[Rare manifestation of nasal polyposis in the Peutz-Jeghers syndrome].
We are relating a case of the Peutz-Jeghers syndrome, a disease characterized by hamartomatous polyposis and by mucocutaneous melanic pigmentation, in a rare variant identified by the presence of nasal polyposis. At the objective E.N.T. examination, the patient's nasal fossae were completely obstructed by translucid masses of a polypoid appearance and, moreover, a melanic pigmentation of the cutis, of the perilabial mucosa and of the oral cavity was encountered. At the X-ray examination and, later, during the operation, also the right maxillary sinus appeared completely obstructed by polypoid masses. At the histologic examination, such polypoid neoformations were quite similar to common polyps of an inflammatory nature and, so, absolutely without any peculiar structural features. The nasal polyposis in patients suffering from the Peutz-Jeghers syndrome has been mentioned by Peutz since his first observation but it has been seldom described in the subsequent publications, even in relation to the presentation of rare causes to Congresses and Magazines of an exclusively gastroenterological interest, in which the main concern was that of identifying both the clinical and histological aspects of gastrointestinal hamartomatous polyposis and the correlation between the syndrome and the formation of malignant neoplasias in other organs to the prejudice of the secondary manifestations of the disease. Nevertheless, the nasal polyposis and, at any rate, the tendency to the formation of polyps in other districts (urinary tract, bronchi) are described among the less important features also in gastroenterological treatises of most recent publication. Clinical observations, even occasional, are completely missing in the E.N.T. ambit.(ABSTRACT TRUNCATED AT 250 WORDS)
Cerqua
N
N
USL RM 12, Divisione ORL, Ospedale San Filippo Neri, Roma.
D'Ottavi
L R
LR
Perrotti
V
V
Coen Tirelli
G
G
Piccirillo
E
E
Spaziani
G
G
D000328:Adult; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D007417:Intestinal Polyps / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D008297:Male; D008548:Melanosis / Q000150:complications; D009298:Nasal Polyps / Q000150:complications / Q000175:diagnosis* / Q000473:pathology*; D010256:Paranasal Sinuses / Q000473:pathology / Q000648:ultrastructure; D010580:Peutz-Jeghers Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology
Rare manifestazioni della poliposi nasale nella sindrome di Peutz-Jeghers.