scholarly journals Transsphenoidal Surgery of Invasive Corticotroph Adenomas: Results in A Series of 86 Consecutive Patients

2021 ◽  
Author(s):  
Congxin Dai ◽  
Ming Feng ◽  
Lin Lu ◽  
Bowen Sun ◽  
Yanghua Fan ◽  
...  
Keyword(s):  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Partial Resection ◽  
Gross Total Resection ◽  
Subtotal Resection ◽  
Total Resection ◽  
Persistent Disease ◽  
Biochemical Remission ◽  
Corticotroph Adenomas

Abstract Objective: Surgery is first-line treatment for corticotroph adenomas. Although most of corticotroph adenomas are noninvasive microadenomas that show expansive growth to surrounding tissues, a small subset of them is locally invasive and difficult to manage. The aim of this study was to evaluate surgical outcome of invasive corticotroph adenomas from a single-center. Patients and Methods: The clinical features and outcomes of CD patients who underwent transsphenoidal surgery (TSS) between January 2000 and September 2019 at Peking Union Medical College Hospital were collected from medical records. The clinical, endocrinological, radiological, histopathological, surgical outcomes and a minimum 12-month follow-up of 86 consecutive CD patients with invasive corticotroph adenomas were retrospectively reviewed. Results: Eighty-six patients with invasive corticotroph adenomas were included in the study. The average age at TSS was 37.7 years (range, 12 to 67 years), with a female-to-male ratio of 3.1:1 (65/21). The median duration of symptoms was 52.6 months (range, 1.0 to 264 months). The average of maximum diameter of tumor was 17.6 mm (range, 4.5–70 mm). All 86 patients with invasive corticotroph adenomas were performed TSS by microscopic or endoscopic approach. Gross-total resection was achieved in 63 patients (73.3%), subtotal resection in 18 (20.9%), and partial resection in 5 (5.8%). After surgery, the overall postoperative immediate remission rate was 48.8% (42/86), 51.2 % (44/86) of patients maintained persistent hypercortisolism. In 42 patients with initial remission, 16.7 % (7/42) of them experienced a recurrence. In these patients with persistent disease and recurrent CD, data about further treatment was available for 30 patients. The radiotherapy was used for 15 patients, and 4 (26.7%) of them achieved biochemical remission. Repeat TSS was performed in 5 patients, and none achieved remission. Medication was administrated in 4 patients, and one of them obtained disease control. Adrenalectomy was performed in 6 patients, and 5 (83.3 %) achieved biochemical remission. At last follow-up, (33.3%) 10 of 30 patients were in remission, and 20 patients still had persistent disease. The remission rate in patients with invasive corticotroph adenomas who underwent gross-total resection and first TSS were significantly higher than that in patients undergoing subtotal resection, partial resection, and a second TSS (all P＜0.05). However, there was no significant difference in the remission rate between patient with different tumor size, Knosp Grade and surgical approaches (P＞0.05).Conclusion: The management of invasive corticotroph adenomas remain a therapeutic challenge due to incomplete resection of invasive and/or a large adenoma. With application of multiple techniques assistance, approximately half of the patients could achieve gross-total resection and biochemical remission via TSS by experienced neurosurgeons. The extent of tumor resection and number of operations were associated with surgical remission rate in invasive corticotroph adenomas. If the remission was not achieved by surgery, other treatments including radiotherapy, medical therapy, and even bilateral adrenalectomy are required.

Surgical Technique and Outcomes in the Treatment of Spinal Cord Ependymomas: Part II: Myxopapillary Ependymoma

2011 ◽  
Vol 68 (suppl_1) ◽  
pp. ons90-ons94 ◽  
Author(s):  
Elisa J Kucia ◽  
Peter H Maughan ◽  
Udaya K Kakarla ◽  
Nicholas C Bambakidis ◽  
Robert F Spetzler
Keyword(s):  
Spinal Cord ◽  
Radiation Therapy ◽  
Gross Total Resection ◽  
Subtotal Resection ◽  
Myxopapillary Ependymoma ◽  
Postoperative Radiation ◽  
Total Resection ◽  
Postoperative Radiation Therapy ◽  
Duration Of Symptoms

Abstract BACKGROUND: Myxopapillary ependymomas usually occur in the filum terminale of the spinal cord. OBJECTIVE: This report summarizes our experience treating myxopapillary ependymomas. METHODS: The records of 34 patients (14 men, 20 women; mean age 45.5 years; age range, 14-88 years) who underwent resection of a myxopapillary ependymoma between 1983 and 2006 were reviewed for age, sex, tumor location, symptoms at diagnosis, duration of symptoms, treatment before presentation, extent of surgical resection, adjuvant therapy, length of follow-up, evidence of recurrence, and complications. Neurological examinations performed at presentation, immediately after surgery, and last follow-up were graded according to the McCormick grading scale. RESULTS: The average duration of symptoms before diagnosis was 22.2 months. The most common symptom was pain followed by weakness, bowel/bladder symptoms, and numbness. The rate of gross total resection was 80%. All patients with a subtotal resection (20%) underwent postoperative radiation therapy. Presentation and outcomes of patients who underwent subtotal resection followed by radiation therapy were compared with those who underwent gross total resection. There was no significant difference in neurological grade between the groups at presentation or final follow-up. The overall recurrence rate was 10% (3/34 patients). CONCLUSION: The goal of surgical treatment of myxopapillary ependymomas is resection to the greatest extent possible with preservation of function. In cases of subtotal resection, postoperative radiation therapy may improve outcome. If neurological function is maintained at treatment, these indolent lesions allow years of good function.

Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation

2013 ◽  
Vol 11 (5) ◽  
pp. 518-525 ◽  
Author(s):  
Yui Mano ◽  
Toshihiro Kumabe ◽  
Ichiyo Shibahara ◽  
Ryuta Saito ◽  
Yukihiko Sonoda ◽  
...  
Keyword(s):  
Malignant Transformation ◽  
Gross Total Resection ◽  
Subtotal Resection ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Total Resection ◽  
Who Grade ◽  
Adjuvant Radiochemotherapy ◽  
First Case ◽  
Complex Forms

Dysembryoplastic neuroepithelial tumors (DNETs) have conventionally been regarded as benign and stable tumors and considered curable with surgery without adjunctive therapy. Recently, recurrent DNETs with or without malignant transformation have been described. The authors report 2 unusual cases of DNET: 1) an enlarging lesion that developed an enhancing component over the natural course of 4 years, and 2) a recurrent DNET that developed an enhancing component 10–11 years after gross-total resection. The patient in the first case was treated with subtotal resection and adjuvant radiochemotherapy; histological examination of the tumor led to the diagnosis of DNET, WHO Grade I, for the nonenhancing component and anaplastic oligodendroglioma, WHO Grade III, for the enhancing component. The patient in the second case was treated with repeat gross-total resection; the original tumor had been histologically diagnosed as DNET, and the nonenhancing and enhancing components of the recurrent tumor were diagnosed as simple and complex forms of DNET, respectively. These and previous reports suggest an aggressive subtype of DNETs. If follow-up MRI reveals progressive behavior, resection should be performed without delay. Additional radiochemotherapy is needed if the histological diagnosis demonstrates malignant transformation.

Patient Outcome at Long-term Follow-up after Aggressive Microsurgical Resection of Cranial Base Chondrosarcomas

Neurosurgery ◽  
2006 ◽  
Vol 58 (6) ◽  
pp. 1090-1098 ◽  
Author(s):  
Fotios Tzortzidis ◽  
Foad Elahi ◽  
Donald C. Wright ◽  
Nancy Temkin ◽  
Sabareesh K. Natarajan ◽  
...  
Keyword(s):  
Cranial Base ◽  
Gross Total Resection ◽  
Cerebrospinal Fluid Leakage ◽  
Subtotal Resection ◽  
Incomplete Resection ◽  
Total Resection ◽  
Long Term Follow Up ◽  
Microsurgical Resection

Abstract OBJECTIVE: To evaluate patient clinical outcome and survival at long-term follow-up after aggressive microsurgical resection of chondrosarcomas of the cranial base. METHODS: Over a 20-year period, 47 patients underwent 72 operative procedures for resection of cranial base chondrosarcomas. Thirty-three patients were previously untreated, whereas 14 patients previously had undergone surgery or radiation. Twenty-three patients had a single operation and 24 underwent staged (more than one) operations because of extensive disease. Patients who underwent subtotal resection also underwent radiotherapy or radiosurgery. Patients were evaluated at follow-up clinically and by imaging studies. RESULTS: Gross total resection was accomplished in 29 (61.7%) patients, and subtotal resection was accomplished in 18 patients (38.3%). The resection was better in patients who underwent a primary operation (gross total resection, 68.8 versus 46.7%) rather than a reoperation. Patients who underwent incomplete resection underwent postoperative radiotherapy, which included proton beam radiotherapy (15.6%), radiosurgery (68%), and fractionated radiation (15.6%). There were no operative deaths. Postoperative complications (cerebrospinal fluid leakage, quadriparesis, infections, cranial nerve palsies, etc.) were observed in 10 patients (18%). The follow-up ranged from 2 to 255 months, with an average of 86 months. At the conclusion of study, 36 (76.6%) patients were alive, and 21 (44.7%) patients were alive without disease. Recurrence-free survival was 32% at 10 years in all patients, 42.3% in primary patients and 13.8% in those who underwent reoperation. The Karnofsky performance score was 82.4 ± 9.8 before surgery, 85 ± 12.5 at 1 year after surgery, and 85.3 ± 5.8 at the latest follow-up. Two patients died as a result of radiotherapy complications (malignancy, radiation necrosis). CONCLUSION: Cranial base chondrosarcomas can be managed well by complete surgical resection or by a combination of surgery and radiotherapy. The study cannot comment about the efficacy of radiotherapy. Approximately half of the patients survived without recurrence at long-term follow-up (>132 mo). The functional status of the surviving patients was excellent at follow-up.

Long-term outcomes of transsphenoidal surgery for management of growth hormone–secreting adenomas: single-center results

2020 ◽  
Vol 133 (5) ◽  
pp. 1360-1370 ◽  
Author(s):  
Mohammed J. Asha ◽  
Hirokazu Takami ◽  
Carlos Velasquez ◽  
Selfy Oswari ◽  
Joao Paulo Almeida ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Recurrence Rate ◽  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Repeat Surgery ◽  
Significant Difference ◽  
Remission Rates ◽  
Biochemical Remission

OBJECTIVETranssphenoidal surgery is advocated as the first-line management of growth hormone (GH)–secreting adenomas. Although disease control is defined by strict criteria for biochemical remission, the length of follow-up needed is not well defined in literature. In this report, the authors present their long-term remission rate and identify various predictive factors that might influence the clinical outcome.METHODSThe authors conducted a single-institute retrospective analysis of all transsphenoidal procedures for GH-secreting adenomas performed from January 2000 to June 2016. The primary outcome was defined as biochemical remission according to the 2010 consensus criteria and measured at the 1-year postoperative mark as well as on the last recorded follow-up appointment.Secondary variables included recurrence rate, patterns of clinical presentation, and outcome of adjuvant therapy (including repeat surgery). Subgroup analysis was performed for patients who had biochemical or radiological “discordance”—patients who achieved biochemical remission but with incongruent insulin-like growth factor 1 (IGF-1)/GH or residual tumor on MRI. Recurrence-free survival analysis was conducted for patients who achieved remission at 1 year after surgery.RESULTSEighty-one patients (45 female and 36 male) with confirmed acromegaly treated with transsphenoidal surgery were included. In 62 cases the patients were treated with a pure endoscopic approach and in 19 cases an endoscopically assisted microscopic approach was used.Primary biochemical remission after surgery was achieved in 59 cases (73%) at 1 year after surgery. However, only 41 patients (51%) remained in primary surgical remission (without any adjuvant treatment) at their last follow-up appointment, indicating a recurrence rate of 31% (18 of 59 patients) over the duration of follow-up (mean 100 ± 61 months). Long-term remission rates for pure endoscopic and endoscopically assisted cases were not significantly different (48% vs 52%, p = 0.6). Similarly, no significant difference in long-term remission was detected between primary surgery and repeat surgery (54% vs 33%, p = 0.22).Long-term remission was significantly influenced by extent of resection, cavernous sinus invasion (radiologically as well as surgically reported), and preoperative and early postoperative GH and IGF-1 levels (within 24–48 hours after surgery) as well as by clinical grade, with lower remission rates in patients with dysmorphic features and/or medical comorbidities (grade 2–3) compared to minimally symptomatic or silent cases (grade 1).CONCLUSIONSThe long-term surgical remission rate appears to be significantly less than “early” remission rates and is highly dependent on the extent of tumor resection. The authors advocate a long-term follow-up regimen and propose a clinical grading system that may aid in predicting long-term outcome in addition to the previously reported anatomical factors. The role of repeat surgery is highlighted.

Long-term endocrinological follow-up evaluation in 115 patients who underwent transsphenoidal surgery for acromegaly

1998 ◽  
Vol 89 (3) ◽  
pp. 353-358 ◽  
Author(s):  
Pamela U. Freda ◽  
Sharon L. Wardlaw ◽  
Kalmon D. Post
Keyword(s):  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Postoperative Radiotherapy ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Content Type ◽  
Biochemical Remission ◽  
Fine Print

Object. The results of surgical therapy for acromegaly were assessed using carefully conducted endocrinological testing in 115 patients who underwent transsphenoidal surgery from 1981 to 1995. Methods. Ninety-nine of the 115 patients could be contacted for follow-up review; in 57 of the patients an endocrinological assessment was performed by the authors. Biochemical cure was strictly defined as a normalization of the insulin-like growth factor(IGF)—I level (obtained in 100 patients) and/or a basal or glucose-suppressed growth hormone (GH) level of 2 ng/ml or less. The mean length of follow up from transsphenoidal surgery to laboratory testing was 5.4 years with a maximum of 15.7 years. After transsphenoidal surgery alone, 61% of the patients achieved a biochemical remission; the remission rate was 88% for patients with microadenomas and 53% for those with macroadenomas. Tumor size and preoperative GH level tended to correlate negatively with outcome of surgery. Early postoperative GH level tended to correlate with long-term outcome; in cases in which the early postoperative GH level was lower than 3 ng/ml, the chance of long-term remission was 89%. Thirty-two patients received postoperative radiotherapy: in 10 (31%) of these patients the disease is currently in remission after surgery and radiotherapy only and in three others the disease is in remission with the addition of medical therapy. The overall complication rate was 6.9% with no cerebrospinal fluid leaks, meningitis, permanent diabetes insipidus, or new hypopituitarism. The overall recurrence rate was low at 5.4%. Conclusions. This series shows, based on IFGF—I measurements and strict GH supression criteria to define remission, that transsphenoidal surgery provides an excellent chance for long-term cure in patients with microadenomas. Surgery alone is successful in most patients with noninvasive macroadenomas; however, most patients with invasive macroadenomas will require adjunctive therapy. Recurrences are uncommon when biochemical remission is clearly documented postoperatively.

The Long-term Outcome After Resection of Intraspinal Nerve Sheath Tumors

Neurosurgery ◽  
2015 ◽  
Vol 77 (4) ◽  
pp. 585-593 ◽  
Author(s):  
Charlotte Marie Halvorsen ◽  
Pål Rønning ◽  
John Hald ◽  
Tom Børge Johannesen ◽  
Frode Kolstad ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Progression Free Survival ◽  
Gross Total Resection ◽  
Subtotal Resection ◽  
Total Resection ◽  
Free Survival ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath

Abstract BACKGROUND: The existing literature on recurrence rates and long-term clinical outcome after resection of intraspinal nerve sheath tumors is limited. OBJECTIVE: To evaluate progression-free survival, overall survival, and long-term clinical outcome in a consecutive series of 131 patients with symptomatic intraspinal nerve sheath tumors. METHODS: Medical charts were retrospectively reviewed. Surviving patients voluntarily participated in a clinical history and physical examination that focused on neurological function and current tumor status. RESULTS: Follow-up data are 100% complete; median follow-up time was 6.1 years. All patients (100%) had surgery as the first line of treatment; gross total resection was performed in 112 patients (85.5%) and subtotal resection in 19 patients (14.5%). Five-year progression-free survival was 89%. The following risk factors for recurrence were identified: neurofibroma, malignant peripheral nerve sheath tumor, subtotal resection, neurofibromatoses/schwannomatosis, and advancing age at diagnosis. More than 95% of patients had neurological function compatible with an independent life at follow-up. The rate of tumor recurrence in nonneurofibromatosis patients undergoing total resection of a single schwannoma was 3% (3/93), in comparison with a recurrence rate of 32% (12/38) in the remaining patients. CONCLUSION: Gross total resection is the gold standard treatment for patients with intraspinal nerve sheath tumors. In a time of limited health care resources, we recommend that follow-up be focused on the subgroup of patients with a high risk of recurrence. The benefit of long-term, yearly magnetic resonance imaging follow-up with respect to recurrence in nonneurofibromatosis patients undergoing gross total resection of a single schwannoma is, in our opinion, questionable.

scholarly journals Outcomes After Surgery and Radiotherapy for Spinal Myxopapillary Ependymoma

Neurosurgery ◽  
2014 ◽  
Vol 75 (3) ◽  
pp. 205-214 ◽  
Author(s):  
Chiaojung Jillian Tsai ◽  
Yucai Wang ◽  
Pamela K. Allen ◽  
Anita Mahajan ◽  
Ian E. McCutcheon ◽  
...  
Keyword(s):  
Cox Regression ◽  
Postoperative Radiotherapy ◽  
Gross Total Resection ◽  
Entire Group ◽  
Subtotal Resection ◽  
Myxopapillary Ependymoma ◽  
Adjuvant Radiation ◽  
Total Resection ◽  
Cox Regression Analysis

Abstract BACKGROUND: The role of radiotherapy after surgery for myxopapillary ependymoma (MPE) is unclear. OBJECTIVE: To review long-term outcomes after surgery, with or without radiation, for spinal MPE. METHODS: Fifty-one patients with spinal MPE treated from 1968 to 2007 were included. Associations between clinical variables and overall survival (OS), progression-free survival (PFS), and local control (LC) were tested with Cox regression analysis. RESULTS: The median age at diagnosis was 35 years (range, 8-63 years). Twenty patients (39%) had surgery alone, 30 (59%) had surgery plus radiotherapy (RT), and 1 (2%) had RT only. At a median follow-up of 11 years (range, 0.2-37 years), 10-year OS, PFS, and LC for the entire group were 93%, 63%, and 67%, respectively. Nineteen patients (37%) had disease recurrence, and the recurrence was mostly local (79%). Twenty-eight of 50 patients who had surgery (56%) had gross total resection; 10-year LC was 56% after surgery vs 92% after surgery and RT (log-rank P = .14); the median time of LC was 10.5 years for patients receiving gross total resection plus RT, and 4.75 years for gross total resection only (P = .03). Among 16 patients with subtotal resection and follow-up data, 10-year LC was 0% after surgery vs 65% for surgery plus RT (log-rank P = .008). On multivariate analyses adjusting for resection type, age older that 35 years at diagnosis and receipt of adjuvant radiation were associated with improved PFS (hazard ratio [HR]: 0.14, P = .003 and HR: 0.45, P = .009) and LC (HR: 0.22, P = .02 and HR: 0.45, P = .009). CONCLUSION: Postoperative radiotherapy after resection of MPE was associated with improved PFS and LC.

Surgical Technique and Outcomes in the Treatment of Spinal Cord Ependymomas, Part 1: Intramedullary Ependymomas

2011 ◽  
Vol 68 (suppl_1) ◽  
pp. ons57-ons63 ◽  
Author(s):  
Elisa J Kucia ◽  
Nicholas C Bambakidis ◽  
Steve W Chang ◽  
Robert F Spetzler
Keyword(s):  
Spinal Cord ◽  
Radiation Therapy ◽  
Surgical Resection ◽  
Complication Rate ◽  
Gross Total Resection ◽  
Subtotal Resection ◽  
Total Resection ◽  
Tertiary Center ◽  
The Mean

Abstract BACKGROUND: Intramedullary spinal ependymomas are rare tumors. OBJECTIVE: To provide a large retrospective review in the modern neuroimaging era from a tertiary center where aggressive surgical resection is favored. METHODS: Charts of intramedullary spinal ependymomas treated between 1983 and 2006 were reviewed. RESULTS: Sixty-seven cases were reviewed. The mean age was 45.6 years (range, 11-78 years) with a male-to-female ratio of 2:1. The most common location was the cervical spine, followed by the thoracic and lumbar spine. The average duration of symptoms was 33 months, with the most common symptom being pain and/or dysesthesias, followed by weakness, numbness, and urinary or sexual symptoms. Gross total resection was achieved in 55 patients and a subtotal resection was performed in 12 patients; 9 patients were treated with adjuvant radiation therapy. Mean follow-up was 32 months. The mean McCormick neurological grade at last follow-up was 2.0. The preoperative outcome correlated significantly with postoperative outcome (P < .001). A significant number of patients who initially worsened improved at their 3-month follow-up examination. Outcomes were significantly worse in patients undergoing subtotal resection with or without radiation therapy (P < .05). There were 3 recurrences. The overall complication rate was 34%. The primary complications were wound infections or cerebrospinal fluid leaks. CONCLUSION: Spinal cord ependymomas are difficult lesions to treat. Aggressive surgical resection is associated with a high overall complication rate. However, when gross total resection can be achieved, overall outcomes are excellent and the recurrence rate is low.

Long-term tumor control after endoscopic endonasal resection of craniopharyngiomas: comparison of gross-total resection versus subtotal resection with radiation therapy

2021 ◽  
pp. 1-9
Author(s):  
Saniya S. Godil ◽  
Umberto Tosi ◽  
Mina Gerges ◽  
Andrew L. A. Garton ◽  
Georgiana A. Dobri ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Diabetes Insipidus ◽  
Endocrine Function ◽  
Gross Total Resection ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Total Resection ◽  
Endoscopic Endonasal

OBJECTIVE Surgical management of craniopharyngiomas (CPAs) is challenging. Controversy exists regarding the optimal goals of surgery. The purpose of this study was to compare the long-term outcomes of patients who underwent gross-total resection with the outcomes of those who underwent subtotal resection of their CPA via an endoscopic endonasal approach. METHODS From a prospectively maintained database of all endoscopic endonasal approaches performed at Weill Cornell Medicine, only patients with CPAs with > 3 years of follow-up after surgery were included. The primary endpoint was radiographic progression. Data were collected on baseline demographics, imaging, endocrine function, visual function, and extent of resection. RESULTS A total of 44 patients with a mean follow-up of 5.7 ± 2.6 years were included. Of these patients, 14 (31.8%) had prior surgery. GTR was achieved in 77.3% (34/44) of all patients and 89.5% (34/38) of patients in whom it was the goal of surgery. Preoperative tumor volume < 10 cm3 was highly predictive of GTR (p < 0.001). Radiation therapy was administered within the first 3 months after surgery in 1 (2.9%) of 34 patients with GTR and 7 (70%) of 10 patients with STR (p < 0.001). The 5-year recurrence-free/progression-free survival rate was 75.0% after GTR and 25.0% after STR (45% in subgroup with STR plus radiotherapy; p < 0.001). The time to recurrence after GTR was 30.2 months versus 13 months after STR (5.8 months in subgroup with STR plus radiotherapy; p < 0.001). Patients with GTR had a lower rate of visual deterioration and higher rate of return to work or school compared with those with STR (p = 0.02). Patients with GTR compared to STR had a lower rate of CSF leakage (0.0% vs 30%, p = 0.001) but a higher rate of diabetes insipidus (85.3% vs 50%, p = 0.02). CONCLUSIONS GTR, which is possible to achieve in smaller tumors, resulted in improved tumor control, better visual outcome, and better functional recovery but a higher rate of diabetes insipidus compared with STR, even when the latter was supplemented with postoperative radiation therapy. GTR should be the goal of craniopharyngioma surgery, when achievable with minimal morbidity.

scholarly journals Clinical presentation and surgical outcomes of intramedullary neurenteric cysts

2015 ◽  
Vol 23 (1) ◽  
pp. 99-110 ◽  
Author(s):  
Tao Yang ◽  
Liang Wu ◽  
Jingyi Fang ◽  
Chenlong Yang ◽  
Xiaofeng Deng ◽  
...  
Keyword(s):  
Case Reports ◽  
Clinical Manifestations ◽  
Gross Total Resection ◽  
Neurological Deficits ◽  
Motor Deficit ◽  
Cervical Cord ◽  
Subtotal Resection ◽  
Total Resection ◽  
Cyst Recurrence

OBJECT Intramedullary neurenteric cysts (NECs) are exceedingly rare lesions and have been previously reported in case reports. The aim of this study was to determine the clinical manifestations, radiological features, and long-term prognosis of patients with such lesions. METHODS The authors retrospectively reviewed the records of 13 patients with an intramedullary NEC. Each patient underwent MRI, laminotomy, and microsurgery. The accurate diagnosis was based on imaging and pathology findings. Each patient's follow-up status was determined through individual office visits and a structured telephone interview. RESULTS The series included 7 male and 6 female patients. Progressive or intermittent motor deficit was the main symptom associated with or without pain or sensory disturbance. Five cysts were located in the cervical cord, 1 in the cervicothoracic cord, 3 in the thoracic cord, and 4 in the conus medullaris. Concurrent malformations included scoliosis (3 cases), fusion of rib (1 case), enlarged spinal canal (1 case), tethered spinal cord (1 case), and ectocardia (1 case). Gross-total resection of the cyst was achieved in 8 cases, and subtotal resection (STR) was achieved in 5 cases. All patients were followed up, with a mean duration of 66.5 months. Cyst recurrence was observed in 4 cases after STR. In 2 cases the patients underwent reoperation; the other 2 patients remained clinically stable and did not undergo reoperation. At the last evaluation, neurological function was improved in 11 patients and remained stable in 2 patients. CONCLUSIONS Intramedullary NECs should be considered in the differential diagnosis of a middle-aged patient with intermittent neurological symptoms and concurrent malformations. Early surgery is advocated to prevent permanent neurological deficits. When gross-total resection cannot be achieved, maximally safe removal under the protection of intraoperative neuromonitoring is advised. Because of the high risk of cyst recurrence, routine follow-up MRI is needed. If a residual cyst shows obvious regrowth and results in neurological deficits, timely reoperation with a goal of STR should be performed.

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