|
Case Presentation
1) Castlemen's disease
2) B/L retroperitoneal lymphangiectasia
|
|
| |
By,
Dr.R.S.Shah (Professor and Head of the unit-c, Department of surgery, Smt. SCL. Hsp, Saraspur)
Dr.M.B.Patel (Associate Professor, Unit-c, Department of surgery, Smt. SCL Hsp, Saraspur)
Dr.H.N.Vyas (Assistant Professor, Unit-c, Department of surgery, Smt. SCL Hsp, Saraspur)
Dr Kirti Prajapati, Dr.Aman Bhatia, Dr Keyur Surti (Resident, Unit-c, Department of Surgery, Smt SCL Hsp, Saraspur) |
| |
|
CASTLEMEN’S DISEASE |
| |
| |
| A) CASE HISTORY: |
| |
| A 42 years old Vijayaben Chandubhai Patel presented to the hospital with vomiting, abdominal distension and constipation from last 6days. Vomiting was persistent and bilious in nature. Patient was known case of diabetes mellitus and on regular OHA. Patient was operated for cholecystectomy l-1/2 years ago. On examination, patient had 100F fever, tachycardia of 110/min. Locally abdomen was distended with absent bowel sounds and stool loaded rectum. Rest of the history was not informative. |
| |
| B) INVESTIGATION: |
| |
Hb: 9.0gm%
RBS: 98gm%
Blood Urea:30mg%
S.cr: 0.8
S.Na: 137
S.K: 3.9
Blood group:B+ve
S. acetone: absent |
| |
| ON RADIOLOGY |
X-ray Abdomen erect: multiple air fluid levels spread all over the abdomen
Usg: 5*5*7 cm mixed echogenic lesion on right side of abdomen surrounded by dilated edematous bowel loops. ? Retroperitoneal mass. |
| |
| C) TREATMENT: |
| |
| Patient was prepared for emergency laparotomy and operated via midline abdominal incision. |
| |
| Peroperative findings: |
- About 50 ml of exudative fluid
- Dilated edematous small bowel.
- 3*3*3cm sized lymph node mass in the mesentry of jejunum 2 feet distal
to the DJ flexure
- Rest of the mesentry and retroperitoneum was normal.
|
| Post op period was satisfactory except fluctuation of RBS. Drain was removed on 5th day. Patient was discharged after 14 days of stay. |
| |
| D) DISCUSSION: |
| |
| It is also known as giant LN hyperplasia which was initially described by Castlemen. Most common age of presentation is middle age. Although mediastinum is most common site for the disease in initial reports, it can occur wherever the lymph nodes are present— retroperitoneum, cervical, axillary, mesenteric etc. |
| |
| Histologically, there are 2 distinct etiologies. |
| |
| 1. Hyaline vascular, characterized by small hyaline follicles and intrafollicular capillary proliferation. (90%) |
| |
2. Plasma cell type: characterized by large follicles with intervening sheets of plasma cell.
|
| |
| Tumours most significantly appear as single demarcated lesion. They are considered as benign lesion. Most of the patients are discovered on routine chest radiograph. Patient may present with cough, chest pain or as a mass lesion. |
| |
| Occasionally, it may present as multi-centric disease characterized by generalized lymphadenopathy associated with hepatospleenomegaly. |
| |
| It is investigated mainly by radiological imaging, CT scan of the disease part being the best. Treatment is the removal of lymph node mass in question with good prognosis. |
| |
| E) CONCLUSION: |
| |
| Though it is one of the rare diseases it is important differential diagnosis of mediastinal mass and any lymph node mass. Essentially it is a benign lesion and can be treated with its removal in toto. |
| |
| F) REFERENCES: |
| |
| * Castlemen B, IVersion L, Menendeg VP; localized mediastinal lymphoid hyperplasia resembling lymphoma. Cancer, 9; 822, 1956. |
| |
| * Day JRS; Bew D, Ali M, et al; Castlemen's disease association with myasthenia gravis. Ann Thoracic Surgery, 75:1648-50, 2003. |
| |
|
| |
|
|
