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Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 316-322
Histiocytic and follicular dendritic cell sarcoma: Diagnostically challenging rare entities

Department of Oncopathology, The Gujarat Cancer and Research Institute, New Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India

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Date of Submission29-Apr-2020
Date of Decision29-May-2020
Date of Acceptance28-Oct-2020
Date of Web Publication9-Apr-2021


Background: Follicular dendritic cell sarcomas (FDCSs) and histiocytic sarcomas (HSs) are exceedingly rare tumors. Most of the data on those entities are based on case reports or small case series. The natural history and response to different treatment modalities have not been well established. Aims: To analyze the clinicopathologic features, immunophenotypic profile, treatment responses and to add to the existing data on FDCS and HS. Study Design: Retrospective descriptive study. Materials and method: The study was conducted at the department of Oncopathology at a tertiary care cancer hospital in India, retrospectively within the time period of four years (2016-2019). Total eight (8) cases were diagnosed: four cases of FDCS and four cases of HS involving nodal and extra-nodal sites. Clinical, histopathological, immunohistochemistry (IHC) and therapeutic data of the eight cases were retrieved and analyzed. Statistics: Descriptive statistics. Result: Among the four patients of FDCS, two had nodal and two had extra-nodal disease. Mean tumor size was 6 cm. Tumor cells expressed CD23, CD21, CD45, CD68 and S100. One patient received adjuvant chemotherapy (Gemcitabine and Docetaxel). Median survival was 36 months. None of them developed distant metastasis. Two of the patients having HS, developed bone metastasis. Median survival was 8.5 months. CD68 was consistently expressed in all cases of HS. Other applied IHC markers were negative in all the eight cases. Conclusion: FDCS and HS are under-recognized and easily prone to a wrong diagnosis. Therefore, considering these rare entities in differential diagnoses and inclusion of proper IHC biomarkers are necessary to avoid potential misdiagnosis.

Keywords: Dendritic cell sarcoma, extra-nodal, histiocytic sarcoma, immunohistochemistry

How to cite this article:
Patra S, Trivedi P, Kaur K. Histiocytic and follicular dendritic cell sarcoma: Diagnostically challenging rare entities. Indian J Pathol Microbiol 2021;64:316-22

How to cite this URL:
Patra S, Trivedi P, Kaur K. Histiocytic and follicular dendritic cell sarcoma: Diagnostically challenging rare entities. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 16];64:316-22. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/316/313296

   Introduction Top

Follicular dendritic cell sarcoma (FDCS) and histiocytic sarcoma (HS), two exceedingly rare entities are grouped with the Histiocytic and dendritic cell neoplasm by the World Health Organization classification of tumors and account for less than one percent (<1%) of tumors presenting in the lymph nodes or soft tissue.[1] In 1986, Monda et al. originally described FDCS.[2] Most of the data on FDCS and HS are based on case reports or small case series. The natural history and response to different treatment modalities have not been well established. Therefore, to add to the existing data on FDCS and HS for better understanding of the disease, we analyzed clinicopathologic features, immunophenotypic profile, treatment responses and outcome with a review of the literature on these rare entities.

   Materials and Method Top

The study was conducted at the department of OncoPathology at a tertiary care cancer hospital in India, retrospectively within 4 years (2016-2019). Total eight cases were diagnosed in this time, four cases with FDCS and four cases with HS, involving different parts of body. Clinical, histopathological, immunohistochemistry (IHC) and therapeutic data of the eight cases were retrieved from the pathology archives and analyzed. Descriptive statistics was used.


Immunohistochemical localization was performed in all the cases on formalin fixed paraffin embedded tissue by fully automated machine (Ventana Benchmark XT) using monoclonal antibodies to Myeloperoxidase (MPO) (Thermo-1:50, Ab1), CD117 (Cellmarque-1:100, YR145), CD68 (Cellmarque-1:50 PG-M1), CD34 (DAKO-1:50, QBEnd10), CD45 (Thermo-1:100, RA/RO), CD43 (DAKO-1:50, DF-T1), CD99 (Thermo-1:50, H036-1.1), Terminal deoxynucleotidyl transferase (TdT) (BioGenex-1:50, EP266), Vimentin (BioGenex-1:200, B9), Desmin (BioGenex-1:30, D33), Friend Leukemia Integration-1(FLI-1) (Cellmarque-1:75, MRQ-1), Multiple Myeloma1 (MUM1) (Cellmarque-1:100, MRQ43), CD2 (Cellmarque-1:100, MRQ11), CD3 (DAKO-1:50 F7.2.38), CD10 (Ventana-RTU, SP67), CD23 (Cell marque-1:50, MRQ12), CD21 (Cell marque-1:50, MRQ32), CD20 (Cellmarque-1:100, L26), CD79a (Cellmarque-1:100, JCB117), CD1a (BioGenex-1:30, O10), Epithelial Membrane Antigen (EMA) (Cellmarque-1:100, E29), Anion Exchanger1/3(AE1/AE3) (Thermo-1:100, AE1/AE3), Inhibin alpha (Cellmarque-1:100, R1), CD19 (Cellmarque-1:50, MRQ36), Synaptophysin (Thermo-1:50, SP11), Chromogranin A (Cellmarque-1:100, LKH110), Placental alkaline phosphatase (PLAP) (BioGenex-1:30, PL8-F6) and Prostate specific antigen (PSA) (BioGenex-1:30, ErPr-8), p40 (Ventana-RTU, BC28), Human Melanoma Black-45 (HMB 45) (Cellmarque-1:50, HMB45), Anaplastic lymphoma kinase -1 (ALK-1) (Thermo-1:50, 5A4), and Calretinin (BioGenex-1:30, 2E7).

   Results Top

A total of eight cases were diagnosed within 4 years (2016-2019). Out of the eight cases, four patients had FDCS and another four had HS. Total number of surgical specimens, soft tissue sarcomas and lymphomas examined in our department in the above-said period were 1, 19, 324; 2216 and 2136, respectively. Hence, FDCS and HS, each constituted 0.18% of total soft tissue sarcomas and 0.19% of total lymphoma cases.


Among the four patients having FDCS, a male and female ratio was 1:3 with median age of 57 years. Two of the patients presented with levels I and II cervical lymphadenopathy and another two had extra-nodal disease [Table 1]. Clinically and radiologically all had localized disease.
Table 1: Demographic data, clinical and therapeutic parameters of patients

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Grossly, FDCSs were well-circumscribed pink or tan-gray nodular solid masses with a mean tumor size of 6 cm. The tumor cells were elongated with oval to spindle nuclei, vesicular chromatin with small nucleoli, and pale-eosinophilic cytoplasm with indistinct cell borders [Figure 1]a and [Figure 1]b. Tumors cells were arranged in fascicles and storiform patterns. The background showed variably vascularized fibro-collagenous stroma with sprinkling of small mature lymphocytes. Plasma cells and eosinophils were also evident in one of the tumors. Pleomorphism, mitosis and necrosis were recorded [Table 2]. Vimentin (4/4) and CD23 (4/4) were strong positive [Figure 1]c. CD21 (1/4, Weak), CD45 (2/4), CD43 (2/4), CD68 (2/4), S100 (3/4, Focal) and Mib 1 [Figure 1]d,[Figure 1]e,[Figure 1]f were also expressed in variable proportions [Table 3]. Focal TdT positive immature T lymphoblastic cells were identified in two cases [Case No. 3 and 4, [Table 1]] [Figure 1]g,[Figure 1]h,[Figure 1]i. All other applied epithelial, mesenchymal, lymphoid, neuroendocrine and germ cell markers were negative [Table 2].
Figure 1: Follicular dendritic cell sarcoma (a) A low-power view shows fascicles and whorls of spindle cells in fibro-collagenized stroma (H&E, 10×); (b) The high power view shows oval to spindle tumor cell nuclei with small nucleoli, indistinct cell borders, and a sprinkling of small lymphocytes (H&E, 40x); (c) Staining with antibody to CD23 (IHC, 40×); (d) antibody to CD45 (IHC,10×); (e) antibody to S-100 (IHC,40×); (f) MIB-1 index (IHC, 40×); (g-i) TdT reactivity by immature T-lymphoblastic cells in FDCS (IHC, 40×); H and E - Hematoxylin and eosin; IHC - Immunohistochemistry

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Table 2: Histopathology and IHC findings in FDCS

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Surgery was done in all the four cases. One patient received three cycles of adjuvant chemotherapy (CT) with gemcitabine and docetaxel after surgery. Another one patient received radiotherapy (RT) after surgical intervention. Median survival of the three patients was 36 months after diagnosis. None of them had developed distant site metastasis [Table 1]. One patient was lost to follow up after 1 month of surgery.


The mean age of the patients having HS was 43.5 years with a male-female ratio of 3:1. Three patients presented with localized nodal disease, cervical (two cases) and inguinal lymph node (one case) involvement. One patient had extra-nodal presentation with involvement of the spleen.

Typical histology of HS was a diffuse growth pattern composed of large polygonal cells. The cells were predominantly arranged in non-cohesive sheets. The neoplastic cells were large, with moderate to abundant eosinophilic cytoplasm, round to irregular nuclei with vesicular chromatin, and one to multiple distinct nucleoli [Figure 2]a,[Figure 2]b,[Figure 2]c. Moderate to marked pleomorphism was evident in two cases. Mitotic activity was variable [Table 4]. Coagulative tumor cell necrosis was noted in three cases [Figure 2]a. Infiltrating neutrophils, lymphocytes, and occasional eosinophils were present in the background. Neoplastic cells strongly expressed Vimentin (4/4), CD68 (4/4) [Figure 2]d, CD43 (4/4) and CD45 (3/4) [Table 3]. S100 was expressed focally in two cases [Figure 2]e. Mib-1 index was variable (20%-50%) [Figure 2]f. All other applied IHC markers, considering differential diagnoses, were completely negative in tumor cells[Table 4]. Apart from the conventional type histology, other morphological variations were found in HS: intranuclear cytoplasmic inclusions [Figure 2]c, cytoplasmic vacuolation or foamy appearance [Figure 3]a, neoplastic spindle cells [Figure 3]b, hemophagocytosis with erythrophagocytosis [Figure 3]c and tumor cells containing dusky brown hemosiderin pigment that could be easily misdiagnosed as melanin [Figure 3]d.
Figure 2: Histiocytic sarcoma (a) Low-power view showing sheets of tumor cells with an adjacent area of coagulative tumor necrosis (H&E, 10×); (b)High-power view showing large polygonal tumor cells having pleomorphic hyperchromatic nuclei and abundant eosinophilic granular to slightly bubbly cytoplasm (H&E, 40×); (c) Intranuclear pseudo-inclusion in a tumor cell (H&E,40x); (d) Staining with antibody to CD68 (IHC, 40×); (e) Antibody to S-100 (IHC, 40×); (f) MIB-1 index (IHC, 40×);H&E- Hematoxylin and eosin; IHC - Immunohistochemistry

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Figure 3: Variation of morphology in HS; (a) Foamy appearance of neoplastic cells; (b) Spindle-cell morphology; (c) Tumor cells showing hemophagocytosis and erythrophagocytosis; (d) Tumor cells having yellowish-brown hemosiderin pigment in their cytoplasm (H&E, 40×)

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Table 3: Frequency of expression of immunohistochemistry markers in FDCS and HS

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Table 4: Histopathology and IHC findings in HS

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Primary surgical intervention was done in all patients. Three patients [Case no. 5, 6, 7; [Table 1]] received adjuvant CT with Cyclophosphamide, Doxorubicin, Vincristine and Prednisone. One patient with inguinal nodal involvement [Case no. 8; [Table 1]] received CT with Cyclophosphamide, Etoposide and Mitoxantrone. Two cases developed bone metastasis and another one case had bone marrow involvement [Table 1]. Median survival was 8.5 months after diagnosis.

   Discussion Top

Follicular dendritic cell sarcoma

FDCS involves young to middle-aged patients, with a median age of approximately 50 years. Most cases occur in the lymph nodes, typically cervical nodes. Around 30% of tumors are initially diagnosed at the extra-nodal sites. Approximately 10%-20% of FDCS may be associated with Castleman disease, usually hyaline vascular type.[3] Recent studies demonstrated that FDCS contains indolent T-lymphoblastic proliferations (immature TdT positive T-cells) in approximately 45% of cases and is frequently associated with paraneoplastic autoimmune multiorgan syndrome. Recognition of this phenomenon is important to avoid misdiagnosis as thymoma or T-lymphoblastic lymphoma.[4],[5] In our series also, we demonstrated immature TdT positive indolent lymphoblastic cells focally in two cases [Figure 1]g (case No. 3),1h and 1i (case No. 4)]. However, none of them had any features of autoimmune multiorgan syndrome.

In our study, the median age of the patients having FDCS was similar to other study.[6] We found intra-abdominal FDCS involving ovary/pelvic cavity and uterus/broad ligament in two cases. Other different extra-nodal sites of involvement reported in literature were oropharyngeal region, head and neck, thyroid, abdominal wall, mediastinum, cranium and colon.[7],[8],[9],[10],[11],[12],[13]

We assessed nuclear pleomorphism, mitotic activity and coagulative tumor cell necrosis in FDCS. Two of the cases with extra-nodal location, moderate pleomorphism and mitotic count of ≥5 per 10 high-power field showed tumor recurrence within one year. Among these two cases, one having tumor necrosis also died of the disease [Table 2]. In a study, Saygin et al., showed that age <40 years, large tumor size >6 cm, high mitotic index >5/10 high-power field, coagulative necrosis, and marked cellular atypia were associated with poor prognosis.[14]

The pathological diagnosis of FDCS cannot be based solely on morphological grounds but must be confirmed by IHC. Most widely used FDCS markers are CD21, CD23, CD35, and D2-40 (podoplanin), which can distinguish FDCS from other spindle-cell neoplasms.[15],[16],[17] The entities that can mimic nodal FDCS are metastatic carcinoma, malignant lymphoma, interdigitating dendritic cell sarcoma, metastatic melanoma, myeloid sarcoma (MS), spindle-cell thymoma, inflammatory myofibroblastic tumor (IMT) and IgG4-related sclerosing disease. Metastatic spindle cell carcinoma usually shows frank nuclear atypia and positivity of cytokeratin but not FDC markers, and clinical history of carcinoma is also helpful in establishing the correct diagnosis. Lymphoma cells are usually discrete and show positivity for B or T lymphoid markers. Interdigitating dendritic cell sarcoma shows diffuse sheet-like distribution or characteristic interfollicular growth pattern, but lacking prominent reactive lymphocytes. Moreover, the tumor cells are immunoreactive for S100, but lack expression of FDC markers. Metastatic melanoma may have spindle-cell morphology, lacking prominent lymphocytic reaction. In addition, melanoma cells are positive for S100 and HMB45 but negative for FDC markers. MS demonstrates the proliferation of immature myeloid blasts and positive for MPO, CD117 and CD45. Spindle-cell thymoma shows spindle-cell proliferation with admixed lymphocytes. The thymoma cells express cytokeratin but not FDC markers. The background lymphocytes of thymoma are immature T-cells, expressing CD1a, CD99 and TdT.IMT has significant morphologic overlap with FDCS. IMT shows a mixed inflammatory infiltrate composed of lymphocytes, plasma cells, eosinophils, and histiocyte, and the spindle cells are positive for smooth muscle act in but negative for FDC markers. Approximately 40% cases of IMT express ALK. IgG4-related disease morphologically may resemble FDCS but it shows a mixed inflammatory background with abundant lymphoplasmacytic cells. Moreover, the fibroblasts in IgG4-related disease are negative for FDC markers. Owing to a small number of the reported cases in the literature, the clinicopathological characteristics of extra-nodal FDCS remained under-recognized. Almost one-third of cases were misdiagnosed at initial evaluation as documented in the literature.[6],[12],[13],[15] The main cause of misdiagnosis was not to consider a poorly differentiated tumor in the extra-nodal site to be FDCS when it is first evaluated. Another cause was lack of practice to use of the FDC markers routinely to detect FDCS in the extra-nodal sites. For intra-abdominal presentation, more attention to be given to rule out Gastrointestinal stromal tumor (GIST), Malignant peripheral nerve sheath tumor (MPNST), Leiomyosarcoma and IMT in addition to the list of tumors mimicking nodal FDCSs. In this scenario, inclusion of CD21 and CD23 to the panel of IHC markers solved the problem as none of the other tumors expressed CD23 and CD21 other than FDCS in our study. CD35 was not done in any case. In a study by Barbara Pro. et al. (n = 14), CD21, CD23, CD35, CD68 and S100 were positive in 83%, 90%, 40%, 57% and 9% of the cases, respectively.[3] In another study by A. Agaimy et al.,(n = 15), CD21, CD23, CD35 and Podoplanin were expressed in 61.5%, 15.3%, 66.6% and 50% of the cases, respectively.[18]

FDCS was previously considered an indolent tumor with low tendency towards recurrence or metastasis. However, findings of studies with larger patient cohorts and longer follow-up have shown that FDCS behaves more aggressively and should be considered an intermediate-grade malignancy. It has been documented that at least 40% of reported FDCS have recurred and 25% have metastasized with a mortality rate of 16.7%.[17],[19] In our study, abdominopelvic lesions showed recurrence and mortality as documented in other published series.[20] However, none of the patients in our series developed distant metastasis. Surgical excision is the treatment of choice for localized FDCS,[8],[21] whereas chemotherapy is indicated for patients with unresectable disease or multiorgan involvement.[22],[23] Conry published a case series of two (2) patients in which combination chemotherapy with gemcitabine and docetaxel was tried for the first time in metastatic FDCS with good response rates.[24]

Histiocytic sarcoma

The term 'histiocytic sarcoma' was introduced in 1970 by Mathex et al.[25] Since then, emphasis has been given on cytochemical and immunohistochemical properties of these neoplasms in an attempt to establish true histiocytic lineage.[26]

Tumor size appeared to be an important predictor of clinical behavior. The mean tumor size in our study was 7 cm. In a study, JA Vos et al. showed each case that had poor survival, had a presenting tumor with size of ≥3.5 cm in greatest dimension (range of 3.5–9.5 cm).[27] In our study, the patients having tumor of more than 3.5 cm had tumor recurrence. Among them, two patients died of the disease. One patient having involvement of cervical lymph node with largest tumor size of 3 cm had no tumor recurrence after initial surgery and CT.

The HS usually shows a diffuse architecture involving either nodal or extra-nodal sites. A sinusoidal or paracortical pattern can be seen in lymph node.[28] Variations in morphology do occur in HS at different sites or even within the same tumor. Besides those we found [Figure 2]c and [Figure 3]a,[Figure 3]c,[Figure 3]d, several other features mentioned in the literature are the presence of admixed Langerhans cells as a minor component,[29] formation of epithelioid clusters and sheets.[30] The presence of a prominent lymphoid infiltrates in case of the presumed trans-differentiation to the HS from a chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) or synchronous development of the HS and a CLL/SLL.[31]

We analyzed nuclear pleomorphism, mitosis and necrosis in HS and correlated with disease recurrence and mortality. All the cases showing marked nuclear atypia, necrosis and mitosis of >5/10 HPF, were associated with poor survival.

The important differential diagnoses for HS are metastatic carcinoma, sarcomas with epithelioid morphology, MS, malignant melanoma, large-cell lymphoma, germ cell tumor, epithelioid GIST and IMT. Sometimes, Erdheim-Chester disease (ECD) and Rosai-Dorfman disease (RDD) also pose diagnostic challenge. Morphologically, ECD contains lipid-laden macrophages and scattered Touton giant cells in a background of fibrosis and reactive plasma cells. The histiocytes in ECD are negative for S100 and CD1a. Characteristic osteosclerosis in radiology also helps to differentiate ECD from other conditions. The histiocytes in RDD are much larger with characteristic emperipolesis and lack expression of CD1a. In many cases of HS, the cell-in-cell appearance can be found due to phagocytosis rather than true emperipolesis, that is, cells present in the cytoplasm are phagocytosed generating cellular debris. HS can include areas with relatively more uniform sized of histiocytoid cells resembling MS. In contrast to HS, MS has a less mature chromatin pattern, a more monotonous look, and less cytoplasm. Although HS and MS both can express CD68, but MPO, CD13 and CD33 reactivity is shown only in MS. In our study, CD68 consistently showed intense immunoreactivity in all of the four cases. Moreover, CD68 (KP1 clone) has been reported in melanoma with similar frequency to that of traditional melanoma markers (i.e. S-100, HMB-45), although the staining is usually not as intense as in HS.[32],[33]All of the cases were negative for HMB-45 in our study. S-100 was focally positive in two cases. In literature, S-100 reactivity has been reported in benign as well as malignant histiocytic lesions.[34],[35] MIB-1 index was variable (20%–50%). CD45 and CD43 were expressed by tumor cells in variable proportions [Table 3]. All B- and T-cell, epithelial, mesenchymal, melanocytic and accessory/dendritic cell markers were uniformly negative [Table 4].

Several literature had documented that extra-nodal HSs with localized involvement were often treated with surgery. In case of an advanced stage, chemotherapy and radiotherapy may be considered, but the number of cases was too small to predict the effect of the CT/RT on prognosis.[8],[30] In our study, surgery was the primary treatment followed by CT. Three patients developed bone metastasis. The median survival of the patients having HS was 8.5 months similar to the finding of other studies.[27]

   Conclusion Top

FDCSs and HSs are extremely rare tumors. Consistent with their rarity, they remain under-recognized and easily prone to a misdiagnosis. The treatment of these tumors is not being specified. Accurate staging is mandatory to distinguish between localized and systemic forms to precise the therapeutic decision. Therefore, considering these rare entities in differential diagnoses and inclusion of IHC biomarkers for FDC as well as histiocytic differentiation are necessary to avoid potential misdiagnosis.


We acknowledge all the technical staffs in histopathology and immunohistochemistry sections of our department for their support.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

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Correspondence Address:
Priti Trivedi
Department of Oncopathology, The Gujarat Cancer and Research Institute, New Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat - 380 016
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_461_20

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