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

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.

Reduced angiogenic gene expression in morbillivirus‐triggered oncolysis in a translational model for histiocytic sarcoma

“…Histiocytic sarcoma (HS) represents such a rare disease in human beings, predominantly affecting intestine, skin and soft tissue or occurring systemically . The biological behaviour of HS and the underlying molecular mechanisms have been subject to detailed research infrequently, and an ideal treatment scheme is still lacking . Resembling the human counterpart in many aspects, the relatively higher prevalence of HS in dogs emphasizes the dog as an interesting translational animal model .…”

Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas

“…Histiocytic sarcoma is an extremely rare tumour of mature tissue histiocytes (non-Langerhans), with fewer than 40 cases reported in the literature [3,5,8–10]. HS typically presents in the gastrointestinal tract, skin, and soft tissues, but lymph node involvement has also been observed.…”

Characteristics, management, and outcomes of patients with follicular dendritic cell sarcoma

“…Management of FDCS is challenging for multiple reasons. First, it is a rare condition, comprising <0·4% of soft tissue sarcomas (Perkins & Shinohara, ), so data are limited, with information derived primarily from case reports and series (Perez‐Ordonez et al , ; Chan et al , ; Fonseca et al , ; Duan et al , ; Conry, ; Wang et al , ), pooled analyses (Saygin et al , ) and single centre experiences with small patient numbers (Soriano et al , ; Dalia et al , ; Gounder et al , ). Second, it is difficult to diagnose and may be confused with other neoplasms, such as lymphoma, sarcoma, meningioma and melanoma (Ohtake & Yamakawa, ; Pai et al , ).…”