Ann Thorac Surg 2006;82:1901-1904
© 2006 The Society of Thoracic Surgeons
Case Reports
Acute Presentation of Thymoma With Infarction or Hemorrhage
Cameron D. Wright, MD*,
John C. Wain, MD
General Thoracic Surgical Unit, Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
Accepted for publication February 27, 2006.
* Address correspondence to Dr Wright, Blake 1570, Massachusetts General Hospital, Boston, MA 02114. (Email: wright.cameron{at}mgh.harvard.edu).
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Abstract
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The presentation of thymomas is variable; most are asymptomatic and others present with local compression symptoms or a parathymic syndrome. Rarely thymomas present as an acute emergency with severe chest pain from either infarction or hemorrhage of the tumor. This rare presentation usually leads the clinician initially away from the diagnosis of thymoma. We present 4 patients who presented with infarction (3 patients) and hemorrhage (1 patient) who were initially believed to have a lymphoma. Preoperative biopsies were unrevealing. All had a complete resection and were in the early Masaoka stage. There have been no recurrences in follow-up. The astute clinician should be aware of this unusual presentation. The prognosis seems to be good in patients who present with infarction or hemorrhage.
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Introduction
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Most thymomas are asymptomatic [1]. Symptoms referable to thymomas are usually due to either mass effect (ie, compression of the superior vena cava or expansion beneath the sternum) or a parathymic syndrome (eg, myasthenia gravis). Rarely thymomas may undergo infarction or hemorrhage, which leads to acute, atypical presentations with acute severe chest pain. This may lead the clinician away from thymoma or suggest a more aggressive tumor, which is not the case. There are several case reports on infarction and hemorrhage in thymomas, but no clinically oriented reviews of this unusual presentation [2–5].
From 1976 to 2006, 190 patients with thymoma underwent resection at the Massachusetts General Hospital, of which 4 presented clinically with chest pain and either infarction or hemorrhage (2%). The patient characteristics are shown in Table 1. Permission to review these patients was obtained from the Human Studies Committee on November 7, 2005, and individual consent was waived because this was a retrospective study. All 4 patients presented with acute chest pain. Standard roentgenograms suggested an anterior mediastinal mass in all, and 2 patients had pleural effusions (Fig 1). A malignant tumor other than thymoma was originally suspected in all 4 cases, either a lymphoma or a germ cell tumor in the men (Fig 2). All men had serum tumor markers sent and were normal. All patients had a preliminary biopsy first, prior to a resection due to the suspicion of lymphoma. Three patients had an anterior mediastinotomy and biopsy done and all were nondiagnostic. One biopsy showed necrotic tissue only, and two had normal thymic tissue with one also having areas of hemorrhage and granulation tissue. One patient had a core needle biopsy that just showed normal thymic elements (patient 4) (Fig 3). Both pleural effusions were sampled and had reactive cells only. The 4 patients went on to have resections performed through a median sternotomy. All the tumors seemed to be inflamed and edematous, and one was hemorrhagic as well. In 2 patients there was adherence of the tumor to the pericardium; thus the pericardium underneath the tumor was excised. Patient 2 also had pericarditis clinically and at operation. Her pericardium had changes consistent with fibrinous pericarditis on histologic examination. Patient 2, with an abscess in the necrotic tumor, had apparent invasion into the lung requiring partial lung resection to obtain a clean margin. Final pathology showed inflammatory adherence to the lung only. All patients were believed to have undergone a complete resection. Our preoperative diagnosis was infarcted and hemorrhagic thymoma in our four cases, because preliminary biopsies did not suggest lymphoma despite apparent adequate tumor sampling. All cases seemed readily resectable at exploration and were resected without further tissue sampling. Frozen sections were obtained on all four resected specimens and three (ie, the infarcted thymomas) were nondiagnostic, with 1 showing hemorrhage and thymoma. There were no postoperative complications. Two patients with Masaoka stage II tumors were referred to radiation oncologists who proceeded to administer adjuvant radiation. All patients remain free of disease so far.
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Fig 1. Chest roentgenogram from patient 1 demonstrating bilateral pleural effusions, left lower lobe atelectasis, and an anterior mediastinal mass.
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Fig 2. Computed tomographic chest scan of patient 3 demonstrating an anterior mediastinal mass with indistinct margins and heterogeneous interior, with a low density central area. Pathology confirmed an infarcted thymoma.
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Fig 3. Computed tomographic chest scan of patient 4 demonstrating an anterior mediastinal mass with indistinct margins and a high-density interior consistent with hemorrhage. Core needle biopsy was nondiagnostic, but final pathology showed an early stage thymoma with hemorrhage.
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Comment
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These four cases illustrate rare presentations of thymomas with either infarction or hemorrhage. The combination of the symptoms and computed tomographic scan findings seem to suggest a clinically, very malignant tumor, such as an aggressive lymphoma that can be misleading for the clinician. All patients had early-stage tumors that should have a good prognosis in contradistinction to the supposition at the original presentation. Astute clinicians should recognize this rare presentation to treat these types of patients in a proper fashion.
There have been several case reports of infarction of thymoma [3–5]. The key feature is the acute presentation with chest pain. Two of the cases were Masaoka stage I, and the stage was not recorded in the other case. Follow-up information was not provided. Hemorrhage of a thymoma is also quite rare with only three prior case reports, again with presentations of chest pain [2]. Two of these cases were Masaoka stage I and one case was stage II. Follow-up information was not provided. The differential diagnosis of an anterior mediastinal mass and acute chest pain includes true thymic carcinoma (World Health Organization type C), lymphoma, germ cell tumors, hemorrhagic cystic substernal goiters, hemorrhagic ectopic parathyroid adenomas [6], and a hemorrhagic hemangiopericytoma [7].
A large series of thymomas with cystic, hemorrhagic, and infarcted areas were recently reported, concentrating on the pathologic aspects of these cases [8]. Twenty-five cases among 600 thymomas had these pathologic findings with no symptoms, but only 13 presented with chest pain (2 %, 13 of 600). The areas of infarction were associated with ischemic necrosis and were always associated with vaso-occlusive and thrombotic changes in the adjacent tissue. Hemorrhagic areas were associated with cystic areas that showed severe acute and chronic inflammation with granulation tissue. Twenty-three of these cases were Masaoka stage I and 2 cases were stage III. The prognosis was excellent in the 14 patients who had recorded follow-up with no recurrences with a median follow-up of 9 years.
The issue arises as to whether induction therapy should be given to thymomas such as these as the computed tomography suggests an apparently invasive lesion. We would suggest if there is unequivocal evidence of invasion (ie, a cutoff of the innominate vein surrounded by tumor) and tissue diagnosis of at least a B type of thymoma, then induction therapy be given. However if a ready tissue diagnosis can not be achieved (which seems to be common), or if the computed tomography only shows indistinct margins (falsely suggesting invasion) in the setting of acute chest pain, then resection for diagnosis and treatment is appropriate. In the end, this is a difficult call to make, but this report should alert clinicians when faced with patients who present with severe acute chest pain to search for signs of infarction and hemorrhage that greatly diminish the ability of the computed tomographic scan to predict subtle signs of invasion.
The spectrum of the presentation of thymomas is quite broad, not only including anterior mediastinal tumors, but also other locations such as cervical, pleural, pericardial, and within the lung. Thymomas are commonly found as an incidental finding, but they may compress mediastinal structures causing cough, superior vena cava syndrome, and chronic dull chest pain. Various autoimmune syndromes may be present as well, such as myasthenia gravis, red cell aplasia, or hypogammaglobulinemia. Finally, one must keep in mind a rare presentation of thymoma that can be either with infarction or hemorrhage leading to a presentation as an emergency with severe acute chest pain.
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References
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Abstract
Introduction
