Article

Intracranial follicular dendritic cell sarcoma. Case report

University of Bonn, Bonn, North Rhine-Westphalia, Germany
Journal of Neurosurgery (Impact Factor: 3.74). 01/2004; 99(6):1089-90. DOI: 10.3171/jns.2003.99.6.1089
Source: PubMed

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: 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.
    No preview · Article · Aug 2006 · HNO
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    ABSTRACT: 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 11 years 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 11 year 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.
    No preview · Article · Oct 2006 · HNO
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    ABSTRACT: Follicular dendritic cell sarcomas (FDCS) are grouped with the histiocytic and dendritic cell neoplasms. The natural history and response to different treatments have not been well established. The cases of 14 patients with FDCS who were seen at M. D. Anderson between 1995 and 2005 were reviewed. Median patient age was 48 years (range, 25-69 years). Histologically, four cases showed low-grade features, three cases showed low-grade features with focal high-grade features, and five cases showed high-grade features. Tumors were positive for CD21, CD23, and CD35 in 83, 90, and 44% of cases, respectively. Twelve (92%) of 13 tumors were strongly positive for epidermal growth factor receptor. Information on initial treatment was available in 11 patients, which included surgery alone in one patient, surgery and radiation in two, surgery and chemotherapy in one, chemotherapy alone in three, chemotherapy and radiation in one, surgery followed by radiation and chemotherapy in three patients. In eight patients the initial chemotherapy regimen was cyclophosphamide, doxorubicin, vincristine, and prednisone. Complete remission was achieved in 7 (63%) of 11 patients. Ten patients were alive at a median follow up of 22 months, 3 (23%) of 13 had no evidence of disease, and 7 (53%) of 13 patients were alive with disease. Follicular sarcoma is an aggressive neoplasm. Although most of the patients initially responded to treatment, the majority of them (81%) relapsed. A better understanding of the biology of FDCS could guide our efforts in the development of new treatment modalities for this rare disease.
    Full-text · Article · Aug 2007 · American Journal of Hematology
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