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J Thorac Cardiovasc Surg 2006;131:e1-e2
© 2006 The American Association for Thoracic Surgery
Brief Communication |
a Department of Thoracic and Vascular Surgery, Evangelismos General Hospital, Athens, Greece
b Department of Pathology, Evangelismos General Hospital, Athens, Greece
c Department of Chest Disease and Intensive Care Unit, University of Athens, Evangelismos General Hospital, Athens, Greece.
Received for publication August 30, 2005; accepted for publication September 9, 2005. * Address for reprints: Charalambos Zisis, MD, PhD, 17a, Patriarchou Grigoriou str, 166 74 Glyfada, Greece (Email: chzisis{at}otenet.gr).
Primary mediastinal Hodgkin disease (HD) is usually located in the middle or anterior mediastinal compartments and is the most common mediastinal lymphoma,
1
whereas fibrosing mediastinitis is a benign, slowly progressing inflammatory process characterized by excessive fibrotic tissue in the mediastinum. The disease has a tendency to invade and obliterate normal structures such as pulmonary arteries, veins, trachea, main bronchi, superior vena cava, and esophagus.
2
Imaging and clinical and histopathologic features sometimes overlap with HD.
Clinical Summary
A 36-year-old white man had a slowly growing mass on the right anterior thoracic wall present during the previous 6 months. The patient was free of symptoms, and the chest x-ray film showed mediastinal widening. A computed tomographic scan of the chest revealed a large mediastinal mass occupying the whole anterior mediastinum, invading the anterior thoracic wall and the soft tissues (muscles, subcutaneous fat), and extending into both hemithoraces (Figure 1).
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Histologic examination showed an inflammatory and fibrotic process with the presence of T and B lymphocytes, compatible with sclerosing or fibrosing mediastinitis.
Median sternotomy was performed and a tumor solid and hard in consistency was found to be densely adherent to the aorta, proximal superior vena cava, and brachiocephalic artery, with invasion of the pericardium. A large part of the tumor was excised, including part of the pericardium. The histologic examination showed dense fibrotic tissue containing inflammatory cells with patchy distribution, as well as aggregates of epithelioid cells. The most important finding was the presence of a few mummified cells, as well as very few cells with features of lacunar cells (Figure 2).
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The above findings were consistent with the diagnosis of Hodgkin lymphoma nodular sclerosis type, grade I, according to the World Health Organization classification.
Discussion
Nodular sclerosing HD is the most common subtype of HD and has a predilection for the anterior mediastinum, especially the thymus. HD presents as lymphadenopathy or as a discrete mass. Superior vena cava syndrome and chest wall invasion are uncommon.
Although suspicion of the disease can arise from the imaging features, the histologic confirmation may prove strenuous. As nodular sclerosing HD is characterized by a large inflammatory cell reaction within a fibrotic stroma, extensive fibrosis may be the dominant histologic feature. Choice of the suitable surgical access is a difficult point at the decision-making process. In many cases the histologic characteristics of the tumor mandate total resection, whereas for lymphomas a representative specimen is required for an accurate diagnosis to be achieved. Mediastinoscopy, mediastinotomy, video-assisted thoracic surgery, thoracotomy, and even sternotomy are the alternatives; a careful and paced procedure is essential to fulfill our diagnostic and therapeutic aims.
3
Nearly 10% of the patients with primary mediastinal lymphoma are free of symptoms and the disease is an incidental finding. In the remainder, compression symptoms from the mediastinal anatomic structures are present. It is extremely uncommon for such a sizeable tumor with extended invasion and palpable mass of the muscular and skeletal elements of the anterior chest wall to occur without any symptoms or even pain.
Histologically, the mass had features of mediastinal fibrosing mediastinitis. The previous biopsy specimens obtained showed extended fibrosis with inflammatory reaction. Neoplastic cells were not identified and the findings were compatible with a chronic sclerosing mediastinal disease. The clinical, radiologic, and histologic findings are similar to HD, and distinguishing between them can be very problematic. Such a case with HD masquerading as sclerosing mediastinitis has been reported once
4
; in that case biopsy from the celiac node had revealed the correct diagnosis of the nodular sclerosing type of HD.
The prognosis for patients with chest wall invasion from HD is suboptimal. Chest wall invasion is considered an adverse prognostic factor among clinical stage I-II Hodgkin lymphoma patients treated with combined modality therapy,
5
but in all published cases the patients had symptoms.
Conclusion
Distinguishing Hodgkin lymphoma from fibrosing mediastinitis may be very difficult, and safe documentation may demand aggressive surgical management to obtain the correct diagnosis. Thorough histopathologic study and representative tissue material are the prerequisites of an efficacious assessment.
References
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