Functional or non-secretory ectopic pituitary neuroendocrine tumors (PitNET) can form around the sella turcica during the development of the adenohypophysis by differentiating and detaching from the ...pharyngeal roof. These tumors usually appear in the sphenoid sinus, clivus, cavernous sinus, infundibulum, and suprasellar cistern. Ectopic PitNETs typically display the characteristic magnetic resonance imaging findings of pituitary adenomas. However, preoperative diagnosis of PitNETs is usually challenging because of the variety of clinical and imaging presentations, locations, and sizes. Ectopic suprasellar PitNETs resemble mass lesions in the pituitary stalk. Ectopic cavernous sinus of PitNETs are typically microadenomas in the medial wall. Ectopic sphenoclival tumors are characterized by more aggressive tumor activity than the other ectopic PitNETs. Although ectopic PitNETs are exceedingly rare, they should be considered as a differential diagnosis for masses around the sella turcica. Treatment of the disease should be individualized and may include medical care, surgical resection, gamma-knife radiosurgery, and radiotherapy.
Background
Tumors of the middle fossa or cavernous sinus (CS), or intraorbital tumors, can penetrate each other through the superior orbital fissure (SOF) or neighboring tissue. These complicated ...pathologies are often treated with highly invasive surgical procedures. In this article, we demonstrate surgical anatomic dissections of the CS, SOF, orbital apex (OA), and dura mater extending to the periorbita from the middle fossa, by performing an epidural dissection via a lateral orbitotomy approach, and discuss findings that may provide guidance during surgery in these regions.
Methods
Lateral orbitotomy was performed on latex-injected cadaver heads by making a 2-cm skin incision lateral to the lateral canthus, drilling the lesser and greater sphenoid wings that form the SOF borders, and removing the bone section between the middle fossa and orbit. Dura mater from the middle fossa to the periorbita was exposed to perform anterior clinoidectomy. Meningeal dura was dissected from the endosteal dura, which forms the lateral wall of the CS, to expose the CS, SOF, and OA for dissections.
Results
Changing the orientation of the microscope from posterior to anterior enabled regional control for dissection from the Gasserian ganglion to the OA. Cranial nerves that pass through the CS, SOF, and OA were dissected and exposed. The annular tendon was opened, revealing the oculomotor nerves and its branches, as well as the abducens and nasociliary nerves, which pass through the oculomotor foramen and course within the OA and orbit.
Conclusions
This approach causes less tissue damage; provides control of the surgical area in spheno-orbital tumors invading the fissure and foramen by changing the orientation of the microscope toward the orbit, OA, SOF, CS, and middle fossa; and expands the indication criteria for lateral orbitotomy surgery. This approach, therefore, represents an alternative surgical method for excising complicated tumors in these regions.
Aneurysmal bone cysts (ABCs) are defined as benign cystic lesions of bone composed of blood-filled spaces. ABCs may be a secondary pathology superimposed on fibrous dysplasia (FD). Concomitant FD and ...ABC in relation with McCune-Albright syndrome is an extremely rare condition. Here, we report concomitant, double ABCs in bilateral occipital regions and FD from the skull base to the C2 vertebra with Chiari type 1 malformation. A 14-year-old female with a diagnosis of McCune-Albright syndrome presented with swellings at the back of her head. The lesions were consistent with ABCs and were totally resected with reconstruction of the calvarial defects. The coexistence of FD, bilateral occipital ABCs, and Chiari malformation type 1 in a McCune-Albright patient is an extremely rare condition and, to our knowledge, has not been reported to date. Exact diagnosis and appropriate surgical treatment usually lead to a good outcome.
Chordoma is a rare malignant bone tumor with a poor outcome. Although radiotherapy and gamma knife surgery have been used for treatment, providing a cure for the tumor is not easy, because of the ...frequent recurrences. Molecular targeted therapy against tyrosine kinases has been effective in the treatment of malignancies such as breast and lung cancers and brain tumors. We aimed to analyse the histopathological features of chordomas and the immunoexpression profiles of the three receptor tyrosine kinases of EGFR, c-Met and c-Erb-B2 in chordomas. We have correlated these results with recurrence and overall survival status of the patients.
We studied 49 chordoma patients in order to evaluate the histopathological features and immunohistochemical stainings by EGFR, c-Met and c-ErbB2 antibodies. Of the 49 patients, follow up data was available for 40 patients. Clinical data of the patients were correlated with histopathological features and survival analysis was performed.
The immunostaining rate by EGFR and c-Met was 73.5% and 12.2% respectively. None of the cases showed immunoreactivity by c-ErbB2 (0%). Of the 40 cases, 17 cases showed recurrences. EGFR expression was detected in 14 recurrent (14/17) and 17 non-recurrent cases (17/23). Four of the 17 recurrent cases (4/17) were positive by c-Met, while none of the non-recurrent cases (0/23) were positive by this antibody. Significantly, all cases with positive c-Met expression showed recurrences (p < 0.05).
Our study indicates that EGFR expression is detected in the majority of chordoma cases. c-Met expression can be used as a prognostic indicator for chordoma.
Extra-axial hydatid disease (HD) is rare and may create a diagnostic challenge. Herein, we report an extremely rare case of calvarial HD with superior sagittal sinus invasion and an extension to the ...scalp with unusual magnetic resonance imaging findings simulating osteomyelitis and abscess. Recognition of detached endocystic membranes was the most important clue for the correct diagnosis. HD should be included in the differential diagnosis for a patient having a destructive skull lesion with peripheral enhancing extra-axial soft tissue component. Preoperative correct imaging diagnosis is crucial to improving patient management.
Abstract
Background
We and others have identified mutually exclusive molecular subgroups of meningiomas; however, the implications of this classification for clinical prognostication remain unclear. ...Integrated genomic and epigenomic analyses implicate unique oncogenic processes associated with each subgroup, suggesting the potential for divergent clinical courses. The aim of this study was to understand the associated clinical outcomes of each subgroup, as this could optimize treatment for patients.
Methods
We analyzed outcome data for 469 meningiomas of known molecular subgroup, including extent of resection, postoperative radiation, surveillance imaging, and time to recurrence, when applicable. Statistical relationships between outcome variables and subgroup were assessed. Features previously associated with recurrence were further investigated after stratification by subgroup. We used Kaplan–Meier analyses to compare progression-free survival, and identified factors significantly associated with recurrence using Cox proportional hazards modeling.
Results
Meningioma molecular subgroups exhibited divergent clinical courses at 2 years of follow-up, with several aggressive subgroups (NF2, PI3K, HH, tumor necrosis factor receptor–associated factor 7 TRAF7) recurring at an average rate of 22 times higher than others (KLF4, POLR2A, SMARCB1). PI3K-activated tumors recurred earlier than other subgroups but had intermediate long-term outcome. Among low-grade tumors, HH and TRAF7 meningiomas exhibited elevated recurrence compared with other subgroups. Recurrence of NF2 tumors was associated with male sex, high grade, and elevated Ki-67. Multivariate analysis identified molecular subgroup as an independent predictor of recurrence, along with grade and previous recurrence.
Conclusion
We describe distinct clinical outcomes and recurrence rates associated with meningioma molecular subgroups. Our findings emphasize the importance of genomic characterization to guide postoperative management decisions for meningiomas.
There are many kinds of extra-axial brain tumors and tumor-like lesions, and definitive diagnosis is complicated in some cases. In this pictorial essay, we present rare and challenging extra-axial ...brain lesions including neuroenteric cyst, primary leptomeningeal melanomatosis, isolated dural neurosarcoidosis, intradiploic epidermoid cyst, ruptured dermoid cyst, intraventricular cavernoma, and cavernous hemangioma of the skull with imaging findings and clinico-radiological differential diagnosis, including the pathologic correlation. Familiarity with these entities may improve diagnostic accuracy and patient management.
Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for ...syringomyelia secondary to Chiari's malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.
Background
Low-grade gliomas have good overall survival rates in pediatric patients compared to adults. There are some case series that reported the effectiveness and safety of Gamma Knife ...radiosurgery, yet they are limited in number of patients. We aimed to review the relevant literature for pediatric low-grade glial tumors treated with stereotactic radiosurgery, specifically Gamma Knife radiosurgery, and to present an exemplary case.
Case description
A 6-year-old boy was admitted to clinic due to head trauma. He was alert, cooperative, and had no obvious motor or sensorial deficit. A head CT scan depicted a hypodense zone at the right caudate nucleus. The brain magnetic resonance imaging (MRI) depicted a mass lesion at the same location. A stereotactic biopsy was performed. Histopathological diagnosis was low-grade astrocytoma (grade II, World Health Organization (WHO) classification, 2007). Gamma Knife radiosurgery was applied to the tumor bed. Tumor volume was 21.85 cm
3
. Fourteen gray was given to 50 % isodose segment of the lesion (maximal dose of 28 Gy). The tumor has disappeared totally in 4 months, and the patient was tumor-free 21 months after the initial treatment.
Discussion and conclusion
The presented literature review represents mostly single-center experiences with different patient and treatment characteristics. Accordingly, a mean/median margin dose of 11.3–15 Gy with Gamma Knife radiosurgery (GKRS) is successful in treatment of pediatric and adult low-grade glial tumor patients. However, prospective studies with a large cohort of pediatric patients should be conducted to make a more comprehensive conclusion for effectiveness and safety of GKRS in pediatric low-grade glial tumors.
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