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Indian Pediatr 2011;48: 146-147 |
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Pleuropulmonary Blastoma With Cervical Lymph
Node Metastasis at Presentation |
M Choudhury, SK Gupta, M Jain and R Chadha*
From Departments of Pathology and *Pediatric Surgery,
Lady Hardinge Medical College and Kalawati Saran Children’s Hospital, New
Delhi.
Correspondence to: Dr Santosh Kumar Gupta, G-1/329 Ground
Floor, Dal Mill Road, Uttam Nagar (West),
Delhi110 059, India.
Email: [email protected]
Received: April 20, 2009;
Initial review: May 11, 2009;
Accepted: October 1, 2009.
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Pleuropulmonary blastoma is a rare childhood neoplasm accounting for
less than one percent of all primary malignant lung tumors of children
less than six years of age. Metastasis to CNS, orbit and iris, bone,
contralateral lung and rarely adrenal glands, liver, kidney and pancreas
has been described. This report presents a rare case of pleuropulmonary
blastoma with cervical lymph node metastasis at the time of
presentation.
Key words: Child , Metastasis, Pleuropulmonary blastoma,
Tumor.
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Pleuropulmonary
blastoma is a rare, dysembryonic childhood neoplasm first described in
eleven children by Manivel, et al. [1] in 1988. The tumor is a
dysembrayonic neoplasm of thoracopulmonary mesenchyme and arises from the
lung, pleural surface or both [2]. The exact histogenesis is unknown.
Pleuropulmonary blastoma is classified into. type I (cystic), type II
(cystic and solid) and type III (predominantly solid) lesions [3].
Metastasis to cervical lymph node is very rare [4].
Case Report
A 9 year old boy presented to the pediatric OPD with
persistent cough, left sided chest pain and cervical lymphadenopathy.
Imaging and bronchoscopic findings were suggestive of left sided
consolidation with collapse and mediastinal lymphadenopathy. Computerized
tomography of the chest revealed a large heterogenous mass lesion
occupying the whole of left hemithorax with areas of punctuate, coarse
calcification. Lytic areas with sclerosis were seen in D3 vertebral body,
suggesting metastasis.
Section of the cervical lymph node showed effacement of
nodular architecture with a rim of preserved lymphoid tissue at the
periphery. The node was infiltrated by a biphasic tumor comprising of
blastemal and sarcomatous elements. The blastemal cells were present in
nests and cords, had scanty cytoplasm with hyperchromatic and pleomorphic
nuclei, granular chromatin and inconspicuous nucleoli. These cells blended
with the surrounding sarcomatous areas comprising of spindle shaped cells
in fibrous stroma exhibiting fascicular pattern (Solid Type III pattern).
Sections from the lung biopsy obtained on exploratory thoracotomy showed
type II pattern with solid and cystic areas. Histo-pathology of the
resected lung tumour was consistent with type II pattern of
pleuropulmonary blastoma. Immunohistochemistry for vimentin, desmin, and
smooth muscle actin were positive in the interstitial cells present in the
solid areas, further confirming the diagnosis of pleuropulmonary blastoma.
Cytokeratin and Epithelial membrane antigen were strongly positive in the
lining epithelium in the cystic areas.
Discussion
Priest, et al. [4] studied the
clinicopathological correlates in 50 patients with pleuropulmonary
blastoma, The most common presentation was respiratory distress. Other
symptoms included fever, chest or abdominal pain, cough and malaise and
common sites for metastasis were the central nervous system (11 patients),
orbit and iris [5]. Other sites of metastasis included bone, adrenal,
liver, kidney, pancreas and rarely ovary and lymph nodes [4]. Two patients
had recurrences in the contralateral chest. None showed metastasis to the
lymph nodes. Our case presented with cervical lymphadenopathy, which is a
rare presenting feature [4].
The lymph node and lung biopsies showed two different
morphological patterns – Type II and Type III. In the lung biopsy Type II
cystic areas were lined by cuboidal epithelium. However, biopsy from
cervical lymph node showed predominantly type III solid areas
characterized by blastemal cells and sarcomatous areas arranged in a
fascicular pattern. Since the morphology of solid Type III lesion exhibits
blastemal and stromal components [4]; hence this lesion must be
distinguished from adult type pulmonary blastoma which also consists of
epithelial and stromal components. Skeletal muscle and cartilaginous
differentiation have also been described but was not seen in our case.
Transition from Type I lesion to Type III lesion has been previously
described by Wright, et al. [6].
Five year survival for type II and III lesions is
approximately 42% after multimodality therapy. Patients with pleural,
mediastinal and extrapulmo-nary involvement have worse prognosis.
Treatment of pleuropulmonary blastoma is radical surgery followed by
chemotherapy and radiotherapy or both, because response to radiotherapy is
not so good. The patient was treated with VAC chemotherapy and follow up
has so far been uneventful.
Contributors: All authors were involved in
literature search and writing of the report.
Funding: None.
Competing interests: None stated.
References
1. Priest JR, Watterson J, Strong L, Huff V, Woods WG,
Byrd RL, et al. Pleuropulmonary blastoma: a marker for familial
disease. J Pediatr. 1996;128:220-4.
2. Manivel JC, Priest JR,Watterson J, Steiner M,
WoodsWG, Wick MR, et al. Pleuropulmonary blastoma. The so-called
pulmonary blastoma in childhood. Cancer. 1988; 62:1516-26.
3. Dehner LP, Watterson J, Priest J, Pleuropulmonary
blastoma. A unique intrathoracic pulmonary neoplasm of childhood. Perspect
Pediatr Pathol. 1995;18:214-26.
4. Priest JR, McDermott MB, Bhatia S, Watterson J,
Manivel JC, Dehner LP. Pleuropulmonary blastoma: a clinicopathological
study of 50 cases. Cancer. 1997;80:147-61.
5. Lallier M, Bouchard S, Di Lorenzo M, Youssef S,
Blanchard H, LaPierre JG, et al. Pleuropulmonary blastoma; a rare
pathology with an even rarer presentation. J Peditric Surg.
1999;34:1057-9.
6. Wright JR Jr. Pleuropulmonary blastoma: A case
report documenting transition from type I (cystic) to type III (solid).
Cancer. 2000;88:2853-8.
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