Malignant peripheral nerve sheath tumors of the head and neck: two cases and a review of the literature.Abstract
Malignantperipheral nerve sheath tumors are uncommon lesions that occasionally affect the head and neck. We describe 2 new cases of head and neck pathology Head and Neck pathology is the subspecialty of surgical pathology which deals with the diagnosis and characterization of neoplastic and non-neoplastic diseases of the neck, scalp, face, ears, paranasal sinuses, nasal cavity, oral cavity, salivary glands, pharynx, and larynx. . One tumor involved the parotid gland and resulted in erosion of the temporal bone, and the other affected the lower lip. A rapid diagnosis has significant implications for management because of the tumor's potential for aggressive behavior and its high rate of recurrence. To the best of our knowledge, lip involvement is rare and temporal bone involvement has not been previously described.
Malignant peripheral nerve sheath tumors derive from the mesenchymal cells of the neural crest. (1) They account for approximately 5% of all malignant soft-tissue tumors (2); of these, only 15% occur in the head and neck. (3-6) When head and neck tumors do occur, most are located in the parotid gland or in the infratemporal fossa; they have also been reported in the mandible and, very rarely, on the lips. Invasion of the temporal bone, resulting in otorrhea and conductive heating loss, has not been heretofore described. Surgical resection with adjuvant radiotherapy (even with clear resection margins) is the recommended treatment modality. Radiotherapy is desirable even when resection margins are clear because of the tumor's high rate of recurrence. Regional lymph node metastases are rare.
In this article, we describe 2 new cases of malignant peripheral nerve sheath tumor of the head and neck. One case featured temporal bone involvement, and the other was characterized by the location of the tumor on the lip.
Patient 1. A 32-year-old man had been referred to his local hospital for evaluation of a 4-year history of fight-sided hearing loss and recent otorrhea. Examination of the fight ear at that time revealed an attic polyp. No external swelling, parotid enlargement, or cervical lymphadenopathy had been noted. A pure-tone audiogram au·di·o·gram
A graphic record of hearing ability for various sound frequencies.
A chart or graph of the results of a hearing test conducted with audiographic equipment. revealed a 65-dB conductive heating loss in the right ear. Computed tomography (CT) of the temporal bones detected a soft-tissue mass that involved the medial part of the ear canal, the mesotympanum, and the epitympanum (figure 1, A and B). Destruction of the bone of the glenoid fossa of the temporomandibular joint and tegmen tegmen /teg·men/ (teg´men) pl. teg´mina [L.] a covering structure or roof.
tegmen tym´pani tympani was evident.
[FIGURE 1 OMITTED]
A mastoid exploration was performed, and the polyp and biopsy specimens obtained from the middle ear and mastoid were subjected to histologic examination, which identified only a nonspecific inflammatory reaction. Because the histology was not consistent with the clinical findings, the patient was referred to the Department of Otolaryngology and Skull Base Surgery at our hospital for further evaluation.
We obtained magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI), which showed that a well-defined, lobulated lobulated /lob·u·lat·ed/ (lob´ul-at-id) made up of lobules.
made up of lobules. , enhancing mass had arisen from the deep lobe of the fight parotid gland (figure 1, C). The mass involved the external auditory meatus laterally (figure 1, D), and it extended inferiorly posterior to the temporomandibular joint and the ramus of the mandible The ramus of the mandible (perpendicular portion) is quadrilateral in shape, and has two surfaces, four borders, and two processes. Surfaces
The lateral surface is flat and marked by oblique ridges at its lower part; it gives attachment throughout nearly the whole . It also extended medially into the parapharyngeal space anterior to the carotid sheath (figure 1, C). The brain appeared to be normal, as there was no evidence of intracranial extension. No cervical lymphadenopathy was detected.
Angiography of the right internal and external carotid arteries excluded aneurysmal dilation and a vascular tumor. A chest radiograph was normal, as were the complete blood count, erythrocyte sedimentation rate Erythrocyte Sedimentation Rate Definition
The erythrocyte sedimentation rate (ESR), or sedimentation rate (sed rate), is a measure of the settling of red blood cells in a tube of blood during one hour. , autoantibody autoantibody /au·to·an·ti·body/ (-an´ti-bod?e) an antibody formed in response to, and reacting against, an antigenic constituent of one's own tissues.
n. screen, and levels of angiotensin-converting enzyme, antineutrophil cytoplasm antibody (ANCA), and the immunoglobulins. The C-reactive protein level was elevated at 45 mg/L (range of normal: 0 to 6).
The histology of a second biopsy identified fragments of chronically inflamed connective tissue that were covered by hyperplastic and hyperkeratotic squamous epithelium. No evidence of dysplasia was seen, and malignancy was not suspected. In order to obtain tissue for definitive histology, we performed a right temporal bone exploration. The previous mastoid cavity was enlarged, and the incus incus /in·cus/ (ing´kus) [L.] the middle of the three ossicles of the ear, which, with the stapes and malleus, serves to conduct vibrations from the tympanic membrane to the inner ear. Called also
characterized by the development of lesions resembling myxomas. mass was noted to involve the middle ear cavity, and it extended anterosuperiorly to the middle fossa dura. The mastoid cavity and eustachian tube were obliterated with fat and fascia and secured with Tisseel, and a blind sac closure of the ear canal was performed.
Histologic examination revealed that the tumor was largely made up of spindle cells that had a myxomatous appearance secondary to the presence of abundant intercellular connective tissue mucin mucin: see glycoprotein. . The spindle cells contained small hyperchromatic nuclei and long, wavy cytoplasmic processes. This cell morphology blended into a more epithelioid epithelioid /ep·i·the·li·oid/ (-the´le-oid) resembling epithelium.
Of or resembling epithelium.
resembling epithelium. appearance with clumps and cords of nuclei. Focally, there was a cribriform cribriform /crib·ri·form/ (krib´ri-form) perforated like a sieve.
Perforated like a sieve.
perforated like a sieve. pattern created by cells and cell processes that encompassed pools of connective tissue mucin.
Immunohistochemistry showed strong staining of tumor cells for neuron-specific enolase (NSE), patchy staining for S-100 protein, and weak but definite diffuse positivity for protein gene product (PGP) 9.5. The reactions to epithelial membrane antigen (EMA) and cytokeratin were negative. Mild-to-moderate nuclear pleomorphism pleomorphism /pleo·mor·phism/ (-mor´fizm) the occurrence of various distinct forms by a single organism or within a species.pleomor´phicpleomor´phous
1. was noted, but mitoses were not apparent. The proliferation marker MIB-1 showed only a low proportion of cells in cycle.
The combined morphologic and immunohistochemical features were consistent with the epithelioid variant of malignant peripheral nerve sheath tumor. The infiltrative behavior of this tumor clearly confirmed its malignant nature.
The patient underwent complete excision of the tumor by means of an extended total conservative parotidectomy Parotidectomy Definition
Parotidectomy is the removal of the parotid gland, a salivary gland near the ear.
The main purpose of parotidectomy is to remove cancerous tumors in the parotid gland. . This was performed through a postauricular incision extended into the neck, reflecting the pinna pinna /pin·na/ (pin´ah) auricle (1).pin´nal
n. pl. pin·nae
pin forward with the skin flaps elevated over the parotid gland. The main trunk of the facial nerve was identified, which allowed us to preserve its branches while removing the superficial and deep lobes of the parotid gland. The fat obliterating the temporal bone was removed, which resulted in complete exposure of the tumor in the parapharyngeal space and in the anterior part of the temporal bone. A complete macroscopic excision of the tumor was achieved with the assistance of intraoperative frozen sections. The deep lobe of the parotid gland was completely excised medial to the facial nerve. Despite wide surgical clearance, the pinna, the meniscus of the temporomandibular joint, and the head of the mandible were preserved. The temporal bone was obliterated with fat and fascia in the same fashion as the mastoid cavity and eustachian tube were obliterated in the previous surgical procedure.
The final histology report confirmed that the tumor arose from a large myelinated myelinated /my·eli·nat·ed/ (mi´e-li-nat?ed) having a myelin sheath.
Having a myelin sheath.
having a myelin sheath. nerve and was made up of interlacing spindle cells set in a mucinous mucinous /mu·ci·nous/ (mu´si-nus) resembling, or marked by formation of, mucin.
relating to, resembling or containing mucin. matrix. Focally, these cells coalesced to form acinar acinar /ac·i·nar/ (as´i-nar) pertaining to or affecting one or more acini.
Relating to an acinus.
pertaining to or affecting an acinus or acini. and cribriform patterns. Immunohistochemistry yielded a strong cytoplasmic staining for S-100 protein and moderate staining for PGP 9.5 and NSE. The reactions for EMA and desmin were negative. No evidence of lymph node involvement was noted. As was the case with the previously analyzed specimen, mitoses were scant and proliferation marker MIB-1 showed only a low proportion of cells in cycle.
The patient subsequently received postoperative radiotherapy. Follow-up was carried out regularly for 3 years at our center with serial MRIs. Subsequent follow-up was conducted at the referring hospital, and there has been no sign of recurrence.
Patient 2. A 64-year-old man was referred to us by his general practitioner for evaluation of a progressive bilateral sensorineural hearing loss Sensorineural hearing loss
Hearing loss caused by damage to the nerves or parts of the inner ear governing the sense of hearing.
Mentioned in: Tinnitus
sensorineural hearing loss , primarily in the high frequencies (4 to 8 kHz).
During clinical examination, we made an incidental finding of a firm, nonulcerated, 3-mm nodule on the lower lip just to the left of the midline. Findings on the remainder of the examination were completely normal. The patient reported that the lip lesion had been present for at least 3 months, and it had become more sensitive to touch over the previous 3 weeks. Our clinical impression was that this lesion represented a fibroma fibroma /fi·bro·ma/ (fi-bro´mah) pl. fibromas, fibro´mata a tumor composed mainly of fibrous or fully developed connective tissue. , and a wedge excision was performed.
On macroscopic examination, we noted that the gray-tan soft-tissue mass was covered by surface epithelium. The mass contained a 0.4-cm nodule, which was identified on histologic examination as a cellular infiltrative lesion made up of pleomorphic pleomorphic adjective Referring to a variable appearance or morphology spindle cells and cuboidal cells with indistinct eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik)
1. readily stainable with eosin.
2. pertaining to eosinophils.
3. pertaining to or characterized by eosinophilia. cytoplasm (figure 2, A and B). The nuclei were enlarged and hyperchromatic, and a few mitotic figures were seen. No evidence of necrosis was noted. Cells were arranged in fascicles in some places and in loose cords of less dense cells in other areas. There was a prominent myxoid myxoid /myx·oid/ (mik´soid) mucoid.
Containing or resembling mucus; mucoid.
myxoid adjective 1. change of the intervening stroma. Nerve twigs within the biopsy specimen (figure 2, B) indicated perineural infiltration by the tumor.
[FIGURE 2 OMITTED]
On immunocytochemistry im·mu·no·cy·to·chem·is·try
The study of cell constituents by immunologic methods, such as the use of fluorescent antibodies.
immunocytochemistry , the tumor cells expressed S-100 protein, confirming the tumor's neural origin (figure 2, C). The cells also expressed glial fibrillary acidic protein Glial fibrillary acidic protein (GFAP) is an intermediate filament (IF) protein that is found in glial cells such as astrocytes. First described in 1971, GFAP is a type III IF protein that maps, in humans, to 17q21. (GFAP GFAP glial fibrillary acidic protein. ); although GFAP is often positive in low-grade lesions, it is usually lost in high-grade neoplasms. The tumor did not express smooth-muscle actin or pancytokeratin markers. The tumor's morphologic appearance and immunocytochemical phenotype were consistent with a low-grade epithelioid-type malignant peripheral nerve sheath tumor.
Despite the clear resection margins, the patient underwent postoperative radiotherapy because of the perineural invasion. No recurrence was observed during follow-up.
The estimated yearly incidence of malignant peripheral nerve sheath tumors is 1 per 100,000 population. (3-6) These tumors usually occur between the third and fifth decades of life, and their distribution between the sexes is equal. (3-6) They have been associated with neurofibromatosis Neurofibromatosis Definition
Neurofibromatosis (NF), or von Recklinghausen disease, is a genetic disease in which patients develop multiple soft tumors (neurofibromas). These tumors occur under the skin and throughout the nervous system. in as many as 38% of cases. (5-9) Malignant peripheral nerve sheath tumors usually arise in major nerve trunks, such as the sciatic nerve, brachial plexus, and sacral plexus. (10) Therefore, they typically occur in the extremities and the trunk. (3-5,11) These tumors can arise de novo as a solitary mass or multiply, as is seen in cases of neurofibromatosis type 1. (9)
Roughly 15 % of all malignant peripheral nerve sheath tumors occur in the head and neck. (3-6) Approximately 100 cases of malignant peripheral nerve sheath tumors of the head and neck have been reported, some of which involved the parotid and infratemporal fossa (table). (3-6,11-15)
The overall prognosis is poor, and reported 5-year survival rates range from 23 to 67%. (3-6,11-15) Survival is worse in patients with centrally located or large tumors and in patients with neurofibromatosis type 1.
Malignant peripheral nerve sheath tumor, which is a sarcoma that arises from a nerve or displays features of neural differentiation, must not be confused with its benign counterpart, neurilemoma. (2) Malignant peripheral nerve sheath tumor can originate in any cell of the nerve sheath, including Schwann's cells and fibroblasts that surround motor and sensory nerve axons. (2) These tumors grow longitudinally along the length of a nerve. They assume a fusiform fusiform /fu·si·form/ (-form) shaped like a spindle; tapered at each end.
Tapering at each end; spindle-shaped.
spindle-shaped. appearance without compromising the morphologic or functional integrity of the nerve. As a result, they can be surgically separated from their nerve of origin.
In our patient 1, no neurologic deficit was elicited pre- or postoperatively as a consequence of the tumor or its excision (with the exception of some weakness of the mandibular branch of the facial nerve after surgical exposure). That tumor might have arisen from any of the lower four cranial nerves where they pass through the skull base, from the auriculotemporal nerve, or from the otic ganglion (which acts as a relay station for parasympathetic parasympathetic /para·sym·pa·thet·ic/ (-sim?pah-thet´ik) see under system.
Of, relating to, or affecting the parasympathetic nervous system. secretomotor fibers to the parotid gland).
The histologic appearance of these tumors can vary considerably, (16) but most masses are made up primarily of a monomorphic monomorphic /mono·mor·phic/ (-mor´fik) existing in only one form; maintaining the same form throughout all developmental stages.
mon·o·mor·phic or mon·o·mor·phous
1. population of small spindle cells that are usually arranged in fascicles in a herringbone pattern. These more solid areas normally alternate with areas that have a loose or myxoid appearance. Microvascular proliferation and perineural spread are common findings, and in approximately 20% of cases there is focal divergent differentiation toward mesenchymal elements (e.g., rhabdomyosarcoma rhabdomyosarcoma /rhab·do·myo·sar·co·ma/ (mi?o-sahr-ko´mah) a highly malignant tumor of striated muscle derived from primitive mesenchymal cells. , chondrosarcoma, osteosarcoma osteosarcoma /os·teo·sar·co·ma/ (os?te-o-sahr-ko´mah) a malignant primary neoplasm of bone composed of a malignant connective tissue stroma with evidence of malignant osteoid, bone, or cartilage formation; it is subclassified as , angiosarcoma angiosarcoma /an·gio·sar·co·ma/ (an?je-o-sahr-ko´mah) a malignant neoplasm arising from vascular endothelial cells; the term may be used generally or may denote a subtype, such as hemangiosarcoma. , and liposarcoma) or toward glandular and squamous-type epithelial tumors. (8) The explanation for this wide range of neoplastic phenotypes is that cells of the neural crest also contribute to the formation of leptomeninges leptomeninges /lep·to·me·nin·ges/ (lep?to-me-nin´jez) sing. leptome´ninx the pia mater and arachnoid taken together; the pia-arachnoid.leptomenin´geal
n. , bone, cartilage, and muscle in the head, neck, and face. (17) Secondary features of malignant peripheral nerve sheath tumors include myxoid change, hyalinization, and geographic necrosis. Approximately two-thirds of cases feature a benign neurofibromatous component, supporting the view that malignant peripheral nerve sheath tumors arise as a result of malignant transformation in a preexisting pre·ex·ist or pre-ex·ist
v. pre·ex·ist·ed, pre·ex·ist·ing, pre·ex·ists
To exist before (something); precede: Dinosaurs preexisted humans.
v.intr. benign lesion. (9)
Imaging with CT, MRI, and angiography can differentiate this tumor from other possible pathologies, such as an aneurysmal formation, vascular tumor, lipomata, or a lytic lytic /lyt·ic/ (lit´ik)
1. pertaining to lysis or to a lysin.
2. producing lysis.
1. Of, relating to, or causing lysis.
2. lesion in the temporal bone or infratemporal fossa, such as a cholesteatoma, meningioma meningioma /me·nin·gi·o·ma/ (me-nin?je-o´mah) a benign, slow-growing tumor of the meninges, usually next to the dura mater, which may invade the skull or cause hyperostosis, and often causes increased intracranial pressure; it is usually , metastasis, ossifying ossifying /os·si·fy·ing/ (os´i-fi?ing) changing or developing into bone.
changing or developing into bone. fibroma, osteosarcoma, or fibrous dysplasia. In this regard, CT and MRI CT and MRI
Two high technology methods of creating images of internal organs. Computerized axial tomography (CT or CAT) uses x rays, while magnetic resonance imaging (MRI) uses magnet fields and radio-frequency signals. Both construct images using a computer. are superior to other imaging techniques and to physical examination.(18) Although CT and MRI do not provide information regarding a specific histologic diagnosis, they may yield useful information that may differentiate benign from malignant tumors. (10,19) Bony destruction of the mandible or skull base is best visualized on CT (figure 1, A and B), whereas bone marrow involvement is best demonstrated on MRI. (20)
MRI is superior to CT in demonstrating the internal architecture of salivary gland tumors Salivary Gland Tumors Definition
A salivary gland tumor is an uncontrolled growth of cells that originates in one of the many saliva-producing glands in the mouth. in a multiplanar fashion and in delineating the interface between the tumor and the normal parotid gland. (20) Neurogenic neurogenic /neu·ro·gen·ic/ (-jen´ik)
1. forming nervous tissue.
2. originating in the nervous system or from a lesion in the nervous system. tumors usually enhance intensely with gadolinium diethylenetriamine-penta-acetic acid (DTPA DTPA diethylenetriamine pentaacetic acid; see pentetic acid.
diethylenetriamine penta-acetic acid. ), whereas glomus tumors have the characteristic serpiginous serpiginous /ser·pig·i·nous/ (ser-pij´i-nus) creeping; having a wavy or much indented border.
adj. areas of flow void (salt-and-pepper pattern) on MRI. (20) On MRI, most malignant peripheral nerve sheath tumors demonstrate moderate enhancement on T1-weighted imaging and marked enhancement on T2-weighted imaging, especially with gadolinium contrast (figure 1, C and D). (19)
The diagnosis is based on histology and confirmed by a positive reaction to immunohistochemical markers. Malignant peripheral nerve sheath tumors usually demonstrate focal S-100 protein positivity, (21) although as many as one-third of tumors may be S-100-negative. They may show reactivity for Leu-7 or myelin basic protein, but they are negative for cytokeratins. (22) Tumors are occasionally positive for the melanocytic marker HMB-45. NSE has been widely used to demonstrate neural or neuroendocrine differentiation in tumors, and it may also be found in many gliomas and carcinomas of nonendocrine origin. NSE is best used only in conjunction with other markers to provide supportive evidence of neural differentiation. (21)
The optimum treatment is combined surgical excision with radiotherapy. (3,5,23) Radiotherapy is indicated because of the tumor's high recurrence rate (18 to 76%) and discrete 5-year survival rate (23 to 67%). (3-6,11-15) These poor outcomes are even worse in patients with high-grade tumors. (12,23) Survival is also worse in patients with head and neck tumors because of the technical difficulties posed by anatomic constraints to achieving complete clearance. (11)
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Pablo Marinez Devesa, FRCS (Edin); Timothy E. Mitchell, MA, FRCS (ORL-HNS); Ian Scott, FRCPath; David A. Moffat, BSc, FRCS
From the Department of Otolaryngology and Skull Base Surgery (Dr. Martinez Devesa, Dr. Mitchell, and Dr. Moffat) and the Department of Histopathology (Dr. Scott), Addenbrooke's NHS Trust.
Reprint requests: Mr. Pablo Martinez Devesa, ENT Department, Wexham Park Hospital, Slough, Berkshire SL2 4HL, UK. Phone: 44-1753-633-090, fax: 44-118-322-7147; e-mail: email@example.com
Table. Summary of reported cases of malignant peripheral nerve sheath tumors of the head and neck (excluding triton tumor) Author No. patients Treatment Das Gupta and Brasfield, (3) 1970 18 Sx + RT Ghosh et al, (4) 1973 16 (4 w/NF) Sx Sordillo et al, (5) 1981 18 (? w/NF) Sx + RT Greager et al, (12) 1985 8 (2 w/NF) Sx + RT Basso-Ricci, (13) 1989 6 Sx + RT Colmenero et al, (11) 1991 7 (2 w/NF) Sx + RT Meis et al, (6) 1992 16 (4 w/NF) Sx Dvornik et al, (14) 1996 1 Sx Loree et al, (15) 2000 17 (7 w/NF) Sx + RT Martinez Devesa et al, ([dagger]) 2006 2 Sx + RT 5-year Recurrence Author survival * (%) * (%) Das Gupta and Brasfield, (3) 1970 49 46 Ghosh et al, (4) 1973 66 18 Sordillo et al, (5) 1981 23 52 Greager et al, (12) 1985 67 50 Basso-Ricci, (13) 1989 66 44 Colmenero et al, (11) 1991 50 60 Meis et al, (6) 1992 NR 14 Dvornik et al, (14) 1996 NR NR Loree et al, (15) 2000 52 76 Martinez Devesa et al, ([dagger]) 2006 -- -- * Reported rates include patients without head and neck cancers. ([dagger]) Present report. Key: Sx = surgery; RT = radiotherapy; NF = neurofihromatosis; NR = not reported.