Ann Thorac Surg 2008;85:658-660. doi:10.1016/j.athoracsur.2007.08.012
© 2008 The Society of Thoracic Surgeons
Case Reports
Pleuropulmonary Blastoma in the Area of a Diagnosed Congenital Lung Cyst
Lidia Libretti, MDa,*,
Paola Ciriaco, MDa,
Monica Casiraghi, MDa,
Gianluigi Arrigoni, MDb,
Piero Zannini, MDa
a Department of Thoracic Surgery, University and Scientific Institute H San Raffaele, Milan, Italy
b Department of Pathology, University and Scientific Institute H San Raffaele, Milan, Italy
Accepted for publication August 8, 2007.
* Address correspondence to Dr Libretti, Department of Thoracic Surgery, University and Scientific Institute H San Raffaele, via Olgettina 60, Milan, 20132, Italy (Email: libretti.lidia{at}hsr.it).
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Abstract
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Pleuropulmonary blastoma is a rare and aggressive neoplasm typically occurring in young children, even in newborns. Onset of the disease can be identified in the area of a previously diagnosed lung cyst. We report a case of a child previously diagnosed as having a right congenital lung cyst who had a pleuropulmonary blastoma developing in the same area. He underwent surgical resection of the neoplasm followed by chemotherapy. After 20 months he is alive and well.
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Introduction
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Pleuropulmonary blastoma (PPB) is a rare and aggressive embryonic neoplasm that occurs almost exclusively in young children [1]. The tumor can arise in the lung, pleura, or mediastinum. Dehner and colleagues [2] have proposed a classification dividing this lesion into three types: (1) predominately cystic (type I), (2) cystic and solid (type II), and (3) predominately solid (type III). Each type reflects increasing histologic evidence of malignancy [2]. The first appearance of the tumor can be identified in the area of a pre-existing congenital lung cyst [3]. We report the development of PPB in a 22-month-old child in the area of a pulmonary cyst.
A 22-month-old Moroccan child was admitted to our department presenting with a 1-week history of persistent cough and high-grade fever. His medical history reported a diagnosis at age 8 months of a congenital cyst (4.5 cm in size) of the right lung upper lobe. Clinical examination revealed decreased breath sound of the right middle and lower chest. A chest x-ray film showed enlargement of the cyst (Fig 1). A computed tomographic chest scan showed a large multi-loculated cystic lesion of the right upper lobe with a maximum diameter of 10 cm that contained fluid (Fig 2). The middle and lower lobe appeared medially displaced. Fibrobronchoscopy was normal, and then surgical resection was planned. The child underwent a right muscle-sparing posterolateral thoracotomy. The cyst appeared almost extraparenchymal displaying benign features and fluid content. The cyst was removed by means of an upper lobe wedge resection leaving macroscopically free margins. The postoperative period was uneventful and the child was discharged on postoperative day 4. The resected specimen was a multi-loculate cystic formation (10 x 9 x 4 cm with 1 to 1.5 cm thick white walls). A fragment of normal lung was attached to one pole. Immunohistochemical analysis revelead positivity for vimentin and desmins, focally to smooth muscle actin, occasionally to S100. The cells focally contained periodic acid Schiff positive, diastase sensitive, material. The final diagnosis was type II PPB with blastematous and sarcomatous areas (ie, embryonal type rhabdomyosarcoma with focal botryoid component) and tumor-free margins (Figs 3A, B).
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Fig 3. Pleuropulmonary blastoma with blastematous and sarcomatous areas, embryonal type rhabdomyosarcoma. (A) Hematoxylin and eosin; x100. (B) Hematoxylin and eosin; x1,000 oil.
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The child was then referred to the oncologist who prescribed six cycles of chemotherapy with isofosfamide, dactinomycin, and vincristine.
Presently, 20 months after surgery, the child is healthy with no evidence of disease recurrence.
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Comment
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Pleuropulmonary blastoma is a rare, aggressive dysontogenetic neoplasm of childhood [3–5]. Its classification as type I, II, or III seems to have some prognostic value with a different survival rate, even though the better prognosis of type I lesion is reported as not being statistically significant [4]. It has also been speculated that the natural history of PPB is a progression from type I to type III with time [5].
Symptoms can be nonspecific, such as dry cough, fever, and chest or abdominal pain, or with pulmonary infections that persist despite medical therapy. Uncommon symptoms are dyspnea and pneumothorax [2–4]. Differential diagnosis between hydatid or bronchogenic cyst, pulmonary abscess, and cystic adenoma is mandatory [4]. The prognosis of PPB is extremely poor; local and regional spread at presentation of the disease usually precludes adequate resection [3, 6]. At the time of surgical resection, two-thirds of the lesions are type II or type III, whereas type I seems to be the least common of the three types of PPB. Types II and III lesions have a tendency to recur, even at remote or contralateral sites, despite presumed complete primary resection [4].
In most cases pulmonary cysts were identified radiologically prior to surgery and were often presumed to be developmental of infections; the children were simply followed until a more suspicious solid component developed [4]. In our case the child had been conservatively treated when the congenital bronchogenic cyst was diagnosed. The onset of the symptoms of infection and fluid collection led us to schedule surgery and resection of the cyst. Indications for surgery, for Priest and colleagues [4], were the presence of pulmonary cysts, pneumothorax, a presumed inflammatory-infectious process, and the presence of intrathoracic mass lesions.
Various hypotheses have been formulated on the association of PPB with pre-existing cystic disease of the lung. Priest and colleagues [4] reported that PPB and congenital lung cysts may be associated in 38% of cases, which is why they recommend an early surgical approach when a lung cyst is discovered. Other reports suggest that lung cysts may be markers of embryonal instability, but their prophylactic surgical resection does not protect patients from having PPB develop later [3]. However, PPB has recently been identified in utero, indicating that these lesions may be congenital in origin and thus malignant from the beginning [7].
The surgical procedure of choice for PPB is lobectomy, as the border between the lesion and normal parenchyma may be difficult to determine grossly, and usually the tumor spreads into lung parenchyma [4, 8]. In our case, a formal lobectomy was not necessary because the lesion was located outside the lobe and was joined to it only by a pedicle.
Postoperative treatment of PPB varies according to the type of disease [4]. Although the necessity for chemotherapy has not been clearly established on a statistically significant level, it should be considered for all types of PPB because of the aggressiveness of types II and III and the possibility that type I may recur with the pathologic features of types II or III [5, 6].
In our case, the oncologist believed that postoperative chemotherapy could offer a better chance of preventing distant metastases. It was well tolerated by the child, who is still alive, well, and has no evidence of disease after 20 months. Authors agree that patients with PPB, especially types II and III, should be treated as aggressively as is tolerable, although the most active chemotherapeutic agent or combination of agents has not been identified yet. However, a multi-modal approach, including radiotherapy in types II and III, is advocated [4].
In conclusion, congenital cystic lesions should be followed-up and never underestimated because they may conceal a PPB [3, 4]. Early definitive surgery in PPB may have a positive impact on survival, although diagnosis is usually histologic [4]. After resection, postoperative chemotherapy is strongly recommended to prevent distant spread of the disease or transition from PPB type I to PPB type III [5, 6].
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References
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- Manivel JC, Priest JR, Watterson J, et al. Pleuropulmonary blastoma: the so called pulmonary blastoma of childhood Cancer 1988;62:1516-1526.[Medline]
- Dehner LP, Watterson J, Priest J. Pleuropulmonary blastoma: a unique intrathoracic pulmonary neoplasm of childhood Perspect Pediatr Pathol 1995;18:214-216.
- Papagiannopoulos KA, Sheppard M, Bush AP, Goldstraw P. Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective? Ann Thorac Surg 2001;72:604-605.[Abstract/Free Full Text]
- Priest JR, McDermott MB, Bathia S, et al. Pleuropulmonary blastoma: a clinicopathological study of 50 cases Cancer 1997;80:147-161.[Medline]
- Wright JR. Pleuropulmonary blastoma: a case report documenting transition from type I (cystic) to type III (solid) Cancer 2000;88:2853-2858.[Medline]
- Tagge EP, Mulvihil D, Chandler JC, Richardson M, Uflaker R, Othersen Jr HB. Childhood pleuropulmonary blastoma: caution against nonoperative management of congenital lung cysts J Pediatr Surg 1996;31:187-189.[Medline]
- Miniati DN, Chintagumpala M, Langston C, et al. Prenatal diagnosis and outcome of children with pleuropulmonary blastoma J Pediatr Surg 2006;41:66-71.[Medline]
- Laberge JM, Puligandla P, Flageole H. Asymptomatic congenital lung malformations Semin Pediatr Surg 2005;14:16-33.[Medline]
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Abstract
Introduction
