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Table of Contents
Year : 2018  |  Volume : 15  |  Issue : 2  |  Page : 118-120

A rare cause of retroperitoneal mass: Castleman's disease

1 Department of Surgical Gastroenterology, Indraprastha Apollo Hospital, New Delhi, India
2 Department of Radiology, Indraprastha Apollo Hospital, New Delhi, India

Date of Web Publication5-Jul-2018

Correspondence Address:
G K Adithya
Department of Surgical Gastroenterology, Indraprastha Apollo Hospital, New Delhi - 110 076
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_35_18

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Castleman's disease is a rare lymphoproliferative disorder. It is a pathological diagnosis and rarely diagnosed before surgery. Presentation of disease may be unicentric or multicentric. We are reporting a case of unicentric Castleman's disease presenting as an asymptomatic retroperitoneal mass. The ultrasound-guided aspiration of the lesion was inconclusive. Complete en bloc resection was done and was reported as Castleman's disease after pathological analysis. Pathogenesis of Castleman's disease is not well defined, but interleukin-6 and vascular endothelial growth factor are thought to have a significant role. Two pathological variants have been described as follows: hyaline vascular and plasma cell type. The treatment of unicentric disease is complete resection. Multicentric disease may be associated with human immunodeficiency virus and human herpes virus-8 infection and requires systemic treatment in the form of steroids, chemotherapy, radiotherapy, or immunotherapy.

Keywords: Castleman's disease, human herpes virus-8, human immunodeficiency virus, lymphoproliferative disorder, retroperitoneal mass

How to cite this article:
Jindal S, Adithya G K, Madaan V, Gupta R, Vohra S, Tandon V, Govil D. A rare cause of retroperitoneal mass: Castleman's disease. Apollo Med 2018;15:118-20

How to cite this URL:
Jindal S, Adithya G K, Madaan V, Gupta R, Vohra S, Tandon V, Govil D. A rare cause of retroperitoneal mass: Castleman's disease. Apollo Med [serial online] 2018 [cited 2023 Jan 27];15:118-20. Available from: https://apollomedicine.org/text.asp?2018/15/2/118/236000

  Introduction Top

Castleman's disease (CD) is a rare lymphoproliferative disorder and may present as unicentric or multicentric disease. Unicentric disease is localized to a single lymph node group and most of the patients are diagnosed incidentally on imaging studies. A few patients may be symptomatic as a result of the pressure effects of the mass. Multicentric CD has various presentations, ranging from asymptomatic discrete lymphadenopathy to recurrent episodes of diffuse lymphadenopathy and other systemic symptoms including splenomegaly, anemia, and systemic inflammatory symptoms. Multicentric disease may be seen in the setting of human herpes virus-8 (HHV-8) infection in association with the human immunodeficiency virus (HIV) infection. Optimal therapy for unicentric disease is surgical resection and is curative if lesion is completely resected. Multicentric disease is primarily treated with systemic therapies. CD is a pathological diagnosis and is characterized by features such as germinal center atrophy, onion skin appearance, and central vasculature giving a lollipop appearance. Here, we present a case of unicentric CD located in retroperitoneum and which was resected completely.

  Case Report Top

A 42-year-old male patient presented with a history of mild abdominal discomfort for 6 months. He had no other associated symptoms or comorbidity. An ultrasonography abdomen performed outside had revealed a mass lesion in the left hypochondriac region (? retroperitoneal) and a fine-needle aspiration cytology from the same was reported to be inconclusive. A contrast-enhanced computed tomography (CECT) abdomen was performed which showed a well-defined homogenous enhancing mass lesion in the retroperitoneum, displacing the body, and tail of pancreas anterosuperiorly. The tumor was hypervascular with multiple feeding vessels and distinctly separated from the pancreas and other nearby visceral structures [Figure 1]. A differential diagnosis of neuroendocrine tumor, retroperitoneal sarcoma, and neurogenic tumor was kept in mind. His serum chromogranin A level and other routine investigations were normal. He was planned for surgical excision of tumor. At laparotomy, a large mass of size 8 cm × 6 cm was found to the left of the duodenojejunal flexure, (DJ flexure) at the root of the mesentery, displacing the superior mesenteric vessels to the right side, and pancreas anterosuperiorly [Figure 2]a. The tumor was distinctly separate from the pancreas, bowel loops, and adrenal gland. The tumor was resected en bloc [Figure 2]b. Histopathology revealed hyaline-vascular variant of CD [Figure 3]. Immunoelectrophoresis, IgG, IgM, and IgA levels were normal. Postoperative recovery was uneventful. After 2 months of follow-up, the patient continues to remain asymptomatic. He is planned for a CECT abdomen and chest at 3 months postsurgery.
Figure 1: (a) Coronal computed tomography abdominal image showing the location of the tumor with its increased vascularity. (b) Axial computed tomography image showing uniform arterial enhancement. (c) Axial computed tomography image showing venous phase enhancement. (d) Multiple lymph nodes near the primary tumor showing similar enhancement charateristics

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Figure 2: (a) Intraoperative image showing the exact location of the lesion. It is seen lying below the pancreas and to the left of the DJ flexure. (b) Complete excision of the mass

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Figure 3: (a) Hematoxylin and eosin staining of cut sections showing atretic germinal center (1) and prominent hyalinized capillaries (2). (b) Showed typical features of CD lollipop and onion peel appearance. (c) Bcl-2 staining shows reactive pattern (d) CD31 highlights vasculature with peculiar central location

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  Discussion Top

CD was first reported by Benjamin Castleman in 1956.[1] It is a nonclonal lymphoproliferative disorder, which has been described in the literature by various names such as angiofollicular or giant lymph node hyperplasia, giant benign lymphoma, and lymph node hamartoma. CD may present in a unicentric or multicentric form. According to the histological pattern, CD is divided into two variants as follows: hyaline-vascular variant and plasma-cell variant. A mixed variant has also been reported in some patients.[2] The hyaline-vascular variant presents as a unicentric disease in 76%–90% of cases, while the plasma-cell variant commonly presents as multicentric disease in 80%–90% of cases.

Unicentric disease is localized to a single lymph node or a lymph node group. Patients are generally asymptomatic and may be diagnosed incidentally on imaging, while some may present with symptoms of mass effect. The most common site involved is the mediastinum. Other common sites reported in the literature are the retroperitoneum, abdomen, and neck.[3] The plasma-cell variant of unicentric disease may present with systemic manifestations such as fevers, night sweats, weight loss, and anemia. Multicentric CD affects multiple lymph node regions. These patients often present with systemic symptoms such as fever, night sweats, general malaise, weight loss, and anemia. Generalized lymphadenopathy and hepatosplenomegaly are commonly seen in these patients. Multicentric disease commonly occurs in the setting of HIV infection along with HHV-8 infection. Kaposi sarcoma is commonly associated with CD due to a common association with HHV-8.[4]

Although CD is not a malignant condition, it may be associated with increased risk of certain malignancies such as large B-cell lymphomas and follicular dendritic cell sarcomas. CD may be asscociated with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormality).[5] The hyaline-vascular variant most often present as a highly vascular solitary mass with intense homogeneous CECT.[6] It is difficult to distinguish CD from malignant lesions by radiological imaging including positron-emission tomography.

CD is a pathological diagnosis, made on excisional biopsy of affected lymph node tissue. If complete excision is not possible, then core needle biopsy is preferred over fine-needle aspiration cytology for diagnosis as it retains the architecture of the germinal center and interfollicular zone which is essential for diagnosis. Fine-needle aspiration cytology is not sensitive for diagnosis of this entity and normal appearing cells may be misleading. From a pathological standpoint, common differential diagnosis includes both benign and neoplastic entities, which can be excluded on the basis of careful histological examination, with the use of immunohistochemistry. Other molecular studies such as flow cytometry and molecular genetics may be helpful in some cases.[7]

CD presents in two primary pathologic variants as follows: hyaline vascular type and plasma cell type. A mixed type may be seen in a few patients. As reported in the literature, 80%–90% of unicentric CDs are the hyaline-vascular variant and 10%–20% are plasma cell and remaining are mixed type. Hyaline-vascular variant is characterized by increased numbers of small, hyalinized blood vessels within and between follicles with obliteration of the medullary sinuses. Lymphoid follicles are increased in number with predominance of dendritic cells within germinal centers with a relative paucity of lymphocytes in mantle zones. Plasma cells may be found in the interfollicular region, but they are generally few and present in small clusters. Abundant plasma cells may be seen in the mixed variant.[7] The plasma-cell variant is characterized by a preserved architecture of the lymph nodes with hyperplastic follicles. Follicular dendritic cells are normal in this variant, while interfollicular plasma cells are increased. These plasma cells are usually polyclonal.

The pathogenesis of CD is not clearly defined. However, interleukin 6 (IL6) has been thought to play a pivotal role. IL6 induces a pro-inflammatory state and results in constitutional symptoms. Anti-IL6 therapy is associated with an improvement in symptoms and involution of CD. Surgical removal of bulk of the disease leads to a decrease in levels of IL6 and clinical improvement. The increased vascularity in hyaline-vascular variant is suggestive of a possible role of an angiogenic factor. IL6 is associated with increase expression of vascular endothelial growth factor, which can explain increase angiogenesis in CD. Other proposed mechanisms in case of multicentric disease include human herpes virus-8 (HHV-8) infection, immunodeficiency, and primary defect in germinal center formation.[8] HHV-8 infection is reported to induce human IL-6.

Surgical resection is considered curative for unicentric disease in more than 90% of cases. Small satellite nodules if present subside after resection of bulk of disease. The option for irresectable disease is radiation therapy, which results in complete response in nearly one-half of reported cases.[9]

Multicentric disease is difficult to treat and there is no specific guideline for treatment. Use of corticosteroids is beneficial during acute episodes, but may be associated with relapse during tapering. Higher doses of steroids are not recommended for long term and may lead to complications of the steroids. Lymphoma-like chemotherapy (e.g., cyclophosphamide, doxorubicin, vincristine, and prednisone or cyclophosphamide, vincristine, doxorubicin, and dexamethasone) have been described in treatment with partial response. Rituximab has been used in chronic disease alone or along with steroid and chemotherapeutic agents, but the prognosis is dismal. IL-6 targeted therapy including suramin, tocilizumab, and siltuximab has also been proposed and is under trial at present. Prognosis is unpredictable with each treatment. In the long-term multicentric disease may progress to lymphoma and other malignancies, and hence, regular surveillance is necessary for these patients.[10]

  Conclusion Top

CD is a relatively rare entity and preoperative diagnosis is difficult. It is a pathological diagnosis and requires an experienced pathologist for the same. Unicentric disease is considered curable after complete resection, but multicentric disease is difficult to treat. Multicentric disease may be associated with HIV and HHV-8 infection and these infections should be evaluated in these cases. Association with other malignancies has also been reported; hence, the patient should be properly screened for these disorders in case of multicentric disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Casper C. The aetiology and management of Castleman disease at 50 years: Translating pathophysiology to patient care. Br J Haematol 2005;129:3-17.  Back to cited text no. 1
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;29:670-83.  Back to cited text no. 2
Flendrig JA, Schillings PM. Benign giant lymphoma: The clinical signs and symptoms and the morphological aspects. Folia Med 1969;12:119-20.  Back to cited text no. 3
Gaba AR, Stein RS, Sweet DL, Variakojis D. Multicentric giant lymph node hyperplasia. Am J Clin Pathol 1978;69:86-90.  Back to cited text no. 4
Herrada J, Cabanillas F, Rice L, Manning J, Pugh W. The clinical behavior of localized and multicentric Castleman disease. Ann Intern Med 1998;128:657-62.  Back to cited text no. 5
Ko SF, Hsieh MJ, Ng SH, Lin JW, Wan YL, Lee TY, et al. Imaging spectrum of Castleman's disease. AJR Am J Roentgenol 2004;182:769-75.  Back to cited text no. 6
Ferry J, Harris N. Atlas of Lymphoid Hyperplasia and Lymphoma. Philadelphia: WB Saunders; 1997.  Back to cited text no. 7
Polizzotto MN, Uldrick TS, Wang V, Aleman K, Wyvill KM, Marshall V, et al. Human and viral interleukin-6 and other cytokines in kaposi sarcoma herpesvirus-associated multicentric Castleman disease. Blood 2013;122:4189-98.  Back to cited text no. 8
Dispenzieri A, Armitage JO, Loe MJ, Geyer SM, Allred J, Camoriano JK, et al. The clinical spectrum of Castleman's disease. Am J Hematol 2012;87:997-1002.  Back to cited text no. 9
El-Osta HE, Kurzrock R. Castleman's disease: From basic mechanisms to molecular therapeutics. Oncologist 2011;16:497-511.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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