|Year : 2001 | Volume
| Issue : 2 | Page : 163-167
Castleman's disease - a urologic enigma
Sanjeev Mehrotra, Reji K Varghese, Nitin S Kekre, Sheila Nair, Ganesh Gopalakrishnan
Departments of Urology and Pathology, Christian Medical College & Hospital, Vellore, India
Nitin S Kekre
Department of Urology, Christian Medical College and Hospital, Vellore (Tamilnadu) - 632 004
Source of Support: None, Conflict of Interest: None
Keywords: Castleman′s Disease; Adrenal Tumour.
|How to cite this article:|
Mehrotra S, Varghese RK, Kekre NS, Nair S, Gopalakrishnan G. Castleman's disease - a urologic enigma. Indian J Urol 2001;17:163-7
| Case Report|| |
A 40-year-old male presented with episodic left loin pain of 1-year's duration. He had fever and malaise at the onset, both of which settled later. Physical examination was non-contributory. Haematological and biochemical investigations were normal. He underwent Ultrasound and subsequently CT scan of the abdomen which showed what was thought to be a left adrenal mass [Figure - 1]. CT-guided FNAC was non-diagnostic. At exploration there was a large vascular mass in the suprarenal region pushing the adrenal medially. Dissection of the mass was extremely difficult. The left renal vessels which were in close proximity to the mass were injured during the dissection and they had to be repaired. Histopatological examination of the mass was reported as Castleman's disease.
| Comment|| |
Castleman first reported this disease in 1954 while studying lymphoid tumours of the mediastinum.  It is a rare benign disorder characterised by hypervascular lymphoid hyperplasia. It usually manifests as an asymptomatic solitary mediastinal tumour and seldom as an isolated retroperitoneal mass.
Two histological subtypes have been described.  The hyaline vascular type accounts for around 90% of all the cases. It is usually asymptomatic. On gross examination it appears as a well-circumscribed very vascular mass. Histologically numerous follicles showing central blood vessel with swollen endothelial cells are seen. The richly vascular interfollicular space contains hyaline material and admixture of cells predominantly small lymphocytes. The plasmacystic variety which accounts for 10% differ from the hyaline vascular type in that (i) patients usually have fever, raised ESR and hypergammaglobulinaemia; (ii) majority of cells in the interfollicular area are plasma cells rather than small lymphocytes; (iii) hyalinization and vascularisation are less prominent.
The cause of this disease is unknown. Reactive proliferation in response to some unidentified agents is the most popular explanation. Some believe it is hamartomatous in origin.  Radiological differentiation from lymphoma is difficult despite characterisitc CT and angio features.  A frozen section examination will help in confirming this as a lymphoid lesion though differentiation from lymphoma is again difficult.
Castleman's disease mimicking adrenal tumour has not been reported in the past. It is a benign disease. If as a Urologist, one were to encounter a similar lesion, we strongly recommend a frozen section examination to avoid an unnecessary extensive operation and associated haemorrhage.
| References|| |
|1.||Castleman B, Iverson C, Pardo-Menedez V. Localised mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956; 9: 822-830. |
|2.||Keller AR, Hochholzer C, Castleman B. Hyaline vascular and plasma cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972; 29: 670-683. |
|3.||Paradinas F. Primary and secondary immune disorders. In: Stansfelt AG (ed.). Lymphnode biopsy interpretation, Churchill Livingstone. Edinburgh, London. 1985; 184-189. |
|4.||Dahnert W. Radiology Review Manual, 2nd edn. Williams & Wilkins, Baltimore. 1993: 354. |
[Figure - 1]