Pleomorphic undifferentiated sarcoma |
Micrograph of a pleomorphic undifferentiated sarcoma. H&E stain.
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Classification and external resources |
Specialty |
Oncology |
ICD-10 |
C49 (ILDS C49.M10) |
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.
It is considered a diagnosis of exclusion for sarcomas that cannot be more precisely categorized.[1][2]
Contents
- 1 Epidemiology
- 2 Presentation
- 3 Diagnosis
- 4 Treatment
- 5 Prognosis
- 6 Human-human transmission
- 7 References
- 8 External links
Epidemiology
PUS is regarded as the most common soft tissue sarcoma of late adult life. It rarely occurs in children.[3] It occurs more often in Caucasians than in those of African or Asian descent and is a male-predominant disease, afflicting two males for every female.
Presentation
PUS occurs most commonly in the extremities and retroperitoneum, but has been reported in other sites. Metastasis occurs most frequently in the lungs (90%), bones (8%), and liver (1%).
In the extremities, it presents itself as a painless enlarging soft tissue mass.
Diagnosis
It can be detected by magnetic resonance imaging (MRI), but a biopsy is required for the definitive diagnosis. MRI findings typically show 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.
Pathology
Pleomorphic undifferentiated sarcomas are, by definition, undifferentiated, meaning (as the name implies) that 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
Treatment consists of surgical excision (the extent of which ranges from tumor excision to limb amputation, depending on the tumor) and in almost all cases radiation. Radiation eliminates the need for limb amputation and there is level I evidence to show that it leads to equivalent rates of survival (Rosenberg et al. NCI Canada). Radiation may be delivered either pre-op or post-op depending on surgeon and multidisciplinary tumor board's recommendations. Radiation can be omitted for low grade, Stage I excised tumors with >1cm margin (NCCN). Chemotherapy remains controversial in MFH.
The usual site of metastatic disease is the lungs, and metastases should be resected if possible. Unresectable or inoperable lung metastasis may be treated with stereotactic body radiation therapy (SBRT) with excellent local control. However, neither surgery nor SBRT will prevent emergence of additional metastasis elsewhere in the lung. Therefore, role of chemotherapy needs to be further explored to address systemic metastasis.
Prognosis
Prognosis depends on the primary tumor grade (appearance under the microscope as judged by a pathologist), size, resectability (whether it can be completely removed surgically), and presence of metastases. The five-year survival ranges from 35 to 60%.
Human-human transmission
There is a case report of human-human transmission of cancer, in a surgeon who injured his hand while working on a PUS case.[4]
References
- ^ 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.
- ^ "Neoplasia".
- ^ 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. Retrieved 2010-01-05.
External links
- Malignant Fibrous Histiocytoma (MFH) - Sarcoma Learning Center
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Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215)
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Not otherwise specified |
- Soft-tissue sarcoma
- Desmoplastic small-round-cell tumor
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Connective tissue neoplasm |
Fibromatous |
Fibroma/fibrosarcoma: |
- Dermatofibrosarcoma protuberans
- Desmoplastic fibroma
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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 fibrosis
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Histiocytoma/histiocytic sarcoma: |
- Benign fibrous histiocytoma
- Malignant fibrous histiocytoma
- Atypical fibroxanthoma
- Solitary fibrous tumor
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Myxomatous |
- Myxoma/myxosarcoma
- Cutaneous myxoma
- Superficial acral fibromyxoma
- Angiomyxoma
- Ossifying fibromyxoid tumour
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Fibroepithelial |
- Brenner tumour
- Fibroadenoma
- Phyllodes tumor
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Synovial-like |
- Synovial sarcoma
- Clear-cell sarcoma
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Lipomatous |
- Lipoma/liposarcoma
- Myelolipoma
- Myxoid liposarcoma
- PEComa
- Chondroid lipoma
- Intradermal spindle cell lipoma
- Pleomorphic lipoma
- Lipoblastomatosis
- Spindle cell lipoma
- Hibernoma
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Myomatous |
general: |
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smooth muscle: |
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skeletal muscle: |
- Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
- Alveolar rhabdomyosarcoma
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- Leiomyoma
- Angioleiomyoma
- Angiolipoleiomyoma
- Genital leiomyoma
- Leiomyosarcoma
- Multiple cutaneous and uterine leiomyomatosis syndrome
- Multiple cutaneous leiomyoma
- Neural fibrolipoma
- Solitary cutaneous leiomyoma
- STUMP
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Complex mixed and stromal |
- Adenomyoma
- Pleomorphic adenoma
- Mixed Müllerian tumor
- Mesoblastic nephroma
- Wilms' tumor
- Malignant rhabdoid tumour
- Clear-cell sarcoma of the kidney
- Hepatoblastoma
- Pancreatoblastoma
- Carcinosarcoma
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Mesothelial |
- Mesothelioma
- Adenomatoid tumor
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Index of muscle
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Description |
- Anatomy
- head
- neck
- arms
- chest and back
- diaphragm
- abdomen
- genital area
- legs
- Muscle tissue
- Physiology
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Disease |
- Myopathy
- Soft tissue
- Connective tissue
- Congenital
- abdomen
- muscular dystrophy
- Neoplasms and cancer
- Injury
- Symptoms and signs
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Treatment |
- Procedures
- Drugs
- anti-inflammatory
- muscle relaxants
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Tumors: urogenital neoplasia: urinary organs (C64–C68/D30, 188–189/223)
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Abdominal |
Kidney |
Glandular and epithelial neoplasm: |
- Renal cell carcinoma
- Renal oncocytoma
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Complex and mixed tumor: |
- Wilms' tumor
- Mesoblastic nephroma
- Clear-cell sarcoma of the kidney
- Angiomyolipoma
- Cystic nephroma
- Metanephric adenoma
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by location: |
- Renal medullary carcinoma
- Juxtaglomerular cell tumor
- Renal medullary fibroma
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Ureter |
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Pelvic |
Bladder |
- Transitional cell carcinoma
- Inverted papilloma
- Squamous-cell carcinoma
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Urethra |
- Transitional cell carcinoma
- Squamous-cell carcinoma
- Adenocarcinoma
- Melanoma
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Retroperitoneum |
- Malignant fibrous histiocytoma
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Index of the urinary system
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Description |
- Anatomy
- Physiology
- Development
- Cells
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Disease |
- Electrolyte and acid-base
- Congenital
- Neoplasms and cancer
- Other
- Symptoms and signs
- Urine tests
- Blood tests
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Treatment |
- Procedures
- Drugs
- Intravenous fluids
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