ABSTRACT
BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare lymphoid neoplasm presenting in the head and neck. There are no pooled analyses of head and neck FDCS cases in the English language literature. METHODS: A MEDLINE and PubMed review of cases from 1978 to February 2014 was performed. Demographics, clinicopathologic data, and outcomes were summarized. RESULTS: We presented 2 patients and analyzed 97 cases. The mean age was 42.7 years (SD = 16.3 years). Outcomes were available for 76 patients. Tumors ≤4 cm had better disease-free survival (63% vs 28% at 5 years; p = .0282). Locoregional recurrence was significantly less likely with surgery and radiation compared to surgery alone (15% vs 45%; p = .019) and in patients receiving a neck dissection (10% vs 43%; p = .046). CONCLUSION: This pooled analysis provides the largest sample size of FDCS of the head and neck to date and suggests that radiation and neck dissection may be beneficial to locoregional oncologic control. © 2015 Wiley Periodicals, Inc. Head Neck 38: E2241-E2249, 2016.
Subject(s)
Dendritic Cell Sarcoma, Follicular/radiotherapy , Dendritic Cell Sarcoma, Follicular/surgery , Head and Neck Neoplasms/radiotherapy , Head and Neck Neoplasms/surgery , Adult , Disease-Free Survival , Female , Humans , Male , Middle Aged , Neck Dissection , Neoplasm Recurrence, Local , Treatment OutcomeABSTRACT
Follicular dendritic cell sarcoma is a rare and aggressive tumor and its management is a major clinical challenge. Surgery is considered the mainstay of treatment and no adjuvant approach has demonstrated the ability to reduce the rate of relapses. We report on a case of a man with a 26-year clinical history of mediastinal follicular dendritic cell sarcoma, with several relapses after multiple surgical interventions. The impact of chemotherapy was very small, unlike the radiation therapy that was performed twice, with an interval time of 8 years, through an intensity-modulated technique and an altered fractionation schedule.
Subject(s)
Dendritic Cell Sarcoma, Follicular/pathology , Dendritic Cell Sarcoma, Follicular/radiotherapy , Mediastinum/pathology , Mediastinum/radiation effects , Adult , Humans , Male , Neoplasm Recurrence, Local/pathologySubject(s)
Codon, Nonsense , Dendritic Cell Sarcoma, Follicular/diagnosis , Dendritic Cell Sarcoma, Follicular/therapy , Mutation, Missense , Radiotherapy, Intensity-Modulated , Thyroid Nodule/diagnosis , Thyroid Nodule/therapy , Thyroidectomy , Adult , Biopsy, Fine-Needle , DNA Mutational Analysis , Dendritic Cell Sarcoma, Follicular/pathology , Dendritic Cell Sarcoma, Follicular/radiotherapy , Dendritic Cell Sarcoma, Follicular/surgery , Female , Gene Expression Profiling , Hashimoto Disease/complications , Humans , Lymphatic Metastasis , Neoplasm Grading , PTEN Phosphohydrolase/genetics , Proto-Oncogene Proteins c-ret/genetics , Radiotherapy, Adjuvant , Thyroid Nodule/pathology , Thyroid Nodule/radiotherapy , Thyroid Nodule/surgery , Tumor Suppressor Protein p53/geneticsABSTRACT
Follicular dendritic cell sarcomas are a recently described entity, with biphenotypic characteristics of lymphomas and sarcomas. The treatment is hardly consensual in the literature. We report two head and neck cases, of favorable outcome after surgery and radiotherapy. Histopathology and differential diagnoses are discussed as well as the therapeutic strategies used.