Aggressive desmoplastic fibromatosis - a clinicians dilemma case report and review of literature

Abstract

Fibromatoses are a heterogeneous group of distinct entities which differ in biological behaviour, but arehistologically very similar. This group of fibrous tumor or tumor like lesions, present considerable difficulties in pathologic diagnosis. Aggressive fibromatosis (AF) of the oral or para-oral structures is a very uncommon finding and its intra-osseous component is even relatively unusual. Such lesions with their origin from within the bone are termed desmoplastic fibromatosis (DF). These lesions must be distinguished from other fibroblastic tumors of the head and neck such as benign fibrous histiocytoma (BFH), fibrosarcoma, nerve sheath tumors and tumors of muscular origin. The major challenge in dealing with lesions of fibromatosis is to avoid an overdiagnosis of fibrosarcoma or an underdiagnosis of reactive fibrosis.Problems of differential diagnosis concern a wide range of diseases and immunohistochemical analysis may be helpful in diagnosis. With respect to the patient's post-operative well-being and if periodic follow-ups are guaranteed, the tumor should be carefully resected with only narrow safety margins. A rare case of aggressive desmoplastic fibromatosis in a 12-year-old girl is presented in this article with emphasis on the need and challenges for diagnosing such lesions as they have to be differentiated from other soft tissue tumors which display borderline pathological features regarding benign or malignant behaviour. Synonyms listed for the same include extra-abdominal desmoids, extra-abdominal fibromatosis, desmoids tumor, aggressive fibromatosis, juvenile desmoids-type fibromatosis, infantile fibromatosis.

Keywords: Aggressive; Desmoplastic; Fibromatosis.

[Table/Fig-1]:

Photograph showing swelling in the left anterior maxillary region. Inset showing an expansion of the buccal cortical plate with normal palatal borders

[Table/Fig-2]:

Panaromic radiograph showing a diffuse radiolucent lesion with displacement of roots of # 24 and #26. Inset shows periapical radiograph revealing radiolucent lesion with thick ill defined septae

[Table/Fig-3]:

Waters view showed haziness of the left maxillary sinus with expansion and thinning of the sinus walls

[Table/Fig-4]:

Spindle cells arranged in storiform to parallel fashion, mixed with short interlacing fascicles and intertwining strands (H& E, X10). Inset showing the same in X40

[Table/Fig-5]:

Diffuse positive staining seen for vimentin (IHC, X40)

[Table/Fig-6]:

Non reactive stain for CD68 (IHC, X40)