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Home , Adipose tissue neoplasm, Histiocytoma, Mesothelioma, Pleuropulmonary blastoma

Int J Clin Exp Pathol 2015;8(10):13571-13577 www.ijcep.com /ISSN:1936-2625/IJCEP0014587

Case Report Infant pleuropulmonary : report of a rare case and review of literature

Hong Zhang1, Chun-Wei Xu2, Jian-Guo Wei3, Guang-Jun Zhu4, Sheng Xu2, Jin Wang2

Departments of 1Out-Patient, 2Pathology, The General Hospital of Beijing Military Region, Beijing 100700, People’s Republic of China; 3Department of Pathology, The People’s Hospital of Shaoxing Zhejiang, 312000, People’s Republic of China; 4Department of Central Laboratory, 281 Hospital of PLA, Qinhuangdao 066105, Hebei, People’s Republic of China Received August 16, 2015; Accepted September 23, 2015; Epub October 1, 2015; Published October 15, 2015

Abstract: In infants, is a rare but aggressive tumor. The typical histopathological pre- sentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid , fetal , synovial , and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous containing well-differentiated cartilage islands or cartilaginous nodes. was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clini- cal data should help in determining the diagnosis of pleural pulmonary blastoma.

Keywords: Pleuropulmonary blastoma, pathological diagnosis, differential diagnosis, immunohistochemistry

Background clinical pathology and immunohistochemistry features will be described. A special focus has Pleuropulmonary blastoma (PPB) is a rare, been placed on the differential diagnosis of aggressive tumor that is most common in PPB. infants with a familial predisposition [1]. Although Manivel et al. first proposed PPB in Case presentation 1988 [2], it was not until 1989 that PPB was considered as an independent entity in clinical The patient was a 4-month-old male infant, who pathology. The World Health Organization mainly presented with a cough and dyspnea (WHO) classified PPB as a tumor of and occasional choking while drinking milk. the lung that is distinct from that of other pul- During crying episodes, he experienced cyano- monary blastomas. Consisting of types I, II, and sis for 3 days and was hospitalized for pneumo- III, PPB presents with unique clinical and histo- nia. On February 21, 2011, a pathological manifestations. We will report on showed intact ribs and thorax. There were den- one infant with PPB who was treated in our hos- sities in the right upper lung field with unobvi- pital. Combined with a literature review, the ous mediastinal and tracheal displacement. Infant pleuropulmonary blastoma: a case study

Figure 1. A: Chest radiograph: intact thorax and ribs, with densities in the right upper lung field and unobvious me- diastinal and tracheal displacement. The majority of the left lung field was obscured because it was blocked by the cardiac shadow; the , hilar shadow, and cardiac border were obscured. Moreover, the diaphragmatic surface and bilateral costophrenic angles were obscured. B: Chest CT scan: an irregular soft tissue mass is shown in the right upper lung that was sized 6.0 × 6.0 × 4.0 cm. The CT value was approximately 18-40 Hu and had a non-uniform density. There were cystic-solid lesions. An aerial bronchogram could be locally observed; the inner and outer lateral borders were connected to the mediastinum, cardiac border, and chest wall; and the mediastinum and cardiac shadow were considerably displaced to the left. C: A postoperative chest radiograph demonstrated the following: intact thorax and ribs; increased but obscured striates in bilateral lung fields; no mediastinal widening; thickened right hilar tumor shadow; no right hilar enlargement; cardiac shadow with a normal size and normal mor- phology; smooth diaphragmatic surface; sharpened bilateral costophrenic angles; a shadow cast by cannulation in the superior vena cava; and a drainage tube shadow in the right thorax.

The majority of the left lung field was obscured possibility of pulmonary blastoma could not be because it was blocked by the cardiac shadow. excluded. A right was per- The mediastinum, hilar shadow, and cardiac formed (Figure 1B). On March 2, 2011, a post- border were obscured. Moreover, the diaphrag- operative chest radiograph was performed that matic surface and bilateral costophrenic angles demonstrated the following: an intact thorax were obscured, but not significantly Figure ( and ribs, with increased but obscured striates 1A). On February 23, 2011, a chest computed in bilateral lung fields; no mediastinal widening; tomography (CT) scan showed an irregular soft a thickened, right hilar tumor shadow, with no tissue mass in the right upper lung that was right hilar enlargement; a cardiac shadow of sized 6.0 × 6.0 × 4.0 cm, with a CT value of normal size and morphology; a smooth dia- approximately 18-40 Hu and a non-uniform phragmatic surface; sharpened bilateral costo- density. Cystic-solid lesions were present. An phrenic angles; a shadow cast by cannulation aerial bronchogram could be locally observed; in the superior vena cava; and a drainage tube the inner and outer lateral borders were con- shadow in the right thorax (Figure 1C). nected to the mediastinum, cardiac border, and chest wall; the mediastinum and cardiac shad- ow were considerably displaced to the left. The diagnosis of pulmonary blastoma was made The tumor mass in the right lung was sized 9.0 preoperatively. During surgery, it was found × 6.0 × 4.0 cm, with a solid, soft-textured gray- that the mass was located in the right upper ish-white cross section. The mass was clearly lobe and almost occupied the right thoracic distinguishable from the lung, and a large part cavity. The envelope was intact, and a cross of the lung was occupied by the tumor. The section of the tumor was fish-like. The right specimen was fixed with 4% neutral formalde- middle and lower lobes were already com- hyde and conventionally made into paraffin pressed by the tumor. The tumor mass was sections. HE staining was performed. The cells resected, and a small portion of it was immedi- were detected using EnVison immunohisto- ately frozen for pathological examination. The chemistry followed by DAB color development. results showed that the tumor was composed The primary antibodies used for staining were of atypical spindle-shaped cells. An adenoid Ki-67, AE1/AE3, EMA, CEA, TTF-1, vimentin, structure was sporadically observed, and the S100, NSE, bcl-2, CD99, Fli-1, CD34, SMA, des-

13572 Int J Clin Exp Pathol 2015;8(10):13571-13577 Infant pleuropulmonary blastoma: a case study

Figure 2. A: The tumor mass demonstrated an aggregation of primitive cells with a round or orbicular-ovate shape and with residual mature . B: (medium magnification of a) Cells were medium sized and had little cyto- plasm, and the nuclei were round or orbicular-ovate and deeply stained. The cells were highly malignant. C: (high magnification of a) The cytoplasm contained vacuoles; the nuclei were deeply stained; and the nucleolus was clearly visible. Karyokinesis was active. D: A -like lesion was interwoven with spindle-shaped cells and short spindle-shaped cells. E: A malignant fibrous histiocytoma contained well-differentiated cartilage. F: A junction be- tween the tumor and lung tissue. The right comprises malignant tumor cells, while the left comprises normal lung tissue.

Figure 3. A: Positive staining for vimentin; B: Positive for residual bronchial epithelia with TTF-1 staining. min, MyoD-1, myoglobin, calponin, MC, and cal- cytoplasm was eosinophilic and contained vac- retinin. All antibodies and EnVison kits were uoles; the nuclei were round or orbicular-ovate purchased from Beijing Zhong Shan Golden and deeply stained. The nucleolus was clearly Bridge Biological Technology Co, Ltd. The tumor visualized, and karyokinesis was active; the was mostly of a solid texture and locally nodu- tumor mass had an aggregation of cells (Figure lar. Medium-sized primitive fetal cells of a round 2A-C). Some portion of the tumor was biphasic: or orbicular-ovate shape were observed. The fasciculated sarcoma (composed of spindle-

13573 Int J Clin Exp Pathol 2015;8(10):13571-13577 Infant pleuropulmonary blastoma: a case study shaped cells and short spindle-shaped cells) mass was lined with a multi-cystic structure of and malignant fibrous histiocytoma. The cells mature respiratory epithelial cells. There were varied in density (Figure 2D), and well-differen- primitive, malignant small cells aggregating tiated cartilage islands or cartilaginous nodes under the . These cells were small (Figure 2E) were observed. In some regions, and round and in a dense arrangement with a sunken or residual mature bronchioles or an single-focal, multi-focal, or diffuse distribution. alveolar epithelium was found. At the junction Rhabdomyoblastic cells of varying differentia- between the extensive solid tumor and lung tis- tion were also observed. The tumors mainly sues, a nodular structure was seen locally presented with discontinuous septa or an (Figure 2F). Immunohistochemistry revealed island-like structure, with or without cartilagi- the following: vimentin (+) (Figure 3A), NSE nous nodes [9]. Type II PPB had a solid focal (focal +), SMA (mesenchymal cells, focal +), region based on type I PPB. The septal mesen- CD99 (weak +), Bcl-2 (weak +), desmin (-), myo- chyma overgrew locally or completely. The globin (-), calretinin (-), calponin (-), FLI (-), MyoD- mass was composed of sheets of insignificantly 1 (-), and CD34 (-); in the residual epithelial differentiated primitive malignant small cells, components, CK (AE1/AE3), EM/A and TTF-1 (+) embryonal , and bundle- (Figure 3B), CEA (-), S-100 (cartilage, mesen- like fasciculated sarcoma formed by patches or chymal cells, focal +), and Ki-67 index (tumor nodules. The solid region of the type III PPB cells accounting for about 45%; residual epithe- exhibited mixed features including blastoma lial cells-). A pathologic diagnosis of type III and sarcoma, with round or spindle-shaped (solid) pleuropulmonary blastoma was made. cells. Histologically, the mass consisted of car- tilage islands, cartilaginous nodes, rhabdomyo- Discussion ma, and anaplastic cells that were observed either separately or in a mixed condition. PPB usually occurs in infants and exhibits dif- Bleeding, necrosis, and fibrosis of various ferent clinical and epidemiological features extents were also occurred [10]. Because of from that of other pulmonary blastomas. About progression or incomplete resection of PPB, 93% of PPB patients are under 6 years of age type I PPB may develop into type II or III. With an [3, 4], and adult cases are rare [5, 6]. PPB does early stage tumor, there is a transition between not demonstrate an obvious gender difference. normal lung tissue and the tumor. The alveolar There are three PPB types, namely, type I (mul- mesenchymal cells overproliferate, causing the ticystic), type II (multicystic tumor complicated alveolar septa to expand and thicken and to by solid nodules), and type III (solid). The aver- progress to type II. This point could be corrobo- age age of onset for type I, type II, and type III is rated by the observation of residual cystic 10 months, 36 months, and 44 months, lesions in type II PPB [9]. The proliferating mes- respectively [7]. Presenting with non-specific enchymal cells form solid nodes to replace the clinical manifestations, all three types are cystic region, and the tumor progresses to type mainly associated with a prolonged cough, III PPB. One salient feature of this development , difficulty breathing, and that is the change in quantity of mesenchymal cells are complicated by and atelec- that only account for 6% in type I PPB. However, tasis [8]. the proportion could be as high as 76.5% and 90.3% in type II and type III PPB, respectively. In The gross specimen of the type I PPB in our our case, the solid region comprised the largest study had a multicystic structure and thin cystic proportion, showing nodular edges, residual wall. The air-filled could be easily distin- bronchioles, and locally expanded alveoli. guished from the surrounding tissues, and solid nodules or patches were not seen with the Currently, there are no specific molecular mark- naked eye. The type II PPB specimen had a cys- ers that could be used to diagnose PPB, espe- tic-solid structure and thick cystic wall, com- cially if the marker expressions diverge in primi- bined with cystic type I PPB and solid type III tive small cells. The tumor cells could express PPB features. The solid nodules had grown into vimentin, resulting in the corresponding immu- the cystic cavity in this case. The type III PPB nophenotype with the differentiation of primi- mass was solid, with a soft, fish-like cross sec- tive mesenchymal cells. The commonly used tion, and contained bleeding or necrotic muscle cell markers include desmin, Myo-D1, regions. Under a microscope, the type I PPB MSA, and SMA. The residual epithelium

13574 Int J Clin Exp Pathol 2015;8(10):13571-13577 Infant pleuropulmonary blastoma: a case study expresses CK, EMA, TTF-1, and other proteins. vation would reveal the presence of a simple The results of the immunohistochemical stain- cystic structure, which creates the need for dif- ing showed that vimentin (spindle-shaped cells ferentiation with a histopathological examina- +) and muscle cell markers were not significant- tion. Type I PPB has to be pathologically differ- ly expressed in our case. This suggested that entiated from congenital pulmonary airway the primitive mesenchymal cells did not under- malformation (CPAM) in some cases. CPAM4 go intensive myogenic differentiation. The and type I PPB share common histological fea- expressions of CK (AE1/AE3) and EMA were tures, and the differentiation of clinical and only confined to the epithelium. The lack of imaging manifestations is difficult. The former expression of CEA and Ki-67 also indicated the is associated with a distal airway malformation existence of non-tumor respiratory epithelial and the presence of large cysts. Squamous epi- cells growing into the tumor. thelium or cubic epithelium overlaid the cystic wall, but no primitive malignant small cells were PPB may present as unilateral or bilateral local seen. In contrast, type I PPB contains primitive air-filled cysts upon an imaging examination. malignant small cells, which may be accompa- This could occur following difficulty breathing nied by fibroblast-like or adipose-like differenti- [11]. Alternately, PPB is found as a solid, mixed- ation; the tumor cell nuclei usually show consid- density space-occupying lesion, or an irregular erable atypia [9]. The immunohistochemical soft tissue mass with an obscure boundary and staining was positive for p53. Types II and III a non-uniform density. The septa are widened, PPB need to be differentiated from the follow- and the cystic mass is visible. These imaging ing tumors: (1) sarcomatoid mesothelioma. findings indicate that PPB is not a non-congeni- Sarcomatoid mesothelioma usually contains tal adenomatoid malformation or congenital no primitive malignant small cells. Immu- emphysema. However, Mut et al. [12] reviewed nohistochemical staining is positive for actin three PPB cases and concluded that PPB pre- and desmin. The diagnosis of sarcomatoid sented with diversified imaging findings. Some mesothelioma could be made if both the anti- PPB may originate from a congenital pulmonary calretinin antibody and mesothelial cell anti- . body were positive. Furthermore, the age of onset for sarcomatoid mesothelioma is much In PPB, dyskaryosis is the amplification of chro- older. (2) Typical pulmonary blastoma: if PPB is mosome 8, and a p53 is implicated associated with sunken epithelial components, [13]. One recent research study showed that all it may be mistakenly diagnosed as typical pul- included PPB cases with a familial predisposi- monary blastoma even though the epithelial tion carried a mutated DICER1 gene. In all cell components are non-cancerous [15]. However, types, one normal DICER1 gene and one abnor- typical pulmonary blastoma is more common in mal DICER1 gene existed. It was suggested adults, and the microscopic manifestations that PPB might derive from a mutation in include well-differentiated tubular glands and DICER1 in one or several lung cells that is detri- abundant mesenchymal components in cells. mental to cell function. Another research study For either epithelial pulmonary blastoma or showed that the occurrence of PPB was caused bidirectional pulmonary blastoma, the epitheli- by an unknown mechanism for tumor induction. al components are cancerous. The epithelium An abnormal DICER1 gene would downregulate is primitive and malignant, as in fetal adenocar- signaling of the surrounding cells, inducing the cinoma, which makes it easily distinguishable transformation of normal cells to tumor cells. from the small quantity of normal respiratory However, the cell carrying the abnormal DICER1 epithelium in PPB. In addition, typical pulmo- gene would not become a tumor cell. nary blastoma contains no primitive malignant Diagnosis or a relevant differential diagnosis of small cells. (3) Embryonal rhabdomyosarcoma: PPB is usually made based on a pathological type II and III PPB may present with the differ- examination (microscopically or with the naked entiation of rhabdomyoblastic cells and could eye) combined with clinical data and imaging be confused with embryonal rhabdomyosarco- findings (chest radiograph). However, a patho- ma. However, they differ in vulnerable organs: logical examination is the only diagnostic tool embryonal rhabdomyosarcoma is more com- that could be used to confirm the case. For type mon in the and vagina because II PPB, both imaging findings and visual obser- they have a lumen, while PPB occurs mostly in

13575 Int J Clin Exp Pathol 2015;8(10):13571-13577 Infant pleuropulmonary blastoma: a case study the lung and rarely in the chest wall. Moreover, receiving or not receiving ; the embryonal rhabdomyosarcoma is associated two-year survival was 64% and 65%, respec- with unidirectional myogenic differentiation tively [18]. Like other tumors, PPB is also asso- and is positive for myogenic markers [1]. (4) ciated with metastatic loci: over 25% of type II : this tumor could be easily and type III PPBs have metastatic loci. distinguished by age of onset and position of Moreover, PPB is more likely to result in metas- occurrence. Synovial sarcoma is most common tasis to the central nervous system, as com- in young people in deep soft tissues near the pared with other pediatric . Therefore, large joints in the four limbs. The spindle- the brain is the organ most vulnerable to shaped cells are morphologically different from . Other parts of the body that could those in PPB. In the former, the spindle-shaped be affected are the bones, lymph nodes, , cells are arranged near the vessels; in the lat- , , and [7, 19]. Type I ter, the cells are loosely arranged. Synovial sar- PPB is associated with a better prognosis than coma features bidirectional differentiation, but type II and type III PPB [20]. Rubinas et al. [19] with no differentiation of the primitive mesen- believed that the long-term survival of type I chymal cells and rhabdomyoblastic cells. PPB was 83%, as opposed to only 42% for types Immunohistochemical staining may be simulta- II and III PPB [20]. neously positive for CK, EMA, and vimentin. (5) Primitive neuroectodermal tumor (PNET): the Conclusion prominent feature of PNET is the presence of Homer-Wright rosettes, ependymal tubules, PPB is a rare but highly aggressive neoplasm in and Flexner-Wintersteiner rosettes. Immu- children. Its typical histopathological presenta- nohistochemical staining indicates differentia- tion is the aggregation of primitive malignant tion into neural cells. There are other non-can- small cells. Combining imaging and histopatho- cerous lung lesions, from which PPB needs to logical examinations and clinical data should be differentiated by analyzing both the clinical help in determining the diagnosis of PPB. manifestations and pathological examinations. Acknowledgements Surgery is the first choice for PPB treatment and is usually combined with chemotherapy Here we express our thanks to Professor Guang and radiotherapy, but the postoperative relapse Liu (Department of Pathology, the General rate is high. Autologous stem cell transplanta- Hospital of Beijing Military Region, Beijing, tion following large-dose chemotherapy is cur- China.) for his help in correcting this manu- rently considered for clinical treatment [15]. In script. Written informed consent was obtained type II PPB that does not have any extrapulmo- from the patient for publication of this Case nary involvement, simple tumor resection is Report and accompanying images. sufficient; however, for type II and III PPB, tumor Disclosure of conflict of interest resection should be combined with chemother- apy and radiotherapy [16]. The administration None. of chemotherapy is to help relieve symptoms or for preoperative preparation and prevention of Address correspondence to: Dr. Chun-Wei Xu, De- postoperative relapse. The frequently used partment of Pathology, General Hospital of Beijing chemotherapy scheme is vincristine (V), actino- Military Region, 5 Nanmen Warehouse, Dongsishi- mycin D (A), and cyclophosphamide (C), or VAS. tiao Street, Dongcheng District, Beijing 100700, P. Douglas et al. [17] found that eight cases of 18 R. China. Tel: +86-10-66721576; Fax: +86-10- infants with type I PPB who received only surgi- 66721576; E-mail: [email protected] cal treatment had relapsed. In contrast, none of the 14 infants receiving postoperative che- References motherapy relapsed. Chemotherapy seems to [1] Fosdal MB. Pleuropulmonary blastoma. J reduce PPB relapse considerably, although Pediatr Oncol Nurs 2008; 25: 295-302. large-dose chemotherapy may induce compli- [2] Manivel JC, Priest JR, Watterson J, Steiner M, cations and even death. Chemotherapy is pri- Woods WG, Wick MR, Dehner LP. Pleu- marily reserved for large tumors. However, one ropulmonary blastoma. The so-called pulmo���- research study indicated that no obvious differ- nary blastoma of childhood. 1988; 62: ence existed regarding the survival of patients 1516-1526.

13576 Int J Clin Exp Pathol 2015;8(10):13571-13577 Infant pleuropulmonary blastoma: a case study

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