2015, Volume 31, Number 1, Page(s) 068-071
Pleuropulmonary Blastoma: A Case Report
Sümeyye EKMEKCİ1, Anıl AYSAL1, Nur OLGUN2, Mustafa OLGUNER3, Handan ÇAKMAKCI4, Erdener ÖZER1
1Department of Pathology, Dokuz Eylül University, Faculty of Medicine, İZMİR, TURKEY
2Department of Children Health and Disease, Dokuz Eylül University, Faculty of Medicine, İZMİR, TURKEY
3Department of Pediatric Surgery, Dokuz Eylül University, Faculty of Medicine, İZMİR, TURKEY
4Department of Radiology, Dokuz Eylül University, Faculty of Medicine, İZMİR, TURKEY
Keywords: Lung neoplasms, Child, Pleuropulmonary blastoma
Pleuropulmonary blastoma is rare embryonal tumor of infancy and
early childhood and it often arises from lung and more rarely from
the parietal pleura. We present this entity which has no systematic
data associated with its incidence in order to discuss clinical,
histopathological, immunohistochemical features and the differential
diagnosis. A three-year-old boy presented with fever showed signs of
upper respiratory tract infection. Radiological examination revealed
a solid mass filling the right hemithorax. The patient underwent
core needle biopsy, wedge biopsy and lobectomy. Biopsy and
surgical material were examined histopathologically. The tumor was
composed of predominantly solid areas consisting blastemal cells
with spindle, polygonal and round nuclei in the myxoid stroma.
Immunohistochemical staining of the tumor cells were positive
with vimentin and desmin. MIB-1 labeling index was above 90%.
Histological diagnosis was pleuropulmonary blastoma type 3. The
surgically sampled adjacent diafragma was also infiltrated with the
tumor. The patient was treated with chemotherapy and showed no signs
of recurrence in the follow-up of 9 months. Pleuropulmonary blastoma
is a very rare childhood cancer that needs to be kept in mind in the
pathological differential diagnosis of thoracic tumors in the children.
Pleuropulmonary blastoma (PPB) is a lung cancer1,2
that is very uncommon in children. Manivel et al.1
described the presence of PBP in children in 1988 that is
different from the classical adult type biphasic epithelialstromal
morphology. PPB is observed in children of
different ages varying from one month to twelve years. The
incidence rates for PPB between genders are similar. Initial
findings are usually flu-like symptoms with or without fever3
Dehner4 described three different types of PPB
morphologically according to gross and microscopic
features. The prognosis is strongly correlated with the
histological type. Five-year survival rate was 80-90% in
patients with type 1, while 50% in type 2 and 3. In this report we present a case of type 3 PPB in order to discuss
the clinical, histopathological, immunohistochemical
features and the differential diagnosis of the disease.
A three-year-old boy presenting with fever and symptoms
of upper respiratory tract infection was admitted to the
hospital. A solid tumoral lesion filling the right hemithorax
and accompanying by the pleural fluid was observed
radiologically (Figure 1
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|Figure 1: Magnetic resonance imaging revealed a huge pulmonary
tumor with a maximum 14 cm diameter occupying the right
Core needle biopsy of the lesion demonstrated histologically
small tissue fragments consisting of primitive round
embryonal cells in a loose myxoid stroma (Figure 2).
Although these cells were predominantly monotonous,
some showed eosinophilic cytoplasm and larger nucleus (Figure 3). These morphological findings were suspicious
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|Figure 2: Primitive round embryonal cells in a loose myxoid
stroma (H&E; X100).
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|Figure 3: Rhabdomyomatous transformation of the tumor cells
with eosinophilic cytoplasm (H&E; X200).
A surgical biopsy was also performed later by thoracotomy.
On histopathological evaluation, predominantly solid
partially necrotic tumor tissue was observed. The tumor
tissue also contained a few cystic areas. The tumor cells
present in the myxoid stroma were round or fusiform in
shape and their nuclei were hyperchromatic. These blastomatous
looking tumor cells were organised in some areas
resembling the cambium layer of the rhabdomyosarcomas.
Immunohistochemically the tumor cells were stained diffusely
positive with vimentin, weakly positive for desmin
and negative Mic-2 (CD99) and actin. MIB-1 index was
After debulking of the tumor by four cycles VAC (Vincristine,
actinomycin-D, cyclophosphamide) chemotherapy, a
right upper lobectomy was performed. Macroscopically
the lobectomy specimen showed the tumoral lesion of
7x9 cm with solid tan-coloured cut surface (Figure 4).
Histopathological diagnosis was type 3 PPB characterized
by solid areas consisting of rhabdomyosarcomatous cells.
These cells appeared primitive and atypical but some showed
large eosinophilic cytoplasm. Immunohistochemical
staining revealed positive immunoreactivity with desmin
and vimentin (Figure 5). The final pathological diagnosis
was PPB type 3. A diaphragmatic tissue sampled surgically
also revealed a tumoral infiltration that was similar
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|Figure 4: The cut surface of the macroscopical specimen. Note the
tumor consisting of solid areas.
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|Figure 5: Strong positivity by immunhistochemistry in rhabdomyosarcomatous
areas. (desmin, X100).
The hilar lymph nodes were negative. ICE (ifosfamide,
carboplatin, etoposide) treatment protocol was started and
the overall prognosis in the 9 months follow-up is very
good without any evidence of recurrence.
Although intrathoracic neoplasia and neoplasms of the
chest wall are not common, pulmonary metastases are
the most frequent intrathoracic neoplastic lesions in
. Pleuropulmonary blastoma (PPB) is an
aggressive tumor that includes less than 1% of all primary
malignant pulmonary tumors in the pediatric population.
A family history is evident in 25-30% of the cases usually
presenting with cystic lesions and childhood cancers5
There are presently over 100 cases registered with The
Pleuropulmonary Blastoma Registry (www.ppbregistry.
org). Initial symptoms are usually flu-like illness with or
. Some patients with type 2 and 3 PPB
may present with lung cysts. The radiologic findings of a
PPB include different patterns of masses lesions and/ or
cysts such as unilateral, rarely bilateral, localized air-filled
cysts or a purely solid mass. Rarely, PPB is diagnosed when
metastatic involvement of brain, bone, lymphoid tissue,
liver, pancreas, renal and surrenal tissue occurs6,7
Dehner4 defined three types of PPB morphologically.
Type 1 was characterized as multilocular cysts surrounded
by ciliated columnar respiratory type benign metaplastic
epithelium separated by fibrous septa6. Beneath the respiratory
epithelium a continuous or interrupted cell layer
including immature or primitive tumor cells morphologically
varying from small round to fusiform-shaped cells resembles
the cambium layer of botryoid rhabdomyosarcoma6.
Histopathologically, solid areas of type 2 and type 3
tumors show the characteristics of both blastomatosis
and sarcomatosis. In type 2 lesions cysts are observed
macroscopically, however there are some solid areas
including anaplastic cell clusters, cartilage, and nodules of
blastoma. Type 3 lesions seem like type 2 lesions; however
they include predominantly solid areas. Features like nodules
of cartilage with malign appearance, cellular aggregates of
anaplastic and pleomorphic cells, areas like fibrosarcoma,
foci of rhabdomyosarcomatosis, liposarcomatosis, or
chondrosarcomatosis and areas that seem like condensed
blastoma separated with loose fusiform cells can be seen
alone or combined6.
Most of the neoplastic cells were immunoreactive for
vimentin. It is positive in focal areas of rhabdomyoblastic
cell population, cartilage, fibrosarcoma, or histiocytoma
and foci of blastomatosis. Only respiratory epithelial cells
are cytokeratin (CK) positive, smooth muscle actin and
desmin are positive mainly in rhabdomyoblastic cells; on
the other hand they can be positive focally in primitive
cells6,9. S100 expression in nodules of cartilage may be
helpful to distinguish cystic lesions of lung and chest wall
from synovial sarcomas. Moreover, negative CD99, EMA
(epithelial membrane antigen) and CK are also useful in the
differential diagnosis from synovial sarcoma9.
In differential diagnosis there are other tumors that contain
mainly small round cells10. Primitive neuroectodermal
tumors that appear in the thoracic soft tissue show more
cellular areas including rosette formation. The differential
diagnosis of PPB also includes rhabdomyosarcoma,
mestastatic neuroblastoma, extrarenal rhabdoid tumor,
desmoplastic small cell tumor, congenital infantile
fibrosarcoma, metastatic myxoid chondrosarcoma and
other biphasic childhood tumors that may affect the chest8. Rhabdomyosarcomas are treated with a different
protocol, therefore the differential diagnosis is important.
In PPBs of the mediastinum lymphoblastic lymphomas
should be kept in mind in the differential diagnosis.
Pulmonary blastoma, which is important in differential
diagnosis, is a rare congenital lung tumor, but it is usually
seen in adults. Malignant epithelial cells are not seen in
PPB and this morphology is an important key point to
distinguish it from pulmonary blastoma8.
Although present symptoms are similar, the clinical
prognosis depends the histological type and five-year
survival appears better in type 1 patients, while it is worse
in type 3. In type 1, besides a better prognosis, recurrences
can occur locally. Central nerve system is the major area for
metastasis. Spinal cord and skeletal system also carry risk of metastasis. Ocular and pancreatic metastases have also been
reported2. The management of PPBs is a multimodal
approach including chemotherapy, radiotherapy, and
transplantation of autologous hematopoietic stem cells,
however an effective treatment has not been defined yet11-13. Indolfi P et al. reported14 that extrapulmonary
invasion is the parameter of worse prognosis and
neoadjuvant chemotherapy for 9-10 weeks should be
followed by a successful total resection.
In conclusion PPB is a very rare childhood cancer that needs
to be considered in the pathological differential diagnosis of
thoracic tumors in the children. This case is reported also
because of its diagnosis performed by needle biopsy.
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