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Although perineural metastases have been studied extensively for various regions of the body, there is a relative paucity of data on cancers of the head and neck region. A case of perineural metastasis from a squamous-cell carcinoma of the lower lip is presented, with symptoms mimicking other neurologic diseases.
We report a case of a malignant peripheral nerve sheath tumor arising from a retroperitoneal ganglioneuroma in an adult male. There was no known previous history of a childhood neuroblastoma or of radiation therapy. In addition, the patient had no stigmata or family history of von Recklinghausen's neurofibromatosis. This case provides evidence that, although rare, spontaneous malignant transformation of ganglioneuroma can occur. Immunohistochemical studies supported that the spindle cell component was of nerve sheath origin.
Tumors of peripheral nerve origin are usually slow growing and minimally symptomatic, making differentiation from other soft tissue neoplasms difficult. Yet failure to recognize a nerve tumor may result in irreversible loss of neurologic function. This article provides current information on the history, pathologic identification, and treatment of upper extremity nerve tumors. Other neoplastic and tumor-like lesions that occur within the peripheral nerve are also considered.
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Peripheral nerve tumors show an interesting histologic variety despite being composed ofa limited array of cellular constituents. As we learn more about the interplay between the Schwann cells, perineurial cells, and ganglion cells that comprise these tumors, it is likely that we will better understand the biologic behavior of these important tumors. Key issues for the pathologist include distinguishing schwannomas from neurofibromas, ganglioneuromas from neurofibromas involving ganglia, and MPNSTs from cellular schwannomas or neurofibromas. The association of each of these tumors with genetic tumor disorders provides a unique window into discovering basic mechanisms of cell regulation and tumorigenesis that may ultimately shed light on the biology of a much wider array of human disease.
Soft-tissue tumors have proved to be a fruitful area for the identification of reproducible cytogenetic aberrations, especially among pediatric round-cell sarcomas and lipomatous tumors. Thus far, however, data regarding sarcomas of monomorphic spindle cell type have been limited and somewhat disappointing, with the notable exception of synovial sarcoma. As part of an ongoing international collaborative study, 130 karyotyped spindle-cell sarcomas were reviewed and classified histologically, without knowledge of the clinical and karyotypic data, with the aim of identifying objective correlations between morphology, karyotype, and clinical parameters. Clonal chromosomal abnormalities were identified in 82 cases studied (63%), but only in the group of synovial sarcomas was there clear correlation between the cytogenetic findings, in the form of a consistent t(X;18)(p11;q11), and morphology. Among leiomyosarcomas (41 cases) and malignant peripheral nerve sheath tumors (MPNSTs; 27 cases) as well as in individual examples of rarer entities, there was a general tendency for karyotypic complexity associated with frequent loss or rearrangement of chromosome arms 1p, 10p, 11q, 12q, 17p, and 22q. Rearrangements of 17q (the region of the NF1 gene) were seen in 9/27 (33%) of MPNSTs. Among nine cases of solitary fibrous tumor (in which previous cytogenetic data are very limited) no consistent aberrations were identified. We conclude that, with the exception of synovial sarcoma, most spindle-cell sarcomas share with pleomorphic sarcomas the tendency for karyotypic complexity. There was no indication (in most of these lesions) that detectable cytogenetic aberrations could either facilitate their diagnosis or help to determine prognosis. There is a clear need to further study and understand the significance of multiple chromosomal abnormalities in this group of mesenchymal neoplasms with the particular goal of determining their role in the process of tumor development.
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Brachial plexus injury with resultant pain and attendant impairment may occur in cancer patients from either disease recurrence or as a consequence of radiation therapy. Distinguishing between these two causes of plexus dysfunction may be difficult, but physical findings and appropriate workup may assist in diagnosis. Additionally, lymphedema may be seen as a result of surgical, radiation, or combination therapy for breast cancer. Patients typically present with arm swelling, but neurological impairment, including pain, may also be noted. This case report describes an individual who developed pain and weakness, initially from radiation injury, and later developed local disease recurrence.
Cytogenetic analyses of malignant peripheral nerve sheath tumors (MPNST) and malignant triton tumors (MTT) are few to date. Two separate triton tumor specimens from one patient and a MPNST specimen from another patient, both with peripheral neurofibromatosis (NF-1, von Recklinghausen disease), showed complex near-triploid complements and partial deletion of the short arm of chromosome 1. Notably, a structural abnormality of chromosome 17 was detected in the MPNST, and loss of chromosome 22 was detected in the MTT. The genes for peripheral neurofibromatosis (NF-1) and central neurofibromatosis (NF-2) have been mapped to these two chromosomes respectively.
Two unique, poorly-differentiated neuroepithelial tumors are described, one in a 35-year-old woman with an anterior mediastinal tumor and one in a 71-year-old woman with a left femoral mass. Immunocytochemical stains demonstrated Neuron specific enolase in both tumors and Chromogranin in one. Electron microscopy showed the cells of both neoplasms to contain abundant, thick, vermiform, organelle-free processes, previously described solely in large cell lymphomas. Rare dense-core granules were present, and very few processes were suggestive of neurites. These observations enlarge the spectrum of poorly differentiated neuroepithelial tumors.
Intrinsic hemangiomas of peripheral nerves are extremely rare. Eight cases have been reported in the literature and only two of them involved the median nerve. An extensive cavernous hemangioma with carpal tunnel symptomatology is reported.
A case of cavernous hemangioma of the palm in combination with an intraneural hemangioma of the ulnar digital nerve of the index finger is described. Treatment consisted of resection of both lesions with the involved part of the digital nerve and bridging of the defect with a nerve graft.
Four cases of lipofibromatous infiltration of the median nerve with long-term follow-up (average 11 years) are reported. Progressive compromise of median nerve function has been noted. Initial treatment considerations should be directed toward biopsy and alleviation of the compression neuropathy. The role of microsurgical debulking procedures remains unclear at this time.
SEPs have been recorded in 15 patients with spinal cord tumors (mostly ependymomas) and in 5 patients with cord compression due to meningiomas (3 cas), neuromas (1 case) or neurosarcoma (1 cas). All the patients have been operated on so that precise informations were available on the histology and the location of the tumor. SEPs were monitored during surgery in 8 patients. The main conclusions of this study are as follows: There was a good correlation between the somatosensory troubles for touch, vibration sense and joint position sense, and SEPs abnormalities, however, SEPs may be clearly abnormal in the absence of any somatosensory deficit when the dorsal columns are compressed and not infiltrated by the tumor. In the latter situation the reverse dissociation (i.e., normal SEPs with somatosensory troubles) may be observed. The possibility of a dissociation between normal N11 and N13 cervical components and absent P14 far field components (non-cephalic reference) has been confirmed in 6 patients with cervicomedullary tumors. In 3 of them, this dissociation was found to be reversible and early SEPs returned to normal after surgery. Peroperative monitoring of scalp SEPs recorded with a non-cephalic reference electrode allowed the detection of transient SEP abnormalities related with a traction on the dorsal columns of the cord. SEP monitoring is recommended for any surgical removal of tumor that needs a posterior myelotomy.
Malignant peripheral nerve sheath tumors (MPNST) are rare. They account for 10% of all soft tissue sarcomas. The incidence of MPNST in patients with neurofibromatosis type 1 (NF-1) is 4%. A retrospective study was undertaken to evaluate the prognosis of patients with MPNST and NF-1 vs patients with MPNST alone. Twenty-two patients with MPNST were diagnosed. The MPNST was diagnosed with NF-1 in 11 patients (50%). Treatment was defined as curative in 20 patients (90%), non-curative in one patient (5%), and one patient received no treatment. The median disease-free survival (DFS) was 14 months (range 0-153 months). The median overall survival 24 months (range 1-153 months). There were three patients with isolated local failures (14%), four patients with local and distant failures (18%), and seven patients with isolated distant failures (32%). There was no statistical difference between patients with and without NF-1. Five patients with NF-1 developed a second MPNST (45%), none of the patients without NF-1 did (P = 0.018). A statistically worse survival was found when the duration of the first symptoms had been longer than six months. Patients with MPNST have a poor prognosis with a high risk for local and distant failures (> 50%). There is no statistical difference in the final outcome for MPNST with or without NF-1, but patients with NF-1 have a high risk for developing a second MPNST.
Peripheral nerve haemangiomas are rare and their association with muscular haemangiomas is exceptional. We report a case of haemangioma of antebrachial flexor muscles in a 19-year-old patient. The diagnosis was established during a Scaglietti-Page type of release operation.
PURPOSE: To review the radiotherapy department experience in treating primary spinal cord ependymomas (PSCE), analyse prognostic factors and provide treatment recommendations regarding literature review. MATERIALS AND METHODS: Sixteen patients with PSCE received postoperative radiotherapy between 1972 and 1997. There were 10 male and 6 female patients with a mean age of 34 years (range 2-63). Surgery was gross total resection in 2 cases, subtotal resection in 9, biopsy in 4 and of unknown type in one patient. All patients were treated with radiotherapy to the craniospinal axis (4 cases), spinal cord (1 case) and to the site of primary tumour in 11 cases. RESULTS: Five year-survival rate was 73%. Two patients had recurrent tumours within the primary site. Gender, extent of surgery and treatment field extent were not prognostic factors. Histologic type was the only variable predictive of outcome. Patients with myxopapillary type had a 5-year survival rate of 100% compared with 47% for those with other histology types. CONCLUSION: We conclude that aggressive surgery is not necessary in the management of PSCE, localised field radiotherapy is associated with favourable outcome, and tumour grade is an important prognostic factor.
The authors describe the unusual case of a patient requiring reoperation for ependymoma of the filum terminale after a symptom-free period of 42 years: treatment of the primary tumor consisted of macroscopically complete surgical excision and postoperative radiotherapy. This case brings to light some interesting observations: a late recurrence, as in the case reported here, appears to be rare especially if the tumor presents in adult age; the long preoperative clinical history (26 months) and the initial aspect of the tumor that infiltrated 2 caudal roots, removed together with the tumor, may have been factors correlated with the risk of recurrence: postoperative radiotherapy may have helped to delay clinical manifestation of the recurrence. Ependymomas of the filum terminale may clinically recur even after complete removal. The latest clinical recurrence described in the literature occurred after 26 years.