Follicular dendritic cell sarcoma: clinicopathologic study of 15 cases with emphasis on novel expression of MDM2, somatostatin receptor 2A, and PD-L1

Agaimy, Abbas; Hadravský, Ladislav

doi:10.1016/j.anndiagpath.2016.05.003

Cited by 40 publications

(47 citation statements)

References 25 publications

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“…First, EBV was found to be clonal in splenic cases, as well as in primary liver IPT-like FDC tumors [93,106,111]. Also, although splenectomy is almost always curative, some cases present aggressive outcome, with local recurrence and/or lymph node infiltration [89,104]. Morphological atypia and complex karyotype were also described in these tumors [92].…”

Section: Follicular Dendritic Cell Tumors Of the Spleenmentioning

confidence: 99%

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Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

et al. 2017

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Follicular dendritic cell (FDC) tumor is an uncommon neoplasm. It generally presents as a slow-growing, painless mass, without systemic symptoms. Histological features usually include low grade spindle cell proliferation. This tumor occurs primarily in lymph nodes, especially cervical and axillary, however, involvement of extranodal sites such as the tonsils, spleen, liver, and gastrointestinal tract has been reported. Inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDCT) is a rare, distinctive histological subtype of this low-grade malignant neoplasm, with consistent Epstein-Barr virus (EBV) association. The differential diagnosis with other fibro-inflammatory tumor proliferations, as inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT), may be challenging. In the present article, two cases of IPT-like FDCT of the spleen are presented, with a broad overview of the literature: one 77-year-old male and one 70-year-old female. A large immunohistochemical panel should be used for diagnosis, as no single specific and totally sensitive markers are available, including markers for CD21, CD23, CD35, CNA42, and clusterin. Individual cases may express one or more of these markers, so that all of them should be investigated. In situ hybridization for EBV is constantly positive. Immunostaining for ALK should be negative, as it is present in roughly half of the cases of IMT. This panel should be used in combination of clinical, laboratory, and topographic evidences. Importantly, inclusion of this lesion as a possible option in clinical and pathological investigation represents the basis for a correct diagnosis.

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Section: Follicular Dendritic Cell Tumors Of the Spleenmentioning

confidence: 99%

“…From 1996 to date we retrieved 22 publications reporting IPT-like FDC tumors [11,15,35,[89][90][91][92][93][94][95][96][97][98][99][100][101][102][103][104][105][106][107]. There were 46 patients described: 24 female, 22 male (ratio 1.1:1), median age 57y (range 29-79y).…”

Section: Follicular Dendritic Cell Tumors Of the Spleenmentioning

confidence: 99%

Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

et al. 2017

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“…The miRNA expression profile of FDCS tumor cells is similar to fibroblasts and myopericytomas or to FDC from Castleman disease, re‐emphasizing the mesenchymal rather than hematolymphoid origin of these tumors. Molecular studies have shown recurrent alterations in nuclear factor‐κB regulatory genes; a minority of cases harbors BRAF, PTEN or TP53 mutations or MDM2 amplification . BRAF V600E mutations, which occur in about 20% of FDCS cases, can be used as for diagnostic or theranostic purposes.…”

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confidence: 99%

“Medusa‐Head” cells, “Starfish” cells, and interconnecting long cytoplasmic processes as diagnostic cytologic clues for follicular dendritic cell sarcoma in fine needle aspiration samples

Czapla

Omman

Nam

et al. 2017

Diagnostic Cytopathology

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“…An inflammatory pseudotumor subtype has been described in which chronic inflammatory cells predominate; this lesion is more common in women, preferentially involves the liver and spleen, and is associated with Epstein‐Barr virus 12 . Lesional cells of FDCS typically stain positively for FDC markers such as CD21, CD23, CD35, and CNA42 by immunohistochemistry 8,13 . CD68, S100‐protein, and EMA may stain positively, which can cause diagnostic difficulties 8‐10 .…”

Section: Introductionmentioning

confidence: 99%

Fine needle aspiration of an intranodal follicular dendritic cell sarcoma: A case report with molecular analysis and review of the literature

Andersen

Kerr

Lisovsky

et al. 2020

Diagnostic Cytopathology

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Follicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm of follicular dendritic cell origin which can present a diagnostic challenge. Due to the rarity of this neoplasm, its molecular pathogenesis has not been fully elaborated. A previous series of 13 cases reported that 38% contained mutations of genes encoding proteins involved in negative regulation of NF‐κB. NF‐κB is a family of transcription factors regulated through multiple cellular processes known as the canonical and noncanonical pathways. Here we present the case of a 62‐year‐old man who presented with abdominal pain and systemic symptoms and was found to have a mass in the porta hepatis. Fine needle aspiration cytology demonstrated a spindle cell neoplasm with vesicular chromatin and prominent nucleoli with admixed lymphocytes. Surgical resection showed an intranodal, 7.3 × 5.5 × 3.5 cm, solid mass composed of plump, spindle to histiocytoid cells with ovoid nuclei and small, prominent nucleoli arranged in a whorled and fascicular pattern. The lesional cells stained positively for CD21, CD23, and CD35 by immunohistochemistry, consistent with a diagnosis of FDCS. Next‐generation sequencing revealed pathologic mutations in three genes involved in NF‐κB regulation pathways: NFKBIA, TNFAIP3, and TRAF3. A pathologic TP53 mutation was also identified. This case report supports prior associations of the NF‐κB pathway dysregulation and FDCS. Additionally, it is the first reported FDCS case with TRAF3 mutation as well as the first reported case to suggest disruption in both the canonical and noncanonical NF‐κB pathways in the same lesion.

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Follicular dendritic cell sarcoma: clinicopathologic study of 15 cases with emphasis on novel expression of MDM2, somatostatin receptor 2A, and PD-L1

Cited by 40 publications

References 25 publications

Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

“Medusa‐Head” cells, “Starfish” cells, and interconnecting long cytoplasmic processes as diagnostic cytologic clues for follicular dendritic cell sarcoma in fine needle aspiration samples

Fine needle aspiration of an intranodal follicular dendritic cell sarcoma: A case report with molecular analysis and review of the literature

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