Bombay Hospital Journal] Case Reports[Contents][Home][Archives][Search][Books][Feedback]

 CASTLEMANNíS DISEASE - A Surgical Enigma

GV Bhagwat, N Chakrabarti, RV Patankar,K Patel, VV Dewoolkar
Department of Surgery, KJ Somaiya Medical College, Mumbai
INTRODUCTION

First described in 1954, Castlemannís disease usually occurs in areas where lymphnodes are commonly present and in the mediastinum. It is essentially a hamartoma. An atypical case of Castlemannís disease is presented and the medical literature reviewed.

CASE REPORTS

A girl aged 16 yrs presented with history of slowly growing 12.5 cm x 10 cm swelling in the left infraclavicular region since 6 months. There was no history of tuberculosis. Swelling was nodular, mobile and not adherent to the underlying pectorals. Routine investigations including ESR, chest radiograph and HIV were normal. Excision biopsy of the swelling was carried out. Histopathology revealed small hyaline follicles with abnormal germinal centres with a background of vascularised and fibrous lymphoid tissue, suggestive of hyaline vascular type of Castlemannís disease.

Gross appearance of specimen.
Fig 1
Gross appearance of specimen.


DISCUSSION

Commonest site of the condition is in the mediastinum, followed by retroperitoneal region, cervical, axillary and pelvic sites. [1] They are often confused with neurogenic tumours and pericardial cysts when present in posterior mediastinum. [2]

There are 3 histological entities:

1.Hyaline vascular type : 80-90% characterised by abnormal germinal centres in a background of vascularised and fibrous lymphoid tissue with small hyaline follicles. [3]

Histopathology of hyaline vascular variety - low power.
High power view.
Fig 2
Fig 3
Histopathology of hyaline vascular
variety - low power.
High power view.


2.Plasma cell type : 10-15% characterised by large hyaline follicles with intervening sheets of plasma cells. [3]

3.Mixed type : Castlemannís disease is commonly solitary and asymptomatic but fever and weight loss may sometimes be present. Association with immunodeficiency states such as AIDS, lymphoma and Kaposiís sarcoma is often seen with multicentric type and it has poor prognosis. [4]

REFERENCES

  1. Castlemann B, Thowne VW. Case reports of Massachusetts Gen. Hospital. New England Journal of Medicine 1954; 250 : 26-31.
  2. Lather R, Patche MR. Localised Mediastinal lymphnode hyperplasia. Cancer 1956; 9 : 822-30.
  3. Kelier AR, Hochholzer I, Cast B. Hyaline vascular and plasma cell types of Castlemannís disease. Cancer 1972; 29 : 670-83.
  4. Henry K, W St. C. Symmers - Pathology of lymphatics and lymphnodes. 1997; 7 : 208-18.

To Section TOC

Sponsor-Dr. Reddy's Lab