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A pulmonary perfusion defect was caused by an extremely large anterior mediastinal mass subsequently proved by I-131 imaging to be an intrathoracic extension of a cervical goiter. Substernal goiter is not known to be reported previously as one of the causes of a perfusion defect in lung scanning.
Substernal goiter with vena caval obstruction remains an interesting diagnostic and therapeutic problem. Since 85 to 90 percent of superior vena caval obstructions are due to a malignant disease, an intrathoracic goiter represents one of the benign, curable causes of this syndrome. This report describes the clinical course of a 44-year-old woman who presented with this entity and was successfully corrected by surgical intervention. A discussion of diagnostic, operative, and prognostic considerations is presented.
The authors are presenting a retrospective study on 218 retrosternal goiters operated between 1968 and 1991. 33% of the goiters were incidentally discovered on a plain X-ray of the chest. Symptoms of compression were present in 50.5% of patients and hyperthyroidism in 16.5%. Respiratory manifestations were more frequent and more severe in patients aged 70 and more. Moreover 90% of those old patients were symptomatic. Goiters migrated anteriorly in 57.7% of cases, posteriorly in 33.9%, both anteriorly and posteriorly in 5.5%. Type of migration was unknown in 2.5%. 3.7% were malignant. 27 patients with respiratory symptoms received corticosteroids to reduce the airway compression. Among 52 patients with hyperthyroidism, 36 were given antithyroid drugs. Among them, some received corticosteroid drugs in order to reduce risks of mediastinal compression. A simple cervicotomy was adequate in 92.7% of case and was completed by sternotomy in 7.3%. The operation was iterative for relapsing or forgotten thoracic goiters in 24 cases. Morbidity was slight even for sternotomized patients. Mortality was noted in 2 cases (0.9%) one of them operated on for poorly-differentiated and suffocating carcinoma of the thyroid. We advise a straightforward surgical attitude as a result of the slight morbidity and mortality, even in sternotomized patients in order to avoid severe compressive complications of the airways.
In a series of 363 patients operated for goiter, 60 were retrosternal (16.5%). The female incidence although less than in cervical goiters remains predominant (2.5:1). Most patients are in older age group, 68% being above 40 years. The majority of goiters are multinodular and of long duration. Bilateral (37%) and left sided location (43%) were common. The incidence of cancer was 7%, thyrotoxicosis was noted in 5 patients (8%). Most patients were symptomatic (97%). Diagnosis is easily done by physical examination, chest X-ray and thyroid scintigraphy. Computed tomography might be helpful. Retrosternal goiter is an indication to surgery except in high risk patients. The cervical approach has been used in 98% of cases. There were no post-operative death and no major complications (compressive hematoma, laryngeal nerve paralysis, hypocalcemia).
In a series of 363 patients operated for goiter, 60 were retrosternal (16.5%). The female incidence although less than in cervical goiters remains predominant (2,5:1). Most patients are in older age group, 68% being above 40 years. The majority of goiters are multinodular and of long duration. Bilateral (37%) and left sided location (43%) were common. The incidence of cancer was 7%, thyrotoxicosis was noted in 5 patients (8%). Most patients were symptomatic (97%). Diagnosis is easily done by physical examination, chest X-Ray and thyroid scintigraphy. Computed tomography might be helpful. Retrosternal goiter is an indication to surgery except in high risk patients. The cervical approach has been used in 98% of cases. There were no post-operative death and no major complications (compressive hematoma, laryngeal nerve paralysis, hypocalcemia).
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Parathyroid carcinoma is a rare tumor responsible for 0.5-5% of primary hyperparathyroidism. It is usually small (not more than 27 g) and the precise diagnosis of malignancy is made when local or distant metastases are found. We describe a case of a 37 yr old male presenting with a substernal goiter and no specific symptoms except hypertension. This mass had cysts and calcifications and it was in the anterior upper mediastinum. The patient had severe hypercalcemia (Ca greater than 14 mg/dl), high PTH levels and mild renal failure. Bone scanning showed signs of hyperparathyroidism. The patient was subjected to total thyroidectomy and removal of the mass en block. The tumor was circumscribed lobulated and mostly cystic. It weighed 1,200 g (380 g after evacuation of cysts) and measured 12 x 9 x 4.5 cm. Histologic examination showed a highly differentiated adenocarcinoma of parathyroid with metastasis in a regional lymph node. Almost 4 years later the patient is alive and well without hypercalcemia and without evidence of distant metastases.
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We reviewed the cases of 52 patients with substernal goiters to examine clinical presentation, workup, technique of removal, malignancy, and outcome. Half of the patients were asymptomatic; half had at least one compressive symptom. Chest film was the most used; computed tomography or magnetic resonance imaging was by far the most useful study. Thyroid scans often failed to show the intrathoracic goiter. Fine-needle aspiration was not helpful because of the gland's inaccessibility. Seventeen percent (nine) of the thyroids showed malignancy, 21% (11) including incidental papillary carcinomas. These were not identified by duration of goiter, symptoms, or fine-needle aspiration. Except for lymphomas, prognosis was good after resection. Removal was almost always accomplished via cervical incision, with low morbidity and no deaths. The threat of compression, the substantial chance of malignancy, and the safety of resection mean that the presence of substernal goiter is an indication for surgery.
The authors relate their experience with 7 cases of mediastinal goiter residual to a subtotal thyroidectomy for substernal goiter. The differential diagnosis with ordinary recurrence was based on the absence of connection with the cervical remnant. The reasons for surgical decision-making was mediastinal compression in 4 patients, hyperthyroidism in 1 patient and absent diagnosis in 1 patient; surgery was systematic in 1 asymptomatic patient. Sternal splitting incision was required in 6 patients: alone in 3, associated with cervical incision in 3 others; excision by an exclusively cervical route was possible in one patient. No malignancy was discovered. Postoperative outcome was uncomplicated in all patients. The residual goiter has the same clinical and paraclinical presentation as the ordinary intrathoracic goiter; treatment should be principally surgical for the same reasons. Nevertheless, for this mediastinal tumor, sternum-splitting incision will be required in most cases.
PURPOSE: To review the diagnosis and management of thyroid diseases, both metabolic (thyroid storm and myxedema coma) and anatomic (substernal goiter, lymphoma, anaplastic carcinoma) that may require intensive care therapy. DATA SOURCES: English-language articles were identified through a search of the MEDLINE and Index Medicus databases. Bibliographies of retrieved articles were examined for relevant articles. STUDY SELECTION: Approximately 250 articles were reviewed. Those articles deemed most representative were utilized. Case reports were included to highlight rare, but potentially lethal complications. DATA EXTRACTION: All data were analyzed by one observer. Limitations of the data are discussed. DATA SYNTHESIS: Guidelines for determining the etiology of thyrotoxic crisis are outlined. Criteria for distinguishing critically ill hypothyroid patients from those patients with the euthyroid sick syndrome are given. Therapy for both disorders must be aggressive and multifaceted, and detailed management is indicated. Substernal goiter is almost always benign, but may cause various acute complications, including dyspnea, respiratory failure, superior vena caval syndrome, esophageal varices (downhill), and others. Surgery almost always corrects the problem. Lymphomas present with rapid thyroid enlargement. Recent studies suggest that surgery may assume more of a supportive role, used principally to obtain adequate tissue for histologic classification. CONCLUSIONS: Thyroid disorders can produce emergencies requiring the attention of multiple medical and surgical specialists. While management is often successful, future studies should address the following three areas: a) optimization of thyroid hormone dose for treatment of myxedema coma; b) clarification of the role of radiotherapy and/or chemotherapy for thyroid lymphoma; and c) more effective therapy for anaplastic carcinoma.
An 81-year old man who had a plasmacytoma of the ileum presented with melena for which no bleeding site could be established before surgery. He developed intussusception and the tumor was located at laparotomy. Metastases to the skin and axillary lymph nodes were documented prior to his operation, to mesenteric lymph nodes at surgery and to intrathoracic lymph nodes and a substernal goiter at autopsy. The liver, spleen, bones and marrow were not involved by myeloma or amyloid. Immunoelectrophoresis demonstrated an Ig-A (lambda) monoclonal protein in the serum (with nondiagnostic serum zone electrophoresis patterns) and lambda light chains in the urine. Immunoflourescense of Ig-A (lambda) globulin was present in imprint preparations from the bowel tumor.
Goitres of the thyroid gland whose major component resides substernally would appear to have a different presentation and management protocol than those goitres that reside mainly in the neck. These goitres, as opposed to their supraclavicular counterparts, usually present with symptoms referrable to compression of the trachea or esophagus. Even in those patients who are asymptomatic, a precise history may elicit pertinent findings. Furthermore, these goitres do not respond to suppression and require surgical removal. The trans-cervical approach is the most desirable, but the surgeon must be willing to enter the chest on that rare occasion. We reviewed the charts of 938 patients undergoing thyroid surgery who were registered in the Head and Neck Tumour Registry of the University of Toronto. Approximately 2.4% of patients had substernal goitres. Their history, pathology and ultimate surgical management will be discussed.
A patient with a superior mediastinal mass on an admission chest radiograph was initially evaluated by an 131I thyroid scan which failed to demonstrate a substernal thyroid. However, the tomographic 67Ga scan clearly showed an abnormal uptake in the area corresponding to the mass lesion on radiographic examination. Subsequent resection and biopsy of the substernal mass revealed a poorly differentiated follicular carcinoma with foci of anaplastic carcinoma. The differential diagnosis of the anterior mediastinal mass and the usefullness of the tomographic gallium scan are briefly discussed.
A case of varices of the entire esophagus in a patient who had undergone resection of a substernal thyroid gland 29 years previously is reported. Dilated esophageal veins, varices, may serve as collateral channels between the portal and systemic systems. When the superior vena cava is obstructed, blood from the upper extremities and head is returned to the heart via downhill varices. Obstruction of the superior vena cava proximal to the azygos vein results in varices of the upper esophagus. Obstruction distal to the asygos vein, as in this patient, results in varices of the entire esophagus. Portacaval shunt has no place in the management of these patients. Downhill varices should be considered in any patient with superior vena caval obstruction from any cause.
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