Intrathoracic psammomatous melanotic schwannoma associated with the Carney complex.
The Carney complex is a multiple neoplasia syndrome characterized by myxomas, schwannomas, mucocutaneous spotty pigmentations, and endocrine overactivity with or without endocrine tumors. Herein, we report the rare case of a 49-year-old man with a paravertebral intrathoracic tumor, a history of bilateral adrenalectomy, and resection of an atrial myxoma. A thoracoscopic en-bloc tumor extirpation with minimal safety margins was performed. Histopathologic examination revealed the diagnosis of a malignant psammomatous melanotic schwannoma that is associated with the Carney complex in 50% of these patients. Prognosis of all melanotic schwannomas is usually poor due to local recurrences or metastases. Although treatment guidelines for this rare tumor do not exist, radiotherapy was performed in our patient to prevent possible recurrence or regrowth of this malignant tumor. Twenty-four months after operation the patient showed no signs of tumor recurrence or metastases.
Mees
Soeren Torge
ST
Department of General Surgery, University Hospital of Muenster, Muenster, Germany. soerentorge.mees@ukmuenster.de
Spieker
Tilmann
T
Eltze
Elke
E
Brockmann
Jens
J
Senninger
Norbert
N
Bruewer
Matthias
M
D003480:Cushing Syndrome / Q000453:epidemiology; D006325:Heart Atria; D006338:Heart Neoplasms / Q000453:epidemiology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009232:Myxoma / Q000453:epidemiology*; D009378:Neoplasms, Multiple Primary / Q000453:epidemiology*; D009442:Neurilemmoma / Q000453:epidemiology* / Q000378:metabolism; D010859:Pigmentation Disorders / Q000453:epidemiology*; D013577:Syndrome; D013899:Thoracic Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000378:metabolism; D014057:Tomography, X-Ray Computed