|Year : 2015 | Volume
| Issue : 1 | Page : 14-17
Verocay bodies and myxoid change in fibrosarcomatous variant of dermatofibrosarcoma protuberans: A case report and review of literature
Mary Theresa Sylvia, Bhawana Ashok Badhe
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
|Date of Web Publication||16-Jul-2015|
Dr. Mary Theresa Sylvia
Plot No. 19, 2nd Floor, Poorani Ammal Street, Srinivasapuram, Gorimedu, Puducherry - 605 011
Source of Support: None, Conflict of Interest: None
Dermatofibrosarcoma protuberans (DFSP) is a fibro-histiocytic tumor of intermediate malignant potential. Unusual morphologic patterns can be misleading. Sarcomatous change in DFSP is a rare occurrence. Presence of Verocay bodies and myxoid change in DFSP is uncommon. We report a case where the trucut biopsy was initially reported as neurogenic tumor based on prominent Verocay bodies, myxoid change and increased mitoses. Resection specimen had classic areas of DFSP with foci of Verocay bodies, prominent myxoid change, myofibroblastic differentiation, sarcomatous transformation and a satellite nodule. We also briefly discuss the relevant literature.
Keywords: Fibrosarcomatous variant, myxoid change, verocay bodies
|How to cite this article:|
Sylvia MT, Badhe BA. Verocay bodies and myxoid change in fibrosarcomatous variant of dermatofibrosarcoma protuberans: A case report and review of literature. Indian J Dermatopathol Diagn Dermatol 2015;2:14-7
|How to cite this URL:|
Sylvia MT, Badhe BA. Verocay bodies and myxoid change in fibrosarcomatous variant of dermatofibrosarcoma protuberans: A case report and review of literature. Indian J Dermatopathol Diagn Dermatol [serial online] 2015 [cited 2020 Apr 6];2:14-7. Available from: http://www.ijdpdd.com/text.asp?2015/2/1/14/160983
| Introduction|| |
Dermatofibrosarcoma protuberans (DFSP) constitutes 1% of the soft tissue sarcomas. , It is more common in early and mid adult age; however, there are increasing case reports in pediatric population.  Unusual morphological variants of DFSP can cause diagnostic dilemma especially on trucut biopsies. Sarcomatous transformation in DFSP is a very rare occurrence and has uncertain clinical outcome.
| Case Report|| |
A 14-year-old boy presented with recurrent mass in the right shoulder and adjacent chest wall. The initial mass had been excised incompletely at a private hospital and reported as schwannoma. Fine needle aspiration of the recurrent mass showed features of a neurogenic tumor. Trucut biopsy from the mass showed a spindle cell lesion in background of extensive myxoid change. There was prominent nuclear palisading forming Verocay bodies. Some foci had nuclear atypia and increased mitosis. The trucut biopsy was reported as a low grade malignant peripheral nerve sheath tumor arising from a benign nerve sheath tumor and was advised complete surgical excision.
Gross examination of the resected specimens showed infiltrative grey white glistening lesions in the dermis and subcutis adherent to the overlying skin.
Microscopic examination showed a tumor composed of fascicles of spindle cells arranged in storiform/cartwheel pattern [Figure 1]a with tongues of lace-like infiltration into the subcutaneous fat [Figure 1]b. Focally they were adherent to the epidermis, trying to protrude out [Figure 1]e and f Other areas had a clear zone in between the tumor and the epidermis. There were foci of myxoid change (40%) [Figure 1]c, nuclear palisading replicating Verocay bodies [Figure 1]d and giant cells with 1-2 mitosis/10hpf. There were foci of increased cellularity [Figure 2]a, herring-bone pattern [Figure 2]b, nuclear atypia [Figure 2]c and increased mitosis (11/10hpf) [Figure 2]c. These areas occupied >5% of the tumor.
|Figure 1: (a) Dermal spindle cell tumor. (H and E, ×100) (b) Lace-like infiltration in subcutaneous fat (c) Myxoid change (d) Verocay body like areas. (H and E, ×400) (e) High power view of the tumor protruding through the epidermis. (H and E, ×400) (f) High power view showing the storiform areas. (H and E, ×400)|
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|Figure 2: (a) Storiform areas with increased cellularity (b) Focal herring bone pattern (c) Increased mitosis. (H and E, ×400) (d) Focal fuschinophilic areas. (Masson Trichrome ×400)|
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Masson's trichrome stain showed increased collagen deposition in between the tumor cells. There were focal fuschinophilic areas [Figure 2]d. Immunohistochemical stains showed uniform positivity for vimentin, [Figure 3]a and patchy for CD34 (negative in the sarcomatous areas) [Figure 3]b. S-100 was negative [Figure 3]c. Focal positivity for smooth muscle actin was seen proving myoid differentiation [Figure 3]d.
|Figure 3: (a) Diffuse positivity for Vimentin (b) Patchy CD34 positivity (c) Negative for S-100. Control fat cells are positive (d) Focal positivity for smooth muscle actin. (IHC ×400)|
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The tumor was finally diagnosed as fibrosarcomatous variant of DFSP with satellite nodule.
| Discussion|| |
Dermatofibrosarcoma protuberans is a very rare and locally aggressive fibro-histiocytic tumor.  The origin is considered to be fibroblastic, neuro-ectodermal, histiocytic or from pluripotent progenitor cells that have the capacity to differentiate into these three cell types.  The histological variants of DFSP are pigmented (Bednar tumor), myxoid, myoid, granular cell, sclerotic, atrophic DFSP, giant cell fibroblastoma, and DFSP with fibrosarcomatous areas.  DFSP is described as a tumor with a uniform storiform appearance, low mitosis (1-3/hpf), no atypia or necrosis. 
Verocay bodies are formed by nuclear palisading around a collagenous stroma. It was first described by Jose Juan Verocay in 1910. They are considered to be pathognomic of Schwanomma. The possible cause is increased expression of laminin causing the adhesion of the nuclei. Such palisading has been reported in few cutaneous neoplasms described as the rippled pattern.  Verocay bodies have not yet been included in the histo-morphological description of DFSP. There are four cases reported in literature. Three cases were of pigmented variant of DFSP  and one case was classic DFSP.  It has not been previously reported in a fibrosarcomatous variant of DFSP.
Myxoid change can be seen focally beneath the epidermis except in the myxoid variant where it is >50%.  Such extensive myxoid areas sampled in the trucut lead to a consideration of neural tumor.
Inflammatory infiltrates, hemosiderin deposits, multinucleated giant cells, and foamy histiocytes are reported to be uncommon in literature in DFSP.  Our case had many multinucleated giant cells.
The incidence of sarcomatous change in DFSP ranges from 10 to 20%. It can have foci of fibrosarcoma (FS-DFSP) or very rarely malignant fibrous histiocytoma. The diagnostic criteria for FS-DFSP are the presence of sarcomatous foci in >5% of the tumor, loss of storiform pattern with fascicular or herring bone architecture, nuclear atypia, increased mitosis in the range of 7 to 15/10hpf compared to 1-3/hpf in the areas of classic DFSP and loss of CD34 expression. , Average age of cases reported in literature is 30 years. Our case documents the rare occurrence in the pediatric age group. ,,
More than 90% of DFSP and FS-DFSP are characterized by reciprocal chromosomal translocation between chromosomes 17 and 22, t(17;22) (COL1A1-PDGF-B fusion) or supernumerary ring chromosomes. 
The prognostic significance of fibrosarcomatous change in DFSP is not clear. Goldblum et al. reported 18 cases of FS-DFSP and found no prognostic difference.  However, Palmerini et al. found more recurrences and metastasis in their series of 13 cases.  Similarly, Szollosi et al. reported an aggressive course in eight cases and stated that adequate surgical excision was important for improved outcomes. 
Hence to consider it as a variant of DFSP or sarcomatous transformation is still debatable. 
DFSP has tongues of infiltration into the subcutaneous fat; hence it is treated by wide local excision. Moh's micrographic surgery where the margins are pathologically confirmed, is also used.  Radiotherapy and imatinib are effective in very aggressive lesions with metastasis. 
Our case had prominent Verocay bodies, myxoid change, giant cells, myoid differentiation, fibrosarcomatous transformation and satellite nodule. This supports the fact that DFSP originates from pluripotent cells capable of differentiation into these three cell lines; the stratification into several variants is arbitrary and the classic variant can have foci of these variants. Hence the accurate diagnosis would help in targeted therapy and surgery and avoid unnecessary chemotherapy.
| Conclusions|| |
The morphological patterns described as variants of DFSP can occur together in the same tumor. Fibrosarcomatous change is very rare with aggressive course. Verocay bodies in a fibrosarcomatous variant of DFSP has not been previously reported in literature to the best of our knowledge.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]