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Primary pulmonary ganglioneuroblastoma:
a clinicopathologic and immunohistochemical study of two cases.
Ann
Diagn Pathol. 1998 Jun;2(3):154-8.
We present two
patients with primary ganglioneuroblastoma involving the bronchial
wall. The first, a 38-year-old woman, presented with signs and
symptoms suggestive of multiple endocrine neoplasia, including
gastric ulceration and hypercalcemia. Chest radiographic studies
revealed a 3-cm nodule in the hilus of the right lung and two
less-pronounced lesions in the periphery of the right lung. The
second, a 20-year-old asymptomatic woman, was evaluated for a
solitary mass in the upper lobe of the left lung that was
peribronchial and that impinged on the lumen of a bronchus. Grossly,
both neoplasms extended from bronchi, were well-circumscribed, firm,
tan or white, and homogeneous, and measured 5 x 5 cm and 3 x 3 cm,
respectively. Histologically, both tumors were characterized by
neuroblastoma with areas of neuropil and multifocal areas of
ganglion cells. Immunohistochemical studies performed in one case
showed focal staining for neurofilament protein and S-100 protein
and diff use staining for neuron-specific enolase. Follow-up
information showed that one patient died a few days after admission
to the hospital; the second patient has remained well and without
evidence of recurrence or metastases 1 year after initial diagnosis.
These two cases confirm that ganglioneuroblastoma can occur as a
primary pulmonary tumor in adults, presumably arising from
sympathetic ganglia of the bronchus.
Primary pulmonary
ganglioneuroblastoma in an adult: maturation, involution and the immune
response.
Histopathology.
1981 Jul;5(4):451-63.
The features of
a primary pulmonary ganglioneuroblastoma occurring in an adult are
presented. The tumour showed evidence of both maturation and
involution. Maturation appeared to be occurring in a centrifugal
manner, a rim of mature ganglioneuromatous tissue enclosing the
primitive neuroblastoma. Necrosis of the neuroblastomatous element
was widespread and associated with deposition in the walls of
numerous small vessels of an amorphous eosinophilic amyloid-like
material. Accumulation of this material had led to occlusion of some
vessels with resultant necrosis of related tumour. A collarette of
lymphocytes surrounded the tumour, and lymphocytic aggregates were
prominent at the interface between neuroblastoma and ganglioneuroma.
Despite widespread vascular invasion, the patient remains well and
apparently tumour-free, 2 1/2 years post-resection. The appearances
may represent a combined cellular and humoral host response, and a
possible relationship of this response to tumour maturation is
suggested. The potential role of immunostimulation in the treatment
of neuroblastoma is discussed.
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