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Home , Mesothelioma, Solitary fibrous tumor

DOI: 10.26717/BJSTR.2017.01.000150 Flavio Colaut. ISSN: 2574-1241 Biomed J Sci & Tech Res Case Report Open Access of the Pleura: Histology, CT Scan Images and Review of Literature over the Last Twenty Years

Giulia Bora1, Flavio Colaut2*, Gianni Segato3, Luisa Delsedime4 and Alberto Oliaro1 1Department of Thoracic Surgery, University of Turin, Italy 2Department of General Surgery and Thoracic, City Hospital , Montebelluna, (Treviso), Italy 3Department of General Surgery, S. Bortolo City Hospital, Vicenza, Italy 4Department of Pathology, University of Turin, Italy Received: June 14, 2017; Published: June 26, 2017

*Corresponding author: Flavio Colaut, Department of General Surgery, City Hospital Montebelluna, Thoracic City Hospital, via Montegrappa 1, 31044 Montebelluna (Treviso), Italy, Tel: ; Fax: 0499367643; Email:

Introduction

Literature up to 800 cases [1-3] have been reported, and these in case of recurrence [10,16,17]. In less than 5% of patients with Solitary fibrous tumor of the pleura is a rare . In numbers show its rarity, despite of , the most pleural SFPTs an increase of insulin-like factor II type occur and this causes refractory to therapy hypoglycaemia (Doege-Potter syndrome) similar in both sexes and there no differences in both benign and [10,18,19]. The incidence of Doege-Potter syndrome in SFPT is tumors represented. Males and females are equal distributed , tobacco or others environmental agents, were found for and the same is true for age. No correlation with exposure to malignantSome patients forms. may also present gynecomastia or galactorrhoea its development. Solitary fibrous tumor of the pleura occurs as localized of the pleura and was initially classified as microscope and , has been possible [1]. Sometimes large size SFPTs may appear with an unusual clinical “localized ”. Recently, with the aid of the electronic emphasize that their origin is mesenchymal and not mesothelial, so presentation, as the two cases reported by Santambrogio et al. [20] and Shaker et al. [21]. The first described a patient with large size the term “localized mesothelioma” has been replaced with “solitary author reported the case of a woman with lower limbs edema and SFPTs manifested with syncope episodes of coughing. The second only in the pleura but recently it has been found located also in fibrous pleural tumor” STFP [4]. In the past STFP were described other sites [5,6] such as abdomen, , , retroperitoneal dyspnea caused by a bulky SFPTs compression to the right atrium spaces, meninges, orbit, thyroid, salivary glands and soft tissues Histologicaland the inferior Characteristics vena cava. synchronous or metacron neoplasms like prostate, , breast, including the breast [7-10]. The STFP can be associated to other England [10] suggested SFPTs originate from sub-mesothelial . Histologically SFPTs occur such as low-grade endometrial and thyroid. Although most of STFP are neoplasms with variable cellularity. The cells present oval or The clinical behavior is unpredictable and probably is due to their benign neoplasms, a part of these could have a malignant behavior. fusiform shape with oval nuclei and is well distributed. Electronic microscopy has identified at least 3 pathological patterns: “subverted order” is the most common. In this type the histological and morphological features. STFP may remain silent for The second most common pattern is “-like”, Clinicalmany years Features before turning into malignant form [11]. cells and the surrounding collagen do not exhibit any architecture.

in which dense anastomoses are highlighted among blood vessels. Often SFPTs are accidentally discovered by radiological The third one is the least frequent and refers to angiofibroma-like, investigation like X-Ray [12,13]. Most patients with SFPTs become -like patterns and synovialsarcoma-like. symptomatic when these tumors reach large size [2,10,14]. About Histologic differential diagnosis is difficult and includes spindle- 54-67% of patients with benign SFPTs are symptomatic, while 75% cell , sarcomatoid mesothelioma and . of cases of malignant SFPTs, show symptoms [15]. These symptoms Recently immunohistochemistry has revealed to be extremely are cough, chest pain and dyspnoea. If there is obstruction of the useful for differential diagnosis. Perrot et al. [2] summarized the airway, also and pneumonia [2,10] could be seen. most important immunohistochemical characteristics: in SFPTs Paraneoplastic syndromes, represented by digital hypocratism vimentin is positive and keratin negative. CD34 is + in most benign and Pierre-Marie-Bamberg syndrome, are observed in 10-20% vanish after the neoplasm has been removed, but may reappear SFPTs as well in malignant one, while it reremains negative in of cases and specially with large size SFPTs. Symptoms usually most of other tumors of the lung. Lately, a cytogenetic procedure Cite this article: DOI: Giulia B, Flavio C, Gianni S, Luisa D, Alberto O. Solitary Fibrous Tumor of the Pleura: Histology, CT Scan Images and Review 188 of Literature over the Last Twenty Years.. Biomed J Sci & Tech Res. 1(1)-2017. BJSTR.MS.ID.000150. 10.26717/BJSTR.2017.01.000150 Flavio Colaut. Biomed J Sci & Tech Res Volume 1- Issue 1: 2017

has also contributed: SFPTs is often associated with trisomy 8 and trisomy 21 and this is helpful for diagnose and differentiate fibrous hybridization has shown that these chromosomal abnormalities are pleural tumor from mesothelioma and other sarcomas. Genomic more frequent in SFPTs larger than 10 cm and this correlation may suggest that gene can promote the growth of neoplasia. Also SFPTs maliciousness prediction is not easy. England et al. [10] definedTable 1: Malignancy criteria criteria (Table by England 1). et al. [10].

A Abundant cellularity with dense and overlapping nuclei C B High mitotic activity with more than 4 mitotic figures per fields Macroscopic Description: these neoplastic cells have bulky, D PleomorphismLarge necrotic with or haemorragic cytonuclear areasatypia ovoid, monomorphic nuclei; mitotic 14/10 HPFs (two E evident mitosis in photo 2); Focal areas of necrosis are present. There are also areas of sclerosis and stromal and F perivascular healing. Atypical localization Figure 2: Hematoxylin eosin stain 400x.

Perrot et al [11] according to the revision of the literature clinic presentation: divided SFPT in four stages, correlating histologic features with

Stage 0: SFPT pedunculated without signs of malignity

Stage 1: SFPT sessile without signs of malignancy

Stage 2: SFPT pedunculated with histologic signs of malignancy

Stage 3: SFPT sessile with signs of malignancy

Stage 4: Multiple and metastatic SFPT the presence of hypercellularity, atypia, cellular pleomorphism, The malignancy criteria used in this classification include necrosis and invasion increased number of mitosis, high number of mitosis per field, Macroscopic Description: Proliferation cells were found to be intensely positive to immunohistochemical reaction for CD34 (see photo 3, 200x) and for CD99 (+). Macroscopicstromal / vascular. Description Of Histological Figure 3: Hematoxylin-Eosin stain 200x. Slides (Figures 1-3)

Turin By gentle courtesy of Luisa Delsedime, Pathology, University of Diagnosis Chest X-Ray

Macroscopic description: 5 gray-whitish rounded suture shrubs: the first cm 12x7.2x7 size, the second cm 13x9x8 size, the third cm 10x9x7 size, the fourth cm 5x5x1.5 size and the fifth one cm 2.5x1.8x1 size. All fragments include irregular surface, with fibrous pseudocapsula, in appearance lobulated with mucoid areas on the surface of the section; multiple nodules are surrounded by unhindered lung rhymes. The nodes macroscopically described are constituted by proliferation of fused cells, circumscribed by fibrous pseudocapsules. Figure 1: Hematoxylin-eosin stain 100x Figure 4: Rx of the Chest.

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Chest X-ray shows a well-defined mass that is localized typically lower lobes [22] (Figure 4). in the surface of the lung. SFPT is often located in the middle and

Figure 5: CT-Scan. Pleural nodulations on the left side.

Chest-CT-Scan

haemangiopericitoma, fibrosarcoma and malignant fibrous hystyocytoma [34-36]. A great help for diagnosis comes from SFPT CT scan typically demonstrates homogeneous immunohystochemical analysis. nodulations, well defined on the pleural surface. In some cases it Therapy results difficult to distinguish SFPT from interlobar masses [23]. Surgical treatment In some SFPT calcifications can also be observed, regardless of the benignity or malignancy of the lesion [10,11] and it can be difficult Scan is not possible differentiate a benign lesion from malignant excision of the neoplasm because of the high rate of recurrences to differentiated them from a bronchial [24]. With CT The best treatment for SFPT is and complete surgical one and even the dimensions do not correlate with the behaviour but sometimes even a lobectomy is necessary, partial pleurectomy [37]. Pedunculated tumors can be resected with a wedge resection, ofMagnetic the neoplasm resonance [25,26] (Figure 5). is adherent to the parietal pleura, dissection must be used by or even a resection in block with part of chest wall. If the neoplasm

Magnetic resonance imaging (RM) has a limited use in pleural extrapleuric via [16,38]. toracotomy, anterior as well as the approach by diseases. RM investigation is better to TC for delineate morphology Regarding surgery access, it can be used postero-lateral and the relationship large-size SFPTs with adjoining mediastinal Angiography structures, like great vessels and with the diaphragm [11,27-29]. Video-Assisted Thoracoscopic approach, according to the size of theAdjuvant tumor and its location.

Angiography is an important diagnostic tool. It allows localizing supply from the frenic artery, intercostal artery or from the the SFPTs vascular peduncle [30]. The demonstration of arterial internal mammary vessels, can be very helpful to determinate the Currently, adjuvant chemotherapy is used after surgery resection and it’s recommended in malignant SFPT, especially in recurrentNeo-Adjuvant forms. chemotherapy extrapulmonaryChest-ultrasound origin of large-size SFPTs. The indication to perform an ultrasound chest scan for In SFPT neo-adjuvant chemotherapy is limited due to difficulty ofPrognosis obtaining a pre-operative histologic diagnosis [39]. diagnoses SFPTs or its evaluation isn’t shared by many authors, exceptPetWith18Fluoroosoxyglucose for a trans-thoracic for typing [31]. 12% of the cases evolves in spread of Prognosis of SFPT is generally good (88%), but approximately

Recently has been documented that FDG-PET can be used to occurs up to 17 years after resection and is usually located in diagnose and for follow up of treated patients. Cardillo et al. [32]. In intrathoracic or non-resectable recurrence. The relapse their study confirmed the high negative predictive value of PET for for compression and for the inferior vena cava assessment of the malignancy behaviour of the lesions. In the case the same emithorax [35]. Intrathoracic recurrence can be lethal of multiple SFPTs and a high metabolic rate highlighted by FDG- generally they are localized to liver, central nervous system, , PET,Differential a malignant Diagnosis nature must be suspected [33]. obstruction [18]. Metastases spread occur by hematogen via and

peritoneum, , gastrointestinal tract, and bone. mesothelioma, neurogenic , , The main differential diagnosis of malignant SFPT includes After resection of malignant sessile SFTP the risk of relapse is higher. Most SFPT relapses of malignant sessile occur within two Submission Link: 190 http://biomedres.us/submit-manuscript.php Flavio Colaut. Biomed J Sci & Tech Res Volume 1- Issue 1: 2017

years from resection and approximately about 50% of the relapses

useful. Indeed, in addition to the reaction with CD34, the recent is the cause of death. Therefore is recommended check with introduction of protein STAT36 seems to allow a better and more Chest X-Ray or CT- Scan every six months during the first 2 years accurate diagnosis. The best treatment remains the surgical and then once a year. In case of recurrence is indicated surgical resection, especially earlier and radical at the presentation. mandatory and in case of recurrences it is necessary to consider the treatment [39]. The most important predictors are morphologic Because of the uncertain behavior after surgery, a long follow-up is and histological indicators [4]. characteristics and prognosis: Referencesoption of a re-surgical approach. De Perrot [11] proposed a SFPT’s classification based on their ( 2006) Solitary fibrous tumor of the pleura. Cancer Control A. A recurrence of 2% in pedunculate benign tumors 1. Robinson LA De 13(4): M, Kurt 264-269. JH, Spiliopoulos (1999) Clinical behavior of solitary tumors of the Thorac B. B recurrence of 8% in sessile benign tumors 2. SurgPerrot AM, Robert Borisch B, A fibrous pleura. Ann C. C recurrence of 14% pedunculated malignant tumors Szkorupa67(5):1456-1459. M, Klein J, T, C, Chudàcek J (2010) Solitary of the . Chir D. D recurrence of 63% and mortality of 30% within the first 3. Bohanes Neoral Chan JKC (1997) Solitary tumour-everywhere, and a diagnosis 2 years in malignant sessile FSTP fibrous tumor Rozel 89(12): 750-753. in 4. fibrous E. E pleural effusion vogue. M, Mark EJ, Dickersin 31(6): 568-576. tumors of the pleura: eight new cases and review of 360 cases in the F.However F atypical more localization than dimension histological features are 5. CancerBriselli GR (1981) Solitary fibrous important [11] and the most important indicator of clinic outcome literature. Vallat-Decouvelaere47(11): 2678-2689. Dry SM, Fletcher CDM ( and malignant solitary tumors in extrathoracic locations: evidence 6. of their comparabilityAV, to intra-thoracic 1998) J AtypicalSurg is the complete recability of the tumor at the first presentation 22(12): fibrous (TableTable 2: 2). SFPT clinical manifestations related with frequency of symptoms. tumors. Am Pathol Ibrahim 1501-1511. JC, Corrin (1993) Double primary localized Benign Malign tumors of the pleura and retro Histopathology 7. NB, Briggs B fibrous peritoneum. Clinical manifestation 22(3):Vaswani 282-284. K, Guttikonda S, Vitellas KM (2000) Case Localized tumor of the J Symptomatic 8. 1. fibrous Sandvliet liver. HeysteegAJR Am M, Roentgenol (2000)175(3): large 875-876. thoracic mass in a + +++ 57-year-old patient: solitary tumor of the Chest 117(3): Casual or Accidental Discovery ++ +/- 9. RH, Paul MA A fibrous pleura. Dyspnea Pain +/- +++ 897-900.England DM, Hochholzer L, McCarthy MJ (1989) Localized benign and Macroscopic characteristics malignant brous tumors of the pleura: a clinico pathologic review +/- +++ 10. of 223 cases J Surg 13 fi De M,. AmFischer S,Pathol (8): 640-658. Sekine Y, S (2002) AtypicalSize <10 Locations cm Infrequent Frequent Solitary tumors of the Thorac Surg 74 Sessile 11. Perrot Bründler MA, Keshavjee Infrequent Frequent Magdeleinatfibrous , M, etinopleura. Le Annochais J , Dulmet E,(1): et 285-293. (2002) Infrequent Frequent Solitary tumors of the pleura: clinical characteristics, surgical 12. treat- ment andPAlifano P Eur J CardiothoracA, R SurgP al. Peduncolate Frequent Infrequent fibrous Santambrogio L,outcome. M, L, 21(6):Tosi D, 1087-1093.et ( 2008) Necrosis Rare Frequent Solitary tumor of the pleura presenting with syncope episodes Bleedind Rare Frequent 13. when Nosotti J Surg PalleschiOncol A, Rosso al. fibrous CalcificationMicroscopic characteristicsFrequent Rare Klemperercoughing. , World ( 6: 86-90. neoplasm of the pleura: a report of Ipercellularity 14. P Rabin CB 1992) Primary Shield T five( 2004)cases. LocalizedArch Pathol 22(1): tumors 1-31. of the In: Shields: Rare Frequent General Thoracic Surgery (4th edn), 15. W fibrous pleura. High mitotic index - Cellular Pleomorphysm Rare Frequent Williams & Wilkins, Baltimore, O, Filosso E, Molinatti M, Di Marzio , et ( 2001) Frequent USA, pp. 722-730. Solitary tumor of the pleura: surgical Eur J Invasion of theNecrosis surrounding tissue Rare- Frequent 16. CardiothoracRena SurgPL, Papalia P al. fibrous treatment. Frequent Chaugle H, 19(2): C, Grotte 185-189. GJ, Keenan DJM ( 1999) Conclusion associated with a solitary tumour of the Eur J 17. CardiothoracParchment Surg fibrous pleura. M, Mark EJ,15: Dickersin 84-86. (1981) Solitary tumors of SFPT is a rare disease with uncertain behaviour, curable in most the pleura: eight new cases and review of 360 cases in the 18. CancerBriselli GR fibrous cases. From a biological point of view, we need more knowledge literature. to optimize the clinical outcome. Immunohistochemistry is very 47(11): 2678-2689.

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