Open AccessNeurofibromatosis type 1‐associated malignant peripheral nerve sheath tumour carcinomatous pleurisy: an autopsy case
Author(s) -
Furukawa Marie,
Ota Hiroki,
Nakamura Yasuhiko,
Nihonyanagi Yasuhiro,
Tochigi Naobumi,
Homma Sakae
Publication year - 2019
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.463
Subject(s) - medicine , autopsy , neurofibromatosis , pleural effusion , pathology , malignant transformation , bloody , metastasis , malignant peripheral nerve sheath tumor , radiology , cancer , surgery
A 57‐year‐old man with neurofibromatosis type 1 (NF‐1) and intrathoracic meningoceles was admitted to hospital after presenting with neck pain and progressive dyspnoea. On admission, a chest computed tomography scan demonstrated right pleural effusion, neck tumour, intrathoracic meningoceles, and rib metastasis. The myelography showed no transportation between the intrathoracic meningoceles and pleural cavity. As a result, these radiological finding indicated the potential for malignant transformation. The appearance of the right pleural effusion was bloody and had no malignant cells. We biopsied the neck tumour, and the tissue showed glass‐like materials but no malignant cells. At 1 month after admission, he developed bladder–rectal disorder, syndrome of inappropriate secretion of antidiuretic hormone, and paralysis of both legs and later died. An autopsy demonstrated glass‐like material in the neck tumour, which was surrounded by malignant cells. NF‐1 appears to have progressed to a malignant peripheral nerve sheath tumour in this patient.