Intra-abdominal Follicular Dendritic Cell Sarcoma (FDCS): Series of 18 cases of a rare entity from Pakistan

Zubair, Maha; Din, Nasir Ud; Arshad, Sidra; Minhas, Khurram; Idress, Romana; Ahmad, Zubair

doi:10.1016/j.anndiagpath.2020.151595

Cited by 3 publications

(2 citation statements)

References 32 publications

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“…The tumor cells are positive for CD21, CD23, CD35, clusterin, and CXCL13 and negative for keratins, EMA, CD30, ALK Protein, CD3, CD20, CD79a, CD1a, MPO, CD34, and HMB-45. 22 GIST must always be considered in the differential diagnosis of ovoid to epithelioid gut neoplasms. Most cases occur in the stomach and small intestine and are composed of fascicles and sheets of spindle to epithelioid cells.…”

Section: Discussionmentioning

confidence: 99%

Anaplastic Large-cell Lymphoma Involving Gastrointestinal Tract: A Clinicopathologic Study of 25 Cases of a Rare Tumor at a Rare Site

et al. 2022

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Background. Anaplastic large-cell lymphoma (ALCL) is an uncommon lymphoma divided into anaplastic lymphoma kinase (ALK) positive, ALK negative, and breast implant-associated (BIA) ALCL. Gastrointestinal tract involvement is very rare and may be difficult to diagnose. Its recognition is crucial as prognostic ramifications are different. Objectives. To describe clinicopathological features of ALCL involving the gastrointestinal tract. Materials and Methods. Slides were reviewed. Diagnosis was confirmed. Histological and immunohistochemical features were described. Results.Twenty-five tumors were diagnosed during the study period. Ages ranged from 14 to 65 years (mean 41 years). Mean age for ALK-negative and ALK-positive patients were 49 and 17 years, respectively. Twenty-one were males and 4 were females. Eighteen involved small intestine. Mean tumor size was 4.2 cm. All showed diffuse sheets of large anaplastic cells with pleomorphic nuclei, abundant pink cytoplasm, and strong positivity for CD30. Epithelial membrane antigen was positive in 17 tumors and keratin was negative in all. Eighteen tumors were ALK negative. Out of 14 patients with follow-up, 12 died within a few months of diagnosis. Seven had stage IE, 5 had stage IIE, and 2 had stage IV disease. Two patients were alive at 35 and 60 months. Twelve received chemotherapy. Conclusion. A marked male predominance was noted. Small intestine was the commonest site of involvement. Majority were ALK negative. ALK-negative tumors occurred in older patients and ALK positive in younger patients. Prognosis was poor. ALCL should be included in the differential diagnosis of anaplastic epithelioid cell neoplasms in the gastrointestinal tract.

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Section: Discussionmentioning

confidence: 99%

Anaplastic Large-cell Lymphoma Involving Gastrointestinal Tract: A Clinicopathologic Study of 25 Cases of a Rare Tumor at a Rare Site

et al. 2022

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“…However, local recurrence is common in 40–50% of cases ( 2 , 3 ). Higher risk FDCS, which are larger in size or located intra-abdominally, display more aggressive spread, which can require the use of chemo or radiation therapy ( 3 , 4 ).…”

Section: Introductionmentioning

confidence: 99%

Case report: Splenic inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS): a trial of immunotherapy and review of the literature

Resnick,

Monroe,

Siddiqi

et al. 2024

Front. Oncol.

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Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is a rare malignancy with fewer than 150 cases in the literature. IPT-like FDCS follows an indolent course with most cases definitively managed with surgical resection. We present a case of IPT-like FDCS with multiple recurrences with a trial of immunotherapy. The patient initially presented with splenic involvement requiring splenectomy, subsequently recurring in the liver requiring hepatic resections. Afterwards, there was recurrence with pelvic/small bowel involvement for which treatment was trialed with ipilimumab and nivolumab. The patient progressed despite dual immune checkpoint inhibitor therapy requiring a small bowel resection. To date, this is the first case of immunotherapy use in IPT-like FDCS. Therefore, more evidence is needed to support additional treatments in recurrent IPT-like FDCS after resection.

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Follicular dendritic cell sarcoma

2021

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Summary Follicular dendritic cells (FDC) are mesenchymal-derived dendritic cells located in B-follicles where they play a pivotal role in triggering and maintaining B-cell adaptive immune response. In 1986 Dr. Juan Rosai first reported a series of neoplasms showing features of FDC and defined it as Follicular Dendritic Cell Tumor, subsequently renamed as “sarcoma” (FDCS). In its seminal and subsequent articles Rosai and colleagues highlighted the heterogeneous microscopic appearance of FDCS and its immunohistochemical and ultrastructural features. FDCS mostly occurs in extranodal sites (79.4% of cases) and lymph nodes (15.1%); in about 7%-10% of cases it is associated with hyaline-vascular Castleman disease. Given its significant growth pattern and cytological variability, FDCS can be confused with various neoplasms and even inflammatory processes. The diagnosis requires the use of a broad spectrum of FDC markers (e.g. CD21, CD23, CD35, clusterin, CXCL13, podoplanin), particularly considering that tumor antigen-loss is frequent. The inflammatory-pseudotumor-like (IPT-like) variant of FDCS, in addition to its peculiar histopathological and clinical features, is characterized by positivity of tumor cells for Epstein-Barr virus, representing a diagnostic requisite. No distinctive genetic and molecular anomalies have been identified in FDCS. It often carries an aberrant clonal karyotype and chromosomal structural alterations, frequently involving onco-suppressor genes. Direct or next generation sequencing showed alterations on genes belonging to the NF-κB regulatory pathway and cell-cycle regulators. In contrast to hematopoietic-derived histiocytic and dendritic cells tumors, FDCS typically lacks mutations in genes related to the MAPK pathway. FDCS recurs locally in 28% and metastasizes in 27% of cases. Extent of the disease, surgical resectability and histopathological features are significantly associated with the outcome. IPT-like FDCS behaves as an indolent tumor, even if it often recurs locally over years. Complete surgical excision is the gold standard of treatment. Data on targeted therapies (e.g.: tyrosine kinase inhibitors) or immune checkpoint inhibitors are very limited and responses are variable. A better understanding of the molecular drivers of this tumor may lead to potential new therapeutic strategies.

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Intra-abdominal Follicular Dendritic Cell Sarcoma (FDCS): Series of 18 cases of a rare entity from Pakistan

Cited by 3 publications

References 32 publications

Anaplastic Large-cell Lymphoma Involving Gastrointestinal Tract: A Clinicopathologic Study of 25 Cases of a Rare Tumor at a Rare Site

Anaplastic Large-cell Lymphoma Involving Gastrointestinal Tract: A Clinicopathologic Study of 25 Cases of a Rare Tumor at a Rare Site

Case report: Splenic inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS): a trial of immunotherapy and review of the literature

Follicular dendritic cell sarcoma

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