Gastrointestinal Stromal Tumour

          

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        Atypical Fibroxanthoma

              Dr Sampurna Roy  MD 

 
April 2008  

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Adnexal Tumours of the eyelid

Mesenchymal Tumour of the Eyelid

Pulmonary Mesenchymal Tumours

Cardiac fibroma

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

Fibrous Histiocytoma (Dermatofibroma) Click here

Epithelioid Cell Histiocytoma Click here

Plexiform Fibrohistiocytic Tumour Click here

Giant Cell Fibroblastoma Click here 

Dermatofibrosarcoma Protuberans Click here 

Atypical Fibroxanthoma Click here 

Malignant Fibrous Histiocytoma  (Pleomorphic ; Angiomatoid ; Myxoid (myxofibrosarcoma) ; Giant cell ; Inflammatory)Click here

Immunohistochemistry

Vimentin:(+)
CD34:(-) or (+/-)
Factor XIIIa:(+)
S100:(-)or(-/+)
HMB45:(-)
HAM56:(+)
HHF-35:(+)to(+/-)
SMA:(+/-)
Desmin: (+/-)
Cytokeratin:(-)

CELLULAR FIBROUS HISTIOCYTOMA: (ESCOP)

IMAGE LINK1  2  3  4 

Characteristic features of cellular variant:

- Extends into the subcutis
- Mitotic figures are present.
- Central necrosis may be present in some cases.
- Lesion may recur, if incompletely excised.
- Focal smooth muscle actin positive in 60% cases. CD34 is negative

D/D-
Dermatofibrosarcoma Protuberans.
Abstract: Cellular benign fibrous histiocytoma. Clinicopathologic analysis of 74 cases of a distinctive variant of cutaneous fibrous histiocytoma with frequent recurrence.Am J Surg Pathol. 1994;18(7):668-76

Metastasizing cellular dermatofibroma. A report of two cases.Am J Surg Pathol.1996 ;20(11):1361-7.

ATYPICAL CUTANEOUS FIBROUS HISTIOCYTOMA:

This is a variant of cutaneous fibrous histiocytoma (dermatofibroma).

Usually presents in middle-aged patients as small nodules occurring on the trunk ,limbs , head & neck , vulva.

Histologically these are primarily dermal lesions. Superficial involvement of the subcutis may occur. The degree of pleomorphism varied from only focal and minimal  or moderate to marked . Number of mitotic figures ranged from 1 to 15 per 10 high power fields.

Similar to the cellular and aneurysmal variants of fibrous histiocytoma, atypical fibrous histiocytoma shows a higher tendency to recur locally than ordinary fibrous histiocytoma and may rarely metastasize.

D/DPleomorphic sarcoma ; Atypical fibroxanthoma  ; Malignant fibrous histiocytomas.

Abstract:

Atypical fibrous histiocytoma of the skin: clinicopathologic analysis of 59 cases with evidence of infrequent metastasis. Am J Surg Pathol. 2002;26 (1):35-46

The value of immunohistochemistry in atypical cutaneous fibrous histiocytoma. Am J Dermatopathol. 2004;26(5):367-71

Atypical cutaneous fibrous histiocytoma.Am J Dermatopathol. 1986;8(6):467-71

Atypical 'pseudosarcomatous' variant of cutaneous benign fibrous histiocytoma: report of eight cases.Histopathology. 1990 Aug;17(2):167-9

Diagnosis of Path Case24:  Aneurysmal variant of dermatofibroma - Case history and images:

                          

This is a common dermal tumour which  usually occurs on the extremities in young adults.  Fibrous histiocytoma is regarded as a neoplastic lesion by some authors and an  inflammatory or reactive process by others.
Gross: These are usually solitary round or ovoid firm nodule,less than 1cm in diameter. Aneurysmal variant may be upto 10 cms in diameter. Dark brown or red in colour. Cream or yellowish cut surface in tumour with abundant lipid.

Microscopic features:(Dr Weems) IMAGE LINK1 ; 2      
1. Symmetric lesion in the dermis.
2. Epidermal hyperplasia is usually present.
3. Grenz zone is noted in almost 70% cases.
4. Rarely extend into superficial subcutis (as a well defined bulge or septal extension).
5. Storiform growth pattern.
6. Mixed population of cells  (fibrocytic,histiocytic or mixed).
7. Hyalinized collagen fibers surrounded by spindle cells at the periphery - (keloidal pattern) 
8. Birefringence of intralesional collagen with polarization present .

Various changes may be present in the overlying epidermis:

1. Acanthosis with basal hyperpigmentation; 2.Seborrheic keratosis like pattern; 3. Epidermal atrophy; 4. Intraepidermal carcinoma; 5. Basaloid hyperplasia with rudimentary pilar structure  (difficult to distinguish from basal cell carcinoma) ; 8. Sebaceous structures   IMAGE1 ; IMAGE2  (Dr Weems) 

Abstract: Anti-cytokeratin 20 staining of Merkel cells helps differentiate basaloid proliferations overlying dermatofibromas from basal cell carcinoma. J Cutan Pathol. 2005 Aug;32(7):491-5

Follicular basal cell hyperplasia overlying dermatofibroma.Am J Surg Pathol.1997;21(6): 711-8

Sebaceous hyperplasia: a clue to the diagnosis of dermatofibroma.J Am Acad Dermatol. 2001 Jan;44(1):94-5

Plate-like sebaceous hyperplasia overlying dermatofibroma. J Cutan Pathol. 1992;19(3):253-5

Proliferation and differentiation of the keratinocytes in hyperplastic epidermis overlying dermatofibroma: immunohistochemical characterization. Am J Dermatopathol. 2001 ;23(2):90-8.

Dermatofibroma may be associated with melanocytic lesions. Melanocytic lesions associated with dermatofibromas: a spectrum of lesions ranging from junctional nevus to malignant melanoma in situ. Mod Pathol. 2005;18(8):1043-7
Dermatofibroma may occur in patients associated with HIV infection. 
Multiple eruptive dermatofibromas in three men with HIV infection. Dermatology.1997; 195(4):344-8

Presence of Leishmania organisms in specific and non-specific skin lesions in HIV-infected individuals with visceral leishmaniasis. Int J Dermatol. 2002 ;41 (10) :670-5

Variants of dermatofibroma:

FIBROCOLLAGENOUS: Mainly collagen and fibroblast like cells in irregular or whorled pattern.

STORIFORM: Prominent storiform pattern. These are often termed fibrous histiocytoma.

LIPIDIZED: Presence of numerous foam cells. Lipidized fibrous histiocytoma: clinicopathologic analysis of 22 cases. Am J Dermatopathol. 2000;22(2):126-34.

ANGIOMATOUS: Presence of small branching vessels in a collagenous stroma. Hemosiderin may be present.

ANEURYSMAL VARIANT: Image Link1 ; Image Link2 ; Image Link3 ; Image Link4 ; Image Link5 ; Image Link6.

IMAGE LINK(Dr.Weems); IMAGE LINK1   2   3   4   5 (ESCOP)
-Narrow clefts to large cavernous cysts
-Histiocytes,fibroblasts, foam cells and hemosiderin

-Mitotic figures may be present
-Solid areas with usual features of dermatofibroma.  D/D-
Kaposi Sarcoma ; angiomatoid malignant fibrous histiocytoma ; spindle cell hemangioendothelioma  

Abstracts: Recurrent aneurysmal fibrous histiocytoma.J Clin Pathol. 2004; 57(3): 312-3.

Aneurysmal fibrous histiocytoma showing transition from factor XIIIa-positive to -negative.J Dermatol. 2002 Nov;29(11):744-7.

Aneurysmal and haemangiopericytoma-like fibrous histiocytoma. J Clin Pathol. 1996;49 (4):313-8.

Aneurysmal fibrous histiocytoma of the skin. A histological, immuno- histochemical,and ultrastructural study. Am J Dermatopathol 1995;17(2):179-84

Aneurysmal benign fibrous histiocytoma: clinicopathological analysis of 40 cases of a tumour frequently misdiagnosed as a vascular neoplasm. Histopathology.1995;26(4):323-31

Aneurysmal ("angiomatoid") fibrous histiocytoma of the skin.Cancer. 1981;47 (8):2053-61.

CLEAR CELL: D/D - Metastatic renal cell carcinoma, xanthogranulomatous reactions, balloon cell naevus/melanoma and clear cell sarcoma

Abstracts: Clear cell dermatofibroma. Histopathology.1997;30(1):64-9.

Clear cell dermatofibroma. Case report of an unusual fibrohistiocytic lesion. Am J Surg Pathol. 1996;20(4):483-91

CHOLESTEROTIC ASSOCIATED WITH HYPERLIPOPROTEINEMIA: Cholesterotic fibrous histiocytoma. Its association with hyperlipoproteinemia.Arch Dermatol. 1990 Apr;126(4):506-8.

GRANULAR CELL: IMAGE LINK (Dr Weems)

Granular cell dermatofibroma.Am J Dermatopathol.1997;19 (2): 168-73.

Granular cell dermatofibroma.Histopathology.1997;31(3):258-62

Dermatofibroma with granular cells: a report of two cases. Dermatology. 1999; 199(1):54-6

MONSTER CELL: Atypical bizarre cells with foamy cytoplasm, hyperchromatic nuclei. IMAGE LINKS (Dr Weems)

Abstract: Giant dermatofibroma with monster cells. Am J Dermatopathol. 2002;24(1):36-8

Dermatofibroma with monster cells.Am J Dermatopathol. 1987 Oct;9(5):380-7

Case of dermatofibroma with monster cells: a review and an immunohistochemical study.Am J Dermatopathol. 1997 Jun;19(3):312-5.

WITH OSTEOCLASTIC LIKE GIANT CELLS:
IMAGE LINKS (Dr Weems)

Dermatofibroma with osteoclast-like giant cells. Am J Dermatopathol. 1992 Oct;14(5):397-401.

OSSIFYING: Ossifying dermatofibroma with osteoclast-like giant cells.Am J Dermatopathol. 1994 Apr;16(2):193-5.

MYOFIBROBLASTIC: Prominent myofibroblastic differentiation. A pitfall in the diagnosis of dermatofibroma.Am J Dermatopathol. 1997 Apr;19(2):138-46.

MYXOID: Stromal mucin is present.(D/D cutaneous myxoma). Myxoid dermato- fibroma. Histopathology.1999 Apr;34(4):357-64.

Decreased CD44 expression and stromal hyaluronate accumulation in myxoid dermatofibroma.Dermatology. 2003;207(1):104-6.

WITH SMOOTH MUSCLE:Smooth-muscle proliferation in dermatofibromas. Am J Dermatopathol. 1994;16(2):155-60.

KELOIDAL: Keloidal dermatofibroma: report of 10 cases of a new variant.Am J Surg Pathol. 1998;22(5):564-8

PALISADING: Palisading subcutaneous fibrous histiocytoma.Pathol Int.2004;54(5):360-3

Palisading cutaneous fibrous histiocytoma. An immunohistochemical study demonstrating differentiation from dermal dendrocytes.Am J Dermatopathol. 1993 Dec;15(6):559-61.

ATROPHIC: The atrophic dermatofibroma: a delled dermatofibroma.J Dermatol. 1995 May;22(5):334-9.

Atrophic dermatofibroma. Elastophagocytosis by the tumor cells.J Cutan Pathol. 2000 Jul;27(6):312-5.

D/D:  Atrophic variants of dermatofibroma and dermatofibrosarcoma protuberans. Histopathology. 1995 Jun;26(6):519-27

SUBCUTANEOUS:
Dermatofibroma extending into the subcutaneous tissue. Differential diagnosis from dermatofibrosarcoma protuberans. Am J Surg Pathol. 1990 Dec;14(12):1156-64.

COMBINED:
Combined dermatofibroma: co-existence of two or more variant patterns in a single lesion.Histopathology. 2000;36(6):529-39

Benign fibrous histiocytoma with indeterminate cells and eosinophils: collision, differentiation, or involution?Am J Dermatopathol. 2004 Jun;26(3):237-41

                          

               Differential diagnosis of dermatofibroma:
Dermatofibrosarcoma protuberans: CD34+ or +/- ; Factor XIIIa negative, monomorphic population of cells, irregularly  infiltrative at the periphery, honeycomb pattern of infiltration into the subcutis).   

Abstract: Dermatofibroma and dermatofibrosarcoma protuberans: an immunohistochemical study reveals distinctive antigenic profiles.J Dermatol Sci. 1996 Jan;11(1):1-9.

Deep penetrating dermatofibroma versus dermatofibrosarcoma protuberans. A clinicopathologic comparison.Am J Surg Pathol. 1994 Jul;18(7):677-86

The pathological distinction between "deep penetrating" dermatofibroma and dermatofibrosarcoma protuberans.Semin Cutan Med Surg. 1999 Mar;18(1):91-8

CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma.Am J Dermatopathol. 1997 Apr;19(2):147-53

Tenascin differentiates dermatofibroma from dermatofibrosarcoma protuberans: comparison with CD34 and factor XIIIa.Hum Pathol. 2001 Jan;32(1):50-6.

Atypical fibroxanthoma ; scar ;  keloid ; some hemangiomas,  Giant cell fibroblastoma; Sclerotic fibroma;  dermal nodular fasciitis; Dermal dendrocytoma;Giant cell collagenoma;Pleomorphic fibroma;

Abstract: Differential expression of factor XIIIa and CD34 in cutaneous mesenchymal tumors. J Cutan Pathol. 1993 Apr;20(2):154-8.

Medallion-like dermal dendrocyte hamartoma: a new clinically and histopathologically distinct lesion.J Am Acad Dermatol. 2004 Sep;51(3):359-63.

CD34-reactive myxoid dermal dendrocytoma.Am J Dermatopathol.2002;24(1):50-3

Giant cell collagenoma: a benign dermal tumor with distinctive multinucleate cells. Am J Surg Pathol 1998 May;22(5):557-63.

Giant cell collagenoma: case report and review of the literature.J Cutan Pathol 2002 Jan;29(1):48-51

Subungual pleomorphic fibroma. J Cutan Pathol. 2003 Oct;30(9):569-71

Sclerotic fibroma-like dermatofibroma: an uncommon distinctive variant of dermatofibroma.Histol Histopathol. 2005 Jul;20(3):801-6.

Dermatofibroma with sclerotic areas resembling a sclerotic fibroma of the skin.J Cutan Pathol. 2002 Jan;29(1):44-7

Sporadic sclerotic fibroma of the oral soft tissues. Am J Dermatopathol. 2004;26 (3):182-7

Solitary sclerotic fibroma of skin: a possible link with pleomorphic fibroma with immunophenotypic expression for O13 (CD99) and CD34 J Cutan Pathol 2003; 30:631-636


                                Atypical Fibroxanthoma  
- Presents as an exophytic nodule.
- It is a dermal tumour that can mimic melanocytic, vascular, epithelial, or other histiocytic lesions. It is usually located on the extremities in adults.
- Microscopically, it is a polypoid lesion with epidermal collarette.
- Most striking feature is the presence of sheets of angulated epithelioid with abundant eosinophilic cytoplasm (resemble
Spitz naevus).
- No junctional component or theques noted.
- Giant cells, xanthoma cells and vascular spaces may be present.
- S100 and HMB45- negative, Factor XIIIa - positive, Vimentin- positive, CD31,CD34- negative in the tumour cells but highlight the blood vessels.

Abstract:Immunohistochemical distinction of epithelioid histiocytic proliferations from epithelioid melanocytic nevi. Am J Dermatopathol. 2000;22(3):237-41

Epithelioid cell histiocytoma - histogenetic and kinetics analysis of dermal microvascular unit dendritic cell subpopulations.JCutan Pathol.2003;30(7):415-22

Epithelioid benign fibrous histiocytoma of skin: clinico-pathological analysis of 20 cases of a poorly known variant. Histopathology. 1994;24(2):123-9.

Epithelioid cell histiocytoma. A report of 10 cases including a new cellular variant.Am J Surg Pathol 1994;18(6):583-90

Epithelioid cell histiocytoma: a simulant of vascular and melanocytic neoplasms. Am J Dermatopathol. 1999 Feb;21(1):1-7

Cellular 'neurothekeoma': an epithelioid variant of dermatofibroma?  Histopathology. 1998 May;32(5):414-22.

ABSTRACTS:

Indeterminate fibrohistiocytic lesions of the skin: is there a spectrum between dermatofibroma and dermatofibrosarcoma protuberans?Am J Surg Pathol.2000;24(7):996-1003

Characterization of factor XIIIa+ dendritic cells in dermatofibroma: Immunohistochemical, electron and immunoelectron microscopical observations.J Dermatol Sci. 2005 Aug;39(2):89-96.

Dermatofibroma. A clinico-pathologic classification scheme.Pathologe.1998; 19(6):412-9

Lichenoid, erosive and ulcerated dermatofibromas. Three additional clinico-pathologic variants. J Cutan Pathol. 2000 Mar;27(3):112-7.

Immunophenotyping of dermal spindle cell tumors: diagnostic value of monocyte marker Ki-M1p and histogenetic considerations.Am J Surg Pathol. 1997;21(7):791-800

Metastasizing fibrous histiocytoma of the skin: a clinicopathologic and immuno- histochemical analysis of three cases. Mod Pathol. 2000;13(6):654-60.

Benign fibrous histiocytoma (dermatofibroma) of the face: clinicopathologic and immunohistochemical study of 34 cases associated with an aggressive clinical course.
Am J Dermatopathol. 2001;23(5):419-26

Immunohistochemistry of dermatofibromas and benign fibrous histiocytomas.J Cutan Pathol. 1995;22(4):336-41.

Dermatofibroma is a clonal proliferative disease .J Cutan Pathol 2001;27 (1), 36-39

Clonal analysis of cutaneous fibrous histiocytoma (dermatofibroma).J Cutan Pathol 2002;29(7):385-9

Dermatofibroma-a critical evaluation.Int J Surg Pathol. 2004;12(4):333-44.

Dermatofibroma: upregulation of syndecan-1 expression in mesenchymal tissue.Am J Dermatopathol. 2003 Oct;25(5):392-8.

Dermatofibroma with diffuse eosinophilic infiltrate.Am J Dermatopathol. 2000 Jun;22(3):281-4.