Hepatic inflammatory pseudotumor-like follicular dendritic cell tumor: a case report

Abstract

Background Inflammatory pseudotumor-like follicular dendritic cell sarcoma is a rare histological variant of follicular dendritic cell sarcoma involving typically the spleen and the liver, often linked to the presence of Epstein–Barr virus infection. Definite diagnosis of this type of sarcoma is difficult to make owing to nonspecific clinical and imaging findings and is based on histopathological features. Inflammatory pseudotumor-like follicular dendritic cell sarcoma is described as a low-aggressivity tumor with a favorable prognosis. Case presentation We report the case of a 34-year-old Caucasian woman, Epstein–Barr virus positive, diagnosed with hepatic inflammatory pseudotumor-like follicular dendritic cell sarcoma and surgically treated in November 2014, who developed 6 years later a recurrence for which she underwent once again surgical treatment. As far as we know, fewer than 30 reports of inflammatory pseudotumor-like follicular dendritic cell liver tumors have been reported in the English literature. Conclusions Although it is an uncommon tumor, inflammatory pseudotumor-like sarcoma is a diagnostic worth being taken in consideration, and surveillance is recommended owing to the possibility of recurrence.

Challenges in the Diagnosis of Epstein-Barr Virus-positive Inflammatory Follicular Dendritic Cell Sarcoma

“…After an extensive survey of the English literature, we found 70 reported cases of EBV + IFDCS 2–8,12–34. The detailed clinical data of the 70 cases from the literature are included in Table S2 (Supplemental Digital Content 2, http://links.lww.com/PAS/B509).…”

A Clinicopathology Review and Update of Epstein–Barr Virus-Associated Mesenchymal Tumors

“…In such instances, the suppression of the immune system can trigger the activation of EBV latency-associated genes, ultimately resulting in the development of tumors. However, this mechanism has not been observed in any of the previously reported cases of IPT-FDCS [80,86,88,89,91,100,101]. This aspect represents an active area of ongoing research and investigation, and further studies are needed to unravel the underlying mechanisms contributing to the development of IPT-FDCS.…”

“…This tumor is categorized into two subtypes: conventional FDCS and IPT-FDCS. IPT-FDCS was first identified in 2001 and represents a distinct subtype of FDCS that consistently exhibits EBV positivity [75,86]. FDCS is known to affect individuals across a wide age range, spanning from 7 to 82 years old, with a prevalence among middleaged adults [85,[87][88][89].…”

“…Surgical excision is considered the treatment of choice for IPT-FDCS. Given its typically indolent behavior, the role of adjuvant therapy remains unclear when it comes to managing this type of tumor [86,91]. However, due to the possibility of a recurrence, regular surveillance is recommended [86].…”