Gastrointestinal Stromal Tumour

          

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                   Path Quiz Case-66    

            Atypical Fibroxanthoma

                  Dr Sampurna Roy MD

   Path Quiz Case- 66:Case history and images

 
 March 2008
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Nodular fasciitis

Proliferative fasciitis

Proliferative myositis

Ischaemic fasciitis

Elastofibroma

Fibrous Hamartoma of Infancy

Infantile Myofibromatosis/ Myofibroma

Juvenile hyaline fibromatosis

Inclusion  Body Fibromatosis

Calcifying aponeurotic fibroma

 Fibromatosis colli

Fibroma of tendon sheath

Desmoplastic fibroblastoma (collagenous fibroma)

Storiform Collagenoma (sclerotic fibroma)

Giant Cell Collagenoma

Pleomorphic Fibroma

Angiomyofibroblastoma

Dermatomyofibroma

Cellular Angiofibroma

Giant Cell Angiofibroma

Fibromatosis

Lipofibromatosis

Solitary fibrous tumour

[Hemangiopericytoma  including Lipomatous Hemangiopericytoma]

Inflammatory myofibroblastic tumour

Low grade myofibroblastic sarcoma

Myxoinflammatory fibroblastic sarcoma

Infantile fibrosarcoma

Adult fibrosarcoma

Myxofibrosarcoma

 Low grade fibromyxoid sarcoma

Hyalinizing Spindle Cell Tumour with Giant Rosettes

Sclerosing epithelioid fibrosarcoma

                    
 

Age: Young to middle aged individuals

Site: Commonly located in the upper limb (fingers) and is usually attached to the tendon.

Gross:   Well circumscribed, lobulated fibrous nodule. They are firm and rubbery and on section have a grayish-white appearance.

Microscopic features:
Image Link1 ; Image Link2 ; Image Link3 ; Image Link4 .

The lesions consist of a dense fibrocollagenous tissue with a variable degree of collagenization and cellularity, the peripheral zone often being most cellular and containing slit-like vascular spaces. In some cases there may be complete hyalinization.  Mitotic activity depends on the cellularity of the lesion.

Other features:  Presence of inflammatory cells and myxoid stroma in some cases. Rare presence of giant cells or foamy cells. 


Immmunohistochemistry:  Vimentin - Positive ;  muscle-specific actin  and smooth muscle actin - Positive.  Desmin - Negative ;  Factor XIIIa- Positive ;  CD34- Positive ; CD68 (+/- ).

Cytogenetics: Transclocation  2;11 has been found in one case.

Electron-microscopy:  Two main cell types were identified: Myofibroblasts and fibroblasts, the former dominating in cellular areas, the latter dominating in collagenized areas.

Differential diagnosis: Giant cell tumour of tendon sheath (localised nodular tenosynovitis) ; Circumscribed fibromatosis ; Nodular fasciitis;  Neurofibroma  ; Leiomyoma ;  Scar tissue ;  Benign fibrous histiocytoma ; 

Comparison with Giant cell tumour of tendon sheath:    This lesion is more common than fibroma of tendon sheath. Histologically the two tumours have separate and distinct histologic features.  Fibroma of the tendon sheath is hypocellular, with slit-like vascular channels within a dense collagen matrix , whereas giant cell tumor of the tendon sheath is much more cellular and contains multiple multinucleated giant cells, foamy histiocytes, and hemosiderin.

 All cases are treated effectively by local excision or reexcision of the growth.

                     

 

Abstracts:

Fibroma of tendon sheath. A clinicopathologic study of 32 cases. Am J Surg Pathol. 1989;13(6):472-9.

Fibroma of tendon sheath arising from the radio-ulnar joint .Pathol Int. 1999;49(12) :1089-92.

Translocation 2;11 in a fibroma of tendon sheath.Histopathology.1998;32(5):433-5.

Fibroma and giant cell tumor of tendon sheath: a comparative histological and immunohistological study.Mod Pathol. 1995;8(2):155-9.

Tendon sheath tumours: a pathological study of the relationship between giant cell tumour and fibroma of tendon sheath.Histopathology. 1992 ;20(3):213-20.

Tendon sheath fibroma. A case report with immunohistochemical studies. Hautarzt. 1992;43(2):92-6.

Fibroma of tendon sheath.J Clin Pathol. 1982 Aug;35(8):842-8.

Fibroma of the tendon sheath. Diagnosis and differential diagnosis. Zentralbl Allg Pathol. 1988;134(4-5):393-8.

Fibroma of tendon sheath: a clinicopathologic study. J Cutan Pathol. 1986 ;13(5): 331-8.

Fibroma of tendon sheath: a tumor of myofibroblasts. A clinicopathologic study of 18 cases.
Acta Pathol Jpn. 1985;35(5):1099-107.

Fibroma of tendon sheath. A light and electron-microscopic study of 6 cases.Acta Pathol Microbiol Immunol Scand [A]. 1984;92(6):401-9

Fibroma of tendon sheath.J Am Acad Dermatol. 1984 Oct;11(4 Pt 1):625-8.

Sclerotic fibroma of tendon sheath. Dermatology. 1997;195(3):563-5.

MR imaging of fibroma of the tendon sheath. AJR Am J Roentgenol.2003;180(5) :1449-53.

Fibroma of tendon sheath.Cancer. 1979;44(5):1945-54.

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