Pembrolizumab in MSI-high Pancreatic Sarcomatoid Carcinoma

We report the outcome of a patient with MSI-high metastatic pancreatic sarcomatoid carcinoma refractory to multiple lines of chemotherapy treated successfully with pembrolizumab. In November 2015, our patient presented with epigastric pain leading to radiologic workup. A lesion in the pancreas as well as liver metastasis were diagnosed; liver biopsy revealed a poorly differentiated sarcomatoid carcinoma. After having received every approved chemotherapy for pancreatic cancer his disease eventually progressed so that we reached out for novel therapeutic strategies. Because of the tumor being MSI high, treatment with pembrolizumab was commenced in May 2017. Clinical response with better overall quality of life was soon reported and repeated CT scans showed an ongoing partial response leading to a near complete remission in the latest scan obtained. Adverse events during the course of therapy included immune mediated arthralgia grade 1, colitis grade 2 and pneumonitis grade 1 which were managed by administration of glucocorticoids without interruption of immunotherapy. To our knowledge, this is the first case of a patient with MSI high metastatic sarcomatoid carcinoma of the pancreas successfully treated with immunotherapy for more than three years.

The emergence of bladder dysfunction secondary to lupus cystitis in a patient with established lupus nephritis: the first case from Pakistan

Lupus cystitis is an uncommon manifestation of systemic lupus erythematosus (SLE). We describe the case of a 23-year-old woman with lupus that complained of abdominal pain, vomiting, and diarrhea for one week. Two years back, she was brought with an affirmative history that satisfied the clinical criteria of SLE which was renal biopsy-proven (class V membranous lupus nephritis). On radiologic workup, she was found to have bilateral hydronephrosis and a thick-walled bladder with diverticula. Immunosuppressive therapy was commenced however augmentation cystoplasty had to be opted for as the symptoms did not subside with medical therapy alone.

Endoscopic ultrasound (EUS) in pancreatic masses with inconclusive radiologic workup – a case series and short review of the literature

Endoscopic ultrasound (EUS) is a very sensitive examination to detect pancreatic masses and can provide useful information in cases where conventional radiologic workup remains inconclusive. We present three cases in which EUS was decisive in establishing the correct diagnosis. Case 1: A 74-year-old female was hospitalized because of acute pancreatitis. Medical history, CT and MRI gave no clue to etiology, but EUS diagnosed a small pancreatic tumor. Case 2: A female patient was admitted because of abdominal pain and weight loss. While MRI suspected a pancreatic tumor, EUS showed typical features of autoimmune pancreatitis. Case 3: A 50-year-old patient was hospitalized with cachexia, ascites and pulmonary embolism. At first, a pancreatic tumor was suspected, but EUS showed a cystic lesion with a solid component (pancreatic pseudocyst).

A naturally occurring TIPS?

Portal-systemic encephalopathy in both cirrhosis and non-cirrhotic patients can occur when a large portal-systemic shunt forms within the venous system. Though most commonly found in patients with portal hypertension, after trauma, surgical intervention, and liver biopsy, one can also be idiopathic. A 78-year-old Latino male developed worsening confusion and lethargy during a hospitalization for bridging of his anticoagulation. An extensive laboratory and radiologic workup did not initially reveal the etiology of his delirium and decreased state of consciousness. On exam, he had tremulousness and fetor hepaticus without corresponding liver function abnormalities in his laboratory testing. An ultrasound of the liver demonstrated a large veno-venous shunt between the right portal vein and the right hepatic vein. After multiple episodes of encephalopathy and progressive heart failure from significant right ventricular overload, the shunt was closed by interventional radiology. This treatable cause of encephalopathy may not be evident without radiologic studies.

Monophasic Synovial Sarcoma of the Liver

We report a hepatic monophasic synovial sarcoma in a 60-year-old woman who presented with right upper quadrant pain subsequent to an intrahepatic bleed from a highly vascular tumor mass. Imaging studies showed a dominant tumor mass in the right hepatic lobe with multiple satellite nodules. A detailed physical examination and radiologic workup failed to reveal a primary tumor elsewhere. A right partial hepatectomy was performed with a preoperative differential diagnosis of angiosarcoma versus hepatocellular carcinoma. The morphologic, immunophenotypic, and cytogenetic findings (t(X;18)(p11.2;icq11.2)) were consistent with a monophasic synovial sarcoma. Postoperative clinical evaluation of the extremities and a positron emission tomographic scan performed 4 weeks after surgery showed no evidence of recurrent or metastatic disease. The patient was started on an aggressive 4-drug chemotherapy regimen, but died 3 months thereafter from widespread metastatic disease. No autopsy was performed. The presence of multiple lesions in the liver certainly suggests the possibility of metastatic disease. It would, however, be very unusual for a synovial sarcoma to present as an occult primary, and the negative radiologic workup 1 month after the partial hepatectomy also argues against this possibility. The clinical presentation, radiographic findings, and subsequent course in this patient was therefore most consistent with a primary monophasic synovial sarcoma of the liver.