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Primary
malignant peripheral nerve sheath tumor of the lung in a young child
without neurofibromatosis type 1.Pediatr
Blood Cancer. 2006 Oct 15;47(5):636-8.
Malignant
peripheral nerve sheath tumors (MPNST) are uncommon in children and
almost half of the cases occur in patients with neurofibromatosis 1
(NF1). We report a child with a primary MPNST of the lung without
NF1. MPNST of the lung has similar clinical and radiologic
characteristics as pleuropulmonary blastoma. We suggest to include
MPNST of the lung in the differential diagnosis of intrapulmonary
masses in children.
Primary pulmonary
tumours of nerve sheath origin.Histopathology.
1995 Mar;26(3):247-54.
Primary
intrapulmonary tumours of nerve sheath origin are extremely rare. We
describe the clinicopathological features of four such peripheral
nerve sheath tumours, two benign and two malignant. Of the benign
tumours, one was a typical schwannoma and the other an ancient or
degenerated schwannoma. The typical schwannoma was endobronchial in
origin, diagnosis being established by small bronchoscopic biopsy.
The histological diagnosis of malignant peripheral nerve sheath
tumour required immunohistochemical and/or ultrastructural evidence
of nerve sheath differentiation. One malignant tumour arose in a
patient with Von-Recklinhausen's disease. Both malignant tumours
behaved aggressively with the development of multiple intrapulmonary
metastases, despite the markedly different histological appearance
of the two tumours. Flow cytometric analysis of nuclear DNA content
was performed on the four tumours, all of which showed a diploid DNA
profile.
Endobronchial neurogenic tumors.Ann
Chir. 1992;46(8):742-7
Endobronchial
neurogenic tumours are exceptional and little known. The authors
report two cases of such tumours: a neurofibroma of the right main
bronchus in a 32 year old man and a schwannoma of the left main
bronchus in a 10 year old child. Pneumonectomy was necessary in both
cases due to destruction of the pulmonary parenchyma distal to the
obstruction. The incidence of this type of tumour is estimated to be
between 0.2 and 4%. They may be either schwannomas or neurofibromas,
the discovery of which always raises the problem of the possible
association of von Recklinghausen's disease malignant forms,
neurogenic sarcomas or malignant schwannomas may also be
encountered. The diagnosis is based on endoscopy which reveals the
tumour and allows biopsy. Treatment of these essentially benign
tumours should be conservative, provided the diagnosis is made
early, prior to parenchymal destruction. The prognosis is poor in
the malignant forms and chemotherapy may be useful in malignant
schwannomas.
A
rare case of a primary intrabronchial neurofibroma.Dtsch
Med Wochenschr. 1997 May 23;122(21):682-4
HISTORY AND
CLINICAL FINDINGS: A 42-year-old woman, a smoker for many years, had
suffered from dry cough for some time. She was admitted because of
haemoptyses, the first one month previously. Physical examination
was unremarkable. INVESTIGATIONS: Chest radiography in two planes
showed no abnormality. Computed tomography revealed a
space-occupying lesion adjacent to the right main bronchus, strongly
suspicious of a central bronchial carcinoma. Bronchoscopy showed a
smoothly circumscribed tumour in the upper lobe bronchus which
almost occluded its lumen. Multiple biopsies failed to establish the
benignity/malignity of the tumour. TREATMENT AND COURSE: The tumour
was removed by upper lobe resection. There was no macroscopic
intraoperative evidence of infiltration. Histology provided the
surprising diagnosis of a benign neurofibroma. CONCLUSION:
Thoracotomy should, if at all possible, be performed whenever biopsy
of a pulmonary mass fails to establish its benignity. Intrapulmonary
neurofibroma is very rare, unless it is part of v. Recklinghausen's
disease.
Triton's malignant intrapulmonary tumor.
Rev
Mal Respir. 1998 Oct;15(5):661-4
The authors
report a case of an intrapulmonary tumour which on histological
analysis was found to be a malignant Triton tumour after surgical
excision. The tumours proved to be of nervous tissue origin and was
a rare variant of malignant tumours of the surrounding of peripheral
nerves with rhabdomyoblastic heterologous differentiation which is
rarely described in the lungs. The characteristics of the tumour as
well as therapeutic approaches are discussed followed by a review of
the literature. A particular aspect of the case presented was to
realise that there was a right to left shunt and this is also
discussed.
Primary malignant 'triton' tumour of the lung.Histopathology.
1997 Feb;30(2):140-4.
Two cases of
malignant 'triton' tumour arising within lung parenchyma are
described. The patients were a three-year-old child and a
53-year-old man. Both patients presented with shortness of breath
and a large intrapulmonary mass on chest X-ray. Neither patient had
a history of von Recklinhausen's neurofibromatosis. The lesions were
treated by pneumonectomy. Grossly, both tumours presented as large,
soft and gelatinous intraparenchymatous masses measuring 130 mm and
80 mm, respectively. Histologically, they were characterized by an
atypical spindle cell proliferation embedded in an abundant myxoid
stroma. Focal areas of rhabdomyoblastic differentiation
characterized by large cells with abundant eosinophilic cytoplasm
and occasional cytoplasmic cross-striations could be seen admixed
with the atypical spindle cell elements. Immunohistochemical studies
showed a focal positive reaction for S-100 protein in the atypical
spindle cells embedded within the myxoid stroma, and a strong
positive reaction for desmin and myoglobin in the rhabdomyoblastic
areas. The child died three months after diagnosis with extension of
the tumour into the thoracic cavity. The second patient has been
lost to follow-up. Although rare, malignant 'triton' tumour should
be considered in the differential diagnosis of primary spindle cell
sarcomas of the lung.
A
case of pulmonary neurilemmoma.Nihon
Kyobu Shikkan Gakkai Zasshi. 1989 Jan;27(1):71-4
Intrapulmonary
or bronchial neurilemmoma are rare neoplasms and there have been
only 29 cases reported in the foreign and domestic literature. We
experienced a case of neurilemmoma of the right upper lobe in a
41-year-old male. The patient had no complaint but, a small coin
lesion (1.2 x 1.4 cm) was pointed out by chest X-ray examination.
The tumor was diagnosed as neurilemmoma by transbronchial lung
biopsy and a right upper lobectomy was performed. He has remained
asymptomatic in the 18 months after operation.
A
case of primary intrapulmonary neurilemoma and review of the
literature.Jpn
J Surg. 1989 Nov;19(6):740-6
In this paper,
we present an extremely rare case of a primary intrapulmonary
neurogenic tumor, in which localization of S-100 protein, neuron
specific gamma-enolase (NSE) and CEA was investigated using
immunohistochemical staining. The patient, who was a 39-year-old
man, experienced no symptoms; however, a routine chest X-ray
revealed a round tumor-like shadow in the infrahilar area of the
right lung. As the tumor appeared to be gradually increasing in
size, surgery was performed and histopathological examination of the
excised tumor revealed it to be a primary intrapulmonary neurilemoma.
Immunohistochemical staining demonstrated the presence of S-100
protein in the tumor cells but NSE and CEA were not detected. For
the 2 years following his operation, the patient has been in good
health and is now under careful observation.
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