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Follicular dendritic cell sarcoma: a novel orbital tumour

Published:December 04, 2020DOI:https://doi.org/10.1016/j.jcjo.2020.11.009
      Follicular dendritic cell sarcoma is a rare neoplasm of lymphoid cells predominantly found in lymph nodes and rarely in extranodal locations. We report the first case of follicular dendritic cell sarcoma occurring in the orbit.
      A 74-year-old previously healthy man presented with blurred near vision. His best-corrected visual acuity was 20/50 right eye and 20/20 left eye. A 2+ right afferent pupillary defect was present. The right upper eyelid was slightly ptotic, and the right globe had mild lateral chemosis with 3 mm proptosis. Ocular ductions were limited in right eye elevation. Fundoscopic examination revealed normal optic nerves. Automated visual fields demonstrated an inferior paracentral scotoma in the right eye (Supplementary Fig. 1, available online). Clinical differential diagnosis included cavernous hemangioma or a cystic schwannoma.
      During surgical resection, the mass was adherent to the inferior rectus muscle and fixed to the orbital apex, precluding complete resection. Postoperatively, vision improved to 20/20, but the patient experienced intermittent diplopia.
      Microscopically, the neoplasm was characterized by a proliferation of atypical elongated cells with eosinophilic cytoplasm, in a syncytial pattern, intermixed with small lymphocytes and arranged in nodules within fibrous tissue (Fig. 1). Focal whorling pattern, cystic degeneration, and rare mitotic figures were present (Fig. 1). The tumour cells were strongly positive for CD21 and CD35 immunostains (Fig. 1), and negative for EMA, STAT6, keratin, desmin, and S100. BRAF mutation analysis and Fluorescence In Situ Hybridization analysis for FUS (16p11.2) gene rearrangement were negative.
      Fig 1
      Fig. 1Follicular dendritic cell sarcoma. On hematoxylin-eosin-stained sections, lower power shows areas of cystic degeneration, hemorrhage, and hematoidin and hemosiderin pigment deposits (A). Medium- and high-power views demonstrate a proliferation of atypical cells with eosinophilic cytoplasm, poorly defined cell borders in a syncytial pattern, elongated nuclei with finely granular chromatin, and small nucleoli (B, C). Focally, whorling pattern (C) and interspersed small lymphocytes were observed (D). The tumour was strongly positive for CD21 (E) and CD35 immunostains (F).
      Follicular dendritic cell sarcoma (FDCS) is an exceedingly rare neoplasm (142 extranodal cases reported)
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      composed of spindled and oval-shaped cells that exhibit morphologic and immunophenotypic features of follicular dendritic cells, which are normal constituents of primary and secondary B-cell follicles.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      These cells are nonmigrating and form a stable meshwork within the follicle but also exist in nonlymphoid organs.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      Thus, neoplasms arising from cells of dendritic origin can be found both nodally and extranodally.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      • Mograbi M
      • Stump MS
      • Luyimbazi DT
      • Shakhatreh MH
      • Grider DJ
      Pancreatic inflammatory pseudotumor-like follicular dendritic cell tumor.
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      Patients typically present with a slow-growing, painless mass. Cervical lymph nodes are the most commonly affected.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      • Mograbi M
      • Stump MS
      • Luyimbazi DT
      • Shakhatreh MH
      • Grider DJ
      Pancreatic inflammatory pseudotumor-like follicular dendritic cell tumor.
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      Extranodal locations include the oral cavity, gastrointestinal tract, and soft tissues, but, to our knowledge, have never included the orbit.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      Systemic symptoms are uncommon but seen with the inflammatory pseudotumour-like variant, commonly found in the liver and spleen.
      • Mograbi M
      • Stump MS
      • Luyimbazi DT
      • Shakhatreh MH
      • Grider DJ
      Pancreatic inflammatory pseudotumor-like follicular dendritic cell tumor.
      The age of presentation is predominantly in adulthood with a mean age of 44 years.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      No sex predilection exists.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      Morphologically, FDCS is characterized by a proliferation of spindled to oval-shaped cells that form fascicles, storiform arrays, whorls, diffuse sheets, and vague nodules as observed in our case.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      ,
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      ,
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      Nuclear pseudo-inclusions, binucleation, and multinucleation have also been documented.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      ,
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      ,
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      Mitotic activity and levels of necrosis can vary.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      The tumour is typically infiltrated by small lymphocytes.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      ,
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      In the orbit, the differential diagnosis includes meningioma, solitary fibrous tumour, and other spindle cell sarcomas.
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      Given the wide differential, immunohistochemical proof of follicular dendritic cell differentiation is imperative.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      FDCSs express follicular dendritic cell markers CD21, CD35, and CD23.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      • Mograbi M
      • Stump MS
      • Luyimbazi DT
      • Shakhatreh MH
      • Grider DJ
      Pancreatic inflammatory pseudotumor-like follicular dendritic cell tumor.
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      FDCS cells are also positive for vimentin, clusterin, fascin, EGFR, and HLA-DR.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      ,
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      FDCS cells express variable positivity for EMA, S100, and CD68.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      ,
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      Rarely CD45 or CD20 can be expressed.
      • Mograbi M
      • Stump MS
      • Luyimbazi DT
      • Shakhatreh MH
      • Grider DJ
      Pancreatic inflammatory pseudotumor-like follicular dendritic cell tumor.
      ,
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      However, CD1a, lysozyme, myeloperoxidase, CD34, CD3, CD79a, and HMB45 are consistently negative.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      ,
      • Ohtake H
      • Yamakawa M
      Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
      The intermixed lymphocytes tend to be more commonly T cells than B cells.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      The behaviour of FDCS is usually indolent, as observed in low- or intermediate-grade soft tissue sarcomas.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      ,
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      Local recurrences occur in about 50% of the cases, with possible metastases to the lymph nodes, lung, and liver.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      ,
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      In some cases recurrence occurs many years after the diagnosis.
      • Wang R-F
      • Han W
      • Qi L
      • Shan L-H
      • Wang Z-C
      • Wang L-F
      Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
      • Amirtham U
      • Manohar V
      • Kamath MP
      • et al.
      Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
      • Chen T
      • Gopal P
      Follicular dendritic cell sarcoma.
      In conclusion, we report the first case of follicular dendritic cell sarcoma arising in the orbit. Although exceedingly rare, clinicians and pathologists should remain cognizant of this uncommon orbital malignancy.

      Footnotes and Disclosure

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      Appendix. Supplementary materials

      References

        • Wang R-F
        • Han W
        • Qi L
        • Shan L-H
        • Wang Z-C
        • Wang L-F
        Extranodal follicular dendritic cell sarcoma: a clinicopathological report of four cases and a literature review.
        Oncol Lett. 2015; 9: 391-398
        • Amirtham U
        • Manohar V
        • Kamath MP
        • et al.
        Clinicopathological profile and outcomes of follicular dendritic cell sarcoma of the head and neck region - a study of 10 cases with literature review.
        J Clin Diagn Res. 2016; 10: XC08-XC11
        • Chen T
        • Gopal P
        Follicular dendritic cell sarcoma.
        Arch Pathol Lab Med. 2017; 141: 596-599
        • Mograbi M
        • Stump MS
        • Luyimbazi DT
        • Shakhatreh MH
        • Grider DJ
        Pancreatic inflammatory pseudotumor-like follicular dendritic cell tumor.
        Case Rep Pathol. 2019; 2019: 1-5
        • Ohtake H
        • Yamakawa M
        Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.
        J Clin Exp Hematop. 2013; 53: 179-184