Small intestine follicular dendritic cell sarcoma with liver metastasis

Abstract

Rationale:Follicular dendritic cell sarcoma (FDCS) is a rare neoplasia composed of spindle or oval cells with follicular dendritic cell differentiation, usually occurring in lymphoid tissue. In this report, we present a case of FDCS of the small intestine with liver metastasis.Patient concerns:A 19-year-old female presented with recent onset of left upper abdominal pain. Abdominal computed tomography scan showed a large tumor mass in the liver lateral segment with compression to the pancreas upper part, and a smaller mass in the terminal ileum, respectively. High serum levels of amylase and lipase were noted. Resection of the tumors was performed. Microscopically, both tumors consisted of ovoid to spindle-shaped nuclei cells admixed with some lymphocytes arranged in fascicles, whorls, storiform arrays. Immunohistochemistry demonstrated that the tumor cells were positive for follicular dendritic cell markers, including CD21, CD23, and CD35. Epstein–Barr virus encoding small RNA (EBER; Inform EBER probe; Ventana Medical Systems, Tucson, AZ) in situ hybridization was negative.Diagnoses:According to the clinicopathological features, diagnosis of FDCS of intestinal origin was made.Interventions:Resection of tumors located in the liver and at the small intestine was performed. After the operation, patient received adjuvant vinblastin chemotherapy.Outcomes:There was no evidence of recurrence at 8-month follow-up.Lessons:It was unusual for FDCS of intestinal origin with liver metastasis and expressing with high serum levels of pancreatic enzymes.

Follicular Dendritic Cell Sarcoma - A Case Report

“…Some other sites affected by FDCS include retroperitoneal, mediastinal, and mesenteric area lymph nodes. [8] In our case, heterogeneously enhancing hypermetabolic well defined soft tissue mass in mesentery in the left lumbar region was observed. In context to the diagnosis of FDCS, imaging modalities can be of great help.…”

“…Ultrasound and CT are the preferred imaging modalities for the initial diagnosis of FDCS. [8] In our case, PET scans helped in the initial assessment of the patient as well as follow-up evaluation to assess disease progression or response to therapy. As per current literature, surgical resection is a preferred choice for FDCS while there is no standard chemotherapy or adjuvant therapy.…”

Clinicopathological characteristics of extranodal follicular dendritic cell sarcoma: A report of two cases

“…Carcinoma and FDCS cells are all ovoid cells with avesicular nuclei; however, carcinomas are positive for CK and negative for CD21, CD23 and CD35 ( 21 , 53 ). Similarly to FDCS, GISTs exhibit fascicles, storiform arrays and whorls patterns, and are negative for CK, but immune histochemical markers are positive for Dog-1, CD34 and CD117, and negative for specific markers for FDCS, which may be used to distinguish GIST from FDCS ( 5 , 19 ).…”

“…The use of the genomic sequencing approach enhanced the understanding of genomic features of FDCS in the thyroid ( 38 ). Extensive mutations were detected, including VEGFR1, CLTCL1 and TP53 mutations and hepatoma-derived growth factor related protein 3 (HDGFRP3) and Src homology 2 domain containing family member 4 (SHC4) ( 10 , 19 ). SHC4 is associated with the EGFR signaling pathway, from which it was deduced that this pathway may serve a role in the etiopathogenesis of FDCS ( 25 ).…”

Follicular dendritic cell sarcoma detected in hepatogastric ligament: A case report and review of the literature

“…About 10-20% of patients usually suffer from hyaline-vascular Castleman disease (HVCD)[6]. Since FDCS with HVCD has increased expression of vascular endothelial growth factor (VEGF), it is speculated that FDCS may be derived from hyperplastic dendritic cells stimulated by VEGF[7,8]. As a malignant tumor of the lymphatic system, no evidence shows that FDCS has any relationship with Epsteim-Barr virus and Epstein-Barr-encoded-RNA, which is typically used as a negative control for diagnosis[9].…”