Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive spindle cell tumors that develop from peripheral nerve sheath cells and account for 5% of all soft tissue sarcomas. MPNSTs involving the lateral skull base are an extremely rare subgroup of these lesions. The article deals with the case of a 51-year-old man with a huge primary MPNST: the disease involved the left frontozygomatic and retroauricolar regions of the scalp with erosion of the outer table of the calvaria and diffuse infiltration of the temporal, infratemporal, and pterygoid fossae. Radical surgery via left transfacial- transparotid approach and reconstruction with microvascular flap were performed. Intraoperative radiotherapy and postoperative radiotherapy were also delivered. Twenty-two months postoperatively, the patient is alive despite recurrence of the tumor in the neck and lung metastases. The article reviews the few series of patients and the small number of case reports of MPNSTs involving the lateral skull base that are available in the English literature describing current concept of pathophysiology, diagnosis, and management of the disease. Although MPNSTs of the lateral skull base have an adverse prognosis, an acceptable survival time and a good quality of life are possible; however, they require an early and correct diagnosis as well as an adequate and aggressive combination therapy.
| Original language | English |
|---|---|
| Pages (from-to) | 805-812 |
| Number of pages | 8 |
| Journal | Journal of Craniofacial Surgery |
| Volume | 19 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - May 2008 |
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Keywords
- Infratemporal fossa
- Lateral skull base
- MPNST
- Pterygoid fossa
- Temporal fossa
ASJC Scopus subject areas
- Surgery
Cite this
Malignant peripheral nerve sheath tumors of the lateral skull base. / Telera, Stefano; Carapella, Carmine; Covello, Renato; Cristalli, Giovanni; Carosi, Maria Antonia; Pichi, Barbara; Spriano, Giuseppe.
In: Journal of Craniofacial Surgery, Vol. 19, No. 3, 05.2008, p. 805-812.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Malignant peripheral nerve sheath tumors of the lateral skull base
AU - Telera, Stefano
AU - Carapella, Carmine
AU - Covello, Renato
AU - Cristalli, Giovanni
AU - Carosi, Maria Antonia
AU - Pichi, Barbara
AU - Spriano, Giuseppe
PY - 2008/5
Y1 - 2008/5
N2 - Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive spindle cell tumors that develop from peripheral nerve sheath cells and account for 5% of all soft tissue sarcomas. MPNSTs involving the lateral skull base are an extremely rare subgroup of these lesions. The article deals with the case of a 51-year-old man with a huge primary MPNST: the disease involved the left frontozygomatic and retroauricolar regions of the scalp with erosion of the outer table of the calvaria and diffuse infiltration of the temporal, infratemporal, and pterygoid fossae. Radical surgery via left transfacial- transparotid approach and reconstruction with microvascular flap were performed. Intraoperative radiotherapy and postoperative radiotherapy were also delivered. Twenty-two months postoperatively, the patient is alive despite recurrence of the tumor in the neck and lung metastases. The article reviews the few series of patients and the small number of case reports of MPNSTs involving the lateral skull base that are available in the English literature describing current concept of pathophysiology, diagnosis, and management of the disease. Although MPNSTs of the lateral skull base have an adverse prognosis, an acceptable survival time and a good quality of life are possible; however, they require an early and correct diagnosis as well as an adequate and aggressive combination therapy.
AB - Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive spindle cell tumors that develop from peripheral nerve sheath cells and account for 5% of all soft tissue sarcomas. MPNSTs involving the lateral skull base are an extremely rare subgroup of these lesions. The article deals with the case of a 51-year-old man with a huge primary MPNST: the disease involved the left frontozygomatic and retroauricolar regions of the scalp with erosion of the outer table of the calvaria and diffuse infiltration of the temporal, infratemporal, and pterygoid fossae. Radical surgery via left transfacial- transparotid approach and reconstruction with microvascular flap were performed. Intraoperative radiotherapy and postoperative radiotherapy were also delivered. Twenty-two months postoperatively, the patient is alive despite recurrence of the tumor in the neck and lung metastases. The article reviews the few series of patients and the small number of case reports of MPNSTs involving the lateral skull base that are available in the English literature describing current concept of pathophysiology, diagnosis, and management of the disease. Although MPNSTs of the lateral skull base have an adverse prognosis, an acceptable survival time and a good quality of life are possible; however, they require an early and correct diagnosis as well as an adequate and aggressive combination therapy.
KW - Infratemporal fossa
KW - Lateral skull base
KW - MPNST
KW - Pterygoid fossa
KW - Temporal fossa
UR - http://www.scopus.com/inward/record.url?scp=44649146652&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=44649146652&partnerID=8YFLogxK
U2 - 10.1097/SCS.0b013e31816aaac4
DO - 10.1097/SCS.0b013e31816aaac4
M3 - Article
C2 - 18520403
AN - SCOPUS:44649146652
VL - 19
SP - 805
EP - 812
JO - Journal of Craniofacial Surgery
JF - Journal of Craniofacial Surgery
SN - 1049-2275
IS - 3
ER -