Castleman's disease, also known as angiofollicular or benign giant lymph node hyperplasia, is an uncommon benign lymphoproliferative disorder [13]. Pathologically, two major histologic types are recognized: the hyaline-vascular type and the plasma cell type. The hyaline-vascular type (Fig. 1A, 1B, 1C, 1D), accounting for approximately 90% of cases, is characterized by germinal follicles with hyalinized vessels surrounded by concentric layers of small lymphocytes with proliferative interfollicular vascular stroma. The plasma cell type (Fig. 2A, 2B, 2C, 2D) is characterized by a paucity of follicular hyalinized vessels in the germinal follicles, although the interfollicular tissues are composed of sheets of dense plasma cells with less vascular stroma. Occasionally, a mixed type may be encountered. Alternatively, on the basis of its biologic behavior, Castleman's disease is also categorized as either a unicentric form or a multicentric form. The former is typically of the hyaline-vascular type and amenable to surgical treatment, although the latter typically belongs to the plasma cell type and is associated with more complicated systemic manifestations [2, 3]. This pictorial essay shows the typical features and highlights the atypical imaging presentations of histologically proven Castleman's disease encountered in our hospital.
Fig. 1A. 25-year-old asymptomatic man with right posterior mediastinal hyaline-vascular Castleman's disease. Digital chest radiograph shows right lower posterior mediastinal mass (open arrows) mimicking neurogenic tumor. Note intratumoral calcifications (solid arrows) with somewhat branchlike appearance.
Fig. 1B. 25-year-old asymptomatic man with right posterior mediastinal hyaline-vascular Castleman's disease. Unenhanced CT scan using bone window setting reveals posterior mediastinal mass (arrow) with typical “arborizing” pattern of intralesional calcifications.
Fig. 1C. 25-year-old asymptomatic man with right posterior mediastinal hyaline-vascular Castleman's disease. Contrast-enhanced chest CT scan shows intense enhancement of mass (arrow).
Fig. 1D. 25-year-old asymptomatic man with right posterior mediastinal hyaline-vascular Castleman's disease. Photomicrograph of histopathologic section shows typical features of hyaline-vascular Castleman's disease with hyalinized vessels within germinal follicle (arrows) formed by onionlike sheets of lymphocytes and intervening prominent vascular stromata. (H and E, ×100)
Fig. 2A. 60-year-old woman with thoracic plasma cell Castleman's disease who presented with chest tightness. Posteroanterior chest radiograph shows widening of upper mediastinum, aortopulmonary and azygos adenopathy, and enlargement of hila (arrows) with extension of lesion below right hilum.
Fig. 2B. 60-year-old woman with thoracic plasma cell Castleman's disease who presented with chest tightness. Coronal (B) and axial (C) T1-weighted images show aortopulmonary, paratracheal, azygos, bilateral hilar, and subcarinal adenopathy, as well as encasement of bronchi and pulmonary vessels.
Fig. 2C. 60-year-old woman with thoracic plasma cell Castleman's disease who presented with chest tightness. Coronal (B) and axial (C) T1-weighted images show aortopulmonary, paratracheal, azygos, bilateral hilar, and subcarinal adenopathy, as well as encasement of bronchi and pulmonary vessels.
Fig. 2D. 60-year-old woman with thoracic plasma cell Castleman's disease who presented with chest tightness. Photomicrograph of histopathologic section shows typical features of plasma cell Castleman's disease with germinal follicle (arrows) and interfollicular infiltrations of dense plasma cells. (H and E, ×200)
Castleman's disease occurs at any age, with a peak incidence in the third to fourth decades of life, but the multicentric form usually affects older individuals [1, 3]. Various hypotheses regarding the pathogenesis of Castleman's disease have been proposed including lymphoid hamartomatous hyperplasia; abnormal autoimmunity; an immunodeficiency state; and, the most widely accepted, chronic low-grade inflammatory process. Approximately 70% of Castleman's disease cases are located in the thorax (Figs. 1A, 1B, 1C, 1D, 2A, 2B, 2C, 2D, 3A, 3B, 4A, 4B, 4C, 5A, 5B, 5C, 6, 7, 8, 9A, 9B, 10A, 10B, 10C); 10–15% in the abdomen, retroperitoneum, and pelvis (Figs. 10A, 10B, 10C, 11A, 11B, 12, 13); and 10–15% in the neck (Fig. 14A, 14B). Castelman's disease has also been described in the extralymphatic tissues, including the lung, larynx, parotid gland, pancreas, and muscle [13].
Fig. 3A. 32-year-old man with anterior mediastinal hyaline-vascular Castleman's disease who presented with anterior chest pain. Posteroanterior chest radiograph shows widened anterior mediastinum (arrows) mimicking thymoma or lymphoma.
Fig. 3B. 32-year-old man with anterior mediastinal hyaline-vascular Castleman's disease who presented with anterior chest pain. Contrast-enhanced chest CT scan shows matted lymphadenopathy (arrows) formed by confluence of inhomogeneously enhancing enlarged lymph nodes confined to anterior mediastinum.
Fig. 4A. 26-year-old asymptomatic woman with hyaline-vascular Castleman's disease in right major fissure incidentally found on chest radiographs; interlobar pleural mass with prominent vessels on lesion surface and marked adhesion to adjacent lung tissues were noted during surgery. Posteroanterior chest radiograph shows incomplete upper border of mass (arrows) over right lower lung field suggestive of pleural lesion abutting fissure.
Fig. 4B. 26-year-old asymptomatic woman with hyaline-vascular Castleman's disease in right major fissure incidentally found on chest radiographs; interlobar pleural mass with prominent vessels on lesion surface and marked adhesion to adjacent lung tissues were noted during surgery. Lateral chest radiograph shows well-defined interlobar mass (arrows).
Fig. 4C. 26-year-old asymptomatic woman with hyaline-vascular Castleman's disease in right major fissure incidentally found on chest radiographs; interlobar pleural mass with prominent vessels on lesion surface and marked adhesion to adjacent lung tissues were noted during surgery. Contrast-enhanced chest CT scan using lung window setting shows well-defined nodule (arrows) along right major fissure.
Fig. 5A. 32-year-old asymptomatic woman with pericardial hyaline-vascular Castleman's disease incidentally found on chest radiographs; ovoid mass embedded within proliferated pericardial fat adjacent to right atrium was confirmed during surgery. Posteroanterior (A) and lateral (B) chest radiographs show focal bulge (arrow) of right posterior heart border simulating pericardial cyst.
Fig. 5B. 32-year-old asymptomatic woman with pericardial hyaline-vascular Castleman's disease incidentally found on chest radiographs; ovoid mass embedded within proliferated pericardial fat adjacent to right atrium was confirmed during surgery. Posteroanterior (A) and lateral (B) chest radiographs show focal bulge (arrow) of right posterior heart border simulating pericardial cyst.
Fig. 5C. 32-year-old asymptomatic woman with pericardial hyaline-vascular Castleman's disease incidentally found on chest radiographs; ovoid mass embedded within proliferated pericardial fat adjacent to right atrium was confirmed during surgery. Contrast-enhanced chest CT scan shows proliferation of right pericardial fat (solid arrows) adjacent to right atrium and homogeneous, well-enhanced intrapericardial nodule (open arrow) with central calcification.
Fig. 6. 28-year-old man presented with obesity and hypertension with lung mass found incidentally on chest radiograph; right upper lobectomy confirmed presence of intrapulmonary hyaline-vascular Castleman's disease. Posteroanterior chest radiograph reveals solitary lung mass (solid arrows) in right upper lobe with inferior border contacting right minor fissure (open arrow).
Fig. 7. 7-year-old girl with hyaline-vascular Castleman's disease who presented with cough. Contrast-enhanced chest CT scan shows well-defined left anterior mediastinal mass with homogeneous enhancement and prominent tumor vessels (arrows) at its periphery.
Fig. 8. 38-year-old man with hyaline-vascular Castleman's disease who presented with intermittent fever and cough. Contrast-enhanced chest CT scan shows right posterior mediastinal mass (open arrow) with extension to azygoesophageal recess, mild erosion of adjacent vertebral body (black arrow), and partial bronchial encasement (solid white arrows).
Fig. 9A. 35-year-old asymptomatic man with right paratracheal hyaline-vascular Castleman's disease treated by thoracoscopic excision. Contrast-enhanced chest CT scan shows well-defined right paratracheal nodule with enhanced rim (arrows) and central hypodensity.
Fig. 9B. 35-year-old asymptomatic man with right paratracheal hyaline-vascular Castleman's disease treated by thoracoscopic excision. Photomicrograph of histopathologic section shows mildly distorted germinal follicle (arrows) and marked central fibrotic and degenerative changes (triangles). (H and E, ×100)
Fig. 10A. 58-year-old woman presented with aggravating dyspnea for 3 months; anemia and hyperalbuminemia were also noted. Histopathologic examination of thoracoscopic biopsied specimen of left basal lung revealed infiltrations of lymphocytes and plasma cells around bronchiole. Diagnosis of multicentric plasma cell Castleman's disease was confirmed by biopsies of enlarged neck and retroperitoneal lymph nodes. Posteroanterior chest radiograph shows interstitial opacities in lower lobes and focal opacity (arrow) in left lower lobe.
Fig. 10B. 58-year-old woman presented with aggravating dyspnea for 3 months; anemia and hyperalbuminemia were also noted. Histopathologic examination of thoracoscopic biopsied specimen of left basal lung revealed infiltrations of lymphocytes and plasma cells around bronchiole. Diagnosis of multicentric plasma cell Castleman's disease was confirmed by biopsies of enlarged neck and retroperitoneal lymph nodes. High-resolution CT scan of lung shows poorly defined centrilobular nodules (white arrows), minimal interlobular septal thickening, mild bronchiectasis, small subpleural thin-walled cysts (black arrow), and subpleural nodules.
Fig. 10C. 58-year-old woman presented with aggravating dyspnea for 3 months; anemia and hyperalbuminemia were also noted. Histopathologic examination of thoracoscopic biopsied specimen of left basal lung revealed infiltrations of lymphocytes and plasma cells around bronchiole. Diagnosis of multicentric plasma cell Castleman's disease was confirmed by biopsies of enlarged neck and retroperitoneal lymph nodes. Contrast-enhanced abdominal CT scan shows hepatosplenomegaly and enlarged retroperitoneal lymph nodes (arrows).
Fig. 11A. 38-year-old woman admitted for preoperative evaluation of cervical cancer; coexistent left retroperitoneal hyaline-vascular Castleman's disease was incidentally found. Abdominal radiograph shows retroperitoneal mass with typical “arborizing” calcifications (arrows).
Fig. 11B. 38-year-old woman admitted for preoperative evaluation of cervical cancer; coexistent left retroperitoneal hyaline-vascular Castleman's disease was incidentally found. Abdominal CT scan at level of lower pole of left kidney (white open arrow) shows heterogeneously enhanced retroperitoneal mass (black open arrow) with multiple hypodense areas and peripherally located calcifications (solid arrows).
Fig. 12. 36-year-old woman with hyaline-vascular Castleman's disease who presented with vague left abdominal pain. Enhanced abdominal CT scan shows well-defined mesenteric mass (arrows) with homogeneously intense enhancement.
Fig. 13. 46-year-old man with hyaline-vascular Castleman's disease who presented with lower leg numbness. Contrast-enhanced pelvic CT scan shows well-defined pelvic mass with areas of focal necrosis (arrows) and erosion of left sacral bone.
Fig. 14A. 42-year-old man with hyaline-vascular Castleman's disease who presented with neck pain and mass in right lower neck that was slowly enlarging for several years. Axial T1-weighted image reveals slightly hyperintense mass (arrow) in right supraclavicular fossa with prominent tumor vessels with flow void.
Fig. 14B. 42-year-old man with hyaline-vascular Castleman's disease who presented with neck pain and mass in right lower neck that was slowly enlarging for several years. Doppler sonogram of neck shows slightly hypoechoic mass with prominent peripheral vascularity (open arrows) and feeding artery (solid arrow) penetrating mass.

Radiographic Appearances

Typical thoracic Castleman's disease usually occurs in the mediastinum and hilum and manifests as a rounded solitary mediastinal or hilar mass in asymptomatic patients [13]. On chest radiographs, mediastinal Castleman's disease may mimic thymoma, lymphoma, or neurogenic tumor (Figs. 1A and 3A), although hilar Castleman's disease may simulate bronchial adenomas [1]. Uncommonly, thoracic Castleman's disease arises from other locations, including the pleura, pericardium, intercostal space, and lung, with atypical imaging features. Pleural Castleman's disease may present as either a well-defined interlobar mass (Figs. 4A and 4B) or massive pleural effusion [4]. Pericardial Castleman's disease may present as a pericardial mass resembling pericardial cyst [5] (Figs. 5A and 5B). Intercostal Castleman's disease may manifest as an extrapulmonary mass with rib erosion [6]. Intrapulmonary Castleman's disease may appear as a solitary lung mass [1] (Fig. 6). Most instances of abdominal or pelvic Castleman's disease may not be visible on abdominal radiographs unless they are massive or calcified (Fig. 11A). It is unusual for Castleman's disease to have calcification sufficient to be visible on radiographs, but when present, the calcification is characteristically coarse or branchlike [13] (Figs. 1A and 11A). Conversely, multicentric Castleman's disease may manifest as bilateral hilar and mediastinal enlargement (Fig. 2A), diffuse reticulonodular pulmonary infiltrations (Fig. 10A), hepatosplenomegaly, and ascites [3].

CT Appearances

On the basis of CT, unicentric thoracic Castleman's disease can be categorized as one of three morphologic patterns: a solitary, noninvasive mass (50% of cases) (Figs. 1B, 4B, 5C, and 7); a dominant mass with involvement of contiguous structures (40% of cases) (Fig. 8); or a matted lymphadenopathy confined to a single mediastinal compartment (10% of cases) [2, 3] (Fig. 3B). Homogeneously intense contrast enhancement, reflecting hypervascularity of the lesion, is considered to be the CT finding characteristic of unicentric thoracic and abdominal Castleman's diseases (Figs. 1C, 5C, 7, and 12). Prominent feeding vessels may occasionally be shown (Fig. 7). However, Meador and McLarney [7] have reported that in the presence of intralesional fibrosis, necrosis, and degeneration, abdominal or pelvic Castleman's disease may have a heterogeneous appearance on CT, especially lesions larger than 5 cm [7, 8] (Figs. 11B and 13). A few instances of mediastinal Castleman's disease may also show intratumoral hypodensity on contrast-enhanced CT (Fig. 9A, 9B). On CT, 5–10% of Castleman's disease showed intralesional calcifications, typically being discrete, coarse, or distinctive with an “arborizing” pattern in morphology [2, 3] (Figs. 1B, 5C, and 11A, 11B). Intrathoracic multicentric Castleman's disease typically exhibits bilateral hilar and mediastinal lymphadenopathy; centrilobular nodular opacities; and, less commonly, ground-glass attenuation, air-space consolidation, and bronchiectasis [3] (Fig. 10B). In the abdomen, multicentric Castleman's disease is characterized by diffuse lymphadenopathy, hepatomegaly, splenomegaly, ascites, and thickening of the retroperitoneal fascia (Fig. 10C).

MRI Appearances

MRI is well suited to the evaluation of thoracic Castleman's disease because of its ability to show the extent of the tumor, to clarify its relationship to the bronchovascular structures, to allow multiplanar anatomic delineation of the lesion (Figs. 2B and 2C), and to show the feeding vessels as flow void structures [2, 8] (Fig. 14A). Most Castleman's disease lesions are isointense or slightly hyperintense relative to the skeletal muscle on T1-weighted images (Figs. 2B, 2C, and 14A) and heterogeneously hyperintense on long-TR images and enhanced T1-weighted images [2, 9]. In contrast to CT, clear depiction of intratumoral calcifications or interstitial lung infiltrations is difficult.

Sonographic Appearances

Most abdominal Castleman's disease lesions appear as nonspecific, well-defined hypoechoic masses on sonography. Konno et al. [9] have described a case of mesenteric Castleman's disease characterized by a large feeding artery penetrating the nodal hilum and prominent arteries in the periphery identified on a Doppler sonogram. Although sonography is of limited value in detecting thoracic Castleman's disease because of hindrance of bony structures and aerated lung tissues, it remains useful for the evaluation of cervical (Fig. 14B) and axillary Castleman's diseases, in which the depiction of prominent peripheral vessels and penetrating feeding vessels on Doppler sonograms can suggest the diagnosis of this uncommon disease.

Conclusion

Although most Castleman's disease lesions typically appear as well-defined mediastinal, hilar, or abdominal masses on radiographs and show good enhancement on CT and MRI, they can exhibit a wide range of atypical imaging features. Understanding the typical and atypical imaging characteristics of Castleman's disease is important for the diagnosis of this uncommon disease entity.

Footnotes

Address correspondence to S.-F. Ko ([email protected]).
Presented at the 2003 annual meeting of the American Roentgen Ray Society, San Diego, CA.

References

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Information & Authors

Information

Published In

American Journal of Roentgenology
Pages: 769 - 775
PubMed: 14975984

History

Submitted: January 22, 2003
Accepted: July 10, 2003

Authors

Affiliations

Sheung-Fat Ko
Department of Radiology, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, 123 Ta-Pei Rd., Niao-Sung Hsiang, Kaohsiung Hsien 833, Taiwan.
Ming-Jeng Hsieh
Department of Cardiovascular and Thoracic Surgery, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, Kaohsiung Hsien, Taiwan.
Shu-Hang Ng
Department of Radiology, Chang Gung Memorial Hospital at Keelung, Chang Gung University, Keelung Hsien, Taiwan.
Department of Radiology, Chang Gung Memorial Hospital at Linkou, Chang Gung University, Taoyuen Hsien, Taiwan.
Jui-Wei Lin
Department of Pathology, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, Kaohsiung Hsien, Taiwan.
Yung-Liang Wan
Department of Radiology, Chang Gung Memorial Hospital at Keelung, Chang Gung University, Keelung Hsien, Taiwan.
Department of Radiology, Chang Gung Memorial Hospital at Linkou, Chang Gung University, Taoyuen Hsien, Taiwan.
Tze-Yu Lee
Department of Radiology, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, 123 Ta-Pei Rd., Niao-Sung Hsiang, Kaohsiung Hsien 833, Taiwan.
Wei-Jen Chen
Department of Pathology, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, Kaohsiung Hsien, Taiwan.
Min-Chi Chen
Department of Public Health and Biostatistics, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, Kaohsiung Hsien, Taiwan.

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