Pleuropulmonary blastoma report of a case presenting with spontaneous pneumothorax
Yükleniyor...
Dosyalar
Tarih
2001
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
European journal of cardio-thoracic surgery
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
A 21
/2-year-old boy was referred to the Department of
Thoracic Surgery with pneumothorax. He had a history of
dif®culty in breathing, cough and fever for the last 6 months.
Prior to admission, he had undergone antibiotic treatment
for pneumonia, which was diagnosed on chest radiogram.
He was dyspneic and had leukocytosis. Chest radiograms
showed right tension pneumothorax and a cystic mass lesion
in the right lower lobe, the radiologic appearance simulating
an air crescent sign. A chest tube was inserted into the right
hemithorax, resulting in lung expansion. Thorax CT
con®rmed a large solid mass with necrotic areas, ®lling
the right inferoposterior hemithorax. Magnetic resonance
examination revealed a supradiphragmatic, suggestively
extraparenchymal, mass compressing the lower lobe.
Bronchoscopy was not performed since the tumor was
peripherally located. At thoracotomy, a predominantly
solid and partially necrotic mass with extraparenchymal
extension was found to originate from the visceral pleura,
extending posteriorly to the mediastinum and inferiorly to
the diaphragm. The mass involved the diaphragmatic
surface of the posterobasal segment through a narrow
base. Although ®rm adhesions were present, involvement
of the parietal pleura was not observed. The mass was
removed completely with local excision and wedge resection.
Lobectomy was not required. The resected 7 £ 7 £ 3
cm mass was histopathologically evaluated to present with
morphological characteristics of pleuropulmonary blastoma
(PPB). The patient did well in the postoperative period and
was discharged on the 7th postoperative day. Eight courses
of cisplatin (100 mg/m2
) and etoposide (100 mg/m2
) were
administered postoperatively at an oncology center. He is
well and free of recurrence at 10 months following surgery.
PPB is de®ned as a distinctive pulmonary and/or pleural
tumor of childhood with blastomatous and sarcomatous
features without any epithelial component, differentiating
it from the classical adult type pulmonary blastoma [1]. It
is pathologically divided into cystic, solid and mixed types
[2]. PPB is considered within the category of disembryonic
neoplasms such as Wilms' tumor, neuroblastoma and hepatoblastoma
[1]. The etiology and predisposing factors are
unclear. However, PPB arising in the presence of cystic
pulmonary disease have been reported [1]. In the present
case, there was no cystic remnant within the tumor and
radiological cystic appearance was principally due to
tumor necrosis.
The common presenting symptoms in PPB are respiratory
distress, fever, chest pain, cough, anorexia and malaise [3].
PPB may also present with pneumothorax. Pneumothorax
was reported in those cases associated with cystic pulmonary
disease [4,5]. In the present case, tension pneumothorax
might have resulted from spontaneous rupture of the tumor.
The recommended initial treatment for PPB has been
complete surgical excision. However, invasion of the
surrounding structures and extreme friability of the tumor
may prevent complete excision. The prognosis is usually
poor. Gender, pathological subtype, tumor size, extent of
surgical resection, presence of necrosis and adjuvant treatments
such as radiotherapy and chemotherapy have been
reported not to in¯uence survival signi®cantly. The best
indicators of long-term survival are the presence of parenchymal
involvement alone and the absence of pleural or
mediastinal involvement [3].
Açıklama
Anahtar Kelimeler
Kaynak
European journal of cardio-thoracic surgery
WoS Q Değeri
Scopus Q Değeri
Cilt
19
Sayı
Künye
Kuzucu, A. Soysal, Ö. Yakıncı, M. C. Aydın, N. E. (2001). Pleuropulmonary blastoma report of a case presenting with spontaneous pneumothorax. European journal of cardio-thoracic surgery. 19; 229-230.
