Follicular Dendritic Cell Sarcoma Presenting As a Thyroid Mass

Starr, Jason S.; Joseph, Richard W.; Menke, David M.; Casler, John D.; Smallridge, Robert C.

doi:10.1200/jco.2013.49.3213

Cited by 23 publications

(39 citation statements)

References 19 publications

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“…The patient in the present study only presented with an abdominal mass and did not present with any other obvious symptoms. The manifestations of the patient are similar to the cases reported in the literature (9,10). Follicular dendritic cells are essential in the lymph nodes, where their primary role is directed towards antigen presentation and antigen-dependent B-cell maturation.…”

Section: Discussionsupporting

confidence: 82%

Follicular dendritic cell sarcoma of the spleen: A case report and review of the literature

Wang

Qiao

et al. 2016

Oncology Letters

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Abstract. Follicular dendritic cell sarcoma (FDCS), which originates in antigen-presenting cells of the B-cell follicles of the lymph nodes, is an uncommon disease, particularly in a splenic location. In a number of cases, FDCS may easily be confused with other solid tumors and be underdiagnosed. The present study reports a case in which such as tumor occurred in a 64-year-old woman. The patient presented with an abdominal mass, and computed tomography was performed. Histological and immunohistochemical studies revealed a diagnosis of FDCS. The patient underwent surgical treatment and had a good remission. At the time of writing, the patient was in good condition. The present study also discusses the clinical characteristics, treatment methods and prognosis of FDCS, with particular reference to the main points of the imaging diagnosis.

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Section: Discussionsupporting

confidence: 82%

Follicular dendritic cell sarcoma of the spleen: A case report and review of the literature

Wang

Qiao

et al. 2016

Oncology Letters

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“…Recently, promising results with gemcitabine and docetaxel were published (Conry, ). Due to limited data on cytogenetic (Perry et al , ) and molecular characteristics (Starr et al , ), few reports have demonstrated efficacy of targeted agents (Azim et al , ). Potential targets have been proposed, such as epidermal growth factor receptor (EGFR) (Sun et al , ).…”

mentioning

confidence: 99%

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

et al. 2017

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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.

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“…On the molecular level, Go et al 10 identified the BRAF V600E mutation, a potentially drugtargetable gain-of-function mutation that is variably present in other histiocytic and dendritic cell tumors, in a subset of follicular dendritic cell sarcoma tumors (18.5%, n = 27). In a case report of a rare, high-grade follicular dendritic cell sarcoma tumor of the thyroid, Starr et al 11 identified pathogenic loss-of-function mutations in PTEN (biallelic, nonsense) and TP53 (missense), and a novel missense mutation in RET using next-generation sequencing for a panel of 236 cancer genes. Griffin et al 12 recently provided the most comprehensive genetic analysis of follicular dendritic cell sarcoma to date using a targeted next-generation sequencing assay to profile mutational and copy-number status across 309 cancer-related genes in 13 tumors.…”

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confidence: 99%

Genomic analysis of follicular dendritic cell sarcoma by molecular inversion probe array reveals tumor suppressor-driven biology

et al. 2017

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Follicular dendritic cell sarcoma is a rare malignant neoplasm of dendritic cell origin that is currently poorly characterized by genetic studies. To investigate whether recurrent genomic alterations may underlie the biology of follicular dendritic cell sarcoma and to identify potential contributory regions and genes, molecular inversion probe array analysis was performed on 14 independent formalin-fixed, paraffin-embedded samples. Abnormal genomic profiles were observed in 11 out of 14 (79%) cases. The majority showed extensive genomic complexity that was predominantly represented by hemizygous losses affecting multiple chromosomes. Alterations of chromosomal regions 1p (55%), 2p (55%), 3p (82%), 3q (45%), 6q (55%), 7q (73%), 8p (45%), 9p (64%), 11q (64%), 13q (91%), 14q (82%), 15q (64%), 17p (55%), 18q (64%), and 22q (55%) were recurrent across the 11 samples showing abnormal genomic profiles. Many recurrent genomic alterations in follicular dendritic cell sarcoma overlap deletions that are frequently observed across human cancers, suggesting selection, or an active role for these alterations in follicular dendritic cell sarcoma pathogenesis. In support of a tumor suppressor-driven biology, homozygous deletions involving tumor suppressor genes CDKN2A, RB1, BIRC3, and CYLD were also observed. Neither recurrent gains nor amplifications were observed. This genomic characterization provides new information regarding follicular dendritic cell sarcoma biology that may improve understanding about the underlying pathophysiology, provide better prognostication, and identify potential therapeutic markers for this rare disease.

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Follicular Dendritic Cell Sarcoma Presenting As a Thyroid Mass

Cited by 23 publications

References 19 publications

Follicular dendritic cell sarcoma of the spleen: A case report and review of the literature

Follicular dendritic cell sarcoma of the spleen: A case report and review of the literature

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

Genomic analysis of follicular dendritic cell sarcoma by molecular inversion probe array reveals tumor suppressor-driven biology

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