Ann Thorac Surg 1995;59:1162-1165
© 1995 The Society of Thoracic Surgeons
Giant Lymph Node Hyperplasia (Castleman's Disease) in the Chest
Joo Hyun Kim, MD,
Tae Gook Jun, MD,
Sook Whan Sung, MD,
Young Soo Shim, MD,
Sung Koo Han, MD,
Young Whan Kim, MD,
Chul Gyu Yoo, MD,
Jeong Wook Seo, MD,
Joon Ryang Rho, MD
Departments of Thoracic and Cardiovascular Surgery, Internal Medicine, and Pathology, Seoul National University Hospital, Seoul, South Korea
Accepted for publication January 27, 1995.
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Abstract
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We have experienced 7 cases of giant lymph node hyperplasia in the chest from 1981 to 1992. The ages of the 1 male and 6 female patients ranged from 9.9 to 40.4 years (mean age, 29.2 ± 10.4 years). In 4 patients, a mass was discovered in routine radiographs. Focal calcification suggesting continual enlargement over a long time was noted in 1 patient. The sites of lesions were unusual in 2 patients (intercostal space and intrapulmonary fissure). All patients underwent surgical removal of the mass. Five cases had typical features of the hyaline-vascular type, and 2 cases revealed a mixture of the hyaline-vascular type and the plasma-cell type. Follow-up was available in all patients (mean follow-up, 31.9 months). In 1 patient, recurrence was observed 9 years after surgical removal. In general, giant lymph node hyperplasia can occur anywhere in the chest, grow without symptoms, and recur in spite of complete resection. Surgical resection and close follow-up are advised.
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Introduction
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Giant lymph node hyperplasia (Castleman's disease) is a benign disease characterized by a peculiar form of lymph node hyperplasia [1, 2]. It can be found wherever lymph nodes are present, but 71% of the cases are located in the chest. The majority are located along the tracheobronchial tree in the mediastinum or lung hilus, but it also can occur in the intrapulmonary fissure or other spaces [3]. It also has been reported in the pelvis, neck, retroperitoneum, and muscle [2]. Keller and associates [3] distinguished two histologic types. The hyaline-vascular variant, the classic type described in 1956 by Castleman and associates, usually is a localized, asymptomatic, solitary mass that occurs most frequently in the mediastinum and is characterized histologically by small hyaline-vascular follicles and interfollicular capillary proliferations. The less frequent plasma-cell variant consists of large hyperplastic lymphoid follicles with intervening sheets of plasma cells. Systemic symptoms, such as fever, fatigue, sweating, anemia, hypergammaglobulinemia, arthralgia, thrombocytosis, and an elevated erythrocyte sedimentation rate may be associated with the disease. A systemic lymphoproliferative disorder histologically related to the plasma-cell variant and eventually following a progressive and fatal course has been described [4].
At Seoul National University Hospital, we have experienced 7 cases of giant lymph node hyperplasia in the chest. This report describes the clinical features and the outcome after surgical removal.
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Material and Methods
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Clinical and pathologic data were drawn from 7 patients who had surgical removal of an intrathoracic mass diagnosed as Castleman's disease at Seoul National University Hospital between 1981 and 1992. Detailed clinical data are shown in Table 1
. Six patients were female and 1 patient was male. Age at operation ranged from 9.9 to 40.4 years, with a mean of 29.2 ± 10.4 years. The ages of half of the patients, including 1 child, were less than 30 years.
No common clinical symptoms were found in any of the patients. One patient exhibited cough, another dyspnea, and a third chest pain. The other 4 patients were hospitalized due to the incidental discovery of a mass during routine radiographs (see Table 1
). None of the patients had any particular findings on physical examination and laboratory tests, even though all had computed tomographic (CT) scans. All patients underwent surgical removal of the mass to rule out malignancy due to the mass effect and had no further treatment.
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Results
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All lesions were solitary. The size of lesion varied from 3 to 7 cm in the CT scans (Table 2). The lesions presented as densely enhancing, homogeneous, well-marginated, soft-tissue masses and were located in various sites: lung hilus (4 cases), posterior mediastinum (1 case), intrapulmonary fissure (1 case), and intercostal space (1 case). Focal calcification suggesting gradual enlargement over a long time was noticed in 1 patient (patient 6).
Surgical removal was done by conventional thoracotomy. Four lesions were either encapsulated or at least relatively well circumscribed. For this reason, it was possible to excise easily only the mass. Adhesions to adjacent tissues, such as the pulmonary artery and the bronchus, existed in 3 patients (patients 3, 4, and 6). Even with these 3 patients, only the masses were excised, but with some difficulty. All lesions showed hypervascular features, and conspicuous bleeding occurred during one operation (patient 6). There were no postoperative complications.
Macroscopically, the masses were moderately firm and usually well encapsulated. The external surfaces were smooth and glistening; 2 showed a lobulated external appearance. The cut surfaces were homogeneous, glistening, and grayish pink. Necrosis was not seen. Lobulation was noted in 3 cases and focal calcification was noticed in 1 case.
Microscopically, 5 cases had morphologic features of the classic hyaline-vascular variant of Castleman's disease with small to medium-sized lymphoid follicles. In the center of a follicle, prominent vascular proliferation and hyalinization were observed. Concentric layering of lymphocytes occurred at the periphery of the follicle. Interfollicular stroma was characterized by numerous hyperplastic hyalinized small vessels and an admixture of plasma cells, eosinophils, and immunoblasts. Two patients (patients 6 and 7) revealed a mixture of the classic hyaline-vascular type and the plasma-cell type. Portions of plasma-cell type showed diffuse proliferation of plasma cells and lymphocytes in the interfollicular area. Deposition of amorphous eosinophilic material also was seen in 1 of these patients (patient 7); the other patient (patient 6) showed scattered atypical spindle cells and exuberant proliferation of endothelial cells.
In patient 6, the first thoracotomy revealed a 3 x 2-cm yellowish-pink mass, which appeared quite vascular and had infiltrated the adjacent lung parenchyme. Biopsy resulted in conspicuous bleeding at the biopsy site. The frozen section was reported erroneously as a normal lymph node and complete removal was not tried. In permanent microscopic sections the lesion was confirmed to be Castleman's disease. During a follow-up of 7 years, the mass progressively increased in size and amount of calcification (Fig 1). Thoracotomy was repeated and a hypervascular mass was removed. The mass exhibited adhesions to adjacent pulmonary artery and fingerlike penetrations into the minor fissure.
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Fig 1. . (Patient 6.) (A) Posteroanterior chest radiograph demonstrating a well-defined mass shadow in the right hilum. Small scattered calcifications in the mass are shown. (B) Chest computed tomographic scan after contrast enhancement. Conglomerated calcifications are shown inside the mass in the right hilar area.
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In patient 7, a left hilar mass was discovered incidentally in an asymptomatic 29-year-old woman in 1983. The CT scan displayed a round mass with a diameter of 6 cm in the left pulmonary hilum (Fig 2A). During the operation, a nonadherent, well-encapsulated mass measuring 7 x 5.5 cm was found. It extended into the interlobar fissure. Microscopic examination revealed the typical features of Castleman's disease (Fig 3A). In 1992, the patient had chest pain and radiographs showed recurrence in almost the same area. The CT scan revealed a mass with a tissue density similar to the distal portion of the left descending pulmonary artery (Fig 2B). Left thoracotomy revealed a 4 x 3.5-cm solid mass adhering to the pulmonary artery at the hilum and extending into the interlobar fissure like the previous one. The microscopic diagnosis was the same (Fig 3B).
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Fig 2. . (Patient 7.) Computed tomographic scan without contrast enhancement at the level of mass. (A) In 1983, a well-marginated, round, soft tissue mass lesion was observed in the left hilar area. (B) In 1992, a recurrent, soft tissue mass lesion was noted in the same area.
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Fig 3. . (Patient 7.) (A) Lymphoid tissue in 1983. Prominent vascular proliferation and diffuse proliferation of lymphoid cells are shown. (B) Lymphoid tissue in 1992. Marked proliferation of lymphoid follicles with central hyalinized vascular structures. (Both hematoxylin and eosin; x250 before 54% reduction.)
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Follow-up was available for all patients with a mean follow-up of 31.9 months (Table 2).
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Comment
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These 7 patients demonstrate the typical features of classic Castleman's disease and also display some unusual features of this disorder. Castleman's disease occurs in all age groups, but more often in the adolescent or young adult. The peak incidence lies from the second to the fourth decade [5]. As in our patients, half are younger than 30 years [6]. There is no known racial or sexual predominance. Interestingly, our patients were all female except 1. Patients are usually asymptomatic or have nonspecific complaints [1, 3]. Symptoms are apparently due to tracheobronchial compression, such as cough, dyspnea, chest pain, respiratory infection, hemoptysis, and back pain [3]. Feigert and associates [7] described a distinct syndrome associated with angiofollicular lymph node hyperplasia, the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonalgammopathy, and skin changes). Our cases revealed no symptoms or signs of POEMS syndrome.
Asymptomatic chest lesions usually are discovered on routine radiographs. Lesions may present themselves as long-standing masses, ranging up to 20 years, with minimal or no evidence of continued growth [8]. However, there were 1 case of recurrence and 1 case of mass growth in our patients, both of whom had a mixture of the hyaline-vascular and the plasma-cell type according to the pathologic findings. This suggests a spectrum of disease with one end being a localized hyaline vascular type and the other being a plasma cell variant with systemic symptoms.
A lesion can occur anywhere along the lymphatic chain, but most are found in the mediastinum. Sometimes the locations are unusual [9–11]. Patient 1 was the fourth case of such a lesion arising in the intercostal space [9, 11]. In patient 4, the lesion occurred in the intrapulmonary fissure, adhering to the anteromedial basal segmental artery and posterior basal segmental artery. The CT scan has aided greatly in the characterization of mediastinal masses. Due to the hypervascularity of these lesions, the CT scan shows enhancement. Although Castleman's disease can be mistaken histologically for follicular lymphoma or thymoma, these entities are not enhanced in the CT scan [12].
A lesion usually varies from 3 to 7 cm in the greatest dimension and are either encapsulated or relatively well circumscribed. The external surface is usually smooth but may be slightly nodular, suggesting the fusion of several nodules [8]. However, in our patients the lesions were all solitary, and most had smooth surfaces.
A lesion is usually quite vascular [13, 14] and tends to bleed profusely. Angiographic preoperative embolization can make operation much easier [14]. In addition, the margin of a lesion is usually well circumscribed. However, our cases showed several unusual features. In 2 patients, the lesion adhered to adjacent tissue, such as the bronchus and the pulmonary artery. In 1 patient (patient 6), the lesion extended from the mediastinum into the interlobar fissure and had fingerlike infiltration into the lung parenchyma.
Histologic diagnosis of Castleman's disease usually is made after excision of the mass. Frozen section examination is often misleading, and our experience also shows a positive correct diagnosis in only half of the cases examined by frozen section. Review of the frozen-section slide revealed a diagnostic mistake in 1 case. In addition the other cases were difficult to diagnose due to inadequate sampling of the specimen. Frozen section is useful, but a correct diagnosis can not be guaranteed unless enough tissue is examined.
Regarding the histologic type of Castleman's disease, the hyaline-vascular type is the most common. The plasma-cell variant is reported as being less than 10% of cases. Two of our cases (patients 6 and 7) showed some features of the plama-cell types associated with typical areas of hyaline-vascular type. An amorphous eosinophilic deposit was present in 1 case, but an atypical spindle cell proliferation, suggestive of vascular sarcoma, was present in the other [15].
The etiology and pathogenesis of Castleman's disease are uncertain. Proposed theories include a hyperplastic reactive (inflammatory) origin [1, 6], a hamartomatous origin [8, 13], a mixed origin [4], and an inappropriate immune response [10]. An inflammatory origin now is accepted widely. Treatment is surgical removal of the tumor mass. In the case of a large inoperable lesion, especially of the plasma-cell type, radiotherapy can be considered [16]. Our experience was not enough to answer the question of how one determines whether to resect totally or to leave some disease behind.
The prognosis generally is good, but the lesion can recur as in 1 of our patients [5, 6]. The presence of these lesions over long periods of time without noticeable increase in size and with an uneventful course after the surgical removal speak strongly for a benign character. However, some lesions, especially plasma-cell type, may possess malignant biological potential [4, 17].
In summary, giant lymph node hyperplasia can occur anywhere in the body, grow over a period without symptoms, and recur in spite of complete resection. Surgical resection and close follow-up are advised.
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Acknowledgments
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This study is supported by grant 02-92-052 from the Seoul National University Hospital Research Fund.
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Footnotes
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Address reprint requests to Dr Kim, Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, 28, Yonkun-Dong, Chongno-gu, Seoul 110-744, South Korea.
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References
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- Tung KS, McCormack LJ. Anogiomatous lymphoid hamartoma: report of five cases with a review of the literature. Cancer 1967;20:525–36.[Medline]
- Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972;29:670–83.[Medline]
- Frizzera G, Massarelli G, Banks PM, Rosai J. A systemic lymphoproliferative disorder with morphologic features of Castleman's disease: pathological findings in 15 patients. Am J Surg Pathol 1983;7:211–31.[Medline]
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Abstract
Introduction
