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| Year : 2009 | Volume
: 52
| Issue : 4 | Page : 582-583 |
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| Intra-abdominal mass: A diagnostic dilemma |
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Asim Qureshi1, Rafay Azhar2, Qasim Ahmed2
1 Aga Khan University Hospital, Karachi, Pakistan
2 Shaukat Khanum Cancer Hospital, Lahore, Pakistan
Click here for correspondence address and email
| Date of Web Publication | 1-Oct-2009 |
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How to cite this article:
Qureshi A, Azhar R, Ahmed Q. Intra-abdominal mass: A diagnostic dilemma. Indian J Pathol Microbiol 2009;52:582-3 |
Ormond's disease (sclerosing fibrosis, inflammatory pseudotumor) is a rare disease of unknown etiology that results in progressive fibrosis with involvement of retroperitoneal structures like pancreas, kidneys and ureters etc. producing symptoms of urinary obstruction and chronic pancreatitis. Biopsy or fine needle aspiration cytology may not be helpful to establish or reach a diagnosis.
A 25-year-old man presented to a tertiary care cancer hospital with history of dull, dragging pain in the abdomen for six months. There were episodes of severe left-sided colicky pain associated with urinary complaints. Physical examination was unremarkable except for tenderness in the para-umbilical and suprapubic region. Ultrasound and computed tomography scan of the abdomen revealed a 7x7 cm mass in the tail and body of pancreas, encircling the left ureter and left renal hilum. Fine needle aspiration of the mass revealed a few neuro-endocrine cells so a possibility of neuro-endocrine tumor of pancreas was taken into account. Considering the young age and performance status of the patient the surgeon decided to resect the tumor. Per-operatively a 7x7x6 cm mass was present in the body and tail of pancreas, encircling the left ureter and left renal hilum. It was grossly stuck to the gastric bed and transverse colon. En bloc resection of the pancreatic tail along with portion of stomach, colon and left kidney was done and sent to the laboratory for histopathology.
Gross examination revealed similar findings as seen per-operatively. Multiple sections were taken from the lesion in the pancreas, adherent stomach, colon and kidney [Figure 1]. Histological examination revealed marked fibrosis with thick bands of collagen containing scattered spindle cells. There were foci of lympho-plasmacytic aggregates. A few germinal centers were also noted [Figure 2]. The fine needle aspiration slides were also reviewed; the "cells" seen were aspirated from endocrine pancreas. The case was discussed at length with the radiologists and surgeons involved and a diagnosis of retroperitoneal fibrosis was made.
Idiopathic retroperitoneal fibrosis is a rare fibrosing reactive process that may be confused with mesenteric fibromatosis. It was well-recognized after publication of two cases by Ormond. [1] It is characterized by diffuse or localized fibroblastic proliferation and a chronic lympho-plasmacytic infiltrate in the retroperitoneum causing compression or obstruction of the ureters, aorta or other vascular structures.
The disease is more common in men and most patients present in the fourth to fifth decade of life. [2] Most patients present with vague abdominal symptoms including dull, poorly localized back or flank pain. Other common symptoms include weight loss, nausea or vomiting. Some patients may present with ureteral or colonic symptoms. [3]
Although most cases are idiopathic some have been associated with administration of methysergide (1%), an ergot derivative used to treat migraine headaches. [4] Symptoms are relieved upon withdrawal of drug. Other cases are secondary to exuberant desmoplasia in response to malignancy at this location. The tumors that are most frequently implicated are Hodgkin's disease and other types of lymphoma, retroperitoneal sarcomas, carcinoid tumors and a variety of carcinomas. [5]
Grossly, the mass is dense grayish white and plaque-like, usually arising at or just below the level of aortic bifurcation. It surrounds the vessels and typically encases the ureters resulting in hydronephrosis.
Microscopic examination reveals a fibrous proliferation, broad anastomosing bands of hyalinized collagen and a lympho-plasmacytic infiltrate with occasional germinal centers. Several studies have found a progression from active inflammation to fibrosis through serial biopsies. [5]
 Acknowledgment | |  |
Ms. Shamsha Noorali Punjwani
| References | | |
| 1. | Arrive L, Hricak H, Tavares NJ, Miller TR. Malignant versus non malignant retroperitoneal fibrosis: Differentiation with MR imaging. Radiology 1989;172:139-43.  |
| 2. | Baker LR, Mallinson WJ, Gregory MC, Menzies EA, Cattell WR, Whitfield HN, et al. Idiopathic retroperitoneal fibrosis: A retrospective analysis of 60 cases. Br J Urol 1987;60:497-503. |
| 3. | Hughes D, Buckley PJ. Idiopathic rekoperitoneal fibrosis is a microphage rich process: Implications for its pathogenesis and treatment. Am J Surg Pathol 1993;17:482-3. |
| 4. | Lepor H, Walsh PC. Idiopathic retroperitoneal fibrosis. J Urology 1979;122:1-6. |
| 5. | Walsh SV, Evangilista F, Khettry U. Inflammatory myofibroblastic tumor of pancreatobiliary region; Morphologic and immunohistochemical study of three cases. Am J of Surgical Pathology 1998;22:412-8. |
Correspondence Address:
Asim Qureshi
Department of Pathology & Microbiology, Aga Khan University, Hospital Stadium Road P.O. Box 3500, Karachi -748 00
Pakistan
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.56126
[Figure 1], [Figure 2] |
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