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CASE REPORT Table of Contents  
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Fibroblastic reticular cell tumor of eyelid: Rare case report and review of literature

1 Department of Oncopathology, Yenepoya Medical College, Mangalore, Karnataka, India
2 Pathology, Yenepoya Medical College, Mangalore, Karnataka, India

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Date of Submission10-Sep-2021
Date of Decision23-Feb-2022
Date of Acceptance24-Feb-2022
Date of Web Publication27-Jan-2023


Fibroblastic reticular cell tumours (FRCT) originate from the fibroblastic reticular cells (FBRC) which are histiocytic cells, belonging to the dendritic cell family. These tumours are extremely rare, with only a few cases reported in literature. Histomorphologically, they resemble follicular dendritic cell sarcoma (FDCS); however, they differ immunophenotypically. Extranodal presentations are rare. We report a case of malignant FBRC tumour of the left eyelid, in a 23-year-old woman, who had presented with a recurrent swelling over left lower eyelid. Microscopy revealed an ill circumbscribed tumour composed of oval to spindle cells in storiform pattern, sprinkled with lymphocytes. Immunohistochemistry was performed and diagnosis of FRCT was offered. To the best of our knowledge, this is the first report of malignant FBRC tumour arising in the eyelid region. Here we present this extremely rare case with review of the available literature.

Keywords: Dendritic cells, eyelid tumour, fibroblastic reticular cell tumours

How to cite this URL:
Mishra PP, Madan K, Biswas S, Kini Rao AC. Fibroblastic reticular cell tumor of eyelid: Rare case report and review of literature. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Mar 27]. Available from:

   Introduction Top

Dendritic cells are antigen-presenting cells present in lymphnode and skin, subdivided into four major subsets on the basis of their location and phenotype: Langerhans cells (LC), Interdigitating dendritic cells (IDCs), Follicular dendritic cells (FDCs) and Fibroblastic reticular cells (FBRCs).[1] FBRCs are located in the capsule and hilar areas of lymph nodes. Primary extranodal FBRC tumours (FRCTs) are a rare occurance.

Most of the well-characterized cases of Dendritic cell tumours reported in the literature represent FDC tumours.[2] The earliest reported cases in the literature thought to be of FBRC origin were included in a series by Turner et al.[3] and Andriko et al.[4] Here, we report a case of recurrent malignant tumour of the eyelid having immunomorphologic features consistent with FBRC derivation.

To the best of our knowledge, this is the first case of a malignant FBRC tumour in eyelid region.

   Case Report Top

A 23-year-old young female presented with recurrent, small, firm swelling in left lower eyelid. She was evaluated elsewhere four months back for a similar swelling. Histopathology of the lesion was suggestive of high grade poorly differentiated malignant neoplasm. Immunologically, the tumour cells showed weak expression of pan-cytokeratin only. Four months later, the patient presented to our Oncology centre with a recurrent lesion on the same site and was evaluated for the same. Local examination of the patient showed two discrete swellings, one measuring 1 × 1 cm and other measuring 0.5 × 0.5 cm in the left lower eyelid region, [Figure 1] near the previous operated scar. The lesion was not adherent to skin or underlying structures. No cervical lymphadenopathy was seen. Routine haemogram and biochemical test results were normal. Fluorodeoxyglucose (FDG), Positron Emission Tomography (PET) showed discrete FDG avid non-enhancing lesion in left lower eyelid. A workup for extra ocular malignancy as a source of metastasis to the eyelid was unrevealing.
Figure 1: Small, 1 × 1 cm nodule in the left lower eyelid region

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With proper pre-surgical workup, the patient was taken up for wide local excision with local advancement flap and the specimen was sent for Oncopathology analysis.

A skin covered, wide local excision specimen was received in the pathology department. Cut surface showed two subcutaneous pale white lesions, larger measuring 1 × 1 cm and smaller measuring 0.5 × 0.5 cm. Histopathologic sections studied revealed two ill circumscribed, focally infiltrative tumour nodules in the subcutaneous and intramuscular planes. The tumour cells were oval and spindle shape [Figure 2] arranged in ill-defined storiform pattern and showed moderate anisonucleosis. Focally, they appeared epithelioid. Brisk mitotic activity was noted along with sprinkling of lymphocytes interspersed. Wide areas of necrosis were seen. The tumour was seen infiltrating the underlying skeletal muscle bundles. All the resected margins were free of tumours. With this morphology, diagnosis of malignant spindle cell neoplasm – probably of lymphohistiocytic origin, favouring FDCS was offered.
Figure 2: Photomicrograph: H and E; 40×: Oval to spindled tumour cells with sprinkled lymphocyte

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A limited initial panel of IHC markers was performed. The tumour cells showed strong immunoreactivity to AE1/AE3 [Figure 3], EMA and focal staining for CD68 [Figure 4], SMA. They were negative for LCA, CD21 and CD23.
Figure 3: Photomicrograph: IHC; 40×: Strong and diffuse Cytokeratin positivity

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Figure 4: Photomicrograph: IHC; 20×: CD 68 positivity in tumour cells

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Correlating histomorphology with the immunological findings, we rendered a final diagnosis of Fibroblastic Reticular Cell Tumour – Malignant.

The patient is currently undergoing radiotherapy and has been on regular follow-up.

   Discussion Top

The first documented FRCT case was detected in the thoracic lymph nodes and was reported by Gould et al.[5] Extranodal involvements include liver, lung, spleen, soft tissue and bone. Very limited data is available on FRCT due to its rare incidence, and no aetiology association has been confirmed. They occur in a wide age range group (13–80 yrs) with a median age of 61 yrs and a slight male predilection. Our patient was a young female who presented to us with a recurrent lower eyelid nodular lesion.

Microscopically, FRCT cells are present in whorls, fascicles or a storiform pattern, of spindle or ovoid, rarely epitheloid cells, with lymphoplasmacytic infiltration in between.[4]

FRCT cells are demonstrated to have certain myofibroblastic-like features with immunoreactivity for Vimentin, Smooth muscle actin and Desmin, whereas they are negative for CD21, CD35 and S-100 protein.[6] Differentiating carcinomas from FBRC tumour subtypes that express Cytokeratins is challenging.[7]

With this morphology, differential diagnoses of carcinoma, leiomyosarcoma and inflammatory myofibroblastic sarcoma were considered. In spite of the diffuse CK positivity, an absence of glandular features and high-grade areas with SMA positivity, excludes carcinoma. The presence of inflammatory cells with concurrent CD68 positivity ruled out Leiomyosarcoma. Inflammatory myofibroblastic sarcoma (IMT) was the closest morphologic differential but the absence of ganglion cell and CD68 positivity ruled it out.

The morphology of FDCs and IDCs are similar to that of FBRCs; however, FDCs are immunoreactive for CD21, CD23 and CD35 whereas IDCs are immunoreactive for S-100 protein, variably immunoreactive for CD1a and histiocytic markers.[8]

Surgical resection is considered as a primary treatment modality for FDCS and FRCT. Localized FRCT cases have been treated with radiotherapy. The current patient received radiotherapy after wide excision. The patient was followed up for four months and the recovery was uneventful.

   Conclusion Top

The present study reports the first case of FRBC tumour in the eyelid region. FRBC tumours are extremely rare and are misdiagnosed owing to the diagnostic complexity. In conclusion, the diagnosis, treatment and prognostic details of the tumour presented in the case report will assist in improving further knowledge of the characteristics of this less known, rarely diagnosed entity.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Sato K, Fujita S. Dendritic cells: Nature and classification. Allergol Int 2007;56:183-91.  Back to cited text no. 1
Fonseca R, Yamakawa M, Nakamura S, van Heerde P, Miettinen M, Shek TW, et al. Follicular dendritic cell sarcoma and interdigitating reticulum cell sarcoma: A review. Am J Hematol 1998;59:161-7.  Back to cited text no. 2
Turner RR, Wood GS, Beckstead JH, Colby TV, Horning SJ, Warnke RA. Histiocytic malignancies: Morphologic, immunologic and enzymatic heterogeneity. Am J Surg Pathol 1984;8:485-500.  Back to cited text no. 3
Andriko JW, Kaldjian EP, Tsokos M, Abbondanzo SL, Jaffe ES. Reticulum cell neoplasms of lymph nodes: A clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells. Am J Surg Pathol 1998;22:1048-58.  Back to cited text no. 4
Gould VE, Warren WH, Faber LP, Kuhn C, Franke WW. Malignant cells of epithelial phenotype limited to thoracic lymph nodes. Eur J Cancer 1990;26:1121-6.  Back to cited text no. 5
Sundersingh S, Majhi U, Krishnamurthy A, Velusami SD. Cytokeratin-positive interstitial reticulum cell sarcoma: Extranodal presentations mimicking carcinoma. Indian J Pathol Microbiol 2013;56:172-5.  Back to cited text no. 6
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Kapucuoglu N, Percinel S, Ventura T, Lang R, Al-Daraji W, Eusebi V. Dendritic cell sarcomas/tumours of the breast: Report of two cases. Virchows Arch 2009;454:333-9.  Back to cited text no. 7
Gaertner EM, Tsokos M, Derringer GA, Neuhauser TS, Arciero C, Andriko JA. Interdigitating dendritic cell sarcoma: A report of four cases and review of the literature. Am J Clin Pathol 2001;115:589-97.  Back to cited text no. 8

Correspondence Address:
K Madan,
Assistant Professor in Oncopathology, Yenepoya Medical College, Mangaluru - 575 018, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_908_21


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


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