumors that presents similarly to nonfunctional pituitary adenoma. Endoscopic transsphenoidal resection of the tumor by a multidisciplinary team allowed total resection despite local adherence of the tumor. Postoperatively, the patient’s visual symptoms improved with persistence of secondary adrenal insufficiency and secondary hypothyroidism.
LESSONS
Careful resection is needed due to SCO’s characteristic hypervascularity and strong adherence to minimize local structure damage. Long-term follow-up is recommended due to the tendency for recurrence.
... Surgery is the treatment of choice for symptomatic PPT usually by the transsphenoidal route (TSS). However, PPTs, including both pituicytomas and SCOs, often show recurrence/ persistence following surgery (16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67). ...
... Even in our pooled patient analysis of only TTF-1 positive neoplasms, there was a 39.3% prevalence of non-remitting disease. This tumour behaviour is seldom attributable to a high proliferation rate or invasiveness (19,21,31,40,41,44,53,55). Interestingly, recurrence/persistence is also seen in tumours that are non-invasive and with a low proliferation rate (Ki-67<3%) (18,19,21,24,31,32). ...
Objective: Posterior pituitary tumours (PPTs) are rare neoplasms with the four recognised subtypes unified by thyroid transcription factor-1 (TTF-1) expression, according to the 2017 WHO classification. Though traditionally defined as low grade neoplasms, a substantial proportion of them show recurrence/persistence following surgery.
Methods: We selected patients with PPTs in our cohort of 1760 patients operated for pituitary tumours over the past ten years. The clinical, radiological, hormonal, histopathological profiles and long-term outcomes of the three cases identified (two pituicytomas and one spindle cell oncocytoma, SCO) were analysed. Following a literature review, data of all published cases with documented TTF-1 positive pituicytomas and SCOs were analysed to determine the predictors of recurrence/persistence in these tumours.
Results: Patients presented with compressive features or hypogonadism. Two had sellar-suprasellar masses. One had a purely suprasellar mass with a pre-operative radiological suspicion of pituicytoma. Two were operated by transsphenoidal surgery and one transcranially guided by neuronavigation. Histopathology confirmed spindle cells in a storiform arrangement and low Ki67 index. Immunohistochemistry showed positive TTF-1, S-100 expression and variable positivity for EMA, vimentin and GFAP. Re-evaluation showed recurrence/persistence in two patients. A literature review of recurrent/persistent pituicytoma (n=17) and SCO (n=9) cases revealed clinical clues (headache for pituicytomas, male gender for SCO), baseline tumour size (≥20.5mm with sensitivity exceeding 80%) and longer follow-up duration as determinants of recurrence/persistence.
Conclusion: PPTs are rare sellar masses with quintessential TTF-1 positivity. Recurrent/persistent disease following surgery is determined by greater tumour size at baseline and duration of follow-up. This warrants intensive and long-term surveillance in these patients.
... SCO is a rare nonfunctioning tumor of the sella turcica and accounts for approximately 0.1% to 0.4% of all sellar regions tumors [1,5,6]. Previously, this tumor was suspected to be derived from folliculostellate cells of the anterior pituitary gland, which are sustentacular cells of adenohypophysis [1]. ...
... Histologically, the tumor is composed of spindle cells arranged in interlacing fascicular structures with intervening blood vessels and should be differentiated with null cell adenoma with oncocytic change, meningioma, schwannoma, granular cell tumor, solitary fibrous tumor, and paraganglioma [8]. Immunohistochemically, SCO is negative for neuroendocrine markers such as synaptophysin and chromogranin, pituitary hormones, cytokeratin, desmin, and smooth muscle actin and typically positive for TTF-1, EMA, vimentin, and galectin-3 [1, [3][4][5][6]. The ultrastructural characteristics of SCO include cytoplasmic accumulation of numerous mitochondria and several cell-cell junctions, mainly short desmosomes [9]. ...
We present a rare case of spindle cell oncocytoma (SCO) of the sella turcica with malignant histologic features and rapid progression. A 42-year-old woman experienced bilateral blurred vision and was preoperatively misdiagnosed as having a pituitary macroadenoma on magnetic resonance imaging. After surgery, SCO was diagnosed by the histopathologic features of interlacing fascicles of spindle tumor cells with finely granular, eosinophilic cytoplasm. Focal anaplastic changes and necrosis were present. Immunohistochemically, the tumor cells were positive for vimentin, epithelial membrane antigen, S-100, galectin-3, and thyroid transcription factor 1. Four months later, the tumor had progressed, and second surgery with adjuvant radiotherapy was performed; the patients remains under observation. In this report, we proposed distinctive radiologic features for differential diagnosis between SCO and other pituitary tumors.
... 2,78 The presence of oncocytic changes may lead to an erroneous diagnosis of an oncocytic pituitary adenoma (especially oncocytic null cell pituitary adenoma) without the appropriate immunohistochemical evaluation. [84][85][86] In contrast to granular cell tumor, cytoplasm is eosinophilic but finely granular (because of accumulation of mitochondria). Staining with PAS is also negative. ...
The pituitary gland is the site of numerous neoplastic and inflammatory processes. The overwhelmingly most frequent tumors arise from cells of the anterior lobe, the pituitary neuroendocrine tumors (PitNETs). Immunohistochemistry assay staining for pituitary hormones is the core tool for classifying PitNETs, resulting in the diagnosis of somatotroph PitNETs, lactotroph PitNETs, and so on. For cases showing no hormonal expression, the updated WHO classification system now considers the assessment of several transcription factors: PIT-1 (pituitary-specific POU-class homeodomain transcription factor); T-PIT (T-box family member TBX19); and SF-1 (steroidogenic factor regulating gonadotroph cell differentiation) before rendering a diagnosis of null cell adenoma. Other tumors and disease processes of this site often mimic PitNETs radiographically and sometimes even clinically (ie, compression of the optic chiasm). These potpourri of processes include germ cell neoplasms (especially germinomas), tumors that originate from Rathke's pouch (craniopharyngiomas, Rathke's cleft cyst), tumors that originate from the posterior lobe of the pituitary (pituicytoma, spindle cell oncocytoma, granular cell tumor), and tumors that originate from the meninges (especially meningiomas). In addition to neoplasms, several described inflammatory and related conditions exist that need to be distinguished from PitNETs. These include lymphocytic hypophysitis and Langerhans cell histiocytosis, a neoplastic disorder of histiocytes. In this review, we aim to briefly describe the main pituitary and sellar lesions, with emphasis on the most common tumors, the PitNETs.
... It accounts for 0.1%-0.4% of all sellar tumors with no sex predilection. [1,2] SCO was first reported by Roncaroli et al. in 2002. [2] The tumor arises from the folliculostellate cells of adenohypophysis. ...
... [1,2] SCO was first reported by Roncaroli et al. in 2002. [2] The tumor arises from the folliculostellate cells of adenohypophysis. In the 2007 classification of the World Health Organization (WHO) for the central nervous system (CNS) tumors, SCO in the neurohypophysis was described as a distinct diagnosis. ...
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.
... According to the current hypothesis, PPTs originate from specific morphological variants of pituicytes (specialized glial cells, i.e. pituicytomas from the light/major variant, granular cell tumors from the granular variant, spindle cell oncocytomas from the oncocytic variant and sellar ependymomas from the ependymal pituicytes) [4]. PPTs show a common immunohistochemical feature, diffuse nuclear expression for the thyroid transcription factor-1 (TTF-1), a homeobox transcription factor [4]. Spindle cell oncocytomas (SCO or oncocytomas) are rare, benign tumors accounting for 0.1-0.4% of all sellar tumors [5]. Until mid-2017, there have been only 34 cases published [3]. ...
Spindle cell oncocytomas (SCO) of the pituitary are rare tumors accounting for 0.1–0.4% of all sellar tumors. Due to their rarity, little information is available regarding their pathogenesis. Our aim was to investigate miRNA expression profile of pituitary oncocytomas. Total RNA was extracted from 9 formalin-fixed paraffin embedded pituitary samples (4 primary, 3 recurrent oncocytomas and 2 normal tissues). Next-generation sequencing was performed for miRNA profiling. Transcriptome data of additional 6 samples’ were obtained from NBCI GEO database for gene expression reanalysis and tissue-specific target prediction. Bioinformatical analysis, in vitro miRNA mimics transfection, luciferase reporter system and AlamarBlue assay were applied to characterize miRNA’s function. 54 differentially expressed miRNAs and 485 genes in pituitary SCO vs. normal tissue and 8 miRNAs in recurrent vs. primary SCO were determined. Global miRNA downregulation and decreased level of DROSHA were detected in SCO samples vs. normal tissue. Transcriptome analysis revealed cell cycle alterations while miRNAs influenced mainly metabolic processes (tricarboxylic acid cycle-TCA, carbohydrate, lipid metabolism). Through miRNA-target interaction network the overexpressed Aconitase 2 potentially targeted by two downregulated miRNAs (miR-744-5p, miR-127-3p) was revealed. ACO2 and miR-744-5p interaction was validated by luciferase assay. MiR-127-3p and miR-744-5p significantly decreased cell proliferation in vitro. Our study firstly reported miRNA profile of pituitary oncocytoma. Our results suggest that tumor suppressor miRNAs may have an essential role in the pathogenesis of pituitary oncocytoma. Earlier reports showed downregulated TCA cycle in SCO which is extended by our results adding the role of miR-744-5p targeting ACO2.
... Also, enlarge transtubercular approach is an alternative possibility when an intraoperative finding or previous histology suggest PPT and increase the chance of total resection [42]. Although a direct comparison between both methods is probably not reliable, craniotomy as might expected is associated with higher risks of visual damage [13,42,60] and transphenoidal approach with subtotal resection [12,13,19,20,23,26,30,31,34,37,41,42,53,55,57,73,85,88,98,104]. Perioperative and postsurgical complications are common in patients with PPT. ...
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.
... Not including the cases reported in this paper (or the subset reported earlier by Miller et al. 23 ), a total of 39 cases of SCO have been reported in the literature as case reports and case series (Table 3). [1][2][3][4][5][8][9][10][11][13][14][15]19,20,[23][24][25][26][27][28][29][30][31][32][33][34][35][36] These lesions are often initially managed by a TS approach because preoperative radiological features are suggestive of a pituitary adenoma. 26 Of 37 cases for which type of resective surgery was reported in the literature, 32 (86.5%) were managed initially with TS resection and 5 (13.5%) were managed initially with craniotomy. ...
... There are also case reports of SCOs managed transcranially, which has been posited to be of benefit given their highly vascular and adherent nature. 9,20,25,29 In this series, one operation at an OSH had to be aborted because of intraoperative bleeding during a standard craniotomy (case 4), and it is possible that the transcranial approach may provide more intraoperative maneuverability to deal with excessive bleeding and better access to adherent areas of tumor. Nevertheless, each of the 6 SCOs reported on here was ultimately managed by the TS approach. ...
OBJECTIVE
The authors report the diagnosis, management, and outcomes of 6 cases of spindle cell oncocytoma (SCO) in an effort to guide clinical diagnosis and management of these uncommon lesions.
METHODS
This study is a retrospective review of cases involving adult patients who underwent resection of pituitary lesions at the authors’ institutions between January 2000 and October 2017. The authors identified patients with histopathological confirmation of SCO and collected clinical data, including preoperative, perioperative, and postoperative management, complications, and outcomes.
RESULTS
Six patients with SCO were identified. Clinical findings at initial presentation included visual disturbances, dizziness, and headache. All patients underwent resection. Four resections were initially performed by the transsphenoidal approach, and 2 resections were performed by craniotomy at an outside institution with subsequent transsphenoidal reoperations. Neither necrosis nor increased mitotic activity was seen in the tumor samples. All samples stained positive for S100 protein and thyroid transcription factor 1 and negative for glial fibrillary acidic protein and pituitary hormones. Five of the samples stained positive for epithelial membrane antigen. The average MIB-1 index was 8.3% (range 2–17). Postoperatively, 3 of the 6 patients received further treatment for progression of residual tumor or for recurrence, 2 have stable residual tumor, and 1 has had no recurrence after gross-total resection. Two patients developed postoperative complications of transient sixth cranial nerve palsy and diplopia. There were no other complications.
CONCLUSIONS
SCO poses both a diagnostic and therapeutic challenge. These tumors are often initially misdiagnosed as nonfunctional pituitary adenomas because of their sellar location and nonspecific symptomatology. Postoperatively, SCO must also be distinguished from other neoplasms of the posterior pituitary gland through histopathological examination. Resection of SCO can be challenging, given its highly vascular and adherent nature. Long-term follow-up is critical, as the tumor is associated with higher recurrence and progression rates compared to other benign neoplasms of the sella.
... в 2002 г. [5]. Она развивается из онкоцитарных клеток аденогипофиза и составляет 0,1-0,4 % всех новообразований хиазмально-селлярной области [30]. К настоящему времени в мировой литературе описано не более 26 случаев ВКО хиазмальной области [1,5,[30][31][32][33][34][35][36][37][38][39][40]. ...
... Она развивается из онкоцитарных клеток аденогипофиза и составляет 0,1-0,4 % всех новообразований хиазмально-селлярной области [30]. К настоящему времени в мировой литературе описано не более 26 случаев ВКО хиазмальной области [1,5,[30][31][32][33][34][35][36][37][38][39][40]. ...
