Síndrome de Good, una causa infrecuente de diarrea crónica / Good Syndrome, a rare cause of chronic diarrhea

Chronic diarrhea is a frequent cause of consultation in daily clinical practice. There are multiple diagnostic algorithms that allow a staggered approach to the most frequent pathologies, leaving out some lesser-known ones. This article reports the case of a 66-year-old female patient with a history of arterial hypertension, dyslipidemia and resected AB thymoma and a history of chronic diarrhea of 8 weeks of evolution. The etiological study ruled out infectious causes, celiac disease and negative viral serology. Due to a history of thymoma, immunoglobulin count was performed, showing severe pan-hypogammaglobulinemia. Good's Syndrome is the combination of thymoma and hypogammaglobulinemia, where patients may present with diarrhea secondary to immunodeficiency. Hypogammaglobulinemia associated with the presence of a thymoma is a rare cause but widely described in the literature as Good's Syndrome. Therefore, it seems relevant to describe a case, its approach and subsequent management.

La diarrea crónica constituye una causa frecuente de consulta en la práctica clínica diaria. Existen múltiples algoritmos diagnósticos que permiten realizar un abordaje escalonado de las patologías más frecuentes y permiten descartar algunas menos conocidas. En el presente artículo se reporta el caso de una paciente de género femenino de 66 años, antecedentes de hipertensión arterial, dislipidemia y timoma AB resecado con historia de diarrea crónica de 8 semanas de evolución. Dentro del estudio etiológico se descartan las causas infecciosas, enfermedad celíaca y serologías virales negativas. Por antecedente de timoma, se realizó recuento de inmunoglobulinas, evidenciando una severa pan-hipogammaglobulinemia. El Síndrome de Good es la combinación de timoma e hipogammaglobulinemia, donde los pacientes podrían presentar diarreas secundarias a inmunodeficiencia. La hipogammaglobulinemia asociada a la presencia de un timoma es una causa poco frecuente pero ampliamente descrita en la literatura como Síndrome de Good. Por lo antes señalado, nos parece relevante describir un caso, su abordaje y manejo posterior.

Síndrome Sjögren y penfigoide de las membranas mucosas en paciente con antecedente de timoma: Reporte de caso y revisión de literatura / Sjögren Syndrome and Mucous Membrane Pemphigoid in a Patient with a History of Thymoma: Case Report and Literature Review

RESUMEN Los timomas son las neoplasias mediastínicas más comunes en adultos. Los pacientes con timoma son frecuentemente diagnosticados con síndromes paraneoplásicos y generalmente están asociados con varias condiciones autoinmunes. El síndrome de Sjögren (SS) es un trastorno inflamatorio autoinmune, que se caracteriza por la destrucción mediada por linfocitos de las glándulas exocrinas, que conduce a la ausencia de secreción glandular, en particular las salivales y las lagrimales; la hiposalivación puede variar desde una ligera reducción en el flujo de saliva con molestias transitorias, a un grave deterioro de la salud oral y malestar psicológico. El penfigoide de membranas mucosas (PMM) es una enfermedad autoinmune crónica, muestra predominante afectación de la mucosa, afectando con mayor frecuencia la cavidad oral, seguida de la conjuntiva, la cavidad nasal y el esófago; intraoralmente incluye gingivitis descamativa, vesículas, erosiones cubiertas por pseudomembranas y úlceras. El objetivo de este trabajo es presentar el primer caso de una paciente con antecedente de timoma, la cual, conjuntamente presentó SS y PMM; además evidenciar la importancia del manejo multidiciplinario para un diagnóstico preciso y temprano, ya que así, aumenta la eficiencia y la eficacia de la estrategia de tratamiento.

ABSTRACT Thymomas are the most common mediastinal neoplasms in adults. Patients with thymoma are frequently diagnosed with paraneoplastic syndromes and are generally associated with several autoimmune conditions. Sjögren's syndrome (SS) is an autoimmune inflammatory disorder, characterized by the lymphocyte-mediated destruction of the exocrine glands, which leads to the absence of glandular secretion, particularly salivary and lacrimal; hyposalivation can vary from a slight reduction in the flow of saliva with transient discomfort, to a serious deterioration of oral health and psychological distress. Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity and the esophagus; intraorally includes scaly gingivitis, vesicles, erosions covered by pseudomembranes and ulcers. The objective of this work is to present the first case of a patient with a history of thymoma, which, jointly presented SS and MMP; also highlight the importance of multidisciplinary management for an accurate and early diagnosis, as this increases the efficiency and efficacy of the treatment strategy.

Timoma esclerosante: relato de caso / Sclerosing thymoma: case report

We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (< 1%), was diagnosed, thus confirming the first report in Brazil. The patient showed partial improvement of symptoms associated with myasthenia gravis.

Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%), sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.

Tumores del timo y cirugía / Tumors of thymus and surgery

Los tumores del timo constituyen menos del 1 por ciento de todas las neoplasias, y es la cirugía el tratamiento de elección. Objetivos: conocer el tipo de tratamiento quirúrgico y la magnitud de la resección, así como la relación con el tamaño del tumor, el sangrado, el tiempo quirúrgico, la morbilidad y la mortalidad. Métodos: se realizó un estudio prospectivo en 22 pacientes con tumor mediastinal, que tuvieron criterios de cirugía durante el ingreso en los servicios de neurología o cirugía general del hospital Hermanos Ameijeiras, desde enero de 2007 hasta febrero de 2009. Los resultados se presentan en por cientos y se empleó el chi cuadrado en la relación de variables. Resultados: 12 fueron del sexo femenino (54,5 por ciento ). El tratamiento más empleado fue la esternotomía total en 9 pacientes (40,9 por ciento ), el tiempo quirúrgico varió de 50 a 260 min con mediana de 127,5, mientras el sangrado por encima de 100 mL estuvo asociado a un tiempo quirúrgico de 61 a 180 min (p= 0,036). Se complicaron 11 pacientes (50 por ciento ) y hubo 1 fallecido (4,5 por ciento ). El tamaño del tumor varió de 3,5 a 20 cm. El paciente con tumor neuroendocrino recidivó a los 10 meses, mientras los pacientes con timomas no muestran hasta la fecha recidiva local ni se ha comprobado actividad metastásica. Conclusión: la cirugía constituye el paso más importante en el tratamiento de los tumores mediastinales, y se logra, en la gran mayoría, la resección completa, a pesar del tamaño y la relación con estructuras vecinas(AU)

Introduction: the tumors of thymus account for less than 1 percent of all neoplasms and the choice treatment is the surgery. Objectives: to know the type of surgical treatment and the magnitude of resection, as well as the relationship with the tumor size, bleeding, surgical time and morbidity and mortality. Methods: a prospective study was conducted in 20 patients presenting with mediastinum tumor with surgery criteria over the admission in the services of neurology or general surgery of the Hermanos Ameijeiras Clinical Surgical Hospital from January, 2007 to February, 2009. Results are showed in percentages and variables relation Chi2 was the test used. Results: twelve patients were of female sex (54.5 percent). The more applied treatment was the total sternotomy in 9 patients (40.9 percent), surgical time varied from 50 to 260 min with a mean of 127,5, whereas the bleeding over of 100 mL was associated with a surgical time from 61 to 180 min (p= 0.036). Eleven patients have complications (50 percent) with a death (4.5 percent). The tumor size varied from 3,5 to 20 cm. The patients with neuroendocrine tumor have a relapse at 10 months, whereas in patients with thymoma until now there was neither relapse nor metastatic activity. Conclusion: surgery is the more important step in the treatment of mediastinum tumors and in most of them it is possible a total resection despite its size and the relationship with neighbouring structures(AU)

Metastatic Thymoma of the Breast

Breast metastasis from nonmammary malignant neoplasms is uncommon, and it accounts for approximately 2% of all breast tumors. Distant metastasis of thymoma is very rare, and especially to extrathorcic areas. We report a female who had a metastatic thymoma in her breast 20 years after undergoing resection for a non-invasive thymoma. She presented with a palpable mass in her left breast. Mammography and ultrasonogram showed a lobular mass at the anterior glandular portion. Histological examination after surgical excision revealed a metastatic thymoma.

Ectopic cervical thymoma--the great mimic: a case report.

Ectopic cervical thymoma is a rare tumour that probably arises from thymic tissue trapped during the migration of the thymus into the anterior mediastinum from the third and fourth pharyngeal pouches. As it occurs in the region of the thyroid gland it mimics thyroid lesions and can cause confusion to the clinician, sonologist and cytologist. Misinterpretation of frozen sections can also occur if knowledge of this entity is not present. We are reporting a case of this rare entity in an elderly lady who presented with a neck mass that mimicked a thyroid lesion. Ectopic cervical thymoma should be considered in the list of differential diagnosis of neck masses in the elderly.

[Guillain-barre syndrome following surgery]

Thymoma is the most common neoplasm of anterior mediastinum with incidence peaks during the fourth, fifth and sixth decades of life. Two-thirds of patients are asymptomatic and identified accidentally by chest x-rays Of the total, 40-70% of patients have one of more parathymic syndromes of which Myasthenia Gravis [MG] is the most common and reported in 10-50% Gullain-Barre Syndrome [CBS] has not been reporterd as a parathymic presentation in present literature. We report a 37 years old woman who presented with CBS after thymoma resection. There is one question: Is there any association between thymoma and CBS or is it a complication of surgery [thymectomy]?

Bócio de tireóide intratorácico e timoma invasivo: apresentação simultânea incomum / Intrathoracic goiter and invasive thymoma: rare concomitant presentation

Apresentamos uma rara situação de ocorrência simultânea de dois tumores de mediastino com diferentes topografias e histologias, encontrados durante a ressecção de volumosa massa mediastinal em paciente assintomático. A possibilidade de diferentes tumores contidos numa mesma massa tumoral está relatada; entretanto, não encontramos na literatura médica relato de diferentes tumores em localizações distintas. Os bócios de tiróide intratorácicos e os timomas representam uma grande parcela dos tumores encontrados no mediastino. O tratamento cirúrgico, sempre que possível, desempenha papel fundamental na perspectiva de cura. A exploração cirúrgica minuciosa é fundamental para uma ressecção completa e possível achado de lesões concomitantes.

We present a rare situation in which two mediastinal tumors of different topology and histology were found during the resection of an extensive mediastinal tumor in an asymptomatic patient. Different histologies within the same mass have been reported, although, to our knowledge, there have been no reports of different tumors at distinct locations. Thymomas and intrathoracic goiters account for a large proportion of the tumors found in the mediastinum. When feasible, surgical resection plays a fundamental role in effecting a cure. In order to identify concomitant lesions and perform a complete resection, detailed surgical exploration is required.

Timoma linfocítico: informe de caso / Lymphocytic thymoma: a case report

La glándula tímica puede ser el sitio de origen de una gran variedad de neoplasias malignas y benignas. Aunque estos tumores son relativamente infrecuentes en la población general, son de gran interés para la literatura médica por sus manifestaciones clínico-patológicas. Se reporta un caso de una mujer de 52 años de edad quien presentó hipertensión arterial y ensanchamiento mediastinal en una radiografía de tórax (vista postero-anterior y lateral). La tomografía computarizada mostró una imagen hiperdensa, no homogénea por encima de la base cardíaca y área de la aorta. Se realizó mediastinotomía transesternal y fue extraído un tumor blando, amarillento, el cual fue después diagnosticado histológicamente como timoma linfocítico. Los principales signos y síntomas, diagnóstico, clasificación y tratamiento de los timomas son expuestos mediante una revisión de la literatura(AU)

The gland tímica can be the place of origin of a great variety of wicked and benign neoplasias. Although these tumors are relatively uncommon in the general population, they are of great interest for the medical literature for their clinical-pathological manifestations. A case of a 52 year-old woman is reported who presented arterial hypertension and ensanchamiento mediastinal in a thorax x-ray (seen postero-previous and lateral). The on-line tomografía showed an image hiperdensa, not homogeneous above the heart base and area of the aorta. He/she was carried out mediastinotomía transesternal and a soft, yellowish tumor was extracted, which was diagnosed histológicamente like timoma linfocítico later. The main signs and symptoms, diagnosis, classification and treatment of the timomas are exposed by means of a revision of the literature(AU)

Good's syndrome: an unusual cause of chronic diarrhea.

A 50-year-old man presented with recurrent episodes of pulmonary infections over a period of 5 years, chronic small bowel diarrhea and weight loss of 6 months' duration. On evaluation he was found to have a thymoma, intestinal infection with giardia, oral candidiasis, and low immunoglobin levels. He was diagnosed to have Good's syndrome. The patient refused further management.

Very Severe Aplastic Anemia appearing after Thymectomy

Aplastic anemia is a rare complication of thymoma and is extremely infrequent after thymectomy. We present a case of a 60-year-old woman with very severe aplastic anemia appearing sixteen months after thymectomy for a thymoma. She underwent thymectomy for a thymoma in April 2000. Preoperative examination revealed no hematologic abnormality. About sixteen months after the operation, she was readmitted because of pancytopenia with cough and fever. Bone marrow aspiration revealed a very severe hypoplasia in all the three cell lines with over 80% fatty tissue, and chest CT revealed no recurrence of thymoma. Her aplastic anemia had responded to cyclosporine A and granulocyte-colony stimulating factor (G-CSF).

Timectomía transesternal en miastenia gravis / Transternal thymectomy in myasthenia gravis

La Miastenia Gravis es una enfermedad poco frecuente caracterizada clínicamente por fatiga después del ejercicio repetido. Se genera por la producción de anticuerpos contra los receptores de acetilcolina los que son producidos por los linfocitos B del timo. Desde 1941, se está practicando la timectomía como tratamiento quirúrgico de estos pacientes. El presente trabajo describe una serie personal de 8 pacientes operados, entre diciembre de 1996 y agosto de 1998, en etapas IIB y IIC de la enfermedad. Hace una descripción clínica y epidemiológica de ellos, con especial énfasis en los resultados funcionales de la cirugía. De la serie de 8 pacientes, todos tienen una respuesta favorable y 6 se consideran curados de la enfermedad. Como conclusión del presente trabajo se desprende que la timectomía es una herramienta útil en el tratamiento de estos pacientes, sobre todo en aquellos menores de 50 años y con poco tiempo de evolución de la enfermedad

Pénfigo vulgar / Pemphigus vulgaris

El pénfigo es un grupo de desórdenes autoimmunes de la piel poco comúnes, en que el sistema inmunológico produce anticuerpos dirigidos contra las uniones intercelulares de la epidermis, lo que clínicamente se traduce en lesiones ampollares o ulceradas, semejantes a quemaduras. Se ha descrito que existiría cierta predisposición genética, presentando factores que gatillarían esta enfermedad. Epidemiológicamente tiene distribución mundial, afectando a hombre y mujeres entre los 30 y 60 años de edad principalmente. Se presenta un caso clínico y se hace una revisión bibliográfica del tema

Asociación entre bronquiectasias, timoma e hipogammaglobulinemia: síndrome de good / Association between bronchiectasis, thymona and hypogammaglobulinemia: good's a syndrome

Se presenta el caso de un paciente con bronquiectasias, hipogamaglobulinemia y timoma, entidad denominada Sindrome de Good. Mostranmos los aspectos clínicosde laboratorio, radiológicos y funcionales de este caso. Se revisa en la literatura la descripción de casos similares

Oligoartrite como síndrome paraneoplásica de timoma maligno: uma nova forma de apresentação? / Oligoarthritis as paraneoplastic syndrome of malignant thymona: a new form of presentation?

Existem diversas neoplasias que se apresentam inicialmente por meio de síndromes paraneoplásicas reumáticas. A presença de poliartrite aguda em pacientes idosos, acompanhada de comprometimento do estado geral, exige investigação de doença maligna subjacente. O objetivo deste relato é apresentar um caso de artrite reumatóide como manifestação inicial e paraneoplásica de carcinoma de timo. Trata-se de um paciente do sexo masculino, 32 anos, com diagnóstico de artrite reumatóide, que procurou atendimento médico em virtude do aparecimento de tosse seca. A história clínica revelou oligoartrite assimétrica de membros inferiores havia quatro meses, sem resposta adequada ao uso de antiinflamatórios não hormonais. Havia um mês vinha apresentando hipertemia leve, e havia 15 dias, tosse seca. O exame físico revelou baqueteamento digital não doloroso em extremidades superiores. Diante de um quadro caracterizado por dor intensa, efusão articular mínima, latéx positivo (1/80) e baqueteamento digital, pensou-se em síndrome paraneoplásica. A radiografia torácica evidenciou massas mediastinais, confirmadas através da tomografia axial computadorizada. O paciente foi submetido a tratamento cirúrgico e radioterapia, com melhora das manifestações articulares. O exame histopatológico confirmou o diagnóstico de carcinoma de timo. Sabe-se que percentual significativo de timomas são diagnosticados durante investigações de síndromes paraneoplásicas, enquanto o carcinoma de timo usualmente não está associado à síndromes paratímicas. Este caso ilustra a necessidade de alto grau de suspeição para o diagnóstico de neoplasias malignas

Myasthenia gravis and thymoma: evaluation of 41 patients

We avaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36 per cent) were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80 per cent of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.

Timoma linfocito : informe de un caso / Lymphocytic thymoma: a case report

Timoma Linfocitico : reporte de un caso. En este artículo se presenta un caso de timona Linfocitico en un paciente masculino de 18 años de edad, diagnosticado mediante biopsia mediastínica en el Hospital Dr. Luis E. Aybar, Santo Domingo, República Dominicana, el cual falleció seis días después de su ingreso en el Hospital Dr. Heriberto Pieter