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Mesenteric Castleman disease
Journal of Pediatric Surgery (2008) 43, 1398–1400
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Mesenteric Castleman disease Ke Ren Zhang⁎, Hui Min Jia Department of Pediatric Surgery, Shengjing Hospital, China Medical University, Liaoning 110004, PR China Received 10 January 2008; revised 4 February 2008; accepted 4 February 2008
Key words: Children; Castle disease
Abstract Castleman disease is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. It can present at any extrathoracic site where there is lymphoid tissue. The sites include retroperitoneum, mesentery, axilla, and pelvis. Mesenteric Castleman disease is very rare in children. © 2008 Elsevier Inc. All rights reserved.
1. Case report A 10-year–old girl with no significant medical history was found, on routine physical examination, to have a nontender, mobile, 4-cm mass in the umbilical region. Results of routine laboratory tests were normal. Initial assessment with ultrasound examination revealed an ovoid 4 × 4 × 4-cm predominantly hypoechoic mass and increased vascularity on color Doppler. Computed tomography of the mass demonstrated a well-defined homogeneous soft-tissue mesenteric mass with 50 HU computed tomography (CT) value and dense uniform contrast enhancement (Fig. 1). As a result of the hypervascularity demonstrated on ultrasound and CT, percutaneous biopsy was not performed. The patient underwent a laparotomy with resection of a well-encapsulated mesenteric mass that was solid, homogeneous, and deep red on cut surface (Fig. 2). Histologic examination revealed multiple small lymphoid follicles separated by broad irregular bands of tissue containing a striking number of capillary vessels (Fig. 3). This pattern was consistent with the diagnosis of the hyaline-vascular type of Castleman disease. The postoperative course was uneventful, and the ⁎ Corresponding author. Tel.: +86 24 83956608; fax: +86 24 83955029, +86 24 23892617. E-mail address: [email protected] (K.R. Zhang). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.02.002
patient was discharged from the hospital in good condition on the seventh postoperative day. His recovery was uncomplicated, and he remains healthy and free of disease 6 months later.
Fig. 1 Abdominal CT showing a mass with 50 HU CT value in abdomen.
Mesenteric Castleman disease
1399
2. Discussion Castleman disease is a benign condition characterized by proliferation of mature lymphocytes and/or plasma cells. The condition was first reported by Castleman [1] in 1956 when he described a group of patients with large thymomalike masses in the anterior mediastinum. Since then, similar benign proliferations of lymphoid tissue have been reported in all areas where lymphoid tissue is normally found [2-5]; but more than 70% of cases involve the mediastinum, with less than 10% being intraabdominal. Although usually known as Castleman disease, it also has been called giant lymph node hyperplasia, lymph node hamartoma, angiofollicular mediastinal lymph node hyperplasia, and angiomatous lymphoid hyperplasia. The pathogenesis is unknown. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular type, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell type, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergammaglobulinemia. From a clinical staging point of view, Castleman disease also is classified into 2 subgroups [6]: localized or disseminated. The former is associated with a benign outcome; but the disseminated form, in contrast, is associated with various symptoms, such as fever, splenomegaly, and leukocytosis. Ultrasonography, CT scan, and magnetic resonance imaging have proven to be useful in the diagnosis of masses located in the retroperitoneum. However, the images of Castleman disease resemble other masses including lymphoma, tuberculosis, sarcoidosis, and retroperitoneal sarcomas [7]. From radiological and clinical points of view, disseminated Castleman disease may be indistinguishable from lymphoma. Ultimately, biopsy of an enlarged lymph node or mass is
Fig. 3 Histologic examination revealing multiple small lymphoid follicles separated by broad irregular bands of tissue containing a striking number of capillary vessels (hematoxylin and eosin, 100×).
mandatory for differential diagnosis. Surgical resection has been recommended in cases with a solitary lesion but not with the disseminated form. After resection of the tumor, symptoms and laboratory data improved in some cases. Radiotherapy, chemotherapy, steroid therapy, and/or immunosuppressive agents have been applied in refractory cases, resulting in the improvement of clinical symptoms [8-9]. It has been reported recently the interleukin 6 (IL-6) [10] is involved in the mechanism of clinical manifestation. Human herpesvirus 8 encodes a gene product that is homolog of human IL-6 (vIL-6), and it is thought to be responsible for the clinical symptoms. Clinical symptoms and signs of the disease resolved and most of the abnormal laboratory values greatly improved within a few days after treatment with IL-6 antibody in patients with elevated serum IL-6 levels. This therapy may be useful in the future. In our patient, a complete surgical excision was accomplished; and thus far, there is no evidence of recurrence.
References
Fig. 2
Laparotomy revealing the mesenteric mass.
[1] Castleman B, Towne VW. Case report of Massachusetts General Hospital; weekly clinicopathological exercises; founded by Richard C. Cabot. Case 40011. N Engl J Med 1954;250:26-30. [2] Powell RW, Lightsey AL, Thomas WJ, et al. Castleman's disease in children. J Pediatr Surg 1986;21:678-82. [3] Kim SH, Min BW, Kim WB, et al. Mesenteric Castleman's disease. Yonsei Med J 2005;46:289-91. [4] Papaziogas B, Chatzimavroudis G, Koutelidakis I, et al. A rare form of isolated mesenteric Castleman's disease presenting as an abdominal mass (isolated mesenteric Castleman's disease). J Gastrointestin Liver Dis 2006;15:171-4. [5] Jongsma TE, Verburg RJ, Geelhoed-Duijvestijn PH. Castleman's disease: a rare lymphoproliferative disorder. Eur J Intern Med 2007;18: 87-97. [6] Herrada J, Cabanillas F, Rice L, et al. The clinical behavior of localized and multicentric Castleman disease. Ann Intern Med 1998;128: 657-62.
1400 [7] Ko SF, Hsieh MJ, Ng SH, et al. Imaging spectrum of Castleman's disease. Am J Roentgenol 2004;182:769-75. [8] Shroff VJ, Gilchrist BF, DeLuca FG, et al. Castleman's disease presenting as a pediatric surgical problem. J Pediatr Surg 1995;30: 745-7.
K.R. Zhang, H.M. Jia [9] Bowne WB, Lewis JJ, Filippa DA, et al. The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature. Cancer 1999;85:706-17. [10] Yoshizaki K, Matsuda T, Nishimoto N, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's disease. Blood 1989;74:1360-7.
www.elsevier.com/locate/jpedsurg
Pediatric surgical images
Mesenteric Castleman disease Ke Ren Zhang⁎, Hui Min Jia Department of Pediatric Surgery, Shengjing Hospital, China Medical University, Liaoning 110004, PR China Received 10 January 2008; revised 4 February 2008; accepted 4 February 2008
Key words: Children; Castle disease
Abstract Castleman disease is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. It can present at any extrathoracic site where there is lymphoid tissue. The sites include retroperitoneum, mesentery, axilla, and pelvis. Mesenteric Castleman disease is very rare in children. © 2008 Elsevier Inc. All rights reserved.
1. Case report A 10-year–old girl with no significant medical history was found, on routine physical examination, to have a nontender, mobile, 4-cm mass in the umbilical region. Results of routine laboratory tests were normal. Initial assessment with ultrasound examination revealed an ovoid 4 × 4 × 4-cm predominantly hypoechoic mass and increased vascularity on color Doppler. Computed tomography of the mass demonstrated a well-defined homogeneous soft-tissue mesenteric mass with 50 HU computed tomography (CT) value and dense uniform contrast enhancement (Fig. 1). As a result of the hypervascularity demonstrated on ultrasound and CT, percutaneous biopsy was not performed. The patient underwent a laparotomy with resection of a well-encapsulated mesenteric mass that was solid, homogeneous, and deep red on cut surface (Fig. 2). Histologic examination revealed multiple small lymphoid follicles separated by broad irregular bands of tissue containing a striking number of capillary vessels (Fig. 3). This pattern was consistent with the diagnosis of the hyaline-vascular type of Castleman disease. The postoperative course was uneventful, and the ⁎ Corresponding author. Tel.: +86 24 83956608; fax: +86 24 83955029, +86 24 23892617. E-mail address: [email protected] (K.R. Zhang). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.02.002
patient was discharged from the hospital in good condition on the seventh postoperative day. His recovery was uncomplicated, and he remains healthy and free of disease 6 months later.
Fig. 1 Abdominal CT showing a mass with 50 HU CT value in abdomen.
Mesenteric Castleman disease
1399
2. Discussion Castleman disease is a benign condition characterized by proliferation of mature lymphocytes and/or plasma cells. The condition was first reported by Castleman [1] in 1956 when he described a group of patients with large thymomalike masses in the anterior mediastinum. Since then, similar benign proliferations of lymphoid tissue have been reported in all areas where lymphoid tissue is normally found [2-5]; but more than 70% of cases involve the mediastinum, with less than 10% being intraabdominal. Although usually known as Castleman disease, it also has been called giant lymph node hyperplasia, lymph node hamartoma, angiofollicular mediastinal lymph node hyperplasia, and angiomatous lymphoid hyperplasia. The pathogenesis is unknown. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular type, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell type, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergammaglobulinemia. From a clinical staging point of view, Castleman disease also is classified into 2 subgroups [6]: localized or disseminated. The former is associated with a benign outcome; but the disseminated form, in contrast, is associated with various symptoms, such as fever, splenomegaly, and leukocytosis. Ultrasonography, CT scan, and magnetic resonance imaging have proven to be useful in the diagnosis of masses located in the retroperitoneum. However, the images of Castleman disease resemble other masses including lymphoma, tuberculosis, sarcoidosis, and retroperitoneal sarcomas [7]. From radiological and clinical points of view, disseminated Castleman disease may be indistinguishable from lymphoma. Ultimately, biopsy of an enlarged lymph node or mass is
Fig. 3 Histologic examination revealing multiple small lymphoid follicles separated by broad irregular bands of tissue containing a striking number of capillary vessels (hematoxylin and eosin, 100×).
mandatory for differential diagnosis. Surgical resection has been recommended in cases with a solitary lesion but not with the disseminated form. After resection of the tumor, symptoms and laboratory data improved in some cases. Radiotherapy, chemotherapy, steroid therapy, and/or immunosuppressive agents have been applied in refractory cases, resulting in the improvement of clinical symptoms [8-9]. It has been reported recently the interleukin 6 (IL-6) [10] is involved in the mechanism of clinical manifestation. Human herpesvirus 8 encodes a gene product that is homolog of human IL-6 (vIL-6), and it is thought to be responsible for the clinical symptoms. Clinical symptoms and signs of the disease resolved and most of the abnormal laboratory values greatly improved within a few days after treatment with IL-6 antibody in patients with elevated serum IL-6 levels. This therapy may be useful in the future. In our patient, a complete surgical excision was accomplished; and thus far, there is no evidence of recurrence.
References
Fig. 2
Laparotomy revealing the mesenteric mass.
[1] Castleman B, Towne VW. Case report of Massachusetts General Hospital; weekly clinicopathological exercises; founded by Richard C. Cabot. Case 40011. N Engl J Med 1954;250:26-30. [2] Powell RW, Lightsey AL, Thomas WJ, et al. Castleman's disease in children. J Pediatr Surg 1986;21:678-82. [3] Kim SH, Min BW, Kim WB, et al. Mesenteric Castleman's disease. Yonsei Med J 2005;46:289-91. [4] Papaziogas B, Chatzimavroudis G, Koutelidakis I, et al. A rare form of isolated mesenteric Castleman's disease presenting as an abdominal mass (isolated mesenteric Castleman's disease). J Gastrointestin Liver Dis 2006;15:171-4. [5] Jongsma TE, Verburg RJ, Geelhoed-Duijvestijn PH. Castleman's disease: a rare lymphoproliferative disorder. Eur J Intern Med 2007;18: 87-97. [6] Herrada J, Cabanillas F, Rice L, et al. The clinical behavior of localized and multicentric Castleman disease. Ann Intern Med 1998;128: 657-62.
1400 [7] Ko SF, Hsieh MJ, Ng SH, et al. Imaging spectrum of Castleman's disease. Am J Roentgenol 2004;182:769-75. [8] Shroff VJ, Gilchrist BF, DeLuca FG, et al. Castleman's disease presenting as a pediatric surgical problem. J Pediatr Surg 1995;30: 745-7.
K.R. Zhang, H.M. Jia [9] Bowne WB, Lewis JJ, Filippa DA, et al. The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature. Cancer 1999;85:706-17. [10] Yoshizaki K, Matsuda T, Nishimoto N, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman's disease. Blood 1989;74:1360-7.