British Journal of Radiology (2004) 77, 161-163
© 2004 British Institute of Radiology
doi: 10.1259/bjr/30098414
Castleman's disease presenting as an adnexal mass: ultrasound, CT and MRI features
S D Chang, MD
1 and
R F Thoeni, MD
2
1 Department of Radiology, University of British Columbia, Vancouver Hospital & Health Sciences Centre, 899 West 12th Avenue, Vancouver, British Columbia, Canada V5Z 1M9 and 2 Department of Radiology, University of California San Francisco, 505 Parnassus Avenue, San Francisco, CA 94143-0628, USA
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Introduction
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Castleman's disease is a rare benign lymphoproliferative disorder of unknown aetiology. Castleman first described this disease entity also known as angiomatous lymphoid hamartoma or giant lymph node hyperplasia in 1954 in a group of patients with mediastinal lymphadenopathy [1]. Castleman's disease most commonly occurs in the thorax (70%), but can be seen in any location where lymphoid tissue is present and even in areas devoid of lymphoid tissue such as the central nervous system [2]. Castleman's disease in the pelvis is rare with only a few cases reported in the literature [3–6]. Imaging of Castleman's disease by CT has been well described in the literature, but MR reports on this disease are scarce [7, 8] and no cases of pelvic Castleman's disease have been reported using MRI with gadolinium. We describe one case of pelvic Castleman's disease imaged by three cross-sectional modalities: ultrasound (US), CT and MRI with gadolinium. An additional case of Castleman's disease of the pelvis is described that included both US and CT with coronal and sagittal reformations. To our knowledge, no case report has been published so far that used three cross-sectional imaging modalities or multiplanar reformations for assessing vascular supply in masses due to pelvic Castleman's disease.
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Case reports
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Case 1
A 30-year-old woman with no previous medical illness, presented with right pelvic discomfort. A firm right adnexal mass was palpated on physical examination. The ultrasound showed a 3 cm solid adnexal mass with lobulated margins and Doppler interrogation revealed a vascular mass with a low resistance arterial waveform (Figure 1a
). MRI demonstrated the mass as isointense to muscle on T1 weighted sequences (Figure 1b) and hyperintense to muscle on the T2 weighted sequences (Figure 1c). Following administration of gadolinium, strong homogeneous enhancement was present (Figure 1d). A diagnostic CT was then performed followed by an image-guided biopsy. Contrast enhancement was used to avoid necrotic areas in the mass during biopsy. The CT demonstrated a 3 cm x 3.8 cm right pelvic mass with marked contrast enhancement (Figure 1e). Cytology of the fine-needle aspirate revealed atypical lymphoid proliferation but a definitive diagnosis could not be made. The patient underwent surgical excision of the right pelvic mass. The pathological examination revealed prominent reactive follicles containing small sclerotic germinal centres, associated with hyalinized small blood vessels and prominent interfollicular vascular proliferation consistent with hyaline-vascular subtype of Castleman's disease. The patient has done well since the surgical resection with no clinical or imaging evidence of recurrence.
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Figure 1. 30-year-old woman with right pelvic mass. (a) Ultrasound with Doppler shows a large solid homogeneous vascular mass (arrow) with low arterial resistance flow. (b) T1 weighted and (c) T2 weighted MR images. (b) MR imaging before gadolinium demonstrates the right adnexal mass (arrows) as an area of intermediate signal intensity. (c) The T2 weighted MR image demonstrates the mass (arrows) as hyperintense to muscle. b, bladder. (d) T1 weighted MR image with fat suppression after gadolinium. Following gadolinium enhancement, the right adnexal mass enhances homogeneously. b, bladder, c, cervix, r, rectum. (e) Intravenous contrast-enhanced CT scan demonstrates strong homogeneous enhancement of the mass (arrow) draining into the common femoral vein (v). b, bladder, white v, vagina, r, rectum, a, common femoral artery, black v, common femoral vein.
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Case 2
A 28-year-old woman presented with a large left pelvic mass, lethargy and vaginal bleeding. The ultrasound showed a large 11 cm x 8.4 cm x 5.9 cm solid lobulated mass in the left adnexa. Doppler examination revealed vascularity within the mass with a low resistance arterial waveform similar to Case 1. A CT was performed on the same day and demonstrated marked contrast enhancement of this mass with a large draining ovarian vein (Figure 2). The pelvic mass was surgically resected and pathological examination revealed prominent interfollicular vascular proliferation. A diagnosis of hyaline-vascular subtype of Castleman's disease was made.
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Figure 2. 28-year-old woman with left pelvic mass. Coronal reformation of a contrast-enhanced axial CT image. CT shows marked opacification of the left adnexal mass (white arrows) and a large draining ovarian vein (black arrow). A, aorta, S, spine.
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Discussion
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In pelvic Castleman's disease, US, CT or MR imaging reveal features of a very vascular mass. On ultrasound, Castleman's disease usually appears as a homogeneous hypoechoic mass similar to that of lymphoma [9, 10] but a cystic appearance mimicking a cystic ovarian tumour has been described [5]. Colour Doppler ultrasound of Castleman's disease has been reported to show hypervascularity [3] as was the case with both of our patients. However, there have been no previous reports of the spectral waveform of these lesions. Both of our cases demonstrated a low resistance arterial waveform with resistive indices of less than 0.4 and thus can mimic a malignant ovarian neoplasm.
On enhanced CT or MRI, Castleman's disease has been reported to present as well-circumscribed mass(es) with homogeneous contrast enhancement due to marked hypervascularity [6–8]. On CT, both our cases demonstrated well-defined masses with marked homogeneous enhancement. Similar findings were also seen in the one patient who had undergone MRI with gadolinium enhancement. This later case demonstrates how readily MR visualizes the hypervascularity of this lesion. Larger lesions (>5 cm) may show heterogeneous enhancement due to associated necrosis or degeneration [6]. On MRI, Castleman's lesions are usually hypointense with T1 weighting and hyperintense with T2 weighting. Although calcifications were not seen in our cases, they are not uncommon. In a series of 16 cases of Castleman's disease involving the abdomen and pelvis, calcification was seen in 31% of cases [6]. These calcifications have been described as punctate or coarse, and as peripheral or arborizing.
There has been only one reported case of pelvic Castleman's disease imaged with MR. This case report of a male patient did not mention the use of gadolinium [11]. The authors concluded that MRI added no benefit over CT in the imaging of Castleman's disease. However, one should consider the fact that the use of MR with gadolinium can benefit patients with renal failure or patients in whom the administration of intravenous iodinated contrast material is contraindicated. In comparison with CT, MRI also has the added advantage of direct imaging in multiple planes and superior soft tissue contrast. However, multidetector helical CT with its improved reconstructive capabilities has diminished this difference between MR and CT. In the second case, CT reformations helped to determine that the vascular mass was originating from the adnexa because of visualization of a large draining ovarian vein. The use of reformations and 3D reconstructions has not been previously reported for pelvic Castleman's disease. Both CT and MR can provide improved surgical planning, particularly if the tumour is large and/or has large feeding or draining vessels where pre-operative embolisation may be needed.
On angiography, a prolonged dense homogeneous "blush" during the capillary phase is characteristic [12]. The vascularity of these lesions can result in haemorrhage during intraoperative resection of the tumour requiring transfusion. Pre-operative embolisation can be performed to facilitate surgical excision and decrease intraoperative bleeding. Most cases of pelvic Castleman's disease derive their blood supply from branches of the internal iliac arteries [11]. Large draining veins were seen in both of our cases, which has not been previously reported. Castleman's disease has also been evaluated with PET imaging. The uptake in Castleman's disease is less than that seen in patients with low and intermediate grade lymphomas [4].
The differential diagnosis of Castleman's disease presenting as a hypervascular adnexal mass includes sarcoma, unusual ovarian neoplasm, endometriosis, Kaposi's sarcoma, metastasis, lymphoma and an inflammatory mass such as seen in AIDS (bacillary angiomatosis, tuberculosis). Clinical correlation with the patient's immune status, tissue or fluid cultures and at times only biopsies might provide the definitive diagnosis. The presence of calcification and hypervascularity help to distinguish Castleman's disease from lymphoma, which usually is hypovascular and not associated with calcification unless treated.
In summary, pelvic Castleman's disease is a rare entity, but should be considered in the differential diagnosis of a well-circumscribed adnexal mass that is hypervascular and enhances homogeneously. Ultrasound with Doppler scanning can demonstrate the hypervascularity of the lesion but may confuse it with a malignant ovarian neoplasm. Contrast-enhanced CT usually demonstrates the typical features, but MRI can be used in patients with a contraindication to the use of iodinated contrast material. CT reformations or MR scanning in multiple planes can aid in determining that the mass originates from the adnexa and help in planning the surgical removal of this hypervascular mass. If needed, MR or CT angiography can provide a vascular road map for planned resection in the selected cases.
Received for publication March 6, 2002.
Revision received May 2, 2003.
Accepted for publication June 13, 2003.
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References
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Introduction
Case reports
