Retrospective Analysis of 20 Patients Affected by Schwannomas in the Upper and Lower Limbs / Análise retrospectiva de 20 pacientes acometidos por schwannoma nos membros superior e inferior

Abstract Objective To analyze the epidemiological profile and evolution of 20 patients diagnosed with upper- and lower-limb schwannomas. Methods A group of patients was defined for a retrospective evaluation comprising the period between February 2002 and June 2018, in which we studied and evaluated 20 medical records of patients undergoing surgery due to schwannoma; the diagnosis was confirmed by an anatomopathological examination. Results Male and female patients were equally affected. The average age was 50.85 years, ranging from 12 to 77 years. There was a predominance of the upper limb and of the flexor face. The most affected nerve was the ulnar nerve. In total, 6 (30%) patients had transient postoperative complications. No cases of tumor recurrence were identified. Conclusion Schwannoma is a rare and difficult-to-diagnose lesion. It should always be considered as a hypothesis when facing a soft-tissue tumor affecting the limbs. The Tinel sign should be regarded, given its higher correlation with complications. The patients should be informed of the possible postoperative complications, which are frequent but usually transient.

Resumo Objetivo Analisar o perfil epidemiológico e a evolução de 20 pacientes diagnosticados com schwannoma nos membros superiores e inferiores. Métodos Definiu-se um grupo de pacientes para avaliação retrospectiva, compreendendo o período entre fevereiro de 2002 e junho de 2018, no qual foram estudados e avaliados 20 prontuários de pacientes submetidos a procedimento cirúrgico devido a schwannoma; a confirmação diagnóstica foi feita pelo exame anatomopatológico. Resultados Tanto os pacientes do sexo masculino quanto do feminino foram igualmente acometidos, e a média de idade foi de 50,85 anos, variando de 12 a 77 anos. Houve predomínio do membro superior e da face flexora. O nervo mais acometido foi o ulnar, e 6 (30%) pacientes apresentaram complicações pós-operatórias transitórias. Não foi identificado nenhum caso de recidiva tumoral. Conclusão O schwannoma é uma lesão rara e de difícil diagnóstico. Deve sempre ser considerada como hipótese quando se estiver diante de um tumor de partes moles acometendo os membros. O sinal de Tinel deve ser levado em consideração por conta de sua maior correlação com as complicações. Os pacientes devem ser informados quanto às possíveis complicações pós-operatórias, que são frequentes, mas, geralmente, transitórias.

Accelerated perineural invasion in colitis-associated cancer: A retrospective cohort study.

Perineural invasion (PNI) is a prognostic factor in patients with colorectal cancer. Neurotrophic factors, molecular determinants of PNI, are altered in their expression levels in patients with ulcerative colitis. In this study, we evaluated the frequency of PNI in colitis-associated cancer (CAC) and sporadic cancer.We retrospectively reviewed 778 colorectal cancers with pathological T3-T4 in 761 patients all of whom were surgically resected without preoperative treatment. The lesions were classified into either CAC or sporadic cancer based on the clinical information. Clinicopathological findings including PNI were compared between CACs and sporadic cancers. Moreover, we analyzed the risk factors for positive PNI by multivariate analysis using a logistic regression model.Ten of the cancers (1.3%) were diagnosed as CACs, and the remaining 768 as sporadic cancers. CACs were characterized by being nonobstructive and predominantly located in the rectum. The CACs had a larger size and more frequent undifferentiated histology than sporadic cancers. PNI was observed more frequently in CACs (90%) than in sporadic cancers without obstruction (45%, P = .007). On multivariate analysis, CAC was one of the significant factors associated with PNI (odds ratio: 9.05, P = .040).Our results suggest that CAC was more likely to exhibit PNI than sporadic colorectal cancer.

Perineural Invasion Is a Prognostic but not a Predictive Factor in Nonmetastatic Colon Cancer.

BACKGROUND: Perineural invasion is associated with adverse oncological outcomes in colorectal cancer. However, data regarding the prognostic and predictive impact in colon cancer are scarce. OBJECTIVE: This study aims to clarify the role of perineural invasion in patients with nonmetastatic colon cancer. DESIGN: This study is a retrospective review of a prospectively maintained database. SETTINGS: This study took place at a tertiary medical center. PATIENTS: Patients with stage I to III colon cancer who underwent elective surgery at our tertiary center between 2004 and 2015 (n = 1145) were included. MEAN OUTCOME MEASURES: The primary long-term outcomes include disease-free survival, disease-specific survival, and overall survival. Differences were determined by multivariate Cox regression models adjusted for stage and potential confounders. RESULTS: Perineural invasion was identified in 215 patients (18.8%) and associated with emergency procedures, male sex, and advanced disease. Histopathological features including lymphatic and extramural vascular invasion, poor differentiation, and infiltrating tumor borders were correlated with perineural invasion. Compared with patients with perineural invasion-negative tumors, patients who had perineural invasion-positive tumors had worse disease-free, overall, and disease-specific survival (all p < 0.001). Moreover, patients with perineural invasion-positive node-negative disease had worse overall survival than patients with perineural invasion-negative node-positive disease (p < 0.001). After adjustment, perineural invasion remained significantly associated with worse disease-free survival (HR, 1.45; 95% CI, 1.03-2.03; p = 0.033), worse overall survival (HR, 1.75; 95% CI, 1.33-2.31; p < 0.001), and worse disease-specific survival (HR, 1.52; 95% CI, 1.00-2.30; p = 0.048). However, we did not find a significant predictive response with adjuvant chemotherapy in perineural invasion-positive node-negative tumors (HR, 2.10; 95% CI, 0.80-5.51; p = 0.122). The predictive value was only demonstrated in stage III disease with a significant impaired overall survival in patients with perineural invasion-positive tumors who did not receive adjuvant therapy (HR, 0.23; 95% CI, 0.13-0.40; p < 0.001). LIMITATIONS: This study was limited by its retrospective design. CONCLUSION: Our study confirms the prognostic value of perineural invasion in stage I to II and III colon cancer. However, patients with node-negative disease and perineural invasion did not significantly benefit from adjuvant therapy. More information regarding postoperative treatment in node-negative perineural invasion-positive colon cancer is required. See Video Abstract at http://links.lww.com/DCR/A988. LA INVASIÓN PERINEURAL COMO FACTOR PRONÓSTICO NO PREDICTIVO EN EL CÁNCER DE COLON NO METASTÁSICO: La invasión perineural se encuentra asociada a resultados oncológicos adversos en casos de cáncer colorrectal. Sin embargo, los datos sobre el impacto pronóstico y predictivo en caso de cáncer de colon son pocos. OBJETIVO: Definir el papel de la invasión perineural en pacientes con cáncer de colon no metastásico. DISEÑO:: Revisión retrospectiva de una base de datos alimentada prospectivamente. AJUSTES: Centro hospitalario de atención terciaria. PACIENTES: Todos aquellos portadores de un cáncer de colon estadío I-III que se sometieron a cirugía electiva en nuestro centro entre 2004-2015 (n = 1145). PRINCIPALES RESULTADOS: Los resultados a largo plazo incluyeron la supervivencia sin enfermedad, la supervivencia específica de la enfermedad y la supervivencia general. Las diferencias se determinaron mediante modelos de regresión multivariantes de Cox, ajustados para el control de factores de confusión durante el análisis por estratificación. RESULTADOS: La invasión perineural fué identificada en 215 pacientes (18.8%) y se la asoció con procedimientos de emergencia, al género masculino y a la enfermedad avanzada. Las características histopatológicas que incluyeron la invasión vascular linfática y extramural, la diferenciación deficiente y los bordes tumorales infiltrantes se correlacionaron con la invasión perineural. Comparativamente con los tumores sin invasión perineural, los pacientes positivos a la invasión perineural tuvieron una peor supervivencia general, libre y específica de la enfermedad (todos p < 0.001). Asimismo, aquellos pacientes con invasion-perineural con ganglios negativos tuvieron una supervivencia global mucho peor que aquellos pacientes con ganglios positivos e invasión perineural negativa (p < 0.001). Después del ajuste, la invasión perineural se asoció significativamente con una peor supervivencia sin la enfermedad (HR, 1.45; IC 95%, 1.03-2.03; p = 0.033), supervivencia general (HR, 1.75; IC 95%, 1.33-2.31; p <0.001), así como con una peor supervivencia específica de la enfermedad (HR, 1.52; IC 95%, 1.00-2.30; p = 0.048). Sin embargo, no encontramos una respuesta predictiva significativa con quimioterapia adyuvante en los tumores acompañados de invasion-perineural con ganglios negativos (HR, 2.10; IC del 95%, 0.80-5.51; p = 0.122). El valor predictivo solo fué demostrado en aquellos casos de estadio III con un deterioro significativo de la supervivencia global en pacientes con tumores perineurales positivos a la invasión y que no recibieron tratamiento adyuvante (HR, 0.23; IC 95%, 0.13-0.40; p < 0.001). LIMITACIONES: Diseño retrospectivo. CONCLUSIÓN:: Nuestros resultados confirman el valor pronóstico de la invasión perineural en el cáncer de colon estadios I-II y III. Sin embargo, los pacientes con enfermedad ganglionar negativa e invasión perineural no se beneficiaron significativamente de la terapia adyuvante. Se requiere más información sobre el tratamiento postoperatorio en el cáncer de colon positivo para la invasión perineural con ganglios negativos. Vea el Resumen del video en http://links.lww.com/DCR/A988.

Malignant peripheral nerve sheath tumors: Analysis of the national cancer database.

OBJECTIVES: Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcoma (STS) that carries significant mortality. Given the rarity of MPNST, current knowledge on the disease is limited. We aim to characterize patient, tumor, and treatment factors, and determine variables associated with 5-year overall survival (OS) in individuals with MPNST. MATERIALS AND METHODS: We utilized the National Cancer Database (NCDB) to identify adult patients diagnosed with MPNST from 2004 to 2015, and analyze patient, tumor, treatment, and survival data. American Joint Committee on Cancer (AJCC 8) staging criteria for STS was utilized. RESULTS: In our cohort of 2,858 patients with MPNST, median age at diagnosis was 47. The 5-year OS rate was 52%. Age, race, tumor size, and treatment regimen significantly predicted 5-year OS on univariate analysis. AJCC 8 stage appeared to predict 5-year OS in head and neck tumors (though not significantly), but not trunk and extremity tumors. CONCLUSION: While our cohort of patients with MPNST are typically relatively young and healthy, 5-year OS is only 52%. Notably, age extremes, large tumor size, and certain treatment regimens are associated with worse outcomes. While AJCC 8 staging guidelines may have some value in predicting OS in head and neck tumors, they do not reliably risk stratify patients with trunk and extremity MPNST. Ultimately, MPNST is associated with high mortality with few modifiable risk factors, and survival may not be adequately predicted by current staging systems.

Epidemiology and Estimated Cost of Microsurgical Treatment of Peripheral Nerve Tumor Conducted by the Brazilian Unified Health Care System (2008­2016)

Introduction Primary nerve tumors correspond to 5% of the soft tissue neoplasms affecting the upper limbs, with benign lesions being more frequent than malignant lesions. Objective To describe the epidemiological data of the microsurgical treatment of peripheral nerve tumors performed by the Brazilian Unified Health System (SUS, in the Portuguese acronym), with the code 0403020131, from 2008 to 2016, regarding the number of annual procedures, hospital and professional expenses, the average duration of hospital stay, and the number of deaths. Methods This is a descriptive epidemiological study whose data were obtained by consulting the database provided by the Health Informatics Department of the Brazilian Ministry of Health (DATASUS, in the Portuguese acronym). Results/Discussion A total of 6,012 procedures were performed during the period studied, with an average of 688 procedures per year. The average hospital stay was of 2.13 days. There was nomortality rate. The average annual cost of the professional was BRL 50,091.45; and the average hospital expenses were BRL111,887.94. Conclusion The microsurgical treatment of peripheral nerve tumors is a safe surgical procedure with zero mortality rate and short hospital stay. There was no national data on tumors of this nature in the medical literature.

Cauda equina schwannoma with concomitant intervertebral disc herniation: A case report and review of literature.

The coexistence of lumbar intervertebral disc (IVD) herniation and cauda equina schwannoma in symptomatic patients is uncommon. We experienced a case with a surgically treated cauda equina schwannoma with concomitant lumbar IVD herniation (L2/L3); both were relatively small but presented with painful symptoms in combination. A 45-year old man complained of low back pain and right anterior thigh pain six months prior to surgery. He underwent conservative treatment; however, his pain suddenly worsened three months before surgery, with abnormal sensations and pain in his right buttock and posterior thigh. A magnetic resonance imaging (MRI) scan revealed an L2/L3 disc herniation extruding to the center of the lumbar spinal canal and compressing the thecal sac. Incidentally, an intrathecal tumor was suspected, and enhanced MRI revealed a round tumor measuring 10-mm in diameter at the L2/L3 intervertebral level, which was uniformly enhanced by gadolinium chelate, radiologically diagnosed as a schwannoma. Microscopic discectomy and tumor resection were performed and the patient's symptoms disappeared immediately. Schwannoma is generally slow-growing, and usually asymptomatic when the tumor size is small. Therefore, we suspected that the tumor occasionally impinged on the left L3 root with L2/3 IVD herniation, leading to acute worsening of the patient's symptoms. Altogether, in case of tumor in patients undergoing conservative treatment for cauda equina schwannoma, we should consider that symptoms can acutely worsen with the occurrence of lumbar IVD herniation.

Intraneural lipomas: institutional and literature review.

BACKGROUND: Adipose lesions of nerve can be envisioned as a spectrum ranging from intraneural/extraneural lipomas to lipomatosis of nerve (LN). We have noticed that intraneural lipomas are not as a homogenous group as previously thought and demonstrate differences which have clinical implications. To better understand intraneural lipomas, we conducted a search of cases at our institution and published cases in the world's literature. MATERIALS AND METHODS: Mayo Clinic's database was searched between years 1994-2018. Published cases were identified using PubMed and Google Scholar databases. Following terms were used: intraneural lipoma, lipoma and nerve, lipoma and neuropathy, lipofibroma and nerve, fibrolipoma and nerve and neural lipoma as well as lipofibroma and fibrolipoma alone. Cases that could be clearly identified as intraneural lipomas by the location of the lipoma within the epineurium were included for analysis. These cases were then sub-classified as encapsulated intraneural lipomas or hybrid intraneural lipomas (demonstrating features of both intraneural/extraneural lipomas and LN) based on their characteristics. RESULTS: We identified 12 cases at our institution (8 encapsulated, 4 hybrid) and 24 published cases (21 encapsulated, 3 hybrid). The most commonly affected nerve was median both at our institution and in the published cases. Encapsulated cases were found to be relatively easy to resect. Hybrid cases demonstrated variable degree of interdigitating fat between the fascicles and were relatively difficult to resect. CONCLUSION: Intraneural lipomas exist as two separate entities with distinct clinical implications. Although rare, this should be taken in account when planning surgery. Terminology should be clarified to prevent ambiguity and confusion.

Livestock and poultry density and childhood cancer incidence in nine states in the USA.

BACKGROUND: Parental occupational and childhood exposures to farm animals have been positively associated with childhood brain tumors, whereas associations with childhood leukemia are equivocal. The developing immune system may be influenced by allergen, virus, or other exposures from animal sources, which may contribute to childhood cancer incidence. METHODS: Incident cancers (acute lymphoblastic leukemia [ALL], acute myeloid leukemia [AML], central nervous system [CNS], peripheral nervous system [PNS]) for children aged 0-4 diagnosed between 2003 and 2008 were obtained from nine National Cancer Institute Surveillance, Epidemiology and End Results (SEER) registries and were linked to U.S. Census of Agriculture data from 2002 and 2007 by county of diagnosis. Animal densities (animal units [AU]/km2; one animal unit is 1000 pounds of animal weight) were estimated for hogs, cattle, chickens (layers and broilers, separately), equine (horses, ponies, mules, burros, donkeys), goats, sheep, turkeys, and total animals. Animal density was examined in models as both continuous (AU per km2) and categorical variables (quartiles). Animal operation densities (per km2) by size of operation (cattle, hogs, chickens, sheep) were modeled continuously. Rate ratios and 95% confidence intervals were estimated using Poisson regression. RESULTS: We found positive associations between AML and broiler chicken densities (RRper 10AU/km2 = 1.14, 95% CI = 1.02-1.26). ALL rates increased with densities of hog operations (RRper operation/100km2 = 1.06, 95% CI = 1.02-1.11). PNS cancer rates were inversely associated with layer chicken density (RRper log of AU/km2 = 0.94, 95% CI = 0.89-0.99). No association was found between any cancer type and densities of cattle, equine, or goats. CONCLUSIONS: Although limited by the ecologic study design, some of our findings are novel and should be examined in epidemiological studies with individual level data.

Genetic variants in the promoter region of the calcium-sensing receptor gene are associated with its down-regulation in neuroblastic tumors.

We have previously reported that the calcium-sensing receptor (CaSR) is expressed in benign, differentiated neuroblastic tumors, and epigenetically silenced in undifferentiated, malignant cases. Furthermore, cinacalcet, an allosteric activator of the CaSR, reduces neuroblastoma tumor growth in preclinical models. However, to identify patients that might benefit from this treatment, a complete understanding of mechanisms governing CaSR expression in these tumors would be required. We have now analyzed two polymorphisms in the promoter region of the CASR gene (rs7652589 and rs1501899) by allelic discrimination in neuroblastoma patients and cell lines. Association of genotypes and haplotypes with CaSR mRNA levels and CASR promoter P2 methylation status was determined. Data presented show that minor alleles rs7652589 and rs1501899, present either in homo- or heterozygosis, were correlated with reduced CaSR mRNA levels in matching primary tumors and this association was independent of CASR promoter P2 hypermethylation. Haplotype AA was independently associated with reduced CaSR expression after adjusting by promoter P2 methylation status. These polymorphisms were identified in some ganglioneuromas in which CaSR expression is low despite exhibiting a high degree of differentiation. Furthermore, homozygous variants rs7652589 and rs1501899 were detected in SH-SY5Y cells, which are devoid of CaSR expression in the absence of hypermethylation of CASR promoter P2. In summary, minor alleles rs7652589 and rs1501899 are associated with reduced CaSR expression in neuroblastic tumors and neuroblastoma cell lines in which the CASR gene promoter P2 is not hypermethylated. Therefore, they potentially represent an additional mechanism of CASR transcriptional regulation in this group of developmental malignancies. © 2016 Wiley Periodicals, Inc.

Insight into the epidemiology of cutaneous squamous cell carcinoma with perineural spread.

BACKGROUND: Perineural spread (PNS) of cutaneous squamous cell carcinoma of the head and neck (SCCHN) can be associated with poor outcomes. Disease understanding and awareness is limited leading to delayed diagnosis and treatment. The purpose of this study was to identify epidemiological features of patients with PNS of cutaneous SCCHN. METHODS: Tumor characteristics and demographics of patients with PNS of cutaneous SCCHN managed through a single institution were collected between 1998 and 2013. RESULTS: One hundred twenty patients were included in this study. The majority had a history of skin cancer (85.8%). The median time from primary tumor treatment to PNS symptom onset was 16 months (range, 1-86 months). A total of 34.2% had no perineural invasion (PNI) detected in the primary, and 22.5% had no known primary tumor. Only 5.8% of the patients had nodal involvement at presentation. CONCLUSION: Patients can present with PNS from cutaneous SCCHN with no known primary tumor or with primary tumors without PNI. The majority of patients presented without regional nodal involvement. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1416-1420, 2016.

Agricultural crop density and risk of childhood cancer in the midwestern United States: an ecologic study.

BACKGROUND: There is limited evidence for an association between agricultural pesticide exposure and certain types of childhood cancers. Numerous studies have evaluated exposure to pesticides and childhood cancer and found positive associations. However, few studies have examined the density of agricultural land use as a surrogate for residential exposure to agricultural pesticides and results are mixed. We examined the association of county level agricultural land use and the incidence of specific childhood cancers. METHODS: We linked county-level agricultural census data (2002 and 2007) and cancer incidence data for children ages 0-4 diagnosed between 2004 and 2008 from cancer registries in six Midwestern states. Crop density (percent of county area that was harvested) was estimated for total agricultural land, barley, dry beans, corn, hay, oats, sorghum, soybeans, sugar beets, and wheat. Rate ratios and 95% confidence intervals were estimated using generalized estimating equation Poisson regression models and were adjusted for race, sex, year of diagnosis, median household income, education, and population density. RESULTS: We found statistically significant exposure-response relationships for dry beans and total leukemias (RR per 1% increase in crop density = 1.09, 95% CI = 1.03-1.14) and acute lymphoid leukemias (ALL) (RR = 1.10, 95% CI = 1.04-1.16); oats and acute myeloid leukemias (AML) (RR = 2.03, 95% CI = 1.25, 3.28); and sugar beets and total leukemias (RR = 1.11, 95% CI = 1.04, 1.19) and ALL (RR = 1.11, 95% CI = 1.02, 1.21). State-level analyses revealed some additional positive associations for total leukemia and CNS tumors and differences among states for several crop density-cancer associations. However, some of these analyses were limited by low crop prevalence and low cancer incidence. CONCLUSIONS: Publicly available data sources not originally intended to be used for health research can be useful for generating hypotheses about environmental exposures and health outcomes. The associations observed in this study need to be confirmed by analytic epidemiologic studies using individual level exposure data and accounting for potential confounders that could not be taken into account in this ecologic study.

Gender dimorphism and age of onset in malignant peripheral nerve sheath tumor preclinical models and human patients.

BACKGROUND: Gender-based differences in disease onset in murine models of malignant peripheral nerve sheath tumor (MPNST) and in patients with Neurofibromatosis type-1-(NF-1)-associated or spontaneous MPNST has not been well studied. METHODS: Forty-three mGFAP-Cre+;Ptenloxp/+;LSL-K-rasG12D/+ mice were observed for tumor development and evaluated for gender disparity in age of MPNST onset. Patient data from the prospectively collected UCLA sarcoma database (1974-2011, n = 113 MPNST patients) and 39 published studies on MPNST patients (n = 916) were analyzed for age of onset differences between sexes and between NF-1 and spontaneous MPNST patients. RESULTS: Our murine model showed gender-based differences in MPNST onset, with males developing MPNST significantly earlier than females (142 vs. 162 days, p = 0.015). In the UCLA patient population, males also developed MPNST earlier than females (median age 35 vs. 39.5 years, p = 0.048). Patients with NF-1-associated MPNST had significantly earlier age of onset compared to spontaneous MPNST (median age 33 vs. 39 years, p = 0.007). However, expanded analysis of 916 published MPNST cases revealed no significant age difference in MPNST onset between males and females. Similar to the UCLA dataset, patients with NF-1 developed MPNST at a significantly younger age than spontaneous MPNST patients (p < 0.0001, median age 28 vs. 41 years) and this disparity was maintained across North American, European, and Asian populations. CONCLUSIONS: Although our preclinical model and single-institution patient cohort show gender dimorphism in MPNST onset, no significant gender disparity was detected in the larger MPNST patient meta-dataset. NF-1 patients develop MPNST 13 years earlier than patients with spontaneous MPNST, with little geographical variance.

Malignant peripheral nerve sheath tumors of the spine: a SEER database analysis.

Peripheral nerve sheath tumors are uncommon neoplasms that can affect any area of the body. Spinal lesions, especially those that are malignant, pose difficult management challenges, and data regarding these lesions are limited by the disease rarity. This study provides a population-based analysis using the Surveillance, Epidemiology, and End Results (SEER) database, focusing on patient characteristics and treatments. Surgery is associated with improved survival, whereas radiation therapy is associated with decreased survival in this cohort with malignant peripheral nerve sheath tumor in the spine.

Paraneoplastic disorders of the central and peripheral nervous systems.

Paraneoplatic neurologic syndromes (PNS) have been seminally defined as acute or subacute neurological syndromes resulting from nervous system dysfunction that is remote from the site of a malignant neoplasm or its metastases. However, in respect to our current understanding of their pathogenesis we may redefine these disorders as cancer-related dysimmune neurologic syndromes. We first deal with the epidemiology and the pathogenesis of PNS, then the different classic PNS are reviewed with clinical features according to the associated onconeuronal antibodies. Finally, therapeutic approaches are discussed.

Fibroproliferative neuromas may occur after iatrogenic injury for lipomatosis of nerve.

BACKGROUND: Lipomatosis of nerve (LN) is a condition associated with nerve-territory overgrowth. We have noted a unique type of neuroma at sites of LN injury; the neuroma extends beyond the epineurium, enhances, and appears to enlarge over time. OBJECTIVE: We sought to understand the relationship between fibroproliferative scarring and surgery performed on the nerve. METHODS: A review of the searchable records for LN at our institution found 52 cases, confirmed by pathology or pathognomonic appearance on magnetic resonance imaging (MRI). Clinical histories were reviewed to categorize the surgeries performed by the degree of iatrogenic injury to the nerve. Postoperative MRI was performed in 22 of the 46 patients who had surgery, which was then retrospectively reviewed for fibroproliferative neuromas. RESULTS: Complex and masslike neuromas were found on MRI, correlating with the degree of iatrogenic injury to the nerve. These fibrous neuromas proliferated beyond the epineurium, disrupted fascicular architecture, were contrast enhancing when contrast was administered, indicative they were unique and unlike stump or traction neuromas. Of the 8 patients who underwent surgery involving nerve decompression alone, none developed fibroproliferative neuromas. Of the 7 patients who underwent surgery involving nerve debulking, fibroproliferative neuromas developed in 4. Of the 11 patients who underwent surgery involving nerve transection, all developed fibroproliferative neuromas (P < .001). There was also a high incidence of hypertrophic scarring of the skin incision (21.3%). CONCLUSION: Surgical injury of LN appears to be strongly associated with the development of fibroproliferative neuromas. It is possible that the pathological overgrowth stimulus associated with LN promotes exuberant scar formation.

Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007.

BACKGROUND: Primary tumors of the spinal cord, spinal meninges, and cauda equina are relatively rare, and a paucity of population-based data exist on tumors in these sites. This study intends to augment the current literature by examining incidence of these tumors on a national level. METHODS: Data from central cancer registries in the National Program of Cancer Registries (NPCR) and Surveillance, Epidemiology, and End Results (SEER) programs for 2004-2007 (covering 99.2% of US population) and 1999-2007 (covering 89.4% of US population) were analyzed. Analyses for diagnosis years 2004-2007 included cases of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors. Descriptive statistics including estimated age-adjusted incidence rates standardized to the 2000 US standard population were conducted for both malignant and nonmalignant primary spinal tumors from cases diagnosed during 2004-2007 as well as trend analyses on malignant cases of primary spinal tumors (n = 5103) for cases diagnosed during 1999-2007 using SEER Stat 6.6.2 software. RESULTS: There were 2576 cases of malignant primary spinal tumors and 9136 cases of nonmalignant primary spinal tumors in 2004-2007. The incidence of malignant and nonmalignant primary spinal tumors combined differed by age, sex, race, and ethnicity. Results of trend analyses indicated that malignant primary spinal tumors have been stable throughout the 1999-2007 period. CONCLUSIONS: This large population-based study adds new insights into the descriptive epidemiology of primary spinal cord, spinal meninges, and cauda equina tumors by providing in-depth analyses of the incidence of these tumors on a national level.

Tumors of central and peripheral nervous system associated with inherited genetic syndromes.

There are several genetic syndromes that predispose to the development of tumors of the nervous system. In the present study, we provide a review of the tumors that are associated with neurofibromatosis type 1, neurofibromatosis type 2, tuberous sclerosis complex, von Hippel-Lindau disease, Li-Fraumeni syndrome, Cowden disease, Turcot syndrome, nevoid basal cell carcinoma syndrome (Gorlin syndrome) and rhabdoid predisposition syndrome, which are the most common.

[Spinal tumors - part 2: intradural tumors. Epidemiology, clinical aspects and therapy]. / Spinale Tumoren - Teil 2: Intradurale Tumoren. Epidemiologie, klinische Aspekte und Therapie.

Intradural spinal tumors are classified in extra- and intramedullary tumors. The most frequent intradural-extramedullary tumors are meningiomas and neurinomas. Among the intradural-intramedullary tumors the most frequent ones are ependymomas and astrocytomas. Independent of their origin, spinal tumors usually manifest with progressive local or radicular pain and neurological deficits. The treatment of choice for most of these tumors is a complete surgical resection. In tumors with infiltrative growth into the intramedullary area, a marginal tumor tissue has to be left in situ in order to avoid additional neurological deficits. In particular if neurological deficits appear, a fast surgical intervention is indicated, since the prognosis depends on duration and severity of the preoperative existing deficits. Below, clinical presentation and relevant treatment options of spinal intradural tumors are discussed.

Survey of orbital tumors at a comprehensive cancer center in the United States.

BACKGROUND: The purpose of this study was to evaluate the frequencies of various types of orbital lesions seen at a comprehensive cancer center in the United States. METHODS: In this retrospective case series, we reviewed the medical records of 268 consecutive patients referred to our orbital oncology service for evaluation of an orbital mass between November 1998 and February 2009. Each orbital lesion was documented by CT and/or MRI, and in most cases diagnosis was established with a tissue biopsy. Patients who were seen for second opinions and had inadequate follow-up data were excluded, as were patients with thyroid eye disease or orbital hemorrhage. RESULTS: The study included 134 men and 134 women aged 1 to 89 years at diagnosis (median, 55 years). Follow-up ranged from 0.06 to 192 months (median, 15 months). Of the tumors, 171 (64%) were primary orbital, 69 (26%) were secondary orbital, and 28 (10%) were metastatic tumors. Lesion types were as follows: secondary orbital tumors, 69 (26%); lymphoproliferative lesions, 68 (25%); metastases, 28 (10%); epithelial lacrimal gland tumors, 27 (10%); inflammatory conditions, 21 (8%); vascular lesions, 20 (7%); mesenchymal tumors, 18 (7%); optic nerve and nerve sheath tumors, 7 (3%); peripheral nerve tumors, 3 (1%); histiocytic lesions, 3 (1%); cystic lesions, 3 (1%); and other lesions, 1 (<1%). The most common histopathologic diagnoses were lymphoma, 50 cases (19%); orbital extension of sinus tumor, 25 (9%); lacrimal gland adenoid cystic carcinoma, 18 (7%); cavernous hemangioma, 15 (6%); orbital extension of brain tumor, 14 (5%); idiopathic orbital inflammation, 14 (5%); plasmacytoma, 8 (3%); reactive lymphoid hyperplasia, 7 (3%); metastatic breast cancer, 7 (3%); orbital extension of ocular adnexal basal cell carcinoma, 7 (3%); orbital extension of ocular adnexal melanoma, 6 (2%), rhabdomyosarcoma, 6 (2%); metastatic gastrointestinal cancer, 5 (2%); sarcoidosis, 5 (2%); and other less common lesions. Forty-two lesions (16%) were intraconal, and 226 (84%) were extraconal. There were 169 (63%) malignant tumors and 99 (37%) were benign tumors. The rate of malignant lesions was 65% in children and 63% in adults (≥18 years). Malignant conditions encountered at a higher rate than previously reported included lymphoma (19% vs 8% to 13%), secondary orbital tumors (26% vs 13% to 20%), orbital metastases (10% vs 2% to 7%), and malignant epithelial lacrimal gland tumors (9% vs 2% to 3%). CONCLUSION: Our findings highlight the distinctive nature of the orbital oncology experience at a comprehensive cancer center. We found higher than previously reported rates of malignant tumors (63% of all tumors), particularly secondary tumors, malignant epithelial lacrimal gland tumors, and orbital metastases.

Clinical presentation, histology, and treatment in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina.

OBJECT Patients having a primary tumor of the spinal cord, spinal meninges or cauda equina, are relatively rare. Neurosurgeons encounter and treat such patients, and need to be aware of their clinical presentation, tumor types, treatment options, and potential complications. The purpose of this paper is to report results from a series of 430 patients with primary intraspinal tumors, taken from a larger cohort of 9661 patients with primary tumors of the CNS. METHODS Extensive information on individuals diagnosed (in the year 2000) as having a primary CNS neoplasm was prospectively collected in a Patient Care Evaluation Study conducted by the Commission on Cancer of the American College of Surgeons. Data from US hospital cancer registries were submitted directly to the National Cancer Database. Intraspinal tumor cases were identified based on ICD-O-2 topography codes C70.1, C72.0, and C72.1. Analyses were performed using SPSS. RESULTS Patients with primary intraspinal tumors represented 4.5% of the CNS tumor group, and had a mean age of 49.3 years. Pain was the most common presenting symptom, while the most common tumor types were meningioma (24.4%), ependymoma (23.7%), and schwannoma (21.2%). Resection, surgical biopsy, or both were performed in 89.3% of cases. Complications were low, but included neurological worsening (2.2%) and infection (1.6%). Radiation therapy and chemotherapy were administered to 20.3% and 5.6% of patients, respectively. CONCLUSIONS Data from this study are suitable for benchmarking, describing prevailing patterns of care, and generating additional hypotheses for future studies.