ABSTRACT
Thymic carcinoid is an uncommon component of Multiple Endocrine Neoplasia Type 1 (MEN1).The low penetrance and the high mortality made TH-NET one of the most fatal tumor components of MEN1.Here we are discussing the pathology and pathogenesis, as well as epidemiology, screening strategy, diagnosis, therapies, and analysis of prognostic factors of thymic carcinoid associated to MEN1.
ABSTRACT
Thymic carcinoid is a rare neoplasm with unclear risk factors and controversial classifications .Clinical manifestations vary from asymptomatic to many nonspecific symptoms , among which endocrinopathy seems to be as-sociated with poor prognosis .Image studies show no specificity both in CT and PET/CT, but are of great value in clinical staging .Ki67 index has been found to be a powerful tool for grading neuroendocrine tumors and further studies should be made .The diagnosis of thymic carcinoid mainly depends on pathology and immunohistochemistry plays a role in differential diagnosis .Radical resection is the first choice in treatment , and target therapy becomes possible with the development in molecular pathology .
ABSTRACT
Hombre de 54 años con antecedentes de enfermedad de Cushing 32 años antes de la consulta. Ingresó por edemas asociados a astenia y adinamia. En el laboratorio se constató hipopotasemia y alcalosis metabólica. Se realizó diagnóstico humoral de síndrome de Cushing secundario a secreción ectópica de hormona adrenocorticotropa (ACTH). En la tomografía de tórax se halló un tumor de 3 × 3 cm en el mediastino anterior. La anatomía patológica de la pieza quirúrgica fue compatible con un carcinoide tímico. Este paciente sufrió en dos oportunidades un síndrome de Cushing, la primera por enfermedad (adenoma hipofisiario) y la segunda vez por secreción ectópica de ACTH (SEA) una asociación no descripta, en nuestro conocimiento, en la literatura médica.
A 54-year-old man, with a history of Cushing’s disease diagnosed 32 years earlier, presented with edema, asthenia and general malaise. Abnormal laboratory studies depicted hypokalemia and metabolic alkalosis. A CT scan of the chest revealed a 3 × 3 cm tumor in the anterior mediastinum. The pathology was consistent with a thymic carcinoid. These findings led to a diagnosis of biochemical Cushing’s syndrome secondary to ectopic secretion of ACTH. Thus, this patient suffered twice of Cushing’s syndrome. The first instance was the consequence of an ACTH - secreting pituitary adenoma and the second of an ectopic secretion of ACTH. To the best of our knowledge this is the first such case reported in the medical literature.
Subject(s)
Humans , Male , Middle Aged , Thymus Neoplasms/complications , ACTH Syndrome, Ectopic/etiology , Neuroendocrine Tumors/complications , Cushing Syndrome , Thymus Neoplasms/diagnosis , ACTH Syndrome, Ectopic/diagnosis , Neuroendocrine Tumors/diagnosisABSTRACT
Thymic carcinoids are rare mediastinal tumours. These are aggressive tumours that often present late and have poor prognosis. Primary surgical treatment is recommended even in metastatic tumours since the role of adjuvant therapy is not well established. We present a case of metastatic thymic carcinoid managed with surgical excision.
Subject(s)
Adult , Carcinoid Tumor/pathology , Carcinoid Tumor/surgery , Humans , Male , Positron-Emission Tomography , Preoperative Care , Thoracotomy/methods , Thymectomy/methods , Thymus Gland/pathology , Thymus Gland/surgery , Thymus Neoplasms/pathology , Thymus Gland/surgery , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Primary neuroendocrine carcinoma of the thymus (formerly known as thymic carcinoids) are unusual tumors that account for 5% of all anterior mediastinal neoplasms. Thymic neuroendocrine carcinomas exhibit a much more aggressive behavior than those originating from other foregut locations and are associated with the ectopic ACTH syndrome in 38% of cases. Patients having a thymic neuroendocrine carcinoma usually present with symptoms such as chest tightness, dyspnea, a palpable neck mass and sometimes a weight gain. We report on 2 patients with thymic neuroendocrine carcinoma who showed different clinical manifestations each other. One presented with a typical. Cushingoid appearance and the other had symptoms of fatigue and weight loss which is a common symptom of malignant tumors.
Subject(s)
Humans , ACTH Syndrome, Ectopic , Carcinoma, Neuroendocrine , Dyspnea , Fatigue , Mediastinal Neoplasms , Neck , Thorax , Thymus Gland , Weight Gain , Weight LossABSTRACT
Ectopic ACTH syndrome is frequently caused by lung cancer and uncommonly by other tumors such as thymic carcinoid. For its treatment, early diagnosis and complete resection is irresponsible, but some cases are remained unlocalized in spite of all diagnostic modalities. Here we report a case of ectopic ACTH syndrome which was localized by PET but could not be localized by conventional technique. A tumor at thymic area was ACTH secreting thymic carcinoid which was operated but couldnt resect completly. Glucocorticoid hypersecretion was persisted with chemotherapy, radiotherapy, and ketoconazole treatment. Patient died of sepsis after 12 months of diagnosis.
Subject(s)
Humans , ACTH Syndrome, Ectopic , Adrenocorticotropic Hormone , Carcinoid Tumor , Diagnosis , Drug Therapy , Early Diagnosis , Ketoconazole , Lung Neoplasms , Radiotherapy , SepsisABSTRACT
The syndrome of ectopic production of ACTH by non-pituitary neoplasm occurs with various types of tumors. ACTH-producing thymic carcinoid tumor is rare and has not been reported in Korea. We have experienced a 36-year-old male patient with ACTH-producing thymic carcinoid tumor, presenting symptoms of generalized weakness and weight gain. Clinical presentation, radiologic and hormonal evaluation, and pathologic examination were compatible with ACTH-producing thymic carcinoid tumor. The immunohistochemical staining of the thymic carcinoid tumor showed positivity for ACTH. Primary and metastatic tumor and both adrenal glands were resected. Radiation therapy and chemotherapy were performed. The prognosis of this patient is thought to be poor.
Subject(s)
Adult , Humans , Male , ACTH Syndrome, Ectopic , Adrenal Glands , Adrenocorticotropic Hormone , Carcinoid Tumor , Drug Therapy , Korea , Prognosis , Weight GainABSTRACT
Until 1972, many carcinoid tumors of the thymus were not recognized as distinct lesions and were mistakenly labeled as variants of thymomas. Thymic carcinoid tumors are unusual neoplasms that show different morphological, functional, and behavioral characteristics than those of thymomas. We report a case of a 65-year-old woman with thymic carcinoid tumor. The cinicopathological findings are discussed with a review of the literature.