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Year : 2014  |  Volume : 57  |  Issue : 2  |  Page : 342-344
A rare case of neurofibroma with lipomatous differentiation and floret like giant cells-cytologically disguising as pleomorphic lipoma

Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India

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Date of Web Publication19-Jun-2014

How to cite this article:
Siddhi Gaurish SK, Bhatt NC, Joshi A. A rare case of neurofibroma with lipomatous differentiation and floret like giant cells-cytologically disguising as pleomorphic lipoma. Indian J Pathol Microbiol 2014;57:342-4

How to cite this URL:
Siddhi Gaurish SK, Bhatt NC, Joshi A. A rare case of neurofibroma with lipomatous differentiation and floret like giant cells-cytologically disguising as pleomorphic lipoma. Indian J Pathol Microbiol [serial online] 2014 [cited 2023 Sep 27];57:342-4. Available from:


Benign nerve sheath tumors of soft tissue can occasionally adopt unusual or unfamiliar morphologic appearance that may introduce difficulties for diagnosis such as multinucleation, bizarre nuclei, intranuclear vacuoles, and other degenerative changes. [1] To the author's knowledge, this is the first case of neurofibroma reported exhibiting both lipomatous differentiation and floret like giant cells (FLGC) and mimicking pleomorphic lipoma cytologically, which has prompted us to report it along with cytohistological correlation and role of immunohistochemical analysis to confirm the diagnosis and prove its benign nature.

A 53-year-old male patient presented with a well-defined, firm, mobile, nontender subcutaneous swelling over lateral aspect of left upper arm since 6 months. The swelling has gradually increased to present size of 1.5 cm × 1 cm. There was no history of trauma and patient had not received intramuscular injection at the site of swelling. Systemic examination findings were unremarkable. Fine-needle aspiration (FNA) was performed, which showed fragments of mature adipocytes admixed with few benign spindle shaped cells and many FLGC with hyperchromatic nuclei. The background was myxoid. A cytological diagnosis of pleomorphic lipoma was offered [Figure 1]. Subsequently, the swelling was excised and sent for histopathological examination. Postoperatively, the patient is disease free with no evidence of recurrence for 9 months.
Figure 1: Fine needle aspiration cytology: Mature adipose tissue; scattered spindle shaped cells and large floret like multinucleated giant cell (Leishman, ×400); inset showing large floret like multinucleated giant cell (Leishman, ×1000)

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On gross examination, the swelling was 2 cm × 2 cm × 1.3 cm, well-defined and glistening tan-white. On cut section white firm areas admixed with yellow soft greasy areas were noted [Figure 2]. On microscopic examination, a well-defined lesion composed of interlacing bundles of elongated spindle shaped cells with wavy darkly stained nuclei and moderate to scanty amount of cytoplasm was seen. Lobules of mature adipocytes were seen embedded in the lesion. At the periphery, nerve fiber bundles were observed. Many FLGC with hyperchromatic nuclei were seen diffusely scattered within the tumor. A diagnosis of neurofibroma with lipomatous differentiation and FLGC was considered [Figure 3]. Benign fibrous histiocytoma was kept as a differential diagnosis (d/d). Representative sections were taken and submitted for immunohistochemical examination. Immunohistochemistry (IHC) was performed with the following panel of antibodies viz. vimentin (clone V9, Dako), S-100 (Dako), CD34 (clone QBEnd10, Novacastra), CD68 (clone 514H12), p53 (clone Do-7, dako) and Ki-67 (clone MM1, Novacastra). The spindle cells showed strong cytoplasmic immunoreactivity for vimentin, and CD34. Both nuclear and cytoplasmic immunoreactivity was seen for S-100. CD68 was nonreactive. The FLGC showed cytoplasmic immnunoreactivity for S-100 whereas CD34, vimentin and CD68 were nonimmunoreactive. p53 was negative and Ki-67 index was <1% [Figure 4]. A diagnosis of neurofibroma with degenerative changes and lipomatous differentiation was made.
Figure 2: Macroscopic features of tumor (external and cut surface): (a) External surface shows a partially capsulated (capsule thickeness varying from area to area), well-defined and glistening tan-white swelling
measuring 2 cm × 2 cm × 1.3 cm. (b) On cut section white firm areas admixed with yellow soft greasy areas were noted

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Figure 3: Microscopic details of tumor: (a) Areas showing interlacing bundles of elongated spindle shaped cells singly scattered mature adipocytes with many floret like giant cells (FLGC) with hyper chromatic nuclei seen diffusely scattered within the tumor (H and E, ×100). Inset showing many FLGC (H and E, ×400). (b) Lobules of mature adipocytes were seen embedded in the lesion

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Figure 4: Immunohistochemical findings: (a) Spindle cells showing strong cytoplasmic immunoreactivity for CD34 and floret like giant cells (FLGC) showing negativity (×400), and (b) spindle cells showing nuclear and cytoplasmic immunoreactivity for S-100 and FLGC showing cytoplasmic immnunoreactivity for S-100 (×400), (c) spindle cells and giant cells showing nonimmunoreactivity for CD68 (×400)

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Sporadic neurofibroma with FLGC is rarely reported in literature and can pose diagnostic dilemma. [2] Numerous FLGC apparent on low magnification (×100) were identified only in 5.3% of cases in a study of 94 cases which included both sporadic and neurofibromatosis-1 (NF-1) associated neurofibromas. [3] FLGC are occasionally reported in the breast tissue, in the background of pseudoangiomatous stromal hyperplasia in men with NF-1 and gynecomastia. [3] Lipomatous neurofibroma is a new entity, its incidence being 6.9%. [4] Lipomatous differentiation is more commonly seen in schwannoma. [1] To the best of our knowledge, both lipomatous differentiation and FLGC in a neurofibroma has not been mentioned in the literature so far. The etiology of the FLGC is unknown. If it is an incidental finding or is unique to patients with NF is not known. [2],[5] Some regard them as morphological reactive change of the indigenous dermal or endoneurial fibroblasts or dendritic cells in response to unknown micro environmental stimuli. [1] Others think of it as reparative change or a change occurring as a result of hypoxia. [2] Trauma is also thought to be one of the causative factors. [2] Interestingly all the cases are mentioned in males, significance of this finding is although unknown. [2] The lipomatous component in neurofibroma can be focally present with spindle cells or regularly scattered, within the neurofibroma. [4] Such neurofibromas with lipomatous differentiation are found more commonly on head and neck unlike our case. [4] It is regarded as either metaplastic change or aberrant adipose differentiation from mutipotential cells of neural crest origin. [4] Some regard them as senescent change or a result of chronic injury. [6] The d/d of tumors with FLGC is giant cell fibroblastoma, pleomorphic lipoma, multinucleate angiohistiocytoma and giant cell collagenoma. [5] In our case, FNA showed presence of lobules of adipocytes and FLGC with few spindle cells on myxoid background. So diagnosis of pleomorphic lipoma was offered. However d/d of fibrohistiocytic lesion was considered. The hyperchromatic, pleomorphic multinucleated giant cell and the morphological overlap between various components in neurofibroma with both lipomatous differentiation and FLGC can mislead to malignant diagnosis. [5] Cytologically we need to differentiate it from other benign spindle cell tumors and low grade spindle cell sarcomas such as solitary fibrous tumor, low grade fibromyxoid sarcoma, dermatofibrosarcoma protuberance, myxoid synovial sarcoma and low grade malignant peripheral nerve sheath tumor. The absence of cytological features like pleomorphism and mitosis along with IHC studies would help in ruling out malignancy. [5] In our case, spindle cells were immunoreactive for vimentin, CD34 and S-100. FLGC showed immunoreactivity for S-100. CD68 was negative in both spindle cells and FLGC. Thus diagnosis of neurofibroma with lipomatous differentiation with FLGC due to degenerative changes was confirmed. Ki-67 labeling index of <1% and negative p53 confirmed its benign nature. In various studies, the FLGC shows varying patterns of immunoreactivity for S-100, CD34 and CD68. [2] Our experience with present case highlights the rarity of lesion, cytohistological correlation and significance of immunohistochemical analysis in diagnosis. It is hoped that more case studies regarding lipomatous differentiation and etiopathogenesis of FLGC in a neurofibroma, will elucidate the understanding further.

   References Top

1.Plaza JA, Wakely PE Jr, Suster S. Lipoblastic nerve sheath tumors: Report of a distinctive variant of neural soft tissue neoplasm with adipocytic differentiation. Am J Surg Pathol 2006;30:337-44.  Back to cited text no. 1
2.Satter E. Floret-like multinucleated giant cells in a neurofibroma outside the context of neurofibromatosis type 1. Am J Dermatopathol 2009;31:724-5.  Back to cited text no. 2
3.Magro G, Amico P, Vecchio GM, Caltabiano R, Castaing M, Kacerovska D, et al. Multinucleated floret-like giant cells in sporadic and NF1-associated neurofibromas: A clinicopathologic study of 94 cases. Virchows Arch 2010;456:71-6.  Back to cited text no. 3
4.Val-Bernal JF, González-Vela MC. Cutaneous lipomatous neurofibroma: Characterization and frequency. J Cutan Pathol 2005;32:274-9.  Back to cited text no. 4
5.Shaktawat SS, Golka D. Floret-like multinucleated giant cells in neurofibroma. Diagn Pathol 2007;2:47.  Back to cited text no. 5
6.Ahn SK, Ahn HJ, Kim TH, Hwang SM, Choi EH, Lee SH. Intratumoral fat in neurofibroma. Am J Dermatopathol 2002;24:326-9.  Back to cited text no. 6

Correspondence Address:
Sinai Khandeparkar Siddhi Gaurish
E-517, The Island, Wakad, Pune - 411 057, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.134745

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

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