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Other spindle cell tumors on FNA smears processed by the ultrafast Papanicolaou protocol. (A) Leiomyosarcoma (× 400). (B) Fibrosarcoma (× 400). (C) Schwannoma (× 400). (D) Gastrointestinal stromal tumor (× 400).
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Follicular dendritic cell (FDC) tumor is a rare tumor derived from accessory cells in the lymphoid follicles. FDC tumors are typically diagnosed on histology based on immunoreactivity to at least 1 of the FDC markers (CD21, CD23 or CD35) or based on the characteristic ultrastructural feature of long, interwoven, cytoplasmic, dendritic processes con...
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Context 1
... interconnecting network of long, multipolar, cytoplasmic processes should also be observed in UFP-stained direct smears of interdigitating dendritic cell sarcoma (S100+, CD21−) and dendrit- ic cell sarcoma, not otherwise specified (CD1a+, S100+, CD21−) 16 but cannot possibly occur in other tumors in the broad differential diagnosis, as mentioned above. The cytoplasmic processes of other spindle cell tumors, such as leiomyosarcoma ( Figure 3A), fibrosar- coma ( Figure 3B), peripheral nerve sheath tumor ( Figure 3C) and gastrointestinal stromal tumor ( Figure 3D), are bipolar, rather than multipolar, as characteristic of dendritic cell sarcomas and dendrites of the neuron. This case illustrates the importance of executing proper smearing technique and the advantage of using air-dried, re- hydrated, direct smears incorporating the UFP protocol to maxi- mize information obtained for cytomorphologic analysis. ...
Context 2
... interconnecting network of long, multipolar, cytoplasmic processes should also be observed in UFP-stained direct smears of interdigitating dendritic cell sarcoma (S100+, CD21−) and dendrit- ic cell sarcoma, not otherwise specified (CD1a+, S100+, CD21−) 16 but cannot possibly occur in other tumors in the broad differential diagnosis, as mentioned above. The cytoplasmic processes of other spindle cell tumors, such as leiomyosarcoma ( Figure 3A), fibrosar- coma ( Figure 3B), peripheral nerve sheath tumor ( Figure 3C) and gastrointestinal stromal tumor ( Figure 3D), are bipolar, rather than multipolar, as characteristic of dendritic cell sarcomas and dendrites of the neuron. This case illustrates the importance of executing proper smearing technique and the advantage of using air-dried, re- hydrated, direct smears incorporating the UFP protocol to maxi- mize information obtained for cytomorphologic analysis. ...
Context 3
... interconnecting network of long, multipolar, cytoplasmic processes should also be observed in UFP-stained direct smears of interdigitating dendritic cell sarcoma (S100+, CD21−) and dendrit- ic cell sarcoma, not otherwise specified (CD1a+, S100+, CD21−) 16 but cannot possibly occur in other tumors in the broad differential diagnosis, as mentioned above. The cytoplasmic processes of other spindle cell tumors, such as leiomyosarcoma ( Figure 3A), fibrosar- coma ( Figure 3B), peripheral nerve sheath tumor ( Figure 3C) and gastrointestinal stromal tumor ( Figure 3D), are bipolar, rather than multipolar, as characteristic of dendritic cell sarcomas and dendrites of the neuron. This case illustrates the importance of executing proper smearing technique and the advantage of using air-dried, re- hydrated, direct smears incorporating the UFP protocol to maxi- mize information obtained for cytomorphologic analysis. ...
Context 4
... interconnecting network of long, multipolar, cytoplasmic processes should also be observed in UFP-stained direct smears of interdigitating dendritic cell sarcoma (S100+, CD21−) and dendrit- ic cell sarcoma, not otherwise specified (CD1a+, S100+, CD21−) 16 but cannot possibly occur in other tumors in the broad differential diagnosis, as mentioned above. The cytoplasmic processes of other spindle cell tumors, such as leiomyosarcoma ( Figure 3A), fibrosar- coma ( Figure 3B), peripheral nerve sheath tumor ( Figure 3C) and gastrointestinal stromal tumor ( Figure 3D), are bipolar, rather than multipolar, as characteristic of dendritic cell sarcomas and dendrites of the neuron. This case illustrates the importance of executing proper smearing technique and the advantage of using air-dried, re- hydrated, direct smears incorporating the UFP protocol to maxi- mize information obtained for cytomorphologic analysis. ...
Similar publications
Follicular dendritic cells (FDC) are non-lymphoid, non-phagocytic accessory cells in the immune system that are essential for antigen presentation and germinal center reaction regulation. These cells are CD21+, CD35+, CD1a- and S100 protein +/- and they show desmosomes ultrastructurally. The most commonly involved sites by FDC tumors are lymph node...
Citations
... Their shape is variable, but most reports describe them as oval to spindle with some showing epithelioid or polygonal morphology. Different authors have made an emphasis on long, slender cytoplasmic processes (Figure 2b) variably described as stellate cells [23], interwoven dendritic (spider web-like) [30], or interconnecting [36]. Probably, the most remarkable description was that made by the group of Pambuccian [36] that mentions such cytoplasmic meshwork as resembling the head of the mythical monster Medusa. ...
Follicular dendritic cells (FDCs) are antigen-presenting cells located in the germinal centers of the lymph nodes. Among the few tumors showing FDC differentiation are follicular dendritic cell sarcoma (FDCS) and Castleman disease (CD), more precisely the unicentric hyaline vascular (HV) variant. Both are relatively rare tumors, and the diagnostic cytological experience is limited to descriptions of isolated cases or small series. The purpose of this review is to bring together all the available cytological published information, and our personal experience, in order to obtain a global idea of the cytological features of these peculiar FDC-derived tumors. The different descriptions of FDCS are very similar, reflecting a tumor that shows repetitive and characteristic cytological features. It shows a dimorphic population of mature lymphocytes and large tumoral cells with partial spindle morphology. Most cases of HV variant of CD can be recognized as benign upon cytology, however a precise diagnosis seems more difficult. It is characterized by reactive lymphocytes mixed with vessels and FDCs, either single or forming syncytial aggregates. Both, FDCS and CD are challenging for cytological diagnosis in which a high index of suspicion is necessary for a correct preoperative assessment. Cytology is very useful for follow-up of recurrences and metastases.
... The characteristic cytological features of FDCS was used for cytomorphological diagnosis in many cases (Czapla et al., 2017;Duan et al., 2017Duan et al., , 2020Granados et al., 2008;Hang et al., 2017;H. Liu et al., 2020;Lu et al., 2020;Massoth et al., 2021;Patra et al., 2021;Song et al., 2009;Yang et al., 2006). However, overlapping morphological characteristics with other neoplasms make it difficult to diagnose accurately (Chang et al., 2017;Czapla et al., 2017;Granados et al., 2008;Tao et al., 2019;Wright et al., 1997;Yang et al., 2006). ...
... Liu et al., 2020;Lu et al., 2020;Massoth et al., 2021;Patra et al., 2021;Song et al., 2009;Yang et al., 2006). However, overlapping morphological characteristics with other neoplasms make it difficult to diagnose accurately (Chang et al., 2017;Czapla et al., 2017;Granados et al., 2008;Tao et al., 2019;Wright et al., 1997;Yang et al., 2006). It could easily be misdiagnosed (Loo et al., 2001;Mohanty et al., 2003;Ryley et al., 1999) because it has morphological features similar to other mesenchymal tumors such as inflammatory myofibroblast tumor, smooth muscle, and fibrohistiocytic tumors, metastatic sarcoma, and undifferentiated carcinoma (Song et al., 2009). ...
Follicular dendritic cells (FDCs) are unique accessory immune cells that contribute to the regulation of humoral immunity. They are multitasker cells essential for the organization and maintenance of the lymphoid architecture, induction of germinal center reaction, production of B memory cells, and protection from autoimmune disorders. They perform their activities through both antigen-driven and chemical signaling to B cells. FDCs play a crucial role in the physiological regulation of the immune response. Dis-regulation of this immune response results when FDCs retain antigens for years. This provides a constant antigenic stimulation for B cells resulting in the development of immune disorders. Antigen trapped on FDCs is resistant to therapeutic intervention causing chronicity and recurrences. Beyond their physiological immunoregulatory functions, FDCs are involved in the pathogenesis of several immune-related disorders including HIV/AIDS, prion diseases, chronic inflammatory, and autoimmune disorders. FDCs have also been recently implicated in rare neoplasms of lymphoid and hematopoietic tissues. Understanding FDC biology is essential for better control of humoral immunity and opens the gate for therapeutic management of FDC-mediated immune disorders. Thus, the biology of FDCs has become a hot research area in the last couple of decades. In this review, we aim to provide a comprehensive overview of FDCs and their role in physiological and pathological conditions.
... The most common location of these tumors was lymph nodes, and only a total of 3 cases were diagnosed correctly on cytology, all of which were known cases of FDCS [8][9][10] . In other cases, a diagnosis of metastatic carcinoma, malignant tumor of uncertain origin, Hodgkin disease, and melanoma was proposed. ...
Background:
Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm arising from follicular dendritic cells of germinal centers. The most common site of origin is lymph nodes and it may mimic a variety of tumors at that location, including carcinomas and sarcomas. Diagnosis is frequently missed on cytology as there are very few case reports describing the cytological characteristics of the lesion. Even on histology, a high degree of suspicion is required for an appropriate diagnosis.
Case:
A 60-year-old male presented with a gradually increasing left submandibular mass that had been present for 3 months. Fine-needle aspiration cytology (FNAC) was performed, showing many clusters as well as scattered epithelioid cells with spindled to oval nuclei, nuclear pleomorphism, grooves, inclusions, and uniformly dispersed mature lymphocytes throughout the smears. The diagnosis of FDCS was suspected and was confirmed on histopathology and immunohistochemistry.
Conclusion:
FNAC can be a cheap, easy, and helpful tool in obtaining a diagnosis of FDCS as there are few characteristic cytological features that are better recognized than histology.
... Classical FDC sarcoma is characterized by dual populations of cells, attributed by neoplastic proliferation of large spindled to ovoid cells with various pleomorphisms in a background of abundant reactive chronic inflammatory cells. [2][3][4][5] Classic cytologic findings for FDC sarcoma feature solid sheets or fascicular or whirl-like patterns of large oval to spindle cells in a background of inflammatory cells. In contrast, the imprint smears of the present case showed many tumor cells with epithelioid or Reed-Sternberg cell-like shapes dispersed in a background of inflammatory cells, which can be misleading, and result in erroneous diagnosis of lymphoid malignancy in preference to Hodgkin lymphoma. ...
Follicular dendritic cell (FDC) sarcoma is a neoplastic proliferation of FDCs. Because its cytologic findings can vary widely, both the cytomorphology and histopathology of FDC sarcoma can impose a significant diagnostic dilemma. We present cytologic features of FDC sarcoma assessed by intraoperative touch imprint. Intra-abdominal lymphadenopathies were noted in 54-year-old male with hepatitis B-virus associated liver cirrhosis. In contrast to cytologic features of classical FDC sarcoma, the tumor cells featured a large epithelioid or Reed-Sternberg cell-like shape scattered in a background with abundant inflammatory cells, which led to a mistaken diagnosis of malignant lymphoma. However, in accordance with cytologic features previously described in the literature, the tumor cells were characterized by a fragile cytoplasm with cytoplasmic processes in dendritic or reticulated patterns reminiscent of the ultrastructural features of FDC. Cytoplasmic features rendering nuclei with a tendency to form clusters or syncytial aggregates associated with reactive lymphocytes appear to be the most valuable finding in diagnosis of FDC sarcoma.
... However, dendritic cell processes, a cytologic clue for dendritic cell sarcomas, were previously reported only by Banner et al. 12 in IDCS and by Yang et al. in FDCS. 15 The diagnosis of all neoplasms having histiocytic and/ or dendritic differentiation is problematic for both cytopathologists and surgical pathologists. Clinical presentation, cellular and nuclear features, the presence of dendritic cell processes, and selective use of commonly available immunohistochemical stains (S100 and CD68) on an inevitably limited FNA specimen will hopefully point the pathologist in the right direction to make at least a broad and presumptive diagnosis of such a neoplasm. ...
A hybrid histiocytic sarcoma-interdigitating dendritic cell sarcoma was found in a small perinephric lymph node of an asymptomatic 80-year-old man, who presented a year ago with two small foci of lung metastasis found during routine chest X-ray. Fine needle aspiration cytology demonstrated interconnecting long and thin cell processes radiating from dendrite-like neoplastic cells with oval, enlongated, reniform, and irregular nuclei with vesicular chromatin and distinct nucleoli. Histology showed spindled epitheliod and histiocytic cells with abundant, slightly eosinophilic cytoplasm with indistinct cell borders and forming fascicles in a vague storiform pattern with interspersed T-lymphocytes. Immunohistochemically, the neoplastic cells were strongly positive for histiocytic markers: CD163, CD68, lysozyme, and PU.1, as well as strongly positive for dendritic cell markers: S100 and fascin, but were negative for CD1a (Langerhans cell marker), CD21/CD35 (follicular dendritic cell markers), B-cell, and T cell markers. This case is compared to the four hybrid histiocytic-dendritic sarcomas reported since 1983. Diagn. Cytopathol. 2010. © 2009 Wiley-Liss, Inc.
... 6 There are only 12 cytological reports of FDC tumors in the literature. [7][8][9][10][11][12][13][14][15][16][17] In four of them, the tumors were intraabdominal. Two aroused in the mesentery, 11,14 and the other two were metastatic liver lesions from primary splenic 16 and peri-splenic 17 tumors. ...
... There are only 12 cytological reports of FDC tumors in the literature. [7][8][9][10][11][12][13][14][15][16][17] Eight were seen in lymph nodes and four were intraabdominal. From the latter, two aroused in the mesentery, 11,14 and the other two were metastatic liver lesions from primary splenic 16 and peri-splenic 17 tumors. ...
... In a recent report, multiple cytoplasmic processes interconnecting tumor cells were observed with a modified Papanicolaou-stained technique. 17 We saw long cytoplasmic cell processes in our case (Fig. 5). Most of them were bipolar, indistinct from those found in other sarcomas, such as fibrosarcomas or leiomyosarcomas. ...
Follicular dendritic cell (FDC) sarcoma is an exceedingly uncommon tumor of lymph nodes and extranodal tissues. The inflammatory pseudotumor (IPT)-like variant of FDC sarcoma of intraabdominal location is considered a separate entity, with different clinical and pathological features than those of the classic FDC tumor.
There have been only 12 cytological reports of FDC sarcomas in the literature. Two of them were metastases to the liver and, like our case, had features of IPT. Fine-needle aspiration biopsy (FNAB) and imprint and scrape cytology from the surgically excised tumor here reported revealed spindle tumor cells with moderate pleomorphism, nuclear grooves, prominent nucleoli, and cytoplasmic processes, admixed with inflammatory cells. To the best of our knowledge, this is the first cytology report of a primary hepatic FDC tumor. The cytological findings permit the recognition of this tumor. However, confirmation by inmunohistochemistry (IHQ) is mandatory for a definitive diagnosis. Diagn. Cytopathol. 2008;36:42–46.
Background:
Follicular dendritic cell sarcoma is a malignant neoplasm derived from germinal center follicular dendritic cells, which both share a characteristic immunophenotype (namely CD21, CD23, and CD35). Cytomorphologic descriptions are few, consisting of only 26 prior cases from 24 publications. Identification by cytologic means appears challenging as the majority of previous reports disclose an erroneous or indeterminate initial cytologic diagnosis. Herein, we present the largest cytology series to date with the aim of expanding upon this small body of literature and discuss possible factors resulting in misinterpretation.
Materials and methods:
A retrospective search was conducted from 2 academic medical centers to identify histologically confirmed cases of follicular dendritic cells with an associated cytologic component. Clinicopathologic data were tabulated and a comparative analysis of cytomorphologic and immunohistochemical features was performed.
Results:
Seven separate cases were identified. All cases showed cohesive tumor cells with a characteristic voluminous, ill-defined cytoplasm with interconnecting fibrillary processes and intimately admixed mature lymphocytes. Features were maintained across various cytologic preparations, including conventional smear, liquid-based cytology, and touch imprint. Unusual immunohistochemical profiles were noted in a subset of cases.
Conclusions:
Cytomorphology is highly conserved across cases and preparations; however, a propensity for aberrant immunoexpression may contribute to diagnostic errors. Cytomorphologic features, supported by immunohistochemistry, suggest fine-needle aspiration as a reasonable diagnostic modality. Tumors with these features should include CD21, CD23, and/or CD35 in the workup.
Follicular dendritic cell sarcomas (FDCS) are rare tumours of lymph nodes and extranodal tissues which are grouped with the histiocytic and dendritic cell neoplasms. The diagnosis is usually made after thorough clinical and pathological examination with immunohistochemical analysis. Difficulties persist in diagnosing FDCS on cytological preparations. We report herein a case of a 57-year-old female who presented with a right neck mass of 5 months duration. Computed Tomography (CT) imaging of the neck reported a necrotic right level IIb lymph node and asymmetric fullness of the right palatine tonsil. Fine needle aspiration (FNA) biopsy revealed numerous spindle, oval and stellate neoplastic cells, arranged singly and in syncytia with moderate nuclear pleomorphism, vesicular chromatin pattern, and prominent nucleoli, sprinkled with small lymphocytes. The tumour cells were strongly diffusely positive for CD21, CD23, and D2-40 immunostaining on cell bock sections, but were negative for CD1a and CD34, supporting the diagnosis of FDCS. Follow-up surgical pathology on the resection showed histopathological features and an immunohistochemical profile consistent with FDCS.
Abstract
The diagnosis of follicular dendritic cell sarcoma (FDCS) is frequently missed on cytology as there are very few case reports outlining the cytological characteristics. In this case report, the authors present a case of cervical FDCS diagnosed on FNA cytopathology and describe the cytomorphological and histopathological features.
Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm of the accessory immune cells that can present at both nodal and extranodal sites¹. A high index of suspicion is crucial so as to include FDCS in the differential diagnosis of a spindle cell neoplasm in the appropriate clinical and morphological context. However, the morphological overlap with other tumors makes this entity a diagnostic challenge unless supplemented by other ancillary tests like immunohistochemistry and ultrastructural studies.
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