Subtotal Tumor Resection Research Articles

ATTEMPTED RADICAL REMOVAL OF PARASAGITTAL MENINGIOMA: RISKS AND BENEFITS

Background: Parasagittal meningiomas involving the superior sagittal sinus (SSS) pose formidable obstacles to surgicalmanagement. Invasion is often considered a contraindication to surgery because of associated morbidity, such as cerebral venous thrombosis. Aim of the work: was to evaluate the risk/benefit ratio in attempting radical excision of parasagittal meningiomas involving the superior sagittal sinus. Patients and methods: The study consisted of 25 patients who had undergone surgery for parasagittal meningioma. Patients with meningioma involving the anterior third of the sinus underwent radical removal. Patients with meningioma that was involving the middle and posterior third of the sinus had a radical removal if the sinus was completely obliterated, and subtotal removal of tumors that are infiltrating but not obliterating the SSS. Results: 23 patients (92%) had radical tumor resection achieving Simpson GI and 2 patients (8%) had subtotal tumor resection achieving Simpson GIV. There were 3 postoperative transient neurological deterioration (12%) and 2 postoperative deaths (8%). The recurrence rate in the study was 5%, with a follow-up for 24 months.Conclusion: The benefits must be carefully weighed against the risks deciding between more aggressive, radical, or less aggressive subtotal resections. The less aggressive subtotal resections if the sinus ispartially occluded may be a reasonable choice.

Non-functioning pituitary macroadenoma: surgical outcomes, tumor regrowth, and alterations in pituitary function-3-year experience from the Iranian Pituitary Tumor Registry.

The aim of this study was to assess clinical features and therapeutic outcomes in patients with clinically non-functioning pituitary macroadenomas (NFPMAs). This is a retrospective cohort study of NFPMA patients treated surgically and followed periodically between 2015 and 2017 in a tertiary care center in Iran. Descriptive analysis was performed applying appropriate tests. Binary logistic regression models were used to determine the predictive factors for subtotal tumor resection (STR) and hormonal recovery. Data were analyzed by Stata software. A total of 71 patients with a mean age of 50.6 ± 1.4years were studied. The mean diameter of the adenoma was 26.8 ± 1.1mm. The most frequent symptoms were headache (85.75%), visual field defect (VFD) (78.3%), and hypogonadal symptoms (40.3%). Gross total resection (GTR) was achieved in 45.1%. Preoperative hypopituitarism was observed in 50.7% of patients. Recovery of at least one axis occurred in 36.1% of the patients suffering from hypopituitarism preoperatively, while new-onset postoperative hormonal deficiency appeared in 14.3% of patients. Multivariate analyses showing preoperative tumor size (OR = 38.2; P = 0.008) and cavernous sinus extension (OR = 13.4; P = 0.020) were predictors of STR. Moreover, hormonal recovery was observed not to be related to age, gender, tumor size, or the extent of tumor resection. Tumor size and cavernous sinus extension are the main predictors for STR. Notably, recovery of the gonadal axis in a large proportion of patients supports the surgical resection of NFPAM in patients suffering from gonadal deficiency, even in the absence of VFD.

Extension Pathways of Pituitary Adenomas with Cavernous Sinus Involvement and Its Surgical Approaches

Resection and remission rates are low in pituitary adenomas that invade the cavernous sinus (CS) because of the complexity of the region. In this study, the authors describe the invasion patterns and surgical approaches for these corridors. Endoscopic transsphenoidal surgery was performed in 1849 patients between August 1997 and 2017; 381 of these patients were included in the study because of the presence of CS invasion. CS invasion pathways and CS compartments were defined, and these patients were divided into 3 groups according to their invasion corridors. The cases were also categorized according to the Knosp classification. A total of 381 patients were included, 199 of whom were men. The mean follow-up duration was 34 months. In preoperative magnetic resonance imaging measurements, 2 of 381 patients had microadenoma, 350 of 381 had macroadenoma, and 29 of 381 had giant adenoma. When the early postoperative magnetic resonance images were compared with the preoperative images, 91 of 381 (23.9%) patients had subtotal tumor resection, 83 of 381 (21.8%) had near-total tumor resection, and 207 of 381 (54.3%) had gross total tumor resection. Considering the invasion classification with endoscopic confirmation, there were 168 of 381 (44.1%) cases of isolated medial corridor involvement, 74 of 381 (19.4%) cases of isolated lateral corridor involvement, and 139 of 381 (36.5%) cases of total involvement cases. Medial wall defect has no importance in the invasion. Similarly, seeing the existence of pitholes is not an invasion criterion. Pituitary adenomas invade 4 compartments: the superior, anteroinferior, posterior, and lateral compartments of the CS via 2 corridors. Increased experience, novel approaches, and endoscopic developments have contributed immensely to this field.

Clinical features, surgical management, and long-term prognosis of intraventricular meningiomas: A large series of 89 patients at a single institution.

Intraventricular meningiomas are rather rare and only represent a small proportion of all intracranial meningiomas. Data are still limited toward this peculiar entity and surgical resection remains challenging for neurosurgeons. The purpose of present study is to demonstrate clinical features, surgical treatment, and potential risk factors determined long-term prognosis of intraventricular meningiomas.A total of 89 surgically treated and histopathologically confirmed intraventricular meningiomas were identified in our institution from 2008 to 2018. Clinical features, neuroimaging findings, surgical records, and prognosis data were extracted and reviewed retrospectively. Group comparison and recurrence-free survival analysis were performed.Female predominance was well established with a sex radio of 2.1:1. Raised intracranial pressure and decline of visual acuity were 2 chief symptoms that patients generally complained of. Preoperative magnetic resonance imaging (MRI) scans were performed in all patients and there was a trend toward lateral ventricular meningiomas were larger in size than others (P = .07). Superior parietal lobule and temporal approach were widely adopted and lateral/4th ventricular meningiomas were more easily to acquire total tumor resection as compared with 3rd ventricular meningiomas (P = .03). After an average follow-up of 57.3 months, 6 patients experienced recurrence of disease in our series. Individuals with subtotal resection (P < .001) and higher World Health Organization classification (P = .001) were more prone to relapse.Intraventricular meningiomas presented with a wide variety of symptoms. Surgery remained 1st treatment of choice and optimal surgical approach should be planned individually based on preoperative MRI evaluation. Patients underwent subtotal tumor resection and with malignant tumor nature should be carefully monitored during follow-up.

Subdural hydroma; A postoperative complication of desmoplastic infantile ganglioglioma and astrocytoma: A report of two cases and literature review

Abstract Introduction Desmoplastic infantile ganglioglioma (DIG) and desmoplastic infantile astrocytoma (DIA) are rare benign childhood intracranial tumors that are classified under neural and mixed glioneuronal tumors. Although many authors have written on surgical treatment of these disease conditions and indicated satisfactory outcomes, no author have written on the postoperative formation of a subdural hydroma after surgery. Case presentation We present two cases of DIG and DIA infants in whom we achieved total or subtotal tumor resection and observed postoperative hydroma as complications. Their main symptoms were vomiting, seizures and head enlargement. In both cases magnetic resonance imaging (MRI) revealed huge solid-cystic mass located in left frontal, temporal and parietal lobes. Histopathological staining confirmed the diagnosis of DIG and DIA in both cases. A second surgery may be required in patients with this kind of postoperative complication if clinical and radiological imaging evaluation reveal deteriorating of the patient's condition otherwise conservative treatment usually gives a total resolution of this complication. Conclusion The pathophysiology of this occurrence after surgery is as the result of a potential space created after excision of tumor and subsequent influx of CSF into the potential space between the subdural.

Results of Chordoma Patients Treated by Different Approaches in a Single Institution.

To discuss the optimal and recent treatment options based on clinical review of 16 chordoma patients. Data of the patients diagnosed and treated between 1999 and 2017 in Gazi University School of Medicine has been collected through patients’ files and the electronic database of hospital records. Statistical analysis was applied to evaluate the correlation between the progression free survival and treatment modalities. Nine of the 16 patients were women (56.3%). Half of the patients had intracranially located tumors, whereas the other 50% of the sample had spinal (n=5) and sacral (n=3) chordomas. The median follow-up time was 51.7 months. Recurrence was observed in 50% of patients, while the median recurrence time equaled to 27.6 months. Multivariate analysis results showed that age, gender tumor size, intra or extracranial location of tumor, treatment modalities, subtotal or grosstotal resection of tumor, radiotherapy dose, and techniques were not associated with recurrence. On the other hand, 2 patients are still under chemoterapy (imatinib, bevacizumab) without evident of recurrent disease. Despite the fact that surgery remains to be the cornerstone of treatment, total resection is not reasonable for all patients with chordomas. For this reason, adjuvant treatment for ensuring local control is highly important. If the residual tumor is of a small volume, SBRT may provide more advantages. Targeted treatment or chemotheapeutic agents may also be benificial for maintanence therapy. As the clinical awareness about chordomas is based on our series, aggressive multi-modality treatment options should be applied in the adjuvant therapy.

Effects of total and subtotal glial tumor resection on survival

Effects of total and subtotal glial tumor resection on survival

Evaluation of a Novel General Pituitary Hormone Score to Evaluate the Function of the Residual Anterior Pituitary (Adenohypophysis) in Patients Following Surgery for Pituitary Adenoma.

BackgroundThe aim was to develop and assess a general pituitary hormone score to evaluate the function of the anterior pituitary (adenohypophysis) in patients following resection of pituitary adenomas.Material/MethodsSixty-six patients with pituitary null cell macroadenoma (1–3 cm diameter) (N=38) and pituitary null cell giant adenoma (≥3 cm diameter) (N=28) had preoperative and postoperative data including magnetic resonance imaging (MRI) and measurement of six pituitary hormones levels, adrenocorticotropic hormone (ACTH), growth hormone (GH), thyroid-stimulating hormone (TSH), prolactin (PRL), follicle-stimulating hormone (FSH), and luteinizing hormone (LH). The postoperative general pituitary hormone score, for 57 patients who underwent subtotal resection (>60%) and nine patients who underwent partial resection (≤60%), was 1–5 for each hormone level (score range, 6–30).ResultsACTH, GH, TSH, PRL, FSH, and LH levels in 38 patients with pituitary null cell macroadenoma were not statistically different from the 28 patients with pituitary null cell giant adenoma; the general pituitary hormone score in the former group was significantly increased compared with the latter group (P<0.05). ACTH, GH, TSH, PRL, FSH, and LH levels in the 57 patients with subtotal tumor resection were not significantly different from the nine patients with partial tumor resection; the general pituitary hormone score in the former group was significantly reduced compared with the latter group (P<0.05).ConclusionsA general pituitary hormone score was developed that might be relevant to the evaluation of pituitary function following surgical resection of pituitary null cell macroadenoma and giant adenoma.

Efficacy of laminoplasty and bone graft in the treatment of multilevel intraspinal tumor

Objective To investigate the clinical effect of laminoplasty technique with bone grafting in the treatment of multiple segmental intraspinal tumors. Methods Retrospectively review the clinical data of 35 patients suffering multiple segmental intraspinal tumors, who underwent the laminoplasty with bone grafting for intraspinal tumor resection in Department of Neurosurgery, Clinical Medical College of Yangzhou University, from June 2013 to May 2016. Observation indicators: (1) surgical and postoperative recovery situations, including tumor resection, number of lamina reset, tumor pathological results, clinical efficacy evaluation, complications, etc; (2) follow-up situations, including spinal stability evaluation, rate of bone graft fusion, tumor recurrence. Follow-up using outpatient examination and telephone interview was performed by the end of December 2017. Results (1) Surgical and postoperative recovery situations: of the 35 patients, 31 achieved total tumor resection, 3 subtotal tumor resection and 1 partial tumor resection. There were no spinal dura mater injuries when laminectomy was performed, and 84 laminae were repositioned and implanted. The neurological function of the spinal cord was evaluated based on McCormick scoring system: 32 cases were improved and 3 cases were controlled. Incision healing was bad in 2 patients, and cerebrospinal fluid leakage occurred in 1 case, which all recovered after treatment. (2) Follow-up situations: all the 35 patients were followed up for 8 to 36 months, with a median time of 14.2 months. Imaging examination more than 6 months after operation: no spinal instability was found in standing spine X-ray examination; CT scan showed a total of 142 sides in the 84 vertebral plates (168 sides) had achieved bone fusion, the fusion rate was 84.5%, and no titanium nail removal and connecting piece shift was found; MRI showed no tumor recurrence in all patients. Conclusion Use of the laminoplasty technique with bone grafting in the treatment of multiple segmental intraspinal tumors can achieve good clinical efficacy, high fusion rate for resected laminae and satisfactory spinal stability. Key words: Spinal canal; Perioperative period; Laminoplasty; Bone grafting; Intraspinal tumor

Advances in the Surgical Treatment of Neuroblastoma.

Objective:This study was to review the efficacy of surgical resections in different clinical situations for a better understanding of the meaning of surgery in the treatment of neuroblastoma (NB).Data Sources:The online database ScienceDirect (2016–2018) was utilized. The search was conducted using the keywords “neuroblastoma,” “neuroblastoma resection,” “neuroblastoma surgery,” and “high-risk neuroblastoma.”Study Selection:We retrospectively analyzed of patients who underwent surgical resections in different clinical situations. The article included findings from selected relevant randomized controlled trials, systematic reviews, and meta-analyses or good-quality observational studies. s only, letters, and editorial notes were excluded. Full-text articles and abstracts were extracted and reviewed to identify key articles discussing surgery management of NB, which were then selected for critical analysis.Results:A total of 7800 English language articles were found containing references to NB (2016–2018). The 163 articles were searched which were related to the surgical treatment of NB (2016–2018). Through the analysis of these important articles, we found that the treatments of NB at low- and intermediate-risk groups were basically the same. High-risk patients remained controversial.Conclusions:NB prognosis varies tremendously based on the stage and biologic features of the tumor. After reviewing the relevant literature, patients with low-risk disease are often managed with surgical resection or observation alone with tumors likely to spontaneously regress that are not causing symptoms. Intermediate patients are treated with chemotherapy with the number of cycles depending on their response as well as surgical resection of the primary tumor. High-risk patients remain controversial. Multidisciplinary intensive treatment is essential, especially for patients who received subtotal tumor resection. Minimally invasive surgery for the treatment of NBs without image-defined risk factors in low- to high-risk patients is safe and feasible and does not compromise the treatment outcome. We conclude that ≥90% resection of the primary tumor is both feasible and safe in most patients with high-risk NB. New targeted therapies are crucial to improve survival.

Multidisciplinary Treatment of Pediatric Low-Grade Glioma: Experience of Children Cancer Hospital of Egypt; 2007-2012

Abstract Background: Pediatric gliomas comprise a clinically, histologically, and molecularly heterogeneous group of central nervous system tumors. The survival of children with gliomas influenced by histologic subtype, age, and extent of resection. Tumor grade emerged as the most determinant of survival except in the young age groups. The aim of this study was to evaluate the role of multidisciplinary therapeutic approach including surgery and chemotherapy, and their impact on the outcome in pediatric patients with low-grade glioma (LGG). Procedure: Study patients were prospectively enrolled onto the study. All patients were below 18-year-old, diagnosed as LGG between July 2007 and June 2012. Upfront surgical resection was attempted in all tumors other than optic pathway sites. Systemic chemotherapy was given according to CCG-A9952 protocol. Results: Total/near-total resection in 105/227 (46.3%) without adjuvant treatment, while 49/227 patients (21.5%) underwent subtotal tumor resection followed by chemotherapy for big residual (n = 26). Follow-up only was indicated for asymptomatic/small residual (n = 23). The radiological diagnosis was set in 18/227 (7.9%) patients; 13/18 had optic pathway glioma. The 3-year overall survival (OS) was 87.3% versus 65.5% event free survival (EFS) for the whole study patients with a follow-up period of 1–5 years. The OS and EFS for patients who did surgery with no adjuvant treatment (n = 128) were, respectively, 95.2% and 77.3% versus 87.4% and 65.1% for adjuvant chemotherapy group (n = 99); (P = 0.015 and P = 0.016 for OS and EFS, respectively). Conclusion: Pediatric LGGs comprise a wide spectrum of pathological and anatomical entities that carry a high rate of prolonged survival among children and adolescents. Surgical resection is the mainstay of treatment in most of tumors. Combined chemotherapy can be an acceptable alternative when surgery is not safely feasible.

Papillary Tumor of the Pineal Region: Case Report and Literature Review

AbstractPapillary tumor of the pineal region (PTPR) is a neuroectodermal tumor thought to originate from cells of the subcommissural organ. Its oncologic properties are still under investigation, as well as the most suitable therapeutic measures for this type of neoplasm. We report the case of a 36-year-old woman with a 1-year history of headache and intermittent diplopia. The magnetic resonance imaging (MRI) scan showed a heterogeneously enhancing mass in the pineal region that caused an acute hydrocephalus, and an emergency shunt derivation was necessary. One week later, the patient was submitted to subtotal tumor resection, and remained asymptomatic in the post-operative period. In the follow-up, the patient remained asymptomatic; in the imaging control 3.5 years after the surgical resection, local recurrence was identified, and the patient was submitted to a local radiation protocol. Our literature review showed an early clinical onset due to intracranial hypertension signs. Definitive clinical onset might be reached only through a histopathological examination. Gross total resection followed by radiotherapy is the current standard of care. Local recurrence is often observed, with rare dissemination to the cerebral spinal fluid. The natural history of the PTPR remains unknown, as well as the best treatment strategy. Large case series with longer follow-ups are necessary for further conclusions.

Clinical outcomes of pediatric patients with newly diagnosed rhabdomyosarcoma treated by two consecutive protocols - A single institution report in Taiwan.

Taiwan Pediatric Oncology Group (TPOG) initiated two consecutive protocols for treating pediatric patients with rhabdomyosarcoma since 1995. However, the results have not been analyzed and reported yet. The aim of this study is to investigate the treatment results of these two protocols in our hospital and to assess whether the results are comparable to other large-scaled studies. Treatment of pediatric patients with rhabdomyosarcoma according to TPOG protocols at National Taiwan University Hospital began in 1995. Between 1995 and 2006, patients were treated by TPOG RMS 95 protocol, which was based on IRS-III/IV. After 2007, patients were treated by TPOG RMS 2007 protocol which was adapted from IRS-V study. The clinical data of patients were obtained retrospectively by reviewing medical records. The date of the latest follow-up was December 31, 2016. Thirty-seven patients were enrolled in this study. The 5-year overall survival (OS) and event-free survival rates of them were 54.7±8.8% and 48.5±8.6%, respectively. The 5-year OS rates for patients treated by TPOG RMS 95 and TPOG RMS 2007 protocols were 55.0±11.1% and 55.9±14.0%, respectively. Age at diagnosis of less than ten years old and receiving operation with gross total or subtotal tumor resection were identified as independent prognostic factors that predicted better outcomes in the multivariate analysis. The clinical outcomes of pediatric patients with rhabdomyosarcoma in Taiwan improved dramatically after incorporating two consecutive protocols from TPOG. In addition, the treatment results of these two protocols were comparable to large-scale studies of other countries.

Tissue Compatibility of SN-38-Loaded Anticancer Nanofiber Matrices.

Delivery of chemotherapy in the surgical bed has shown preclinical activity to control cancer progression upon subtotal resection of pediatric solid tumors, but whether this new treatment is safe for tumor-adjacent healthy tissues remains unknown. Here, Wistar rats are used to study the anatomic and functional impact of electrospun nanofiber matrices eluting SN-38-a potent chemotherapeutic agent-on several body sites where pediatric tumors such as neuroblastoma, Ewing sarcoma, and rhabdomyosarcoma arise. Blank and SN-38-loaded matrices embracing the femoral neurovascular bundle or in direct contact with abdominal viscera (liver, kidney, urinary bladder, intestine, and uterus) are placed. Foreign body tissue reaction to the implants is observed though no histologic damage in any tissue/organ. Skin healing is normal. Tissue reaction is similar for SN-38-loaded and blank matrices, with the exception of the hepatic capsule that is thicker for the former although within the limits consistent with mild foreign body reaction. Tissue and organ function is completely conserved after local treatments, as assessed by the rotarod test (forelimb function), hematologic tests (liver and renal function), and control of clinical signs. Overall, these findings support the clinical translation of SN-38-loaded nanofiber matrices to improve local control strategies of surgically resected tumors.

Application of intraoperative MRI combined with neuronavigation in microsurgical resection for insular glioma

To evaluate the value of intraoperative magnetic resonance imaging (iMRI) combined with neuronavigation for the resection of insular gliomas. Methods: From August 2014 to October 2017 in the First Hospital Affiliated to Sun Yat-sen University, clinical data of 41 patients with insular glioma, who underwent the surgery assisted with 3.0T iMRI and neuronavigation, were analyzed retrospectively, and the resection extent, complications and prognosis were evaluated. Results: Subtotal tumor resection was achieved in 21 patients and partial resection was done in 20 after iMRI scanning. After further resection, total tumor resection was achieved in 16 patients, subtotal resection in 18 and partial resection in 7. There was a statistical significant difference in tumor resection between pre-iMRI and post-iMRI according to the Fisher test (P<0.05). In the follow-up from 3 months to 3 years, the symptoms of the 41 patients had improved. Conclusion: iMRI corrected the shift of brain. Neuronavigation can accurately and timely assess the degree of resecting tumor. The combination of neuronavigation with surgery can maximally and safely resect insular glioma.

The incidence of brainstem primitive neuroectodermal tumors of childhood based on SEER data.

Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children. Data were collected using the Surveillance Epidemiology and End Results cancer registry. From 1973 to 2013, we identified 83 pediatric patients (aged 0-21years). Patients were divided into two age groups (0-3years and 4-21years). Median overall survival was 53months. Patients in the older age group had a significant survival advantage (P < 0.001), as did those who received three modalities of therapy (surgery, chemotherapy, and radiation therapy) (P < 0.001) and patients with gross or subtotal tumor resection (P < 0.001). This study presents the first estimate of incidence and the largest cohort of pediatric BS-PNETs to date. A high index of suspicion of BS-PNET in similar cases is crucial for diagnosis, treatment, and outcome.

Olfactory groove meningiomas: An analysis based on surgical experience with 129 cases.

The surgical strategy and outcome for 129 patients operated for an olfactory groove meningioma is retrospectively analyzed. One hundred and twenty nine patients with an olfactory groove meningioma operated between the years 1987 and 2016 were analyzed on the basis of clinical and radiological factors that appeared to affect the conduct of surgery and its outcome. Each factor was given points, and the points were added to obtain a score. On the basis of the score, the tumors were divided into three grades. The grades determined the difficulties that could be anticipated during surgery. In the initial part of the series, a bifrontal craniotomy was done to resect the tumor in all patients. In the later half of the series, majority of the patients were operated by the use of a unifrontal craniotomy. The aim of surgery was to resect the tumor completely. Total tumor resection was achieved in 86 patients, and subtotal tumor resection with less than 10% tumor left behind was achieved in 43 patients. Seven patients died in the immediate postoperative period. All patients where a subtotal tumor resection was done and where the patient died after surgery had a higher grade tumor. The average follow-up period was 82 months. There was non-symptomatic recurrence in 11 patients. The location and size of the tumor, extent and duration of visual symptoms, extent of tumor adjoining cerebral edema, encasement of anterior cerebral artery complex and extension in the extracranial compartment were more important factors that affected the outcome of surgery.

Endoscopic Endonasal Surgery for Subdiaphragmatic Type Craniopharyngiomas.

Subdiaphragmatic type craniopharyngiomas are tumors that originate within the sella. They are divided into two types; those localized within an enlarged sella (intrasellar type) and those accompanying a suprasellar extension (suprasellar extended type). The clinicopathological features and the recent outcomes of endoscopic endonasal surgery were retrospectively reviewed in 32 patients, with 11 surgeries for recurrence. These tumors showed a preponderance in young patients (19 patients were younger than 18-year-old) and suprasellar extended type (25 cases), were mostly composed of a large cyst (96.9%) and were frequently adamantinomatous type (68.8%). Combined transcranial-endoscopic endonasal surgery was applied in three patients with extremely large tumors and significant frontal extension. Total tumor resection and stalk preservation were achieved in 26 and 17 patients, respectively. No complications developed after surgery apart from pituitary dysfunction and visual deterioration. 5 of 6 patients with subtotal tumor resection and 6 of 7 patients with no improvement or deterioration of visual function were in the recurrent cases. Although this type is basically an extraarachnoidal tumor, the suprasellar portion of the tumor showed adherence to important tissues in some patients with recurrence. Pituitary function remained normal in only one third of patients with stalk preservation. To avoid pituitary dysfunction after surgery, sharp excision of firm adherence to the stalk should be considered in some patients.

Inflammatory myofibroblastic tumor of the orbit: A clinico-pathological study of 25 cases

BackgroundInflammatory myofibroblastic tumor (IMT) is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma, which typically occurs in children or young adults. The IMT is considered generally a benign lesion, although about 20% of cases may experience recurrence, and most rarely develop metastasis. Herein, we present the largest series of primary orbital IMT ever reported. Patients and methodsThe clinical records of 25 patients, collected between the 1995 and 2015, with biopsy-proven diagnosis of orbital IMT were retrospectively reviewed to determine demographic, clinical, radiologic and pathological features, management, and outcome. ResultsThe study included 13 females and 12 male patients, age ranged from 5 to 76 years. Disease onset was in all cases unilateral (25/25), with posterior location (10/25) or extending anterior to posterior (7/25). The most common signs and symptoms were: proptosis (19/25), ptosis (18/25), diplopia (10/25), periocular swelling (9/25), pain (8/25), redness (7/25). All patients underwent to incisional biopsy which included total or subtotal tumor resection avoiding arming of the adjacent structure, followed by systemic steroid therapy (22/25) or radiotherapy (3/25). The disease recurred in 6 (24%) patients who responded to the subsequent therapy. No one developed metastasis or died because of the disease. ConclusionIMT is a distinct entity which may occur in the orbit primarily. It should be considered in differential diagnosis in all orbital masses, particularly with onset of acute or subcronic inflammation. Surgical biopsy associated to a partial debulcking of the tumor, avoiding to damage adjacent vital structure may contribute to improve the outcome. Steroid therapy, seems to be the suitable as first line medical therapy, although, as reported in literature, not all cases respond to this treatment regimen. Radiotherapy, may be considered as an alternative therapy. Recurrences occurred in 24% of patients and may be treated with additional surgical resection and a new course of steroid or radiotherapy. No specific pathological features which may correlate with the prognosis have been found in this series.

Collision tumor of the sellar region

Objective To summarize clinical and pathological features, diagnosis and differential diagnosis, as well as treatment and prognosis through analyzing one case of collision tumor in the sellar region (pituitary adenoma combined with malignant triton tumor). Methods and Results A 59-year-old male was admitted to hospital with decreased vision, vision field defect and dizziness. Head MRI demonstrated space-occupying lesion in the sellar region. Therefore, the patient underwent a transsphenoidal surgery with subtotal tumor resection. Three months later, the patient was admitted to hospital again with vision loss and headache. Head CT demonstrated space-occupying lesions in sellar/suprasellar region, therefore, the patient underwent saddle tumor resection. Histological findings revealed that the tumor was composed of two different types of tumors, one was pituitary adenoma with epithelial cells arranged in nest-like and trabecular pattern, and the other was diffused spindle cell tumor. The two tumors were distributed separately or mixed togenther. The morphology of spindle cell tumor was bland with scarce mitosis in the first surgery, whereas additional features including increased cell density, dark nuclei, frequent mitosis and scattering distributed rhabdomyoblasts were found in the second surgery. Immunohistochemically, the pituitary adenoma (epithelial cell region) cells were positive for cytokeratin 8 (CK8), chromogranin A (CgA), synaptophysin (Syn) and thyroid stimulating hormone (TSH), while the malignant triton tumor cells (spindle cell region) were positive for CD56, CD57, calretinin (CR) and focally positive for S-100 protein (S-100), desmin (Des) and myogenin. Ultrastructural findings revealed abundant rough endoplasmic reticules and secretary granules in the cytoplasm of pituitary adenoma cells, and discontinuous basal lamina located outside the spindle cell membrane. The final pathological diagnosis was sellar collision tumor (non-functional TSH adenoma combined with malignant triton tumor). The patient died 6 months after the second surgery. Conclusions Collision tumor of the sellar region with pituitary adenoma and malignant triton tumor is a rare tumor which can hardly be diagnosed by clinical and neuroimaging examination. The diagnosis relies on morphological characteristics, immunophenotype and ultrastructural features. The prognosis of collision tumor with malignant triton tumor component is poor. DOI: 10.3969/j.issn.1672-6731.2017.11.009