Cyclic ACTH-secreting thymic carcinoid: a case report and review of the literature

SUMMARY Cyclic Cushing's syndrome (CS) due to thymic carcinoid is a rare disorder. We report a case of cyclic CS due to ectopic adrenocorticotropic hormone (ACTH)-secreting atypical thymic carcinoid tumor and reviewed similar cases published in the literature. Our patient had hypercortisolemia lasting approximately one month, followed by normal cortisol secretion, with relapse one year later. Histopathology revealed an atypical ACTH-positive thymic carcinoid. Ectopic CS can be derived from atypical thymic carcinoids, which can be aggressive tumors with early relapse, suggesting that this type of tumor probably needs aggressive treatment.

Cyclic ACTH-secreting thymic carcinoid: a case report and review of the literature.

Cyclic Cushing's syndrome (CS) due to thymic carcinoid is a rare disorder. We report a case of cyclic CS due to ectopic adrenocorticotropic hormone (ACTH)-secreting atypical thymic carcinoid tumor and reviewed similar cases published in the literature. Our patient had hypercortisolemia lasting approximately one month, followed by normal cortisol secretion, with relapse one year later. Histopathology revealed an atypical ACTH-positive thymic carcinoid. Ectopic CS can be derived from atypical thymic carcinoids, which can be aggressive tumors with early relapse, suggesting that this type of tumor probably needs aggressive treatment.

Intrathyroidal thymic carcinoma misdiagnosed as a medullary thyroid carcinoma.

The intrathyroidal thymic carcinoma is a rare neoplasm. The probable origin of this neoplasm is the presence of ectopic thymic tissue or remnants of the third and fourth branchial arch. The case of a 49-year-old female with an initial diagnosis of medullary thyroid cancer is presented. When she was operated for regional recurrence, 16 years later, the pathology report demonstrates the presence of a intrathyroidal thymic carcinoma. Intrathyroidal thymic carcinoma is considered an independent type of thyroid carcinoma because this entity has specific clinical-pathological characteristics similar to thymic carcinomas and different prognosis than known thyroid carcinomas. We present the case of a patient initially treated as having a medullary thyroid carcinoma who, upon presenting recurrence, the presence of intrathyroidal thymic carcinoma was demonstrated.

El carcinoma tímico intratiroideo es una neoplasia rara. El origen probable de esta neoplasia es la presencia de tejido tímico ectópico o de restos del tercer y cuarto arcos branquiales. Se presenta el caso de una mujer de 49 años con diagnóstico inicial de cáncer medular de tiroides. Cuando fue operada por recurrencia regional, 16 años después, se demostró la presencia de un carcinoma tímico intratiroideo, que se considera un tipo independiente de carcinoma tiroideo debido a que tiene características clínico-patológicas específicas similares a los carcinomas tímicos y un pronóstico diferente a los carcinomas de tiroides conocidos. Este caso se trató inicialmente como carcinoma medular de tiroides y al presentar recurrencia se demostró la presencia de un carcinoma tímico intratiroideo.

Tumor carcinoide atípico solitario de timo: una entidad inusual que no debemos olvidar / Solitary atipic carcinoid tumor of the thymus: an unusual entity that we should not forget

Resumen: Los tumores neuroendocrinos primarios (NET) del mediastino son muy raros. Presentamos el caso de un tumor carcinoide atípico primario de timo. Un hombre de 52 años que fue a un examen médico porque se quejaba de tos no productiva sin hemoptisis. Se solicitó una radiografía de tórax donde se evidenció un mediastino notablemente ensanchado, con una tomografía de tórax que mostro una masa tumoral de 90 x 50 mm en el mediastino anterosuperior que comprime el arco aórtico y la arteria pulmonar sin un plano de clivaje adecuado. Debido a que el tumor era infiltrativo, se proporcionó escisión quirúrgica completa, quimioterapia y radioterapia al mediastino. El objetivo principal de nuestro estudio fue evaluar los hallazgos de CT y MRI con relación a la literatura mundial.

Abstract: Primary neuroendocrine tumors (NET) of the mediastinum are very rare. We present the case of a primary atypical carcinoid tumor of the thymus. A 52-year-old man who went to a medical examination because he complained of a non-productive cough without hemoptysis. A chest radiograph was requested where a markedly widened mediastinum was evidenced, with a thoracic tomography showing a tumor mass of 90 x 50 mm in the anterosuperior mediastinum that compresses the aortic arch and pulmonary artery without an adequate cleavage plane. Because the tumor was infiltrative, complete surgical excision, chemotherapy and radiotherapy were provided to the mediastinum. The main objective of our study was to evaluate the findings of CT and MRI in relation to universal literature.

Revisando un órgano olvidado: Evaluación del timo en PET-CT / Revisiting a Forgotten Organ: Thymus Evaluation in PET-CT

Resumen La pesquisa incidental de lesiones tímicas ha aumentado. Una adecuada aproximación a esas lesiones en la tomografía computada por emisión de positrones (PET-CT) es fundamental, pues se usa como parte de la mayoría de los procedimientos de planificación oncológica. Se han seleccionado casos representativos respecto de los aspectos más importantes de las imágenes de timo en PET-CT y cómo esa técnica puede contribuir a un diagnóstico preciso o a la planificación del tratamiento. Específicamente, presentamos una descripción general de las lesiones tímicas comunes y los imitadores de enfermedad, con énfasis en los hallazgos en PET-CT, incorporando también ejemplos de resonancia magnética (RM).

Abstract Incidental thymic lesion findings have increased. An adequate characterization of these lesions in positron emission computed tomography (PET-CT) is essential, since it is used as part of most oncological planning procedures. Representative cases have been selected regarding the most important aspects of thymus imaging in PET-CT and how this technique can contribute to an accurate diagnosis or treatment planning. Specifically, we present a general description of common thymic lesions and disease mimics, with an emphasis on PET-CT findings, also incorporating examples of magnetic resonance imaging.

Long-term survival of a patient with an inoperable thymic neuroendocrine tumor stage IIIa under sole treatment with Viscum album extract: A CARE compliant clinical case report.

RATIONALE: Thymic neuroendocrine tumor (TNET) is very rare and characterized by a tendency to invade adjacent structures, frequent metastasis, resistance to therapy, and a poor prognosis. Viscum album extracts (VAE) have shown immunological, apoptogenic, and cytotoxic properties. PATIENT CONCERNS: A 54-year-old Peruvian man was suffering from constant fatigue, cough, dyspnea, and fever for a couple of months. DIAGNOSES: He was diagnosed with TNET (12.8 cm × 10 cm × 7 cm) stage IIIa, G1. Due to the size and extensive invasiveness (vena cava superior, also obstructing 85% of its lumen, pericardium, and pleura), the TNET was inoperable. INTERVENTIONS: We report the case of this patient who declined chemotherapy and was treated instead with sole subcutaneous VAE 3 times per week for 85 months. No other tumor-specific intervention was applied. OUTCOMES: Quality of life (QoL) improved substantially. The patient returned to work, and the tumor remained stable for 71 months. Thereafter, the tumor progressed, and the patient died 90 months after initial diagnosis. Besides self-limited local skin reactions around the application site, no side effects occurred. LESSONS: This is an exceptionally good course of disease of an inoperable, large, obstructing, and invasive TNET with a reduced baseline condition (Karnofsky index: 50-60) due to pronounced symptoms. Given the considerable reduction of symptoms and improved QoL following the onset of VAE therapy and other reports describing long disease stability and improvement of the QoL using VAE in different cancer types, we presume that the VAE treatment was supportive in this case. As TNETs are rare and few trials are available, future treatments of TNETs using VAE should be carefully documented and published to help determine whether further investigation of the use of VAE in TNET treatment is worthwhile.

[Thymoma in childhood. A case report and review of literature]. / Timoma en edad pediátrica. Reporte de un caso y revisión de la bibliografía.

BACKGROUND: Mediastinal tumours in children are rare. Around 25% of them can be malignant. The thymoma is an uncommon neoplasm, and during adulthood it corresponds to 30% of anterior mediastinum tumours. The peak incidence is between 55-65 years. CLINICAL CASE: A case of lymphocytic thymoma case is reported in a 4 year old patient with no previous or associated symptomatology. There was only a volume increase on the anterior neck region. The neck radiography and neck and chest tomography confirmed an anterior mediastinal mass surrounding the aorta and vena cava, as well as multiple mediastinal lymph nodes CONCLUSIONS: Early diagnosis and complete resection are the basis for management and prognosis.

Initial Experience of 18F-FDG PET/MRI in Thymic Epithelial Tumors: Morphologic, Functional, and Metabolic Biomarkers.

PURPOSE: The aim of this study was to investigate the value of morphologic, functional, and metabolic biomarkers acquired concurrently at PET/MRI in patients with thymic epithelial tumors. PATIENTS AND METHODS: During 1 year, 9 patients with suspected thymic epithelial tumors at contrast-enhanced chest CT were prospectively enrolled and underwent preoperative 18F-FDG PET/MRI. Two chest radiologists prospectively reviewed the CT and MRI scans of PET/MRI in consensus, and 2 nuclear physicians reviewed the PET images. Visual assessment of the tumor morphology, functional biomarkers such as apparent diffusion coefficient from diffusion-weighted images, and metabolic biomarkers (including SUVmax, metabolic tumor volume, total lesion glycolysis, and heterogeneity index) were recorded. All patients underwent operation, and their pathologic reports served as the reference standard. RESULTS: Thymic epithelial tumors were demonstrated in all 9 patients at pathologic examination. Tumor contour (P = 0.012) and shape (P = 0.033) had an association with the World Health Organization subtype, and the presence of septum (P = 0.048) on MRI scans had an association with the Masaoka stage. In terms of functional and metabolic biomarkers, SUVmax (ρ = 0.683, P = 0.042) and SUV/apparent diffusion coefficient (ρ = 0.703, P = 0.035) correlated with the Masaoka stage. Metabolic tumor volume (P = 0.024), heterogeneity index (P = 0.024), and total lesion glycolysis (P = 0.048) were useful for classification between low- and high-risk thymic epithelial tumors. CONCLUSIONS: Although limited by the small number of patients enrolled, morphologic, functional, and metabolic biomarkers derived from PET/MRI scans were useful for the stratification of thymic epithelial tumors.

Sonographic characteristics of thymoma compared with mediastinal lymphoma.

Thymomas are uncommon neoplasms that are typically located in the cranioventral mediastinum in domestic species, which generally affects older animals. Medical records and sonograms of 50 animals diagnosed with either mediastinal thymoma (n = 35) or lymphoma (n = 15) between January 2005 and February 2012 were reviewed. Cases with definitive cytological and/or histological diagnoses of either thymoma or lymphoma were included in this study. Masses were characterized by echogenicity, presence or absence of cysts, shape, margination, vascularity, and presence of effusion. Many thymomas (57.1%) were sonographically cystic in appearance and nearly all were heterogeneous (94%) in echogenicity compared with lymphoma (P = .0028). Lymphomatous lymph nodes were more likely to be solid (80%) and were equally divided between hypoechoic (47%) and heterogeneous (53%) echogenicities. Sonographic findings of either internal cysts or a heterogeneous echogenicity in mediastinal masses may be suggestive of a thymoma.

Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients.

Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

Invasive thymoma in a child: a rare case report.

Thymomas are neoplasms of the anterior mediastinum and generally occur between the fourth and sixth decades of life. In children, they are rare, with few reported cases. We describe a 9-year-old boy with invasive thymoma treated successfully by surgery alone. The patient was previously healthy and under treatment for a community-acquired pneumonia. A chest radiograph showed an opacity at the left lung base, and thoracic computed tomographic scan showed a mass with thick walls and liquid content situated in the lingula with no cleavage plane with the mediastinum. The patient underwent a left anterolateral thoracotomy, which showed a mass extending from the anterior mediastinum, infiltrating the left upper lobe of lung, phrenic nerve, and pericardium. A left upper lobectomy and resection of the mediastinal mass and lymph nodes were carried out. The pathologic analysis showed it to be a type B3 fusiform-cell thymoma, infiltrating the left upper lobe and 1 peribronchial lymph node. A multidisciplined tumor group decided to observe the patient and followed with thoracic computed tomographic scans every 3 months. After 2 years of follow-up, the patient has no evidence of recurrent disease.

Characteristics associated with complete surgical resection of primary malignant mediastinal tumors.

OBJECTIVE: To identify preoperative characteristics associated with complete surgical resection of primary malignant mediastinal tumors. METHODS: Between 1996 and 2006, 42 patients with primary malignant mediastinal tumors were submitted to surgery with curative intent at a single facility. Patient charts were reviewed in order to collect data related to demographics, clinical manifestation, characteristics of mediastinal tumors and imaging aspects of invasiveness. RESULTS: The surgical resection was considered complete in 69.1% of the patients. Cases of incomplete resection were attributed to invasion of the following structures: large blood vessels (4 cases); the superior vena cava (3 cases); the heart (2 cases); the lung and chest wall (3 cases); and the trachea (1 case). Overall survival was significantly better among the patients submitted to complete surgical resection than among those submitted to incomplete resection. The frequency of incomplete resection was significantly higher in cases in which the tumor had invaded organs other than the lung (as identified through imaging studies) than in those in which it was restricted to the lung (47.6% vs. 14.3%; p = 0.04). None of the other preoperative characteristics analyzed were found to be associated with complete resection. CONCLUSIONS: Preoperative radiological evidence of invasion of organs other than the lung is associated with the incomplete surgical resection of primary malignant mediastinal tumors.