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CT Features of Castleman Disease of the Abdomen and Pelvis

¹ Department of Radiologic Pathology, Armed Forces Institute of Pathology, Rm. M-111, 14th St. N.W. and Alaska Ave., Washington, DC 20306-6000.
² Present address: Asheville Radiology Associates, P. O. Box 2959, Asheville, NC 28802.
³ Present address: Department of Radiology, USA MEDDAC, Fort Carson, CO 80913.

Received September 23, 1999; accepted after revision December 8, 1999.

 
The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as representing the views of the Department of the Army or the Department of the Defense.

Presented at the annual meeting of the American Roentgen Ray Society, New Orleans, May 1999.

Abstract

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OBJECTIVE. The purpose of this study was to describe the CT features of Castleman disease in the abdomen and pelvis.

CONCLUSION. The most frequent appearance of abdominal or pelvic Castleman disease is of a single, well-defined enhancing mass. Smaller tumors (<5 cm) display homogeneous contrast enhancement; larger tumors (>5 cm) show heterogeneous enhancement and attenuation when correlated with central necrosis and degeneration. Calcification was seen in 31% of the cases. Castleman disease may be considered in the differential diagnosis of a discrete enhancing mass in the abdomen or pelvis.

Introduction

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First described in 1954 [1], Castleman disease (also known an angiofollicular lymph node hyperplasia and giant lymph node hyperplasia) is a rare, usually benign process of unknown cause, characterized by lymphocyte proliferation. Castleman disease is most frequently described in the radiology and pathology literature as located in the mediastinum but may be found in other anatomic locations. The description of Castleman disease in the abdomen or pelvis in the radiology literature is generally limited to case reports. The two principal histologic types of Castleman disease are hyaline-vascular and plasma cell types. A rare and potentially fatal, systemic form of Castleman disease has also been described [2]. The cytology and core histologic diagnosis of Castleman disease may be difficult, and the misdiagnosis of lymphoma may result. The purpose of this study was to describe the radiologic features of Castleman disease in the abdomen and pelvis.

Materials and Methods

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A retrospective review of all cases of Castleman disease in the archives of the Department of Radiologic Pathology of the Armed Forces Institute of Pathology from 1985 to 1999 was performed. Cases imaged with CT were selected. All lesions were assessed by two radiologists aware of diagnosis for size, location, pattern of enhancement, and density characteristics and then coded by consensus. Medical records were reviewed for presenting symptomatology. Pathology reports were reviewed for classification as hyaline-vascular, plasma cell, or mixed types of Castleman disease.

Results

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Sixteen cases of Castleman disease of the abdomen or pelvis were found. The pathologic diagnosis was established by surgical excision in all patients. Ten women and six men, ranging in age from nine to 79 years (mean age, 39 years), were included. All patients had been examined with contrast-enhanced CT.

The hyaline-vascular type was found in 11 patients (69%), the plasma cell type in three patients (19%), and a mixed form in two cases (13%). Of the 11 patients with the hyaline-vascular type, eight (73%) were symptomatic. The most common symptom was abdominal pain (n = 5); additional signs and symptoms included weight loss (n = 3), fatigue (n = 2), anemia (n = 2), and elevated erythrocyte sedimentation rate (n = 1). All three patients with the plasma cell type were symptomatic. Symptoms included fatigue (n = 2), failure to thrive (n = 2), fever (n = 1), anemia (n = 1), polyclonal hypergamma-globulinemia (n = 1), elevated erythrocyte sedimentation rate (n = 1), and splenomegaly (n = 1). Both patients with the mixed form of the disease were symptomatic, both presenting with abdominal pain and one presenting with anemia. The lesion was an incidental finding in two patients. One patient presented with a palpable abdominal mass. Clinical history was unavailable for one patient.

Fifteen patients (94%) were found to have a single mass on CT: nine in the retroperitoneum or pelvis, three in the porta hepatis, and three in the mesentery. One patient presented with generalized lymphadenopathy in the chest, with diffuse abdominal lymph nodes measuring approximately 1 cm. Of the nine cases of focal retroperitoneal or pelvic Castleman disease, seven were located on the left side and two on the right side. Lesions ranged in size from 1 to 18 cm in maximum diameter (average size, 4.5 cm). All tumors had well-defined margins. Tumors less than 5 cm in diameter (n = 8) generally showed homogeneous enhancement (enhancement defined as increased attenuation relative to the psoas muscle) (Fig. 1A,1B,1C), whereas tumors greater than 5 cm in diameter (n = 8) generally showed heterogeneous enhancement (Fig. 2). Calcification was seen in five (31%) of 16 patients; punctate calcification was seen in four patients and an "arborizing" or radial pattern of calcification was seen in one patient (Fig. 3). Although calcification tended to be seen in larger lesions (average size, 15 cm), calcification was also seen in one lesion measuring 3.5 cm.

View larger version (101K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A. —9-year-old boy who presented with abdominal pain. Contrast-enhanced pelvic CT scan shows well-defined, homogeneously enhancing mass (arrow). Surgical pathology revealed hyaline-vascular type Castleman disease.

View larger version (188K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B. —9-year-old boy who presented with abdominal pain. Photomicrograph of histopathologic specimen shows typical target appearance of follicular center in hyaline-vascular type Castleman disease. (H and E)

View larger version (175K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C. —9-year-old boy who presented with abdominal pain. Photomicrograph of histopathologic specimen shows concentric-ring pattern of flattened cells, hyalinized germinal center, and capillary proliferation. (H and E)

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2. —46-year-old man. Clinical presentation history was not available. Contrast-enhanced abdominal CT scan shows well-defined heterogeneously enhancing retroperitoneal mass. Surgical pathology revealed hyaline-vascular type Castleman disease.

View larger version (131K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3. —43-year-old woman who presented with abdominal pain. Contrast-enhanced CT scan shows heterogeneously enhancing mass with "arborizing" or radial pattern of calcification. Less than 1-cm aortocaval lymph nodes are identified. Surgical pathology revealed mixed-type Castleman disease.

Discussion

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Castleman disease is a rare, generally benign disease characterized by atypical proliferation of lymphocytes. The cause is unknown, but it has been proposed to represent either a response to chronic inflammation or a hamartoma of the lymph system [1,3,4]. The disease has been described in patients who were 2-85 years old (median age, 23 years) [3]. Castleman disease is most often found in the mediastinum (67%) but has been identified in multiple anatomic locations, including the neck (14%), the pelvis (4%), and the axilla (2%) [5]. Castleman disease most commonly manifests as a localized mass or masses. A rare, potentially fatal systemic form of Castleman disease has been identified [2].

Two major histologic forms of Castleman disease, the hyaline-vascular and plasma cell types, and, uncommonly, a transitional or mixed form may be seen. The hyaline-vascular type of Castleman disease is found in 90% of affected patients, and 70% of cases are diagnosed in patients younger than 30 years old [3]. Patients with the hyaline-vascular type are usually asymptomatic; only 3% of patients present with systemic complaints [3]. Histologically, the hyaline-vascular type shows hypervascularhyaline germinal centers. The follicle center cells are concentrically layered and flattened, with extensive capillary proliferation between follicles and complete effacement of lymphoid sinuses [6].

Compared with patients with the hyalinevascular type of Castleman disease, 50% of patients with the plasma cell type are symptomatic at presentation [3]. Systemic signs and symptoms may include fever, elevated erythrocyte sedimentation rate, anemia, hypergammaglobulinemia, and splenomegaly [2,3]. Pediatric patients may present with growth disturbance or failure to thrive. The plasma cell type histologically shows sheets of mature plasma cells with normal- to large-size follicular centers [6]. Effacement of lymphoid sinuses is rare.

Castleman disease in the abdomen and pelvis is most commonly described in the radiology literature as a focal enhancing mass of varying locations, including the retroperitoneum, mesentery, porta hepatis, and pancreas [7,8,9]. In comparison, Castleman disease in the mediastinum is described as having a variety of appearances depending on histology. The hyaline-vascular type is described as having three patterns on CT: a solitary, noninvasive mass; a dominant infiltrative mass with associated lymphadenopathy; or matted lymphadenopathy without a dominant mass [10]. In contrast, the plasma cell type of Castleman disease manifests as diffuse mediastinal lymphadenopathy. All lesions show enhancement, regardless of histopathology.

In our series, 15 of 16 patients had a single, well-circumscribed enhancing mass. No difference was seen between the CT appearance of the hyaline-vascular or plasma cell types. Smaller lesions (<5 cm in diameter) tended to display homogeneous contrast enhancement whereas larger tumors (>5 cm) displayed heterogeneous enhancement, with low-attenuation areas consistent with necrosis. Because of its imaging appearance, Castleman disease may mimic a visceral neoplasm [8,9,10]. Although these lesions are usually well defined, a single case report describes Castleman disease as an enhancing retroperitoneal mass that appears to infiltrate the surrounding fat [11].

Castleman disease in the abdomen and pelvis displays a variety of calcification patterns, including punctate, coarse, peripheral, and "arborizing" [7,8,12]. In our series, five tumors (31%) showed calcification, one with an arborizing pattern and the remaining lesions with punctate calcification.

The differential diagnosis of an enhancing retroperitoneal mass may include lymphoma, metastasis, infection (abscess, tuberculosis), sarcoma, schwannoma, paraganglioma, and hemangiopericytoma. Based on its CT appearance, the most commonly stated presurgical radiologic differential diagnosis for Castleman disease in our series was lymphoma or sarcoma. In fact, two patients had presurgical diagnoses of lymphoma based on percutaneous biopsy; in one patient, a histologic diagnosis of lymphoma was made on frozen section. On CT, lymphoma is typically a mass of low attenuation, without significant enhancement or calcification. Retroperitoneal sarcomas may show enhancement and calcification, depending on histologic type. In contrast, tuberculosis is typically seen on CT as a mass or multiple masses with necrotic, low-attenuation centers and peripheral enhancement.

Limitations of our study include a small sample size and a selection bias. In addition, because the cases were submitted to the Armed Forces Institute of Pathology from many different institutions, a variety of CT imaging techniques was included.

In our series, Castleman disease in the abdomen and pelvis most commonly manifested as a discrete, enhancing mass. Masses less than 5 cm in diameter showed homogeneous contrast enhancement, and larger masses (>5 cm in diameter) showed heterogeneous enhancement. A variety of calcification patterns may be seen. Castleman disease may be considered in the differential diagnosis of an enhancing mass in the abdomen and pelvis.

References

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