Mesenteric Castleman's disease: case report

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Mesenteric Castleman's disease: case report. G. Demirpolat,1 A. Pourbagher,1 M. Hekimgil,2 N. Elmas,1 O. Kitis,1 M. Korkut,3 A. Memis1. 1Department of ...
Abdom Imaging 25:551–553 (2000) DOI: 10.1007/s002610000090

Abdominal Imaging © Springer-Verlag New York Inc. 2000

Mesenteric Castleman’s disease: case report G. Demirpolat,1 A. Pourbagher,1 M. Hekimgil,2 N. Elmas,1 O. Kitis,1 M. Korkut,3 A. Memis1 1

Department of Radiology, Ege University School of Medicine, 35100 Izmir, Turkey Department of Pathology, Ege University School of Medicine, 35100 Izmir, Turkey 3 Department of Surgery, Ege University School of Medicine, 35100 Izmir, Turkey 2

Received: 5 January 2000/Accepted: 26 January 2000

Abstract An asymptomatic case of hyaline vascular–type Castleman’s disease localized to the mesentery and detected incidentally by ultrasound is presented. Computed tomographic, angiographic, and histologic findings are reported. This type of Castleman’s disease predominates in the thorax and is very rare in the mesentery. Key words: Castleman’s disease—Mesentery—Computed tomography—Angiography—Histopathology.

Castleman’s disease is a rare lymphoproliferative disorder of unknown etiology. Benign masses of lymphoid tissue are seen in the mediastinum, lung, neck, axilla, mesentery, pelvis, retroperitoneum, and within the muscle.

Case report Abdominal ultrasound of a 24-year-old asymptomatic female, examined in another hospital, demonstrated a mass in the left lower quadrant. Because the mass was thought to be intestinal in origin, a small bowel follow-through series was requested. The small bowel follow-through series showed displacement of bowel loops by an extrinsic mass. Abdominal ultrasound was repeated and color Doppler examination was performed. A round, hypoechogenic, hypervascular mass with smooth contours was detected in close proximity to the small bowel loops. Dynamic three-phase spiral computed tomography (CT) examination showed spotty calcification and marked enhancement of the mass, which had prominent vascular supply from the superior mesenteric artery. Opacification of the superior mesenteric vein during the arterial phase suggested early venous drainage. The mass showed a dense homogeneous blush on angiography. Arterial supply from the jejunal branches of the superior mesenteric artery and early venous drainage to the

Correspondence to: G. Demirpolat

superior mesenteric vein were seen. No cluster of vessels, suggesting an arteriovenous malformation, was detected. The initial diagnosis was a benign tumor of the mesentery, and the patient had surgery. A 20-cm segment of the small intestine was resected with the mass. At macroscopic examination, the mass, measuring 5 ⫻ 4 ⫻ 3 cm and surrounded by an intact capsule, was totally removed. The cut surface was tan to white, soft, fleshy, and homogeneous. Four lymph nodes measuring between 1 cm and 1 mm were dissected for further examination. Histopathologic examination showed that the mass was composed of a giant lymph node, with a largely obliterated architecture. Fibrous thickening of the capsule and an increased number of lymphoid follicles with small, involuted, partly collagenized germinal centers and broad mantles were evenly distributed throughout the entire parenchyma, effacing the normal structure of both the cortex and the medulla. Occasional sinuses were observed at the periphery of the node. At higher magnification, atrophic, lymphocyte-depleted, hyalinized germinal centers with multiple concentric layering of mantle-zone lymphocytes were seen. Radially penetrating hyalinized vessels and the surrounding mantle of small lymphocytes, giving the characteristic appearance of a “lollipop,” were prominent features in most follicles. Abundant vascular proliferation, small round lymphocytes, scattered plasma cells, and immunoblasts were noted in interfollicular areas. Infiltration of lymphoid follicles with atrophic hyalinized germinal centers were also noted in the thick fibrous capsule and within the pericapsular adipose tissue. The histopathologic diagnosis was angiofollicular lymph node hyperplasia (Castleman’s disease) of the hyaline vascular type. The microscopic diagnosis of the other four lymph nodes was reactive follicular hyperplasia.

Discussion

Primary neoplasms of the mesentery are very rare. They are usually of mesenchymal origin and include desmoid tumor, lipoma, liposarcoma, and fibrosarcoma. Metastatic tumors and lymphoma are more common [1]. Our patient had no systemic symptoms, and the mass was wellcircumscribed and showed homogeneous enhancement.

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Fig. 1. A Nonenhanced CT shows a well-circumscribed mass of soft tissue density with spotty calcification in the left lower quadrant. B The mass shows marked enhancement during the arterial phase. Early venous drainage is also seen.

G. Demirpolat et al.: Mesenteric Castleman’s disease

mass shows a homogeneous blush. Arterial supply from the jejunal branches is seen. Fig. 3. Follicle center containing deposits of hyaline, transfixed by penetrating capillaries (hematoxylin and eosin, 400⫻).

Fig. 2. Selective angiography of the superior mesenteric artery. The

An initial diagnosis of primary benign tumor of the mesentery was made. Castleman’s disease is a lymphoproliferative disorder of unknown etiology. It can be localized or multicentric. The localized form is considered to be benign, and surgical removal of the mass provides cure. The multicentric form may have an aggressive course. It has two histologic types: hyaline vascular and plasma cell. The hyaline vascular type predominates in the thorax and is rare in the mesentery [2]. The enlarged lymph nodes are highly vascular and show

marked enhancement on contrast-enhanced CT [3, 4]. The mesenteric disease is usually of the plasma cell type, which is associated with systemic symptoms such as hematologic and endocrine disorders or peripheral neuropathies [5, 6]. Our patient was asymptomatic. The disease was localized to the mesentery and was of the hyaline vascular type. Although it is rare in the mesentery, hyaline vascular–type Castleman’s disease should be included in the differential diagnosis of hypervascular, well-demarcated soft tissue masses localized to the mesentery.

G. Demirpolat et al.: Mesenteric Castleman’s disease

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