Nerve sheath tumor malignant peripheralC47.-
Synonym(s)
DefinitionThis section has been translated automatically.
Group of malignant neurogenic tumors (MPNST) originating from peripheral nerves or showing nerve sheath differentiation. Occurring in approximately 2-5% in peripheral neurofibromatosis (NF type I), de novo origin and malignant transformation of a preexisting neurofibroma are discussed. MPNST can also occur in pre-irradiated body parts after decades of latency. Molecularly, 17q and 9p deletions are encountered.
ManifestationThis section has been translated automatically.
In case of spontaneous emergence no gender preference; in this case the first manifestation age is >50 years. In connection with neurofibromatosis, malignancy development occurs earlier (35-40 years), in this collective men are preferentially affected.
LocalizationThis section has been translated automatically.
Head and neck, extremities, trunk
Clinical featuresThis section has been translated automatically.
Variably sized, rapidly growing, deeply cutaneous or palpable as a subcutaneous mass, painless also painful, solid nodule. The tumour may develop de novo (rather rarely) or in the context of neurofibromatosis from a long-known neurofibroma.
HistologyThis section has been translated automatically.
Large, blurred, cutaneous-subcutaneous localized tumor formations consisting of densely packed spindle-shaped cells with wavy or comma-shaped nuclei. Rarely palisade formations with Meissner`s corpuscles. Pronounced cell and nuclear atypia, abundant mitoses, many immature fibroblasts, fine argyrophilic neurofibrils. Immunohistochemistry shows positivity for S100 and p53 protein.
Rare variants of the tumor are:
- Epithelioid malignant peripheral nerve sheath tumor (epithelioid cell component).
- Glandular malignant peripheral nerve sheath tumor (glandular structures).
- Malignant triton tumor(rhabdomyosarcomatous differentiation )
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
High recurrence rate even with sufficient excision. 5-year survival rate is 50% for sporadic tumors; 5-year survival rate for underlying neurofibromatosis 20-25%.
LiteratureThis section has been translated automatically.
- Feng CJ et al (2015) Superficial or cutaneous malignant peripheral nerve sheath tumor--clinical experience at Taipei Veterans General Hospital. Ann Plast Surg 74 Suppl 2: S85-88.
- Linos K et al (2015) A misdiagnosed melanoma: a case of cutaneous epithelioid malignant peripheral nerve sheath tumor. Dermatol Online J. 21 pii: 13030/qt319176xb.
- Moulin G et al (1990) Incidence of the degeneration of cutaneous nerve tumors in Recklinghausen's disease. Ann Dermatol Venerol 114: 807-811
- Neville H et al (2003) Pediatric neurofibrosarcoma. J Pediatr Surg 38: 343-346
- Sangueza OP et al (1998) Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms. Am J Dermatopathol 20: 89-102
- Sayed AK et al (1987) Malignant schwannoma in siblings with neurofibromatosis. Cancer 59: 829-835