Malignant schwannoma associated with von Recklinghausen's neurofibromatosis

Abstract

A series of 46 malignant schwannomas occurring in soft parts of patients having von Recklinghausen's neurofibromatosis was analyzed. The diagnosis of malignant schwannoma was based upon the occurrence of malignant spindled cells closely resembling Schwann cells in the neoplasm and the close association or origin of the malignant schwannoma in a neurofibroma (27 tumors), or a large peripheral nerve (31 tumors). Additional histologic features useful in making the diagnosis of malignant schwannoma included the arrangement of the spindled tumor cells in a whorled pattern about thin-walled, gaping blood vessels, perivascular cellular proliferation and the presence of prominent myxoid stroma containing abundant hyaluronidase-sensitive acid mucopolysaccharides. Nuclear palisading was present in only one case. Eight tumors containing both neoplastic Schwann cells and rhabdomyoblasts and five containing both neoplastic Schwann cells and rhabdomyoblasts (malignant "Triton" tumors) and five containing foci of malignant cartilage cells were included in the series. The neoplasms occurred principally in adults (median age, 34 years) and were most common in the lower extremity (18 cases) and retroperitoneum (11 cases). A mass with or without pain was the most common presenting symptom (28 cases). The median size of excised tumors was 11 cm. The malignant schwannomas were highly malignant neoplasms, causing the death of 39 patients within five years and two patients within 6--10 years after diagnosis. Only four patients were alive and free of tumor 5--15 years after diagnosis.

Malignant Peripheral Nerve Sheath Tumors

“…In fact, our 5-year local recurrence rate of 18% was notably lower than all other series, which may be explained by the fact that fewer patients in others reports received combination therapy (35–64% of patients received RT vs. 100% in the current series, Table 4). 6,8,9,10,12,14,15,17 For comparison, two older series from Memorial Sloan Kettering Cancer Center (MSKCC) and Mayo Clinic reported 5-year local relapse rates of 40% and 42%, respectively. 8,14 Additionally, Kahn and colleagues from the National Cancer Institute (NCI) recently reported a 5-year recurrence rate of 55% in patients not receiving RT.…”

Spécificité de la transformation sarcomateuse de la maladie de Recklinghaussen : a propos de deux cas et revue de la littérature

“…Ces TMNP peuvent être multiples. Pour Guccion et al [8], les TMNP liés à la NF1 présentent une prédominance masculine nette: 8 hommes pour 2 femmes. L’âge au diagnostic est nettement inférieur à l’âge moyen de diagnostic d'un STMA dans la population générale: âge moyen de 29 ans dans la série de Ducatman et al [9], de 32 ans dans la série de Guccion et al [8], de 36 ans dans la série de Hruban et al [10].…”

Atypical and malignant peripheral nerve-sheath tumors of the brachial plexus: Report of three cases and review of the literature

“…The greatest risk is for patients who have had neurofibromatosis for more than 10 years, and only rarely do MPNST arise within 5 years of diagnosis of neurofibromatosis. 13 …”