Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature

Abstract

BACKGROUD Follicular dendritic cell (FDC) sarcomas are rare neoplasms that occur predominantly in the lymph nodes. They can also occur extranodally. Extranodal FDC sarcomas most commonly present as solitary masses. Inflammatory pseudotumor (IPT)-like FDC sarcomas, a subcategory of FDC sarcomas, are rarer than other sarcoma subtypes. They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration. Paraneoplastic pemphigus (PNP), also known as paraneoplastic autoimmune multiorgan syndrome, is a rare autoimmune bullous disease that is associated with underlying neoplasms. PNP has a high mortality, and its early diagnosis is usually difficult. CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis, conjunctivitis, and skin blisters and erosions as her first symptoms of PNP with an intra-abdominal IPT-like FDC sarcoma. The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment. She showed no recurrence at the 1-year follow-up. CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP. PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis. Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.

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).…”

Case report: Hepatic inflammatory pseudotumor-like follicular dendritic cell sarcoma: A rare case and minireview of the literature

“…Patients are mainly asymptomatic or present with abdominal distension or pain, occasionally accompanied by systemic symptoms such as back pain, waist soreness, significant weight loss, fever, and weakness. In rare cases, IPT-like FDCS exhibits paraneoplastic arthritis ( 7 ), and paraneoplastic pemphigus ( 8 – 10 ).…”

“…Only 9.65% of patients ( n = 11) experienced recurrence or metastasis during follow-up. Yet, PNP-associated IPT-like FDCS predominantly occurs in intraabdominal sites, indicating poor prognosis ( 8 – 10 ). Surgery is the most effective therapy for IPT-like FDCS, and only two cases (Cases 49 and 63) received chemotherapy or targeted therapy.…”

Inflammatory pseudotumor-like follicular dendritic cell sarcoma with first clinical manifestation of thrombocytopenia: A case report

“…The pathogenesis of IPT-like FDCS is associated with EB virus infection. It has also been documented that cases of IPT are associated with surgical or traumatic inflammation, autoimmune diseases, and infections, such as Castleman disease and pallidococcus, [5,[8][9][10] although other pathogenic mechanisms remain unclear. This case was positive for EBV and was accompanied by significant thrombocytopenia, suggesting that its pathogenesis was related to EB virus infection and pathological changes in the spleen.…”

“…It has been reported that paraneoplastic pemphegus monkeys, myasthenia gravis, and other diseases can be combined, and more than 90% of the cases are associated with EB virus. [2][3][4][5][6] The diagnosis of IPT-like FDC sarcoma is problematic because of the rarity written informed consent was obtained from the patient for publication of the case details and accompanying images.…”