A 37-year-old African-American male with past medical history of HIV (detected in 2001; transmitted by heterosexual intercourse; latest CD4 count 320 cells/mm3 with undetectable viral load; compliant with highly active antiretroviral therapy (HAART); no opportunistic infections in past) and nephrolithiasis presented to emergency department of our hospital with progressively worsening epigastric pain and nausea for one week. Pain was constant and was rated severe and radiated to his back. The patient denied hematemesis, hematochezia, or diarrhea. However, he reported weight loss of 12 lbs in last 2 months but denied night sweats. There was no history of recent travel, sick contacts, or illicit drug use. Vital signs were notable for tachycardia of 120 beats/minute and low-grade fever of 38.8 C. The physical examination revealed epigastric tenderness with no rebound. Initial laboratory evaluation showed elevated levels of lipase 629 IU/L (33-200), amylase 250 IU/L (30-110), lactate dehydrogenase (LDH) 936 IU/L (313-618), and C-reactive protein 3.6 mg/dL (0-1), with normal liver and renal function tests. White cell count was 3.8 x 109cells/L (3.8-9.8 x 109), hemoglobin was 13.2 g/dL (13.5 to 17.5), and platelets were 139 x 109 cells/L (150-450 x 109). Cardiac troponins came out negative with unchanged EKG. Preliminary diagnosis of acute pancreatitis was made. Subsequently, abdominal computed tomography (CT) demonstrated multiple, well-defined, peripancreatic, soft tissue masses to left of the uncinate process (Figure 1), posterior to the pancreatic tail, and near the hepatic hilum (Figure 2). The peripancreatic masses were found to be compressing the body of pancreas, causing acute pancreatitis in our patient. Furthermore, there was partial compression of the portal vein with multiple surrounding portal venous system collaterals.
Differential diagnoses included lymphoma, mycobacterium avium complex (MAC), disseminated fungal infection, and tuberculosis (TB). The case was discussed in the multidisciplinary tumor board. It was concluded that, due to the advanced disease, tissue diagnosis will be difficult employing surgery or interventional radiology. Thereafter, an uneventful endoscopic ultrasound (EUS)-guided fine-needle aspiration biopsy of one of the masses was performed, and preliminary pathology suggested lymphoma. Cancer antigen 19-9 (CA 19-9), carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), and serotonin levels were within normal limits. Sputum AFB culture and QuantiFERON-TB Gold (QFT) were negative ruling out TB. Fungal cultures and isolated bacterial cultures for MAC also came out negative. Total body scan showed subcentimeter bilateral mediastinal and axillary lymphadenopathy. Histopathology analysis revealed diffused infiltration of large B-cells with plasmablastic (cells with rounded nuclei, coarser chromatin, and smaller two to three nucleoli) and immunoblastic morphological features (cells with vesicular enlarged nuclei and single prominent nucleolus). A high proliferative index with frequent apoptosis and focal necrosis was demonstrated. Immunohistochemistry of the peripancreatic specimen revealed neoplastic cells positive for BOB1, MUM1, and EBER-ISH, partially positive for CD79a, and negative for CD20, CD56, CD138, CD3, CD5, AE1/AE3, and HHV8, most consistent with plasmablastic lymphoma. A bone marrow biopsy was performed for staging purposes. It revealed normocellular marrow with maturing trilineage hematopoiesis with no evidence of excess blasts or lymphoma. Furthermore, lumber puncture cytology was negative for malignant cells and CSF flow cytometry did not show any abnormal cells; based on these findings, the patient was deemed as stage III.
Subsequently, epigastric pain improved with oral opioids and other pancreatitis-related symptoms resolved with the symptomatic treatment. The patient was discharged from the hospital with oncology out-patient follow-up. Therein, he was initiated on cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) therapy. Central nervous system prophylaxis was achieved by employing intrathecal chemotherapy. Filgrastim (G-CSF) was administered twenty-four hours after CHOP cycles and was continued daily for seven days. Our patient tolerated the treatment well, with no significant interactions with HAART for his HIV-infection, and achieved remission after six cycles of chemotherapy. Contrast-enhanced CT at the level of pancreas 3 months after initiation of CHOP treatment demonstrated near complete resolution of previously seen soft tissue masses (Figure 3). The patient has been disease-free for over a year now without any additional specific therapy.