Castleman's Disease
M Dakak, O Genç, S Gürkök, A Gözübüyük, A Özcan, K Balkanlý
Citation
M Dakak, O Genç, S Gürkök, A Gözübüyük, A Özcan, K Balkanlý. Castleman's Disease. The Internet Journal of Thoracic and Cardiovascular Surgery. 2002 Volume 5 Number 2.
Abstract
Castleman's disease (CD) was first recognized in the 1920s and was further described as a clinicopathologic entity in 1956. It is an unusual condition with idiopathic massive proliferation of lymphoid tissues. The disease remains as a clinicopathologic diagnosis and usually occurs in young adults. Three histological variants (hyaline vascular, plasma-cell and mixed) and two clinical types (localized and multicentric) of Castleman's disease have been described. Hyaline-vascular type (80%) is the most common form of the disease followed by plasma-cell type (20%) and the rarest form is mixed type. Although a variety of cutaneous manifestations in CD have been described, little is known about the specific cutaneous histological findings.
Introduction
Castleman's disease is a lymphoproliferative disorder secondary to lymphoid follicle hyperplasia and marked capillary proliferation with endothelial hyperplasia. The disease usually seen in young man and divided in two groups, hyaline vascular and plasma cell type. Hyaline vascular type is generally localized, plasma cell type is multisentric. The disease is seen with certain immune deficiency conditions. Localized type treated surgically and also in subtotal resections recurrence is unusual. For the reason is seen rarely, we wanted to introduce and discuss the one of the patients is diagnosed pathologically as Castleman's Disease.
Case Report
F.S. 21 year old male patient, coughing and fever was the chief complaint to take him to the hospital. There was no pathological sign in his physical examinations. Erythrocyte sedimentation rate was 120 mm/h, HIV serologically negative. Thoracic radiographic examination reveals, a well rounded 2.5x3x3 cm mass lesion in soft tissue density was in right hiler region (Fig. 1-3-4). Pulmonary digital subtraction angiogram showed, right axillary subclavian vein and vena cava superior, right atrium and ventricle and pulmonary arteries and branches were seen as normal (Fig. 2)The patient underwent right posterolateral thoracotomy in general anesthesia. There were a mass lesion in diameter of 3x3x3 in hilum adherent to intermedier bronchi, touching pulmonary artery and superior pulmonary vein. The mass excised from the hilum. In frozen section at peri-operatively was diagnosed lymphoid hyperplasia. Thoracotomy line closed anatomically. There were no postoperative complication and the chest tubes were removed 2 nd and 4th days postoperatively. The mass diagnosed histopathologcally hyaline vascular type Castleman's disease. The patient is now under observation.
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Discussion
Castleman's disease is a lymhoproliferative disorder secondary to lymphoid follicle hyperplasia and marked capillary proliferation with endothelial hyperplasia. It is a reactive condition rather than neoplasm. The disease arises upon distorted immunoregulations or unidentified antigenic stimulus (1). The histopathologic appearance is characteristic but not specific so seen in other conditions such as drug reactions, Sjogren syndrome and Hodgkin disease (2).Castleman disease is seen mostly in thorax (%64) also in abdomen (%14 retroperitoneum), neck and axilla. There are two forms. Hyaline vascular type (generally localized) and plasma cell type (generally multisentric), transition between two types histologically also seen. Hyaline vascular type is seen percent 90, the patients is usually asymptomatic (%58-97). Many of the patients identified in routine chest radiographic examinations with solitaire mediastinal mass. 70 percent of the patients under 30 years old and the disease is seen mostly in male than female (2). But in some review, opposite findings are claimed. In symptomatic patients dry cough, dyspnea, infection due to airway compression are seen (5). In our patient there were no pathologic sign on physical examination, fewer and dry cough was the only symptom. The lesions tend to grow slowly. There are dense capillary proliferations in affected lymph nodes. There are lymphocyte predominant infiltrations around little germinal centers. The disease subdivided clinically in two groups, the first localized or hyaline vascular type, the second systemic or plasma cell type. The localized form is seen as well rounded or lobulated mass on chest roentgenograms. But in plasma cell types, contrast enhancement may not seen as in localized forms (5). Calcification is seen 5-10 percent (5). Our patient had a well-rounded, soft tissue density mass in thorax CT and chest roentgenogram.
The localized form treated surgically. Also in subtotal resections, recurrence is rare, we excised the mass totally, in rare conditions, vascular neoplasm develops in Caposi sarcoma and Hodgkin disease (2). Plasma cell type seen percent 10, typically has generalized lymph node involvement (Thoracic, mesenteric, retroperitoneal) histologically there are plasma cell sheets around large germinal centers. There are no vascular proliferations in the pathogenesis of this condition, deregulations of interleukin VI is suspected (5). In this patients fever, weight loss, slight anemia, elevated erythrocytes sedimentation rates, polyclonal hypergamma globulunemia and hypoalbuminemia is seen, in this patients, Non Hodgkin lymphoma and Caposi sarcoma is usually arise. The patients are respectively in 5th and 6th decades(2), in their treatments steroids and chemotherapotic agents is used. Prognosis is not good. The mortality is 50 percent and average life is 26 month (2).Castleman's disease is seen rarely, in localized types the surgery establish complete remission. Especially in immunocompromised patients with a mass mediastinum or hilum, the Castleman's disease should be considered.
