Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease
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 u...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2019-01-01
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| Series: | Diagnostic Pathology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13000-019-0779-3 |
| _version_ | 1818963190047834112 |
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| author | You-Li Wu Feng Wu Cheng-Ping Xu Guo-Lei Chen Yu Zhang Wei Chen Xiao-Chu Yan Guang-Jie Duan |
| author_facet | You-Li Wu Feng Wu Cheng-Ping Xu Guo-Lei Chen Yu Zhang Wei Chen Xiao-Chu Yan Guang-Jie Duan |
| author_sort | You-Li Wu |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-20T12:41:16Z |
| format | Article |
| id | doaj.art-4cda11d7242343088b6338b61d37d260 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-20T12:41:16Z |
| publishDate | 2019-01-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-4cda11d7242343088b6338b61d37d2602022-12-21T19:40:28ZengBMCDiagnostic Pathology1746-15962019-01-0114111110.1186/s13000-019-0779-3Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed diseaseYou-Li Wu0Feng Wu1Cheng-Ping Xu2Guo-Lei Chen3Yu Zhang4Wei Chen5Xiao-Chu Yan6Guang-Jie Duan7Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University)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.http://link.springer.com/article/10.1186/s13000-019-0779-3Follicular dendritic cell sarcomaMediastinumRadiologyPathological diagnosisPrognosis |
| spellingShingle | You-Li Wu Feng Wu Cheng-Ping Xu Guo-Lei Chen Yu Zhang Wei Chen Xiao-Chu Yan Guang-Jie Duan Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease Diagnostic Pathology Follicular dendritic cell sarcoma Mediastinum Radiology Pathological diagnosis Prognosis |
| title | Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease |
| title_full | Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease |
| title_fullStr | Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease |
| title_full_unstemmed | Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease |
| title_short | Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease |
| title_sort | mediastinal follicular dendritic cell sarcoma a rare potentially under recognized and often misdiagnosed disease |
| topic | Follicular dendritic cell sarcoma Mediastinum Radiology Pathological diagnosis Prognosis |
| url | http://link.springer.com/article/10.1186/s13000-019-0779-3 |
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