Myopericytoma at the Craniocervical Junction: Clinicopathological Report and Review of a Rare Perivascular Neoplasm

Neurosurgery ◽  
2018 ◽  
Vol 85 (2) ◽  
pp. E360-E365 ◽  
Author(s):  
Jimmy C Yang ◽  
Andrew S Venteicher ◽  
Matthew J Koch ◽  
Christopher J Stapleton ◽  
Gabriel N Friedman ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Myopericytoma is an emerging class of neoplasm originating from the perivascular myoid cellular environment, previously classified as a variant of hemangiopericytoma. Most reported myopericytomas are found in soft tissues of the extremities; however, infrequent cases are described involving the central nervous system. Intracranial myopericytoma remains rare. Here, we describe the first report of myopericytoma occurring at the cervicomedullary junction in close proximity to the vertebral artery, mimicking a vascular lesion. CLINICAL PRESENTATION A 64-yr-old woman presented with radiating neck pain. Magnetic resonance imaging revealed a well-circumscribed enhancing lesion adjacent to the vertebral artery-accessory nerve complex. She underwent a far lateral craniotomy and cervical laminectomy to obtain proximal vertebral artery control and adequate exposure of the lesion, which appeared most consistent with neoplasm at surgery. Histopathology revealed a grade I myopericytoma. A gross total resection was achieved, and the patient has no evidence of recurrence 3 yr after surgery. CONCLUSION Tumors of perivascular origin include hemangiopericytoma, glomus tumor, myofibroma, and myopericytoma and are uncommon lesions intracranially. Consideration of and distinction among these perivascular tumors is critically important, as they each have distinct clinical behaviors and management. Myopericytoma can mimic other neoplastic and cerebrovascular pathologies, but it most commonly has a benign course and can be surgically cured if a gross total resection can be achieved. Rarer myopericytoma variants that adopt a more malignant course have been described, and ongoing molecular studies may identify mutations or activated signaling pathways that can be targeted to offer chemotherapeutic options in the future.

2021 ◽  
pp. 1-12
Author(s):  
Arianna Fava ◽  
Paolo di Russo ◽  
Valentina Tardivo ◽  
Thibault Passeri ◽  
Breno Câmara ◽  
...  

OBJECTIVE Craniocervical junction (CCJ) chordomas are a neurosurgical challenge because of their deep localization, lateral extension, bone destruction, and tight relationship with the vertebral artery and lower cranial nerves. In this study, the authors present their surgical experience with the endoscope-assisted far-lateral transcondylar approach (EA-FLTA) for the treatment of CCJ chordomas, highlighting the advantages of this corridor and the integration of the endoscope to reach the anterior aspect and contralateral side of the CCJ and the possibility of performing occipitocervical fusion (OCF) during the same stage of surgery. METHODS Nine consecutive cases of CCJ chordomas treated with the EA-FLTA between 2013 and 2020 were retrospectively reviewed. Preoperative characteristics, surgical technique, postoperative results, and clinical outcome were analyzed. A cadaveric dissection was also performed to clarify the anatomical landmarks. RESULTS The male/female ratio was 1.25, and the median age was 36 years (range 14–53 years). In 6 patients (66.7%), the lesion showed a bilateral extension, and 7 patients (77.8%) had an intradural extension. The vertebral artery was encased in 5 patients. Gross-total resection was achieved in 5 patients (55.6%), near-total resection in 3 (33.3%), and subtotal resection 1 (11.1%). In 5 cases, the OCF was performed in the same stage after tumor removal. Neither approach-related complications nor complications related to tumor resection occurred. During follow-up (median 18 months, range 5–48 months), 1 patient, who had already undergone treatment and radiotherapy at another institution and had an aggressive tumor (Ki-67 index of 20%), showed tumor recurrence at 12 months. CONCLUSIONS The EA-FLTA provides a safe and effective corridor to resect extensive and complex CCJ chordomas, allowing the surgeon to reach the anterior, lateral, and posterior portions of the tumor, and to treat CCJ instability in a single stage.


2019 ◽  
Vol 80 (S 04) ◽  
pp. S355-S357
Author(s):  
Robert T. Wicks ◽  
Xiaochun Zhao ◽  
Celene B. Mulholland ◽  
Peter Nakaji

Abstract Objective Foramen magnum meningiomas present a formidable challenge to resection due to frequent involvement of the lower cranial nerves and vertebrobasilar circulation. The video shows the use of a far lateral craniotomy to resect a foramen magnum meningioma. Design, Setting, and Participant A 49-year-old woman presented with neck pain and was found to have a large foramen magnum meningioma (Fig. 1A, B). Drilling of the posterior occipital condyle was required to gain access to the lateral aspect of the brain stem. The amount of occipital condyle resection varies by patient and pathology. Outcome/Result Maximal total resection of the tumor was achieved (Fig. 1B, C), and the patient was discharged on postoperative day 4 with no neurologic deficits. The technique for tumor microdissection (Fig. 2) is shown in the video. Conclusion Given the close proximity of foramen magnum meningiomas to vital structures at the craniocervical junction, surgical resection with careful microdissection and preservation of the overlying dura to prevent postoperative pseudomeningocele is necessary to successfully manage this pathology in those patients who are surgical candidates.The link to the video can be found at: https://youtu.be/Mds9N1x2zE0.


2013 ◽  
Vol 12 (3) ◽  
pp. 274-280 ◽  
Author(s):  
Smruti K. Patel ◽  
James K. Liu

Neurenteric cysts are rare and benign lesions that consist of ectopic alimentary tissue residing in the central nervous system. They tend to occur most frequently in an intraspinal rather than intracranial location. Intracranial neurenteric cysts are a rare occurrence in the pediatric population. These lesions typically present as unilateral cystic structures in the lower cerebellopontine angle and craniocervical junction. To the authors' knowledge, there have been no reported cases of bilateral localization of intracranial neurenteric cysts. In this report, they present an unusual case of a 10-year-old girl who was found to have bilateral intracranial neurenteric cysts at the pontomedullary junction. The patient was successfully treated with staged, bilateral far-lateral transcondylar resection of the cysts. The authors also provide a brief overview of the literature describing intracranial neurenteric cysts in children.


2019 ◽  
Vol 80 (S 04) ◽  
pp. S349-S351
Author(s):  
Alexander X. Tai ◽  
Kathleen Knudson ◽  
Walter C. Jean

AbstractWe present a case in which a retrocondylar far-lateral approach was utilized to resect a hemangioblastoma at the craniocervical junction. The patient was a 33-year-old man presenting with 2 months of symptoms referable to compression at the craniocervical junction (i.e., dizziness and gait instability). Though neurologically intact on exam, his imaging demonstrated a highly vascular cystic lesion dorsolateral to the medulla on the left consistent with a hemangioblastoma. Virtual reality software was critical to visualize the patient's lesion in relationship to the vertebral artery and draining vein (Fig. 1). A far-lateral retrocondylar suboccipital craniectomy with a C1 hemilamiectomy permitted resection of this lesion. The operative strategy was to address the lesion similar to an arteriovenous malformation by detaching the lesion from its arterial feeders first, and then addressing the lesion's draining vein (Fig. 2). Postoperative imaging demonstrated a gross-total resection and the patient had an uncomplicated postoperative course. This case demonstrates not only the lateral reach of a retrocondylar far-lateral approach; but also, clearly demonstrates an effective dissection strategy when approaching a hemangioblastoma.The link to the video can be found at: https://youtu.be/M0szMOdhjfE.


2019 ◽  
Vol 80 (S 04) ◽  
pp. S360-S362
Author(s):  
Sima Sayyahmelli ◽  
Mustafa K. Başkaya

Foramen magnum meningiomas are one of the most challenging tumors for skull base neurosurgeons due to their proximity to critical neurovascular structures. The far-lateral approach demonstrated here facilitates access to lesions involving the anterior portion of the foramen magnum.In this video, we present a 62-year-old woman with hand numbness and weakness. The patient had significant difficulty in fine motor movements of both hands. In the neurological examination, she had a significant right-hand intrinsic muscle weakness and mild quadriparesis.Magnetic resonance imaging (MRI) showed a dural-based homogeneously enhancing extra-axial mass in the anterior foramen magnum with a significant mass effect on the brain stem and the upper cervical cord. The decision was made to proceed with a far lateral transcondylar skull base approach including partial C1 laminectomy.The surgery and postoperative course were uneventful. The postoperative MRI showed gross total resection of the mass. The histopathology indicated a WHO (World health Organization) grade-I meningioma. The patient's postoperative course was uneventful. She improved to normal neurological function within several weeks and continues to do well without recurrence at 20 months' follow-up.In this video, we demonstrated important steps for the microsurgical resection of these challenging lesions.The link to the video can be found at: https://youtu.be/_nuX2Y7YU9w.


2019 ◽  
Vol 80 (S 04) ◽  
pp. S363-S364
Author(s):  
Ciro Vasquez ◽  
Alexander Yang ◽  
A. Samy Youssef

We present a case of a foramen magnum meningioma in a 42-year-old female who presented with headaches for 2 years, associated with decreased sensation and overall feeling of “heaviness” of the right arm. The tumor posed significant mass effect on the brainstem, and given the location of the tumor in the anterolateral region of the foramen magnum, a right far lateral approach was chosen. The approach incorporates the interfascial dissection technique to safely expose and preserve the vertebral artery in the suboccipital triangle. After drilling the posteromedial portion of the occipital condyle and opening the dura, the tumor can be entirely exposed with minimal retraction on the cerebellum. The working space offered by the far lateral approach allows careful dissection at the lateral craniocervical junction, and preservation of the V4 segment of the vertebral artery and the lower cranial nerves. Simpson's grade-2 resection was achieved with coagulation of the dural base around the vertebral artery. The postoperative course was unremarkable for any neurological deficits. At the 2-year follow-up, imaging identified no recurrence of tumor and the patient remains asymptomatic.The link to the video can be found at: https://youtu.be/IMN1O7vO5B0.


2019 ◽  
Vol 80 (S 04) ◽  
pp. S358-S359
Author(s):  
Yong Yan ◽  
Hongxiang Wang ◽  
Tao Xu ◽  
Zhenyu Gong ◽  
Fan Hong ◽  
...  

Tumors located in the craniocervical junction region are significantly challenging for surgical resection. We shared our experience of a meningioma at craniocervical junction resected through far lateral approach in a 68-year-old female. The patient presented with intermittent headache with discomfort in the neck and shoulders for 3 years without any positive signs. Magnetic resonance imaging (MRI) revealed a tumor of 3.6 cm × 3.0 cm × 2.5 cm lying at the ventral side of medulla oblongata, with T1 hypointensity, T2 hyperintensity, and a significant enhancement on T1-contrast image. The far lateral approach on the right side was planned to resect the tumor with a park-bench position. The patient underwent a standard craniotomy using a lazy S-shaped incision. The transposition of vertebral artery was performed carefully therein, followed by removal of part of the arches of atlas and axis. After exposure of the tumor, vertebral artery (VA) and posterior inferior cerebellar artery (PICA) adhesive to the lesion could be seen operatively. Truncating the supplying blood vessels of the tumor was taken as the first step, followed by resecting the tumor mass in a piecemeal manner. While preserving VA, PICA, posterior nerves, medulla oblongata, and cervical cord, gross-total resection was achieved under the careful operation. The patient tolerated the procedure well without any neurological deficits. Histological examination confirmed the tumor as a meningioma (World Health Organization [WHO] grade I). Postoperative MRI scan depicted complete resection of the tumor. The patient remained symptom free without any evidence of recurrence during the follow-up period of 1 year. Informed consent was obtained from the patient.The link to the video can be found at: https://youtu.be/i9H-wS4fF10.


2009 ◽  
Vol 11 (1) ◽  
pp. 84-87 ◽  
Author(s):  
R. Shane Tubbs ◽  
Nemil A. Shah ◽  
Brian P. Sullivan ◽  
Nicholas D. Marchase ◽  
Aaron A. Cohen-Gadol

Object The vertebral artery (VA) and its branches may be encountered during various neurosurgical procedures such as far lateral suboccipital approaches to the skull base and spinal operations. Therefore, a working knowledge of the distribution and significance of such VA branches may be advantageous to the surgeon. To date, quantitation of these branches is lacking in the literature. Methods The authors evaluated the branches of 20 VAs from 10 adult cadavers and assessed the distribution and surgical significance of the branches from the V2 and V3 segments. Results In terms of target tissues, the VA branches encountered at the C1–2 level were most likely to be muscular, branches at C2–3 osseous, and those at C3–6 radicular. No radicular or medullary branches were identified arising from any V3 segment of the VA or C1–2 level of the V2 segment. The greatest concentration of branches per level was found arising from the V2 segment at C2–3. Posterior branches of the VA tended to be radicular or muscular, whereas anterior branches tended to be radicular or osseous. Lateral branches were most commonly radicular and medial branches tended to be osseous or muscular in nature. The largest branches of the VA originated from its V3 segment or the C2–3 part of its V2 segment. Rarely, branches to the extracranial glossopharyngeal and spinal accessory nerves were identified originating from the V3 and V2 segments of the VA, respectively. Conclusions Although seemingly diverse in their distribution, the branches of the V2 and V3 segments of the VA may follow a certain consistent arrangement. The potential for injury to neural branches of the VA is minimal at its V3 segment and the C1–2 portion of its V2 segment. Such knowledge may be of use to the neurosurgeon who operates in the neck and at the craniocervical junction.


2020 ◽  
Vol 11 ◽  
pp. 200
Author(s):  
Ricardo Malcata Nogueira ◽  
Luis Santos Cardoso ◽  
Lino Fonseca ◽  
Pedro Branco ◽  
Miguel Correia ◽  
...  

Background: Melanoma is the third most common primary tumor to metastasize to the central nervous system (CNS). However, primary CNS melanoma is very rare, and primary intramedullary melanoma is even less frequently encountered, with only 27 cases published in the literature. There are no pathognomonic imaging characteristics, therefore, the diagnosis must be confirmed immunohistologically and the preferred treatment is the gross total resection. Case Description: A 68-year-old male presented with low back pain of 2 months duration, and 1 week of urinary retention/anal sphincter incontinence. The neurologic examination revealed bilateral paraparesis (3/5 level) with bilateral Babinski signs, and a T10–T11 pin level. The lumbar CT-Scan showed a hyperdense intramedullary tumor arising from the conus medullaris. The patient underwent a D12–L2 laminectomy with myelotomy for gross-total tumor resection. Postoperatively, he regained motor function but the urinary incontinence remained unchanged. The diagnosis of a primary malignant melanoma was confirmed both histopathologically and immunohistochemically (e.g., staining revealed positive immunoreactivity for S100 protein and Melan A). Conclusions: Primary intramedullary spinal melanoma is very rare, and the diagnosis must be biopsy/operatively confirmed. Whether gross total resection is feasible depends on the extent of tumor infiltration of the cord/ adherence as well as the potential for clinical deterioration with overly aggressive removal.


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