Related Experiment Video
Updated: Jun 5, 2025
Endoscopic Ultrasound-Guided Biliary Drainage: Endoscopic Ultrasound-Guided Hepaticogastrostomy in Malignant Biliary Obstruction
Published on: March 25, 2022
Interdigitating dendritic cell sarcoma presenting as retroperitoneal mass and obstructive jaundice. An uncommon
Alejandro Nieto-Jara1, Marta García Calonge2, Fernando Fernández Cadenas3
1Gastroenterology, Hospital Universitario Central de Asturias, España.
Insights
Interdigitating dendritic cell sarcoma (IDCS) was diagnosed in a patient presenting with obstructive jaundice. Surgical resection and chemotherapy led to regression of retroperitoneal adenopathies and stable metastatic liver disease.
Area of Science:
- Oncology
- Pathology
- Gastroenterology
Background:
- A 60-year-old male presented with obstructive jaundice due to a retroperitoneal lesion.
- Initial diagnosis suspected IgG4-related disease based on fine needle biopsy (FNB).
Purpose of the Study:
- To diagnose and manage a rare retroperitoneal malignancy.
- To differentiate between IgG4-related disease and other potential diagnoses.
Main Methods:
- Ultrasound endoscopy (UES) with FNB and endoscopic retrograde cholangiopancreatography (ERCP) with stenting.
- Computed tomography (CT), upper endoscopy, colonoscopy, cephalic duodenopancreatectomy, and immunohistochemistry (IHQ).
Main Results:
- Histopathology and IHQ confirmed interdigitating dendritic cell sarcoma (IDCS).
- Patient underwent extensive surgery and initiated R-CHOP chemotherapy.
- Post-chemotherapy, retroperitoneal adenopathies regressed, and liver metastases remained stable.
Conclusions:
- Interdigitating dendritic cell sarcoma (IDCS) can present as a retroperitoneal mass causing obstructive jaundice.
- Multidisciplinary approach including advanced imaging, biopsy, surgery, and chemotherapy is crucial for managing IDCS.
- R-CHOP chemotherapy showed efficacy in controlling advanced IDCS with metastatic disease.
Abstract:
A 60-year-old male was admitted for obstructive jaundice secondary to a 2 cm retroperitoneal lesion. Ultrasound endoscopy (UES) with fine needle biopsy (FNB) was performed, as well as endoscopic retrograde cholangiopancreatography (ERCP) with placement of a metal stent for bile duct drainage. Initially IgG4-related disease was suspected from FNB. After 8 months of treatment the patient attended with a progression evidenced in computed tomography (CT) with retroperitoneal adenopathies and liver metastasis, with involvement of duodenum and fistulation towards hepatic angle of colon. Upper endoscopy and colonoscopy was performed. The patient underwent a cephalic duodenopancreatectomy with antrectomy and right hemicolectomy. The histological study of the specimen revealed cells of fascicular distribution, fusiform aspect, with ovoid nuclei, accompanied by a polymorphic inflammatory infiltrate with mononuclear cells. Immunohistochemistry (IHQ) was positive for S100, CD21, CD23, D2-40 and Bcl-2 and negative for CD1a, CD2, CD3, CD4, CD5, CD8, CD20, CD79a, PD1, desmine, langherin, c-kit, PDGFR. These findings were suggestive of interdigitating dendritic cell sarcoma (IDCS). Systemic chemotherapy with R-CHOP was initiated and after the first 4 cycles of treatment, retroperitoneal adenopathies regressed, with stability of metastatic liver disease.
