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BackgroundThe en bloc resection of inferior vena cava (IVC) leiomyosarcoma often necessitates IVC reconstruction. The objective of this study is to examine outcomes after IVC reconstruction and determine optimal graft sizing.MethodsA retrospective review was conducted of all IVC reconstructions after IVC leiomyosarcoma resection at a single institution. Cross-sectional dimensions at the IVC resection margins were measured on preoperative imaging. The tumor location was based on the most superiorly involved region of the IVC and was classified as infrarenal, between hepatic and renal veins, or superior to the hepatic veins. Perioperative details and long-term outcomes including graft sizing, graft patency, morbidity, and mortality were recorded.ResultsBetween 2007 and 2017, 12 patients (6 females, mean age: 64.5 years, age range: 46-80 years) underwent IVC leiomyosarcoma resection and reconstruction. All reconstructions were performed with ringed polytetrafluoroethylene (PTFE); graft sizes ranged from 12 mm to 16 mm. The tumor location was exclusively infrarenal in seven patients, between the renal and hepatic veins in two patients, and involved multiple segments in three patients. Larger graft sizes were utilized in reconstructing more superior segments of the IVC. Grafts were typically undersized and based on the diameter of the superior resection margin with 12 mm grafts approximately correlating to a 20 mm diameter, 14 mm to 25 mm, and 16 mm to 30 mm. The average undersizing ratio was 0.6. At a mean follow-up time of 43 ± 27 months, radiographic graft patency was 92%, overall survival was 83%, and disease-free survival was 25%.ConclusionsAfter en bloc resection of IVC leiomyosarcoma, caval reconstruction with an undersized ringed PTFE has acceptable patency. Grafts sizes should be based on the IVC diameter superior to the tumor and undersizing by approximately 40% appears to be associated with acceptable patency rates. Further multiinstitutional studies should be performed to best determine the optimal treatment of this rarely encountered tumor.

BACKGROUND:Pediatric intracavernous sinus tumors are exceedingly rare and thus poorly characterized. Their neurosurgical management is challenging and diagnostic, and management guidelines are limited. OBJECTIVE:To report our institutional experience with the surgical resection of pediatric intracavernous sinus tumors. We also compare and contrast our results with the 14 cases of pediatric intracavernous sinus lesions in the current literature. METHODS:A retrospective descriptive analysis of consecutive pediatric patients (ages 0-18 yr) presenting to our institution with a diagnosis of an intracavernous sinus lesion was performed. From January 2012 to January 2017, 5 cases were identified. Eleven patients with secondary invasion of the cavernous sinus (2 meningiomas, 7 pituitary adenomas) or dermoid tumors involving the cavernous sinus (2) were not included in our review. RESULTS:Surgical resection via a frontotemporal orbitozygomatic approach was performed in all cases by a single senior neurosurgeon (M.L.). There were no perioperative or postoperative complications attributable to the surgery or approach. Four of 5 patients remained neurologically stable throughout the perioperative and postoperative period. The fifth patient had a complete resolution of their cranial neuropathies postoperatively. A pathological diagnosis that guided long-term management was obtained in all cases. CONCLUSION:Neurosurgical management of pediatric cavernous sinus lesions can be safely performed and critically guide future therapies. Surgeon familiarity with cavernous sinus and skull-base anatomy is critical to the successful management of these patients. The benefits of surgery should be balanced against the potential complications and need for a tissue diagnosis in children. The senior author had a significant experience with cavernous sinus approaches in adults prior to initiating use of the approach in the pediatric population.

IntroductionMany patients who undergo coronary artery bypass surgery have a prior history of cancer and potentially chest radiation which is a known risk factor for coronary atherosclerosis. Prior radiation increases fibrosis and can make the dissection of the left internal mammary artery (LIMA) more challenging.Case reportA 72-year-old woman with a history of stage IIA pT2N0M0 left breast intraductal carcinoma treated with lumpectomy, adjuvant chemotherapy and radiation therapy 11 years prior presented to the emergency room with a non-ST elevation myocardial infarction and was taken for cardiac catheterization followed by three-vessel coronary artery bypass grafting. The LIMA was found to be encased in scar tissue and was deemed unsuitable as a conduit, and a saphenous vein graft was bypassed to the left anterior descending artery in its place. Pathologic review of the LIMA showed nests of tumor cells infiltrating within dense fibrous tissue with areas of necrosis and calcifications consistent with recurrent breast cancer. Interestingly the patients original breast cancer was positive for estrogen receptors (ER) and progesterone receptors (PR) ER and PR and negative for HER2 and she had therefore been treated with 5 years of hormonal therapy. The recurrent cancer found in the LIMA dissection bed at the time of bypass surgery was ER, PR, and HER2 negative, suggesting hormonal therapy driven clonal selection of these metastatic tumor cells.Discussion and conclusionsScarring in the LIMA dissection bed in patients with a history of cancer and prior chest radiation should be carefully evaluated for the possibility of recurrent cancer. The gross appearance of tissue can be misleading and sending a biopsy for a formal frozen section histologic evaluation should be considered if there is any question of recurrent malignancy.

Infantile hemangiomas (IHs) are the most common vascular tumors in infants, appearing in early infancy and ultimately regressing with time. Clinical presentation may vary, with a minority of lesions causing impairment of vital function (e.g., respiratory or visual obstruction), permanent scarring, and/or disfigurement. The pathogenesis of IH is complex and poorly understood. Risk factors implicated in their development include preterm birth and placental anomalies. IH presents a myriad of clinical challenges, including correct diagnosis and whether or not to pursue treatment. This article is a review of the current literature regarding pathogenesis, clinical presentation, treatment, and prognosis of IH. Birth Defects Research 109:809-815, 2017. © 2017 Wiley Periodicals, Inc.

Characteristic but rare vascular neoplasms in the adult liver composed of small vessels with an infiltrative border were collected from an international group of collaborators over a 5-year period (N=17). These tumors were termed hepatic small vessel neoplasm (HSVN), and the histologic differential diagnosis was angiosarcoma (AS). The average age of patients was 54years (range, 24-83years). HSVN was more common in men. The average size was 2.1cm (range, 0.2-5.5cm). Diagnosis was aided by immunohistochemical stains for vascular lineage (CD31, CD34, FLI-1), which were uniformly positive in HSVN. Immunohistochemical stains (p53, c-Myc, GLUT-1, and Ki-67) for possible malignant potential are suggestive of a benign/low-grade tumor. Capture-based next-generation sequencing (using an assay that targets the coding regions of more than 500 cancer genes) identified an activating hotspot GNAQ mutation in 2 of 3 (67%) tested samples, and one of these cases also had a hotspot mutation in PIK3CA. When compared with hepatic AS (n=10) and cavernous hemangioma (n=6), the Ki-67 proliferative index is the most helpful tool in excluding AS, which demonstrated a tumor cell proliferative index greater than 10% in all cases. Strong p53 and diffuse c-Myc staining was also significantly associated with AS but not with HSVN or cavernous hemangioma. There have been no cases with rupture/hemorrhage, disseminated intravascular coagulation, or Kasabach-Merritt syndrome. Thus far, there has been no metastasis or recurrence of HSVN, but complete resection and close clinical follow-up are recommended because the outcome remains unknown.

Intravascular large cell lymphoma (IVLCL) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia (AIHA) has been described in a single case report, but the true prevalence of this co-occurrence is not known because of declining autopsy rates. Here, we report a case of a 41-year-old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA. At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL, most likely of B-cell lineage. This case illustrates a potential association between IVLCL and AIHA, highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality.

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.