Graves disease and metastatic hormonal-active Hürthle cell thyroid cancer: A case report.

RATIONALE: A hormone-active metastatic Hürthle cell thyroid carcinoma (HCTC) and Graves disease (GD) present a therapeutic challenge and is rarely reported. PATIENT CONCERNS: We present a 64-year-old male patient, who had dyspnea and left hip pain lasting 4 months. He had clinical signs of hyperthyroidism and a tumor measuring 9 cm in diameter of the left thyroid lobe, metastatic neck lymph node and metastases in the lungs, mediastinum, and bones. DIAGNOSIS: Laboratory findings confirmed hyperthyroidism and GD. Fine-needle aspiration biopsy and cytological investigation revealed metastases of HCTC in the skull and in the 8th right rib. A CT examination showed a thyroid tumor, metastatic neck lymph node, metastases in the lungs, mediastinum and in the 8th right rib measuring 20 × 5.6 × 4.5 cm, in the left acetabulum measuring 9 × 9 × 3 cm and parietooccipitally in the skull measuring 5 × 4 × 2 cm. Histology after total thyroidectomy and resection of the 8th right rib confirmed metastatic HCTC. INTERVENTIONS: The region of the left hip had been irradiated with concomitant doxorubicin 20 mg once weekly. When hyperthyroidism was controlled with thiamazole, a total thyroidectomy was performed. Persistent T3 hyperthyroidism, most likely caused by TSH-R-stimulated T3 production in large metastasis in the 8th right rib, was eliminated by rib resection. Thereafter, the patient was treated with 3 radioactive iodine-131 (RAI) therapies (cumulative dose of 515 mCi). Unfortunately, the tumor rapidly progressed after treatment with RAI and progressed 10 months after therapy with sorafenib. OUTCOMES: Despite treatment, the disease rapidly progressed and patient died due to distant metastases. He survived for 28 months from diagnosis. LESSONS: Simultaneous hormone-active HCTC and GD is extremely rare and prognosis is dismal. Concomitant external beam radiotherapy and doxorubicin chemotherapy, followed by RAI therapy, prevented the growth of a large metastasis in the left hip in our patient. However, a large metastasis in the 8th right rib presented an unresolved problem. Treatment with rib resection and RAI did not prevent tumor recurrence. External beam radiotherapy and sorafenib treatment failed to prevent tumor growth.

Verification of the diagnostic strategy for anterior mediastinal tumors.

BACKGROUND: For thymic epithelial tumors (TETs), the National Comprehensive Cancer Network guideline has suggested that complete excision of the tumor should be performed without a preoperative biopsy when resectable. However, little evidence has been provided to support this strategy. The purpose of this study was to review our diagnostic process and to evaluate the validity of radical resection of anterior mediastinal masses (AMMs) without pathological confirmation. METHODS: A total of 254 patients underwent surgical resection for AMMs between 2004 and 2015. This study included 181 patients with likely TETs according to clinical features, serum levels of tumor markers and autoimmune-antibodies, and radiological findings. In addition, AMMs likely TETs were classified into resectable or unresectable tumors. We retrospectively reviewed the diagnostic process of those patients and validated surgical resection of AMMs without a definitive diagnosis. RESULTS: Among 254 patients, 181 were suspected of having a TET based on the serum levels of tumor markers and autoimmune-antibodies and the radiological findings. Of them, 157 patients were deemed resectable and underwent surgical resection without histological confirmation, and 144 (92%) were diagnosed with TETs in the final pathological examinations. In 13 patients with non-TETs, the tumors were difficult to differentiate from TETs by imaging and clinical findings alone. CONCLUSIONS: A total of 92% of patients suspected of having a TET and who underwent complete resection without pathological confirmation were accurately diagnosed and properly treated. Surgical resection without a definitive diagnosis was feasible in patients suspected of having a TET when they were considered resectable.

Anterior mediastinal masses - A multidisciplinary pathway for safe diagnostic procedures.

OBJECTIVE: The objective of this study was to report our multidisciplinary diagnostic approach for patients with anterior mediastinal masses (AMM). METHODS: A retrospective review of patients with AMM at a tertiary pediatric surgical oncology centre (January 2011-December 2016) was performed. We analyzed data on clinical presentation, mode of tissue diagnosis, anesthetic techniques, and complications. RESULTS: Of the 44 patients admitted with AMM (median age 11 years, 27 males and 17 females), 22 had respiratory symptoms. Imaging revealed tracheobronchial compression in 26 children. Twenty patients had a lymph node biopsy. Ten patients had image-guided core biopsy of the mediastinal mass, and 2 had mediastinoscopic biopsy of a paratracheal lymph node. One patient with likely recurrence of a relapsed metastatic ethmoid carcinoma did not have a biopsy. The diagnosis was made from alternative tissues, such as pleural fluid in 4 and peripheral blood in 7 patients. Twenty-five anesthetics were assessed, as 14 patients required no or only local anesthesia, and 5 had unavailable anesthetic notes. Eighteen of 25 patients were anesthetized maintaining spontaneous breathing, mostly by means of ketamine sedation. There were no major anesthetic complications. CONCLUSION: Safe tissue diagnosis of anterior mediastinal masses can be obtained by a personalized multidisciplinary approach. Use of alternative tissues, local anesthesia, and ketamine sedation help minimize the need for general anesthesia, muscle paralysis, and controlled ventilation. LEVEL OF EVIDENCE: IV (Case Series with no Comparison Group).

[Transthoracal puncture biopsy under ultrasonic induction in tumors of the anterior mediastinum].

Differential diagnostics of tumors of anterior mediastinum is one of the most difficult problems of thoracic surgery and oncology. The authors conducted a retrospective analysis of the results of transthoracal puncture biopsy under ultrasonic induction in 44 patients with the tumors of the anterior mediastinum. Transthoracal biopsy was carried out using the device for biopsy (Multiple Biopsy Device-MBD-23) and ultrasonic scanner (Voluson 730 Expert), the patients being under intravenous (38 patients) and under local (6 patients) anesthetization. The diagnosis was made as a result of morphological research in all 100% of patients, the biopsy being performed the second time in one patient. The general information value of puncture biopsy under ultrasonic induction was 98%. A complication (localized pneumothorax, cupping off by itself) took place only in one of the follow-up study (2%). The analysis of presented material let the authors make a conclusion that transthoracal puncture biopsy under ultrasonic induction is a safe and highly informative method of diagnostics of tumors of the anterior mediastinum in selected patients.

Transsternal surgical biopsy of a mediastinal mass under local anesthesia.

We describe a minimally invasive transsternal surgical approach to obtain biopsies from retrosternal masses under local anesthesia. This original procedure was carried out in a patient with superior vena cava syndrome because she was unfit to undergo a CT-guided biopsy and at high risk for narcosis. In patients with such features this procedure could be preferable to conventional techniques. The transsternal approach is reliable, produces minimal trauma and no risk of pleural or vessel injury, and is very fast.

[Linear endobronchial ultrasound as the initial diagnostic tool in patients with indications of mediastinal disease]. / La ultrasonografía endobronquial lineal como instrumento de diagnóstico inicial en el paciente con ocupación mediastínica.

BACKGROUND AND OBJECTIVE: Linear endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has proven useful for sampling mediastinal masses and nodes and for staging lung cancer. The aim of this study was to assess the usefulness of this diagnostic tool in patients with indications of mediastinal disease that could not be diagnosed by noninvasive methods or white light bronchoscopy. PATIENTS AND METHODS: All patients undergoing linear EBUS-TBNA for the diagnosis of mediastinal masses and/or adenopathy at our endoscopy unit were included in the study. Diagnoses obtained by linear EBUS-TBNA or any surgical technique performed after a nondiagnostic EBUS-TBNA were considered as final. RESULTS: In the study population of 128 patients with a mean (SD) age of 62.0 (11.2) years, a total of 294 TBNAs were performed on 12 masses and 282 nodes. Satisfactory samples were obtained in 11 cases (91.7%) from masses and in 233 cases (82.6%) from nodes. Linear EBUS-TBNA was diagnostic, obviating the need for mediastinoscopy in 115 patients (diagnostic sensitivity, 89.8%). The technique confirmed the diagnosis in 85 of the 94 patients with cancer (90.4%), in 8 of the 10 patients with tuberculosis (80.0%), and in the 5 patients with sarcoidosis. CONCLUSIONS: Linear EBUS-TBNA is a useful diagnostic tool in patients with mediastinal disease for whom a pathologic diagnosis is not achieved by noninvasive methods or white light bronchoscopy.

The anaesthetic management of children with anterior mediastinal masses.

Children with anterior mediastinal masses may experience serious complications during general anaesthesia. We retrospectively surveyed the records of children with an anterior mediastinal mass who had been admitted to our hospital over a 7 year period. The presence of pre-operative symptoms or signs, findings of any special investigations performed and the anaesthetic outcome were noted. All radiological investigations were studied and tracheal compression measured. The majority of patients presented with severe clinical signs. There was a poor relationship between clinical signs and size of tumour or tracheal compression on CT scan. Corticosteroids were used prior to diagnosis in 33% of patients, all of whom were considered high risk. A clear diagnosis was made in 95% of these patients. The overall complication rate was 20% and 5% of patients had a serious complication related to anaesthesia. Stridor was the only sign that predicted an anaesthetic complication. Peri-operative respiratory complications were confined to patients with an isolated tracheal cross-sectional area less than 30% normal or less than 70% and associated with bronchial compression.

The anterior mediastinum: anatomy and imaging procedures.

The mediastinum is divided into compartments (anterior, middle, posterior) on the basis of lateral chest radiographs. Several anatomical and radiological classifications of the mediastinum are reported in the literature. Most mediastinal abnormalities are initially suspected following chest radiography; the need for further investigation and the most appropriate imaging modality are largely dictated by the tentative diagnosis made on this examination. Although routine chest radiography initiates the evaluation of mediastinal disorders, it is rarely diagnostic: notable exceptions are teeth or bones within a mass, which are diagnostic of a teratoma; air/fluid levels suggest an oesophageal origin, hernia, cyst, or abscess. Chest radiography is followed by spiral computed tomography (sCT). However, even sCT with contrast material is occasionally diagnostic (a confident diagnosis can be made of some lesions such as mature teratoma and mediastinal goiter) but is usually sufficient for preoperative evaluation before mediastinotomy or mediastinoscopy: it is instrumental in planning further diagnostic workup. In certain cases, magnetic resonance imaging (MRI) may be complementary to sCT, but its use is not considered routine. Besides, although the anterior mediastinum is suitable for sonographic examination, the diagnostic value of ultrasonography has not been fully exploited. Thyroid scanning with radioactive iodine is useful in identifying and evaluating masses of suspected thyroid origin. The role of fluorodeoxyglucose positron emission tomography (FDG-PET) in mediastinal diseases continues to be evaluated: it has potential for differentiating between benign and malignant disease and is expected to play a more extensive role in the imaging of mediastinal neoplasms in the future. In this paper, the radiological features of masses located in the anterior mediastinum are discussed, with particular reference to radiographic and CT patterns useful to the clinician's everyday practice.

Ganglioneuroma masquerading as spinal pain.

OBJECTIVE: To discuss the clinical features of ganglioneuroma and to propose it as a differential diagnosis for a young patient suffering from chronic back pain. CLINICAL FEATURES: A 25-year-old patient suffered from chronic mid-thoracic pain and a history of scoliosis. The physical examination result was unremarkable; thus radiographs were obtained. A posteroanterior and lateral chest radiograph demonstrated a well-defined opacity extending from the region of the left hilum to below the diaphragm. A differential diagnosis of a posterior mediastinal mass was advanced. Computed tomography revealed a homogenous, nonenhancing left posterior mediastinal mass with adjacent posterior rib deformity. Computed tomography (CT)-guided biopsy subsequently defined the mass as a ganglioneuroma. INTERVENTION AND OUTCOME: The mass was surgically resected. Although the patient experienced some postsurgical discomfort, she has fared well. CONCLUSION: Twenty percent of mediastinal tumors are neurogenic, and 10% of neurogenic tumors are ganglioneuromas. In spite of the rarity of this tumor, ganglioneuroma should be considered in the differential diagnosis of young patients suffering from back pain. The diagnosis is important to ascertain because surgical resection is curative and can relieve the symptoms.

Abnormal flow volume loops in patients with intrathoracic Hodgkin's disease.

STUDY OBJECTIVES: To study the incidence of upper airway obstruction, as measured on the flow volume loop (FVL), in patients with bulky mediastinal Hodgkin's disease; to correlate the FVL with CT of the chest; and to follow the changes in the FVL after treatment of the tumor. DESIGN: Retrospective study of pulmonary function tests (PFTs) and chest CTs performed as part of a clinical trial for Hodgkin's disease. SETTING: Memorial Sloan-Kettering Cancer Center, a comprehensive cancer care center. PATIENTS: Twenty-five patients (15 men and 10 women; age range, 20 to 57 years) with bulky mediastinal Hodgkin's disease enrolled in a clinical trial of chemotherapy followed by external beam radiation therapy. MEASUREMENTS AND RESULTS: Fourteen of 25 patients (56%) had an abnormal FVL prior to therapy; after chemotherapy, only 7 of 25 patients (28%) had an abnormal FVL. The abnormal patterns seen were either those typical of fixed obstruction or variable extrathoracic obstruction. No patient had a pattern typical of variable intrathoracic obstruction. On chest CT scan, 16 patients had grade-I tracheal deformity; 6 had grade-II deformity, and 3 had grade-III deformity. All three patients with grade-III deformity had a fixed obstruction pattern, as did three patients with a grade-I pattern. Patients with a fixed pattern on FVL had significant decreases in inspiratory and expiratory flow rates. CONCLUSION: FVL abnormalities suggesting upper airway obstruction occurred in > 50% of patients with bulky mediastinal Hodgkin's disease. A fixed pattern of obstruction was associated with the lower flow rates and severe tracheal distortion on CT; these patients may warrant special attention prior to general anesthesia or invasive procedures. Asymptomatic patients with abnormal FVLs but normal tracheal profiles need not undergo extensive evaluation. No patients showed the expected pattern typical of intrathoracic obstruction, but rather the major effect was on the inspiratory loop. The authors speculate on the mechanism for this unexpected finding.

MR imaging of the chest: mediastinum and chest wall.

This review presents the options and limitations of MRI in non-vascular diseases of the mediastinum and the chest wall. In numerous thoracic pathologies, MRI is a useful supplement to spiral CT. This imaging procedure also allows a contrast-media-free differentiation of solid tumors and vascular lesions (e. g., aortic aneurysms). The advantages of MRI over CT are particularly useful when multiplanar tumor imaging is required prior to surgery to establish the exact spatial relationship between tumor and the other mediastinal structures. Primary indications for MRI in diseases of the mediastinum and chest wall are therefore: (a) tumors of the posterior mediastinum for determining their position in relation to the neural foramina and the spinal canal; (b) chest wall tumors; (c) preoperative multiplanar imaging of primary mediastinal tumors; and (d) contraindications against CT exams with iodine contrast media.

[Diagnosis and staging of thoracic tumors]. / Diagnosi e stadiazione dei tumori toracici.

During the last 5 years researches, studies, training and practical experiences in both fields of diagnosis and therapy have been carried out with the specific aim to promote and support thoracic surgery in hospital departments where general surgery is mainly performed. Criteria and methods suggested as guidelines for the diagnosis and staging of thoracic neoplasms are herein shortly described. This phase has gained fundamental importance due also to the more and more frequent use of integrated therapy. The article reports the problems related to the III A-B stage of NSCLC, to the differential diagnosis between thymoma and lymphoma in mediastinal masses as well as the problems related to the diagnosis of mesothelioma. It is also acknowledged the great importance of VTS during the diagnostic phase, and in solving many thoracic problems.

Mediastinal tumors in children: experience with 196 cases.

BACKGROUND: Mediastinal masses are relatively common in infants and children. These lesions are often neoplastic in origin and have a high risk of malignancy. METHODS: This report concerns 196 infants and children with mediastinal tumors. Fifty-five cases (28%) were benign, and 141 (72%) were malignant. Diagnosis included Hodgkin's disease (47), neuroblastoma (46), non-Hodgkin's lymphoma (37), teratoma (18), ganglioneuroma (14), cystic hygroma (11), Schwannoma (five), germ-cell tumors (three), lipoma (three), thymic tumor (three), malignant histiocytosis (two), neurofibroma (two), mesenchymal sarcoma (one), rhabdomyosarcoma (one), peripheral neuroectodermal tumor (one), hamartoma (one), and hemangioma (one). Diagnoses were usually made by assessing the patient's age, radiologic evidence of tumor location, the presence of calcium in the tumor, and the presence of tumor markers (alpha-fetoprotein, vanillmandelic acid, human chorionic gonadotropin). Diagnoses were verified by histologic evaluation. Resection was the only treatment for benign tumors. Biopsy and chemotherapy (and/or radiation) were employed for lymphoid tumors, and resection and adjuvant therapy were used for other solid malignancies. RESULTS: Survival was achieved in 53 of 55 (96.3%) patients with benign tumors and 105 of 141 (74.4%) patients with malignant tumors. CONCLUSIONS: Seventy-two percent of mediastinal tumors in this study were malignant. Early diagnosis followed by biopsy and chemotherapy for lymphoid tumors or resection of nonlymphoid tumors along with aggressive adjuvant therapy result in high survival rates (74.4%). Children with benign tumors almost always survive (96.3%) after resection.

Local anesthesia for mediastinoscopy: experience with 450 consecutive cases.

Mediastinoscopy has been widely applied in the evaluation of patients with suspected bronchogenic carcinoma over the past decade. Though there remain those who feel that such findings should not play a significant role in determining resectability, we have found a number of patients who have been saved from exploratory thoracotomy in obviously incurable situations. We have had a relatively high degree of success in the diagnosis of benign diseases. Wtih no mortality and a morbidity of 1.1%, we feel that mediastinoscopy under local anesthesia is applicable in many clinical circumstances in which the requirement for general anesthesia would preclude such evaluation. Though it is not necessary that local anesthesia be exclusively applied, we feel that our experience with over 450 cases in the last six years has demonstrated safety and efficacy in universal application of local anesthesia for cervical mediastinal exploration.

Subxiphoid anterior mediastinal exploration.

A new surgical technique for anterior mediastinal exploration is described. The technique involves application of a Carlens mediastinoscope by means of a subxiphoid incision. The procedure provides complete exploration of the anterior mediastinal compartment and allows the surgeon to obtain a positive biopsy of the tumor or its lymph node metastases without sternotomy.