Published online Sep 14, 2018. doi: 10.3748/wjg.v24.i34.3958
Peer-review started: April 27, 2018
First decision: July 4, 2018
Revised: July 10, 2018
Accepted: July 22, 2018
Article in press: July 22, 2018
Published online: September 14, 2018
Case 1 is a 48 year old female patient who presented with lymph node enlargement; and Case 2 is a 57 year old female patient who presented with tiredness and fever.
In Case 1, the patient initially had no apparent discomfort or noteworthy positive physical signs. In Case 2, the patient felt tired and had a fever with no other discomfort.
Neuroendocrine tumor, paraganglioma and lymphoma.
In Case 1, laboratory examinations revealed that the HIV antibody was negative. Carbohydrate antigen 19-9, carcinoembryonic antigen and other laboratory test results were also within the normal range. In Case 2, laboratory examinations showed that the HIV antibody was negative and the white blood cell count was 14.6 × 109/L. Carbohydrate antigen 19-9, carcinoembryonic antigen and other laboratory test results were also within the normal range.
In Case 1, MRI of the epigastrium showed that there was an elliptical hyperintense signal below the pancreas on the T1WI, and slight hyperintensity on the T2WI. In addition, marked enhancement on the arterial phase and reduced enhancement on the delayed phase were observed. In Case 2, MRI of the epigastrium showed that there was an elliptical hyperintense signal below the pancreas on the T1WI and slight hyperintensity on the T2WI. Marked enhancement on the arterial phase was inhomogeneous. In addition, the venous phase and delayed phase continued to intensify.
In Case 1, the retroperitoneal single lymph node was compatible with Castleman disease. In addition, immunohistochemical staining revealed that cells were slightly positive for CD20, CD3, CD21, bcl-6, and bcl-2, a small number of cells were positive for CD138, and 20% of cells were positive for Ki-67. In Case 2, the diagnosis was retroperitoneal Castleman disease together with a follicular dendritic cell tumor. Immunohistochemical staining showed that a portion of cells were positive for CD21 and cells were negative for CK, a small number of cells were positive for S-100 and slightly positive for CD 1a and CD23, and about five percent of cells were positive for Ki-67.
In Case 1, laparotomy was performed to remove the retroperitoneal peripancreatic tumor without subsequent radiochemotherapy. In Case 2, laparoscopic surgery was performed to remove the retroperitoneal peripancreatic tumor without subsequent radiochemotherapy.
Unicentric Castleman disease (UCD) is a rare disease of lymph nodes and related tissues, and occurrence in the retroperitoneal peripancreatic region is quite rare. Only a few cases of UCD together with follicular dendritic cell tumor have been identified. At present, complete resection of the tumor is still the most effective treatment for retroperitoneal peripancreatic UCD with low recurrence and high cure rates.
Follicular dendritic cell tumor is an uncommon neoplasm that has features of follicular dendritic cell differentiation, and typically presents as a slow-growing, painless mass without systemic symptoms. The tumor can occur on various parts of the body such as lymph nodes, tonsils, armpits and mediastinum, but is most common in the neck lymph nodes.
At present, complete resection of the tumor is still the most effective treatment for retroperitoneal peripancreatic UCD with low recurrence and high cure rates. The diagnosis of retroperitoneal peripancreatic UCD is still dependent on histopathology. The two rare cases of retroperitoneal peripancreatic UCD included in this report will improve our understanding of this disease and provide a significant reference for future cases.