Neurofibrosarcoma (Neurofibrosarcomas)

A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with NEUROFIBROMATOSIS 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)
Also Known As:
Neurofibrosarcomas; Neurogenic Sarcomas; Neurogenic Sarcoma; Sarcomas, Neurogenic; Sarcoma, Neurogenic
Networked: 94 relevant articles (1 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neoplasms (Cancer)
2. Sarcoma (Soft Tissue Sarcoma)
3. Melanoma (Melanoma, Malignant)
4. Liposarcoma
5. Fibrosarcoma


1. Dokrimare, Nikhil: 1 article (10/2015)
2. Rao, Arunkumar: 1 article (10/2015)
3. Rajurkar, Pawan: 1 article (10/2015)
4. Goyal, Vishav: 1 article (10/2015)
5. Ingle, Sachin B: 1 article (10/2015)
6. Agrawal, Amit: 1 article (12/2013)
7. Baskaran, V: 1 article (12/2013)
8. Jayant, H B: 1 article (12/2013)
9. Jaiswal, Shyam S: 1 article (12/2013)
10. Czekajska-Chehab, Elżbieta: 1 article (01/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neurofibrosarcoma:
1. follicular Thyroid cancerIBA
2. malignant SchwannomaIBA
3. Neurofibromin 1 (Neurofibromin)IBA
4. Dermatofibrosarcoma protuberansIBA
05/01/1990 - "Diagnosis and differential diagnosis between fibrosarcoma and leiomyosarcoma, neurofibrosarcoma, dermatofibrosarcoma protuberans are discussed."
07/01/1988 - "The most frequently involved site was the area near the nasal cavity and maxillary sinus for fibrosarcoma (66%), the base of tongue for hemangiosarcoma (60%), scalp for dermatofibrosarcoma protuberans (54%), parapharyngeal space and soft tissue of the neck for neurogenic sarcoma (45%). "
05/01/1990 - "Ultrastructure of 9 cases of fibrosarcoma was observed and compared with that of fibrous connective tissue of 4 human embryos and 9 cases of spindle cell sarcoma, including 3 each of leiomyosarcoma, neurofibrosarcoma and dermatofibrosarcoma protuberans. "
08/01/2003 - "Analysis of grading of individual sarcomas revealed a concordance in 92% of neurofibrosarcomas, 78% of leiomyosarcomas, 70% of synovial sarcomas, 67% of dermatofibrosarcoma protuberans, 67% of fibrosarcomas, 50% of hemangiopericytomas, and 50% of cases of malignant mesenchymal tumors, spindle-cell type unclassified. "
08/01/2003 - "To evaluate the applicability and accuracy of grading spindle-cell sarcomas on fine-needle aspiration cytology (FNAC) smears, 54 cases of histologically documented spindle-cell sarcomas, consisting of synovial sarcomas (20 cases), neurofibrosarcomas (12 cases), leiomyosarcomas (9 cases), dermatofibrosarcoma protuberans (DFSP; 6 cases), fibrosarcomas (3 cases), hemangiopericytomas (2 cases), and spindle-cell sarcomas, unclassified (2 cases), were graded according to a three-tier system proposed earlier for FNAC smears, while the histological sections were graded using the French Federation of National Cancer Centers (FNCLCC) grading system. "
5. S100 Proteins (S 100 Protein)IBA
01/01/1984 - "This study serves to document the range of fine structure that may be found in neurogenic sarcomas, to correlate the ultrastructural findings with the light microscopic appearance of these tumors, to determine the specificity of the electron microscopic findings, and immunohistochemistry for S-100 protein and assess their possible value in differential diagnosis."
12/01/1982 - "Peripheral nerve tumors, i.e., schwannomas, neurofibromas, granular cell tumors, and neurogenic sarcomas were demonstrated to contain variable amounts of S-100 protein in the tumor cell cytoplasm and nuclei. "
07/01/1997 - "The neurofibrosarcoma was vimentin and S-100 protein-positive and alpha-smooth muscle actin- and desmin-negative. "
01/01/1997 - "Using a streptavidin-biotin-immunoperoxidase procedure, we examined the reactivity of 65 MM (46 conventional, 1 polypoid, 6 desmoplastic [DMM], and 12 metastatic), 21 neurofibromas, 1 neurofibrosarcoma, 10 schwannomas, 1 perineurioma, 2 neurothekeomas, and 14 blue and 26 other nevi for CD-68, HMB-45-defined antigen, S 100 and neurofilament protein. "
07/01/1997 - "Sections from 35 formalin-fixed, paraffin-embedded, canine gastrointestinal stromal tumors consisting of 14 leiomyomas (five stomach, three small intestine, two colon, four rectum), 18 leiomyosarcomas (one stomach, five small intestine, nine cecum, three rectum), two undifferentiated sarcomas (two stomach), and one neurofibrosarcoma (small intestine) were examined for the expression of vimentin, S-100 protein, alpha-smooth muscle actin, and desmin via immunoperoxidase methodology using an avidin-biotin complex technique. "
6. Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA
7. Estrogen Receptor alphaIBA
8. Angiogenesis Modulating AgentsIBA
9. Vascular Endothelial Growth Factor Receptors (VEGF Receptors)IBA
10. ThymidineIBA

Therapies and Procedures

1. Radiotherapy
2. Drug Therapy (Chemotherapy)
3. Heterologous Transplantation (Xenotransplantation)
4. Free Tissue Flaps
5. Sternotomy