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| Year : 2011 | Volume
: 54
| Issue : 1 | Page : 206-207 |
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| Desmoplastic fibroblastoma (collagenous fibroma): Report of a case |
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Naorem Gopendro Singh, A. A. S. Rifat Mannan, Mirza Kahvic
Department of Pathology, Al-Jahra Hospital, Kuwait
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| Date of Web Publication | 7-Mar-2011 |
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How to cite this article:
Singh NG, Mannan AR, Kahvic M. Desmoplastic fibroblastoma (collagenous fibroma): Report of a case. Indian J Pathol Microbiol 2011;54:206-7 |
Desmoplastic fibroblastoma (DF) is a benign relatively soft tissue tumor and fewer than 100 cases have been reported in the English literature.
We report a case of desmoplastic fibroblastoma (DF) in a 31-year-old male. The patient came with the complaint of a swelling over the left leg of 6-months duration. On examination, a hard, non-tender subcutaneous swelling measuring about 4.5 cm in diameter was identified. His laboratory investigation was non-contributory. The patient underwent an excisional biopsy of the lump. At surgery, a hard, well-circumscribed, sclerotic lesion measuring about 4.5 × 4.5 cm was identified subcutaneously. His postoperative period was uneventful and he was discharged on the fifth postoperative day.
We received a well-circumscribed, grey white soft tissue measuring about 4.5 × 4.5 × 4.3 cm. The mass was cut with gritty sensation. The cut section of the mass was homogeneous, grey white, firm to hard in consistency with focal areas of hemorrhage [Figure 1]a. Microscopically the lesion was nodular and well circumscribed. It was hypocellular and the predominating area displayed hyalinized collagen. The sparse cellular component revealed haphazardly arranged 'reactive like' spindle and stellate-shaped cells with thin elongated nuclei having delicate nuclear chromatin [Figure 1]b. The cells were embedded in a hypovascular, densely fibrotic collagenous stroma [Figure 1]c. No mitosis or necrosis was observed. The cells were immunoreactive to vimentin and smooth muscle actin (SMA) [Figure 1]d and nonreactive to S100, CD34, and epithelial membrane antigen (EMA). A diagnosis of DF was made. | Figure 1: (a) Cut section of the tumor showing homogenous grey white area with foci of hemorrhage; (b) photomicrograph showing hypocellular, spindle- and stellate-shaped cells embedded in a collagenous stroma (H and E, ×200); (c) Masson's tricrome stain confirming the collagenous nature of the tumor (Masson's trichrome, × 200); (d) photomicrograph of spindle- and stellate-shaped cells showing immmunoreactive to SMA (avidin and biotin, ×200).
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Desmoplastic fibroblastoma also known as collagenous fibroma is a benign relatively paucicellular, soft tissue tumor characterized by spindle- to stellate-shaped fibroblasts and myofibroblasts embedded in a prominent collagenous background. [1] It is a recently defined benign fibroblastic/myofibroblastic tumor. The lesion usually presents as a painless, firm, well-circumscribed subcutaneous mass of long standing duration and behaves in a benign fashion. [2] There is no evidence of bone involvement except for a rare case with surface erosion. It usually appears in adult males with peak incidence at fifth and sixth decade of life. It occurs in a variety of peripheral sites with the most common location being the arm, shoulder, lower limb, back, forearm, hands, and feet. [2] Other rare sites of DF include tongue, palate, and parotid.
The lesion is considered to be a neoplasm because of its association with the 11q12 breakpoints, a lack of inciting events, and the initiation of reactive fibrous proliferation. [3] This idea is also supported by the fact that a flow cytometry analysis has demonstrated a diploid DNA consistent with a low S-phase fraction.
Histologically, the tumors often appear well delineated under a low power microscope. The tumor cells are relatively stellate or bland spindle fibroblasts separated by a collagenous or myxocollagenous matrix. These fibroblasts lack any atypical or hyperchromatic nuclei. Mitotic activity is either absent or minimal. Ultrastructurally and immunohistochemically the tumor cells present features compatible with a fibroblastic-myofibroblastic profile. [1],[2] The cells are positive for vimentin, and focal immunoreactivity to SMA and muscle-specific actin. Immunostaining for desmin, S100 protein, CD34, and EMA is negative. [1],[2] Some studies reported that SMA-positive cells are more frequent at the periphery of the tumor suggesting that the most active area of DF are located peripherally. However in the present case, most cells were positive for SMA regardless of their location in the tumor, suggesting that active area were spread throughout the specimen.
The differential diagnosis of DF includes benign, locally aggressive, and lowgrade malignant soft tissue tumors consisting of relatively uniform stellate or spindle-shaped cells embedded in a myxocollagenous matrix, such as nodular fascitis, fibromatosis, fibroma of tendon sheath, nuchal fibroma, sclerotic fibroma of the skin, neurofibroma, solitary fibrous tumor, and low-grade fibromyxoid sarcoma. [2]
A peculiar histopathologic finding observed in the present case was the presence of areas resembling sclerotic fibroma. Sclerotic fibroma is a benign fibrous neoplasm consisting of thick collagen fibres arranged in a storiform pattern with scattered spindle to stellate-shaped mononuclear cells with occasional multinucleated giant cells. The tumor cells are usually positive for vimentin and CD34, and negative for S-100 protein, SMA, and EMA. [4] Although considered to be a distinct entity, sclerotic fibroma-like changes have been described for other tumors such as dermatofibromas, neurofibromas, intradermal nevus, and angiofibromas. [5] In the present case, the tumor cells found in the sclerotic fibroma-like areas were positive for SMA and negative for CD34. This immunoprofile is in contrast to that commonly reported for sclerotic fibromas.
In comparison to a DF, a desmoid tumor is more cellular, more vascular, more infiltrative, and less circumscribed at the periphery than a DF. A low-grade fibromyxoid sarcoma is composed of generally smaller cells that have denser chromatin than reactive fibroblasts. Nodular fasciitis is also more cellular than DF and frequently shows mitosis, inflammatory infiltration, or fascicles of collagen fibers which point in a variety of directions.
Although DF is characterized by distinct histopathologic features, the findings observed in this case highlight the importance for careful microscopic analysis of these tumors, which may be combined with histochemistry and immunohistochemistry.
 References | |  |
| 1. | Evans HL. Desmoplastic fibroblastoma. A report of seven cases. Am J Surg Pathol 1995;19:1077-81. 
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| 2. | Miettinen M, Fetsch JF. Collagenous fibroma (desmoplastic fibroblastoma): A clinicopathologic study of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. Hum Pathol 1998;29:676-82.
[PUBMED] |
| 3. | Bernal K, Nelson M, Neff JR, Nielsen SM, Bridge JA. Translocation (2;11)(q31;q12) is recurrent in collagenous fibroma (desmoplastic fibroblastoma). Cancer Genet Cytogenet 2004;149:161-3.
[PUBMED] [FULLTEXT] |
| 4. | Alawi F, Freedman PD. Sporadic sclerotic fibroma of the oral soft tissues. Am J Dermatopathol 2004;26:182-7.
[PUBMED] [FULLTEXT] |
| 5. | High WA, Stewart D, Essary LR, Kageyama NP, Hoang MP, Cockerell CJ. Sclerotic fibroma-like change in various neoplastic and inflammatory skin lesions: Is sclerotic fibroma a distinct entity? J Cutan Pathol 2004;31:373-8.
[PUBMED] [FULLTEXT] |
Correspondence Address:
Naorem Gopendro Singh
Department of Pathology, Al-Jahra Hospital, P.O.Box 62276, Jahra 02153
Kuwait
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.77408
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