Intracranial follicular dendritic cell sarcoma. Case report.
ABSTRACT Intracranial occurrence of follicular dendritic cell (FDC) sarcoma, a rare tumor derived from dendritic cells of the lymphoid follicle, has not yet been described. Therefore, the case of a 53-year-old man presenting with an intracranial mass invading the clivus is reported. The diagnosis of FDC sarcoma was confirmed by immunohistochemical staining for dendritic cell markers, that is, CD21, CD23, and CD35. Due to some similarities with meningioma, intracranial FDC sarcoma might be an underdiagnosed disease.
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ABSTRACT: Dendritic cell tumors are extremely rare and current knowledge on these tumors is limited. The characteristics of three dendritic cell sarcoma subtypes and their optimal treatment approaches are not fully clarified. We aimed to make a systematic review of the literature and enrich the current data with five new cases. Pooled analysis of 462 reported cases revealed that the tumor had no age, gender or racial predilection. Our analysis suggests that the young age, advanced stage, intraabdominal involvement and unfavorable histological features (i.e. large tumor size, absence of lymphoplasmacytic infiltration, coagulative necrosis, high mitotic count) may predict poor prognosis. Subtypes of this tumor have different clinical behaviors with interdigitating dendritic cell sarcoma being the most aggressive form. In general, surgery is the most effective treatment modality and adjuvant radiotherapy has no significant effect on overall survival of patients. The role of chemotherapy for the management of advanced disease is controversial.Critical reviews in oncology/hematology 06/2013; · 5.27 Impact Factor
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ABSTRACT: The aim of the present study was to characterize the clinicopathological features of follicular dendritic cell sarcoma (FDCS), and to report the experience of the Xin Hua Hospital Affiliated to Shanghai Jiaotong University School of Medicine (Shanghai, China) with this entity. The clinicopathological findings of four cases that had recently been encountered and 142 previously reported cases in the English literature were evaluated. The current tumors were found in two male and two female patients, aged 49-76 years old, who exhibited a mean tumor size of 8.7 cm. Three of the four cases were misdiagnosed during the initial diagnosis and one experienced intra-abdominal recurrence six months after the first diagnosis. Assessment of all 142 cases in the literature revealed a mild female predominance. The tumors exhibited a mean tumor size of ~7.0 cm. Histologically, the tumors were composed of plump spindle- or oval-shaped cells that exhibited eosinophilic cytoplasm and were arranged in sheets, storiform patterns or whorls. Immunohistochemically, the neoplastic cells expressed at least one of the FDC markers. Among the 130 cases with follow-up data, the overall recurrence, metastasis and mortality rates were 49.2% (64 cases), 21.5% (28 cases), and 13.8% (18 cases), respectively. FDCS can appear deceptively similar to other soft-tissue tumors, even poorly-differentiated carcinomas. A correct diagnosis requires a high degree of suspicion and immunohistochemical evaluation.Oncology letters 01/2015; 9(1):391-398. · 0.99 Impact Factor
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ABSTRACT: Das follikulre dendritische Retikulumzell- (FDC-)Sarkom ist ein seltener Tumor der dendritischen Retikulumzellen von Lymphfollikeln. Wenige Flle extranodaler Manifestationen im Kopf-Hals-Bereich wurden bisher beschrieben. Wir berichten ber eine 47-jhrige Patientin mit einem FDC-Sarkom der linken Tonsille, das vor 11Jahren erstmals auftrat und seither in immer krzeren Abstnden 3-mal rezidivierte. Histologisch zeigt sich eine Lsion mit extremer Vermehrung von spindelzellfrmigen Zellen mit z.T. gering entrundeten und hyperchromatischen Zellkernen. Die Patientin erhielt nach anfnglichen lokalen Tumorexzisionen bei Auftreten des 3.Rezidivs zustzlich eine adjuvante Chemotherapie mit Doxorubicin und Ifosfamid. Der bisher ber 11Jahre verfolgte Krankheitsverlauf besttigt eine grere Aggressivitt des Tumors als ursprnglich angenommen. Daher scheint die grozgige Tumorexzision, ggf. mit lokaler Lymphknotenausrumung, bei Rezidiven sinnvoll.Follicular dendritic cell (FDC) sarcoma is a rare tumor deriving from dendritic cells of the lymphoid follicle. Only a few cases of extranodal manifestation of FDC sarcoma of the head and neck have been reported. We describe the case of a 47-year-old woman with a FDC sarcoma of the left tonsil that first manifested 11years ago. She has suffered three recurrences since this time, at continually reducing intervals. Histologically, the neoplasm consisted of an increased number of spindle-shaped cells with partly hyperchromatic nuclei. The patient underwent local tumor excisions, and then received an adjuvant chemotherapy with doxorubicin and ifosfamide when she presented with the third recurrence. The 11year follow-up bears out a more aggressive behaviour of FDC sarcoma than first assumed. Therefore, a wide local excision, eventually combined with neck dissection in the case of recurrences, seems to be necessary.HNO 08/2006; 54(9):701-704. · 0.54 Impact Factor