皮膚線維肉腫

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/06/20 00:25:40」(JST)

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Dermatofibrosarcoma protuberans (DFSP) ^[1] is a very rare tumor. It is a rare neoplasm of the dermis layer of the skin,^[2] and is classified as a sarcoma. There is only about 1 case per million per year. DFSP is a fibrosarcoma,^[3] 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[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 of a suspected DFSP is usually done with a core needle or a surgical incision.^[4]

Presentation[edit]

Dermatofibrosarcoma Protuberans starts off 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.^[5] 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. All 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[edit]

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 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.^[6]^[7]

Treatment[edit]

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^[8] 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. The type of surgery often used is Moh's Micrographic Surgery. Usually, complete surgical resection with margins of 2 to 4 cm (recommened) 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.^[9] 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 Moh's 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[edit]

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

References[edit]

^ Mendenhall WM, Zlotecki RA, Scarborough MT (December 2004). "Dermatofibrosarcoma protuberans". Cancer 101 (11): 2503–8. doi:10.1002/cncr.20678. PMID 15503305. Review.
^ "Dorlands Medical Dictionary:dermatofibrosarcoma".
^ "dermatofibrosarcoma" at Dorland's Medical Dictionary
^ http://sarcomahelp.org/dermatofibrosarcoma-protuberans.html
^ http://ghr.nlm.nih.gov/condition/dermatofibrosarcoma-protuberans>
^ Sirvent N, Maire G, Pedeutour F (May 2003). "Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment". Genes Chromosomes Cancer 37 (1): 1–19. doi:10.1002/gcc.10202. PMID 12661001.
^ Patel KU, Szabo SS, Hernandez VS, et al. (February 2008). "Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays". Hum. Pathol. 39 (2): 184–93. doi:10.1016/j.humpath.2007.06.009. PMID 17950782.
^ http://wwwu.tsgh.ndmctsgh.edu.tw/commcpc/images/nccn/dfsp%20NCCN%202004.pdf
^ Gloster HM, Harris KR, Roenigk RK (July 1996). "A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans". J Am Acad Dermatol. 35 (1): 82–7. PMID 8682970.

External links[edit]

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：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
2. 隆起性皮膚線維肉腫：治療 dermatofibrosarcoma protuberans treatment
3. 頭頸部肉腫 head and neck sarcomas
4. モース手術 mohs surgery
5. 皮膚の良性病変の概要 overview of benign lesions of the skin

English Journal

Recurrent Dermatofibrosarcoma Protuberans With Pulmonary Metastases Presenting Twelve Years After Initial Diagnosis: 18F-FDG PET/CT Imaging Findings.

Suman S, Sharma P, Jain TK, Sahoo MK, Bal C, Kumar R.Author information From the Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.AbstractDermatofibrosarcoma protuberans is a rare cutaneous tumor that is locally aggressive and has a high rate of recurrence after surgical excision. The tumor grows slowly, typically over years. On rare occasions, metastasis to distant sites (especially the lung) or regional lymph nodes may occur. Here, we present F-FDG PET/CT imaging findings of a 52-year-old man with a local recurrence of dermatofibrosarcoma protuberans in the anterior abdominal wall with metastases to bilateral lungs.
Clinical nuclear medicine.Clin Nucl Med.2014 Jan;39(1):77-8. doi: 10.1097/RLU.0b013e318279f28e.
Dermatofibrosarcoma protuberans is a rare cutaneous tumor that is locally aggressive and has a high rate of recurrence after surgical excision. The tumor grows slowly, typically over years. On rare occasions, metastasis to distant sites (especially the lung) or regional lymph nodes may occur. Here,
PMID 23531730

Fibrosarcoma: a review and update.

Folpe AL.Author information Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.AbstractAdult fibrosarcoma, defined by the World Health Organization as a 'malignant neoplasm composed of fibroblasts with variable collagen production and, in classical cases, a "herringbone" architecture', is a very rare soft tissue sarcoma. Once considered the most common adult sarcoma, the incidence of adult fibrosarcoma has declined dramatically over the past several decades. This is due to (i) evolution in the classification of soft tissue tumours (ii) recognition of clinically, morphologically and genetically distinctive subtypes of fibrosarcoma and (iii) increased understanding of the many other mesenchymal and non-mesenchymal tumours that may mimic fibrosarcoma. This review article will summarize the current state of our knowledge about strictly defined adult fibrosarcoma and discuss important entities in its differential diagnosis, including various fibrosarcoma variants, monophasic synovial sarcoma and other potential mesenchymal and non-mesenchymal mimics.
Histopathology.Histopathology.2014 Jan;64(1):12-25. doi: 10.1111/his.12282. Epub 2013 Nov 22.
Adult fibrosarcoma, defined by the World Health Organization as a 'malignant neoplasm composed of fibroblasts with variable collagen production and, in classical cases, a "herringbone" architecture', is a very rare soft tissue sarcoma. Once considered the most common adult sarcoma, the incidence of
PMID 24266941

Targeted therapies for sarcomas: new roles for the pathologist.

Al-Zaid T, Somaiah N, Lazar AJ.Author information Department of Pathology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.AbstractSarcomas are a heterogeneous family of mesenchymal malignancies that employ an impressive variety of pathogenetic mechanisms. The traditional role of the pathologist in this field has been to ensure accurate diagnosis and histological grading to direct therapy. More recently, with the advent of targeted therapies directed at particular molecular alterations, the role of the pathologist has expanded and increased awareness of the genetic features of sarcomas is needed to deliver optimal multidisciplinary care. This review discusses these trends and briefly enumerates many of the molecular derangements and targeted agents currently used or under investigation in soft tissue sarcoma. A few sarcomas are highlighted in more detail to illustrate how pathologists can exert positive influence on patient care - not just through diagnosis and grading, but also with molecular characterizations. Featured sarcomas include alveolar soft part sarcoma, dermatofibrosarcoma protuberans, gastrointestinal stromal tumour, inflammatory myofibroblastic tumour and PEComas.
Histopathology.Histopathology.2014 Jan;64(1):119-33. doi: 10.1111/his.12297. Epub 2013 Dec 2.
Sarcomas are a heterogeneous family of mesenchymal malignancies that employ an impressive variety of pathogenetic mechanisms. The traditional role of the pathologist in this field has been to ensure accurate diagnosis and histological grading to direct therapy. More recently, with the advent of targ
PMID 24117526

Japanese Journal

症例 COL1A1-PDGFB融合遺伝子の検出が診断に有用であった隆起性皮膚線維肉腫の1例

小林忠弘,平野貴士,石井貴之 [他]
皮膚科の臨床 54(9), 1273-1277, 2012-09
NAID 40019429248

生下時より後頭部に発生した Bednar 腫瘍 (Pigmented dermatofibrosarcoma protuberans) の1例

井上麻由子,三川信之,檜垣浩一
日形会誌 : 日本形成外科学会会誌 = Journal of Japan Society of Plastic and Reconstructive Surgery 31(11), 778-783, 2011-11-20
NAID 10030261731

Related Pictures

★リンクテーブル★

リンク元	「隆起性皮膚線維肉腫」

「隆起性皮膚線維肉腫」

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

　　[★]

ラ: dermatofibrosarcoma protuberans
同: 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

[1] Mendenhall WM, Zlotecki RA, Scarborough MT (December 2004). "Dermatofibrosarcoma protuberans". Cancer 101 (11): 2503–8. doi:10.1002/cncr.20678. PMID 15503305. Review.

[urlDorlands_Medical_Dictionary:dermatofibrosarcoma-2] "Dorlands Medical Dictionary:dermatofibrosarcoma".

[3] "dermatofibrosarcoma" at Dorland's Medical Dictionary

[4] ttp://sarcomahelp.org/dermatofibrosarcoma-protuberans.html

[5] ttp://ghr.nlm.nih.gov/condition/dermatofibrosarcoma-protuberans>

[pmid12661001-6] Sirvent N, Maire G, Pedeutour F (May 2003). "Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment". Genes Chromosomes Cancer 37 (1): 1–19. doi:10.1002/gcc.10202. PMID 12661001.

[pmid17950782-7] Patel KU, Szabo SS, Hernandez VS, et al. (February 2008). "Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays". Hum. Pathol. 39 (2): 184–93. doi:10.1016/j.humpath.2007.06.009. PMID 17950782.

[8] ttp://wwwu.tsgh.ndmctsgh.edu.tw/commcpc/images/nccn/dfsp%20NCCN%202004.pdf

[9] Gloster HM, Harris KR, Roenigk RK (July 1996). "A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans". J Am Acad Dermatol. 35 (1): 82–7. PMID 8682970.

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dermatofibrosarcoma