April 2004: woman in her early fifties with a cervical intraspinal and extraspinal mass lesion.
April 2004: A 53-year-old woman presented with a large club-shaped intra- and extraspinal tumor of the C6 nerve root which had intradurally grown through the enlarged C5-6 neural foramen and focally infiltrated the dura mater. Microscopy revealed a melanin-pigmented tumor with spindle-shaped and epithelioid cells and scattered psammoma bodies, a so-called psammomatous melanotic schwannoma (PMS). More than half of the patients with PMS have Carney complex, a genetically heterogeneous multiple neoplasia syndrome of autosomal-dominant inheritance, which in our patient, however, could not be detected unequivocally. Prognosis of all melanotic schwannomas (MSs) is not good with local recurrences or metastases in over 40% of cases. In our case, frequent versicular nuclei with distinct nucleoli, occasional mitoses and apoptoses and increased focal MIB-1 labeling indices prompted us to diagnose a malignant PMS. However, histologic criteria for malignancy in MSs are not clearly defined and there is no reliable histopathological indicator of malignant clinical behavior in MSs. Therefore, designation of "PMS with malignant histologic features" may be more appropriate. Since tumor recurrences and metastases im MSs may occur after more than 5 years, long-term follow-up of affected patients is required. One year after operation our patient showed no signs of tumor recurrence or metastases.
Kuchelmeister
Klaus
K
Institute of Neuropathology, University Hospital Giessen, Germany.
Lotz
Claudia
C
Schönmayr
Robert
R
Schachenmayr
Walter
W
D000951:Antigens, Neoplasm; D005260:Female; D006801:Humans; D058965:MART-1 Antigen; D008279:Magnetic Resonance Imaging / Q000379:methods; D058950:Melanoma-Specific Antigens; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000378:metabolism; D009442:Neurilemmoma / Q000378:metabolism / Q000473:pathology*; D010524:Peripheral Nervous System Neoplasms / Q000378:metabolism / Q000473:pathology*; D009418:S100 Proteins / Q000378:metabolism; D013120:Spinal Cord Neoplasms / Q000378:metabolism / Q000473:pathology*
D000951:Antigens, Neoplasm; D058965:MART-1 Antigen; C548667:MLANA protein, human; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; D009418:S100 Proteins