dermatofibrosarcoma protuberans

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/20 16:38:26」(JST)

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Dermatofibrosarcoma protuberans
Histopathological image of dermatofibrosarcoma protuberans. Local recurrence long after the first excision. H&E stain
Classification and external resources
ICD-10	C49 (ILDS C49.M24)
ICD-9-CM	8832/3
ICD-O	8833/3
OMIM	607907
DiseasesDB	31601
eMedicine	derm/97
MeSH	D018223

Dermatofibrosarcoma protuberans (DFSP) is a very rare tumor. It is a rare neoplasm of the dermis layer of the skin, and is classified as a sarcoma. There is only about one case per million per year. DFSP is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. In many respects, the disease behaves as a benign tumor, but in 2–5% of cases it can metastasize, so it should be considered to have malignant potential. It occurs most often in adults in their thirties; it has been described congenitally, in children, and the elderly. It accounts for approximately 2–6% of soft tissue sarcoma cancers.

Diagnosis

Dermatofibrosarcoma protuberans is diagnosed with a biopsy, when a portion of the tumor is removed for examination. In order to ensure that enough tissue is removed to make an accurate diagnosis, the initial biopsy of a suspected DFSP is usually done with a core needle or a surgical incision.

Presentation

Dermatofibrosarcoma protuberans begins as a minor firm area of skin most commonly about to 1 to 5 cm in diameter. It is a slow growing tumor and is usually found on the torso but can also be found on the arms, legs, head and neck. About 90% of DFSPs are low grade sarcomas. About 10% are mixed; they contain a high-grade sarcomatous component (DFSP-FS); therefore, they are considered to be intermediate-grade sarcomas. DFSPs rarely lead to a metastasis (fewer than 5% do metastasise), but DFSPs can recur locally. DFSPs most often arise in patients who are in their thirties, but sometimes have been described in children or the elderly.

Pathophysiology

More than 90% of DFSP tumors have the chromosomal translocation t(17;22). The translocation fuses the collagen gene (COL1A1) with the platelet-derived growth factor (PDGF) gene. The fibroblast, the cell of origin of this tumor, expresses the fusion gene in the belief that it codes for collagen. However the resulting fusion protein is processed into mature platelet-derived growth factor which is a potent growth factor. Fibroblasts contain the receptor for this growth factor. Thus the cell "thinks" it is producing a structural protein, but it actually produces a self-stimulatory growth signal. The cell divides rapidly and a tumor forms.

The tissue is often positive for CD34.

Treatment

Dermatofibrosarcoma protuberans of the left axilla. CT, coronal reconstruction

Treatment is primarily surgical, with chemotherapy and radiation therapy sometimes used.

The NCCN guideline recommends CCPDMA or Mohs surgery for the best cure rate of DFSP. Mohs surgery can be extremely effective. It will remove the tumor and all related pathological cells without a wide-area excision that may overlook sarcoma cells that have penetrated muscle tissue.

The standard of care for patients with DFSP is surgery. Usually, complete surgical resection with margins of 2 to 4 cm (recommended) is performed. The addition of adjuvant radiotherapy (irradiation) improves local control in patients with close or positive margins during the surgery. A special surgical technique, the "Mohs micrographic surgery" (MMS), can be employed in patients with DFSP. MMS is technically possible if the DFSP is in an anatomically confined area. A high probability of cure of DFSP can be attained with MMS as long as the final margins are negative. Patients who have a recurrent DFSP can have further surgery, but the probability of adverse effects of surgery and/or metastasis is increased in these patients. The Mohs surgery is highly successful.

Imatinib is approved for treatment. As is true for all medicinal drugs that have a name that ends in "ib," imatinib is a small molecular pathway inhibitor; imatinib inhibits tyrosine kinase. It may be able to induce tumor regression in patients with recurrent DFSP, unresectable DFSP or metastatic DFSP. There is clinical evidence that imatinib, which inhibits PDGF-receptors, may be effective for tumors positive for the t(17;22) translocation.

Additional images

• Subcutaneous tissue infiltration (i.e. "honeycomb" growth pattern)

• Monotonous, plexiform structure of tumour

• DFSP formed both by fibroblastic and histiocytic elements

• Hemosiderin deposits beneath the tumour

• Immunostain positive for CD34

See also

• List of cutaneous conditions

References

External links

• humpath #346 (Pathology images)
• Dermatofibrosarcoma Protuberans in the Sarcoma Learning Center
• Thesis on DFSP

UpToDate Contents

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• 1. 隆起性皮膚線維肉腫：治療 dermatofibrosarcoma protuberans treatment
• 2. 隆起性皮膚線維肉腫：疫学、病因、臨床症状、診断、および病期分類 dermatofibrosarcoma protuberans epidemiology pathogenesis clinical presentation diagnosis and staging
• 3. 頭頸部肉腫 head and neck sarcomas
• 4. 皮膚の良性病変の概要 overview of benign lesions of the skin
• 5. 軟部および骨肉腫の病原因子 pathogenetic factors in soft tissue and bone sarcomas

English Journal

• [Czech eponyms in pathology].

• Steiner I.AbstractThe 24th European Congress of Pathology taking place in Prague is an opportunity to remind our society of the Czech names appearing as eponyms in pathological terminology: Karel Rokitanský - R. protuberance in dermoid cyst; R. thrombogenic theory of atherosclerosis; Mayer - R. - Küster - Hauser - Winckel syndrome (congenital malformation of the vagina and uterus); Václav Treitz - T. duodenal ligament; T. retroperitoneal hernia; T. uremic colitis; Vilém Dušan Lambl - L. excrescences of heart valves; Lamblia (Giardia) intestinalis, and also the foundation of urological cytology; Stanislav Provázek - Prowazek - Halberstädter bodies (trachoma), Rickettsia Prowazeki (typhus fever); Josef Vaněk - V. tumor (gastric inflammatory fibroid polyp), and also discovery of the etiology of pneumocystic pneumonia; Otto Jírovec - Pneumocystis Jiroveci; Blahoslav Bednář - B. tumor (pigmented dermatofibrosarcoma protuberans). Keywords: Rokitansky - Treitz - Lambl - Prowazek - Vaněk - Jírovec - Bednář - history of pathology.
• Ceskoslovenská patologie.Cesk Patol.2013 Dec;49(1):51-4.
• The 24th European Congress of Pathology taking place in Prague is an opportunity to remind our society of the Czech names appearing as eponyms in pathological terminology: Karel Rokitanský - R. protuberance in dermoid cyst; R. thrombogenic theory of atherosclerosis; Mayer - R. - Küster - Hauser -
• PMID 23432077

• Dermatofibrosarcoma Protuberans: Long-term Outcomes of 53 Patients Treated With Conservative Surgery and Radiation Therapy.

• Castle KO, Guadagnolo BA, Tsai CJ, Feig BW, Zagars GK.SourceDepartment of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.
• International journal of radiation oncology, biology, physics.Int J Radiat Oncol Biol Phys.2013 Jul 1;86(3):585-590. doi: 10.1016/j.ijrobp.2013.02.024. Epub 2013 Apr 26.
• PURPOSE: To evaluate outcomes of conservative surgery and radiation therapy (RT) treatment in patients with dermatofibrosarcoma protuberans.METHODS AND MATERIALS: We retrospectively reviewed the medical records of 53 consecutive dermatofibrosarcoma protuberans patients treated with surgery and preop
• PMID 23628134

Japanese Journal

• 症例 COL1A1-PDGFB癒合遺伝子を検出した幼児の隆起性皮膚線維肉腫

• 平野 郁代,八田 尚人
• 皮膚科の臨床 53(10), 1455-1458, 2011-10
• NAID 40019022258

Related Links

• Dermatofibrosarcoma Protuberans: Diagnosis, Treatment ...

Dermatofibrosarcoma Protuberans is explained for patients and practitioners in this 2012 article, with patient stories and support group listings. ... What are the risks factors or causes of DFSP? It is not clear what causes this type of ...

• Dermatofibrosarcoma protuberansとは - goo Wikipedia ...

Diagnosis [edit] Dermatofibrosarcoma protuberans is diagnosed with a biopsy, when a portion of the tumor is removed for examination. In order to ensure that enough tissue is removed to make an accurate diagnosis, the initial biopsy ...

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リンク元	「隆起性皮膚線維肉腫」

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dermatofibrosarcoma protuberans

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皮膚線維肉腫 dermatofibrosarcoma、花むしろ状線維性黄色腫 storiform fibroxanthoma、進行性再発性皮膚線維腫 progressive recurring dermatofibroma