Home | About IJMPO | Editorial board | Search | Ahead of print | Current Issue | Archives | Instructions | Subscribe | Advertise | Contact us |  Login 
Indian Journal of Medical and Paediatric Oncology
Search Article 
Advanced search 

 Table of Contents      
Year : 2013  |  Volume : 34  |  Issue : 4  |  Page : 317-319  

Follicular dendritic cell sarcoma with paraneoplatic pemphigus: Rare case and a brief review of literature

1 Department of Medical Oncology, Dr. BRA IRCH, AIIMS, New Delhi, India
2 Department of Radiology, AIIMS, New Delhi, India
3 Department of Pathology, AIIMS, New Delhi, India

Date of Web Publication18-Jan-2014

Correspondence Address:
Saphalta Baghmar
Department of Medical Oncology, Dr. BRA IRCH, AIIMS, New Delhi - 110 029
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-5851.125255

Rights and Permissions

Paraneoplastic pemphigus (PNP) is often a fatal autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. We describe a patient with PNP associated with follicular dendritic cell sarcoma (FDCS), a rare neoplasm originating from follicular dendritic cells, which are non-lymphoid, non-phagocytic accessory cells of the lymphoid system and play an integral role in regulation of the germinal center reaction and present antigens to B-cells. The presence of rich vascularity around the tumor and few hyalanized vascular follicles found in histopathological examination gives the clue that the tumor might have developed from Castleman's disease (CD). As for the mechanisms by which CD induces PNP, it has been proposed that autoantibodies secreted from the Castleman's tumor play pivotal role. This hypothesis seems to be supported by the present case, in which CD may have triggered both the FDCS and the PNP.

Keywords: Castleman′s disease, follicular dendritic cell sarcoma, paraneoplatic pemphigus

How to cite this article:
Baghmar S, Kumar S, Gupta S D, Raina V. Follicular dendritic cell sarcoma with paraneoplatic pemphigus: Rare case and a brief review of literature. Indian J Med Paediatr Oncol 2013;34:317-9

How to cite this URL:
Baghmar S, Kumar S, Gupta S D, Raina V. Follicular dendritic cell sarcoma with paraneoplatic pemphigus: Rare case and a brief review of literature. Indian J Med Paediatr Oncol [serial online] 2013 [cited 2020 Jun 7];34:317-9. Available from: http://www.ijmpo.org/text.asp?2013/34/4/317/125255

  Introduction Top

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm (0.4% of soft tissue sarcomas) [1] originating from follicular dendritic cells (FDCs), a nonlymphoid, nonphagocytic antigen presenting cells of the lymphoid system. [2] The tumor was first characterized by Monda [3] et al., and subsequently, specific immunohistochemical markers [2] have been identified. Since that time, only 3 cases of FDCS arising from castleman's disease (CD) with paraneoplastic pemphigus (PNP) have been reported in the literature. [4],[5],[6]

  Case Report Top

A 20-year-old boy presented with 7 months history of multiple polymorphous vesiculobullous mucocutaneous eruptions around the lips and subsequently in buccal mucosa causing dysphagia. He later developed conjuntivitis associated with blurred vision and atypical targetoid lesions with crusts surrounded by erythema scattered on the trunk and extremities including prepuce and perianal region [Figure 1]. He lost 5 kg during this period. Direct immunoflorescence of biopsy specimen from Skin lesion showed deposition of Immunoglobulin G (+) and C3 in the intercellular region of epidermal cells mainly in the suprabasal region. Linear deposition of IgG is also seen in dermo-epidermal junction suggestive of PNP. Computed tomography (CT) scan revealed a well defined intensely enhancing mass lesion with central necrosis in the right hemipelvis, 6 cm in size, abutting the right obturator internus muscle and displacing the urinary bladder anteriorly. Engorged vessels were seen around the mass [Figure 2]. Exploratory laprotomy was done but the mass was found to be unresectable. Histopathlogy of the mass showed oval to spindle cells in a background of vascular stroma infiltrated by lymphocytes. The cells were immunopsitive for CD21, CD35, CD23, S-100, Claustrin, CD34, CD31, S-100; Leucocyte common antigen, and H uman leukocyte antigen-DR were also positive in some cells. Hyaline-vascular follicles were identified at the peripheral portion or occasionally scattered in the center of the tumor. Rich vascularity was highlighted by the CD34 immunostaining [Figure 3] and [Figure 4]. Overall features favoured FDCS. The presence of rich vascularity around the tumor and few hyalanized vascular follicles found in histopathological examination (HPE) gave the clue that the tumor might have developed from CD. He tested negative for human immunodeficiency virus. He was started on systemic steroids. His truncal lesions marginally improved after steroids but there was no improvement in arm lesions. He was then given Rituximab, cyclophosphamide, Adriamycin, vincristin, prednisolone. He expired post 1 st course of chemotherapy due to pseudomonas infection.
Figure 1: (a) Vesiculobullous mucocutaneous eruptions around the lips (b) Crusts surrounded by erythema scattered on the extremities

Click here to view
Figure 2: Well defined intensely enhancing mass lesion with central necrosis in the right hemipelvis, 6 cm in size, abutting the right obturator internus muscle and displacing the urinary bladder anteriorly

Click here to view
Figure 3: Photomicrograph shows cells are arranged in sheets with small lymphocytic nodules forming germinal center. [Fig a, H&E ×40 and b ×100]. The tumor cells are oval to slindle with ill defined border. The nuclei are oval to spindle with vesicular chromatin. [Fig c, ×200, d ×400]

Click here to view
Figure 4: Photomicrograph shows positivity of CD20 in the nodules [Fig 4e, IHC-CD20 ×100], the nodules are negative for CD3 (4f, ×100), the spindle cells are immunopositive for CD35, CD43, Clusterin [Fig 4g, IHC-CD35 ×100, h-CD43 ×200, j-Clusterin ×200]

Click here to view

  Discussion Top

FDCS arises from FDCs which are localized within lymphoid follicles; morphologically have entangled cytoplasmic processes and frequent desmosomes; have the ability to trap and retain antigens for long periods of time in the form of immune complexes, but have a non-phagocytic activity; and express molecules involved in the proliferation and differentiation of B-cells. [2] The only predisposing factor identified for FDCS is hyaline-vascular CD (10-20% of cases). [7] FDCS are associated with Epstein barr virus (12%) especially those occurring in the liver. [7] It generally affect young to middle-aged adults, with a mean age of 43 years with no sex predilection. [8] They usually present as painless coalescent nodal mass (60%) of variable size [8] (1-20 cm diameter), but extranodal disease is most commonly seen in the head and neck (tonsil, nasopharynx) and intraabdominal organs (in particular the pancreas and peripancreatic tissues). [7] Other locations: axilla, gastrointestinal tract (17%), liver, oral cavity (13%), mediastinum (8%) and spleen (2%). [7]

Grossly it appears as a firm white mass. HPE showed ovoid to spindled cells whorls with long cellular processes, distinctive nuclei, speckled to vesicular chromatin; sometimes multinucleated cells. Hemorrhage or necrosis is an uncommon finding. [7] Immunohistochemistry positive for CD21, CD23, CD35, Fascin, Clusterin, podoplanin, Desmoplakin Epithelial membrane antigen, Epithelial growth factor receptor, Anti-Follicular Dendritic Cells antibody(Ki-M4), Anti-Follicular Dendritic Cells antibody CNA 42, CD68, Fragment crystallizable region, S100 ± (but not strong) Factor XIIIa ± while negative for Leucocyte common markers (CD1a), cytokeratin, lysozyme, CD34, CD3, CD79a, CD30, Human Melanoma Black HMB45. [2]

A subset of these tumors has been reported to arise in or concomitantly with foci of CD [7] as in our case. The presence of rich vascularity around the tumor and few hyalanized vascular follicles found in HPE gave the clue that the tumor might have developed from CD. In fact, some argue that FDCS actually develops from CD as a hyperplasia - dysplasia - neoplasia sequence [7] so FDCS may share some of the radiologic features of CD. In our patient, we saw a large lobulated soft-tissue mass with engorged vessels seen around it on CT, the feature of CD.

FDCS behaves more like low-grade sarcoma than lymphoma. 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 disease. Cyclophosphamide, Adriamycin, vincristin, prednisolone CHOP [9] have shown good response. In a small series by Soriano et al. [9] patients who received all three modalities of treatment upfront had the longest disease-free intervals. Sarcoma-based regimen gemcitabine and taxane; [9] imatinib in combination with gemcitabine and cisplatin, [10] rituximab [11] was also reported to have some activity against FDCS. Our patient was given R-CHOP. But response could not be assessed as he expired post first cycle due to pseudomonas infection.

Poor prognostic factors like extensive necrosis, a mass larger than 6 cm, cytologic atypia, intraabdominal location, and a high proliferative index increase the likelihood of recurrence, metastasis, and mortality. [7] The lung, liver, and lymph nodes are the most common sites for metastasis, and the tumor may be highly aggressive and fatal within 2 years of diagnosis. [7] Local recurrence rate was reported to be 43% while distant metastases - 24% (lung, liver, peritoneum, pancreas and lymph nodes). Five-year recurrence-free survival rates of 27% to 32%. And overall 5-year survival rate of 79%. [7]

Our patient presented with PNP, an autoimmune bullous disease charaecterized by severe stomatitis, polymorphous skin eruptions that occurs in association with various forms of underlying neoplasia. It is characterized by the presence of autoantibodies that react with intermediate filament-associated proteins in desmosomes and hemidesmosomes; desmoplakin, bullous pemphigoid antigen 1, and envoplakin. [4]

A review of 163 case reports of PNP reported that 84% of cases were associated with hematologic malignancies (Non hodgkin lymphoma [39%], Chronic lymphocytic leukemia [18%], CD [18%], thymoma [6%], Waldenstrom macroglobulinemia [1%], Hodgkin's lymphoma [1%], and monoclonal gammopathy [1%]). The remaining 16% were associated with nonhematologic neoplasms (epithelial origin carcinoma [9%], mesenchymal origin sarcoma [6%], and melanoma [1%]). Oral ulcerations were the presenting feature in 45% of all cases and appear to be a key feature of PNP. [12]

In conclusion, our case report is unique because it reports for the 1st time to our knowledge as a pathological constellation of follicular dendritic cell sarcoma with castleman's disease and paraneoplastic pemphigus arising from extranodal pelvic region, a rare disease at a rare site. Its importance lies in suspicion of diagnosis and considering the treatment of paraneoplastic pemphigus according to the underlying pathology. Being rare its optimal treatment yet to be defined.

  References Top

1.Sharpe M, Easthope SE, Keating GM, Lamb HM. Polyethylene glycol-liposomal doxorubicin: A review of its use in the management of solid and haematological malignancies and AIDS-related Kaposi's sarcoma. Drugs 2002;62:2089-126.  Back to cited text no. 1
2.Maeda K, Matsuda M, Suzuki H, Saitoh HA. Immunohistochemical recognition of human follicular dendritic cells (FDCs) in routinely processed paraffin sections. J Histochem Cytochem 2002;50:1475-86.  Back to cited text no. 2
3.Monda L, Warnke R, Rosai J. A primary lymph node malignancy with features suggestive of dendritic reticulum cell differentiation. A report of 4 cases. Am J Pathol 1986;122:562-72.  Back to cited text no. 3
4.Lee IJ, Kim SC, Kim HS, Bang D, Yang WI, Jung WH, et al. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman's tumor. J Am Acad Dermatol 1999;40:294-7.  Back to cited text no. 4
5.Marzano AV, Vezzoli P, Mariotti F, Boneschi V, Caputo R, Berti E. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma and Castleman disease. Br J Dermatol 2005;153:214-5.  Back to cited text no. 5
6.Yamada H, Nobeyama Y, Matsuo K, Ishiji T, Takeuchi T, Fukuda S, et al. A case of paraneoplastic pemphigus associated with triple malignancies in combination with antilaminin-332 mucous membrane pemphigoid. Br J Dermatol 2012;166:230-1.  Back to cited text no. 6
7.Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer 1997;79:294-313.  Back to cited text no. 7
8.Perez-Ordonez B, Erlandson RA, Rosai J. Follicular dendritic cell tumor: Report of 13 additional cases of a distinctive entity. Am J Surg Pathol 1996;20:944-55.  Back to cited text no. 8
9.Soriano AO, Thompson MA, Admirand JH, Fayad LE, Rodriguez AM, Romaguera JE, et al. Follicular dendritic cell sarcoma: A report of 14 cases and a review of the literature. Am J Hematol 2007;82:725-8.  Back to cited text no. 9
10.Azim HA, Elsedewy E, Azim HA Jr. Imatinib in the treatment of follicular dendritic sarcoma: A case report and review of literature. Onkologie 2007;30:381-4.  Back to cited text no. 10
11.Meijs M, Mekkes J, van Noesel C, Nijhuis E, Leeksma O, Jonkman M, et al. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma without Castleman's disease; treatment with rituximab. Int J Dermatol 2008;47:632-4.  Back to cited text no. 11
12.Kaplan I, Hodak E, Ackerman L, Mimouni D, Anhalt GJ, Calderon S. Neoplasms associated with paraneoplastic pemphigus: A review with emphasis on non-hematologic malignancy and oral mucosal manifestations. Oral Oncol 2004;40:553-62.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 Follicular dendritic cells and related sarcoma
Fabio Facchetti,Luisa Lorenzi
Seminars in Diagnostic Pathology. 2016;
[Pubmed] | [DOI]
2 Paraneoplastic Pemphigus Associated with Follicular Dendritic Cell Tumor in the Mediastinum
Aparna Mullangath Prakasan,Anne Jennifer Prabhu,Kanmani Velarasan,Selvamani Backianathan,Thomas Samuel Ram
Case Reports in Dermatological Medicine. 2016; 2016: 1
[Pubmed] | [DOI]
3 Retroperitoneal and mediastinal follicular dendritic cell sarcoma: report of 3 cases with review of literature
Suvendu Purkait,Saumyaranjan Mallick,Prashant P. Joshi,Supriyo Mallick,N Vijaya Murugan,Meher C. Sharma,Vaishali Suri,Biplab Mishra,Sandeep R. Mathur
Hematological Oncology. 2015; : n/a
[Pubmed] | [DOI]
4 A Rare Case of Follicular Dendritic Cell Sarcoma with Pseudochylous Effusion and Review of Literature From India
Kamal Kant Sahu,Gaurav Prakash,Sandeep Rao,Amanjit Bal,Pankaj Malhotra,Jasmina Ahluwalia,Rakesh K. Vashistha
Indian Journal of Hematology and Blood Transfusion. 2014;
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
  Case Report
   Article Figures

 Article Access Statistics
    PDF Downloaded328    
    Comments [Add]    
    Cited by others 4    

Recommend this journal