Author Affiliations: Department of Surgery (Cecka F, Ferko A, Jon B and Šubrt Z), Fingerland Department of Pathology (Kašparová P) and Second Department of Internal Medicine (Repák R), Faculty of Medicine and University Hospital Hradec Králové, Sokolská 581, 500 05 Hradec Králové, Czech Republic; Department of Field Surgery, Military Health Science Faculty, Hradec Králové, Defence University Brno, Trebešská 1575, 500 01 Hradec Králové, Czech Republic (Šubrt Z)

Corresponding Author: Filip Cecka, MD, PhD, Department of Surgery, Faculty of Medicine and University Hospital Hradec Králové, Sokolská 581, 500 05 Hradec Králové, Czech Republic (Tel: 420-737-163931; Fax: 420-495-832026; Email: filip.cecka@seznam.cz)

 

© 2013, Hepatobiliary Pancreat Dis Int. All rights reserved.

doi: 10.1016/S1499-3872(13)60053-3

 

Contributors: CF proposed the study. JB and ŠZ wrote the first draft. FA, KP and RR collected and analyzed the data. All authors contributed to the design and interpretation of the study. CF is the guarantor.

Funding: The work was supported by Research Project MZO 00179906 from the Ministry of Health Care, Czech Republic.

Ethical approval: Not needed.

Competing interest: No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.

 

 

BACKGROUND:Castleman disease is an uncommon lympho-proliferative disorder most frequently occurring in the medias-tinum. Abdominal forms are less frequent, with pancreatic localization of the disease in particular being extremely rare. Only seventeen cases have been described in the world literature.

 

METHOD: This report describes an interesting and unusual case of pancreatic Castleman disease treated with laparoscopic resection.

 

RESULTS: A 48-year-old woman presented with epigastric pain. CT scan showed a well-encapsulated mass on the ventral border of the pancreas. Endosonography with fine needle aspiration biopsy was performed. Biopsy showed lymphoid elements and structures of a normal lymph node. The patient was treated with laparoscopic distal pancreatectomy. The pancreas was transected with a Ligasure device and the pancreatic stump was secured with a manual suture. One year after surgery the patient was complaint-free and showed no signs of recurrence of the disease.

 

CONCLUSIONS:Laparoscopic distal pancreatectomy is a feasible and safe method for the treatment of lesions in the body and tail of the pancreas. Transection of the pancreas with a Ligasure device offers the advantages of low bleeding and low risk of pancreatic fistula.

 

(Hepatobiliary Pancreat Dis Int 2013;12:332-334)

 

KEY WORDS: Castleman disease; pancreas; laparoscopic distal pancreatectomy; Ligasure

 

 

Tumors in the body and tail of the pancreas are often asymptomatic or may present with vague and indistinctive abdominal pain. A list of the most common diagnoses of masses in the body and tail of the pancreas include adenocarcinoma, cystic tumors, and functioning or non-functioning neuroendocrine tumors. Other findings such as Castleman disease are quite rare. The authors report a rare case of pancreatic Castleman disease treated with laparoscopic distal pancreatectomy.

 

 

A 48-year-old woman presented with epigastric pain. The pain was temporary with prompt relief after analgesia. Contrast enhanced CT scan (Fig. 1) revealed a well-encapsulated mass 37×37 mm on the ventral border of the pancreas. The CT scan also showed suspicion of infiltration of the gastric wall, indicating an origin from the muscular layer of the gastric wall (e.g. gastrointestinal stromal tumor (GIST)). Endosono-graphy showed a mass 38×26 mm located between the pancreas and gastric wall (Fig. 2). However, it could not confirm the origin of the tumor. Fine-needle aspiration biopsy showed lymphoid elements, structures of a normal lymph node and structures of clear cylindrical epithelium without dysplasia, possibly originating from a benign mucinous tumor. After preoperative diagnosis, a differential diagnosis was made between a cystic tumor of the pancreas, GIST originating from the gastric wall, or an enlarged lymph node. Laparoscopic exploration and resection of the tumor were decided.

 

During the laparoscopic exploration, a lesser sac was opened through division of the gastrocolic ligament. A tumor was found on the ventral border of the pancreas; it did not cohere to the gastric wall (Fig. 3). Laparoscopic resection was made of the tail of the pancreas with the tumor. The pancreas between the body and tail was transected with a Ligasure device. Afterwards, the pancreatic stump was secured with a manual suture. In the postoperative course, a type A pancreatic fistula (according to International Study Group on Pancreatic Fistula) was observed.^[1] No other complications in the postoperative course were noted and the patient was discharged from the hospital on the seventh postoperative day. A final histological examination showed a finding typical for Castleman disease: an enlarged intrapancreatic lymphatic node with angiofolicular hyperplasia – hyaline-vascular type (Fig. 4). The patient is now being followed up at our department and is complaint-free, showing no signs of recurrence of the disease one year after the surgery.

 

 

Castleman disease is a relatively rare disorder characterized by benign proliferation of lymphoid tissue. Its precise incidence is unknown.^[2] It was first described by Castleman et al in 1954.^[3] There are two histological types of the disease.^[4] The more frequent type is the hyaline-vascular type (85%-90% of cases), which is characterized by abnormal lymphoid follicles, numerous vessels, and wide fibrous septa. The disease is usually asymptomatic. The plasma-cell type is less frequent (10%-15%); it is characterized by large follicles with intervening sheets of plasma cells and few vessels.

 

Castleman disease commonly occurs in the mediastinum (60%-70% of cases). Abdominal forms are less frequent (10%-17%), and the majority of them are retroperitoneal.^[5] Other locations are less common. Pancreatic Castleman disease is extremely rare, with only 17 cases having been described in the literature so far.^{[2, 4, 6-20]}

 

Little is known about the etiology of the disease.^[2, 21] A recent report^[22] suggest the association of Castleman disease with HHV-8 and HIV infections. However, the sources of immune activation in the HHV-8 and HIV negative patients are still unidentified. Other exogenous and endogenous factors may induce IL-6 secretion from B-lymphocytes. Local production of IL-6 may contribute to the characteristic B-cell proliferation and vascularization.^[23] Moreover, in patients with multicentric Castleman disease, systemic symptoms may result from increased production and circulation of IL-6.^[23]

 

The main issue in pancreatic Castleman disease lies in the establishment of clinical diagnosis. Imaging methods (ultrasonography, CT and MRI) have been shown to be helpful in the diagnosis; however, localized Castleman disease may be clinically and radiographically indistinguishable from other lymphoid and non-lymphoproliferative disorders.^{[4, 9]} Castleman disease is not usually included in the list of possible diagnosis, one of the reasons may be its low incidence. CT scan often shows a solid mass with well-defined margins. Dense enhancement immediately after the application of contrast medium is seen. Percutaneous fine-needle aspiration biopsy is often not diagnostic in Castleman disease,^{[5, 21]} which was true for our patient as well. The histological diagnosis of the disease is based on cell architecture, and therefore requires the study of the entire surgical specimen. Complete surgical resection is curative in unicentric forms of Castleman disease ^{[24, 25]} with very good prognosis after complete resection.

 

 

1 Bassi C, Dervenis C, Butturini G, Fingerhut A, Yeo C, Izbicki J, et al. Postoperative pancreatic fistula: an international study group (ISGPF) definition. Surgery 2005;138:8-13. PMID: 16003309

2 Goetze O, Banasch M, Junker K, Schmidt WE, Szymanski C. Unicentric Castleman's disease of the pancreas with massive central calcification. World J Gastroenterol 2005;11:6725-6727. PMID: 16425374

3 Castleman B, Towne VW. Case records of the Massachusetts General Hospital: Case No. 40231. N Engl J Med 1954;250: 1001-1005. PMID: 13165944

4 Wang H, Wieczorek RL, Zenilman ME, Desoto-Lapaix F, Ghosh BC, Bowne WB. Castleman's disease in the head of the pancreas: report of a rare clinical entity and current perspective on diagnosis, treatment, and outcome. World J Surg Oncol 2007;5:133. PMID: 18028547

5 Irsutti M, Paul JL, Selves J, Railhac JJ. Castleman disease: CT and MR imaging features of a retroperitoneal location in association with paraneoplastic pemphigus. Eur Radiol 1999; 9:1219-1221. PMID: 10415265

6 Baikovas S, Glenn D, Stanton A, Vonthethoff L, Morris DL. Castleman's disease: an unusual cause of a peri-pancreatic hilar mass. Aust N Z J Surg 1994;64:219-221. PMID: 8117206

7 Brossard G, Ollivier S, Pellegrin JL, Barbeau P, De Mascarel A, Leng B. Pancreatic Castleman's tumor revealed by prolonged fever. Presse Med 1992;21:86. PMID: 1531532

8 Corbisier F, Ollier JC, Adloff M. Pancreatic localization of a Castleman's tumour. Acta Chir Belg 1993;93:227-229. PMID: 8266757

9 Erkan N, Yildirim M, Selek E, Sayhan S. Peripancreatic Castleman disease. JOP 2004;5:491-494. PMID: 15536288

10 Charalabopoulos A, Misiakos EP, Foukas P, Tsapralis D, Charalampopoulos A, Liakakos T, et al. Localized peri-pancreatic plasma cell Castleman disease. Am J Surg 2010; 199:e51-53. PMID: 20079482

11 Chaulin B, Pontais C, Laurent F, De Mascarel A, Drouillard J. Pancreatic Castleman disease: CT findings. Abdom Imaging 1994;19:160-161. PMID: 8199551

12 Le Borgne J, Joubert M, Emam N, Gaillard F, Lafargue JP, Moussu P, et al. Pancreatic localization of Castleman's tumor. Gastroenterol Clin Biol 1999;23:536-538. PMID: 10416122

13 Lepke RA, Pagani JJ. Pancreatic Castleman disease simulating pancreatic carcinoma on computed tomography. J Comput Assist Tomogr 1982;6:1193-1195. PMID: 7174940

14 LeVan TA, Clifford S, Staren ED. Castleman's tumor masque-rading as a pancreatic neoplasm. Surgery 1989;106:884-887. PMID: 2683174

15 Rhee KH, Lee SS, Huh JR. Endoscopic ultrasonography-guided trucut biopsy for the preoperative diagnosis of peripancreatic Castleman's disease: a case report. World J Gastroenterol 2008;14:2115-2117. PMID: 18395917

16 Soler R, Rodríguez E, Bello MJ, Alvarez M. Pancreatic Castleman's disease: MR findings. Eur Radiol 2003;13:L48-50. PMID: 15018166

17 Talarico F, Negri L, Iusco D, Corazza GG. Unicentric Castleman's disease in peripancreatic tissue: case report and review of the literature. G Chir 2008;29:141-144. PMID: 18419976

18 Tunru-Dinh VW, Ghani A, Tom YD. Rare case of Castleman disease involving the pancreas. Am Surg 2007;73:1284-1287. PMID: 18186391

19 Yilmaz R, Ersin S, Makay O, Akgun E, Yuce G, Elmas N. Pancreatic Castleman's tumour: an unusual case. Acta Chir Belg 2004;104:354-356. PMID: 15285556

20 Petrina A, Eugeni E, Badolato M, Boselli C, Covarelli P, Rondelli F, et al. Unicentric Castleman's disease approached as a pancreatic neoplasm: case report and review of literature. Cases J 2009;2:9090. PMID: 20062727

21 Park JB, Hwang JH, Kim H, Choe HS, Kim YK, Kim HB, et al. Castleman disease presenting with jaundice: a case with the multicentric hyaline vascular variant. Korean J Intern Med 2007;22:113-117. PMID: 17616028

22 Yamasaki S, Iino T, Nakamura M, Henzan H, Ohshima K, Kikuchi M, et al. Detection of human herpesvirus-8 in peripheral blood mononuclear cells from adult Japanese patients with multicentric Castleman's disease. Br J Haematol 2003;120:471-477. PMID: 12580962

23 Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol 2005;129:3-17. PMID: 15801951

24 Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, et al. The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature. Cancer 1999;85:706-717. PMID: 10091744

25 Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972;29:670-683. PMID: 4551306