Giant cell fibroblastoma is a rare locally aggressive tumor of subcutaneous mesenchymal tissue, occurring mostly on the trunk in young individuals with maximal incidence in the first decade of life. Local recurrences of giant cell fibroblastoma are common if marginally excised, however, distant metastases do not occur. Giant cell fibroblastoma was labelled as a juvenile variant of dermatofibrosarcoma protuberans (DFSP) due to quite frequent combination of both lesions, morphological similarities, identical immunoprofile, and shared gene fusion t(17;22) COL1A1-PDGFB. In this paper, we report a case of a young man with a slowly growing subcutaneous tumor in the groin. The tumor was excised and histological examination identified a mesenchymal tumor with variable cellularity, presence of multinucleated giant cells and pleomorphic spindle cells, which lined pseudovascular or angiectoid spaces. The CD34 immunohistochemistry showed strong positivity in all of these cells, whereas ERG was positive only in endothelial cells in true vessels. These findings led to a suspicion on giant cell fibroblastoma. Because of its borderline malignant behaviour and positive surgical margins, the lesion was subsequently reexcised. The molecular analysis identified the transcription product of gene fusion COL1A1-PDGFB and thus, final diagnosis was confirmed. The article includes review of the literature and brief historical overview of giant cell fibroblastoma concept as an unique entity.

• Klíčová slova
• dermatofibrosarcoma protuberans, fibroblastoma, giant cell, juvenile,
• MeSH
• dermatofibrosarkom * genetika   patologie   MeSH
• endoteliální buňky patologie   MeSH
• lidé MeSH
• nádory kůže * patologie   MeSH
• obrovské buňky patologie   MeSH
• protoonkogenní proteiny c-sis genetika   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH
• Názvy látek
• protoonkogenní proteiny c-sis MeSH

We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans (DFSP) that, in its second recurrence, contained a pattern of Bednár tumor (BT) and giant cell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation of mucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesion contained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nerve sheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and it shows how broad morphological spectrum can be produced by a composite tumor, especially when the tumor includes unconventional growth pattern or additional non-neoplastic lesion.

• MeSH
• dermatofibrosarkom patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru patologie   MeSH
• nádory kůže patologie   MeSH
• nádory nervové pochvy patologie   MeSH
• nádory obrovskobuněčné patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

We present here ultrastructural findings in three composite tumours having giant cell fibroblastoma--dermatofibrosarcoma protuberans components occurring in adult patients. We further hypothesize that these 3 tumours belong to the group of neoplasms that can show giant cell fibroblastoma, dermatofibrosarcoma protuberans, dendritic pigment cell, myxoid, and fibrosarcomatous differentiation. We consider the giant cell fibroblastoma areas of the tumours to represent a well-differentiated component of these neoplasms.

• MeSH
• buněčné jádro patologie   ultrastruktura   MeSH
• dermatofibrosarkom patologie   ultrastruktura   MeSH
• dospělí MeSH
• elektronová mikroskopie MeSH
• fibrom patologie   ultrastruktura   MeSH
• lidé MeSH
• melaniny analýza   MeSH
• nádory kůže patologie   ultrastruktura   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• melaniny MeSH

We report a light microscopic and ultrastructural appearance, and immunohistochemical profile of two benign tumours of soft tissue recently designated as desmoplastic fibroblastoma (collagenous fibroma). The tumours were located in the foot and well demarcated. Microscopically, they were composed of stellate--or spindle--shaped cells embedded in a hypovascular fibrous of fibromyxoid stroma. Immunohistochemical examination showed that tumours were diffusely positive for vimentin and focaly positive for smooth muscle actin and muscle specific actin. Ultrastructurally, the neoplastic cells had features of fibroblasts and myofibroblasts.

• MeSH
• desmoplastický fibrom chemie   patologie   MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory měkkých tkání chemie   patologie   MeSH
• nemoci nohy (od hlezna dolů) patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• práce podpořená grantem MeSH

A case of giant cell fibroblastoma in a 62-year-old male is described. The 2 x 1.5 x 1.5 cm tumor was excised from the right supraclavicular area. Histologically, it was typical with exceptions that the typical pseudovascular spaces were seen only focally and the neoplastic cells were closely spatially associated with lymphocytes and plasmocytes. This association was suggestive of emperipolesis. The unusual clinicopathologic features caused some diagnostic difficulty.

• MeSH
• dermatofibrosarkom patologie   MeSH
• diferenciální diagnóza MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory kůže patologie   MeSH
• nádory obrovskobuněčné patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

We describe a unique case of giant cell fibroblastoma that contained a component of dermatofibrosarcoma protuberans in the left inguinal region of a 37-year-old man. Our case adds further proof of the close histogenetic relationship between these two tumors.

• MeSH
• dospělí MeSH
• fibrosarkom patologie   MeSH
• lidé MeSH
• nádory kůže patologie   MeSH
• nádory obrovskobuněčné patologie   MeSH
• třísla MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

We present a unique case of composite skin tumor of the vulva consisting of dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) with an intratumoral focus of endometriosis. A 31-year-old female with a 10-year-history of a recurring subcutaneous tumor in the vulvar area underwent excision of the seventh recurrence of the tumor. Microscopic examination revealed a composite fibrohistiocytic tumor consisting of DFSP and GCF. Additionally, a focus of endometriosis within the tumor tissue was found. Malignant transformation of extragonadal endometriosis has already been described; we present, however, the occurrence of a focus of endometriosis within the tissue of a hormonally independent soft tissue tumor. There is a possible link to the occurrence of cutaneous endometriosis at previous surgery sites and in the scars. The presence of endometriosis within the soft tissue tumor represents, to the best of our knowledge, a previously undescribed collision phenomenon.

• MeSH
• antigeny nádorové analýza   MeSH
• dermatofibrosarkom chemie   patologie   chirurgie   MeSH
• dospělí MeSH
• endometrióza metabolismus   patologie   chirurgie   MeSH
• fibrom chemie   patologie   chirurgie   MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• mnohočetné primární nádory chemie   patologie   chirurgie   MeSH
• nádorové biomarkery analýza   MeSH
• nádory měkkých tkání chemie   patologie   chirurgie   MeSH
• nádory obrovskobuněčné chemie   patologie   chirurgie   MeSH
• vulva patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• antigeny nádorové MeSH
• nádorové biomarkery MeSH

A case of dermatofibrosarcoma protuberans in a 39-year old woman excised from the right subclavicular region is described. Beside the typical storiform cellular pattern characteristic of dermatofibrosarcoma protuberans, a considerable part of the tumour displayed a myxoid change. Another part of the tumour exhibited large areas with a fibrosarcoma-like pattern. The transitional areas between these myxoid and fibrosarcoma-like areas resembled a poorly differentiated spindle cell sarcoma. Two years later the tumour recurred. The remarkable feature of the recurrent tumour was the occurrence of multiple giant cells identical to those seen in giant cell fibroblastoma.

• MeSH
• dermatofibrosarkom patologie   chirurgie   MeSH
• dospělí MeSH
• fibrosarkom patologie   chirurgie   MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• nádory kůže patologie   chirurgie   MeSH
• následné studie MeSH
• reoperace MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

The authors present the case-history of a young patient with rapid growth of a relapsing skin tumor--dermamtofibrosarcoma protuberans (DFSP)--in the scar of a previous minor dermal excision. DFSP is a malignant skin tumour, frequently relapsing in the scar after excision, rarely metastasizing into lymph nodes and the lungs. The patient was treated by a major radical excision and a dermal flap plastic operation with subsequent adjuvant actinotherapy. With regard to possible relapses, the authors recommend long-term dispensarization of patients.

• MeSH
• dermatofibrosarkom chirurgie   MeSH
• dospělí MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• nádory kůže chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

Dermatofibrosarcoma protuberans is a quite rare local aggressive tumor of dermis and subcutis, revealing characteristic morphology and chromosomal translocation (17; 22)(q21;q13) with gene fusion COL1A1-PDGFB. The tumour almost never metastasizes and complete excision signs an excellent prognosis. Approximately in 10% of cases, dermatofibrosarcoma undergoes a fibrosarcomatous transformation associated with metastatic disease and worse prognosis. In this paper, we refer a case of a male patient with subcutaneous tumor in back region, in which the small biopsy lead to diagnosis of a spindle cell sarcoma. However, only the histopathological examination of the entire tumor in the material from the radical surgery detected the dermatofibrosarcoma protuberans with fibrosarcomatous transformation. Both components of the tumor showed the characteristic genetic alteration. Identification of fibrosarcomatous component within the DFSP matters in prognosis. Distinction between fibrosarcoma arising within the dermatofibrosarcoma protuberans and fibrosarcoma arising de novo is of therapeutic consequence: the patients with metastatic or inoperable DFSP with fibrosarcomatous transformation may profit form imatinib treatment.

• Klíčová slova
• change, dermatofibrosarcoma protuberans, fibrosarcoma, fibrosarcomatous, transformation,
• MeSH
• dermatofibrosarkom * farmakoterapie   patologie   MeSH
• fibrosarkom * farmakoterapie   patologie   MeSH
• imatinib mesylát terapeutické užití   MeSH
• lidé MeSH
• nádory kůže * farmakoterapie   patologie   MeSH
• translokace genetická MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• imatinib mesylát MeSH