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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 3  |  Issue : 2  |  Page : 39-42

Castleman's disease: A rare case report of unicentric type in a young female


1 Department of Cardiovascular and Thoracic Surgery, SPS Hospitals, Ludhiana, Punjab, India
2 Department of Surgery, SPS Hospitals, Ludhiana, Punjab, India
3 Department of Pathology, SPS Hospitals, Ludhiana, Punjab, India
4 Department of Anaesthesia, SPS Hospitals, Ludhiana, Punjab, India
5 Department of Plastic Surgery, SPS Hospitals, Ludhiana, Punjab, India
6 Department of Cardiac Surgery, SPS Hospitals, Ludhiana, Punjab, India

Date of Web Publication1-Feb-2016

Correspondence Address:
Manpreet S Salooja
Department of Cardiovascular and Thoracic Surgery, SPS Hospitals, Sherpur Chowk, Ludhiana - 141 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-3846.175212

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  Abstract 


Castleman's disease (CD), or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue. Two clinical entities have been described: A unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. In addition, three histopathological subtypes have been described: Hyaline-vascular (80–90%), plasma cell (10–20%), and a mixed variant. Preoperative diagnosis of CD is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy for unicentric type is the complete surgical excision. Overall prognosis is good, particularly in the unicentric variety of disease. We report a case of 22-year-old female presenting with a large subpectoral mass on left side chest wall below clavicle. It was painless and gradually increasing since 6 months.

Keywords: Castleman's disease, computed tomography, unicentric


How to cite this article:
Salooja MS, Mukherjee KC, Khetrapal H, Srivastava K, Patil K, Gupta A, Chhabra K, Bassi A. Castleman's disease: A rare case report of unicentric type in a young female. Saudi Surg J 2015;3:39-42

How to cite this URL:
Salooja MS, Mukherjee KC, Khetrapal H, Srivastava K, Patil K, Gupta A, Chhabra K, Bassi A. Castleman's disease: A rare case report of unicentric type in a young female. Saudi Surg J [serial online] 2015 [cited 2022 May 27];3:39-42. Available from: https://www.saudisurgj.org/text.asp?2015/3/2/39/175212




  Introduction Top


Castleman's disease (CD) is an unusual benign nonneoplastic lymphoproliferative disease. CD can present as a localised mass first described by Castleman et al. in 1956,[1] or in a more aggressive multicentric form primary reported by Gaba et al.[2] The disease generally involves the mediastinum and abdomen. Peripheral involvement, especially, the head and neck localization are rare. A diagnosis can be suspected clinically, but it is achieved only with histologic analysis. Treatment can range from curative surgery for the localized form to the use of chemotherapy for the multicentric one.


  Case Report Top


A 22-year-old female presented with a painless mass appeared 6 months ago and progressively increasing in size [Figure 1]. There were no constitutional symptoms, including fever, night sweats, fatigue, and weight loss and no alteration in the clinical picture was observed. The rest of the history was unremarkable.
Figure 1: Site and size of mass

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On physical examination, a large subpectoral mass (13 cm × 12 cm × 11 cm) on left side chest wall below clavicle was evident. On palpation, the lesion was asymptomatic, hard, movable, and showed well defined and smooth limits. The size of mass did not change with postural position, and there was no pulsation and bruit on auscultation. There was no cervical lymph node enlargement, but a few left side axillary lymph nodes were palpable.

Ultrasound showed a large subpectoral mass on left side chest wall below clavicle, measuring 13 cm × 12 cm × 11 cm. No abnormalities were identified in other routine blood tests. Computed tomography scan (CT) reported a large intensely enhancing soft tissue density mass lesion in the anterolateral chest wall on left side deep to pectoralis muscles [Figure 2]. Fine needle aspiration cytology was suggestive of lymphoproliferative disorder. Surgical excision was done. Operative findings were a mass 13 cm × 10 cm × 9 cm reaching meadially till sternum and posterolaterally till lateral border of scapula and superiorly till left sternoclavicular joint. Mass was adherent to pectoralis minor muscle, so a small part of muscle was sacrificed. Mass was also compressing the axillary vein and displacing the axillary artery, but these structures were saved during excision of mass. Axillary lymph node dissection up to level 3 was done. Mass and lymph nodes were sent for histopathological examination.
Figure 2: Computed tomography scan of mass

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Histopathology of 10 cm × 7 cm × 5 cm mass showed partial effacement of its architecture with hyperplastic postcapillary venules in interfollicular stroma with absence of sinuses and areas of eosinophilic deposition. Many follicles with prominent dendritic reticulum cells and few hyalinized vessels were seen. In paracortical area, polymorphs inflammatory cells infiltrations are seen (lymphocytes, histiocytes, plasma cells and few eosinophils) [Figure 3], suggestive of lymphoproliferative disorder favoring CD hyaline vascular type. Other axillary lymph nodes show reactive changes and congestion.
Figure 3: Histopathology picture of mass showing features suggestive of Castleman's disease

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Immunohistochemistry markers CD3, CD20, CD30, EMA, and PAX-5 were positive. Hence, the histopathology and immunohistochemistry features are consistent with CD. No definitive evidence of malignancy was seen in sections examined.


  Discussion Top


CD is a rare lymphoproliferative disease that affects lymph nodes and other immune-cell structures of the body. CD represents a distinct clinicopathological entity, first described by Castleman et al. in 1956.[1] CD can be classified as (a) unicentric versus multicentric, based on clinical and radiological findings, (b) hyaline vascular (80–90%) versus plasmacytic (10–20%) versus mixed cellularity variety based on histopathology, and (c) HIV-negative (−) versus HIV-positive (+) based on the HIV status of the patient.

There are two pathological types of CD, namely the hyaline vascular variant and the plasma cell variant.[2] The hyaline vascular variant (unicentric) exhibits a prominent proliferation of small hyalinized follicles with marked interfollicular vascular proliferation whereas the plasma cell variant (multicentric) exhibits hyperplastic germinal centers, sheets of plasma cells in the interfollicular region, proliferation of blood vessels, and persistent sinuses. It is postulated that 10–20% of all cases are of the plasma cell variant, with a small percentage being of mixed histological appearance.

The average age of people with unicentric CD is around the third and fourth decade, whereas most patients with the multicentric form are in their age group of the 50s and 60s. However, for those who are HIV (+), the disease presents at a younger age.[3] HIV seropositive individuals appear to be at an increased risk for multicentric CD (MCD).[2] There is no sex predilection for the disease.

The exact cause of CD remains unknown. Infection by a virus called the human herpes virus 8 (HHV-8) is associated with CD, and it is suspected that the virus may play a causal role, especially in MCD. This virus has also been linked to the development of Kaposi's sarcoma (KS), a cancerous tumor of blood vessel walls.

The unicentric form of CD is asymptomatic in almost half of the cases. It is usually not associated with HHV-8 infection. It is often discovered during routine physical examination, chest X-ray, and abdominal sonography. In the other half of cases, when the lesion is large enough to cause compressive symptoms, the mode of presentation is usually chest pain or abdominal pain. The size of lesions varies widely (1–12 cm) with a mean size of 6 cm. The unicentric form remains localized to only one site, as the name suggests, with preferential involvement in decreasing order of frequencies including abdomen, peripheral lymph nodes and mediastinum. However, it can affect any other site as well. The usual signs and symptoms of the unicentric variety include fullness in the chest or abdomen, low-grade fever, anemia, fatigue, weight loss, profuse sweating, and skin rashes.[4],[5],[6]

The multicentric form, on the other, is often symptomatic.[5] The symptoms are mainly because of the elevated production of interleukin-6 (IL-6). Symptoms include fever, night sweats, loss of appetite, nausea and vomiting, weight loss, weakness or fatigue, anemia, enlarged peripheral lymph nodes, usually around the neck, collarbone, underarm and groin areas, along with enlarged liver or spleen, and peripheral neuropathy. Peripheral polyadenopathy is quite common, with a mean of four sites being involved. Another clinical observation is the association of MCD with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes syndrome.[6] Some multicentric forms are associated with KS as well. It has been reported that among HIV (+) patients with MCD who are infected with HHV-8, up to 70% will develop KS at some time in their clinical course.[7],[8],[9],[10] MCD commonly results in a fatal outcome due to infectious complications, multi-organ failure, and development of malignancies such as lymphoma (Hodgkin's and non-Hodgkin) or KS.[7],[9] The diagnosis of CD is based on clinical evaluation, which includes patient history, laboratory studies like IL-6, C-reactive protein and erythrocyte sedimentation rate, complete blood cell, HIV serology testing with consent, HHV-8 serology, HHV-8 DNA polymerase chain reaction, but the correct diagnosis of this disease is achieved only by histological examination of excised lymph nodes.

In the case being reported, apart from gradually increasing mass over left side chest wall, there were no other complaints. Neurological and endocrine examinations were normal. Acute phase reactants, blood counts, and serological testing for HIV and HHV-8 were also unremarkable; hence a disease of uncertain etiology.

Imaging findings include nonenhanced CT where CD manifests as homogeneous or heterogeneous mass of soft tissue density.[3] CD is intensely enhanced following contrast administration.

The differential diagnosis of CD may include lymphoma, infection (abscess, tuberculosis), sarcoma, and paraganglioma. Based on its CT appearance, the radiologic differential diagnosis for CD in our case study was lymphoma or tuberculosis. On CT, lymphoma is typically a mass of low-attenuation, without significant enhancement or calcification while tuberculosis is typically seen on CT as a mass or multiple masses with necrotic, low-attenuation centers having peripheral enhancement.

The treatment of CD depends upon the variety of disease. Unicentric form is generally curable using surgical resection (with or without radiotherapy).[4] At present, there is no consensus on the optimal management strategy for MCD.[2] Successful treatment of MCD has been achieved using chemotherapy, with or without prednisone, given at the time of initial diagnosis. The chemotherapeutic agents used in the various case series that have yielded remissions include doxorubicin, vincristine, cyclophosphamide, melphalan, and chlorambucil.[4] These have been used as single agents, together with steroids or in combination (e.g. CHOP). Azathioprine and bone marrow transplantation have also been attempted, especially following the failure of CHOP, but have yielded mixed results. Another treatment option includes monoclonal antibodies to IL-6 and suramin (reverse transcriptase inhibitor), which can cause resolution of symptoms and regression of lymphadenopathy; however, there is often recurrence with discontinuation of treatment.

Our case is identical in the sense because chest wall is a rare site of presentation and the age of the patient is also quite rare for presentation. It will help the surgeons to deal with such swellings in a better way and keep the differential diagnosis of CD in mind.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956;9:822-30.  Back to cited text no. 1
    
2.
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
    
3.
Martinez S, Mcadams HP, Erasmus JJ. Mediastinum. In: Haaga JR, editor. CT and MRI of Whole Body. 5th ed. Mosby: Mosby; 2009. p. 1016-7.  Back to cited text no. 3
    
4.
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
    
5.
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
    
6.
Oksenhendler E, Duarte M, Soulier J, Cacoub P, Welker Y, Cadranel J, et al. Multicentric Castleman's disease in HIV infection: A clinical and pathological study of 20 patients. AIDS 1996;10:61-7.  Back to cited text no. 6
    
7.
Peterson BA, Frizzera G. Multicentric Castleman's disease. Semin Oncol 1993;20:636-47.   Back to cited text no. 7
    
8.
Rose C, Mahieu M, Hachullla E. Le POEMS syndrome. Rev med interne 1997;18:553-62.  Back to cited text no. 8
    
9.
Zietz C, Bogner JR, Goebel FD, Löhrs U. An unusual cluster of cases of Castleman's disease during highly active antiretroviral therapy for AIDS. N Engl J Med 1999;340:1923-4.  Back to cited text no. 9
    
10.
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. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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