CASE REPORT
Ileal Mesenteric Castleman Disease Selman SoÈkmen, Cem Terzi, Mehmet FuÈzuÈn, Funda Obuz and Aydanur Kargl From the Departments of Surgery, Radiology, and Pathology, Dokuz EyluÈl University, School of Medicine, Izmir 35340, Turkey
Eur J Surg 2001; 167: 634–635
INTRODUCTION Castleman disease (CD) was rst described in 1954 and is a rare disorder of lymphoid tissue (3). It generally presents as a localised mass, usually in the mediastinum, has a benign course and can be cured by local excision (1, 5–9). Its aetiology is still unknown (1, 3– 9). In 70% of cases this idiopathic tumoural lesion occurs in the mediastinum, but pancreas, spleen, neck, pelvic retroperitoneum, and supercial axillary and inguinal lymph nodes are the other regions involved (1, 3–9). It can be multicentric and involves multiple lymph node regions and various organs. The multicentric form is usually associated with severe clinical signs and symptoms, and unlike the localised form, it can be fatal (8). We presented a patient with localised disease in the ileal mesentery, which is an unusual site. We review our tests, the clinicopathological features, and surgical management.
CASE REPORT A 30-year-old woman with a history of mild abdominal pain for the last two years was admitted to hospital for further investigation. Her past history was unremarkable except for a caesarean section. She had no family history of malignancy or inherited disease. She did not have any systemic symptoms such as sweating, weight loss, fatigue, or fever. Her physical examination was completely normal. The only abnormalities in her routine blood count and chemistry were a mildly raised erythrocyte sedimentation rate (ESR) and hypergammaglobulinaemia. There was no serological indication of active HIV, Epstein-Barr virus, cytomegalovirus, brucella, toxoplasma or listeria infection. Concentrations of acute phase reactants were within the reference ranges, and she had no antibodies to antinuclear factor. An abdominal ultrasound scan showed a smoothsurfaced, homogenously hypoechoic mass 5 cm in diameter anterior to the inferior vena cava in the right paracolic region. Computed tomography (CT) conÓ 2001 Taylor & Francis. ISSN 1102–4151
rmed an oval, vascularised solid mass with a smooth margin and punctate calcications in the same region (Fig. 1). Abdominal and retroperitoneal lymph nodes were not enlarged. A lymphomatous mass, lymphoma, and tuberculous lymphadenitis were considered in the differential diagnosis. At the exploratory laparotomy, a eshy, solid mass measuring 5 £ 5 £ 6 cm was found in the mesentery of the ileum 30 cm proximal to the ileocaecal valve. We did a segmental small bowel resection with complete removal of the mass and primary anastomosis. Grossly, the mass was round, well-circumscribed, with a solid grey cut surface showing a nodular and granular pattern. Histopathological examination of the tumour showed the hyaline-vascular type of Castleman disease: large follicles were scattered in a mass of lymphoid tissue. The follicles showed vascular proliferation and hyalinisation in the centre. There was a concentric layer of lymphocytes at the periphery of the follicles (“onion-skin” appearance) (Fig. 2). Her postoperative course was uneventful and she was discharged in seven days. She has been free of symptoms for a year.
Fig. 1. Computed tomogram of the abdomen shows an oval, vascularised solid mass in the right paracolic region. Eur J Surg 167
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Despite histological similarities between multicentric and plasma cell variants of localised Castleman disease, the clinical courses of these two types are strikingly different. Multicentric involvement must be excluded before the denitive operation is done. Treatment of multicentric disease has not been rmly established but systemic treatment with steroids or chemotherapy, or both, resulted in varying success (8). Complete surgical excision is the treatment of choice for localised disease. Even with partial excision of unresectable lesions recurrence or progression of localised disease is not expected (5–9). Fig. 2. Hyaline-vascular type of Castleman disease, showing a vessel in the germinal centre (Haematoxylin and eosin, original magnication £200).
CONCLUSION Castleman disease is a rare lymphoproliferative disorder. Although the aetiology is unknown, it is assumed to be a defective regulation of the immune system causing oligoclonal expansion of B-cells (2, 10). According to the histological criteria, two variants have been described: the hyaline-vascular type, which is the most common variant and the plasma cell variant, the less common type (1, 4–9). Patients with the solitary hyaline-vascular type usually have no symptoms, whereas those with the localised plasma cell type may have fever, excessive sweating, fatigue, anaemia, raised ESR, bone marrow plasmacytosis, and polyclonal hyperglobulinaemia (8). Patients with localised disease are usually children and young adults, and they often have a mediastinal or abdominal mass (1, 4–9). Our patient presented with a localised, unicentric, impalpable abdominal mass with no severe clinical signs, hepatosplenomegaly, or lymphadenopathy. The multicentric or systemic form is nearly always of the plasma cell type (1, 6–9). Radiographic ndings are non-specic (1). The ultrasonographic picture is similar to that seen in lymphoma: uniform hypoechoic mass with enhanced through-transmission (1). CT consistently shows solid density and homogeneously enhanced masses with or without calcication. Magnetic resonance studies in pelvic or retroperitoneal disease yields no better results than CT for the differential diagnosis (6). The disease is still radiographically and operatively indistinguishable from a malignant tumour, as we saw in our patient.
REFERENCES 1. Barki Y, Shadked G, Levy I. Mesenteric Castleman disease: sonographic diagnosis. J Clin Ultrasound 1992; 20: 486–488. 2. Brandt SJ, Bodine DM, Dunbar CE, Nienhuis AW. Dysregulated interleukin-6 expression produces a syndrome resembling Castleman’s disease in mice. J Clin Invest 1990; 86: 592–599. 3. Castleman B, Towne VW. Case records of the Massachusetts General Hospital, Case 40011. N Engl J Med 1954; 250: 26–30. 4. Flendrig JA, Schilling PHM. Benign giant lymphoma. The clinical signs and symptoms. Folia Med Neerl 1969; 12: 119–120. 5. Frizzera G. Castleman’s disease: more questions than answers. Hum Pathol 1985; 15: 202–205. 6. MacDonald SR, Lurain JR, Hoff F, Variakozis D, Fishman DA. Castleman disease presenting as a pelvic mass. Obstet Gynecol 1996; 87: 875–877. 7. Takihara H, Takahashi M, Yamakawa G, Ishihara T, Baba Y. Castleman disease. Urology 1993; 41: 162–164. 8. Wengrower D, Libson E, Okan E, Goldin E. Gastrointestinal manifestations in Castleman’s disease. Am J Gastroenterol 1989; 85: 1179–1181. 9. Ylinen K, Sarlomo-Rikala M, Laatikainen T. Pelvic Castleman disease mimicking an adnexal tumor. Obstet Gynecol 1995; 85: 894–897. 10. Yoshizaki K, Matsuda T, Nishimoto H, Kuritani T, Taeho L, Aozasa K. Pathogenic signicance of interleukin-6 in Castleman’s disease. Blood 1989; 74: 1360– 1367. Submitted 14 July, 2000; Accepted 19 October, 2000. Address for correspondence: Selman So¨kmen, M.D. 651 sok. No. 8/3, Gaziemir TR-35410 Izmir Turkey Phone: ‡90.232.2777777 . Ext. 2911 Fax: ‡90.232.2599723 E-mail:
[email protected]
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