Contents lists available at ScienceDirect

J Ped Surg Case Reports 2 (2014) 360e362

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Pleuropulmonary blastoma in extrapulmonary lung tissue: A casereportq

Mio Tanaka a,*, Rieko Ijiri a, Mariko Yoshida a, Misa Yoshida a, Hiroshi Take b,Kumiko Nozawa c, Noriko Aida c, Hiroaki Goto d, Yukichi Tanaka a

aDepartment of Pathology, Kanagawa Children’s Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama 232-8555, JapanbDepartment of Surgery, Kanagawa Children’s Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama 232-8555, JapancDepartment of Radiology, Kanagawa Children’s Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama 232-8555, JapandDepartment of Hematology and Oncology, Kanagawa Children’s Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama 232-8555, Japan

a r t i c l e i n f o

Article history:Received 27 February 2014Received in revised form15 July 2014Accepted 15 July 2014

Key words:Extralobar pulmonary sequestrationPleuropulmonary blastoma

q This is an open access article under the CCcreativecommons.org/licenses/by-nc-nd/3.0/).* Corresponding author. Tel.: þ81 45 711 2351.

E-mail address: mio@zc4.so-net.ne.jp (M. Tanaka).

2213-5766/$ e see front matter � 2014 The Authors.http://dx.doi.org/10.1016/j.epsc.2014.07.007

a b s t r a c t

We report a case of pleuropulmonary blastoma (PPB) occurring in a very unusual location, extrap-ulmonary lung tissue, as possible extralobar pulmonary sequestration. The patient was a girl aged twoyears and six months with respiratory symptoms. Infection of a congenital pulmonary malformation wassuspected based on computed tomography findings. The operative findings revealed a partly adherentmass located between the upper and middle right lobes, and a feeding vessel. Histological analysisshowed the mass to be PPB. A small portion of normal-looking pulmonary tissues was confirmed in thesubcapsular area of the mass, suggesting that the tumor originated in extrapulmonary lung tissue. Thepatient underwent postoperative chemotherapy. No recurrence or metastasis of the PPB has occurredduring the 10-year follow-up. When considering the treatment options for an extralobar pulmonarylesion, the possibility of PPB should be considered, although its occurrence may be extremely rare.

� 2014 The Authors. Published by Elsevier Inc. All rights reserved.

Pleuropulmonary blastoma (PPB) is a rare intrathoracic tumor ofearly childhood. It has often been described as associated withcystic lung diseases, such as congenital adenomatoid malformation(CCAM) [1e3]. However, its association with extralobar lung tissuehas not been reported in the literature. Here, we report a girl withrepeated respiratory infections, whose initial diagnosis was sus-pected CCAM. However, a histological analysis showed her to havePPB in extralobar lung tissue, possibly within an extrapulmonarysequestration (EPS).

1. Case report

A girl aged twoyears and sixmonths presented at hospital with acough and fever. A chest radiogram showed right pulmonaryconsolidation. The CT findings were suggestive of infection of aCCAM and the patient was referred to our institute for furtherevaluation and treatment. She was febrile at presentation, withreduced respiratory sounds in the right thorax andan elevatedwhite

BY-NC-ND license (http://

Published by Elsevier Inc. All right

blood cell count. Her medical and family histories were unremark-able. She was treated with antibiotics. A follow-up CT obtained amonth later, after her general condition had improved, showed awell-defined, large cystic mass with a solid component in the rightthorax. A right thoracotomy was performed. The right lung showedincomplete lobulation between the upper/middle andmiddle/lowerlobes. The mass was located between the upper and middle lobesand appeared to be covered by its own pleura. It was clearly sepa-rated from the pulmonary lobes, although therewas slight adhesionto the upper lobe. There was no connection to the trachea orgastrointestinal tract. A feeding vessel was confirmed and resected,and the mass was completely removed. The mass measured80� 60� 60mmandweighed 155 g. The cut section revealed cysticand solid lesions with hemorrhage (Fig. 1). The solid lesions werefragile and nodular in appearance. Microscopically, the tumor wascompletely covered by a fibrocollagenous capsule and showeddiffuse growth of neoplastic cells, with normal-looking ciliatedepithelial linings in some areas (Fig. 2A). Some neoplastic cellsshowed differentiation toward striated muscle. Neoplastic cartilageandhemorrhagic necrosiswere alsonoted. Immunohistochemically,theneoplastic cellswerepositive for vimentin, desmin,muscle actin,focally positive for myoglobin, and negative for cytokeratin. Theepithelial linings were positive for cytokeratin and thyroid tran-scription factor 1. These findings are consistent with PPB.

s reserved.

Fig. 1. Macroscopic investigation of the tumor. The cut section of the tumor revealscystic and solid lesions. The solid lesions were fragile and nodular in appearance.

Fig. 3. Preoperative computed tomography image. A well-defined, large cystic masswith a solid component is seen in the right thorax. A feeding artery from the intercostalartery is indicated (white arrow).

M. Tanaka et al. / J Ped Surg Case Reports 2 (2014) 360e362 361

Small amounts of normal-looking pulmonary tissues were notedbetween the fibrocollagenous capsule and the tumor (Fig. 2B),suggesting that the tumor arose from extrapulmonary lung tissue.Careful review of the preoperative CT revealed what appeared to bean intercostal artery as the feeding artery (Fig. 3); however,

Fig. 2. Microscopic findings of the tumor. A: Diffuse growth of neoplastic cells, withround to oval or spindle-shaped nuclei and rough chromatin clumping, partly coveredby normal-looking ciliated epithelial lining. B: At the surgical margin, a small amountof normal-looking pulmonary tissue is noted between the visceral pleura and thetumor.

histological examination proved this to be a muscular-type artery.The postoperative CT showed no defect or distortion of the rightlung, and there was no disruption of the distal bronchial tree. Thefinal diagnosis was PPB arising in extralobar pulmonary tissue. Thepatient was discharged after completing postoperative chemo-therapy. There has been no recurrence or metastasis of the PPBduring 10 years of follow-up.

2. Discussion

Pulmonary sequestration is an uncommon congenital anomaly,characterized byananomalous systemicblood supplyanddysplasticlung tissues that have no connection to the tracheobronchial tree. Itis classified into intralobar and extralobar types, the former con-tained within the normal lung and the latter separated from thenormal pulmonary lobe, outside the visceral pleura. EPS is lesscommon than its intralobar counterpart, and is more often associ-ated with congenital anomalies such as congenital diaphragmatichernia, lung and chest wall deformities, foregut malformations,bronchogenic cysts, and complex cardiac anomalies [4e6].

Interpreting the origin of PPB in our patient was very difficultbecause the artery confirmed during surgery was a muscular typeand may have been a parasitized vessel. However, the resectedtumor was an independent mass existing outside the normal pul-monary lobes, with no connection to the gastrointestinal tract. Itwas covered by fibrocollagenous tissue, separate from the visceralpleura that outlined the normal lung lobes. There was no pedicle-like feature that would have suggested a tumor protruding from anormal pulmonary lobe. Moreover, the postoperative CT scanshowed no defect or distortion of the right lung tissue, and therewas no disruption of the distal bronchial tree. Based on these data,the tumor was considered to have developed from extralobar pul-monary tissue, possibly sequestered lung. Because the growth ofthe tumor was expansive, the elastic-type feeding artery may havebecome indistinguishable, although there was no way to prove this.

The management of pulmonary sequestration is controversial,especially in EPS. Patients with intralobar pulmonary sequestration(IPS) often develop respiratory symptoms, most commonly respi-ratory-tract infections and recurrent pneumonia. There have beenvery few reports of patients with IPS who remained asymptomaticthroughout life [7,8]. Therefore, many authors recommend resec-tion, regardless of the presence or absence of symptoms [8e10]. Incontrast, EPS is often asymptomatic and is less likely to becomeinfected later in life. Some authors advocate observation forasymptomatic cases or recommend intervention only after

M. Tanaka et al. / J Ped Surg Case Reports 2 (2014) 360e362362

symptoms appear [11e13]. Even though the surgical results aresatisfactory and the complication rates are generally low, pneumo-thorax, hemothorax, and emphysema are recognized problems.Moreover, the possibility of infection, destruction of the normalpulmonary parenchyma, and malignant transformation have beenreported by other groups [4,10,14].

Pulmonary sequestration has previously been reported mostcommonly in association with CCAM/CPAM type 2, and there hasbeen a misconception that PPB is a preexisting CCAM/CPAM thathas undergone malignant transformation [15,16]. Approximately50% of EPSs contain multiple small cysts, measuring up to 2.0 cm,similar to CPAM type 2, and these are generally termed “hybridlesions.” However, recent studies by the International Pleuro-pulmonary Blastoma Registry have indicated that CPAM type 2 doesnot undergo malignant transformation. In fact, these cystic lesionsare assumed to be secondarily developed lesions associated with inutero airway obstruction and/or atresia, a pathological entity quitedistinct from CPAM type 2 [15,17]. Although the risk of malignanttransformation of the so-called “hybrid lesions” has been rejected,the occurrence of PPB associated with extralobar lung tissue, likelycommonly EPS, as in our patient, cannot be ignored.

The development of minimally invasive surgery has increasedguardians’ willingness to consent to surgical resection in thesechildren because scarring is minimized, there is less pain, and thehospital stay is shorter than for thoracotomy [15]. Transcatheterarterial embolization (TAE) has recently beenperformed on selectedpatients with pulmonary sequestration. Some TAE studies haveshowngood results,with complete regressionof the lesion, althoughthe long-term efficacy and safety of the treatment have not yet beenestablished [18]. However, guardians are often reluctant to consentto surgery, especially when the patient is asymptomatic. In thesecases, TAE may be a treatment option, although clinicians shouldnote that no tissue is available for pathological examination afterTAE. When considering the treatment options for these lesions,although they are extremely rare, the possibility of PPB associatedwith extralobar lung tissue (possibly EPS) should be considered.

Conflict of interest and sources of funding statementThe authors claim that they have no conflicts of interest or

financial conflicts.

References

[1] Indolfi P, Casale F, Carli M, Bisogno G, Ninfo V, Cecchetto G, et al. Pleuro-pulmonary blastoma: management and prognosis of 11 cases. Cancer 2000;89:1396e401.

[2] Federici S, Domenichelli V, Tani G, Sciutti R, Burnelli R, Zanetti G, et al. Pleu-ropulmonary blastoma in congenital cystic adenomatoid malformation: reportof a case. Eur J Pediatr Surg 2001;11:196e9.

[3] Cohen M, Emms M, Kaschula RO. Childhood pulmonary blastoma: a pleuro-pulmonary variant of the adult-type pulmonary blastoma. Pediatr Pathol1991;11:737e49.

[4] Yucel O, Gurkok S, Gozubuyuk A, Caylak H, Sapmaz E, Kavakli K, et al. Diag-nosis and surgical treatment of pulmonary sequestration. Thorac CardiovascSurg 2008;56:154e7.

[5] Louie HW, Martin SM, Mulder DG. Pulmonary sequestration: 17-year experi-ence at UCLA. Am Surg 1993;59:801e5.

[6] DeParedes CG, Pierce WS, Johnson DG, Waldhausen JA. Pulmonary seques-tration in infants and children: a 20-year experience and review of the liter-ature. J Pediatr Surg 1970;5:136e47.

[7] Skrabski R, Royo Y, Di Crosta I, Pueyo C, Sempere T, Maldonado J. Extralobarpulmonary sequestration with an unusual venous drainage to the portal vein:preoperative diagnosis and excision by video-assisted thoracoscopy. J PediatrSurg 2012;47:e63e65.

[8] Laberge JM, Bratu I, Flageole H. The management of asymptomatic congenitallung malformations. Paediatr Respir Rev 2004;5(Suppl. A):S305e12.

[9] Sfakianaki AK, Copel JA. Congenital cystic lesions of the lung: congenital cysticadenomatoid malformation and bronchopulmonary sequestration. Rev ObstetGynecol 2012;5:85e93.

[10] Cho MJ, Kim DY, Kim SC, Kim KS, Kim EA, Lee BS. Embolization versus surgicalresection of pulmonary sequestration: clinical experiences with a thoraco-scopic approach. J Pediatr Surg 2012;47:2228e33.

[11] Stanton M, Njere I, Ade-Ajayi N, Patel S, Davenport M. Systematic review andmeta-analysis of the postnatal management of congenital cystic lung lesions.J Pediatr Surg 2009;44:1027e33.

[12] Nijagal A, Jelin E, Feldstein VA, Courtier J, Urisman A, Jones KD, et al. Thediagnosis and management of intradiaphragmatic extralobar pulmonary se-questrations: a report of 4 cases. J Pediatr Surg 2012;47:1501e5.

[13] Adzick NS, Farmer DL. Cysts of the lungs and mediastinum. Philadelphia:Elsevier Saunders; 2012.

[14] Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM. Themultiple facets of pulmonary sequestration. J Pediatr Surg 2001;36:784e90.

[15] Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A. Pulmonary cysts in earlychildhood and the risk of malignancy. Pediatr Pulmonol 2009;44:14e30.

[16] Dosios T, Stinios J, Nicolaides P, Spyrakos S, Androulakakis E,Constantopoulos A. Pleuropulmonary blastoma in childhood. A malignantdegeneration of pulmonary cysts. Pediatr Surg Int 2004;20:863e5.

[17] Conran RM, Stocker JT. Extralobar sequestration with frequently associatedcongenital cystic adenomatoid malformation, type 2: report of 50 cases.Pediatr Dev Pathol 1999;2:454e63.

[18] Curros F, Chigot V, Emond S, Sayegh N, Revillon Y, Scheinman P, et al. Role ofembolisation in the treatment of bronchopulmonary sequestration. PediatrRadiol 2000;30:769e73.