|Year : 2014 | Volume
| Issue : 1 | Page : 73-75
Castleman's disease: Hyaline vascular type
Srikanth Shastry, Anandam Gadda
Department of Pathology, Prathima Institute of Medical Sciences, Karimnagar. A.P, India
|Date of Web Publication||10-Dec-2013|
Department of Pathology, Prathima Institute of Medical Sciences, Karimnagar, A.P
Source of Support: None, Conflict of Interest: None
Castleman's disease is a rare disease of lymph node with two identified forms, the hyaline vascular type and plasma cell type. It presents as localized or systemic lymphadenopathy or even as extranodal mass and may give rise to several differential diagnoses. Castleman's disease represents a morphologically distinct form of lymph node hyperplasia rather than a neoplasm or a hamartoma. It occurs most commonly in adults but it can also affect children. Here we present a case of Castleman's disease in a 22 year male patient involving right cervical lymphnode.
Keywords: Castleman′s disease, cervical lymph node, hyaline vascular type
|How to cite this article:|
Shastry S, Gadda A. Castleman's disease: Hyaline vascular type. Med J DY Patil Univ 2014;7:73-5
| Introduction|| |
Castleman's disease (CD) is a rare benign lymphoproliferative disorder that may present as a nodal or extra nodal mass. , Synonyms include angiomatous lymphoid hamartoma, angiofollicular hyperplasia, giant lymph node hyperplasia and follicular lymphoreticuloma.  Microscopically, two distinct histological patterns have been described - the hyaline vascular (HV) type or angiofollicular type and plasma cell (PC) type. ,, CD may be confused with other causes of lymphadenopathy. Though CD is a relatively rare entity it should be strongly considered in the differential diagnosis of cervical lymphadenopathy. Here we present a case of CD hyaline vascular type involving right cervical lymph node in a 22 year male patient.
| Case Report|| |
A 22 year male patient complaining of on and off fever, generalized weakness and swelling in the right cervical region since one year. On examination the cervical lymph node was of size 6x5 cm,firm, non tender and mobile. There is no history of cough and weight loss. Fine Needle Aspiration Cytology or any other preoperative investigation was not done and in the present case we received only excised lymph node. Surgery was done and the lymph node was completely excised and sent for histopathology. On gross the lymph node was of size 6 x 5 cm,firm and cut section of the lymph node was well circumscribed and grey white [Figure 1]. Necrosis was not seen.On microscopy it was diagnosed as Castleman's diease - Hyaline vascular type.
|Figure 1: (a) Gross appearance of cervical lymphnode of size 6 × 5 cm, (b) cut section show well circumscribed and greyish white|
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| Discussion|| |
CD was first described by Castleman et al. in 1954 and again in 1956 in a report of cases of solitary masses in the mediastinum. , Later similar cases were reported in other parts of the body which included localized, multicentric, nodal and even extranodal masses.  Most commonly involved sites are mediastinum (60%), retroperitoneum (11%), and axilla (4%).  It occurs most commonly in adults mostly under 30 years of age but it can also affect children. ,
Microscopically, as stated above, two distinct histological patterns have been described - the hyaline vascular (HV) type or angiofollicular type and plasma cell (PC) type. However, depending on clinical presentation they can be divided into solitary or localized and multicentric or systemic type. More than 90% of the solitary type is HV type and almost all of the multicentric type is histologically PC type.
However, multicentric HV type has been reported. The hyaline vascular variant comprises 90% of CD and mostly presents as a solitary lymphadenopathy - most commonly in mediastinum (60%), but also abdomen, neck, lung, axilla, mesentry, broad ligament, retroperitoneum, soft tissue of the extremities, nasopharynx, leptomeninges, adrenal, paraadrenal and parotid. 
The size of the lesion varies widely ranging from 1-12 cms with a mean diameter of 6 cms.  In our study, the size was 6 cms [Figure 1].
Microscopically, the characteristic features are increased number of scattered large follicles, many of which with hyalinization and marked vascular proliferation of their germinal centers [Figure 2]. The interfollicular area shows marked proliferation of postcapillary venules and presence of plasma cells, eosinophils, immunoblasts, and monocytes.  Sinuses are usually not apparent.  There may be more than one germinal center within a single follicle [Figure 3]. Lymphocyte arrangement in the mantle zone gives a "targetoid" or "stadium seating" or "onion skin" appearance [Figure 4]. Often capillaries penetrating the follicle at right angles giving a "lollipop" appearance  [Figure 5].
|Figure 2: Section showing many follicles with regressively transformed germinal centres surrounded by concentrically arranged mantle zone lymphocytes. [H and E, ×10]|
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|Figure 3: Section showing atrophic germinal centres, with depleted lymphocytes within a large follicle [Figure a and b, X40 H and E].|
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|Figure 4: Section showing a large follicle with concentrically arranged mantle zone lymphocytes giving "onion skin" appearance along with hyalinization and marked vascular proliferation of germinal centre. [H and E, ×40]|
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|Figure 5: Section showing a follicle with concentrically arranged mantle zone lymphocytes along with penetrating hyalinized vessel entering at right angles to the follicle giving a "lollipop" appearance.[H and E, ×40]|
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The etiology is unknown. However, the two main hypotheses are viral infection and abnormal reaction.  Recent works indicate possibility of immunologic pathogenesis Human herpes virus 8 and interleukin 6 are regarded to be linked to the pathogenesis.  If Cervical lymph nodes are involved by Castleman's disease and it may be confused with other common causes of cervical lymphadenopathy like tuberculosis and nodal secondaries.
CD follows a benign course and, in most cases, surgical removal of the affected lymph node is curative as re-growth is not expected. 
This case report is presented for its rarity and CD should be kept as a differential of localized or multicentric lymphadenopathy especially in young patients.
| References|| |
|1.||Yildirim H, Cihangiroglu M, Ozdemir H, Kabaalioglu A, Yekeler H, Kalender O. Castleman′s disease with isolated extensive cervical involvement. Australas Radiol 2005;49:132-5. |
|2.||Rosai J. Lymph nodes. In: Rosai J, editor. Ackerman′s Surgical Pathology. 9 th ed. St Louis: Mosby; 2003. p. 1877-2017. |
|3.||Sarrot-Reynauld F. Castleman′s disease. In: Orphanet encyclopedia [Online].  Available from: http://www.orpha.net/data/patho/GB/uk-castleman.pdf. [Last cited on 2001 Aug]. [last accessed on 2008 Oct 05]. |
|4.||Frizzera G. Castleman′s disease and related disorders. Semin Diagn Pathol 1988;5:346-64. |
|5.||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. |
|6.||Roshong-Denk SL, Bohman SL, Booth RL. A 53-year-old white man with right-sided supraclavicular lymphadenopathy. Arch Pathol Lab Med 2005;129:945-6. |
|7.||Castleman B. Records of the Massachusetts General Hospital-weekly clinicopathological exercises (case 40011). N Eng J Med 1954;250:26-30. |
|8.||Castleman B, Iverson L, Mendez VP. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956;9:822-30. |
|9.||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. |
|10.||Koslin DB, Berland LL, Sekar BC. Cervical Castleman disease: CT study with angiographic correlation. Radiology 1986;160:213-4. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]