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DOI:10.2214/AJR.04.1530
AJR 2007; 188:W554-W556
© American Roentgen Ray Society

Case Report

Follicular Dendritic Cell Sarcoma of the Mediastinum

Jonathon A. Leipsic1, H. Page McAdams1 and Thomas A. Sporn2

1 Department of Radiology, Duke University Medical Center, PO Box 3808, Durham, NC 27710.
2 Department of Pathology, Duke University Medical Center, Durham, NC.

Received September 28, 2004; accepted after revision July 10, 2005.

 
Address correspondence to J. A. Leipsic.

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Keywords: cardiopulmonary imaging • Castleman disease • chest imaging • follicular dendritic cell sarcoma • oncologic imaging


Introduction
Top
 Introduction
Case Report
 Discussion
 Summary
 References
 
Follicular dendritic cell sarcoma is a rare neoplasm originating from follicular dendritic cells, which are nonlymphoid, nonphagocytic accessory cells of the lymphoid system [1]. The existence of a primary neoplasm of the follicular dendritic cell was first described in 1986, and subsequently, specific immunohistochemical markers have been identified [2]. Since that time, scattered cases have been reported in the pathology literature. However, to our knowledge, the radiologic findings of this lesion have yet to be described. In this report, we present the radiographic and CT findings of follicular dendritic cell sarcoma of the mediastinum.


Case Report
Top
 Introduction
 Case Report
Discussion
 Summary
 References
 
A 43-year-old man presented to the emergency department with complaints of intermittent chest pain. There was a vague history of trauma but this was relatively remote and no other contributory history was elicited. The results of an initial cardiac workup were negative. A chest radiograph showed a large, right-sided, lobulated, retrocardiac mass (Figs. 1A and 1B). There was no radiographic evidence of calcification.


Figure 1
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  		Fig. 1A —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Posteroanterior (A) and lateral (B) chest radiographs show large, lobulated retrocardiac mass (arrows) on right. Note absence of calcification.

 

Figure 2
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  		Fig. 1B —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Posteroanterior (A) and lateral (B) chest radiographs show large, lobulated retrocardiac mass (arrows) on right. Note absence of calcification.

 
CT (LightSpeed, GE Healthcare) was performed using a 5-mm beam collimation reconstructed at 5-mm increments; no IV contrast material was administered. CT showed that the mass was large; was of heterogeneous soft-tissue attenuation; and contained multiple foci of irregular, coarse, and ringlike calcification (Figs. 1C, 1D, 1E, 1F). The mass abutted and displaced the esophagus and extended from the level of the carina to the esophageal hiatus. There was also apparent compression of the inferior vena cava and the posterior aspect of the left atrium.


Figure 3
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  		Fig. 1C —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Sequential unenhanced CT images (mediastinal window settings) show that mass is lobulated in contour, is heterogeneous and of soft-tissue attenuation, and contains foci of rimlike and coarse calcification (arrowheads, D and F). Note also mass effect on left atrium (La, C), lateral displacement of esophagus (arrow, D), and small right pleural effusion (arrow, E).

 

Figure 4
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  		Fig. 1D —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Sequential unenhanced CT images (mediastinal window settings) show that mass is lobulated in contour, is heterogeneous and of soft-tissue attenuation, and contains foci of rimlike and coarse calcification (arrowheads, D and F). Note also mass effect on left atrium (La, C), lateral displacement of esophagus (arrow, D), and small right pleural effusion (arrow, E).

 

Figure 5
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  		Fig. 1E —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Sequential unenhanced CT images (mediastinal window settings) show that mass is lobulated in contour, is heterogeneous and of soft-tissue attenuation, and contains foci of rimlike and coarse calcification (arrowheads, D and F). Note also mass effect on left atrium (La, C), lateral displacement of esophagus (arrow, D), and small right pleural effusion (arrow, E).

 

Figure 6
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  		Fig. 1F —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Sequential unenhanced CT images (mediastinal window settings) show that mass is lobulated in contour, is heterogeneous and of soft-tissue attenuation, and contains foci of rimlike and coarse calcification (arrowheads, D and F). Note also mass effect on left atrium (La, C), lateral displacement of esophagus (arrow, D), and small right pleural effusion (arrow, E).

 
At surgery, a nodular tan–white mass measuring 13 x 10 x 6 cm was identified in the middle mediastinum. The mass was closely adherent to adjacent pericardium and esophagus, but there was no evidence of either tissue or vascular invasion. The mass was resected in toto. Biopsies of adjacent enlarged levels 7, 8, and 9 lymph nodes were also performed. Histopathologic assessment of resected specimens (mass and lymph nodes) showed mature-appearing lymphocytes and spindle cells arranged in a haphazard fashion (Fig. 1G). Mitotic figures were not identified. Immunohistochemical staining showed cells that were strongly positive for CD21 (Fig. 1H) and CD35 while being negative for most other markers. These findings were considered diagnostic of follicular dendritic cell sarcoma.


Figure 7
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  		Fig. 1G —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Photomicrograph shows cytologically bland spindle cells arranged in haphazard fashion, which is consistent with follicular dendritic cell sarcoma. Note absence of mitotic figures. (H and E stain, original magnification, x200)

 

Figure 8
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  		Fig. 1H —Follicular dendritic cell sarcoma in 43-year-old man with chest pain. Photomicrograph shows marked immunohistochemical staining for CD21 marker. (CD21 stain, original magnification, x200)

 

Discussion
Top
 Introduction
 Case Report
 Discussion
Summary
 References
 
Follicular dendritic cell sarcoma is a rare neoplasm arising from primary and secondary lymphoid follicles. These cells normally play an important role in the immune response and act as antigen-presenting cells for the B-cell compartment [3]. These tumors have distinct immunohistochemical features, with diagnosis relying on reactivity to CD21 and CD35 markers [4]. These neoplasms generally affect young to middle-aged adults, with a mean age of 43 years [5]. There does not appear to be a sex predilection, and they usually present as a solitary mass or as painless, slowly growing cervical lymphadenopathy [2]. The most common presentation is a coalescent nodal mass, but extranodal disease is most commonly seen in the head and neck. Follicular dendritic cell sarcomas have also been known to occur in other locations, such as the axilla, and extranodal sites, such as the gastrointestinal tract, liver, oral cavity, and spleen [5]. A summary of the clinical and histopathologic features of 47 previously reported cases is shown in Table 1.


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  		TABLE 1: Clinical and Histopathologic Features of 47 Previously Reported Cases of Follicular Dendritic Cell Sarcoma

 

The radiologic findings of follicular dendritic cell sarcoma have not been previously described to our knowledge in the radiology literature. Reports in the clinical and pathology literature suggest that these lesions are variable in size, ranging from 1 to 20 cm in diameter, and that they are usually well circumscribed. The largest reported mediastinal tumor measured 13 cm [5]. Hemorrhage or necrosis is an uncommon finding at histopathologic examination. A subset of these tumors has been reported to arise in or concomitantly with foci of Castleman disease (not noted in our patient) [6]. In fact, some argue that follicular dendritic cell sarcoma actually develops from Castleman disease as a hyperplasia–dysplasia–neoplasia sequence [7]. It is thus possible that follicular dendritic cell sarcoma may share some of the radiologic features of Castleman disease. In our patient, we saw a large lobulated soft-tissue-attenuation mass with foci of coarse and chunklike calcification on CT, features typical of Castleman disease [8]. Further, although there was evidence of significant mass effect on adjacent mediastinal structures, no invasion was seen either at CT or at surgery. Because IV contrast material was not administered, we cannot comment on the vascularity of the lesion. At surgery, however, it was not reported to be particularly hypervascular, unlike the more common hyaline–vascular variant of Castleman disease.

The differential diagnosis for the CT findings evident in our case includes lymphoma, small cell lung carcinoma, Castleman disease, and metastatic lymphadenopathy. A potential differentiating feature in our case may be the relative paucity of necrosis and of hemorrhage in the setting of a large tumor mass. The presence of chunklike or coarse calcification may also help distinguish follicular dendritic cell sarcoma from more common causes of bulky mediastinal masses such as untreated lymphoma or small cell lung carcinoma.

As its name implies, follicular dendritic cell sarcomas generally behave more like low-grade sarcomas than lymphomas [9]. Complete surgical resection is considered the treatment of choice; the role of adjuvant therapy (chemotherapy or radiation) is unclear because of the rarity of the lesion and the retrospective nature of published reports. The prognosis of patients with follicular dendritic cell sarcoma is variable. Poor prognostic factors are reported to include extensive necrosis, a mass larger than 6 cm, cytologic atypia, intraabdominal location, and a high proliferative index suggesting high mitotic activity [10]. Lin and Frizzera [6] reviewed 47 reported cases and found that 17 developed local recurrence after resection and that 13 developed metastatic disease.


Summary
Top
 Introduction
 Case Report
 Discussion
 Summary
References
 
In this case report, we describe the radio-logic manifestations of an uncommon mediastinal tumor. Follicular dendritic cell sarcoma, although unusual, should be considered in the differential diagnosis of bulky mediastinal masses arising from lymphoid tissue, especially in the presence of calcification without prior treatment.


References
Top
 Introduction
 Case Report
 Discussion
 Summary
 References
 

  1. Pileri SA, Grogan TM, Harris NL, et al. Tumors of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology 2002;41 : 1–29[Medline]
  2. Monda L, Warnke R, Rosai J. A primary lymph node malignancy with features suggestive of dendritic reticulum cell differentiation. Am J Pathol 1986; 122:562 –572[Abstract]
  3. Ceresoli G, Zucchinelli P, Ponzoni M, Gregorc V, Bencardino K, Paties CT. Mediastinal follicular dendritic cell sarcoma. Haematologica 2003;88 : ECR04[Free Full Text]
  4. Perez-Ordonez B, Rosai J. Follicular dendritic cell tumor: review of the entity. Semin Diagn Pathol 1998;15 : 144–154[Medline]
  5. Perz-Ordonez B, Erlandson RA, Tosai J. Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity. Am J Surg Pathol 1996; 20:944 –955[CrossRef][Medline]
  6. Lin O, Frizzera G. Angiomyoid and follicular dendritic cell proliferative lesions in Castleman's disease of hyaline-vascular type: a study of 10 cases. Am J Surg Pathol 1997;21 :1295 –1306[CrossRef][Medline]
  7. Chang JKC, Tsang WYW, Ng CS. Follicular dendritic cell tumor and vascular neoplasm complicating hyaline vascular Castleman disease. Am J Surg Pathol 1994;18 : 517–525[Medline]
  8. McAdams HP, Rosado-de-Christenson M, Fish-back NF, Templeton PA. Castleman disease of the thorax: radiologic features with clinical and histopathologic correlation. Radiology1998; 209:221 –228[Abstract/Free Full Text]
  9. Fonseca R, Tefferi A, Strickler JG. Follicular dendritic cell sarcoma mimicking diffuse large cell lymphoma: a case report. Am J Hematol 1997; 55:148 –155[CrossRef][Medline]
  10. Weiss LM, Grogan TM, Muller-Hermelink H-K, et al. Follicular dendritic cell sarcoma. In: Kleihues P, Cavanee WK, eds. World Health Organization classification of tumors. Lyon, France: IARC Press, 2001: 286

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