Adenoid Cystic Carcinoma.

BackgroundSinonasal adenoid cystic carcinoma is a rare malignancy of the head and neck. Cavernous sinus invasion from sinonasal adenoid cystic carcinoma and its related management have rarely been investigated. This study evaluated the relationship between treatment outcome and cavernous sinus involvement in addition to other parameters.MethodsA retrospective case series study was conducted at a tertiary referral center. The medical records of 47 patients diagnosed with primary sinonasal adenoid cystic carcinoma between 1984 and 2015 were retrospectively reviewed. The survival impact of the primary treatment modalities and the anatomic sites of tumor involvement were analyzed.ResultsCavernous sinus invasion was observed in 8 patients (17%), of whom 7 had ACC tumors originating from the maxillary sinus. The results of univariate analysis revealed that tumor stage, primary surgery, and the absence of skull-base and infratemporal fossa invasion were associated with better overall survival (P = 0.033, P = 0.012, P = 0.011, and P = 0.040, respectively) and better disease-free survival (P = 0.019, P = 0.001, P = 0.017, and P = 0.029, respectively). Multivariate analysis identified primary surgery as the only independent prognostic factor for disease-free survival (P = 0.026). Cavernous sinus invasion by sinonasal adenoid cystic carcinoma was not associated with worse overall survival or disease-free survival (P = 0.200 and P = 0.198, respectively).ConclusionsBecause maxillary adenoid cystic carcinoma is associated with a higher rate of cavernous sinus invasion, such cases warrant caution during preoperative planning. Primary surgery as the initial therapy provides better locoregional control and survival for patients with sinonasal adenoid cystic carcinoma. Cavernous sinus invasion did not significantly impact survival; thus, it should not be regarded as a contraindication for curative treatment.

Sinonasal adenoid cystic carcinoma (SNACC) is a rare malignancy that most commonly arises in the maxillary sinus. Characteristics of SNACC are slow growth, perineural invasion, and long clinical course. Because it is a rare tumor, population-based studies are limited. We analyzed the incidence and survival for SNACC using a national population-based database. Retrospective cohort study using national cancer database. The United States National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry was utilized to calculate incidence and survival trends for SNACC between 1973 and 2009. Patient data were then analyzed according to age, sex, and race. Incidence trends were studied for the last 30 years, and survival outcomes were compared across the different demographic parameters. A total of 412 cases of SNACC were identified (57.52% female). Incidence trend analysis revealed a significant decrease in yearly rates from 1973 to 2009 for the overall population, females, whites, blacks, and "others." Overall 5-year survival for SNACC was 68.80%, 10-year survival was 48.03%, and 20-year survival was 22.39%. Significant differences in survival outcomes were noted between whites, blacks, and "others." "Others" had the best 20-year survival outcomes. The overall incidence of SNACC is declining. Sex and race seem to influence the overall survival for this rare tumor. Future studies need to be conducted to investigate these dynamic trends related to SNACC.

Adenoid cystic carcinoma (ACC) is a rare tumor, contributing to less than 1% of head and neck malignancies. It involves the salivary gland, nose, and paranasal sinus. Sinonasal malignancies constitute 1–2% of all malignancies. Out of all sinonasal malignancies, sinonasal adenoid cystic carcinoma (SNACC) is the third most common SNACC and is a slow-growing tumor that manifests in later stages. SNACC can extend into intracranial and intradural space, therefore it requires surgical treatment and radiotherapy. The prognosis of SNACC entirely depends on the histological subtype of the tumor and available treatment options. This case report is presented due to its rare histopathological findings.

We present the case of a 51-year-old patient with sinonasal adenoid cystic carcinoma (SACC). Computed tomography (CT) and magnetic resonance imaging (MRI) exams revealed an expansive process in the right nostril accompanied with perineural spread and invasion to the floor of the middle cranial fossa. Due to the size of the tumor and brain involvement, the Head and Neck Tumor Board (HNTB) recommended radiochemotherapy treatment to decrease the size of the lesion. Presently, the patient is undergoing treatment without major complications.

Objective: To analyze the efficacy of sinonasal adenoid cystic carcinoma (ACC) with perineural invasion (PNI), and explore the prognostic value of PNI on sinonasal adenoid cystic carcinoma. Methods: The clinical data of 105 patients with sinonasal ACC admitted to Cancer Hospital, Chinese Academy of Medical Sciences from January 2000 to December 2016 were retrospectively reviewed. All patients were restaged according to American Joint Committee on Cancer 8th edition. Follow-up visits were conducted to obtain information of treatment failure and survival outcome. The Log rank test was used for univariate analysis of prognostic factors, and Cox regression model was used for multivariate prognostic analysis. Results: The maxillary sinus (n=59) was the most common primary site, followed by the nasal cavity (n=38). There were 93 patients with stage Ⅲ-Ⅳ. The treatment modalities included surgery alone (n=14), radiotherapy alone (n=13), preoperative radiotherapy plus surgery (n=10), and surgery plus postoperative radiotherapy (n=68). The median follow-up time was 91.8 months, the 5-year local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) rates were 72.6%, 73.0%, 52.9% and 78.0%, respectively. There were 33 patients (31.4%) with PNI-positive. The 5-year DMFS, PFS, and OS rates of PNI-positive group were 53.7%, 29.4% and 56.5%, respectively, which were significantly inferior to those of PNI-negative group (80.8%, 63.0% and 86.8%, respectively, P<0.05), while there was no significant difference in the 5-year LC rate between both groups (64.5% vs 76.5%, P=0.273). The multivariate Cox regression analysis showed PNI was one of the poor prognostic factors of DMFS (HR=3.514, 95%CI: 1.557-7.932), PFS (HR=2.562, 95%CI: 1.349-4.866) and OS (HR=2.605, 95%CI: 1.169-5.806). Among patients with PNI-positive, the 5-year LC, PFS and OS rates of patients received surgery combined with radiotherapy were 84.9%, 41.3% and 72.7%, respectively, which were significantly higher than 23.3%, 10.0% and 26.7% of patients receiving surgery or radiotherapy alone (P<0.05). Conclusion: The presence of PNI increases the risk of distant metastasis in patients with sinonasal ACC. Compared with patients with PNI-negative, the prognosis of patients with PNI-positive is relatively poor, and surgery combined with radiotherapy for PNI-positive sinonasal ACC results in good clinical outcomes.

Malignant sinonasal tumors are clinically challenging due to their proximity to vital structures and their diverse histogenesis and biological behavior. To date, no animal models accurately reflect the clinical behavior of these malignancies. We developed an orthotopic murine model of sinonasal malignancy that reproduces the intracranial extension, bony destruction, and spread along neural fascial planes seen in patients with aggressive sinonasal malignancies of various histologies. Human squamous cell carcinoma line (DM14) and adenoid cystic carcinoma line (ACC-3) were implanted in the right maxillary sinus or soft palate in male nude mice. Animals were monitored for tumor growth and survival. Tumor specimens were removed for histopathologic evaluation to assess for intracranial extension, orbital invasion, bony invasion, perineural invasion, and distant metastasis. Statistical analysis was done to calculate P values with the Student's t test for individual tumor volumes. Differences in survival times were assessed using the log-rank test. Mice with DM14 or ACC-3 implanted in either the maxillary sinus or the soft palate developed large primary tumors. A statistically significant inverse correlation between survival and the number of tumor cells implanted was found. Histopathologic evaluation revealed orbital invasion, intracranial extension, pulmonary metastasis, lymph node metastasis, and perineural invasion. We describe the first orthotopic model for sinonasal malignancy. Our model faithfully recapitulates the phenotype and malignant behavior of the aggressive tumor types seen in patients. This model offers an opportunity to identify and specifically target the aberrant molecular mechanisms underlying this heterogeneous group of malignancies.

Image-guided resection of small lesions in the cavernous sinus and Meckel's cave

Objective The microsurgical resection of tumors or vascular lesions in the cavernous sinus and the neighbouring Meckel's cave has been considered as hazardous because of often associated cranial nerve morbidity. Despite increasing consent that many of such tumors should not undergo surgical therapy, the cavernous sinus and Meckel's cave may harbour small lesions of various origin, which are amenable for surgical resection. Surgery in this anatomical area needs a well directed approach. In this setting, neuronavigational guidance may provide a useful tool. We report on a series of patients operated on and guided by neuronavigation. Methods Five patients underwent a pterional approach for microsurgical resection. The procedures were planned and assisted by a pointer based neuronavigation system (Medtronic Stealth Station). Pathological entities included schwannoma, epidermoid, cavernoma and capillary hemangioma. Three lesions were located in the Meckel's cave and two lesions in the cavernous sinus. Results Intraoperative guidance by neuronavigation appeared to be particularly instrumental in identification of the appropriate site of dural incision over the target region for microsurgical resection. Except of a mild increase of facial hypesthesia in one patient, there were no new cranial nerve deficits. In three patients, preoperative symptoms improved immediately after surgery. Conclusion The surgical resection of small tumors or vascular lesions within the Meckel's cave or cavernous sinus is facilitated by neuronavigational guidance with sufficient intraoperative reliability and safety. In consideration of well known anatomical landmarks, targeted entry into the cavernous sinus or Meckel's cave directed by neuronavigation enables a tailored approach for microsurgical resection.

Introduction Sinonasal cancer is very rare and accounts for under 1% of all malignancies of which sinonasal adenoid cystic carcinoma (SNACC) is about 5%. The maxillary sinus is the most common site of SNACC. Misleading symptomatology which overlaps with rhinosinusitis contributes to the delay of diagnosis. Recently, the endoscopic approach is commonly applied with equal or better results to the open approach. Radiofrequency coblation might be used to reduce blood loss. Material and Methods We present a case of an adenoid cystic carcinoma arising from the left inferior turbinate. The patient received endoscopic surgical treatment with the application of radiofrequency coblation in Clarós Clinic at the beginning of 2019 and remains followed up with no sign of recurrence. Afterward, we review the literature on the subject. Conclusion SnACC is a rare malignancy, characterized by slow growth and perineural infiltration with spreading towards the skull base, cavernous sinus, and orbit. The regional nodal involvement is rare. Frequent and delayed distant metastasis in lung, bone, liver, and brain are also typical. Prognosis is poor with the 5-year overall survival between 41%-86%. Surgery with or without complementary radiotherapy provides the best outcomes for the majority of patients. The significance of prognostic factors remains unclear. Lifelong surveillance is obligatory as very late recurrences are common.

A 74-year-old woman who presented initially with trigeminal neuralgia of the left forehead and scalp was later found to have a poorly differentiated squamous cell carcinoma (SCC) with large-nerve perineural and intraneural invasion of the left supraorbital nerve. Negative histopathologic margins were achieved in three stages of permanent fixed tissue en face processing and the final defect was repaired with a large rotation flap. Approximately one month after repair, the patient presented with new-onset diplopia and was found to have a complete left cranial nerve VI palsy suspicious for continued disease spread. MRI confirmed perineural spread along the ophthalmic branch of the trigeminal nerve through the superior orbital fissure into the cavernous sinus. She was subsequently treated with radiation therapy (66Gy in 33 fractions). The involvement of two distinct cranial nerves by perineural invasion is uncommon and has mostly been described involving branches of the trigeminal and facial nerves. This case highlights the rare presentation of perineural invasion involving both the trigeminal nerve and the abducens nerve. Anatomically, this clinical presentation can be explained by the retrograde perineural spread along the ophthalmic branch of the trigeminal nerve through the supraorbital fossa into the cavernous sinus where these two nerves are in close proximity.

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Petroclival meningiomas are vaguely defined as tumours arising from the antero-medial zone to the internal auditory meatus. This report subclassifies petroclival meningiomas based on their origin determined by using radiological and intra-operative findings. Ninety-one patients with petroclival meningioma underwent surgery via the anterior transpetrosal approach. The Meckel's cave was routinely opened. Tumour origin was classified into four subtypes according to the main attachment and trigeminal nerve deviation into, upper clivus (UC), cavernous sinus (CS), tentorium (TE), and petrous apex (PA). Their characteristic clinical symptoms and anatomical features were investigated. The characteristic symptom was ataxia in the UC type (37.5%), abducens nerve palsy in the CS type (64.3%) and trigeminal neuropathy, mainly neuralgia in the PA type (80.0%) with a higher statistical difference from other subtypes. The rate of tumour invasion into Meckel's cave reached 70.3% in average, with the lowest rate in the PA type (25.0%). The rate of middle fossa extension was the highest in the TE type (59.5%). The middle fossa approach was considered to be ideal for UC and TE types because of easier access to the Meckel's cave. Radical dissection without complications was difficult in the CS type. Both the anterior transpetrosal approach and the lateral suboccipital approach could be indicated in the PA type due to the rare invasion of Meckel's cave and middle fossa, and frequent extension into the internal auditory meatus. This classification is useful to predict the relation between the tumour and the cranial nerves based on symptoms and images. The anterior transpetrosal approach could be used for all four subtypes and with an absolute indication in the UC and TE types showing middle fossa extension.

Cavernous sinus syndrome associated with metastatic colorectal cancer and perineural spread along the trigeminal nerve

Background Imaging the trigeminal nerve (TN) presents significant challenges due to its extensive pathway from the brainstem nuclei to its peripheral branches, as well as its complex anastomotic network either through its numerous branches or connections with the facial nerve, which often complicates a topographic approach. Various pathologic conditions may affect each segment of the trigeminal nerve, MRI is the preferred imaging technique for evaluating the majority of conditions that involve various segments of the trigeminal nerve. The majority of the literature surrounding the trigeminal nerve although encompasses a variety of perspectives, but it is focusing on trigeminal nerve involvement in perineural spread from locoregional malignancies and neurovascular compression conflicts. This research manuscript is meant to concentrate on the less familiar trigeminal nerve lesions, escalating perineural sheath of segments distal to the porus trigeminus; those encompassing Meckel’s cave and the cavernous sinus, extending to its peripheral branches, while excluding the preganglionic and brainstem segments. Patients and methods The purpose of our current research is that we can report herein our experience in imaging of different trigeminal nerve related masses, starting from distal to porus trigeminus, so we can separate head and neck/skull base from scope of neuro-radiology. Also, we termed them “non-vascular” to exclude vascular loops, cavernous sinus thrombosis and cortico-cavernous fistulas. This retrospective case study aimed to collect patients’ data from the Archives of radiology department, from January 2022 to January 2024 including MRI imaging data and final pathology results, which were extracted from patients primarily referred for imaging from the “oncology,” “neuro-surgery” and “oto-rhino-laryngology” ward. The “Ethics Committee” of our medical institution approved our proposed protocol. Results Our study had enrolled 48 cases: 24 men and 24 women, with ages ranging between 12 and 79 years, showing a mean of 43 years. The predominant pathology was schwannomas (n = 15), followed by inflammatory pseudotumor/ IGg4 disease ( n = 11), followed by meningioma ( n = 9), followed by metastases ( n = 7). The Cavernous sinus (CS) was the prevalent epicenter location of pathologies ( n = 34; 70% of cases), followed by Meckel’s cave (MC), and Masticator space (MS) and PPF-pterygo-palatine fossa ( n = 3 for each), followed by orbit and orbital apex (sites of V1). Conclusions Perineural spread along the TN is not limited to PNTS from locoregional head and neck malignancies and has more wide differential diagnosis, and differential list includes quite less common lesions. These unfamiliar lesions of the TN involve the nerve in a similar perineural pattern and yet imaging features are overlapping with common PNTS from local malignancies, making optimal diagnosis a difficult task without pathology confirmation. However, radiologists should be aware of such uncommon lesions which should be included in differential diagnosis of PNTS.

The goal of the current study is to describe the transnasal endoscopic anatomy of the cavernous sinus and to provide the surgical approaches to this area. Six silicon-injected adult cadaveric heads (12 sides) were dissected through endoscopic endonasal approach. The cavernous sinus and adjacent structures were exposed; detailed anatomies were demonstrated. High-quality pictures were produced. The cavernous sinus had four walls, namely medial, lateral, posterior and superior walls. Five venous spaces within the sinus were identified by their relation to the carotid artery; those were the medial, lateral, posterosuperior, posteroinferior and anterolateral compartments. Three branches arising from the cavernous segment of internal carotid artery from proximal to distal were meningohypophyseal trunk, inferolateral trunk and McConnell capsular artery. Cavernous sinuses communicated each other by intercavernous sinuses, as well as basilar sinus in middle line, and connected with superior and inferior petrosal sinuses. The third and fourth nerves coursed in superior part of the lateral wall of the cavernous sinus; Meckel's cave located in the posteroinferior part of the lateral wall of the cavernous sinus; V1 sloped to the superior orbital fissure along the lateral wall; the sixth nerve entered the posteroinferior compartment then passed through the internal carotid artery and reached to superior orbital fissure. The approaches to the cavernous sinus included trans-sphenoid-sellar-medial cavernous sinus (medial to the internal carotid artery) and trans-ethmoid-pterygoid-sphenoid-lateral cavernous sinus (lateral to the internal carotid artery). Trans-sphenoid-sellar-medial cavernous sinus approach was able to expose medial compartment and posterosuperior compartment and part of posteroinferior compartment. Trans-ethmoid-pterygoid-sphenoid-lateral cavernous sinus approach was able to expose anteroinferior compartment, lateral cavernous sinus and cranial nerves in lateral wall. An understanding of the complex relationships of the cavernous segment of internal carotid artery and cranial nerves in cavernous sinus is paramount for surgically dealing with the disease involved cavernous sinus and adjacent region.