scholarly journals 31 Intracranial growing teratoma syndrome (IGTS): An international retrospective study

Author(s):  
George Michaiel ◽  
Douglas Strother ◽  
Nicholas Gottardo ◽  
Ute Bartels ◽  
Hallie Coltin ◽  
...  

BACKGROUND: IGTS is a rare phenomenon of paradoxical germ cell tumor (GCT) growth during or following treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of IGTS in patients in 21 North-American and Australian institutions. METHODS: Patients with IGTS diagnosed from 2000-2017 were retrospectively evaluated. RESULTS: Out of 739 GCT diagnoses, IGTS was identified in 33 patients (4.5%). IGTS occurred in 9/191 (4.7%) mixed-malignant GCTs, 4/22 (18.2%) immature teratomas (ITs), 3/472 (0.6%) germinomas/germinomas with mature teratoma, and in 17 secreting non-biopsied tumours. Median age at GCT diagnosis was 10.9 years (range 1.8-19.4). Male gender (84%) and pineal location (88%) predominated. Of 27 patients with elevated markers, median serum AFP and Beta-HCG were 70 ng/mL (range 9.2-932) and 44 IU/L (range 4.2-493), respectively. IGTS occurred at a median time of 2 months (range 0.5-32) from diagnosis, during chemotherapy in 85%, radiation in 3%, and after treatment completion in 12%. Surgical resection was attempted in all, leading to gross total resection in 76%. Most patients (79%) resumed GCT chemotherapy/radiation after surgery. At a median follow-up of 5.3 years (range 0.3-12), all but 2 patients are alive (1 succumbed to progressive disease, 1 to malignant transformation of GCT). CONCLUSION: IGTS occurred in less than 5% of patients with GCT and most commonly after initiation of chemotherapy. IGTS was more common in patients with IT-only on biopsy than with mixed-malignant GCT. Surgical resection is a principal treatment modality. Survival outcomes for patients who developed IGTS are favourable.

2010 ◽  
Vol 68 (3) ◽  
pp. 396-399 ◽  
Author(s):  
Ricardo de Amoreira Gepp ◽  
Jose Mauro Cardoso Couto ◽  
Maria Dorvalina da Silva ◽  
Régis Tavares da Silva ◽  
Eidmar Augusto Neri

Intramedullary tumors are rare. The authors reviewed 24 cases operated between 1996 and 2006. The study assessed the clinical characteristics and surgical results based upon the neurological function. METHOD: Medical records of patients with intramedullary astrocytoma and ependymoma were reviewed. The minimal follow up time was 6 months and, at the end of this period, a comparative analysis of the neurological function was performed based using the McCormick scale score. RESULTS: Most patients had astrocytoma (75%). Male gender was more prevalent (58.3%). The most common type of tumor was graded as I or II, and in three cases these were malignant. The total resection of the tumor was achieved in 20.8% of the cases. The statistical analysis did not show a statistically significant difference between preoperative and postoperative grades at McCormick scale. CONCLUSION: The authors concluded that microsurgery to intramedullary tumors did not significantly alter the neurological function after six months.


2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Sachi Kawagishi ◽  
Naoko Ose ◽  
Masato Minami ◽  
Soichiro Funaki ◽  
Takashi Kanou ◽  
...  

Abstract Background Thymic carcinoma has been classified into 12 subtypes, thymic lymphoepithelioma-like carcinoma (LELC) is a type of them, and has a pathological organization similar to that of lymphoepithelioma, an undifferentiated type of nasopharyngeal carcinoma. According to a report from the International Thymic Malignancy Interest Group (ITMIG), thymic LELC is a rare tumor and accounts for 6% of all thymic carcinoma cases. We report two cases of surgical resection for thymic LELC and perform a search of other reports of thymic LELC, and clinical manifestations and follow-up data thus obtained are summarized. Case presentation Two patients underwent surgical resection for thymic LELC. In both, tumors were detected in the anterior mediastinum and a total thymectomy was performed. Each was diagnosed with thymic LELC and classified in accordance with the Masaoka staging system as modified stage II. In recent examinations, one patient was doing well after undergoing total resection, whereas early recurrence of distant lymph node metastasis was noted in the other at 5 months after the total resection procedure and died thereafter from a different disease. Conclusion We report two cases of surgical resection for thymic LELC. A successful total resection may positively affect prognosis: thus, long-term follow-up examinations must be performed.


2021 ◽  
Author(s):  
Xiaoxu Wang ◽  
Wei Liu ◽  
Lejian He ◽  
Min Chen ◽  
Jianbo Shao ◽  
...  

Abstract Purpose Summarized the clinical characteristics and diagnosis and treatment process of three cases of nodular fasciitis of ear, to provide a basis for clinical diagnosis and treatment. Methods Reviewed the clinical manifestations, images, pathology, treatment and postoperative follow up results of three cases of pediatric nodular fasciitis in the Department of Otorhinolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from 2018 to 2020. Results The average age at diagnosis were 24 months, with two girls and a boy. Two lesions were found in the left ear and one in the right ear. All cases had a history of biopsy before surgery. Two of three cases showed a sign of rapid growth after biopsy and three of which were ineffective in anti-inflammatory treatment. FISH test for USP6 were performed in two of the three cases with positive results. Three lesions show a hypointensity or isointensity on T1-weighted MRI and a heterogeneous hyperintensity on T2-weighted MRI. ‘‘Fascial tail’’ sign was found on image of all three cases. All lesions underwent surgical resection. Follow-up showed no recurrence and had an intact ear appearance. Conclusion The early misdiagnosis rate of nodular fasciitis of the ear is high. Combine clinical features with imaging findings may improve the accuracy of preoperative diagnosis. Besides the appearance of pathology, USP6 gene test is also an important tool in the diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence.


2019 ◽  
Vol 1 (1) ◽  
pp. V25
Author(s):  
Sima Sayyahmelli ◽  
Mustafa K. Başkaya

In this surgical video, we present a 57-year-old man with neck pain, dizziness, and imbalance. MRI showed a heterogeneously enhancing mass lesion within the posterior medulla at the level of the foramen magnum. Because the patient was symptomatic from this cavernous malformation, the decision was made to proceed with surgical resection. The patient underwent a midline suboccipital craniotomy with C1 laminectomy for surgical resection of the cavernous malformation in the medulla oblongata, with concurrent monitoring of motor and somatosensory evoked potentials.The surgery and postoperative course were uneventful. The postoperative MRI showed gross-total resection of the mass with histopathology indicating a cavernous malformation. The patient continues to do well without recurrence at 7 years of follow-up. In this video, we demonstrate important microsurgical steps for the resection of this challenging and rare vascular malformation.The video can be found here: https://youtu.be/gbGleLowzxo.


2018 ◽  
Vol 7 (4) ◽  
pp. 1-5
Author(s):  
Przemysław Krawczyk ◽  
Daniel Majszyk ◽  
Antoni Bruzgielewicz ◽  
Kazimierz Niemczyk

Granular cell tumor is benign neoplasm rarely diagnosed among young children and adolescents. The tumor developed commonly within mucous membrane of upper airways, but precise etiology is not known. Treatment is based on surgical resection of tumor and intense follow up due to risk of recurrence and malignant transformation.


2008 ◽  
Vol 109 (1) ◽  
pp. 126-129 ◽  
Author(s):  
Yuuta Kamoshima ◽  
Yutaka Sawamura ◽  
Motoyuki Iwasaki ◽  
Yoshinobu Iwasaki ◽  
Kazuhiko Sugiyama

The authors report a metachronous germ cell tumor with different histological type occurring 12 years after resection of a pineal germinoma. Histological examination of the original tumor revealed germinoma without any other component of germ cell tumor, and the patient underwent chemotherapy followed by 24 Gy of localized irradiation. Twelve years later, follow-up MR imaging showed a round mass in the genu of the corpus callosum. Two courses of chemotherapy were administered, but the tumor size remained stable. A second operation was performed and this second tumor was completely removed. The histological diagnosis was mature teratoma. The second tumor was considered as a metachronous mature teratoma rather than a recurrence of the original germinoma. To the authors' knowledge, this combination of metachronous germ cell tumor has not previously been reported in the literature


1995 ◽  
Vol 13 (5) ◽  
pp. 1177-1187 ◽  
Author(s):  
E W Steyerberg ◽  
H J Keizer ◽  
S D Fosså ◽  
D T Sleijfer ◽  
G C Toner ◽  
...  

PURPOSE To develop a statistical model that predicts the histology (necrosis, mature teratoma, or cancer) after chemotherapy for metastatic nonseminomatous germ cell tumor (NSGCT). PATIENTS AND METHODS An international data set was collected comprising individual patient data from six study groups. Logistic regression analysis was used to estimate the probability of necrosis and the ratio of cancer and mature teratoma. RESULTS Of 556 patients, 250 (45%) had necrosis at resection, 236 (42%) had mature teratoma, and 70 (13%) had cancer. Predictors of necrosis were the absence of teratoma elements in the primary tumor, prechemotherapy normal alfa-fetoprotein (AFP), normal human chorionic gonadotropin (HCG), and elevated lactate dehydrogenase (LDH) levels, a small prechemotherapy or postchemotherapy mass, and a large shrinkage of the mass during chemotherapy. Multivariate combination of predictors yielded reliable models (goodness-of-fit tests, P > .20), which discriminated necrosis well from other histologies (area under the receiver operating characteristic (ROC) curve, .84), but which discriminated cancer only reasonably from mature teratoma (area, .66). Internal and external validation confirmed these findings. CONCLUSION The validated models estimate with high accuracy the histology at resection, especially necrosis, based on well-known and readily available predictors. The predicted probabilities may help to choose between immediate resection of a residual mass or follow-up, taking into account the expected benefits and risks of resection, feasibility of frequent follow-up, the financial costs, and the patient's individual preferences.


2010 ◽  
Vol 46 (2) ◽  
pp. 121-126 ◽  
Author(s):  
Bart Van Goethem ◽  
Tim Bosmans ◽  
Koen Chiers

A 4-month-old kitten was presented with a large mass over the temporal area involving the base of the left ear. Cytological evaluation of a fine-needle aspirate was not diagnostic. Computed tomography was used to determine tumor extent. Surgical resection was performed, which included parts of the orbital rim, masticatory muscles, the complete ear canal, and the pinna. Reconstruction of the ocular muscles was performed, and the skin defect was reconstructed using a single pedicle advancement flap. Despite unilateral facial paralysis, postoperative clinical function was excellent and aesthetics were good. Histological examination revealed the tumor to be a teratoma. After a follow-up period of 3 years, no signs of recurrence were evident. Extragonadal teratomas should be considered in the differential diagnosis when young animals are presented with a growing mass located outside the abdominal cavity. Surgical excision of a mature teratoma can be considered curative.


2021 ◽  
Author(s):  
Su Weijie ◽  
Li Xixi ◽  
Wu Honglin ◽  
Tang Hongxing ◽  
Deng Zhong ◽  
...  

Abstract The complex anatomical structure of jugular foramen (JF) makes a challenge for the diagnose and treatment of this region’s tumors. The purpose of the study was to investigate the effect of surgical resection in patients with JF tumors. A total of 77 patients with JF tumors treated with surgical resection between 2012 and 2021 were retrospectively reviewed. General characteristics, tumor classification, surgical approach and outcomes were retrospectively analyzed. The mean follow-up period was 4.5 years (ranging 0.5-8.5 years). Of the enrolled patients, 63 patients (81.82%) were diagnosed with JF schwannomas. 67 cases (87.01%) of the total patients received gross-total resection. Preoperatively, hoarseness and/or dysarthria served as the most complained symptoms (n=41, 53.25%), followed by swallowing disturbance (n = 37, 48.05%), hearing disturbance and/or tinnitus (n = 32, 41.56%). Postoperatively, a total of 52 patients (67.53%) were without significant symptoms. Worsened hoarseness and/or dysarthria symptoms were found in 14 patients (18.18%). Besides, 6 cases (7.79%) of the enrolled patients showed worsened swallowing disturbance. 8 patients (10.39%) presented with facial palsy after operation and 3 of them got improvement during the follow-up. Nine patients (12.68%) had tumor recurrence and there was one death (1.3%) associated with surgery during the perioperative period in the cohort. Therefore, surgical resection was an effective treatment of JF tumors with improvements of symptoms and good control of tumor. However, operation on this region was at the risks of worsening cranial nerve deficits and death, which needed advance in skull base surgery and rich experience.


Neurosurgery ◽  
2017 ◽  
Vol 81 (1) ◽  
pp. 120-128 ◽  
Author(s):  
Kara A. Parikh ◽  
Garrett T. Venable ◽  
Brent A. Orr ◽  
Asim F. Choudhri ◽  
Frederick A. Boop ◽  
...  

Abstract BACKGROUND: Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors. OBJECTIVE: To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome. METHODS: A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed. RESULTS: Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection—compared with subtotal resection or biopsy—had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer. CONCLUSION: Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.


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