Chondromyxoid Fibroma of the Skull Base: Our Experience with an Elusive Disease

2017 ◽  
Vol 78 (06) ◽  
pp. 576-581 ◽  
Author(s):  
Giancarlo D'Andrea ◽  
Guido Trasimeni ◽  
Venceslao Wierzbicki ◽  
Veronica Picotti ◽  
Alessandra Serraino ◽  
...  
Keyword(s):  
Skull Base ◽  
Local Control ◽  
Osteolytic Lesion ◽  
Tumor Resection ◽  
Brain Magnetic Resonance Imaging ◽  
High Dose ◽  
Adjuvant Radiation ◽  
Chondromyxoid Fibroma ◽  
Histologic Diagnosis ◽  
The Right

Chondromyxoid fibroma (CMF) is an extremely rare lesion of the skull base. This histologic type typically predilects metaphysis of the long bones. It is locally invasive/infiltrative, and this tendency is more concerning in the skull base, where a radical resection is often technically impossible because of the presence of vital neurovascular structures. We present a case of a 19-year-old woman who presented with a sudden onset of right facial weakness, progressively worsening to a severe disfiguring motor weakness. Gadolinium-enhanced brain magnetic resonance imaging showed an osteolytic lesion located in the right mastoid involving the stylomastoid foramen and the right seventh cranial nerve. A partial mastoidectomy was performed, with an excellent rate of tumor resection and complete local control of the disease at follow-up. The analysis of current literature indicates that a radiologic diagnosis is rarely strictly convincing of CMF. Histologic diagnosis is often difficult due to the lack of a specific immunohistochemical pattern of chondrosarcoma. Surgery is currently recognized as the mainstay to manage this lesion, although a trend toward adjuvant radiation therapy (RT) currently is seen. Although a tendency of local recurrence is well recognized in the literature, the very slow and indolent behavior of this lesion plus the trend to enhance local control of the disease with high-dose RT pushed us to a reappraise the role of radical skull base surgeries burdened by the risks of major complications, cosmetic deformities, and additional neurologic deficits.

scholarly journals ETMR-07. ETANTR: A RARE TUMOR IN A RESOURCE-LIMITED SETTING

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii324-iii324
Author(s):  
Julieta Hoveyan ◽  
Manushak Avagyan ◽  
Anna Avagyan ◽  
Ruzanna Papyan ◽  
Samvel Iskanyan ◽  
...  
Keyword(s):  
Survival Rates ◽  
Tumor Resection ◽  
Large Mass ◽  
Intrathecal Methotrexate ◽  
High Dose ◽  
Ongoing Research ◽  
Resource Limited ◽  
The Right ◽  
Immunohistochemical Analyses ◽  
Limited Setting

Abstract INTRODUCTION Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare aggressive brain tumor with low survival rates. There are about 80 cases reported in literature since 2000 when it was first described. There is no standard treatment scheme for ETANTR yet. CASE REPORT: A 2 years old boy presented with a month-long of headache and inability to hold his head. CT scan and MRI revealed a large mass in the right frontal lobe with midline shift. Subtotal tumor resection was done. Histological and immunohistochemical analyses was consistent with ETANTR in one laboratory and PNET in another. The second opinion suggested by the Center of Pediatric Oncology, Hematology and Immunology in Moscow the diagnosis ETANTR was confirmed. Taking into account certain similarities with medulloblastoma was decided to provide treatment according to HIT-2014 protocol. Control MRI done after 2 cycles of Block SKK Carboplatin/Etoposide found tumor progression and for that reason patient underwent second surgical resection. Considering the age of the child radiation therapy was not expedient and the decision was to continue treatment with HIT 2014 intensified regimen, which includes Cisplatin, Vincristine, Etoposide, Cyclophosphamide and intravenous High dose Methotrexate with intrathecal Methotrexate. Aiming to evaluate the effectiveness of treatment we are planning to perform MRI after this 2nd cycle of intensified regimen. DISCUSSION There are difficulties in diagnosis of rare types of cancers in Armenia. Since there is no approved treatment for ETANTR, there is a need for ongoing research to improve its prognosis.

Microsurgical Resection of Recurrent Cavernous Sinus Hemangioma by Superior and Lateral Approach: 2-Dimensional Operative Video

2019 ◽  
Vol 19 (2) ◽  
pp. E165-E166
Author(s):  
Qazi Zeeshan ◽  
Juan P Carrasco Hernandez ◽  
Michael K Moore ◽  
Laligam N Sekhar
Keyword(s):  
Optic Nerve ◽  
Carotid Artery ◽  
Cavernous Sinus ◽  
Tumor Resection ◽  
Cranial Nerves ◽  
Brain Magnetic Resonance Imaging ◽  
Mass Effect ◽  
Lateral Approach ◽  
Microsurgical Resection ◽  
The Right

Abstract This video shows the technical nuances of microsurgical resection of recurrent cavernous sinus (CS) hemangioma by superior and lateral approach.  A 77-yr-old woman presented with headache and difficulty in vision in right eye for 6 mo. She had previously undergone attempted resection of a right CS tumor in another hospital with partial removal, and the tumor had grown significantly. Neurological examination revealed proptosis, cranial nerve 3 palsy, and loss of vision in right eye (20/200). Left side visual acuity was 20/20.  Brain magnetic resonance imaging (MRI) demonstrated a large CS mass with homogeneous enhancement, measuring 3.3 × 3.3 × 2.6 cm, extending into the suprasellar cistern with mass effect on the right optic nerve. It extended anteriorly to the region of the right orbital apex and abuted the basilar artery posteriorly.  She underwent right frontotemporal craniotomy, posterolateral orbitotomy and anterior clinoidectomy as well as optic nerve decompression, and the CS tumor was removed by superior and lateral approach. An incision was made into the superior wall of the CS medial to the third nerve. On lateral aspect the tumor had extended outside the CS through the Parkinson's triangle. Posteriorly it extended through the clival dura. Anteriorly tumor encased the carotid artery and it was gradually dissected away. At the end of the operation, all of the cranial nerves were intact.  Postoperative MRI showed near complete tumor resection with preservation of the internal carotid artery. At 6 mo follow-up her modified Rankin Scale was 1 and vision in left eye was normal.  Informed consent was obtained from the patient prior to the surgery that included videotaping of the procedure and its distribution for educational purposes. All relevant patient identifiers have also been removed from the video and accompanying radiology slides.

scholarly journals Clinical Outcomes Following Dose-Escalated Proton Therapy for Skull-Base Chordoma

2021 ◽  
Vol 8 (1) ◽  
pp. 179-188
Author(s):  
Adam L. Holtzman ◽  
Ronny L. Rotondo ◽  
Michael S. Rutenberg ◽  
Daniel J. Indelicato ◽  
Alexandra De Leo ◽  
...  
Keyword(s):  
Overall Survival ◽  
Skull Base ◽  
Local Control ◽  
Subtotal Resection ◽  
High Dose ◽  
Disease Specific Survival ◽  
Total Resection ◽  
Skull Base Chordoma ◽  
Disease Specific

Abstract Purpose To evaluate the effectiveness of external-beam proton therapy (PT) on local control and survival in patients with skull-base chordoma. Materials and Methods We reviewed the medical records of patients with skull-base chordoma treated with definitive or adjuvant high-dose PT and updated their follow-up when feasible. We assessed overall survival, disease-specific survival, local control, and freedom from distant metastasis. Radiotherapy toxicities were scored using the Common Terminology Criteria for Adverse Events, version 4.0. Results A total 112 patients were analyzed, of whom 105 (94%) received PT and 7 (6%) received combined proton-photon therapy between 2007 and 2019. Eighty-seven patients (78%) underwent a subtotal resection, 22 (20%) a gross total resection, and 3 (3%) a biopsy alone. The median radiotherapy dose was 73.8 Gy radiobiologic equivalent (GyRBE; range, 69.6-74.4). Ninety patients (80%) had gross disease at radiotherapy and 7 (6%) were treated for locally recurrent disease following surgery. Median follow-up was 4.4 years (range, 0.4-12.6); for living patients, it was 4.6 years (range, 0.4-12.6), and for deceased patients, 4.1 years (range, 1.2-11.2). At 5 years after radiotherapy, the actuarial overall survival, disease-specific survival, local control, and freedom from distant metastasis rates were 78% (n = 87), 83% (n = 93), 74% (n = 83), and 99% (n = 111), respectively. The median time to local progression was 2.4 years (range, 0.8-7). Local control and disease-specific survival by resection status was 95% versus 70% (P = 0.28) and 100% versus 80% (P = 0.06) for gross total, versus subtotal, resection or biopsy alone, respectively. There were no serious acute toxicities (grade ≥ 3) related to radiotherapy. Conclusion High-dose PT alone or after surgical resection for skull-base chordoma reaffirms the favorable 5-year actuarial local control rate compared with conventional techniques with acceptable late-complication–free survival. Outcomes following gross total resection and adjuvant PT were excellent. Further follow-up of this cohort is necessary to better characterize long-term disease control and late toxicities.

Skull Base Meningiomas: Is Surgical Resection Enough? Outcome Evaluation and Prognostic Factors Analysis in a Single-Center Cohort

2021 ◽  
Author(s):  
Federico Pessina ◽  
Pierina Navarria ◽  
Zefferino Rossini ◽  
Elena Clerici ◽  
Maurizio Fornari ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Skull Base ◽  
Surgical Resection ◽  
Brain Magnetic Resonance Imaging ◽  
Outcome Evaluation ◽  
High Recurrence Rate ◽  
Subtotal Resection ◽  
Adjuvant Radiation ◽  
Karnofsky Performance Score ◽  
Skull Base Meningiomas

Abstract Background Surgical resection represents the mainstay of treatment in skull base meningiomas (SBMs). Considering the high recurrence rate reported, an adjuvant radiation therapy (RT) treatment should be considered. The aim of this study was to evaluate the progression-free survival (PFS), overall survival (OS), and prognostic factors conditioning outcome. Methods Patients receiving surgical resection for grade I SBMs were included. The extent of resection (EOR) was dichotomized as gross total resection (GTR) and subtotal resection (STR). RT was administered only in patients receiving STR. Clinical outcome was evaluated by brain magnetic resonance imaging (MRI) performed every 6 months for the first year and yearly thereafter. Results From January 2000 to December 2015, 123 patients were treated. The majority were females (70.7%), with a Karnofsky Performance Score (KPS) ≥80 (95%), and symptoms at diagnosis (91%). GTR was performed in 30% of cases and STR in 70%. RT was performed in 18 (20.9%) patients at diagnosis and in 29 (33.7%) patients at progression. Improvement or stability of neurologic status was obtained in 78.9% of patients. The median follow-up time was 91 months (range: 40–230 months). Local recurrence occurred in 34 (27.6%) patients at a median time of 45 months (range: 6–214 months). The median, 2-, 5-, and 10-year PFS were 193 months, 89.3, 81.8, and 72.5%, respectively. On univariate and multivariate analyses, factors impacting on PFS were EOR, tumor location, neurologic postoperative status, and adjuvant RT in STR. Conclusions A safe surgical resection followed by RT adjuvant treatment could represent the better choice to obtain local control maintaining neurologic integrity. Our data underlined the value of adjuvant RT in incompletely resected meningiomas.

Placement of cesium-131 permanent brachytherapy seeds using the endoscopic endonasal approach for recurrent anaplastic skull base meningioma: case report and technical note

2020 ◽  
Vol 132 (3) ◽  
pp. 921-926 ◽  
Author(s):  
Asif Raza Shafiq ◽  
A. Gabriella Wernicke ◽  
Charles Alex Riley ◽  
Peter F. Morgenstern ◽  
Lucy Nedialkova ◽  
...  
Keyword(s):  
Skull Base ◽  
Local Control ◽  
Tumor Resection ◽  
External Beam Radiation ◽  
Endoscopic Endonasal Approach ◽  
Endonasal Approach ◽  
Endoscopic Endonasal ◽  
Beam Radiation ◽  
Invasive Approach ◽  
Skull Base Meningioma

There are few therapeutic options available for the treatment of recurrent meningiomas that have failed treatment with surgery and external-beam radiation therapy (EBRT). As additional EBRT is clinically risky, brachytherapy offers an important alternative for optimizing local control. In skull base meningiomas, the endoscopic endonasal approach (EEA) has demonstrated an excellent extent of resection. However, in the case of recurrent, atypical, or residual meningiomas, the EEA alone may not be adequate to address microscopic, residual, highly proliferative disease. In this situation, local radioactive seed brachytherapy has been shown to improve control, but few reports of this technique exist. A 48-year-old right-handed man presented on multiple occasions with recurrence of an anaplastic skull base meningioma, after multiple prior gross-total resections and multiple rounds of radiotherapy had failed. The authors performed a maximally safe neurosurgical tumor resection via EEA supplemented by the intraoperative implantation of 131Cs low-dose permanent brachytherapy seeds. They describe a technique for permanent implantation of brachytherapy seeds and provide operative video of this technique. The authors submit that utilizing this technique in combination with EEA tumor resection renders a minimally invasive approach to improving local control in a patient with a recurrent anaplastic or atypical meningioma of the skull base.

scholarly journals RARE-40. OUTCOMES AFTER PROTON THERAPY FOR SKULL-BASE CHORDOMA: A PROSPECTIVE STUDY

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi230-vi230
Author(s):  
Adam Holtzman ◽  
Ronny Rotondo ◽  
Michael Rutenberg ◽  
Daniel Indelicato ◽  
Dinesh Rao ◽  
...  
Keyword(s):  
Skull Base ◽  
Local Control ◽  
Proton Therapy ◽  
High Dose ◽  
Free Survival ◽  
3 Dimensional ◽  
Grade 3 ◽  
Skull Base Chordoma ◽  
Cause Specific Survival

Abstract We evaluated treatment outcomes following definitive or adjuvant high-dose, image-guided proton therapy in 91 patients with skull-base chordoma enrolled on a prospective outcomes tracking protocol and treated between February 2007 and February 2018. The median age was 53 years (range, 22–78 years). Patients received passively scattered 3-dimensional conformal proton therapy to a median dose of 73.8 GyRBE (range, 69.6–75.6 GyRBE). Two patients received a component of intensity-modulated radiotherapy. Seventy percent (n=64) were men and 30% (n=27) were woman. Eighty-two percent (n=75) of patients had macroscopic disease at the time of radiotherapy; 18% (n=16) had undergone a macroscopic gross total resection. Overall survival, cause-specific survival, local control, and RT-related grade 3 toxicity-free survival were calculated. Proton therapy-related toxicities were scored using CTCAE v4.0. With a median follow-up of 3.7 years (range, 0.2–10 years), 26 patients experienced disease recurrence, including 26 local, 0 regional, and 1 distant recurrence. The median time to local progression was 2.2 years (range, 0.4–7.0 years). At the time of last follow-up, 66 patients were alive (56 with no evidence of disease progression) and 25 were deceased (18 with disease progression). There were no acute grade 3 toxicities related to the radiation therapy. The 4-year actuarial rates of overall survival, cause-specific survival, local control, and radiation therapy-related grade 3 toxicity-free survival were 83%, 87%, 76% and 83%, respectively. Definitive or adjuvant high-dose passively scattered 3-dimensional conformal proton therapy for skull-base chordoma provides acceptable local control, comparing favorably to historic photon data, with no acute grade ≥3 radiation-related toxicity and an acceptable rate of grade ≥3 late toxicity. Further follow-up of this cohort is necessary to better characterize long-term disease control and late toxicities.

scholarly journals CS-6 A case of poorly differentiated chordoma with systemic metastasis

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi28-vi28
Author(s):  
Naoki Shinojima ◽  
Keisuke Harada ◽  
Yuji Dekita ◽  
Haruaki Yamamoto ◽  
Mai Itouyama ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Residual Tumor ◽  
Final Diagnosis ◽  
High Dose ◽  
Local Doctor ◽  
Short Period ◽  
Systemic Metastasis ◽  
Poorly Differentiated ◽  
The Right ◽  
Pterygoid Muscles

Abstract A case report: The patient was a 32-year-old man with diplopia. He was diagnosed as sphenoid sinusitis on MRI by a local doctor and visited an otolaryngologist. MRI showed extensive extension of neoplastic lesions from the clivus to the sphenoid sinus to the anterior ethmoid sinuses, bilateral cavernous sinuses, and the right medial and lateral pterygoid muscles. The right Lebiere’s lymph node was enlarged and thought to be a metastatic site. Based on the rapid growth and extension of the tumor, the patient was referred to the Department of Otolaryngology at our hospital on suspicion of sinonasal carcinoma. The possibility of chordoma could not be denied, so the patient was referred to our department. The patient underwent a joint endoscopic extended transsphenoidal tumor resection. The pathological diagnosis showed mitotic and necrotic features, and the majority of the cells showed highly atypical components without mucous substrate. However, brachyury, a marker for chordoma, was diffusely positive, and there was loss of INI1 (SMARCB1) expression. The final diagnosis was poorly differentiated chordoma. Postoperatively, the tumor in the right cavernous sinus grew rapidly, and the right eye became blind due to obstruction of the superior ophthalmic vein. The patient was treated with Gamma Knife as soon as possible in the hope of local control by high-dose irradiation, and after a total of three irradiations, the residual tumor shrank markedly and symptoms improved, but systemic metastasis occurred in a short period of time and the patient died. The number of cases of poorly differentiated chordoma has been reported rarely (more than 50), and it is more common in children and even rarer in adults. We report this case with a review of the literature.

scholarly journals Leptomeningeal Enhancement due to Neurosarcoidosis Mimicking Malignancy

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Sahara N. Saltijeral ◽  
Horiana B. Grosu ◽  
Henriette De La Garza ◽  
Barbara O’Brien ◽  
Gloria Iliescu
Keyword(s):  
Present Report ◽  
Brain Magnetic Resonance Imaging ◽  
Granulomatous Inflammation ◽  
Needle Aspiration ◽  
High Dose ◽  
Lower Extremity Weakness ◽  
Leptomeningeal Enhancement ◽  
Positron Emission ◽  
Endobronchial Ultrasonography ◽  
The Right

The present report describes the case of a 56-year-old African American man experiencing progressive disequilibrium, lower extremity weakness, difficulty walking, and hearing loss. Brain magnetic resonance imaging showed leptomeningeal enhancement. Initial differential diagnosis was broad, including malignant, infectious, and inflammatory etiologies. The cerebrospinal fluid analyses demonstrated lymphocytic pleocytosis, hypoglycorrhachia, and hyperproteinorrachia but no other abnormalities. An extensive infectious disease workup was negative. Positron emission tomography revealed hypermetabolic lymph nodes in the right mediastinum and right hilum, correlating with findings on endobronchial ultrasonography. Subsequently, image-guided fine-needle aspiration of the right upper paratracheal lymph node was performed, and biopsy studies showed noncaseating granulomatous inflammation. Based on the clinical picture, the diagnosis of neurosarcoidosis was made, and high-dose steroids were started and resulted in significant improvement.

scholarly journals Six-year follow-up of a characteristic osteolytic lesion in a patient with tumor-induced osteomalacia

2014 ◽  
Vol 170 (1) ◽  
pp. K1-K4 ◽  
Author(s):  
Sara Piemonte ◽  
Elisabetta Romagnoli ◽  
Cristiana Cipriani ◽  
Federica De Lucia ◽  
Roberta Pilotto ◽  
...  
Keyword(s):  
Osteolytic Lesion ◽  
Tumor Resection ◽  
Complete Recovery ◽  
Lytic Lesion ◽  
Mineral Density ◽  
Skeletal Involvement ◽  
Dihydroxyvitamin D ◽  
Cortical Shell ◽  
The Right

ObjectiveTumor-induced osteomalacia is a rare paraneoplastic syndrome characterized by hypophosphatemia and inappropriately normal or low 1,25-dihydroxyvitamin D.Clinical caseHere, we report a 6-year postoperative follow-up of a patient with oncogenic osteomalacia with a distinctive skeletal manifestation. The latter was characterized by an almost linear lytic lesion of a few millimeters with irregular borders, mainly involving the trabecular compartment but extending into cortical shell, located in the middle third of the right fibula. Six years after tumor resection, a sclerotic repair with a complete recovery was observed. Furthermore, we monitored a striking increase in bone mineral density throughout the observation period, reaching a peak of 73% over basal values at lumbar spine after 2 years; at total femur and radius, the peak was 47.5 and 4.6% respectively, after 4 years from tumor resection.ConclusionsWe report for the first time that an osteolytic lesion may be part of the skeletal involvement in tumor-induced osteomalacia.

Eosinophilic vasculitis: an inhabitual and resistant manifestation of a vasculitis

VASA ◽  
2010 ◽  
Vol 39 (4) ◽  
pp. 344-348 ◽  
Author(s):  
Jandus ◽  
Bianda ◽  
Alerci ◽  
Gallino ◽  
Marone
Keyword(s):  
Skin Biopsy ◽  
Intravenous Iron ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Raynaud Phenomenon ◽  
The Third ◽  
Left Elbow ◽  
Right Hand ◽  
The Right ◽  
Trunk Skin

A 55-year-old woman was referred because of diffuse pruritic erythematous lesions and an ischemic process of the third finger of her right hand. She was known to have anaemia secondary to hypermenorrhea. She presented six months before admission with a cutaneous infiltration on the left cubital cavity after a paravenous leakage of intravenous iron substitution. She then reported a progressive pruritic erythematous swelling of her left arm and lower extremities and trunk. Skin biopsy of a lesion on the right leg revealed a fibrillar, small-vessel vasculitis containing many eosinophils.Two months later she reported Raynaud symptoms in both hands, with a persistent violaceous coloration of the skin and cold sensation of her third digit of the right hand. A round 1.5 cm well-delimited swelling on the medial site of the left elbow was noted. The third digit of her right hand was cold and of violet colour. Eosinophilia (19 % of total leucocytes) was present. Doppler-duplex arterial examination of the upper extremities showed an occlusion of the cubital artery down to the palmar arcade on the right arm. Selective angiography of the right subclavian and brachial arteries showed diffuse alteration of the blood flow in the cubital artery and hand, with fine collateral circulation in the carpal region. Neither secondary causes of hypereosinophilia nor a myeloproliferative process was found. Considering the skin biopsy results and having excluded other causes of eosinophilia, we assumed the diagnosis of an eosinophilic vasculitis. Treatment with tacrolimus and high dose steroids was started, the latter tapered within 12 months and then stopped, but a dramatic flare-up of the vasculitis with Raynaud phenomenon occurred. A new immunosupressive approach with steroids and methotrexate was then introduced. This case of aggressive eosinophilic vasculitis is difficult to classify into the usual forms of vasculitis and constitutes a therapeutic challenge given the resistance to current immunosuppressive regimens.

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