Extranodal follicular dendritic cell sarcoma presenting as colonic mass: A diagnostic and therapeutic challenge.

Folliclular dendritic cell sarcoma (FDCS) is an extremely rare neoplasm originating from folliclular dendritic cells, both nodally and extranodally. Its primary presentation as a large colonic mass is rare and can be misdiagnosed as epithelial tumor/soft tissue tumor both clinically and through histomorphology. Due to its rarity and limited consensus guidelines about its management, it presents as a diagnostic and therapeutic challenge for pathologists and oncologists. However, accurate diagnosis is imperative due to its distinct prognostic and therapeutic implications. Herein we report, two cases of extranodal FDCS of colon with the aim of contributing to the management of this uncommon entity.

Survival analysis in patients with follicular dendritic cell sarcoma: a population-based study.

Follicular dendritic cell sarcoma (FDCS) is a rare low-intermediate grade malignant neoplasm. To date, published data on FDCS clinical courses are sparse, and no conditional survival study has been performed. Thus, we retrospectively analyzed 187 patients diagnosed with FDCS from the Surveillance, Epidemiology, and End Results (SEER) database. In this study, the median age at diagnosis was 50 years and 91 (48.7%) patients were male. The most common primary location was the abdomen/pelvis (82, 43.9%). The 1-year, 3-year, and 5-year overall survival (OS) were 88.7%, 69.0%, and 59.8%, respectively. The 5-year conditional overall survival increased from 65.7% at baseline to 83.8% in 5-year survivors. The 3-year FDCS-specific death rate was 26.7% and the rate of death from other reasons was 3.7%. In addition, the annual death hazard was the highest in the first four years after diagnosis and increased again in the 7th and 8th years. Age > 60 years at diagnosis, metastatic disease, and FDCS in thoracic organs were associated with shorter OS and FDCS-specific survival. In addition, FDCS patients, with either local or metastatic disease, could benefit from surgery therapy. In addition, adjuvant radiotherapy or chemotherapy for local disease provided no significant improvement in overall survival or FDCS-specific survival. We hope these findings may guide treatments and surveillance strategies for FDCS patients in clinical practice.

Histiocytic and follicular dendritic cell sarcoma: Diagnostically challenging rare entities.

BACKGROUND: Follicular dendritic cell sarcomas (FDCSs) and histiocytic sarcomas (HSs) are exceedingly rare tumors. Most of the data on those entities are based on case reports or small case series. The natural history and response to different treatment modalities have not been well established. AIMS: To analyze the clinicopathologic features, immunophenotypic profile, treatment responses and to add to the existing data on FDCS and HS. STUDY DESIGN: Retrospective descriptive study. MATERIALS AND METHOD: The study was conducted at the department of Oncopathology at a tertiary care cancer hospital in India, retrospectively within the time period of four years (2016-2019). Total eight (8) cases were diagnosed: four cases of FDCS and four cases of HS involving nodal and extra-nodal sites. Clinical, histopathological, immunohistochemistry (IHC) and therapeutic data of the eight cases were retrieved and analyzed. STATISTICS: Descriptive statistics. RESULT: Among the four patients of FDCS, two had nodal and two had extra-nodal disease. Mean tumor size was 6 cm. Tumor cells expressed CD23, CD21, CD45, CD68 and S100. One patient received adjuvant chemotherapy (Gemcitabine and Docetaxel). Median survival was 36 months. None of them developed distant metastasis. Two of the patients having HS, developed bone metastasis. Median survival was 8.5 months. CD68 was consistently expressed in all cases of HS. Other applied IHC markers were negative in all the eight cases. CONCLUSION: FDCS and HS are under-recognized and easily prone to a wrong diagnosis. Therefore, considering these rare entities in differential diagnoses and inclusion of proper IHC biomarkers are necessary to avoid potential misdiagnosis.

Multidisciplinary diagnosis and treatment of recurrent follicular dendritic cell sarcoma in abdomen: A case report.

RATIONALE: Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor derived from follicular dendritic cells, and is often associated with Castleman disease. Here we present a rare case of paraneoplastic pemphigus (PNP) with FDCS which required multidisciplinary approach for the diagnosis and treatment. PATIENT CONCERNS: A 28-year-old Chinese female had FDCS recurrence, and primary clinical manifestation was PNP. DIAGNOSES: PNP with FDCS. INTERVENTIONS: The patient received gamma globulin infusion, took anlotinib, and underwent plasma exchange therapy. OUTCOMES: The skin lesions recovered and there was no evidence of tumor recurrence. LESSONS: The diagnosis and management of PNP with FDCS require close cooperation among surgeons, dermatologists, hematologists, otolaryngologists, oncologists, radiologists, pathologists, and respiratory doctors. The interesting clinical manifestations of this patient provide a multifaceted approach to the investigation of the interactions among FDCS, Castleman disease, and PNP.

Follicular dendritic cell sarcoma of the uterine cervix: a case report.

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare mesenchymal tumor that mostly occurs in systemic lymph nodes. FDCS in the uterine cervix has not yet been reported. CASE PRESENTATION: A 49-year-old woman was referred to our department with a cervical tumor, which was histologically suspected to be undifferentiated carcinoma. She underwent hysterectomy, salpingo-oophorectomy, and pelvic lymphadenectomy after neoadjuvant chemotherapy with paclitaxel and carboplatin. The resected specimen contained high numbers of spindle cells and was immunohistochemically confirmed to be FDCS. The tumor was completely resected and recurrence was not detected at a 16-month follow-up. CONCLUSION: FDCS is an extremely rare malignant tumor in the uterine cervix, and an accurate diagnosis and complete resection are essential for a good prognosis.

Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease.

BACKGROUND: Mediastinal follicular dendritic cell sarcoma (FDCS) is extremely rare. Due to potential under-recognization of this disease, it happens to be misdiagnosed, especially on core needle biopsy. We report 3 cases of mediastinal FDCS and provide a literature review to improve better understanding of the tumor and to reduce misdiagnosis. METHODS: Three cases of mediastinal FDCS in our clinic practice were studied, including their core needle biopsy and resected specimens, and those cases reported previously in English literature were retrieved and analyzed. RESULTS: The core needle biopsy of case 1 showed a tumor reminiscent of classical Hodgkin's lymphoma (CHL), while the resected mass was finally diagnosed with FDCS combined with hyaline-vascular Castleman's disease. Both the biopsy and resected tissue of case 2 were constitutive of the clear epithelioid cells with marked atypia. In both cases, definitive diagnoses were not made on core needle biopsy. In case 3, there were some areas morphologically similar to CHL, and some areas contained ovoid to spindle-shaped tumor cells with fascicular pattern. The analysis of 43 cases of mediastinal FDCS showed the age of patients were from 16 to 76 years old, the male to female ratio was 1.5:1, the maximal tumor diameters were 3-17 cm. 18 cases were underwent preoperative biopsy, whereas 15 (83.3%) of which were misdiagnosed initially, often as lymphoma. 32 patients had available follow-up data, the rates of recurrence, metastasis, and mortality were 12.5, 18.8 and 28.1%, respectively. Current limited data suggested no statistical differences between adverse prognosis and gender, age, tumor size, necrosis, or different therapeutics, respectively. CONCLUSIONS: Mediastinal FDCS is a rare malignancy that has yet not been fully understood and been often misdiagnosed, particularly when making a diagnosis on core needle biopsy. Increased awareness of this enigmatic tumor is crucial to avoid diagnostic pitfalls.

Follicular Dendritic Cell Sarcoma of Gastrointestinal Tract: an Uncommon Lesion, Commonly Missed.

INTRODUCTION: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm, accounting for only 0.4% of soft-tissue sarcomas. It shows both nodal and extranodal involvement. Considering the rarity and difficulties in diagnosing this tumor, we consider it very important to share our experience of diagnosing FDCS. Its correct diagnosis cannot be overemphasized as the treatment and prognosis of FDCS are very much different from tumors which come in its differential diagnosis. MATERIAL AND METHODS: We are presenting eight cases of extranodal FDCS in gastrointestinal tract diagnosed at our center in a period of 3 years (Feb 2015 to Feb 2018). Presenting complaints, demographic details, gross description, histologic features, immunostain results, and clinical follow-up were evaluated. RESULTS: Four patients were females and four were males. Tumor ranged in size from 5.5 to 35 cm. In five cases, tumor cells were arranged in storiform and whorling pattern. Lymphocytes were seen sprinkled in between these cells. In one case, lymphocytic infiltrate was extensive. Giant cells and frequent mitoses were noted in two cases. One case showed extensive necrosis. Tumor cells were strongly and diffusely positive for CD21 and CD35. Mean follow up of 11.8 months (range 01 to 24 months) was noted. CONCLUSION: FDCS is a rare tumor having distinct morphology and phenotype which if known can be correctly diagnosed. Therefore, knowledge of its varied location, morphology, and phenotype is very important to correctly diagnose this tumor and to prevent misdiagnosis and mistreatment.

Profiles of genomic alterations in primary esophageal follicular dendritic cell sarcoma: A case report.

RATIONALE: Follicular dendritic cell (FDC) sarcoma is a rare tumor with FDC differentiation that typically arises within lymph nodes but can also occur extranodally. To date, the primary esophageal FDC sarcoma has not been reported in the English literature. PATIENT CONCERNS: We described a 67-year-old female who foremostly presented with dysphagia, and the patient was readmitted due to a dry cough and pain of his right shoulder 2 years after initial treatment. DIAGNOSES: Primary esophageal FDC sarcoma with the right superior mediastinal lymph node metastasis. INTERVENTIONS: The esophageal tumor was removed by endoscopic submucosal dissection at the first hospitalization. At the second hospitalization 2 years after the initial visit, the tracheal stent loaded with (125) iodine radioactive seeds was placed. The profiles of genetic variations and immunotherapeutic biomarkers were also explored by next-generation sequencing protocol from the patient's blood, esophageal primary, and mediastinal metastatic tumor samples. OUTCOMES: The patient's symptom transitorily relieved, but she gave up further treatment and died 2 months after the tracheal stent was placed. As for the genomic alterations, we found 9 gene mutations in all the samples, including checkpoint kinase 2(CHEK2), FAT atypical cadherin 1 (FAT1), tumor protein 53 (TP53), DPYD, ERBB2 interacting protein (ERBB2IP), FBXW7, KMT2D, PPP2R1A, TSC2, whereas amplification of MYC was only in the metastatic example. The analysis of clonal evolution and phylogenetic tree showed the propagation and replay of polyclonal esophageal FDC sarcoma. At the same time, the detection of biomarkers for immunotherapy revealed microsatellite stable and mismatch repair-proficient (pMMR), which predicted a relatively poor anti-programmed death (PD-1)/programmed death ligand (PD-L1) immunotherapy outcome. On the contrary, the tumor mutational burdens were 10 mutations per 1 million bases in both the primary and metastatic tumor sample, which ranked the top 23.3% in solid tumors mutational burdens database of Geneseeq and might be a good predictor of the efficacy of anti-PD-1/PD-L1 immunotherapy. LESSONS: To the best of our knowledge, this case report announced the first case of extranodal primary esophageal FDC sarcoma in the world, and firstly revealed its unique genetic alterations profiles, which might contribute to further in-depth study of this rare disease.

Mediastinitis After Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration of a Follicular Dendritic Cell Sarcoma / Mediastinitis después de una aspiración transbronquial con aguja guiada por ecografía endobronquial de un sarcoma de células dendríticas foliculares

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Follicular dendritic cell sarcoma of the porta hepatis.

Follicular dendritic cell (FDC) sarcoma is a very rare neoplasm which commonly involves the lymph nodes and less commonly involves extranodal organs such as the liver. Most cases of FDC sarcoma are idiopathic, however some cases are associated with other disease states. Management of FDC sarcoma is primarily focused on surgical resection of the mass, and secondarily focused on radiotherapy, chemotherapy and/or biologic pharmacotherapy. We report the case of a patient who was found to have FDC sarcoma presenting as an obstructing mass of the porta hepatis, a manifestation which does not appear to be reported in the literature.

Follicular dendritic cell sarcoma of the duodenum: an extremely rare entity.

Extranodal follicular dendritic cell sarcoma (FDCS) is a very rare tumour, only reported in case reports and case series. It poses diagnostic and management challenge both to the clinician and pathologist. We present such a rare case of duodenal FDCS in a 56-year-old woman who was recently managed in our institution. Repeated pre surgical biopsies were non-diagnostic and the final diagnosis was made only after surgical excision of the tumour and with the help of histopathological and immunohistochemical studies. The patient had a complete en block resection of the tumour and was discharged home well 5 days postsurgery. To the best of our knowledge, this is the first case of FDCS reported arising from the duodenum.

Retroperitoneal follicular dendritic cell sarcoma in a young woman: Diagnosis and treatment challenges.

INTRODUCTION: Follicular dendritic cell sarcoma (FDCS) is an uncommon tumor that usually arises in lymph nodes, especially in the cervical, mediastinal, or axillary areas, but rarely in extranodal sites. Few cases have been reported in English literature so far. The scarcity may be partially due to under-recognition of this entity. Through this case report we analyzed the difficulties of clinical and pathological diagnosis of this rare tumor with its unusual location mistaken it with gynecological cancer's iliac lymph nodes metastases. We also discussed its systemic treatment options. CASE REPORT: A 48-year-old woman presented with a loss of weight and epigastralgia. Computed tomography (CT) showed a mass of 5cm of diameter, located close to iliac vessels. Investigation for gynecologic cancers was negative and a partial tumor resection was performed. Pathological examination readdressed the diagnosis of FDCS. Microscopically, the tumor was composed of a proliferation of spindle to ovoid cells arranged in fascicles, whorls and storiform pattern, accompanied by sprinkling of small lymphocytes. The nuclei of the tumor cells were elongated spindled or ovoid shape with vesicular chromatin and distinct small nuclei. Immunohistochemically, the tumor cells were positive for CD21, CD23 but negative for any type of cytokeratin. Even pathological diagnosis was misleading, therapeutic management was more challenging with this unusual location particularly associated with an aggressive clinical course. Two lines of chemotherapy gave different responses. CONCLUSION: Clinical and pathological diagnosis of retroperitoneal FDCS needs vigilance. Both lymphoma and sarcoma chemotherapy regimens are effective. Due to this pathology's rareness we highlighted a lack of treatment consensus and proposed options.

Follicular dendritic cell sarcoma of the head and neck. Literature review and report of the tonsil occurrence in a Ugandan patient.

We report a case of follicular dendritic cell sarcoma (FDCS) in a 60-year-old Ugandan female who presented with a 6-year history of a progressive left sided tonsillary mass. General systemic examination was unrevealing and the patient underwent left tonsillectomy. She was subsequently lost to follow-up. Grossly, the mass measured 6 cm in diameter and had a mottled appearance due to tissue microhaemorrhages. Markers specific for follicular dendritic cell differentiation (CD21, CD35 and CD23), p53 and EGFR were expressed on immunohistochemical analysis. Review of all of the 49 published reports of tonsil FDCS showed that this entity tended to occur at younger age (mean: 44.5 yrs) in women than in men (mean: 49.4 yrs). Tumour size ranged from 0.8 to 5 cm in maximum dimension (mean 2.9 cm). Only 12.2% of the patients presented with metastatic disease at initial diagnosis, all localised in the cervical lymph nodes. Local or distant recurrences occurred after a mean period of 72.5 months. In conclusion, although the pertinent literature suggests that FDCS should be considered at least of intermediate grade, our review indicates that FDCS of the tonsil region behaves as a low-grade sarcoma.

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

Dendritic cell sarcomas 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 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.

Retroperitoneal and mediastinal follicular dendritic cell sarcoma: report of 3 cases with review of literature.

Follicular dendritic cell sarcoma (FDCS) is a rare malignant histiocytic proliferation of antigen presenting follicular dendritic cell. It is an uncommon primary malignancy first described by Monda et al. in 1986. Most commonly reported cases are lymph nodal. Occasional cases occur in extra nodal sites. Here, we describe the clinicopathological features, histomorphology and outcome of three patients with extranodal FDCS along with a concise review of literature on the topic. All three patients were adult females. Two patients were in third decade, and one had age of 50 years. Among the three cases, two cases are presented as retroperitoneal mass and one as mediastinal mass. CT scans revealed heterogeneously enhancing masses. All the cases showed ovoid to spindle neoplastic cells arranged predominantly in whorling, fascicular and storiform patterns with inflammatory infiltrate. Immunohistochemically, the tumor cells are positive for CD21, CD23, CD35 and Clustrin. In view of rarity and variable clinical presentation in FDCS, accurate diagnosis is necessary. Copyright © 2015 John Wiley & Sons, Ltd.

High efficacy of tumor-targeting Salmonella typhimurium A1-R on a doxorubicin- and dactolisib-resistant follicular dendritic-cell sarcoma in a patient-derived orthotopic xenograft PDOX nude mouse model.

Follicular dendritic-cell sarcoma (FDCS) is a rare and recalcitrant disease. In the present study, a patient-derived orthotopic xenograft (PDOX) mouse model of FDCS was established in the biceps muscle of nude mice. The FDCS PDOX was resistant to both doxorubicin (DOX) and NVP-BEZ235, dactolisib (BEZ) an experimental agent which is a dual pan-phosphoinositide 3-kinase-mammalian target of rapamycin inhibitor. However, in contrast to DOX and BEZ, the FDCS PDOX was sensitive to the tumor-targeting bacterial strain, Salmonella typhimurium A1-R (S. typhimurium A1-R). The combination of S. typhimurium A1-R and either DOX or BEZ did not increase the antitumor efficacy of S. typhimurium A1-R, indicating that DOX and BEZ were not active in this PDOX model. The efficacy of S. typhimurium A1-R in this recalcitrant FDCS gives strong impetus to move bacterial therapy to clinical trials for this disease. The findings of the present study are of particular importance since it demonstrates that S. typhimurium A1-R is effective in a PDOX model of FDCS established from a patient who failed DOX therapy.

Follicular Dendritic Cell Sarcoma.

Follicular dendritic cell sarcoma is an uncommon neoplastic proliferation of spindled to ovoid cells with morphologic and immunophenotypic features similar to normal follicular dendritic cells. While most follicular dendritic cell sarcomas arise from lymph nodes, at least one-third occur in extranodal sites. A broad differential diagnosis can be developed--as this tumor has morphologic features similar to other tumors, hence creating a diagnostic pitfall--but its immunophenotypic profile is quite specific and is diagnostically crucial. Herein, we review the pathogenesis; histologic morphology; and immunohistochemical, electron microscopy, and clinical features, including treatment and prognosis, of follicular dendritic cell sarcomas. We will briefly describe the role of molecular studies including utility of BRAF mutations in diagnosing this tumor.