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Pulmonary blastoma,
so-called because of its resemblance to first trimester fetal lung, is a
rare tumour that occurs at any age, with a mean of about 40 years.
Visit:
Biphasic
Epithelial/Mesenchymal Lung Tumours
This is
a decade or so younger than
carcinosarcomas but considerably later than
blastomas of other organs.
The prognosis is somewhat
unpredictable but the overall five years survival rate is only 16%.
Image Link (Gross)
Differentiation of the connective tissue
component can result in the formation of cartilage, bone, fat and smooth
or striated muscle.
In some tumours the epithelial
element is replaced by compact, undemarcated cords of stromal cells around
a slit-like lumen, others show squamous differentiation.
Image
Link1
;
Image Link2 (microscopic)
Pulmonary blastoma is perhaps just a morphological variant of carcinosarcoma. This
would account for observed transitional forms.
A separate purely sarcomatous growth termed pleuropulmonary blastoma affects the periphery
of the lung of young children and is probably the true blastoma of the
lung.
Pleuropulmonary blastoma- External Image Link
Pulmonary blastoma--a rare tumor with variable presentation.
Eur J Cardiothorac Surg. 2006 Feb;29(2):236-9. Epub 2006 Jan 4.
BACKGROUND: Pulmonary
blastoma is a rare primary lung tumor with poor prognosis that
commonly presents at a younger age than the non-small cell lung
carcinoma (NSCLC). Classically they are large, symptomatic tumors with
lymph nodal metastasis and carry poor prognosis. We report our
experience of 7 patients with pulmonary blastoma who presented with
varying clinical features. METHODS: Retrospective analysis of our
database revealed seven patients with pulmonary blastoma that were
operated between January 1993 and December 2004. During the same time,
889 lung resections were performed for primary NSCLC. Demographic and
clinical details were obtained from hospital notes. The histopathology
reports were reviewed with the department of pathology and the
radiological images were reported. RESULTS: The tumors showed a
bimodal pattern in age at incidence being (four patients were less
than 49 years and three more than 66 years). All patients were
symptomatic and demonstrated variable sized tumors. Lesions were
common in lower lobes (lower:other lobes -4:3) and were staged T2N0
pathologically. All of the patients underwent surgical resections and
had no chemotherapy/radiotherapy following surgery. Three of the seven
patients died during follow-up due to unrelated causes between 24 and
29 months. The longest follow-up was more than 9 years and the
survivor continues to do well. CONCLUSIONS: Pulmonary blastomas are
rare tumors but can present with differing clinical features. Early
detection and treatment may improve prognosis. Further larger series
are needed to evaluate the characteristics of the tumor. |
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