|
Multicentric Castlemanís Disease: Imaging Findings
DAVID R. FELDMAN, MD, and STEPHEN I. SCHABEL, MD, Charleston, SC
ABSTRACT: Multicentric Castlemanís disease is an unusual lymphoproliferative lesion typically associated with systemic manifestations and specific histopathology. We describe two cases of pathologically proven multicentric Castlemanís disease along with radiographic and ultrasonographic findings.
DISTINCT FROM reactive lymph node lesions and from histopathologically malignant lymphomas is a class of atypical lymphoproliferative lesions including angioimmunoblastic lymphadenopathy, transplant lymphoproliferation, polyclonal immunoblastic proliferations, and angiofollicular lymph node hyperplasia (Castlemanís disease).1 Two distinctive histologic forms of Castlemanís disease have been described: a hyaline-vascular type and a plasma cell type. The disease also has two typical clinical appearances: a benign localized type and a multicentric or systemic variety. Most localized Castlemanís lesions are mediastinal and of the hyaline-vascular type. Conversely, most disseminated lymphoproliferative lesions are of the plasma cell type.2-4
A diagnosis of multicentric Castlemanís disease can be suggested only by typical pathologic features of involved lymph nodes, since these findings are nonspecific.1,4 Also, accurate histopathologic interpretation requires either core or open biopsy, because differentiation from lymphoma cannot be made reliably by aspiration biopsy. Associated clinical findings are necessary to make the diagnosis; these include multifocal lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, anemia, elevated sedimentation rate, proteinuria, and cutaneous or neurologic signs and symptoms. Affected patients have constitutional complaints, particularly malaise, fever, and night sweats.1,4,5 An increased incidence of infections and an associated increase in secondary malignancy (non-Hodgkinís lymphoma and Kaposiís sarcoma) have been reported.1,3,5
The most common radiologic appearance of hyaline-vascular Castlemanís disease is focal nodal enlargement.6 Calcification has been reported in these lesions.6,7 On dynamic-infusion computed tomography (CT), contrast enhancement is commonly seen in hyaline-vascular lesions; the typical plasma cell lesion of multicentric Castlemanís disease shows either mild or no enhancement, which can be attributed to its lesser vascularity.4,6,8 Isolated cases of moderate enhancement of plasma cell lesions have been reported.8,9
Ultrasonographic evaluation of Castlemanís lesions usually identifies homogeneous hypoechoic masses, except in instances of lesion calcification, where focal hyperechoic areas with acoustical shadowing can be seen.7,9 Some instances of hyperechoic foci in noncalcified lesions have been reported and have been ascribed to enlarged germinal centers.6
We describe the CT and ultrasonographic features of two cases of pathologically proven multicentric Castlemanís disease.
CASE REPORTS
Case 1. A 40-year-old black woman had diffuse adenopathy and nephrotic syndrome. The adenopathy had progressed for 25 years, involving both axillae, the neck, and both inguinal regions. She denied constitutional complaints. Physical examination confirmed lymphadenopathy and hepatosplenomegaly. Serum protein electrophoresis showed polyclonal hypergammaglobulinemia. Percutaneous renal biopsy revealed mesangioproliferative glomerulonephritis. Dynamic-infusion, contrast-enhanced CT of the chest and abdomen showed hepatosplenomegaly and retroperitoneal, periportal, mediastinal, and hilar lymphadenopathy. There was no contrast enhancement in involved nodes (Fig 1). An axillary node biopsy revealed angiofollicular lymph node hyperplasia (Castlemanís disease) of the hyaline-vascular type.
Case 2. A 54-year-old white woman with a history of chronic renal insufficiency had acute onset of nausea and vomiting. Cholecystitis was suspected, and ultrasonography showed a distended gallbladder without stones and a hypoechoic lesion in the vicinity of the pancreatic head (Fig 2). CT showed a retrogastric mass distinct from the pancreas, with associated para-aortic retroperitoneal nodes. Hepatomegaly was present without splenomegaly (Fig 3). Contrast medium was not used because of the patientís azotemia. Serum protein electrophoresis showed polyclonal hypergammaglobulinemia. Exploratory laparotomy was done and a retrogastric para-aortic lymph node was removed. Pathologically, the node showed Castlemanís disease with both hyaline-vascular and plasma cell features.
DISCUSSION
Castleman described in 1956 a pathologically unique form of lymph node hyperplasia associated with capillary proliferation and endothelial hyperplasia.10 Later, other investigators identified a similar type of lesion characterized by germinal center hyperplasia with intervening sheets of plasma cells.11 Both entities are now recognized under the primary categorization of angiofollicular lymph node hyperplasia (Castlemanís disease); the capillary proliferative lesionótypically seen as isolated mediastinal lymphadenopathyóis designated as the hyaline-vascular type, and the germinal hyperplastic lesion with abundant plasma cells is designated as the plasma cell type. The clinical picture of the plasma cell lesion tends toward multicentric lymph-
adenopathy and multisystem disease associated with constitutional complaints, specific abnormal laboratory findings, and a poor prognosis due to an increased incidence of infection and secondary malignancy. Multicentric Castlemanís disease comprises only about 10% of the total number of cases.11 In previous cases,12 a small percentage of patients (3% to 18%) with hyaline-vascular histology had systemic manifestations, as did both our patients.
These nodal lesions may enhance on contrast-enhanced CT, but often they will not in patients with multicentric disease due to underlying plasma cell histology. Despite the hyaline-vascular features in Case 1, contrast medium did not enhance the patientís lesions. This patient falls into the rare group of patients with Castlemanís disease who clearly manifest a systemic disorder but no nodal enhancement, despite having hyaline-vascular histopathology.
A diagnosis of Castlemanís disease should be considered in any patient with multicentric lymphadenopathy, particularly if involved nodes enhance. The differential diagnosis is extensive and includes infective (tuberculosis), inflammatory (sarcoidosis), neoplastic (renal cell carcinoma, papillary thyroid carcinoma, and Kaposiís sarcoma), and lymphoproliferative (angioimmunoblastic lymphadenopathy) etiologies.13 The multifocal lymphadenopathy seen in Castlemanís disease is frequently suggestive of lymphoma, and increased lesion vascularity on contrast-enhanced CT or color Doppler imaging should help eliminate that diagnosis. Although multicentric Castlemanís disease is typically associated with progressive systemic illness, the patient in Case 1 showed a more indolent process clinically, with a 25-year history of progressive lymphadenopathy. The presence of chronic lymphadenopathy should not eliminate the possibility of multicentric Castlemanís disease from a differential diagnosis. In addition, the absence of contrast enhancement should not be considered a reliable predictor of underlying nodal histopathology.
References
1. Peterson BA, Frizzera G: Multicentric Castlemanís disease. Semin Oncol 1993; 20:636-647
2. Herrada J, Cabanillas FF: Multicentric Castlemanís disease. Am J Clin Oncol 1995; 18:180-183
3. Frizzera G, Peterson BA, Bayrd ED, et al: A systemic lymphoproliferative disorder with morphologic features of Castlemanís disease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985; 3:1202-1216
4. Shahidi H, Myers JL, Kvale PA: Castlemanís disease. Mayo Clin Proc 1995; 70:969-977
5. Libson E, Fields S, Strauss S, et al: Widespread Castleman disease: CT and ultrasound findings. Radiology 1988; 166:753-755
6. Yamashita Y, Hirai T, Matsukawa T, et al: Radiologic presentations of Castlemanís disease. Comput Med Imaging Graph 1993; 17:107-117
7. Loevinger EH, Schabel SI: Calcified abdominal Castlemanís disease: plain film and sonographic features. South Med J 1984; 77:1310-1311
8. Chaulin B, Pontais C, Laurent F, et al: Pancreatic Castleman disease: CT findings. Abdom Imaging 1994; 19:160-161
9. Garber SJ, Shaw DG: Case report: the ultrasound and computed tomography appearance of mesenteric Castleman disease. Clin Radiol 1991; 43:429-430
10. Castleman B, Iverson L, Menendez VP: Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956; 9:822-830
11. 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; 29:670-683
12. Weisenburger DD, Nathwani BN, Winberg CD, et al: Multicentric angiofollicular lymph node hyperplasia: a clinicopathologic study of 16 cases. Hum Pathol 1985; 16:162-172
13. Moon WK, Im JG, Kim JS, et al: Mediastinal Castleman disease: CT findings. J Comput Assist Tomogr 1994; 8:43-46
BACK |
