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 Path Quiz Case-27: Diagnosis

Dermatofibrosarcoma Protuberans

        Dr Sampurna Roy MD

   Case 27- Case history and images: click here

Dermatopathology Quiz Case 64 : click here

 
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Dermatofibrosarcoma protuberans is a fibrohistiocytic tumour of intermediate malignancy ,characterized by a distinctive storiform growth pattern and frequent local recurrences.

This tumour was first described in 1924 by Darier and Ferrand as 'progressive and recurring dermatofibroma'.

Over the years this tumour has been considered to be of fibroblastic, histiocytic and even of neural origin by different authors. Inview of the similarity of the tumour with benign fibrous histiocytoma, this tumour is tentatively classified with fibrohistiocytic tumours.

DFSP usually occurs in young and middle aged adults and is commonly located in the head and neck region followed by upper extremity . Palms and soles are not affected.

Cytogenetic analysis reveals reciprocal translocation t(17;22)(q22;q13) and ring chromosome derived from translocation r(17;22).  
Macroscopically, the tumour presents as a firm and grey white plaque, exophytic nodule or a massive pedunculated tumour.The pigmented variant has a slate-gray appearance.

 
 MICROSCOPIC FEATURES: Image Link1 ; Image Link2 ; Image Link3 ; Image Link4 ; Image Link5 ; Image Link6.

                            

- Dermal tumour  which extends into the subcutis where it infiltrates around small groups of fat cells in a lacy or linear fashion.
- The tumour  is composed of interwoven bundles of spindle cells with plump nuclei arranged in a storiform or cartwheel pattern.
- Superficial grenz  zone is present separating the tumour from the epidermis.
- The overlying epidermis is normal or atrophic.
- Scattered mitotic figures are present ( not more than 5 per 10 high power fields) .
- The tumour cells surround the dermal appendages. The appendages are not destroyed by the tumour.
- Other features include presence of thin walled blood vessels, occasional Touton giant cells , foam cells and granular cells.
- Immunohistochemistry - The most diagnostic marker is CD34 (human progenitor cell antigen). 50-100% cells show positivity. The tumour cells are also positive for Vimentin and p53.  

 

HISTOLOGICAL  VARIANTS:
 
1.  Fibrosarcomatous

2.  Fibrosarcomatous with myoid/ myofibroblastic change.    
3.  Myxoid
4.  Granular cell
5.  Atrophic
6.  Palisaded
7.  Giant cell fibroblastoma

8.  Sclerosing 
9.  Combined
10.Indeterminate
Dermatopathology Quiz Case 64

 

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FIBROSARCOMATOUS CHANGE IN DFSP:

Fibrosarcomatous change in DFSP represents a form of tumor progression in DFSP and is associated with a significantly more aggressive clinical course than in ordinary DFSP, indicating a possible need for treatment intensification in such cases.

Features include-  There is  increased cellularity and mitoses (more than 8 mitoses per 10 high power field).

Other features include focal myxoid change, keloid like hyalinization, giant rosettes, pigmented melanocytes, myoid nodules and bundles.
Less than 50% cells display CD34 positivity.
The tumour may be associated with metastasis.

MYXOID VARIANT OF DFSP:

Storiform pattern is less prominent. Blood vessels are more conspicuous. Features supporting myxoid DFSP:  (1) CD34:positive (2) Pattern of infiltration of the adipose tissue. (3) Genetic alterations: ring / markers t(17;22)

INDETERMINATE  FIBROHISTIOCYTIC  LESIONS:

This tumour is characterized by combined features of dermatofibroma and DFSP. Features include-
-  Keloidal collagen
-  Infiltration of the subcutis in a honeycomb pattern
-  Low mitotic count
-  Dual population of CD34 and factor XIIIa positive cells

                                                     
 DIFFERENTIAL DIAGNOSIS:

-Giant cell fibroblastoma- Giant cell fibroblastoma: a report of three cases with histologic and immunohistochemical evidence of a relationship to dermatofibrosarcoma protuberans.Arch Pathol Lab Med. 1996 Nov;120(11):1052-5.

-Dermatofibroma- Dermatofibromas are small (often less than 1 cm), symmetrical and usually  do not penetrate adipose tissue. Hemosiderin pigment is present. Tumour border is usually infiltrative but regular. Collagen is present at the peripheral border, in a keloidal pattern.There is birefringence of  the collagen in dermatofibroma  with polarization. Unlike DFSP, adnexal structures are not entrapped within the lesion. Heterogenous population of cells (histiocytes,fibroblasts or mixed).In DFSP, the cells are usually regular, small spindle shaped.

-Neurofibroma - Neurofibromatous changes in dermatofibrosarcoma protuberans: a potential pitfall in the diagnosis of a serious cutaneous soft tissue neoplasm. J Cutan Pathol. 2004 Aug;31(7):492-6

-Atypical fibroxanthoma and MFH-  There is prominent cellular pleomorphism in these lesions.

-Classic fibrosarcoma- Deep seated tumour. High mitotic index. The tumour rarely extends to the dermis

-Fasciitis The cells display uniform ' tissue culture' growth pattern.
D/D- MYXOID  DFSP -Myxoid Neurofibroma (immmunohistochemistry is particularly useful in this condition).

-Myxoid liposarcoma Presence of lipoblasts.

-Myxofibrosarcoma - Presence of pleomorphic cells.

ABSTRACTS:

-Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases.Journal of Clinical Pathology 2005;58:751-756

-FNA diagnosis of dermatofibrosarcoma protuberans.Diagn Cytopathol.2005 ;32(5):299-302

-Cellular digital fibromas: distinctive CD34-positive lesions that may mimic dermatofibro- sarcoma protuberans.J Cutan Pathol. 2005;32(6):413-8

-Apo D in Soft Tissue Tumors: A Novel Marker for Dermatofibrosarcoma Protuberans.Am J Surg Pathol. 2004 Aug;28(8):1063-1069

-Dermatofibrosarcoma protuberans: A clinicopathological analysis of 10 cases in Asians. Australas J Dermatol. 2004 Feb;45(1):29-33

-Immunohistochemical expression of matrix metalloproteinases 1, 2, 9, and 14 in dermatofibrosarcoma protuberans and common fibrous histiocytoma (dermatofibroma).Arch Pathol Lab Med. 2004 Oct;128(10):1136-41.

-Dermatofibrosarcoma protuberans association with nuchal-type fibroma. J Cutan Pathol. 2004 Jan;31(1):62-6

-Atrophic dermatofibrosarcoma protuberans: a case report and reappraisal of the literature.Cutis. 2004 Oct;74(4):237-42.

-Differential expression of HMGA1 and HMGA2 in dermatofibroma and dermatofibrosarcoma protuberans: potential diagnostic applications, and comparison with histologic findings, CD34, and factor XIIIa immunoreactivity.Am J Dermatopathol. 2004 Aug;26(4):267-72

-Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data--a study from the French Federation of Cancer Centers Sarcoma Group.Am J Surg Pathol. 2003;27(1):27-39

-CD44 and hyaluronate in the differential diagnosis of dermatofibroma and dermato- fibrosarcoma  protuberans. J Cutan Pathol 2003 Mar;30(3):185-9

-Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment.Ann Surg Oncol. 2003 Nov;10(9):1118-22

-Bednar tumor associated with dermal melanocytosis: melanocytic colonization or neuroectodermal multidirectional differentiation?J Cutan Pathol 2003 Feb;30(2):147-51

-Stromelysin 3 expression: A useful marker for the differential diagnosis dermatofibroma versus dermatofibrosarcoma protuberans.J Am Acad Dermatol 2002;46:408-13

-Indeterminate Fibrohistiocytic Lesions of the Skin: Is There a Spectrum Between Dermatofibroma and Dermatofibrosarcoma Protuberans? Am J Surg Pathol 2000;24:996-1003

-Sarcomas Arising in Dermatofibrosarcoma Protuberans: A Reappraisal of Biologic Behavior in Eighteen Cases Treated by Wide Local Excision With Extended Clinical Follow Up.Am J Surg Pathol 2000;24:1125-1130

-A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile. J Cutan Pathol 2000 Apr;27(4):159-6

-Palisading and verocay body-prominent dermatofibrosarcoma protuberans: a report of three cases. Histopathology. 2000 Nov ;37(5):452-5.

-Atrophic dermatofibrosarcoma protuberans: a case report and review of the literature. Dermatology 1998;196(4):422-4

-Atrophic and plaquelike dermatofibrosarcoma protuberans.Am J Dermatopathol.1998; 20(5): 498-501

-Sclerosing dermatofibrosarcoma protuberans.J Cutan Pathol. 1998 Sep;25(8):440-4

-Fibrosarcomatous ("High-Grade") Dermatofibrosarcoma Protuberans: Clinicopathologic and Immunohistochemical Study of a Series of 41 Cases With Emphasis on Prognostic Significance.American Journal of Surgical Pathology. 22(5):576-587, May 1998

-Myxoid dermatofibrosarcoma protuberans: morphological, ultrastructural and immuno- histochemical features.J Cutan Pathol. 1998 Aug;25(7):386-93

-Dermatofibrosarcoma protuberans with fibrosarcomatous areas: a clinico-pathologic and immunohistochemic study in four cases. Am J Dermatopathol. 1997 Dec;19(6):562-7

-Myoid differentiation in dermatofibrosarcoma protuberans and its fibrosarcomatous variant: clinicopathologic analysis of 5 cases.J Cutan Pathol. 1996 Feb;23(1):30-6

-Pigmented fibrosarcomatous dermatofibrosarcoma protuberans (Bednar tumor). 3 case reports, analogy with the "conventional" type and review of the literature.Pathologica. 1997 Jun;89(3):264-73.

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

-Dermatofibrosarcoma protuberans. A clinicopathologic review with emphasis on fibrosarcomatous areas.Am J Surg Pathol.1992 Oct;16(10):921-5

-Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literature.Histopathology. 1985 Sep;9(9):921-38

Pathopedia-India.com:

Contents ; Introduction of Pathology ; An outline of Diagnostic Techniques available in Pathology ; Cellular Injury ; Diagram showing Structural Changes in Reversible and Irreversible Cell Injury ; Autolysis; Heterolysis ; Necrosis; Coagulation (Coagulative) necrosis ; Caseative (Caseous) necrosis ; Liquefaction necrosis ; Fat necrosis ; Fibrinoid necrosis ; Apoptosis ; Gangrene ; Hyaline Change ; Atrophy ; Hypertrophy ; Hyperplasia ; Metaplasia ; Aplasia ; Hypoplasia ;Cellular Accumulations ; Accumulation of Glycogen, complex lipids and carbohydrates ; Pigments ; Melanin ; Pigments derived from Hemoproteins; Hemosiderin and Hemosiderosis ; Primary Hemochromatosis ; Hematin; Bilirubin; Lipofuscin; Mineral Dusts ; Silica ; Urate ; Amyloid ; Inflammation ; Inflammatory cells in acute and chronic inflammation ; Acute Inflammation; Types of Acute Inflammation; Chemical Mediators ; Chronic Inflammation; Wound Healing ; Circulatory Anatomy, Physiology and Regulation; Normal Fluid Balance; Edema; Morphology of Edema; Diagram showing Capillary System and Mechanisms of Edema Formation; Hyperemia and Congestion; Hemostasis and Thrombosis; Embolism; Fat Embolism; Air Embolism ; Decompression Sickness ; Amniotic Fluid Embolism ; Diagram showing Sources of Arterial Emboli ; Diagram showing Sources of Venous Emboli ; Infarction ; Diagram showing common sites of Systemic Infarction  from Arterial Emboli; Shock; Pathology of Shock; Diagram showing Complications of Shock; Hemorrhage;

    
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