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J Thorac Cardiovasc Surg 2007;134:1376-1378
© 2007 The American Association for Thoracic Surgery
Brief Communication |
a Division of Thoracic Surgery, Department of Cardiac and Thoracic Surgery, University of Pisa, Pisa, Italy
b Division of Neurology, University of Pisa, Pisa, Italy.
Received for publication May 3, 2007; accepted for publication July 9, 2007. * Address for reprints: Marco Lucchi, MD, Division of Thoracic Surgery, University of Pisa, Via Paradisa 2, 56124 Pisa, Italy. (Email: m.lucchi{at}med.unipi.it).
Thymoma is an epithelial tumor that is frequently associated with autoimmune phenomena and paraneoplastic syndromes. The most frequent are neuromuscular1
and hematologic2 disorders; however, neurologic,3 dermatologic,4 ophthalmologic, and other disorders are reported.5 The surgical resection of the tumor and associated adjuvant therapies often result in an improvement or complete remission of the symptoms. On the other hand, a thymus-related syndrome sometimes develops months or years after the treatment of the thymoma.1 Moreover, the recurrence of thymoma may be accompanied by recurrence of the originally associated paraneoplastic syndrome, even after many years.
The association of 2 or more paraneoplastic syndromes is rare in any case. We report a unique case of invasive thymoma and 4 thymus-related syndromes occurring in the preoperative setting.
A 43-year-old man with recurrent fever and limb petechiae was admitted to the internal medicine division of our hospital. Serum platelet count was 16 x 103/µL, red cell count was 2.9 x 106/µL, and hemoglobin concentration was 8.2 g/dL. The diagnosis of pure red cell aplasia and idiopathic thrombocytopenic purpura was made.
The marrow biopsy showed 1) a well-represented granuloblastic group with hyperplastic phenomenon, without morphologic anomalies; 2) a low presentation of red cells with some erythroblastic cells, and 3) anomalies in the distribution of lymphoid cells.
The patient also presented with a rheumatologic disease characterized by intense pain localized at the fingers and arms, which was treated with nonsteroidal anti- inflammatory drugs. The anemia and thrombocytopenia were treated with transfusions.
A standard chest x-ray evidenced the presence of a voluminous mass of the anterior and superior mediastinum prominent on the right side. A chest computed tomographic (CT) scan confirmed the presence of an irregular mass (57 x 75 x 100 mm) in the anterior mediastinum, compressing the right vascular structures (Figure 1).
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During the preoperative period, the patient required transfusion of 3 units of platelets and 2 units of red cells. The preoperative blood test results were as follows: platelet count of 35 x 103/µL, red cell count of 3.39 x 106/µL, mean cell volume of 82.4 fL, and hemoglobin concentration of 10 g/dL.
The lesion was radically resected through a median longitudinal sternotomy. During the procedure, another unit of platelets was administered. The postoperative course was uneventful, and the patient did not require any further transfusion.
The pathologic study described a "Thymoma with rich lymphocritical component, extensively infiltrating the mediastinal fat. The capsula is infiltrated in many parts."
A recent pathologic review, according to the new World Health Organization classification, defined the tumor as "Thymoma B2 infiltrating the capsula and the mediastinal fat; Masaoka stage IIB." At the discharge 1 week later, the patient showed the following laboratory values: red cell count of 3.16 x 106/µL, hemoglobin concentration of 9.3 g/dL, hematocrit level of 26.9%, and platelet count of 372 x 103/µL. The patient was referred for mediastinal radiotherapy (50 Gy), but after 2 fractions he refused the treatment.
At the 3-month follow-up, the patient’s hematologic status was completely recovered. Myasthenia gravis was initially controlled with anticholinesterase and steroid therapy, and then the patient had a complete remission without drugs. The rheumatologic symptoms were still present, and the patient underwent long-term therapy with disease-modifying antirheumatic drugs.
At the last follow-up, 5 years after the operation, the chest CT evidenced the presence of a rounded, solid, 3-cm–wide lesion localized on the right mediastinal pleura (Figure 2).
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Paraneoplastic syndromes associated with thymomas are often characterized by an underlying autoimmune mechanism. Thymomas are often associated with defects of T-cell maturation, leading to tolerance breakage.1
Myasthenia gravis is associated with thymomas in 30% to 45% of the cases, sometimes as the first symptom. Pure red cell aplasia is present in 5% to 10% of the cases, and the association between thymoma and thrombocytopenia has been observed in a few cases.
In our patient, both of these improved in the immediate postoperative period, emphasizing their strict correlation to the tumor. Although the tumor appeared invasive at the CT, we judged it resectable and the patient not eligible for neoadjuvant chemotherapy because of the hematologic symptom.
Remission of pure red cell aplasia after thymectomy is reported in 25% to 30% of cases, but usually it requires some adjuvant therapies such as cyclosporine and antithymocyte globulin.2
The rheumatic symptom slightly decreased on the second postoperative day, but 1 month from the removal of the pleural recurrence, the pain was still present and the specific therapy (steroids and disease-modifying antirheumatic drugs) is ongoing.
Although some thymus-related disorders in patients with thymomas are well described in the literature, the presence of 4 syndromes in the same patient is unique and to our knowledge has never been reported.
It is advisable for physicians to exclude the possibility of a paraneoplastic thymoma–related syndrome whenever a patient with a thymoma does not have well-defined complaints.
References
This article has been cited by other articles:
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  M. Lucchi, F. Basolo, and A. Mussi Surgical treatment of pleural recurrence from thymoma Eur J Cardiothorac Surg, April 1, 2008; 33(4): 707 - 711. [Abstract] [Full Text] [PDF]
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