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Cancers 2014, 6(4), 2275-2295; doi:10.3390/cancers6042275

Clinicopathologic Characteristics and Outcomes of Histiocytic and Dendritic Cell Neoplasms: The Moffitt Cancer Center Experience Over the Last Twenty Five Years

1
Mercy Clinic Oncology and Hematology-Joplin, 3001 MC Clelland Park Blvd, Joplin, MO 64804, USA
2
Department of Malignant Hematology, H. Lee Moffitt Cancer Center, 12902 Magnolia Drive, Tampa, FL 33602, USA
3
IHCFLOW Histopathology Laboratory, University of South Florida, 18804 Chaville Rd., Lutz, FL 33558, USA
*
Author to whom correspondence should be addressed.
Received: 18 June 2014 / Revised: 25 October 2014 / Accepted: 27 October 2014 / Published: 14 November 2014
(This article belongs to the Special Issue Lymphoma)
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Abstract

Neoplasms of histiocytic and dendritic cells are rare disorders of the lymph node and soft tissues. Because of this rarity, the corresponding biology, prognosis and terminologies are still being better defined and hence historically, these disorders pose clinical and diagnostic challenges. These disorders include Langerhans cell histiocytosis (LCH), histiocytic sarcoma (HS), follicular dendritic cell sarcoma (FDCS), interdigtating cell sarcoma (IDCS), indeterminate cell sarcoma (INDCS), and fibroblastic reticular cell tumors (FRCT). In order to gain a better understanding of the biology, diagnosis, and treatment in these rare disorders we reviewed our cases of these neoplasms over the last twenty five years and the pertinent literature in each of these rare neoplasms. Cases of histiocytic and dendritic cell neoplasms diagnosed between 1989–2014 were identified using our institutional database. Thirty two cases were included in this analysis and were comprised of the following: Langerhans cell histiocytosis (20/32), histiocytic sarcoma (6/32), follicular dendritic cell sarcoma (2/32), interdigitating dendritic cell sarcoma (2/32), indeterminate dendritic cell sarcoma (1/32), and fibroblastic reticular cell tumor (1/32). Median overall survival was not reached in cases with LCH and showed 52 months in cases with HS, 12 months in cases with FDCS, 58 months in cases with IDCS, 13 months in the case of INDCS, and 51 months in the case of FRCT. The majority of patients had surgical resection as initial treatment (n = 18). Five patients had recurrent disease. We conclude that histiocytic and dendritic cell neoplasms are very rare and perplexing disorders that should be diagnosed with a combination of judicious morphology review and a battery of immunohistochemistry to rule out mimics such as carcinoma, lymphoma, neuroendocrine tumors and to better sub-classify these difficult to diagnose lesions. The mainstay of treatment for localized disease remains surgical resection and the role of adjuvant therapy is unclear. In patients with multiple areas of involvement, treatment at tertiary care centers with multimodality treatment is likely needed. Accurate subset diagnosis will contribute to better data as well as treatment outcomes analysis of these rare disorders of adult patients in the future. View Full-Text
Keywords: dendritic cell tumor; Langerhans cell histiocytosis; histiocytic sarcoma; follicular dendritic cell sarcoma; interdigitating dendritic cell sarcoma; indeterminate dendritic cell sarcoma; fibroblastic reticular cell tumor dendritic cell tumor; Langerhans cell histiocytosis; histiocytic sarcoma; follicular dendritic cell sarcoma; interdigitating dendritic cell sarcoma; indeterminate dendritic cell sarcoma; fibroblastic reticular cell tumor
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Dalia, S.; Jaglal, M.; Chervenick, P.; Cualing, H.; Sokol, L. Clinicopathologic Characteristics and Outcomes of Histiocytic and Dendritic Cell Neoplasms: The Moffitt Cancer Center Experience Over the Last Twenty Five Years. Cancers 2014, 6, 2275-2295.

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