Zehra Dhanani, MD, Nima Madanchi, MD, Sunita Dia, MD, Ronald Distajo, MD
Georgetown University Hospital and Washington Hospital Center, Washington, DC
Introduction: Retroperitoneal fibrosis can be idiopathic or secondary to drugs, infections, radiotherapy, surgery, trauma, amyloidosis, or malignancies. There are very few reported cases of retroperitoneal fibrosis related to esophageal cancer and none of them have reported retroperitoneal fibrosis as the presenting feature of esophageal adenocarcinoma.
Case Description/Methods: A 68 year old female with a past medical history of psoriasis and type-one Chiari malformation presented with one month of progressive abdominal and lower back pain. CT abdomen pelvis revealed inflammatory changes around the aorta, celiac artery, SMA and renal artery with severe narrowing of both renal veins and near obliteration of the IVC. Infectious work-up including TB, Syphilis, Hepatitis B and C, and rheumatologic work-up including ANA, ENA, RF, CCP and IgG4 were negative. Patient continued to have worsening symptoms and worsening kidney failure despite bilateral nephrostomy, high-dose steroid and empiric antibiotics.
Eventually, patient underwent upper endoscopy for episodes of bilious vomiting which revealed esophagitis and nodularity in lower third esophagus. Esophageal biopsy showed poorly differentiated adenocarcinoma. Retroperitoneal biopsy also demonstrated the same morphological features consistent with an esophageal source.
Discussion: To the best of our knowledge, this is the first report of an esophageal adenocarcinoma presenting with retroperitoneal fibrosis with complete absence of dysphasia, odynophagia and weight loss. Patient did not experience any GI symptoms until late in the disease course.
Malignancy-related retroperitoneal fibrosis can be secondary to either metastasis/invasion from a solid tumor or due to release of fibroblast-stimulating cytokines. Cytokine release is most commonly seen in carcinoid tumors. It is associated with poor prognosis. The nonspecific presenting symptoms, as witnessed in our patient, make it difficult to reach a diagnosis. By the time the patient has more clear symptoms of malignancy, it is often too late for curative treatment.
There is currently a lack of effective therapies to treat retroperitoneal fibrosis. Corticosteroids are commonly employed in idiopathic cases however their use in malignancy-related cases has not been effective. Palliative chemotherapy or surgery might be effective for symptomatic management.
In conclusion, we recommend considering esophageal cancer as a differential diagnosis in unexplained retroperitoneal fibrosis.
Citation: Zehra Dhanani, MD, Nima Madanchi, MD, Sunita Dia, MD, Ronald Distajo, MD. P2152 - RETROPERITONEAL FIBROSIS: A PRESENTING SYMPTOM OF ESOPHAGEAL ADENOCARCINOMA. Program No. P2152. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.