Multimodal Treatment of Mediastinal Follicular Dendritic Cell Sarcoma: a case report successfully treated
Stefano Sanna, Marco Monteverde, Marco Taurchini, William Grossi, Alessandra Dubini and Davide Dell'amore
World Journal of Surgical Medical and Radiation Oncology 2013, 2:1
Follicular Dendritic Cell Sarcoma (FDCS) is an extremely rare malignant tumour of unknown etiology which, just to its heterogeneity, is not always easily recognized, diagnosed and accurately treated. To date only 49 cases of FDCS are reported in Literature. There could be a difficult differential diagnosis with metastatic carcinoma, ectopic thymoma or primary – metastatic malignant fibrous histiocytoma. Superficial and deep lymph nodes in axilla and more rarely in inguinal sites, are usually involved with aspecific symptoms, very rarely intra-abdominal structures are interested. A mediastinal localization of this tumour is even more rare. An accurate diagnosis is very difficult and is often obtained on surgical specimen and only with immunohistochemical studies. The treatment of first choice for this pathology is surgical excision, whatever is the site of the lesion; radio - chemotherapy could be considered as adiuvant treatment, in case of incomplete excisions, or principal treatment of local recurrences or distant metastases, whose rates vary between 24 and 43% of cases. This combinated therapy seems to be the key of good survival rates. We present a case of a 42 years old young man affected by mediastinal FDCS, successfully treated with surgical complete excision of the mass and radiotherapy.
Follicular dendritic cell sarcoma, spindle cell sarcoma, lymph node involvement, mediastinal site, surgical therapy.