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

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 intraabdominal 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.

Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus

Background The presence of autoantibodies against multiple epidermal proteins is an important feature in paraneoplastic pemphigus （PNP）. Circulating anti-desmoglein 3 autoantibody, the major pathogenic autoantibody in pemphigus vulgaris （PV）, has been proved pathogenic in PNP. Because of many clinical differences between PNP and PV, we speculate about the involvement of other autoantibodies in the pathogenesis of PNP. Envoplakin （EPL） and periplakin （PPL） are recognized by most PNP sera. Their linker subdomains are highly homologous and necessary for the association of intermediate filaments. Methods We characterized the autoantibodies against the linker subdomains of EPL and PPL in PNP patients＇ sera and their associated tumors by enzyme-linked immunosorbent assay （ELISA） and immunofluorence. We also applied the purified autoantibodies against EPL and PPL from PNP sera to cultured human epidermal keratinocytes （HEK）, to evaluate the changes of cell-cell adhesion. Results Autoantibodies against EPL and PPL were detected in most PNP patients by ELISA, and the decrease of these autoantibodies after removal of the tumors was roughly comparable to the improvement of clinical symptoms. Cultured tumor cells from PNP patients secreted these autoantibodies. Specific immunoglobulin receptors for EPL and PPL were found on B lymphocytes in tumors from PNP. Furthermore, purified anti-EPL and anti-PPL autoantibodies from PNP sera were capable of dissociating cultured human epidermal keratinocytes. Conclusion Autoantibodies against EPL and PPL may also be pathogenic in PNP.

A Case Report on Paraneoplastic Pemphigus Associated Colonic Carcinoma

Paraneoplastic pemphigus mucocutaneous syndrome, has （PNP）, an acantholytic a universal association with malignancy and a poor prognosis, The clinical picture is characterized by painful mucosal erosions, ulcerations, and polymorphous skin lesions that progress to blistering eruptions on the trunk and extremities. In the reviews of all PNP, hematologic malignancies such as non-Hodgkin＇s lymphoma, chronic lymphocytic leukemia, and Castleman＇s disease account for the most part; nonhematologic malignancies are very seldom especially adenocarciuoma of colon, These will be illustrated in the following paper on the basis of a case report of a patient with colonic adenocarcinoma who presented with PNP.

Retrospective analysis of 45 cases of localized retroperitoneal Castleman disease from a single center

Background:Castleman disease(CD)is a rare lymphoproliferative disease characterized by high heterogeneity in clinical manifestation and prognosis.This study aimed to summarize clinical features of localized retroperitoneal CD and our experiences to improve the diagnosis and treatment of this disease.Methods:Clinical data of 45 patients with localized retroperitoneal CD were retrospectively analyzed.The differences in clinical features between groups with and without paraneoplastic pemphigus(PNP)were compared.Survival was analyzed between groups depending on whether complicating with PNP,bronchiolitis obliterans(BO),gender,age and uni-centric CD(UCD)/multi-centric CD(MCD),respectively.Results:Significant differences were observed between patient groups in the prevalence of retroperitoneal CD with PNP complicated with BO(P=0.010),the constituent ratios of initial symptoms(P<0.001)and the duration from appearance of the initial symptoms to being diagnosed(P=0.009).Among 45 cases,43 tumors had clear margins and intact envelops and were completely resected,40 patients were cured or significantly relieved,3 patients were not significantly relieved,2 patients received palliative surgical therapy and eventually relapsed and died after surgery.There were significant differences in the survival rate between groups depending on complication with BO,gender and age(≤40 and>40 years)(all P<0.05).Conclusions:Prompt and complete removal of the retroperitoneal CD tumor is critical to the management of this disease,as palliative resection tends to cause relapse and lead to a poor prognosis.Retroperitoneal CD patients with PNP may develop complications from BO leading to death.Complication with PNP,complication with BO,male gender and age≥40 years were identified as prognostic risk factors for patients with localized retroperitoneal CD.