|IMAGES IN RHEUMATOLOGY
|Year : 2018 | Volume
| Issue : 1 | Page : 62-63
Signet ring cell gastric adenocarcinoma “Linitis Plastica” Masquerading as retroperitoneal fibrosis: A report of two co-existing uncommon entities
Kapil Chaudhary1, Partho Mukherjee1, Emma Monga2, Antony Devasia1
1 Department of Urology, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Web Publication||26-Feb-2018|
Dr. Kapil Chaudhary
Department of Urology, Christian Medical College, Vellore, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Keywords: Gastric adenocarcinoma, MRI, retroperitoneal fibrosis
|How to cite this article:|
Chaudhary K, Mukherjee P, Monga E, Devasia A. Signet ring cell gastric adenocarcinoma “Linitis Plastica” Masquerading as retroperitoneal fibrosis: A report of two co-existing uncommon entities. Indian J Rheumatol 2018;13:62-3
|How to cite this URL:|
Chaudhary K, Mukherjee P, Monga E, Devasia A. Signet ring cell gastric adenocarcinoma “Linitis Plastica” Masquerading as retroperitoneal fibrosis: A report of two co-existing uncommon entities. Indian J Rheumatol [serial online] 2018 [cited 2020 Jul 2];13:62-3. Available from: http://www.indianjrheumatol.com/text.asp?2018/13/1/62/222115
Retroperitoneal fibrosis (RPF) is an uncommon condition with an estimated incidence of 1.38 cases/100,000 people. An underlying malignancy needs to be considered in patients with suspected RPF and is present in 8%–10% of such cases. Malignancy usually results in RPF through an exuberant desmoplastic response to retroperitoneal metastases (e.g., carcinoma of the prostate, breast, and colon) or to a primary retroperitoneal tumor (e.g., Hodgkin's and non-Hodgkin lymphoma, inflammatory myofibroblastic tumor and various types of sarcomas). It can involve any of the retroperitoneal structures. We present a case of signet ring gastric adenocarcinoma masquerading as retroperitoneal fibrosis.
A 50-year-old diabetic female patient presented with a history of right flank and lower abdominal pain for the past 2 months. There was an episode of urinary tract infection (UTI) but no constitutional symptoms or hematuria. On evaluation, elsewhere, she had been detected to have bilateral hydroureteronephrosis and had undergone bilateral double J stenting. Her body mass index was 20.4 kg/m2 and physical examination was unremarkable. Laboratory investigations revealed serum C-reactive protein of 27.1 mg/dl with hemoglobin of 13 g/dl and a serum creatinine of 0.66 mg%. Serum IgG4 levels were <577 mg/L.
Magnetic resonance imaging (MRI) of the abdomen revealed irregular T2 hypo-intense soft tissue thickenings on both sides of the pelvis involving the bilateral ureters and another similar irregular T2 hypointense soft tissue thickening involving the gastrocolic ligament between the proximal body of the stomach and the distal transverse colon, with associated regional stomach wall thickening [Figure 1]. In view of the suspicious stomach wall thickening, she underwent an upper gastrointestinal endoscopy that revealed an ulcer in the body of the stomach with changes suggestive of linitis plastica [Figure 2]. Biopsy from the stomach ulcer revealed poorly differentiated adenocarcinoma with signet ring cells.
|Figure 1: Coronal magnetic resonance imaging revealing T2 hypointense soft tissue thickening of the body of stomach|
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As RPF could be secondary to underlying causes in 30% of cases, it is prudent to rule these out. Although there are no specific lab tests to rule out malignancy, age-appropriate cancers should be considered and abnegated. In this case, there was no history suggestive of a gastric malignancy. On the contrary, the presence of bilateral hydroureteronephrosis with febrile UTI was suggestive of an idiopathic RPF. IgG4 related disease was considered as a differential before malignancy, and the IgG4 level was done which was within normal limits.
Currently, abdominal computed tomography (CT) and MRI are considered as the imaging studies of choice to diagnose RPF. In this case, it was on imaging that the stomach wall thickening was detected leading to the identification of gastric cancer on gastroscopy and biopsy. Morphological features of RPF on CT or MRI may help to differentiate idiopathic from secondary forms, although there are no absolute differentiating features between the two. The following findings are suggestive:
- Based on proliferation pattern: idiopathic forms are mostly characterized by plaque-like densities with peripheral infiltration, whereas proliferation of secondary forms is usually lobulated and with nodularity
- Based on vessel displacement: malignant RPF tends to be bulkier and larger, displacing the inferior vena cava and aorta anteriorly from the spine and the ureters laterally. Vascular displacement is probably secondary to enlarged retrocaval and para-aortic nodes. On the contrary, idiopathic RPF leads to “tethering” of these structures to the vertebrae
- Based on soft tissue extension: malignant conditions like lymphoma tend to have a more caudal extension, whereas the idiopathic RPF tends to be predominantly below the renal hilum
- Based on diffusion-weighted imaging: active RPF and malignant lesions have similarity in the enhancement pattern, while chronic RPF shows weak enhancement.
Although there are a number of medical modalities described for the treatment of idiopathic RPF, treatment of the secondary forms of RPF requires an approach based on the cause. Unfortunately, RPF caused by untreatable cancers, trauma, major surgery, and radiotherapy, has no clear indications for systemic medical treatment. They may benefit only from palliative approaches, thus making a correct diagnosis essential.
The exclusion of secondary forms of RPF is mandatory as treatment with immunosuppressive agents (as used for idiopathic RPF) may worsen the prognosis of the disease. This case highlights that secondary RPF can present due to gastric linitis plastica, and detection may only be possible with appropriate imaging.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]