- Pleomorphic undifferentiated sarcoma
Pleomorphic undifferentiated sarcoma Classification and external resources
Micrograph of a pleomorphic undifferentiated sarcoma. H&E stain.
ICD-10 C49 (ILDS C49.M10) OMIM 8830/3 DiseasesDB 31471 eMedicine radio/420 MeSH D051677
Pleomorphic undifferentiated sarcoma (abbreviated PUS), also undifferentiated pleomorphic sarcoma and previously malignant fibrous histiocytoma (abbreviated MFH), is a type of soft tissue sarcoma.
PUS are regarded as the most common soft tissue sarcoma of late adult life. It rarely occurs in children. It occurs more often in Caucasians, than those of African or Asian descent, and is a male predominant disease, afflicting two males for every female.
In the extremities, it presents as a painless enlarging soft tissue mass.
It can be detected by magnetic resonance imaging (MRI), but a biopsy is required for the definitive diagnosis. MRI findings typically shows a well-circumscribed mass that is dark on T1-weighted images and bright on T2-weighted images. Central necrosis is often present and identifiable by imaging, especially in larger masses.
Pleomorphic undifferentiated sarcomas are, by definition, undifferentiated (as the name implies) meaning they do not bear a resemblance to any normal tissue.
The histomorphology, otherwise, is characterized by high cellularity, marked nuclear pleomorphism, usually accompanied by abundant mitotic activity (including atypical mitoses), and a spindle cell morphology. Necrosis is common and characteristic of high grade lesions.
Treatment consists of surgical excision (the extent of which ranges from tumor excision to limb amputation depending on the tumor) and in some cases radiation. Chemotherapy remains controversial in MFH. The usual site of metastatic disease is the lungs, and metastases should be resected if possible.
Prognosis depends on the primary tumor grade (appearance under the microscope as judged by a pathologist), size, resectability (whether it can be cut-out completely), and presence of metastases. The five-year survival ranges from 35-60%.
There is a case report of human-human transmission, in a surgeon that injured their hand while working on a PUS case.
- ^ Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C (August 2009). "MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st Century". Expert Rev Anticancer Ther 9 (8): 1135–44. doi:10.1586/era.09.76. PMC 3000413. PMID 19671033. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3000413.
- ^ "Neoplasia". http://library.med.utah.edu/WebPath/NEOHTML/NEOPL045.html.
- ^ Alaggio R, Collini P, Randall RL, Barnette P, Million L, Coffin CM (2010). "Undifferentiated high-grade pleomorphic sarcomas in children: a clinicopathologic study of 10 cases and review of literature". Pediatr. Dev. Pathol. 13 (3): 209–17. doi:10.2350/09-07-0673-OA.1. PMID 20055602.
- ^ Gärtner, Hermine-Valeria; Christian Seidl, Christine Luckenbach, Georg Schumm, Erhard Seifried, Horst Ritter, Burkhard Bultmann (1996-11-14). "Genetic Analysis of a Sarcoma Accidentally Transplanted from a Patient to a Surgeon". N Engl J Med 335 (20): 1494–1497. doi:10.1056/NEJM199611143352004. PMID 8890100. http://content.nejm.org/cgi/content/extract/335/20/1494. Retrieved 2010-01-05.
- Malignant Fibrous Histiocytoma (MFH) - Sarcoma Learning Center
Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215) Not otherwise specified (8800–8809) Connective tissue neoplasmFibromatous (8810–8839)Fibroma/fibromatosis: Aggressive infantile fibromatosis · Aponeurotic fibroma · Collagenous fibroma · Diffuse infantile fibromatosis · Familial myxovascular fibromas · Fibroma of tendon sheath · Fibromatosis colli · Infantile digital fibromatosis · Juvenile hyaline fibromatosis · Plantar fibromatosis · Pleomorphic fibroma · Oral submucous fibrosisHistiocytoma/histiocytic sarcoma: Benign fibrous histiocytoma · Malignant fibrous histiocytoma · Atypical fibroxanthomaSolitary fibrous tumorMyxomatous (8840–8849)Fibroepithelial (9000–9039)Synovial-like (9040–9049) Lipomatous (8850–8889) Myomatous (8890–8929)skeletal muscle: Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma (Sarcoma botryoides) · Alveolar rhabdomyosarcoma Complex mixed and stromal (8930–8999) Mesothelial (9050–9059) see also Template:Connective tissue
Tumors: urogenital neoplasia: urinary organs (C64–C68/D30, 188–189/223) AbdominalKidneyGlandular and epithelial neoplasm: Renal cell carcinoma · Renal oncocytomaComplex and mixed tumor: Wilms' tumor · Mesoblastic nephroma · Clear-cell sarcoma of the kidney · Angiomyolipoma · Cystic nephroma · Metanephric adenomaby location: Renal medullary carcinoma · Juxtaglomerular cell tumor · Renal medullary fibromaUreter Pelvic Retroperitoneum
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