Vulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology.

Vulvar cancer is annually diagnosed in an estimated 6,470 individuals and the vast majority are histologically squamous cell carcinomas. Vulvar cancer accounts for 5% to 8% of gynecologic malignancies. Known risk factors for vulvar cancer include increasing age, infection with human papillomavirus, cigarette smoking, inflammatory conditions affecting the vulva, and immunodeficiency. Most vulvar neoplasias are diagnosed at early stages. Rarer histologies exist and include melanoma, extramammary Paget's disease, Bartholin gland adenocarcinoma, verrucous carcinoma, basal cell carcinoma, and sarcoma. This manuscript discusses recommendations outlined in the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for treatments, surveillance, systemic therapy options, and gynecologic survivorship.

NCCN Guidelines® Insights: Cervical Cancer, Version 1.2024.

The NCCN Guidelines for Cervical Cancer provide recommendations for all aspects of management for cervical cancer, including the diagnostic workup, staging, pathology, and treatment. The guidelines also include details on histopathologic classification of cervical cancer regarding diagnostic features, molecular profiles, and clinical outcomes. The treatment landscape of advanced cervical cancer is evolving constantly. These NCCN Guidelines Insights provide a summary of recent updates regarding the systemic therapy recommendations for recurrent or metastatic disease.

Uterine Neoplasms, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology.

Adenocarcinoma of the endometrium (also known as endometrial cancer, or more broadly as uterine cancer or carcinoma of the uterine corpus) is the most common malignancy of the female genital tract in the United States. It is estimated that 65,950 new uterine cancer cases will have occurred in 2022, with 12,550 deaths resulting from the disease. Endometrial carcinoma includes pure endometrioid cancer and carcinomas with high-risk endometrial histology (including uterine serous carcinoma, clear cell carcinoma, carcinosarcoma [also known as malignant mixed Müllerian tumor], and undifferentiated/dedifferentiated carcinoma). Stromal or mesenchymal sarcomas are uncommon subtypes accounting for approximately 3% of all uterine cancers. This selection from the NCCN Guidelines for Uterine Neoplasms focuses on the diagnosis, staging, and management of pure endometrioid carcinoma. The complete version of the NCCN Guidelines for Uterine Neoplasms is available online at NCCN.org.

NCCN Guidelines® Insights: Uterine Neoplasms, Version 3.2021.

The NCCN Guidelines for Uterine Neoplasms provide recommendations for diagnostic workup, clinical staging, and treatment options for patients with endometrial cancer or uterine sarcoma. These NCCN Guidelines Insights focus on the recent addition of molecular profiling information to aid in accurate diagnosis, classification, and treatment of uterine sarcomas.

NCCN Guidelines Insights: Cervical Cancer, Version 1.2020.

The NCCN Guidelines for Cervical Cancer provide recommendations for diagnostic workup, staging, and treatment of patients with the disease. These NCCN Guidelines Insights focus on recent updates to the guidelines, including changes to first- and second-line systemic therapy recommendations for patients with recurrent or metastatic disease, and emerging evidence on a new histopathologic classification system for HPV-related endocervical adenocarcinoma.

Gestational Trophoblastic Neoplasia, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology.

Gestational trophoblastic neoplasia (GTN), a subset of gestational trophoblastic disease (GTD), occurs when tumors develop in the cells that would normally form the placenta during pregnancy. The NCCN Guidelines for Gestational Trophoblastic Neoplasia provides treatment recommendations for various types of GTD including hydatidiform mole, persistent post-molar GTN, low-risk GTN, high-risk GTN, and intermediate trophoblastic tumor.

Cervical Cancer, Version 3.2019, NCCN Clinical Practice Guidelines in Oncology.

Cervical cancer is a malignant epithelial tumor that forms in the uterine cervix. Most cases of cervical cancer are preventable through human papilloma virus (HPV) vaccination, routine screening, and treatment of precancerous lesions. However, due to inadequate screening protocols in many regions of the world, cervical cancer remains the fourth-most common cancer in women globally. The complete NCCN Guidelines for Cervical Cancer provide recommendations for the diagnosis, evaluation, and treatment of cervical cancer. This manuscript discusses guiding principles for the workup, staging, and treatment of early stage and locally advanced cervical cancer, as well as evidence for these recommendations. For recommendations regarding treatment of recurrent or metastatic disease, please see the full guidelines on NCCN.org.

Uterine Neoplasms, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology.

Endometrial carcinoma is a malignant epithelial tumor that forms in the inner lining, or endometrium, of the uterus. Endometrial carcinoma is the most common gynecologic malignancy. Approximately two-thirds of endometrial carcinoma cases are diagnosed with disease confined to the uterus. The complete NCCN Guidelines for Uterine Neoplasms provide recommendations for the diagnosis, evaluation, and treatment of endometrial cancer and uterine sarcoma. This manuscript discusses guiding principles for the diagnosis, staging, and treatment of early-stage endometrial carcinoma as well as evidence for these recommendations.

Vulvar Cancer, Version 1.2017, NCCN Clinical Practice Guidelines in Oncology.

Vulvar cancer is a rare gynecologic malignancy. Ninety percent of vulvar cancers are predominantly squamous cell carcinomas (SCCs), which can arise through human papilloma virus (HPV)-dependent and HPV-independent pathways. The NCCN Vulvar Cancer panel is an interdisciplinary group of representatives from NCCN Member Institutions consisting of specialists in gynecological oncology, medical oncology, radiation oncology, and pathology. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Vulvar Cancer provide an evidence- and consensus-based approach for the management of patients with vulvar SCC. This manuscript discusses the recommendations outlined in the NCCN Guidelines for diagnosis, staging, treatment, and follow-up.

A review of the literature for intra-arterial chemotherapy used to treat retinoblastoma.

Retinoblastoma is a malignancy of the retina that usually presents before the age of 5 years. Sporadic retinoblastoma is most often unilateral and with no hereditary influence, whereas familial retinoblastoma presents unilaterally or bilaterally in conjunction with genetic inheritance. Several treatments have been attempted with the goals of saving the child's life, salvaging the eye, and preserving vision. Alternative methods including external beam radiation, systemic chemotherapy and focal therapies have been shown to be effective but carry a risk of enucleation and other complications proportional to the severity of the tumor. Selective intra-arterial chemotherapy for retinoblastoma began in 1988 in Japan and has emerged in the last 7 years in the United States as a feasible, effective and minimally invasive treatment option. We review the retinoblastoma treatment literature focusing on intra-arterial chemotherapy.

Neuroimaging findings in children with Keutel syndrome.

BACKGROUND: Keutel syndrome is a rare autosomal-recessive condition characterized by abnormal cartilage calcification. Neuroimaging findings associated with this condition have been randomly described in the literature. OBJECTIVE: To systematically evaluate the neuroimaging findings in a series of children with Keutel syndrome to broaden our base of knowledge. MATERIALS AND METHODS: Four children with confirmed Keutel syndrome were reviewed for the brain, head and neck imaging findings. RESULTS: Three of the four children, all siblings, showed evidence of moyamoya syndrome. All four siblings had pinna cartilage calcification. CONCLUSION: We propose that Keutel syndrome be considered and included among the secondary causes of moyamoya syndrome. In children with petrified auricle and neurological symptoms, Keutel syndrome should be considered and brain MRI with MRA is required.

Analysis of radiation doses incurred during diagnostic cerebral angiography after the implementation of dose reduction strategies.

BACKGROUND: One goal of increasing awareness of radiation dose is to encourage personal and technical modifications in order to reduce the radiation exposure of patients and staff. OBJECTIVE: To analyze the radiation doses incurred during diagnostic cerebral angiography and the angiographic techniques practiced over a 4-year period, in order to demonstrate the effectiveness of implementing radiation dose reduction strategies. METHODS: A retrospective review of the first 50 consecutive adult and pediatric patients undergoing diagnostic cerebral angiography each year from 2010 to 2013 was performed. Angiograms and procedure examination protocols were reviewed for patient age, gender, diagnosis, angiography techniques, fluoroscopy time, reference point air kerma (Ka,r in mGy), and kerma-area product (PKA in µGym2). RESULTS: From January 2010 to June 2013, a total of 231 diagnostic cerebral angiograms were reviewed (200 adults, 31 children). Adult patients were aged from 19 to 94 years and included 77 men and 123 women. Pediatric patients were aged from 2 to 18 years and comprised 11 boys and 20 girls. Median Ka,r and PKA significantly decreased from 2010 to 2013 in adults (1867 mGy; 21 231 µGym2 vs 653 mGy; 7860 µGym2) and children (644 mGy; 6495 µGym2 vs138 mGy; 1465 µGym2), (p<0.001). CONCLUSIONS: Increased awareness and implementation of dose reduction strategies resulted in decreased radiation doses for diagnostic cerebral angiography both in adult and pediatric patients. The use of lower and variable digital subtraction angiography frame rates and tailored examinations contributed significantly to the reduced radiation doses observed during diagnostic cerebral angiography.

Decompression of the Jugular Bulb for Enhanced Infralabyrinthine Access to the Petroclival Region: A Quantitative Analysis.

Objectives To describe an enhanced infralabyrinthine approach to petroclival lesions with jugular bulb decompression, and to quantify surgical access using a flat-panel computed tomography image protocol. Design Retrospective case series and paired comparison of pre- versus post-dissection anatomy. Setting Tertiary academic medical center. Participants Four patients presenting with petroclival lesions. Six fresh cadaveric specimens were used for temporal bone dissection. Main Outcome Measures Axial and coronal dimensions, and access angles of the infralabyrinthine surgical corridor. Results Decompression of the jugular bulb increased the craniocaudal width of the infralabyrinthine corridor from 0.9 to 7.9 mm to 6.5 to 11.6 mm. The mean increase of 4 mm was statistically significant (t = 3.7; p < .05). There was also a significant widening of the infralabyrinthine window along the axial dimension by 0.9 to 4.5 mm or a mean of 2 mm (t = 3.7; p < .05). Angles of access to the petroclival region were wider following jugular bulb decompression, particularly in the coronal plane (mean difference 7.9 degrees; t = 5.0; p < .005) but less so in the axial plane (mean difference 4.7 degrees; t = 2.5; p = .05). Conclusions Jugular bulb decompression enhances infralabyrinthine access to petroclival lesions, permitting the removal of tissue for diagnoses or partial resection, without significant additional morbidity.

Fenestration of the supraclinoid internal carotid artery in a patient with a concomitant intracranial arteriovenous malformation.

Accurately recognising a supraclinoid internal carotid artery (ICA) fenestration, despite its rare location, is important as it can mimic an intracranial aneurysm, intraluminal thrombus or focal dissection on non-invasive imaging. The overwhelming majority of reported supraclinoid ICA fenestrations are associated with intracranial aneurysms; however, a concomitant arteriovenous malformation (AVM) remote from the fenestration site should also be considered. We present the case of a 26-year-old woman with a Spetzler-Martin grade I right frontal AVM in whom a left supraclinoid ICA fenestration was incidentally discovered during cerebral angiography. She underwent n-N-Butyl Cyanoacrylate glue embolisation of two dominant middle cerebral artery feeders followed by neurosurgical resection. She tolerated procedures well without complications and has remained neurologically intact.

Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation.

Venous-lymphatic anomalies (VLA) are rare and benign congenital lesions of the lymphatic system, composed of endothelial-lined lymphatic cysts. They are most frequently located in the region of the head and neck, and represent 4% of all orbital masses. In those patients with extensive orbital VLAs, a strong association with intracranial vascular anomalies has been reported. Factors known to suddenly increase the size of these lesions include upper respiratory tract infections or intralesional haemorrhage; however, complete spontaneous regression is rare. We report on the classic presentation of a patient with a fluctuating right orbital VLA in association with an intracranial cavernous malformation and intracranial developmental venous anomaly.

Practical techniques for reducing radiation exposure during cerebral angiography procedures.

PURPOSE: DSA remains the gold standard imaging method for the evaluation of many cerebrovascular disorders, in particular cerebral aneurysms and vascular malformations. The purpose of this study was to demonstrate the effect of modifying DSA frame rate, fluoroscopic and roadmap pulse rates, and flat panel detector (FPD) position on the radiation dose delivered during routine views for a cerebral angiogram in a phantom model. MATERIALS AND METHODS: Adult skull and abdomen/pelvis anthropomorphic phantoms were used to compare the radiation dose metrics Ka,r (in mGy), PKA (in µGym(2)), and fluoroscopy time (in minutes) after modification of fluoroscopic pulses per second (p/s), DSA frames per second (f/s), and FPD position and collimation in three components of a cerebral angiogram: (1) femoral artery access, (2) roadmap guidance, and (3) biplane cerebral DSA. RESULTS: For femoral artery access, DSA protocols resulted in significantly higher doses than those utilizing fluoroscopy alone (p=0.007). Roadmaps using 3 p/s or 4 p/s delivered significantly less dose than higher pulse rates (p=0.008). The ranges of delivered doses for biplane cerebral DSA were 347.3-1188.5 mGy and 3914.54-9518.78 µGym(2). The lowest radiation doses were generated by the variable frame rate DSA protocols. CONCLUSIONS: Replacing femoral arterial access evaluations by DSA with fluoroscopy, utilizing lower pulse rates during fluoroscopy and roadmap guidance, and choosing variable frame rates for DSA are simple techniques that may be considered by operators in their clinical practices to lower radiation dose during cerebral angiography procedures.

Unruptured intracranial aneurysms in children with sickle cell disease: analysis of 18 aneurysms in 5 patients.

BACKGROUND: Intracranial aneurysms (IAs) are rare in the general pediatric population and account for <2% of all cerebral aneurysms. Only 7 children with sickle hemoglobinopathy and IAs have been reported, the majority of which were discovered after rupture. OBJECTIVE: To report the prevalence of unruptured IAs in a selected population of children with sickle cell disease (SCD) and to describe the aneurysm morphology, hematologic characteristics, and management in this patient population. METHODS: A retrospective review of the electronic database for all children with SCD who underwent brain magnetic resonance imaging or angiography from January 2002 to August 2013 at a single institution was performed. Records were reviewed for IA, age, sex, sickle cell genotype, neurological symptoms, hematologic indexes, transcranial Doppler findings, and management. RESULTS: Five of 179 children (2.8%) with SCD imaged by brain magnetic resonance imaging or angiography were diagnosed with IAs. None presented with subarachnoid hemorrhage. Four patients (80%) had HbSS disease, and 1 patient had hemoglobin sickle cell HbSC disease. A total of 18 aneurysms were detected; the majority of patients had multiple aneurysms (80%) and bilateral involvement (60%). CONCLUSION: Children with SCD are at risk for developing multiple intracranial aneurysms, and a high index of suspicion must be maintained during the interpretation of routine magnetic resonance imaging or angiography of the brain.