Follicular Dendritic Cell Sarcoma Mediastinum - a case report

Bushan, Kirti

doi:10.1007/s13193-014-0349-9

Cited by 7 publications

(7 citation statements)

References 15 publications

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“…Mediastinal origin is uncommon, and only 20 of 334 reported cases arose in the mediastinum [ 3 ]. According to the reports of mediastinal FDCS with specification of size, location, and imaging studies [ 2 – 18 ], many of the mediastinal FDCS cases developed in the subcarinal region as in our case [ 8 – 10 , 12 – 15 ]. Usually several lymph nodes reside in the subcarinal region, and these can be a site for development of FDCS.…”

Section: Case Presentationmentioning

confidence: 74%

“…The FDCS in our case most likely originated from a subcarinal lymph node although histological evidence could not be obtained to verify this. Some FDCSs develop in the anterior mediastinum [ 16 – 18 ] or para-tracheal region [ 11 ], which are usually approximately 10 cm in diameter and range in size from 2.5 to 13.4 cm [ 2 – 18 ]. Imaging studies by CT show that mediastinal FDCS are usually well defined and with heterogeneous soft tissue attenuation, accompanied by the areas of calcification [ 8 ].…”

Section: Case Presentationmentioning

confidence: 99%

“…FDG-PET/CT shows moderate or high uptake within the tumor, suggesting hypermetabolism of the FDCS [ 8 ]. In many reported cases of mediastinal FDCS, complete resection was possible and postoperative radiation therapy was frequently performed [ 2 – 11 , 13 – 18 ]. The prognosis of mediastinal FDCS is unknown because long-term outcome beyond 2 years has not been described in reported cases [ 2 – 18 ].…”

Section: Case Presentationmentioning

confidence: 99%

“…In many reported cases of mediastinal FDCS, complete resection was possible and postoperative radiation therapy was frequently performed [ 2 – 11 , 13 – 18 ]. The prognosis of mediastinal FDCS is unknown because long-term outcome beyond 2 years has not been described in reported cases [ 2 – 18 ]. However, good prognosis has been reported in complete resection cases and complete resection with postoperative irradiation cases as a whole [ 4 ] while poor prognosis of non-surgical cases (chemotherapy and / or radiotherapy) has been reported [ 2 , 4 , 12 ].…”

Section: Case Presentationmentioning

confidence: 99%

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Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case

2016

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Follicular dendritic cell sarcoma is a rare malignant neoplasm originating from follicular dendritic cells, and most of them develop in lymph nodes of the head and neck. One third of follicular dendritic cell sarcomas occur in the extranodal sites such as the tonsils, mesentery, and retroperitoneal organs, but those of mediastinal origin are rare. Here, we present the case of a 16-year-old female with a large follicular dendritic cell sarcoma of posterior mediastinal origin. The tumor was found by a chest X-ray mass examination at her high school, and she had no subjective symptoms or significant past medical history. The tumor was diagnosed as a follicular dendritic cell sarcoma by computed tomography-guided needle biopsy. Although the tumor compressed the mediastinal organs and showed moderate uptake in 18-fluorodeoxyglucose positron emission tomography imaging, it was completely resected through posterolateral incision. Histological examination revealed that spindle-shaped tumor cells formed fascicular or storiform pattern with cellular pleomorphism. By immunohistochemical examination, the tumor cells were found to be positive for CD21 and follicular dendritic cell antigen. Two years after surgery, the patient remains alive with no signs of tumor recurrence.

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Section: Case Presentationmentioning

confidence: 74%

Section: Case Presentationmentioning

confidence: 99%

Section: Case Presentationmentioning

confidence: 99%

Section: Case Presentationmentioning

confidence: 99%

See 2 more Smart Citations

Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case

2016

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“…Other regimens that were used include those that are used for lymphomas (ifosfamide, carboplatin and etoposide (ICE) [ 11 , 13 , 24 ], dexamethasone, cytarabine and cisplatin (DHAP) [ 9 , 13 ], etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin (EPOCH) [ 10 ], etoposide, methylprednisolone, cytarabine and cisplatin (ESHAP) [ 13 , 27 , 30 , 31 ], ifosfamide, gemcitabine, vinorelbine and prednisolone (IGEV) [ 19 ]) and soft tissue sarcomas (doxorubicin, ifosfamide and mesna (AIM) [ 14 , 20 , 21 , 31 ], ifosfamide and etoposide (IE) [ 13 ], gemcitabine and docetaxel (Gem/Doce) [ 13 , 22 , 23 , 32 ]). Notably, all three sarcoma regimens have been demonstrated to induce complete response, with the combination of gemcitabine and docetaxel resulting in complete response in the third-line setting in one case [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

Follicular Dendritic Cell Sarcoma of the Tonsil: A Multimodality Approach

et al. 2020

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Follicular dendritic cell sarcoma (FDCS) accounts for < 0.4% of soft tissue sarcomas. Only 35 cases of tonsillar FDCS have been reported, and majority had localized presentation. We present a case of FDCS of the tonsil, wherein a well-coordinated trimodality approach provided good disease control in advanced disease. A 53-year-old man presented with a painless and enlarging neck mass of 11-month duration, with no other symptoms. Close examination revealed a 10 × 5 cm mass at the left carotid triangle, and a 3.2 × 2.2 cm mass at the left tonsillar fossa. Imaging revealed the tumor to be unresectable due to its attachment to the great vessels. There were no distant metastases. Biopsy and immunohistochemistry were initially deemed consistent with an undifferentiated sarcoma. Palliative chemotherapy was given using single agent doxorubicin and subsequent dacarbazine, resulting in partial response and stable disease, respectively. Pathological re-evaluation was pursued because of the uncharacteristic slow progression of the tumor, revealing diffuse positivity for CD21 and negative for CD1A and CD34, consistent with FDCS. The patient underwent three cycles of gemcitabine plus docetaxel resulting in 50% regression. This allowed dissection of level IB-V lymph nodes and subsequent radiotherapy for the neck and tonsillar mass, with weekly gemcitabine as a radiosensitizer. Evaluation 8 months post-treatment showed no signs of disease progression. Treatment-related complications included radiation dermatitis and swallowing dysfunction, which both resolved on follow-up. This case highlights the multidisciplinary management of a rare type of sarcoma in an uncommon ana-tomic location. Precise pathologic diagnosis is important in soft tissue sarcoma because of its therapeutic implications. For FDCS, effective response may still be achieved in the third-line setting.

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Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease

et al. 2019

Diagn Pathol

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BackgroundMediastinal 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.MethodsThree 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.ResultsThe 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.ConclusionsMediastinal 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.

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Follicular Dendritic Cell Sarcoma Mediastinum - a case report

Cited by 7 publications

References 15 publications

Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case

Completely resected follicular dendritic cell sarcoma of the posterior mediastinum: report of a case

Follicular Dendritic Cell Sarcoma of the Tonsil: A Multimodality Approach

Mediastinal follicular dendritic cell sarcoma: a rare, potentially under-recognized, and often misdiagnosed disease

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