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:

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

Experts

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 12/01/1989 - "Tumors in which there was at least a 50% volume reduction (partial or complete remission) included malignant lymphoma (42/63), fibrosarcoma (1/14), solid follicular thyroid carcinoma (3/13), mammary adenocarcinoma (2/8), hemangiosarcoma (2/8), osteosarcoma (1/6), circumanal carcinoma (3/5), synovial cell sarcoma (2/3), undifferentiated sarcoma (2/3), nasal adenocarcinoma (1/2), liposarcoma (1/2), infiltrating lipoma (1/1), malignant melanoma (1/1), sclerosing mesothelioma (1/1), and neurofibrosarcoma (1/2).(ABSTRACT TRUNCATED AT 250 WORDS)"
2.	malignant SchwannomaIBA 08/01/2007 - "The immunohistochemical and histological features indicated a diagnosis of malignant schwannoma rather than neurofibrosarcoma. " 08/01/1998 - "However, excision of the primary mass may reduce the possibility of malignant degeneration into neurofibrosarcoma or malignant schwannoma. " 03/01/1991 - "The term neurogenic sarcoma (or malignant peripheral nerve sheath tumour) is advocated instead of malignant schwannoma. " 05/01/1989 - "Malignant schwannoma (neurofibrosarcoma) metastatic to spine. " 12/01/1983 - "Computed tomography in retroperitoneal neurofibrosarcoma (malignant schwannoma)."
3.	Neurofibromin 1 (Neurofibromin)IBA 08/13/1998 - "High levels of Kit expression in the neurofibrosarcoma-derived Schwann cells correlate with a decrease in neurofibromin expression. " 05/01/1994 - "Recent studies have demonstrated loss of neurofibromin in tumors from NF1 and non-NF1 patients, including neurofibrosarcomas, neuroblastomas and malignant melanomas. " 02/01/1999 - "It is hypothesized, based on data from neurogenic sarcoma cell lines, that loss of neurofibromin leads to increased levels of activated Ras-GTP, and subsequent uncontrolled mitogenic signals to the nucleus. " 02/01/1996 - "The absence of neurofibromin leads to increased levels of Ras-GTP, which contributes to the proliferation of NF1 neurogenic sarcoma cell lines. " 08/01/1994 - "The NF1 gene on chromosomes 17q and its product, neurofibromin, may be important in the formation of neurofibrosarcomas, while the NF2 gene on chromosome 22q and its product, merlin, are probably involved in the formation of schwannomas and other nervous system tumours. "
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 11/01/1999 - "Inhibiting VEGF function may therefore be a useful adjuvant therapy for neurogenic sarcomas." 11/01/1999 - "In this study, the vascularity, vascular endothelial growth factor (VEGF) expression, and effects of inhibiting VEGF receptor on growth of neurogenic sarcomas were examined. " 11/01/1998 - "Increased Ras GTP was associated with increased tumor vascularity in the NF-1 neurogenic sarcomas, perhaps related to increased VEGF secretion. "
7.	Estrogen Receptor alphaIBA 04/10/2005 - "In this study, the methylation status of RASSF1A, p16, MLH1, MSH2 and ERalpha was investigated in 84 primary soft tissue sarcomas (STSs), including 22 liposarcomas, 18 malignant fibrous histiocytomas (MFHs), 18 leiomyosarcomas, 6 rhabdomyosarcomas, 6 neurogenic sarcomas and several other sarcoma entities. "
8.	Angiogenesis Modulating AgentsIBA 09/01/1990 - "These studies suggest that angiogenesis modulators are worthy of further study as feasible means of treating human neurofibrosarcoma."
9.	Vascular Endothelial Growth Factor Receptors (VEGF Receptors)IBA 11/01/1999 - "In this study, the vascularity, vascular endothelial growth factor (VEGF) expression, and effects of inhibiting VEGF receptor on growth of neurogenic sarcomas were examined. " 11/01/1999 - "The small molecule inhibitor (SU5416) of VEGF receptor 2 had no effect on neurogenic sarcoma cell lines in vitro, but the growth of a human tumor explant xenograft model was reduced by 54.8% compared to vehicle. "
10.	ThymidineIBA 01/01/2003 - "A study of the influence of newly synthesized acyclonucleosides and 1,2,3,4-tetrahydroisoquinoline derivatives on deoxythymidine and deoxycytidine kinase activities in human neurofibrosarcoma and ovarian cancer."

Therapies and Procedures

1.	Radiotherapy 03/01/1952 - "Neurogenic sarcoma treated by surgery and radiotherapy." 03/01/2002 - "Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis. " 12/01/1975 - "Twenty-six years after radiotherapy to the spine for ankylosing spondylitis a patient developed a neurofibrosarcoma initially around the left sacroiliac joint. " 12/01/1975 - "Neurofibrosarcoma following radiotherapy for ankylosing spondylitis." 10/01/1992 - "Treatment of a large high-grade neurofibrosarcoma with concomitant vinblastine, doxorubicin, and radiotherapy."
2.	Drug Therapy (Chemotherapy) 05/15/2004 - "When non-neurogenic sarcomas occur in early childhood, a subsequent malignant peripheral nerve sheath tumor can occur as a second malignant neoplasm, especially after alkylating agent chemotherapy and irradiation. " 08/01/1992 - "The doses of radiation had averaged 4144 centigray (range, 2300 to 8000 centigray) and chemotherapy had been administered, when appropriate, for the primary malignant lesion (Ewing sarcoma, malignant fibrous histiocytoma, Hodgkin lymphoma, neuroblastoma, neurofibrosarcoma, rhabdomyosarcoma, and Wilms tumor). " 01/01/2000 - "A total number of 608 cycles of G-CSF and/or GM-CSF was applied in 280 patients aged from 6 months to 20 years during neutropaenia associated with chemotherapy of children's neoplasms (NHL-124, NBL-42, RMS-36, Nephroblastoma-18, Osteosarcoma-17, Ewing's Sarcoma-14, Hepatoblastoma-6, Neurofibrosarcoma-6, PNET-5, Medulloblastoma-3, Fibrohistiocytoma-3, Angiosarcoma-2, other - 4). "
3.	Heterologous Transplantation (Xenotransplantation) 10/01/1990 - "From a panel of 48 human soft tissue sarcomas growing as permanent xenografts, 10 tumor lines (five leiomyosarcomas, three malignant fibrous histiocytomas, two neurofibrosarcomas) have been selected to determine the radiation response to photons and fast neutrons. " 11/01/1999 - "The small molecule inhibitor (SU5416) of VEGF receptor 2 had no effect on neurogenic sarcoma cell lines in vitro, but the growth of a human tumor explant xenograft model was reduced by 54.8% compared to vehicle. "
4.	Free Tissue Flaps 11/01/1987 - "Rectus abdominis myocutaneous free-flap reconstruction following a cranio-orbital-maxillary resection for neurofibrosarcoma."
5.	Sternotomy 10/15/1998 - "Removal of a thymoma via median sternotomy in a rabbit with recurrent appendicular neurofibrosarcoma."