Characteristics, management, and outcomes of patients with follicular dendritic cell sarcoma

Abstract

Summary Dendritic cell sarcomas (DCS) are rare tumours of antigen presenting cells. Data regarding their biology, management and outcomes are sparse. We analysed 66 patients with follicular dendritic cell sarcoma (FDCS). Six patients also had Castleman disease, 9 had another malignancy and 13 had an autoimmune disease. Fifty-four per cent of patients presented with localized disease and 46% with systemic involvement. The median progression-free (PFS) and overall survival (OS) following frontline therapy was 21 months and 50 months, respectively. Survival outcomes were significantly inferior in patients with extranodal, bulky or intra-abdominal disease at presentation. Stage was not associated with survival. Management approaches were heterogeneous. Patients who underwent an upfront gross total resection (GTR) experienced better PFS and OS (both P<0.0001). In patients who underwent a GTR, consolidative radiotherapy was associated with improved local control (P = 0.03), PFS (P = 0.04) and OS (P = 0.05). In patients with measureable disease, gemcitabine with a taxane yielded an overall response rate of 80%. The pattern of relapse was predominantly locoregional. Salvage rates after recurrence were poor. Studies are underway at our institution to define the genomic profile in FDCS and identify potential novel therapeutic targets.

Follicular Dendritic Cell Sarcoma of Colon Presenting as Colo-colic Intussusception: a Rare Entity in Gastrointestinal Site With Extremely Rare Presentation

“…A number of patients of locally advanced disease may be treated with initial neoadjuvant chemotherapy and then they may become surgically resectable candidate. 33 The efficacy of systemic treatment with chemotherapy and targeted agents for the management of disseminated FDCS is not well defined. The available evidence suggest that the same regimens that are used in advanced soft tissue sarcoma with gemcitabine plus docetaxel are effective in FDCS.…”

Follicular dendritic cell sarcoma of the porta hepatis

“…Unfortunately, there is a scarcity of information regarding long-term outcomes of hepatic FDC sarcoma. Recent management recommendations include surgical resection followed by consolidative radiotherapy and frequent follow-up in patients found to have localised and resectable disease 10. For patients with non-localised and/or non-resectable disease, it is recommended that they undergo cytoreductive chemotherapy with a combination of gemcitabine and a taxane, followed by surgery with or without radiotherapy if remnant disease after chemotherapy is found to be localised and resectable 8.…”

Sarcoma de células dendríticas foliculares: una entidad poco frecuente

“…Monda y col., en 1986, los caracterizaron por primera vez. Se trata de un entidad muy poco frecuente que constituye < 0,4% de los sarcomas de tejidos blandos; el conocimiento de la enfermedad y de su manejo es limitado dados los pocos casos descritos en la literatura; Jain y col.(2017) con 66 casos ofrecen la mayor serie recogida hasta la fecha 1 .…”

“…Estos tumores suelen confundirse con linfomas de células grandes, sarcomas fusocelulares, carcinomas somatoides, variantes de melanomas o sarcomas de células dendríticas interdigitantes. El sarcoma de células foliculares dendríticas no parece tener predominio por el sexo y la edad de presentación: varía entre 14 y 78 años, con predominio en adultos alrededor de los 45 años, aunque también existen casos descri-tos en la infancia 1,2 . Pueden involucrar sitios nodales y extranodales, afectando en la mayoría de casos descritos los ganglios axilares, cervicales y supraclaviculares, mientras que aproximadamente un tercio de los casos han sido descritos en localizaciones extraganglionares.…”

“…Se desconoce la etiología pero en determinados casos se relaciona como factor predisponente la enfermedad de Castleman, que es una hipertrofia ganglionar caracterizada por hiperplasia angiofolicular; también se ha relacionado en algunos casos con enfermedades autoinmunitarias, como el pénfigo [1][2][3] . Los SCDF de localización ganglionar, así como los asociados a mucosas superficiales (paladar, lengua) son tumores que clínicamente suelen presentarse como masas tumorales indoloras de crecimiento lento.…”