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Heliyon Jan 2020Perivascular epithelioid cell tumors (PEComas) of the skull base are extremely rare. Here we report the first description of a malignant PEComa mimicking jugular foramen...
BACKGROUND
Perivascular epithelioid cell tumors (PEComas) of the skull base are extremely rare. Here we report the first description of a malignant PEComa mimicking jugular foramen schwannoma and presenting as Collet-Sicard syndrome, and we review the previous literature on PEComas of the head, neck and skull base.
CASE DESCRIPTION
A 29-year-old woman presented with hoarseness, dysphagia, vomiting, and headache. She was first diagnosed with Collet-Sicard syndrome caused by thrombosis of the sigmoid and transverse sinuses. She was treated with anticoagulant therapy, and the hoarseness and paralysis of the accessory nerve improved. Later, at age 31, the hoarseness again worsened. At another hospital, enhanced computed tomography revealed a tumor in the jugular foramen extending to the neck and medially displacing the internal carotid artery. She was referred to our hospital for further examination and was diagnosed with jugular foramen schwannoma causing thrombosis of the sinuses. At the one-year follow-up, the tumor had grown rapidly and had started to surround the internal carotid artery. We therefore performed a tissue biopsy of the tumor in the jugular foramen and neck. Based on pathological analysis, we made a definitive diagnosis of malignant PEComa.
CONCLUSIONS
It may be extremely challenging to reach an accurate diagnosis of PEComa in the skull-base region, which can cause a delay in treatment initiation. When atypical clinical features for a skull-base tumor are found, we recommend preliminary biopsy to obtain a definitive diagnosis and initiate an appropriate treatment strategy as early as possible.
PubMed: 32042962
DOI: 10.1016/j.heliyon.2020.e03200 -
Acta Bio-medica : Atenei Parmensis Aug 2021Introduction A thorough understanding of skull base anatomy is imperative to perform safely and effectively any skull base approach. In this article, we examine the...
Introduction A thorough understanding of skull base anatomy is imperative to perform safely and effectively any skull base approach. In this article, we examine the microsurgical anatomy of the skull base by proposing a modular topographic organization in the median, paramedian, and lateral surgical corridors in relation to transcranial and endoscopic approaches. Methods Five dry skulls were studied focusing on the intracranial and exocranial skull base. Two lines were drawn parallel to the lateral border of the cribriform plate of the ethmoid bone and foramen lacerum, respectively. Lines 1 and 2 delimited the median, paramedian and lateral corridors of the skull base. The bony structures that formed each corridor were carefully reviewed in relation to the planning and execution of the skull base transcranial and endoscopic approaches. Results The midline corridor involves the crista galli, cribriform plate, planum and jugum sphenoidale, chiasmatic sulcus, tuberculum sellae, sellar region, dorsum sellae, clivus, and foramen magnum. The paramedian corridor includes the fovea ethmoidalis, the root of the lesser and greater sphenoid wing, anterior clinoid process, foramen lacerum, the upper half of the petro-occipital suture, and jugular tubercle. The lateral corridors include the orbital plates, sphenoid wings, squamosal and petrous parts of the temporal bone, caudal aspect of the petro-occipital suture, internal auditory canal, jugular foramen, the sulcus of the sigmoid sinus. Conclusion In-depth three-dimensional knowledge of skull base anatomy based on the modular concept of the surgical corridors is critical for the planning and execution of the transcranial and endoscopic approaches.
Topics: Cadaver; Humans; Orthopedic Procedures; Skull Base
PubMed: 34437364
DOI: 10.23750/abm.v92iS4.12115 -
Ear, Nose, & Throat Journal Sep 2023Paragangliomas are rare, slow-growing, hypervascular, catecholamine-secreting neuroendocrine tumors arising from the paraganglia. Paragangliomas are rarely found in the...
Paragangliomas are rare, slow-growing, hypervascular, catecholamine-secreting neuroendocrine tumors arising from the paraganglia. Paragangliomas are rarely found in the head and neck and are typically benign, presenting as a painless, slow-growing mass. Surgical extirpation in combination with long-term surveillance has been long regarded as the standard of care; however, the advances in imaging, radiation therapy, and embolization techniques have improved diagnostic and therapeutic modalities. We present a case of an 87-year-old female who had previously undergone resection of a paraganglioma in 1998, with no evidence of disease in 2002. Eighteen years later, the patient presented to the clinic with otogenic complaints. Imaging showed an expansive mass from the jugular foramen with bone destruction and opacification within the ear canal. The patient opted for observation. The patient eventually presented to the emergency room with neurologic manifestations. Imaging showed a cerebellar abscess prompting emergency drainage. Intraoperative cultures grew and , and the patient was started on 6 weeks of IV antibiotic therapy. Debulking of the paraganglioma was performed followed several months by mastoid and ear canal obliteration; however, the patient experienced complications, including dehiscence of the external auditory canal and infection. The patient was eventually treated successfully, marked by a reduction in complaints, a return to baseline activities, and imaging showing no increase in tumor size.
Topics: Female; Humans; Aged, 80 and over; Paraganglioma; Diagnostic Imaging; Head and Neck Neoplasms; Neck; Mastoid
PubMed: 37551648
DOI: 10.1177/01455613231187762 -
Journal of Neurological Surgery. Part... Feb 2021We present a case of a sizeable vagal schwannoma that was resected through a lateral suboccipital approach in semisitting position. An extra-axial lesion, occupying the...
We present a case of a sizeable vagal schwannoma that was resected through a lateral suboccipital approach in semisitting position. An extra-axial lesion, occupying the left cerebellomedullary cistern and extending from the pontomedullary junction to the jugular foramen was incidentally discovered in a 40-year-old woman afflicted with secondary progressive multiple sclerosis during repeated magnetic resonance imaging ( Fig. 1 ). On physical examination, a mild deviation of the uvula to the right and a diminished gag reflex were observed. The patient was referred to our department after considerable growth of the lesion was noted and a broad interdisciplinary consensus was reached to treat the lesion surgically. A gross total resection was achieved, histopathology confirmed a WHO I schwannoma with a low proliferation index. Postoperative dysphonia resolved completely within a few weeks, there was no collateral neurological deficit and especially no functional dysphagia. At 3-year follow-up, there was no indication of residual or recurrence. This 2-dimensional video demonstrates pre- and postoperative imaging, positioning and set-up of operating room, anatomical and surgical nuances of the skull base approach, and the operative technique for microdissection of the schwannoma from the critical neurovascular structures ( Fig. 2 ). In summary, the lateral suboccipital approach in semisitting position is a powerful tool in the armamentarium for the microsurgical management of various pathologies residing in the posterior cranial fossa, especially large and vascularized schwannomas. Provided the necessary anesthesiological precautions and intraoperative procedures the semisitting position is safe and effective. The link to the video can be found at: https://youtu.be/-9o_qJGkQhg .
PubMed: 33717819
DOI: 10.1055/s-0040-1701656 -
Cureus Jul 2022Collet-Sicard syndrome is a unilateral palsy of the lower cranial nerves IX, X, XI, and XII, resulting from lesions at the skull base that affect the jugular foramen...
Collet-Sicard syndrome is a unilateral palsy of the lower cranial nerves IX, X, XI, and XII, resulting from lesions at the skull base that affect the jugular foramen and hypoglossal canal. Common causes of the lesions include basilar skull fractures, carotid artery dissections, and malignancy. Infectious and inflammatory etiologies have also been reported. A 63-year-old male with a history of uncontrolled diabetes was admitted for dysphagia, right ear pain, drainage, and right-sided facial droop after recent local trauma and surgical instrumentation of the right ear. Culture of the external auditory canal grew . Triple phase bone scan demonstrated osteomyelitis at the skull base due to complications from otitis externa. The patient's presentation was consistent with Collet-Sicard syndrome, and he was subsequently treated with a six-week course of ciprofloxacin. This patient demonstrates a unique case since his malignant otitis externa spread locally and led to skull base osteomyelitis and subsequently developed Collet-Sicard syndrome. His uncontrolled diabetes likely played a role in his disease progression.
PubMed: 36035057
DOI: 10.7759/cureus.27218 -
BMJ Case Reports Aug 2020A 49-year-old man with chronic obstructive pulmonary disease was hospitalised due to pneumonia and pulmonary embolisms. After subsequently developing septic shock and...
A 49-year-old man with chronic obstructive pulmonary disease was hospitalised due to pneumonia and pulmonary embolisms. After subsequently developing septic shock and acute renal failure, he required dialysis. A haemodialysis catheter was planned inserted into the right subclavian vein, the guidewire was introduced using the Seldinger technique. When the guidewire's 20 cm marker entered the introducer needle, it suddenly encountered resistance. Repeated attempts to remove the guidewire failed. Vital signs and haemodynamic parameters remained unchanged throughout the procedure. CT angiography revealed cranial displacement of the wire into the right internal jugular vein, with the tip of the wire just cranial to the jugular foramen in the right sigmoid sinus. Interventional radiological removal attempts were unsuccessful. Thoracic and neurosurgical interventions were considered impossible and the guidewire was left in place. Due to the pulmonary embolism and the foreign object in the patient, life-long anticoagulation was considered, with close monitoring of compliance with the patient's comorbidity and medication.
Topics: Brain; Catheterization, Central Venous; Equipment Failure; Humans; Jugular Veins; Male; Middle Aged; Renal Dialysis; Subclavian Vein
PubMed: 32843444
DOI: 10.1136/bcr-2019-232535 -
Resection of a Lower Clival Meningioma via Posterolateral Approach: Two-Dimensional Operative Video.Journal of Neurological Surgery. Part... Feb 2021This study aimed to demonstrate resection of a craniovertebral junction (CVJ) meningioma via the posterolateral approach. The study is designed with a...
This study aimed to demonstrate resection of a craniovertebral junction (CVJ) meningioma via the posterolateral approach. The study is designed with a two-dimensional operative video. This study is conducted at department of neurosurgery in a university hospital. A 50-year-old woman who presented with lower cranial nerve findings due to a left-sided lower clival meningioma ( Fig. 1 ). Microsurgical resection of the meningioma and preservation of the neurovascular structures. The patient was placed in park-bench position and a left-sided retrosigmoid suboccipital craniotomy, followed by C1 hemilaminectomy and unroofing the lip of the foramen magnum, was performed. The dural incision extended from the suboccipital region down to the posterior arch of C2 ( Fig. 2 ). The arachnoid overlying the tumor was incised, revealing the course of the cranial nerve (CN) XI on the dorsolateral aspect of the tumor. The left vertebral artery (VA) was encased by the tumor which was originating from the dura below the jugular foramen. The mass was resected in a piecemeal fashion eventually. At the end of the procedure, all relevant cranial nerves and adjacent vascular structures were intact. Postoperative magnetic resonance imaging (MRI) confirmed total resection and the patient was discharged home on postoperative day 3 safely. Microsurgical resection of the lesions of the CVJ are challenging as this transition zone between the cranium and upper cervical spine has a complex anatomy. Since adequate exposure of the extradural and intradural segments of the VA can be obtained by the posterolateral approach, this approach can be preferred in cases with tumors anterior to the VA or when the artery is encased by the tumor. The link to the video can be found at: https://youtu.be/d3u5Qrc-zlM .
PubMed: 33717810
DOI: 10.1055/s-0040-1714407 -
Frontiers in Oncology 2021Complete resection of jugular foramen tumors with minimal cranial nerve complications remains challenging even for skilled neurosurgeons. Here, we introduce a modified...
OBJECTIVE
Complete resection of jugular foramen tumors with minimal cranial nerve complications remains challenging even for skilled neurosurgeons. Here, we introduce a modified paracondylar approach, named the suboccipital paracondylar-lateral cervical (SPCLC) approach for this purpose. We also share the follow-up data of our series and discuss the advantages and limitations of this modified paracondylar approach.
METHODS
We included 64 patients with jugular foramen tumors who underwent surgery by the same senior neurosurgeon between November 2011 and August 2020. All patients were treated with the SPCLC approach, which aimed for gross total tumor removal in a single-stage operation. The clinical characteristics, including preoperative and postoperative neurological status, the extent of surgical resection, and follow-up data were retrospectively acquired and evaluated.
RESULTS
There were 48 schwannomas, nine meningiomas, three paragangliomas, one hemangiopericytoma, one chordoma, one endolymphatic sac tumor, and one Langerhans' cell histiocytosis. The median age of our patients was 43 years (range: 21-77 years). Dysphagia, hoarseness, and tongue deviation were observed in 36, 26, and 28 patients, respectively. Thirty-two patients had hearing function impairments, including hearing loss or tinnitus. Gross total resection was achieved in 59 patients (59/64, 92.2%). Gamma Knife treatment was used to manage residual tumors in five patients. Postoperatively, new-onset or aggravative dysphagia and hoarseness occurred in 26 and 18 cases, respectively. Nine patients developed new-onset facial palsy, and one patient developed new-onset hearing loss. There were no cases of intracranial hematoma, re-operation, tracheostomy, or death. At the latest follow-up, hearing loss and tinnitus had improved in 20 cases (20/32, 62.5%), dysphagia alleviated in 20 cases (20/36, 55.6%), and hoarseness improved in 14 cases (14/26, 53.8%). Over a mean follow-up period of 27.8 ± 19.5 months (range: 3-68 months), tumor recurrence was observed in one patient.
CONCLUSION
The SPCLC approach, modified from the paracondylar approach, and was less invasive, safe, and efficient for certain jugular foramen tumors. Taking advantage of the anatomic understanding, clear operational vision, and appropriate surgical skills, it is possible to achieve gross total tumor removal and the preservation of neurological function.
PubMed: 34722234
DOI: 10.3389/fonc.2021.660487 -
Journal of Neurosurgery. Case Lessons Aug 2021Paragangliomas (PGLs) are rare neoplasms that may be associated with hereditary PGL syndromes and variable risk of metastasis. Middle ear adenomas are extremely rare...
Clinical and histopathological principles for the diagnosis of a recurrent paraganglioma of the jugular foramen initially diagnosed as a middle ear adenoma: illustrative case.
BACKGROUND
Paragangliomas (PGLs) are rare neoplasms that may be associated with hereditary PGL syndromes and variable risk of metastasis. Middle ear adenomas are extremely rare tumors with no known hereditary predisposition and extremely low risk of metastasis. Although often easily differentiated, they may share clinical and pathological features that misdirect and confuse the diagnosis.
OBSERVATIONS
The authors discussed a 35-year-old woman with left-sided hearing loss and bleeding from the external ear canal who presented to an outside hospital. She underwent resection of a middle ear and mastoid mass, initially diagnosed as a middle ear adenoma with neuroendocrine features, with later mastoidectomy and ligation of the sigmoid sinus with microsurgical excision of persistent tumor in the jugular foramen and temporal bone. Histopathologically, her tumor was vascular, composed of benign-appearing epithelioid cells with "salt and pepper" neuroendocrine chromatin arranged in vague nests. Lesional cells were GATA3-immunopositive, glucagon-negative, and succinate dehydrogenase-immunonegative, consistent with PGL rather than middle ear adenoma, and required further workup for hereditary PGL syndromes.
LESSONS
This case demonstrates potential challenges in differentiating a PGL from a middle ear adenoma. The authors offer clinical, histopathological, and imaging principles to aid in diagnosis and workup.
PubMed: 36131578
DOI: 10.3171/CASE21307 -
Interactive Cardiovascular and Thoracic... Jul 2022The course of COVID-19 patients may be complicated by thromboembolic events. We report on a 48-year-old female COVID-19 patient who underwent surgical removal of a large...
The course of COVID-19 patients may be complicated by thromboembolic events. We report on a 48-year-old female COVID-19 patient who underwent surgical removal of a large intracardiac thrombus. As per our centre protocol, critically ill COVID-19 patients are anticoagulated by the direct thrombin inhibitor Argatroban with close monitoring of anti-IIa activity. An intra-atrial thrombus formation fixed in a patent foramen ovale but also large mobile portions in both atria was diagnosed 4 days after weaning and removal of the jugular and femoral extracorporeal membrane oxygenation cannulas. The thrombus was removed surgically via median sternotomy and on cardiopulmonary bypass. The thrombus had a bizarre appearance with several finger-like appendices, and histological analysis revealed a mixed picture of acute and chronic thrombus portions. This case highlights the risk of life-threatening thrombus formation in COVID-19 patients despite therapeutic thrombin inhibition.
Topics: COVID-19; Female; Foramen Ovale, Patent; Heart Atria; Heart Diseases; Humans; Middle Aged; Pulmonary Embolism; Thrombosis
PubMed: 35373826
DOI: 10.1093/icvts/ivac077