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1 Formerly Specialist Registrar, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK. Now, Canniesburn Plastic Surgery Unit, Jubilee Building, Glasgow Royal Infirmary, 84 Castle Street, Glasgow G4 0SF, UK. 2 Oral Pathology, The School of Dental Sciences, Framlington Place, Newcastle NE2 4BW, UK.
Two rare cases of metastases from malignant peripheral nerve sheath tumours (MPNST) of the head and neck are described. The initial lesions were superficial, but despite early diagnosis and complete excision, they metastasised to the ipsilateral parotid gland. Both underwent further complete excision and neither metastatic lesion showed progression in grade. One patient is alive, however, the other died of widespread metastases. There are no other reported cases of these sarcomas in the head and neck metastasising to the parotid gland.
Malignant sarcomas of the peripheral nerves, which include neurofibrosarcoma, malignant schwannoma and malignant peripheral nerve sheath tumours (MPNST) are rare, the estimated incidence of MPNST being 0.001%, however, they still represent 5–15% of soft tissue sarcomas in the head and neck region.
Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen's disease).
which is locally aggressive, but very rarely metastasises, and the mainstay of treatment is based on a general principle of management of sarcomas, where wide local excision is advised with adjuvant radiotherapy for incompletely excised and high grade disease. We describe the treatment of two cases of superficial MPNST of the head and neck, which metastasised and we can find no similar cases, in the literature.
1. Case 1
In 1995, a 31-year-old labourer presented to his GP with a 10 month history of a painless, slowly growing lesion on his left temple, which on incisional biopsy showed low grade MPNST. Staging procedures were negative and he underwent wide excision and reconstruction with a full thickness skin graft.
Follow-up was uneventful, until 1997 when he noticed a swelling in his left parotid gland but no facial palsy. A CT scan showed a 1.6×1.9 cm mass in the superficial part of the gland (Fig. 1) and fine needle aspiration cytology (FNAC) was unhelpful. A superficial parotidectomy was performed, which confirmed a completely excised metastatic MPNST with extracapsular spread (Fig. 2) .
Fig. 1CT head showing the metastatic lesion in the left parotid gland.
Three years later, he developed subcutaneous, pulmonary and then bony metastases and died of disease after palliative chemo and radiotherapy in 2001.
2. Case 2
In July 2000, a 19-year-old man underwent excision of a skin lesion in the right preauricular region, which was diagnosed as a low grade sarcoma and widely excised at a second operation. Nine months later he developed an FNAC positive metastatic lymph node in the ipsilateral parotid gland. CT demonstrated no other foci and review of the original biopsy together with the metastatic deposit established a diagnosis of MPNST, Trojani grade 2. He underwent a right modified radical neck dissection with incontinuity superficial parotidectomy and histology confirmed a single metastatic deposit in an intra-parotid lymph node with no progression of grade. He received 54 Gy in 27 fractions and is currently disease free 16 months later.
3. Discussion
The estimated incidence of MPNST is 0.001%, but represents 5–15% of soft tissue sarcomas of the head and neck.
Colville RJI, Kelly CG, McLean NR. The multidisciplinary management of Head and Neck sarcomas. British Association of Plastic Surgeons, Winter Meeting, London, November 2002. p. 33–4.
arising from major nerve trunks, especially the sciatic nerve, brachial and sacral plexuses and are, therefore, commoner in the trunk, upper and lower extremities.
which arises in the dermis and subcutis from cutaneous nerves and is locally aggressive with a recurrence rate of 78%. The two cases in this report had wide local excision alone and unusually have had nodal metastasis rather than local recurrence, suggesting that adjuvant radiotherapy would not have helped.
Histological diagnosis has become more stringent because of similarities with other spindle cell malignancies e.g. leiomyosarcomas, malignant fibrous histiocytomas and neurotropic malignant melanoma.
however, cutaneous nerves are generally too small to identify grossly and hence electron microscopy and immunochemistry are used to show ultrastructural evidence of Schwann cell differentiation with absence of premelanosomes and absence of epidermal melanocyte proliferation. There are no specific immunohistological markers, therefore, a panel of antibodies is required and immunoreactivity with at least two of the following antibodies: S100, Leu7, myelin basic protein, glial fibrillary acid protein and PGP 9.5
and allow staging. MPNSTs infiltrate local tissue extensively and spread preferentially, as with other sarcomas, via the bloodstream to liver, lungs and bone rather than the lymphatics.
but there are no statistics recorded. No one has previously described metastases from a superficial head and neck primary to the parotid gland or lymph nodes. Punjabi described an MPNST in the left parotid which metastased to the contralateral parotid and auditory canal with intra-cranial extension, but it is unclear whether the right parotid was the site of the metastasis or was invaded secondarily.
although reviews do not suggest a margin of excision. Since lymph node involvement is very unusual, elective neck dissection is not recommended. Adjuvant high-dose radiotherapy is used, particularly after incomplete resection or where radical excision is impossible, and chemotherapy remains controversial.
The superficial form of the disease after primary surgery has a local recurrence rate of 78%, metastatic spread in 22% and a 4 year survival of 66%. Unfortunately Dabski's series was small and only half the lesions were located in the head and neck region.
We conclude that superficial MPNST of the head and neck region should be excised as widely as possible, and elective neck dissection is not recommended. Adjuvant radiotherapy should be given when the disease is incompletely excised, the margins are close or high grade tumour.
References
Hujala K
Martikainen P
Minn H
Grenman R
Malignant nerve sheath tumours of the head and neck: four case studies and review of the literature.
Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen's disease).
Colville RJI, Kelly CG, McLean NR. The multidisciplinary management of Head and Neck sarcomas. British Association of Plastic Surgeons, Winter Meeting, London, November 2002. p. 33–4.
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