Surgical resection for pelvic retroperitoneal Castleman's disease: A case report and review literature
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- Published online on: January 21, 2021 https://doi.org/10.3892/br.2021.1405
- Article Number: 29
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Copyright: © Kitakaze et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
Abstract
Introduction
Castleman disease (CD) is a rare atypical lymphoproliferation disorder, also known as angiofollicular hyperplasia (1). CD was first reported by Benjamin Castleman in 1954 and defined 1956(2). Clinically, CD is classified as unicentric or multicentric CD based on the anatomical distribution (3). Unicentric CD primarily affects the mediastinum, and rarely affects the retroperitoneal or pelvic locations (4). The standard treatment for unicentric CD is complete surgical resection (5). However, in some cases, it may not be possible to resect the problematic mass due to a high degree of attachment with other organs or hypervascularity (6). Preoperative angiography and embolization of the arteries that feed the problematic mass can reduce intraoperative bleeding in cases of CD with hypervascularity (7).
In the present case report, a rare case of unicentric CD presented as a pelvic retroperitoneal mass. Due to the hypervascularity of the mass, preoperative embolization was performed. The mass was completely resected without any complications. Additionally, a review of the literature on pelvic CD and preoperative embolization of CD was performed to provide an up-to-date reference on the management and outcomes of patients with CD.
Case report
A 44-year-old man presented with a history of diarrhea at another hospital. He was diagnosed with acute enteritis with computed tomography (CT), and the diarrhea was relieved after a few days. The CT scan incidentally revealed a pelvic retroperitoneal mass with calcification, and he was referred to Osaka University Hospital. The patient underwent appendectomy for appendicitis 30 years ago, and had no viral infection or history of any other diseases. The pelvic calcification was previously identified in previous abdominal X-rays, but further examination was not performed. Physical examination revealed no abnormal symptoms. Laboratory blood tests, including for tumor makers (CA19-9 and carcinoembryonic antigen) were normal. Any abnormal finding was not detected by colonoscopy. The abdominal contrast-enhanced CT scan revealed a well-defined 50x30 mm mass behind the sigmoid mesenteric, under the bifurcation of the aorta in the pelvic retroperitoneal. Non-enhanced phase imaging revealed coarse calcification inside the mass, and evident contrast enhancement was observed in the mass during the arterial phase (Fig. 1). Magnetic resonance imaging (MRI) also revealed a well-defined 50x30 mm solid mass situated in the pelvic retroperitoneal. The mass demonstrated heterogeneous and moderately hyperintense signal intensity, and the low signal intensity corresponded to calcification in the T2-weighed images and diffusion-weighted images (Fig. 2). A positive emission tomography/CT scan was performed to exclude the possibility of paraneoplastic manifestations of a primary tumor, and it revealed a 50x30 mm space-occupying lesion with hypermetabolic activity (SUVmax at 4.1) (Fig. 3). Possible differential diagnosis based on the images were CD, primary mesenteric gastrointestinal stromal tumor or leiomyoma. At first, a diagnosis of CD was doubted as the tumor had calcification, exhibited a strong contrast in imaging, had an uniform edge and a relatively uniform inside on the abdominal CT scan; the tumor was generally isointense on T1 weighted images and hyperintense on T2 images (8). Surgical resection following embolization was suggested. Angiographically, the tumor was hypervascular with a dense capillary blush, and it was supplied by the middle sacral artery (Fig. 4). The vasculature of the mass was embolized by DMSO and the patient was operated on the following day.
During the laparotomy, the mass was located at the bifurcation of the aorta behind the sigmoid mesentery. Mobilization of the sigmoid mesentery revealed that the mass was 50x30 mm in size, rubbery, rich in vasculature and exhibited a high-degree of attachment to the left common iliac vein. Following surgical ligation and dissection of the vasculature to the mass, the mass was completely resected from the adjacent organs without any complications. The patient lost 160 ml of blood, but no blood transfusion was required. The excised mass was round, well circumscribed and encapsulated. The cut surface was dark red with a central white zone of fibrosis and calcification, and it had a granular appearance (Fig. 5). Histopathological examination revealed the lymphoid tissue had a hyalinized vasculature, calcification and noticeable hemorrhaging. Furthermore, a germinal center was not observed, and thus germinal center atrophy was suspected (Fig. 6). Immunohistochemical analysis showed protein expression of CD3, CD20 and CD79a. Immunohistochemistry did not show an increase in IgG4 antibody expression compared with total immunoglobulin expression. Clonality analysis using genomic DNA extraction from the surgical specimen showed no clonality and DNA fragmentation. These histological findings suggested CD of a hyaline vascular (HV) type. Currently, at 20 months post-operation, the patient has not experienced a recurrence. A schematic of this case is shown (Fig. 7).
Discussion and literature review
The classification of CD into unicentric or multicentric CD is based on the presence of this lymphoproliferative disorder in one or more regions, respectively (9). There are three histopathological types of the disease, HV, plasmacytic (PC) and mixed type (10). HV type occurs in 80-90% of cases and usually appears more frequently as a unicentric localization, whereas PC is primarily multicentric and accounts for 10-20% of cases (11). Furthermore, 90% of patients with unicentric CD are usually asymptomatic (1). The large lymph node due to unicentric CD is located only at a single site, exhibits slow progression and is rarely observed in radiographs (1). CD is often overlooked as a possible diagnosis due to its low incidence rate. The possibility of CD should be considered following the identification of a homogeneous vascular mass (8). CD most commonly affects the mediastinum (63%), followed by the abdomen (11%), retroperitoneum (7%) and axilla (4%) (12).
Unicentric CD in the retroperitoneum is commonly found in the retroperitoneal space (53%), followed by the pararenal (15%), peripancreatic (9.7%) and pelvic regions (6.7%) (4). The most common presentation is abdominal pain (42%) (13). Due to its rarity and lack of disease-specific makers and indications, preoperative diagnosis is difficult. The differential diagnosis includes lymphoma, sarcoma, lymph node metastasis, gastrointestinal stromal tumor, lipomas, leiomyomas, neurofibromas, paraganglioma and infectious/inflammatory diseases (14). The imaging findings of unicentric CD are commonly seen on contrast-enhanced CT as a well-defined, solitary soft tissue tumor with evident contrast enhancement during the arterial phase (12). Most unicentric CD lesions are isointense or slightly hyperintense relative to skeletal muscle on T1-weighted images, and hyperintense on T2-weighted images, reflecting the vascularity of the mass (15). The first choice of treatment for unicentric CD is surgical resection if it is curatively resectable; the 10-year overall survival rate is 95% and the 5-year disease-free survival rate is over 90%, suggesting a good prognosis following complete resection (16).
All previously reported cases of abdominal, retroperitoneal and pelvic unicentric CD were searched in PubMed, focusing on studies published in English with images to support the location of the masses identified. A total of 152 cases of abdominal, retroperitoneal and pelvic unicentric CD were found (as of July 2020). A summary of the areas of the abdomen where unicentric CD has been reported is shown in Fig. 8. The most frequently reported site was the superior mesenteric artery feeding mesentery (25%; 38/152). In the retroperitoneal, the paraaortic and left peri-renal areas were found to be the most common: 13.8% (21/152) and 11.2% (17/152), respectively. A small number of cases have also been reported in solid organs such as the liver, pancreas and kidneys (17-19). Pelvic unicentric CD occurred less frequently than intra-abdominal or extra pelvic retroperitoneal unicentric CD, accounting for 15.1% (25/152) cases of abdominal unicentric CD.
Intraabdominal presentations of CD were the second most common location, and pelvic presentations were rare. The present case report was compared with other reported cases in which unicentric CD was present as a pelvic mass. There were 10 cases, and the clinical data and surgical outcomes of these patients are reviewed and listed in Table I. The mean age of the patients was 35.4 years, and the mean greatest diameter of the lesion was 5.88 cm. HV type was observed in 10 out of 11 cases. Furthermore, 2 cases were treated using a laparoscopic approach. All cases in Table I were treated with complete resection and there were no cases of recurrence. Unicentric CD with calcification was found in 2 cases in Table I. The case reported in the present study was the only case in which calcifications were present, and was resected after embolization for pelvic CD.
Table ISummary of the clinical data and outcomes of patients with pelvic unicentric Castleman's disease who underwent surgical resection. |
Several previous cases were diagnosed with abdominal unicentric CD following post-surgical histological examination. The optimal therapy for unicentric CD is surgical resection, which is usually curative if the disease is amenable to complete resection (5). Surgical resection is a useful approach for the diagnosis and treatment of the disease (8).
The masses found in patients with CD often exhibit a moderate to high degree of attachment contiguous with surrounding anatomical structures (6). A high degree of attachment to the contiguous anatomical structures is often observed in lesions >5 cm in diameter (6). Furthermore, significant bleeding may obstruct surgical procedures (4).
In cases of HV-type CD where there is a notably higher risk of massive bleeding due to the hypervascularity, preoperative angiography and embolization of the arteries that supply the tumor should be considered to reduce intraoperative bleeding (7). Preoperative embolization has also been suggested where there is encasement or invasion of the adjacent structures (20-22).
The present case was compared with the other reported cases in which patients with unicentric CD were treated using complete surgical resection after angiography and embolization of the feeding artery. There were 10 such cases, and the clinical data and surgical outcomes of these patients were reviewed and are listed in Table II. The mean age of the patients was 28.6 years and the mean greatest diameter of the lesion was 8.58 cm. HV type was observed in 10 of 11 cases (aforementioned 10 cases and the present case; Table II) The mean blood loss during operation ranged from minimal to 940 ml, and the clinical course was uneventful in all cases (Table II). Preoperative embolization may affect the histological findings on the resected specimens. In relation to the histological findings after embolization, fibrosis and marked hemorrhage were reported.
Table IISummary of clinical data and outcomes in patients with unicentric Castleman's disease who underwent preoperative embolization. |
In the present case, the patient had previously been shown to possess a pelvic calcification in an abdominal x-ray. It has been reported that calcifications are seen in 31% of patients with abdominal or pelvic CD (23). Pelvic calcifications are usually indicative of neurogenic tumors, teratomas, uterine fibroids and intravesical stones, amongst other potential conditions (24,25). However, it is important to consider the possibility of pelvic CD in the differential diagnosis of a pelvic calcification in an abdominal X-ray.
In conclusion, CD is a rare lymphoproliferation disorder of uncertain etiology. Pelvic CD is extremely rare, so it is important to consider CD as a differential diagnosis when a pelvic lesion is found. Although the clinical course of complete surgical resection for unicentric CD is good, surgical resection may be difficult due to attachment with the surrounding tissues or high hypervascularity. Preoperative angiography and embolization of the arteries feeding the tumor can prevent or limit intraoperative bleeding.
Acknowledgements
Not applicable.
Funding
No funding was received.
Availability of data and materials
All data generated and/or analyzed in the present study are included in this published article.
Authors' contributions
MK, NM, SF, TO, HT, MU, TM, YD and HE contributed to the diagnosis at the preoperative conference, NM and SF performed the resection, and contributed to the follow-up. All authors read and approved the final manuscript.
Ethics approval and consent to participate
Not applicable.
Patient consent for publication
Written informed consent was obtained from the patient for the publication of this case report and the accompanying images.
Competing interests
The author declare that they have no competing interests.
References
Ren N, Ding L, Jia E and Xue J: Recurrence in unicentric Castleman's disease postoperatively: A case report and literature review. BMC Surg. 18(1)2018.PubMed/NCBI View Article : Google Scholar | |
Castleman B, Iverson L and Menendez VP: Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 9:822–830. 1956.PubMed/NCBI View Article : Google Scholar | |
Li XF, Liu JZ, Zhang CJ and Miao Q: Unicentric Castleman's disease with Cardiovascular Involvement. Chin Med Sci J. 32:198–200. 2017.PubMed/NCBI View Article : Google Scholar | |
Gopi P, Potty VS, Kaurav RS and Govindan K: Unicentric Castleman's disease as a localized retroperitoneal mass: A case report and review of literature. Int J Appl Basic Med Res. 8:259–262. 2018.PubMed/NCBI View Article : Google Scholar | |
Soumerai JD, Sohani AR and Abramson JS: Diagnosis and management of Castleman disease. Cancer Control. 21:266–278. 2014.PubMed/NCBI View Article : Google Scholar | |
Ko SF, Ng SH, Hsieh MJ, Lin JW, Huang CC, Lee TY and Chen WJ: Castleman disease of the pleura: Experience with eight surgically proven cases. Ann Thorac Surg. 76:219–224. 2003.PubMed/NCBI View Article : Google Scholar | |
Madan R, Chen J-H, Trotman-Dickenson B, Jacobson F and Hunsaker A: The spectrum of Castleman's disease: Mimics, radiologic pathologic correlation and role of imaging in patient management. Eu J Radiol. 81:123–131. 2012.PubMed/NCBI View Article : Google Scholar | |
Tampakis A, Tampaki EC, Daikeler T and Lardinois D: Intrathoracic tumor of the chest wall: A case of Castleman's disease mimicking myositis of the lower extremities. Gen Thorac Cardiovasc Surg. 65:664–666. 2017.PubMed/NCBI View Article : Google Scholar | |
Svensson JF, Marshall-Heyman M, Lindgren F and Ghaffarpour N: Minimal access surgery in Castleman disease in a child, a case report. J Pediatr Surg Case Rep. 3:289–291. 2015. | |
Bracale U, Pacelli F, Milone M, Bracale UM, Sodo M, Merola G, Troiani T and Di Salvo E: Laparoscopic treatment of abdominal unicentric Castleman's disease: A case report and literature review. BMC Surg. 17(38)2017.PubMed/NCBI View Article : Google Scholar | |
Xu J, Zhou BO, Cao HL, Wang BO, Yan S and Zheng SS: Surgical management of isolated retroperitoneal Castleman's disease: A case report. Oncol Lett. 11:2123–2126. 2016.PubMed/NCBI View Article : Google Scholar | |
Bucher P, Chassot G, Zufferey G, Ris F, Huber O and Morel P: Surgical management of abdominal and retroperitoneal Castleman's disease. World J Surg Oncol. 3(33)2005.PubMed/NCBI View Article : Google Scholar | |
Vassos N, Raptis D, Lell M, Klein P, Perrakis A, Köhler J, Croner RS, Hohenberger W and Agaimy A: Intra-abdominal localized hyaline-vascular Castleman disease: Imaging characteristics and management of a rare condition. Arch Med Sci. 12:227–232. 2016.PubMed/NCBI View Article : Google Scholar | |
Jiang Y, Hou G, Zhu Z, Huo L, Li F and Cheng W: The value of multiparameter 18F-FDG PET/CT imaging in differentiating retroperitoneal paragangliomas from unicentric Castleman disease. Sci Rep. 10(12887)2020.PubMed/NCBI View Article : Google Scholar | |
Wong RSM: Unicentric Castleman disease. Hematol Oncol Clin North Am. 32:65–73. 2018.PubMed/NCBI View Article : Google Scholar | |
Talat N, Belgaumkar AP and Schulte KM: Surgery in Castleman's disease: A systematic review of 404 published cases. Ann Surg. 255:677–684. 2012.PubMed/NCBI View Article : Google Scholar | |
Masoum SHF, Nooghabi AJ and Rezaei R: Castleman's disease: Report of four cases and review of the literature. Acta Medica Iranica. 56:132–136. 2018. | |
Goetze O, Banasch M, Junker K, Schmidt WE and Szymanski C: Unicentric Castleman's disease of the pancreas with massive central calcification. World J Gastroenterol. 11:6725–6727. 2005.PubMed/NCBI View Article : Google Scholar | |
Miyoshi H, Mimura S, Nomura T, Tani J, Morishita A, Kobara H, Mori H, Yoneyama H, Deguchi A, Himoto T, et al: A rare case of hyaline-type Castleman disease in the liver. World J Hepatol. 5:404–408. 2005.PubMed/NCBI View Article : Google Scholar | |
Jang SM, Han H, Jang KS, Jun YJ, Lee TY and Paik SS: Castleman's disease of the renal sinus presenting as a urothelial malignancy: A brief case report. Korean J Pathol. 46:503–506. 2012.PubMed/NCBI View Article : Google Scholar | |
Husainy MA, Sayyed F and McPherson SJ: Castleman's disease: A rare indication for endovascular therapy for hemoptysis. Indian J Radiol Imaging. 27:33–35. 2017.PubMed/NCBI View Article : Google Scholar | |
Swee W, Housseini AM, Angle JF, Jones DR, Daniel TM, Turba UC, Abdel-Gawad EA and Hagspiel KD: Preoperative embolization of Castleman's disease using microspheres. Ann Thorac Surg. 88:1999–2001. 2009.PubMed/NCBI View Article : Google Scholar | |
Robert JH, Sgourdos G, Kritikos N, Didier D and Terraz S: Preoperative embolization of hypervascular Castleman's disease of the mediastinum. Cardiovasc Interven Radiol. 31:186–188. 2008.PubMed/NCBI View Article : Google Scholar | |
Meador TL and McLarney JK: CT features of Castleman disease of the abdomen and pelvis. Am J Roentgenol. 175:115–118. 2000.PubMed/NCBI View Article : Google Scholar | |
Sadamoto Y, Abe Y, Higuchi K, Kato K, Matsumoto S, Arima N and Nawata H: Retroperitoneal Castleman's disease of the hyaline vascular type presenting arborizing calcificatio. Intern Med. 37:691–693. 1998.PubMed/NCBI View Article : Google Scholar | |
Menenakos C, Braumann C, Hartmann J and Jacobi CA: Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: Report of a case and review of the literature. World J Surg Oncol. 5(45)2007.PubMed/NCBI View Article : Google Scholar | |
Sato A: Castleman's disease in the pelvic retroperitoneum: A case report and review of the Japanese literature. Int J Surg Case Rep. 4:19–22. 2013.PubMed/NCBI View Article : Google Scholar | |
Al-Natour S, Sawalhi S, Al-Muhtady D and Hijazi E: Mesenteric Castleman's disease: Case report and literature review. Asian J Surg. 33:150–153. 2010.PubMed/NCBI View Article : Google Scholar | |
Yu G, Cao F, Gong H, Liu P, Sun G and Zhang W: Embolization of blood-supply artery followed by surgery for treatment of mesorectal Castleman's disease: Case report and literature review. Gastroenterol Rep. 7:141–145. 2019.PubMed/NCBI View Article : Google Scholar | |
Benjamin B, Zaltzman R, Shpitz B, Gordon CR and Avital S: Presacral mass discovered during pregnancy followed by myasthenia gravis. Isr Med Assoc J. 17:318–320. 2015.PubMed/NCBI | |
Hwang MR, Chang HJ, Kim MJ, Seo GJ, Yoo SB, Park JW, Choi HS and Oh JH: Castleman's disease of the mesorectum: Report of a case. Surg Today. 41:271–275. 2011.PubMed/NCBI View Article : Google Scholar | |
Watson G, Keane A and Chawdhery Z: Pelvic Castleman's disease shown by angiography and MRI. Eur Radiol. 10:1837–1839. 2000.PubMed/NCBI View Article : Google Scholar | |
Zhang KR and Jia HM: Mesenteric Castleman disease. J Pediatr Surg. 43:1398–1400. 2008.PubMed/NCBI View Article : Google Scholar | |
Schelble AP and Merritt DF: Pelvic Castleman's disease presenting as an adnexal mass in an adolescent. J Pediatr Adolesc Gynecol. 32:86–89. 2019.PubMed/NCBI View Article : Google Scholar | |
Guthrie PJ, Thomas JV, Peker D, Turkbey B and Rais-Bahrami S: Perivesical unicentric Castleman disease initially suspected to be metastatic prostate cancer. Urol Annals. 8:245–248. 2016.PubMed/NCBI View Article : Google Scholar | |
Nagano S, Yokouchi M, Yamamoto T, Kaieda H, Setoguchi T, Hiraki T, Tashiro Y, Yonezawa S and Komiya S: Castleman's disease in the retroperitoneal space mimicking a paraspinal schwannoma: A case report. World J Surg Oncol. 11(108)2013.PubMed/NCBI View Article : Google Scholar | |
Gorospe L, Valdebenito-Montecino AP and Munoz-Molina GM: Preoperative embolization of mediastinal Castleman's disease presenting with stroke. Asian Cardiovasc Thorac Ann. 25:158–159. 2017.PubMed/NCBI View Article : Google Scholar | |
Sanchez-Ros-Sanchez A, Infante-Cossio P, Gonzalez-Garcia A and Borrero-Martin JJ: Preoperative embolization for the treatment of cervical Castleman disease. J Craniofac Surg. 23:e257–e259. 2012.PubMed/NCBI View Article : Google Scholar | |
Aydemir B, Okay T, Imamoglu O, Sahin S and Dogusoy I: Preoperative embolization in mediastinal Castleman's disease. Thorac Cardiovasc Surg. 58:496–498. 2010.PubMed/NCBI View Article : Google Scholar | |
Safford SD, Lagoo AS and Mahaffey SA: Preoperative embolization as an adjunct to the operative management of mediastinal Castleman disease. J Pediatr Surg. 38:E21–E23. 2003.PubMed/NCBI View Article : Google Scholar | |
Williams HR, Millner PA and Coral A: Castleman's disease of the erector spinae muscle. Skel Radiol. 27:637–640. 1998.PubMed/NCBI View Article : Google Scholar | |
Amano Y, Takai D, Ohishi N, Shinozaki-Ushiku A, Fukayama M, Akahane M, Nakajima J and Nagase T: Successful treatment of mediastinal unicentric Castleman's disease using video-assisted thoracoscopic surgery with preoperative embolization. Case Rep Med. 2013(354507)2013.PubMed/NCBI View Article : Google Scholar |