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Table of Contents
Year : 2020  |  Volume : 17  |  Issue : 4  |  Page : 286-288

Castleman's disease in an adolescent

Department of Surgery, Kasturba Medical College, Manipal, Karnataka, India

Date of Submission03-Jun-2020
Date of Decision19-Oct-2020
Date of Acceptance28-Oct-2020
Date of Web Publication03-Dec-2020

Correspondence Address:
Ujjwala Singh
Department of Surgery, Kasturba Medical College, Manipal, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_47_20

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Castleman’s disease is a clinic-pathological entity of unknown etiology with non–neoplastic lymph node hyperplasia. It is extremely rare and can be found in patients of all age groups. It can present as localized with local signs and symptoms or disseminated disease which includes a wide range of systemic problems. Our patient is a 15-year-old adolescent female with nonspecific features of abdominal pain, vomiting. Blood and Imaging studies were inconclusive and the diagnosis of Castleman’s diseases was confirmed by histopathology. Here, we discuss the importance of considering Castleman’s diseases as a differential diagnosis for a case of acute abdomen.

Keywords: 15-year-old, Castleman’s disease, case of acute abdomen

How to cite this article:
Singh U, Nayak MS, Singh S, Shenoy K R. Castleman's disease in an adolescent. Apollo Med 2020;17:286-8

How to cite this URL:
Singh U, Nayak MS, Singh S, Shenoy K R. Castleman's disease in an adolescent. Apollo Med [serial online] 2020 [cited 2021 Jan 25];17:286-8. Available from: https://www.apollomedicine.org/text.asp?2020/17/4/286/302211

  Introduction Top

Dr. Benjamin Castleman described the typical pathology of mediastinal lymph node hyperplasia now carrying his name first in a case report in 1954 and later in a series of 13 patients in 1956 . It is a unicentric(UCD) or multicentric(MCD)disease of the lymph node with or without polyclonal proliferation of B cells. Histopathology identifies a hyaline-vascular type, a plasma-cell variant or a mix of both. It usually presents as localized or systemic lymphadenopathy or even as extranodal mass and may give rise to several differential diagnoses. The most important feature of HVCD (hyaline vascular Castleman disease) is the presence of abnormal germinal centres with hyaline-vascular transformation, sometimes showing multiple germinal centers within a single reactive lymphoid follicle, this outlining HVCD as a disorder of follicular dendritic cells.The pathogenesis of Castleman disease is not fully understood; however , central roles of interlukin (IL) 6 in UCD and both IL-6 and human herpesvirus (HHV) 8 in MCD has been well described.In this review ,we discuss the systemic occurrence of Castleman disease and an important differential to be considered in a case of acute abdomen.

  Case Report Top

A 15-year-old female presented with complaints of pain abdomen on the right side of the abdomen and vomiting for the past 2 weeks. Pain abdomen was acute in onset, intermittent, colicky in nature, and localized to the right side of the abdomen. She also complained of two episodes of vomiting. The vomitus contained food particles and was not bloodstained. Physical examination revealed a nondistended abdomen with tenderness on deep palpation in right hypochondrium and no palpable mass. Bowel sounds were normal.

Complete blood counts, renal function tests and liver function tests were done normal. Computed tomography (CT) of the abdomen was suggestive of well-differentiated vascular cystic structure in the right lumbar/paraspinal area. Ultrasonography (USG) abdomen was suggestive of homogenous wall enhancing lesion with internal vascularity suggestive of retroperitoneal mass, most likely paranganglioma. To rule out phaeochromocytoma, 24 h urine Vanillylmandelic acid (VMA) was advised which revealed a normal level of 5.6. USG-guided fine-needle aspiration cytology was performed which reported the sample as inadequate for opinion. The patient was due to appear for Grade 10th board exams in 3 weeks and sought to be discharged.

However, 4 days later she came with another episode of severe abdominal pain and was admitted for exploration. A 6 cm transverse laparotomy incision was given just above the umbilicus. Intraoperatively, a 4 cm × 3 cm retroperitoneal cyst was noted in the infracolic compartment just below the hepatic flexure [Figure 1]. The cyst was excised and sent for histopathological evaluation [Figure 2]. Postoperative period was uneventful and the patient was discharged on the 5th postoperative day.
Figure 1: Cyst seen in infracolic gutter

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Figure 2: Cyst sent for histopathological examination

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Histopathology examination showed Castleman disease, Hyaline Vascular Type-Stroma Rich Variant. The patient was followed up and at the time of reporting this article has been symptom-free for 8 months.

  Discussion Top

Castleman’s Disease, first described by Castleman in 1956[1] is a clinicopathological entity of unknown etiology. It is a rare non–neoplastic lymphoproliferative disease characterized histologically by angiofollicular lymph node hyperplasia. CD is also known as “Giant lymph node hyperplasia,” “Lymph node hamartoma,” “Benign lymphoma,” and? “angiomatous lymphoid hyperplasia”.

The prevalence of CD is reported to be <1/100,000 in the general population.[2] Even though it is predominantly reported in adults in the available literature it can occur at any age, with a third, fourth, and fifth decade of life being the most common.[3],[4] Few series reports increased incidence among females but overall no sex predilection could be established.[5],[6]




Evidence suggests an interleukin-6 (IL-6) role in the pathogenesis of systemic disease due to activation of various immune-mediated mechanisms and chronic viral antigenic stimulation leading to proliferation and maturation of lymphocytes, mostly by human herpesvirus-8 (HHV–8) and HIV.[7],[8],[9]

Clinical features

The localized form of the disease as seen in our patient usually involves a single lymph node group and is mostly asymptomatic as they are often HV-CD. They are often discovered incidentally during a routine examination. In PC-CD, constitutional symptoms such as fever, night sweats, and weight loss are very frequent like in disseminated CD.[3] The site and frequency of involvement are variable but commonly are abdominal, mediastinal, and peripheral.[10] Mediastinal masses can present with local compression symptoms and abdominal masses can present with abdominal pain.[11],[12] Diagnosis can often be difficult as the symptoms are nonspecific and point towards multiple diagnoses.

On the other hand, disseminated CD often involves multiple lymph nodes with an array of systemic symptoms such as fever, asthenia, anemia, skin rash, and hepatosplenomegaly which are thought to arise due to elevated IL-6 production. Uncommon presentations include renal dysfunction, polyneuropathy, pleural effusion, ascites, etc., Disseminated CD can present in HIV positive patients, who have a prevalence of pulmonary symptoms. These patients often develop Kaposi Sarcoma in the course of their illness and have a higher probability of progression to HHV8 associated NHL.[13]


Blood investigation often reveals nonspecific findings such as anemia, elevated acute phase reactants, raised erythrocyte sedimentation rate, thrombocytosis, and low serum albumin. Contrast enhanced CT reveals a lesser degree of enhancement in PC-CD. T2 weighted magnetic resonance imaging shows hyperintensity in comparison to skeletal muscle. Histopathological examination is the most accurate way to diagnose CD and will show B-lymphocytes with polyclonal surface and cytoplasmic immunoglobulin markers. A HIV test must be administered to the patient as CD is commonly associated with HIV and infection with HHV 8.


The treatment for CD is widely debated but as in our case, localized CD is commonly treated with surgical excision as recurrence rate is low and it has a good prognosis. However, in tumors that cannot be resected partial resection with radiation or only radiation can be given, the former is known to reduce the probability of recurrence. Reports of gradual spontaneous complete remission in asymptomatic disease can also be found.

Contrary to this in cases of disseminated CD, the treatment is rather complicated and there is no treatment consensus so far. The prognosis is considered variable due to various complications of the disease such as POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal antibody, and skin changes), sarcoidosis, amyloidosis, NHL (in HIV positive patients), infections, and autoimmune anemia. Various modalities treatment have been used in disseminated CD such as hemotherapy, radiotherapy, Anti-IL 6 Antibodies, and Anti CD20 antibody (Rituximab). Chemotherapy has been successful in limited cases, regimens used against Hodgkin’s Lymphoma (e.g. cyclophosphamide, vincristine, doxorubicin, and prednisolone) are commonly used. Response to radiotherapy has been reported to be equivocal unlike in localized CD. Recent trials suggest that anti-IL 6 antibodies and rituximab can markedly improve the outcome.

Learning points

  1. Castleman’s disease be considered as a differential diagnosis in a case of acute abdomen
  2. Symptoms of Castleman’s disease depend upon the location of the tumor and are nonspecific
  3. Cases of confirmed Castleman’s disease must be administered an HIV test as it is commonly seen in HIV patients
  4. High degree of suspicion and early intervention are key to a successful outcome in CD patients.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956;9:822-30.  Back to cited text no. 1
Dégot T, Métivier A, Casnedi S, Chenard M, Kessler R. Thoracic manifestations of Castleman’s disease; Rev Pneumol Clin 2009;65:101-7.  Back to cited text no. 2
Chronowski GM, Ha CS, Wilder RB, Cabanillas F, Manning J, Cox JD. Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer 2001;92:670-6.  Back to cited text no. 3
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. 4
Kraus DH, Huo J, Burt M. Surgical access to tumors of the cervicothoracic junction. Head Neck 1995;17:131-6.  Back to cited text no. 5
Smir BN, Greiner TC, Weisenburger DD. Multicentric angiofollicular lymph node hyperplasia in children: A clinicopathologic study of eight patients. Mod Pathol 1996;9:1135-42.  Back to cited text no. 6
Yoshizaki K, Matsuda T, Nishimoto N, Kuritani T, Taeho L, Aozasa K, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman’s disease. Blood 1989;74:1360-7.  Back to cited text no. 7
Amin HM, Medeiros LJ, Manning JT, Jones D. Dissolution of the lymphoid follicle is a feature of the HHV8+ variant of plasma cell Castleman’s disease. Am J Surg Pathol 2003;27:91-100.  Back to cited text no. 8
Oksenhendler E. HIV-associated multicentric Castleman disease. Curr Opin HIV AIDS 2009;4:16-21.  Back to cited text no. 9
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. 10
McAdams HP, Rosado-de-Christenson M, Fishback NF, Templeton PA. Castleman disease of the thorax: Radiologic features with clinical and histopathologic correlation. Radiology 1998;209:221-8.  Back to cited text no. 11
Kim SH, Min BW, Kim WB, Park SS, Um JW, Lee JB, et al. Mesenteric Castleman’s disease. Yonsei Med J 2005;46:289-91.  Back to cited text no. 12
Oksenhendler E, Boulanger E, Galicier L, Du MQ, Dupin N, Diss TC, et al. High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease. Blood 2002;99:2331-6.  Back to cited text no. 13


  [Figure 1], [Figure 2]


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