scholarly journals Intracranial solitary fibrous tumor/hemangiopericytoma – A case series

2020 ◽  
Vol 11 ◽  
pp. 414
Author(s):  
Chi-Man Yip ◽  
Shu-Shong Hsu ◽  
Wei-Chuan Liao ◽  
Szu-Hao Liu ◽  
Yung-Shang Lin ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Adjuvant Radiotherapy ◽  
Tumor Resection ◽  
Case Series ◽  
Extent Of Resection ◽  
Treatment Modalities ◽  
Total Resection ◽  
Initial Surgery ◽  
Fibrous Tumor

Background: Intracranial solitary fibrous tumor/hemangiopericytoma (HPC) is a rare and aggressive tumor. We conducted this retrospective study to investigate the outcome of patients after treatment, the efficacy of postoperative adjuvant radiotherapy, and the factors not conducive to total resection. Methods: We conducted a retrospective review of the medical records of patients harboring fresh intracranial solitary fibrous tumor/HPC treated from January 2009 to December 2019 in our hospital. We reviewed their clinical presentations, radiologic appearances, tumor size and location, extent of resection, estimate intraoperative blood loss, treatment modalities and results, and duration of follow-up. Results: There were seven consecutive patients (three males and four females). The ages of the patients at the time of diagnosis ranged from 35 to 77 years (mean: 52.86 years). Five patients (71.43%) got tumor bigger than 5 cm in dimension and only 1 patient (14.29%) underwent gross total tumor resection in the first operation without complication. Five patients (71.43%) underwent postoperative adjuvant radiotherapy. Follow-up period ranged from 4.24 to 123.55 months and the median follow-up period was 91.36 months. Three patients had favorable outcome with Glasgow Outcome Scale (GOS) equal to 4; four patients had unfavorable outcome with GOS equal to 2 or 3. No mortality was happened. Conclusion: Gross total tumor resection in the initial surgery is very important to achieve a better outcome. Massive intraoperative bleeding and venous sinus or major vessels adjoining are factors not conducive to total resection. Radiotherapy can be administered as adjuvant therapy for cases showing an aggressive phenotype or not treated with gross total resection.

scholarly journals Adult Craniopharyngioma: Case Series, Systematic Review, and Meta-Analysis

Neurosurgery ◽  
2017 ◽  
Vol 83 (4) ◽  
pp. 631-641 ◽  
Author(s):  
Charlotte Dandurand ◽  
Amir Ali Sepehry ◽  
Mohammad Hossein Asadi Lari ◽  
Ryojo Akagami ◽  
Peter Gooderham
Keyword(s):  
Systematic Review ◽  
Adjuvant Radiotherapy ◽  
Meta Analysis ◽  
Case Series ◽  
Extent Of Resection ◽  
Treatment Modalities ◽  
Future Research ◽  
Subtotal Resection ◽  
Risk Of Recurrence ◽  
Institutional Experience

Abstract BACKGROUND The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others advocate for subtotal resection followed by adjuvant radiotherapy (STR + XRT). OBJECTIVE To conduct a systematic review and meta-analysis assessing the rate of recurrence in the follow-up of 3 yr in adult craniopharyngioma stratified by extent of resection and presence of adjuvant radiotherapy. METHODS MEDLINE (1946-July 1, 2016) and EMBASE (1980-June 30, 2016) were systematically reviewed. From1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma at our center and were reviewed for inclusion in this study. RESULTS Data from 22 patients were available for inclusion as a case series in the systematic review. Eligible studies (n = 21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17%, 27%, and 45%, respectively. The risk of developing recurrence was significant for GTR vs STR (odds ratio [OR]: 0.24, 95% confidence interval [CI]: 0.15-0.38) and STR + XRT vs STR (OR: 0.20, 95% CI: 0.10-0.41). Risk of recurrence after GTR vs STR + XRT did not reach significance (OR: 0.63, 95% CI: 0.33-1.24, P = .18). CONCLUSION This is the first and largest systematic review focusing on the rate of recurrence in adult craniopharyngioma. Although the rates of recurrence are favoring GTR, difference in risk of recurrence did not reach significance. This study provides guidance to clinicians and directions for future research with the need to stratify outcomes per treatment modalities.

scholarly journals Idiopathic and radiation-induced myxofibrosarcoma in the head and neck—case report and literature review

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Bin Zhang ◽  
Miao Bai ◽  
Runfa Tian ◽  
Shuyu Hao
Keyword(s):  
Head And Neck ◽  
Tumor Resection ◽  
Diagnosis And Treatment ◽  
Treatment Modalities ◽  
Total Resection ◽  
Extensive Resection ◽  
History Of ◽  
Radiation Induced ◽  
Reasonable Choice

Abstract Background Myxofibrosarcoma (MFS), especially radiation-Induced MFS (RIMFS) in the head and neck, is an extremely rare malignant fibroblastic tumor. The diagnosis and treatment of MFS remain great challenges. In the present study, we presented one case of RIMFS. Combined with previous literature, the clinical features, essentials of diagnosis, and treatment modalities of MFS in the head and neck were reviewed to better understand this rare entity. Case presentation We reported a case of RIMFS under the left occipital scalp in a 20-year-old girl with a history of medulloblastoma surgery and radiotherapy in 2006. A total tumor resection was performed with preservation of the overlying scalp the underlying bone, and no adjuvant therapy was administered after the first operation. The postoperative pathological diagnosis was high-grade MFS. The tumor relapsed 6 months later, and then, a planned extensive resection with negative surgical margins was carried out, followed by radiotherapy. No relapse occurred in a 12-month postoperative follow-up. Conclusions Planned gross total resection (GTR) with negative margins is the reasonable choice and footstone of other treatments for MFS. Ill-defined infiltrated borders and the complicated structures make it a great trouble to achieve total resection of MFS in the head and neck, so adjuvant radiotherapy and chemotherapy seem more necessary for these lesions.

scholarly journals Endoscopic Endonasal Intraconal Approach for Orbital Tumor Resection: Case Series and Systematic Review

2022 ◽  
Vol 11 ◽  
Author(s):  
Xin Zhang ◽  
Wei Hua ◽  
Kai Quan ◽  
Guo Yu ◽  
Zunguo Du ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Case Series ◽  
Oculomotor Nerve Palsy ◽  
Cerebrospinal Fluid Leakage ◽  
Lateral Extension ◽  
Total Resection ◽  
Endoscopic Endonasal ◽  
Pathological Conditions ◽  
Surgical Methods

Intraorbital tumor could be approached by numerous surgical methods. The neuroendoscopic endonasal approach could provide a feasible corridor for indicated tumors. Herein we present a series of 6 consecutive intraorbital tumors from April 2018 to October 2020, which received endonasal endoscopic resection. Cadaveric dissection was performed for the intraconal approach, and the literature was also reviewed. Five tumors were located intraconally, while one extraconally. The pathology revealed 1 angioleiomyoma, 1 cavernous hemangioma, 1 pilocytic astrocytoma, 1 meningioma, and 2 schwannomas. Five of the six achieved gross total resection, including 3 tumors with lateral extension beyond the optic nerve. Preoperative visual deterioration was observed in 4 of the 6 patients, and all got improvement postoperatively. Transient oculomotor nerve palsy was presented in one patient postoperatively. No cerebrospinal fluid leakage, enophthalmos, or strabismus was observed. The median follow-up time is 27 months (11~41 months). At the 6-month follow-up, the visual acuity remained unchanged compared with that at discharge. Proptosis was resolved in 2 of the 3 patients; diplopia was improved in one patient. In conclusion, endoscopic endonasal intraconal approach could be suitable for selected pathological conditions, and for both medial or beyond medial extraconal and intraconal orbital tumors.

Primary Calvarial Ewing Sarcoma: A Case Series

2021 ◽  
Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Tumor Resection ◽  
Case Series ◽  
Radical Excision ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Intradural Tumor ◽  
Age Range

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.

Pazopanib-associated secondary adrenal insufficiency in a patient with malignant solitary fibrous tumor

2021 ◽  
pp. 107815522110160
Author(s):  
Muhammed Muhiddin Er ◽  
Murat Araz ◽  
Meryem Karabacak ◽  
Muzaffer Uğraklı ◽  
Melek Karakurt Eryılmaz ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Solitary Fibrous Tumor ◽  
Single Case ◽  
Soft Tissue Sarcomas ◽  
Pituitary Hormones ◽  
Secondary Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Hypoglycemic Attack ◽  
Fibrous Tumor

Introduction Pazopanib is an agent that is being successfully used in soft tissue sarcomas. Some endocrine side effects may develop during pazopanib treatment. Here, we presented a case diagnosed with secondary adrenal insufficiency while being investigated for etiology of hypoglycemia which developed after pazopanib. Case report A 69-year-old male patient was operated in June 2019 due to a lung mass 26 × 18 × 10 cm in size. Pathological diagnosis revealed a solitary fibrous tumor with malignant behavior. The patient received three lines of chemotherapy. After pazopanib treatment, a hypoglycemic attack was reported. Management and outcome: Blood cortisol and ACTH (Adrenocorticotropic hormone) levels were not increased at the time of the hypoglycemic attack, and levels of other pituitary hormones were found to be normal. Electrolyte levels were in normal range. Since the counteracting hormone did not reach a sufficient level, it was considered secondary adrenal insufficiency. Hypoglycemic attacks did not occur during follow-up while taking steroid therapy and pazopanib. Discussion A single case of primary adrenal insufficiency has been reported in the literature. We here present a case who developed hypoglycemia after pazopanib and was diagnosed with drug-associated secondary adrenal insufficiency. When hypoglycemia develops during pazopanib treatment, we must be aware of adrenal insufficiency.

scholarly journals Anterior mediastinal solitary fibrous tumor resection by da Vinci ® Surgical System in obese patient

2017 ◽  
Vol 38 ◽  
pp. 163-165
Author(s):  
Dario Amore ◽  
Marco Rispoli ◽  
Marcellino Cicalese ◽  
Ilaria De Rosa ◽  
Giuseppe Rossi ◽  
...  
Keyword(s):  
Obese Patient ◽  
Solitary Fibrous Tumor ◽  
Da Vinci ◽  
Tumor Resection ◽  
Fibrous Tumor ◽  
Surgical System

scholarly journals Solitary Fibrous Tumors of the Chest: An Analysis of Fifty Patients

2021 ◽  
Vol 11 ◽  
Author(s):  
Jingwen Zhang ◽  
Jumin Liu ◽  
Zhihao Zhang ◽  
Beizong Tian
Keyword(s):  
Solitary Fibrous Tumor ◽  
Chest Tightness ◽  
Ki 67 ◽  
Imaging Characteristics ◽  
Enhanced Ct ◽  
The Common ◽  
The Difference ◽  
Low Incidence ◽  
Fibrous Tumor

BackgroundA solitary fibrous tumor of the chest (SFTC) is a subtype of solitary fibrous tumor (SFT) with a low incidence rate. The purpose of this study is to analyze the diagnosis and treatment of SFTC and the difference between benign and malignant solitary fibrous tumor of the pleura (SFTP) to improve the understanding of this rare disease.MethodsA retrospective analysis of fifty patients with SFTC (33 cases in the pleura and 17 in the lung) was performed. Clinical and imaging characteristics, pathological features, and treatment follow-up outcomes were analyzed.ResultsThe common symptoms of the 50 patients included a cough, expectoration, chest tightness, fever, and chest pain. Space occupying lesions were found via plain computed tomography (CT) and enhanced CT was used for enhancement of the tumors. It was also found that 18 cases had necrosis, and 5 cases had calcification. The histopathology results showed that frequent nuclear division, obvious morphological variation, necrosis, and the high expression of Ki-67 cells are markers of malignant SFTC. There were significant differences in age, chest tightness, necrotic foci in CT, and expression of Ki-67 between the benign and malignant SFTP cases. All the patients who received treatment were given an excellent prognosis.ConclusionA combination of enhanced CT, histopathology, and immunohistochemistry can be used for the accurate diagnosis of SFTC. Advanced age, chest tightness, necrotic foci in CT, and a high Ki-67 index were more likely to be malignant SFTP. Operation and radiofrequency ablation can provide favorable outcomes for both benign and malignant SFTC.

scholarly journals Clinical presentation, natural history and therapeutic approach in patients with solitary fibrous tumor: A retrospective analysis.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e22522-e22522
Author(s):  
Patrick Schoffski ◽  
Iris Timmermans ◽  
Daphne Hompes ◽  
Marguerite Stas ◽  
Veerle Boecxstaens ◽  
...  
Keyword(s):  
Retrospective Analysis ◽  
Metastatic Disease ◽  
Solitary Fibrous Tumor ◽  
Systemic Therapy ◽  
Single Agent ◽  
Disease Free Survival ◽  
Metachronous Metastasis ◽  
Palliative Intent ◽  
Fibrous Tumor

e22522 Background: Solitary fibrous tumor (SFT), a rare variant of soft tissue sarcoma (STS), is characterized by the presence of a NAB2-STAT6 fusion. Given the orphan character of SFT we performed a retrospective analysis. Methods: We retrospectively reviewed all patients (pts) with SFT treated in our institution between 12/1990 - 09/2017. Results: We identified 94 SFT pts (incl. hemangiopericytoma) with a med. follow-up for 4.7 yrs. Common anatomic sites were chest (33%), abdomen (21.3%), brain (12.8%) and extremities (9.6%). The symptomatology at diagnosis was variable. Only 6.4% presented with synchronous metastasis. Hypoglycemia (Doege-Potter syndrome) was seen in 2.1% of cases. A resection of the primary SFT was done in 86 pts (91.5%), their disease-free survival was 35.5 mo and 43% stayed SFT-free during follow-up. Local recurrence occurred in 26.7% of cases, associated with a med. overall survival (OS) of 45.1 mo. Metachronous metastasis was seen in 30.2% of pts, occurring after a med. follow-up of 36 mo. Med. OS after diagnosis of metastasis was 19.0 mo. Systemic therapy was given to 92.9% of pts with inoperable/metastatic disease. The most common 1st line therapy was doxorubicin single agent (57.7% of pts), achieving responses in 13.3% of pts. 2nd line therapies included ifosfamide and pazopanib (31.3% of pts each), 3rd line treatment was very heterogeneous. Conclusions: SFT is an orphan malignancy with a variable clinical course, low incidence of distant spread at first diagnosis but considerable risk of local failure and metachronous metastasis. Surgery is the only curative option at diagnosis, time of relapse and in case of resectable metastasis. Palliative systemic therapy is considered in pts with inoperable/metastatic disease but achieves low response rates. The natural course and survival outcomes of SFT cases treated with palliative intent tend to be better than in non-selected STS pts.

Solitary fibrous tumor in the abdomen and pelvis: A case series with radiological findings and treatment recommendations

2018 ◽  
Vol 48 ◽  
pp. 48-54 ◽  
Author(s):  
Adrian Fernandez ◽  
Miles Conrad ◽  
Ryan M. Gill ◽  
Won-Tak Choi ◽  
Vishal Kumar ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Case Series ◽  
Treatment Recommendations ◽  
Radiological Findings ◽  
Fibrous Tumor

Diffuse Pigmented Villonodular Synovitis Around the Ankle

2018 ◽  
Vol 108 (2) ◽  
pp. 140-144 ◽  
Author(s):  
Keiichi Muramatsu ◽  
Ryuta Iwanaga ◽  
Yasuhiro Tominaga ◽  
Takahiro Hashimoto ◽  
Toshihiko Taguchi
Keyword(s):  
Ankle Joint ◽  
Tumor Resection ◽  
Tumor Location ◽  
Pigmented Villonodular Synovitis ◽  
Growth Patterns ◽  
Treatment Modalities ◽  
Villonodular Synovitis ◽  
Lateral Incision ◽  
Pigmented Villonodular

Background: Pigmented villonodular synovitis (PVNS) is a rare disorder around the ankle joint. The optimal treatment for diffuse-type PVNS is still controversial because of the high incidence of recurrence. We present the clinical features of our patients and review the current diagnostic and treatment modalities. Methods: Five patients with PVNS located around the ankle were surgically treated. In three patients, diffuse PVNS arose from the ankle joint, and in the other two it arose from the calcaneocuboid and intercuneiform joints. The average follow-up time after surgery was 2.9 years (range, 2–4.6 years). Results: The average time between onset of pain and diagnosis of PVNS was 6.4 years (range, 4–10 years). Arthrotomic tumor resection was performed in all of the patients. In the three patients with ankle joint PVNS, both medial and lateral approaches were used. One patient experienced mild infection at the surgical site, but this healed conservatively. No tumor recurrences had occurred after minimum follow-up of 2 years, although mild pain persisted in the three patients with ankle PVNS. Conclusions: Diagnosis of diffuse PVNS is frequently delayed due to vague symptoms and variable growth patterns. Orthopedic clinicians should be aware of the existence of this lesion, and it should be suspected in patients with persistent ankle swelling. To prevent tumor recurrence, accurate evaluation of tumor location and careful operative planning are mandatory. A combined surgical approach involving medial and lateral incision is necessary to expose the entire joint cavity.

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