Abstract
Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm with a non-specific and insidious presentation further complicated by the difficult diagnostic and therapeutic assessment. It has a low to intermediate risk of recurrence and metastasis. Unlike other soft tissue sarcomas or histiocytic and dendritic cell neoplasms, cytogenetic studies are very limited in FDCS cases. Although no specific chromosomal marker has yet been established, complex aberrations and different ploidy types have been documented. We report the case of a 39-year-old woman with FDCS who presented to the Sultan Qaboos University Hospital in Muscat, Oman, in February 2013. Ultrastructural, immunophenotypical and histological findings are reported. In addition, karyotypic findings showed deletions of the chromosomes 1p, 3q, 6q, 7q, 8q and 11q. To the best of the authors' knowledge, these have not been reported previously in this tumour. Techniques such as spectral karyotyping may help to better characterise chromosomal abnormalities in this type of tumour.
Highlights
Case ReportA 39-year-old woman presented to the Sultan Qaboos University Hospital in Muscat, Oman, in February 2013 with a swelling on the right side of her neck
Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm with a non-specific and insidious presentation further complicated by the difficult diagnostic and therapeutic assessment
Unlike other soft tissue sarcomas or histiocytic and dendritic cell neoplasms, cytogenetic studies are very limited in FDCS cases
Summary
A 39-year-old woman presented to the Sultan Qaboos University Hospital in Muscat, Oman, in February 2013 with a swelling on the right side of her neck. This favoured a diagnosis of follicular dendritic cell sarcoma. As the range of chromosomes were in the hypertriploid category, a composite karyotype was interpreted as per the International System for Human Cytogenetic Nomenclature (2013).4 These were as follows: 72~80,XXXX,-1,del[1] (p32)x2,-3,del[3](q24),-4,del[6](q13)x2,-7,del[7](q11) x2,-8,-8,del[8](q22)x2,-9-9-9,del[11](q13),-12,-13,13,-14,-14,-14,add[16](q24)x2,-18,add[19](q13)x2,20,-21,-21,-21,-21,-22,-22,-22,+mar1,+mar2,+mar3,+ mar4,+mar5[19][cp11]/46,XX[1] [Figure 3] As the range of chromosomes were in the hypertriploid category, a composite karyotype was interpreted as per the International System for Human Cytogenetic Nomenclature (2013). These were as follows: 72~80,XXXX,-1,del[1] (p32)x2,-3,del[3](q24),-4,del[6](q13)x2,-7,del[7](q11) x2,-8,-8,del[8](q22)x2,-9-9-9,del[11](q13),-12,-13,13,-14,-14,-14,add[16](q24)x2,-18,add[19](q13)x2,20,-21,-21,-21,-21,-22,-22,-22,+mar1,+mar2,+mar3,+ mar4,+mar5[19][cp11]/46,XX[1] [Figure 3]
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