Mediastinal atypical carcinoid and neurofibromatosis type 1

Citation
P. Mathew et al., Mediastinal atypical carcinoid and neurofibromatosis type 1, ARCH PATH L, 124(2), 2000, pp. 319-321
Citations number
5
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Research/Laboratory Medicine & Medical Tecnology","Medical Research Diagnosis & Treatment
Journal title
ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
ISSN journal
0003-9985 → ACNP
Volume
124
Issue
2
Year of publication
2000
Pages
319 - 321
Database
ISI
SICI code
0003-9985(200002)124:2<319:MACANT>2.0.ZU;2-O
Abstract
We report a case of mediastinal atypical carcinoid in a 63-year-old woman w ith neurofibromatosis type 1 (NF-1), who presented with shortness of breath and a bulky mediastinal mass. Initial consideration was given to a neuroge nic tumor. However, on thoracoscopic biopsy, the histologic appearance was consistent with an atypical carcinoid. Carcinoid tumors have been reported in association with NF-1 previously, but commonly in such unusual sites as the ampulla of Vater and duodenum and not in the thorax. The bulky, extensi ve, and highly vascular nature of the lesion precluded resection or debulki ng surgery. To our knowledge, there are no previous reports of atypical car cinoid of the lung or mediastinum in a patient with neurofibromatosis. This case report, therefore extends the spectrum of solid neoplasia in general and carcinoid tumors in specific, as they occur in association with NF-1.