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Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease
- Source :
- Diagnostic Pathology, Diagnostic Pathology, Vol 14, Iss 1, Pp 1-11 (2019)
- Publication Year :
- 2019
- Publisher :
- BioMed Central, 2019.
-
Abstract
- Background Mediastinal follicular dendritic cell sarcoma (FDCS) is extremely rare. Due to potential under-recognization of this disease, it happens to be misdiagnosed, especially on core needle biopsy. We report 3 cases of mediastinal FDCS and provide a literature review to improve better understanding of the tumor and to reduce misdiagnosis. Methods Three cases of mediastinal FDCS in our clinic practice were studied, including their core needle biopsy and resected specimens, and those cases reported previously in English literature were retrieved and analyzed. Results The core needle biopsy of case 1 showed a tumor reminiscent of classical Hodgkin’s lymphoma (CHL), while the resected mass was finally diagnosed with FDCS combined with hyaline-vascular Castleman’s disease. Both the biopsy and resected tissue of case 2 were constitutive of the clear epithelioid cells with marked atypia. In both cases, definitive diagnoses were not made on core needle biopsy. In case 3, there were some areas morphologically similar to CHL, and some areas contained ovoid to spindle-shaped tumor cells with fascicular pattern. The analysis of 43 cases of mediastinal FDCS showed the age of patients were from 16 to 76 years old, the male to female ratio was 1.5:1, the maximal tumor diameters were 3–17 cm. 18 cases were underwent preoperative biopsy, whereas 15 (83.3%) of which were misdiagnosed initially, often as lymphoma. 32 patients had available follow-up data, the rates of recurrence, metastasis, and mortality were 12.5, 18.8 and 28.1%, respectively. Current limited data suggested no statistical differences between adverse prognosis and gender, age, tumor size, necrosis, or different therapeutics, respectively. Conclusions Mediastinal FDCS is a rare malignancy that has yet not been fully understood and been often misdiagnosed, particularly when making a diagnosis on core needle biopsy. Increased awareness of this enigmatic tumor is crucial to avoid diagnostic pitfalls.
- Subjects :
- 0301 basic medicine
Adult
Male
Pathology
medicine.medical_specialty
Histology
Adolescent
Dendritic Cell Sarcoma, Follicular
Malignancy
Pathology and Forensic Medicine
Metastasis
03 medical and health sciences
Pathological diagnosis
Young Adult
0302 clinical medicine
Drug Therapy
Biopsy
lcsh:Pathology
medicine
Atypia
Humans
Aged
medicine.diagnostic_test
Radiotherapy
business.industry
Research
Mediastinum
General Medicine
Middle Aged
medicine.disease
Prognosis
Lymphoma
030104 developmental biology
medicine.anatomical_structure
030220 oncology & carcinogenesis
Follicular dendritic cell sarcoma
Female
Biopsy, Large-Core Needle
business
Radiology
Tomography, X-Ray Computed
Epithelioid cell
lcsh:RB1-214
Subjects
Details
- Language :
- English
- ISSN :
- 17461596
- Volume :
- 14
- Database :
- OpenAIRE
- Journal :
- Diagnostic Pathology
- Accession number :
- edsair.doi.dedup.....114c81bfccb3d3cd4c387038a66f50ee