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Resumen INTRODUCCIÓN: Los síndromes paraneoplásicos son manifestaciones a distancia de las neoplasias, que se originan por mecanismos secundarios pero no por invasión tumoral. Son poco frecuentes, anteceden a la manifestación propia del tumor y desaparecen con su curación. Si bien la miastenia gravis tradicionalmente se ha relacionado con timomas, aquí se busca reportar un caso de miastenia gravis como síndrome paraneoplásico de un tumor de ovario y hacer una revisión de la bibliografía disponible referente a su diagnóstico. CASO CLÍNICO: Paciente de 46 años, con múltiples episodios de debilidad generalizada e insuficiencia ventilatoria que requirió ventilación mecánica y estancia en cuidados intensivos. Se estableció el diagnóstico clínico de miastenia gravis. A pesar del adecuado tratamiento y de la timectomía, las crisis persistieron. Al poco tiempo se le diagnosticó un adenocarcinoma seroso de ovario que se trató con cirugía y quimioterapia. Con esto desaparecieron los síntomas neurológicos, con una remisión total después de cuatro años de seguimiento, sin tratamiento médico. Para la revisión bibliográfica se emprendió una búsqueda en Medline, Cochrane, LILACS, Google Scholar y literatura gris con los términos "myasthenia gravis AND ovarian cancer". Se encontraron 18 artículos de los que se incluyeron solo dos publicaciones para la revisión completa. CONCLUSIONES: Los síndromes neurológicos, entre estos la miastenia gravis, asociados con tumores de ovario a modo de síndromes paraneoplásicos son poco frecuentes y plantean la necesidad de incluir el estudio ginecológico en pacientes con miastenia gravis de manifestación atípica.
Abstract BACKGROUND: Paraneoplastic syndromes are distant manifestations of neoplasms originated by secondary mechanisms but not by tumor invasion. They are infrequent, precede the manifestation of the tumor itself and disappear when the tumor is cured. Although myasthenia gravis has traditionally been related to thymomas, here we report a case of myasthenia gravis as a paraneoplastic syndrome of an ovarian tumor and review the available literature on its diagnosis. CLINICAL CASE: 46-year-old patient with multiple episodes of generalized weakness and ventilatory failure requiring mechanical ventilation and intensive care stay. The clinical diagnosis of myasthenia gravis was established. Despite adequate treatment and thymectomy, the crises persisted. Soon after, she was diagnosed with serous adenocarcinoma of the ovary, which was treated with surgery and chemotherapy. With this, the neurological symptoms disappeared, with total remission after four years of follow-up, without medical treatment. For the literature review a search was undertaken in Medline, Cochrane, LILACS, Google Scholar and grey literature with the terms "myasthenia gravis AND ovarian cancer". Eighteen articles were found of which only two publications were included for the full review. CONCLUSIONS: Neurological syndromes, among this myasthenia gravis, associated with ovarian tumors as paraneoplastic syndromes are rare and raise the need to include gynecological study in patients with myasthenia gravis of atypical manifestation.
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Objective To discuss the diagnosis and inductive chemoradiotherapy of stage Ⅲ and stage Ⅳa invasive thymoma.Methods Clinical data of 13 cases with incomplete resected invasive thymoma were analyzed retrospectively,among which 8 cases were in stage Ⅲ and 5 cases in stage Ⅳa.All the 13 cases had invasion of major vessels,pericardial and lung tissues,or had metastasis of pleura and pericardium.Each patient got pathological diagnosis by fine needle aspiration,video-assisted thoracoscopic biopsy or anterior mediastinal biopsy.After 3 cycles of NP or CAP inductive chemotherapy and synchronous radiotherapy,tumors shrank and got palliative or complete surgical resection.The Follow up ranged from 2.8 to 9 years.Results All the 13 cases were malignant thymoma,including 4 cases of type B2,6 cases of type B3 and 5 cases of type C.7 patients got radical resection and 2 got palliative resection.9 patients survived more than 3 years after operation,7 patients survived more than 5 years,one patient even lived 8 years,and the other 4 patients without getting surgery died in 4 years.Conclusion Inductive chemoradiotherapy could shrink stage Ⅲ and stage Ⅳa invasive thymoma,reduce clinical stage,improve the resection rate,and prolong survival.
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Background: Mediastinal masses are relatively uncommon lesions that sometimes pose an interesting diagnostic and therapeutic problem for the clinician. Thymomas are one of the common mediastinal neoplasms and exhibit a wide spectrum of morphologic features and an unrivalled frequency of other autoimmune diseases. The great morphologic variability and heterogeneity in thymomas has rendered their histological classification difficult and highly controversial. Methods: This retrospective and descriptive study on thymoma was done in the department of pathology, Kasturba Medical College Mangalore (Manipal University), India over a period of five years from January 2006 to June 2011. Histopathology sections taken were stained with routine Hematoxylin and Eosin stains in every case. Additional stains and immunohistochemistry were done as required. Results: Total number of mediastinal lesions studied was 66, with thymomas making up 15 cases. The age range of patients with thymomas was 22 to 65 years with a mean of 48 years. The most common histologic sub-type of thymoma was B2. Type AB thymoma was associated with bad prognosis. Five cases of thymomas were associated with Myasthenia Gravis. All thymomas showed cytokeratin positivity. Reticulin fibers were seen around individual tumor cells in Type A thymoma while Type B2 showed around tumor nests. Conclusion: Thymomas are rare & interesting neoplasm located in the mediastinum. A histomorphological analysis aided by immunohistochemistry and radiology permits an exact diagnosis and also allows for differentiation between benign and malignant neoplasms.
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Los timomas representan el tumor más frecuente del mediastino anterior. El objetivo del trabajo es presentar las características clínico patológicas de los timomas tratados quirúrgicamente desde enero de 1971 hasta diciembre del 2008 en el Hospital de Rehabilitación Respiratoria María Ferrer, e investigar los factores que afectan la supervivencia. De los 131 casos analizados, 11 fueron perdidos de seguimiento (6%); de los restantes 120, 26 se hallaban fallecidos al final del estudio (21.7%). Se analizaron las siguientes variables: edad, sexo, síntomas de miastenia gravis al momento del diagnóstico, estadificación de Masaoka(1994) del tumor original, clasificación histológica de la OMS (1999), radioterapia postoperatoria y evolución clínica de la miastenia gravis según la clasificación modificada de Osserman. Fueron analizados 66 hombres (54%) y 56 mujeres (46%), con una mediana de edad de 48 años (rango13-78); 78 presentaban miastenia gravis al momento del diagnóstico (64%), y el 97% de ellos persistían sintomáticos luego del tratamiento quirúrgico. En el análisis univariado, la presencia de miastenia gravis al momento del diagnóstico (HR 0.527, IC 95% 0.243 - 1.141, p = 0.10), la evolución clínica de la enfermedad (HR 18.81, IC 95% 4.070 - 86.886,p = 0.000) y el estadio de Masaoka (HR 2.044, IC 95% 0.934 - 4.474, p = 0.074) se asociaron significativamente con la frecuencia de la muerte en pacientes con timoma. En el análisis multivariado, ese valor resultó ser casi 7 veces mayor en timomas invasores que en los estadios I y II (HR 7.272, IC 95% 2.19 - 24.11, p = 0.001), mientras que la radioterapia adyuvante disminuyó en un 79% este riesgo en nuestra población (HR 0.214 IC 95% 0.0648 - 0.7085, p= 0.12)...
Thymomas are neoplasias originating from epithelial cellsof the thymus. The aim is to present a serie of surgicallytreated thymomas at the Hospital de Rehabilitación Respi -ratoria María Ferrer from January 1971 to December 2008and to determine which factors affected the survival ofthese patients during the follow-up time. All patients withdiagnostic of thymoma surgically treated were included.A total of 131 cases were analized, 11 of which were lostto follow-up (6%); of the remaining 120, 26 were dead atthe end of the study (21.7%). The following variables wereanalyzed: age, gender, symptoms of myasthenia gravis atthe time of diagnosis, staging of Masaoka (1994) of theoriginal tumor, the 1999 WHO classification, postoperativeradiotherapy treatment and clinical course of myastheniagravis defined by the modified Osserman classification.Out of the 120 patients studied, 66 were men (54%) and 56female (46%), with a median age of 48 years old (range 13-78); 78/120 had myasthenia gravis at the time of diagnosis(64%), and 97% of them had persistent symptoms aftersurgical treatment. In univariate analysis, symptoms ofmyasthenia gravis at the time of diagnosis (HR 0.527, 95%CI 0.243 - 1.141, p = 0.10), clinical course of myastheniaaccording to the Osserman classification (HR 18.81, CI95% 4.070 - 86.886, p = 0.000) and staging of Masaoka(HR 2.044, 95% CI 0.934-4.474, p = 0.074) showed a signi -ficant association with the frequency of death in patientswith thymoma. In multivariate analysis, the risk of deathin patients with invasive thymomas proved to be almost7 times higher than in stages I and II (HR 7.272, 95% CI2.19 - 24.11, p = 0.001), whereas the postoperative adjuvanttherapy decreased 79% this risk in our population (HR0.214, 95% CI 0.0648 - 0.7085, p = 0.012)...
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Humans , Mediastinal Diseases , Mediastinum , Thymoma , Mortality , Myasthenia Gravis , Thymus GlandABSTRACT
Objective To investigate the immunoregulatory role of Th17 cell and the related cytokines in myasthenia gravis.Methods Totally 51 myasthenia gravis (MG) patients were divided into MG with thymomas ( TM group) and the MG with normal thymus ( NT group),as well as 22 healthy subjects as controls.Th17 cells from peripheral blood mononuclear cells were measured by flow cytometry.Th17 related cytokines were detected by ELISA and real-time quantitative-PCR.Results The quantity of Th17 cells in MG patients with thymomas (1.53 ± 0.59 )％ were significantly increased compared with that of healthy control (0.94％±0.32％,P ＜ 0.05 ).There was no significant difference in the number of Th17 cells between healthy controls and NT group.The expression levels of IL-17 mRNA ( 23.7 ± 4.5 ) were upregulated significantly versus those in healthy controls (13.4 ± 3.2,P ＜0.01 ).The levels of mRNA expression of IL-1 β,IL-6 and IL-23 were up-regulated significantly in TM group.The mean concentration of IL-17 was up-regulated significantly in TM group (30.4 ±7.3) ng/L versus healthy controls [ ( 19.2 ±4.9)ng/L,P ＜ 0.05].Serum levels of IL-23 and IL-1β were always increased in TM group versus healthy controls.Conclusion The elevated levels of IL-17 and other Th17 related cytokines in thymomas may aggravate the autoimmunity disorder.
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Background: The numerous structures within the mediastinum, make it prone for development of various congenital cysts, benign & malignant neoplasms. Cystic lesions and small tumors are asymptomatic in half of the patients and symptoms develop as a result of compression or invasion. These tumors are thus discovered at very late stages, which proves fatal, or, are diagnosed incidently. Methods: We analysed all resected mediastinal tumor specimens received in Department of Pathology & from few other private laboratories in Rural and Urban areas, in & around Bareilly region of North India. In 10 years duration, a total number of 38 cases were examined. Information was collected from detailed gross & microscopic examination and correlated with that of previous studies. Results: Mediastinal tumors occur more in males, with higher frequency of malignant lesions than benign. Predominant tumors are thymomas occurring in middle age group(20-29yrs), followed closely by neurogenic tumors. Conclusion: This study stresses upon the importance of early screening of all patients with suspicion, with help of radiological modalities along with FNAC & mediastinoscopy. Possible causes of increasing incidence of thymomas in children younger than 16yrs age should be further evaluated.
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For the purpose of investigating the pattern of E-cadherin (E-CD) expression in thymomas, 72 cases were immunostained using monoclonal antibody (HECD-1) and microwave-enhanced immunohistochemical method on formalin-fixed, paraffin-embedded tissue sections. The thymomas were classified according to modified Muller-Hermelink classification. The spindle-shaped, medullary type tumor epithelial cells in medullary (3 cases) and composite type (20 cases) thymomas rarely expressed E-CD except in focal areas showing microcystic change observed in 8 cases. Meanwhile, the cohesive epithelioid tumor cells in every case of well-differentiated thymic carcinomas (WDTC) (29 cases) expressed E-CD. The epithelial cells in cortical type (13 cases) expressed stronger E-CD compared with those of organoid type (7 cases). In cases of WDTC admixed with cortical type, we observed increasing expression of E-CD as the tumor epithelium forms cohesive sheets. We could not find any loss of E-CD expression in invasive foci of the 11 cases of high-staged WDTC examined. Since the results of our study show a strong correlation between E-CD expression and epithelioid morphology of the tumor, E-CD seems to play a major role as a morpho-regulatory factor rather than as a suppressor of invasion in organotypic thymomas.