CASE REPORT Est J Med 21(1): 45-49, 2016 Pulmonry lngerhns cell histiocytosis: Two cses with vried rdiologic findings Huly Guntr 1,*, Alpsln Yvuz 2, Bunymin Sertogullrindn 1, Selmi Ekin 1, Selvi Asker 1, Fut Syır 3, Irfn Byrm 4 1Deprtment of Pulmonry nd Criticl Cre, Yuzuncu Yil University Medicl Fculty, Vn, Turkey 2Deprtment of Rdiology, Yuzuncu Yil University, Medicl Fculty, Vn Turkey 3Deprtment of Pulmonry Surgery, Yuzuncu Yil University Medicl Fculty, Vn Turkey 4Deprtment of Pthology, Yuzuncu Yil University Medicl Fculty, Vn Turkey ABSTRACT Pulmonry Lngerhns Cell Histiocytosis (PLCH) is n idiopthic interstitil lung disese with Lngerhns cell infiltrtion in the lung. PLCH X hs non-spesific symptoms, nd most ptients hve smoking history. A comintion of stellte nodules, reticulr nd nodulr opcities, upper zone cysts or honeycoming, preservtion of lung volume nd costophrenic ngle spring re highly specific for PLCH. To contriute to the literture, two cses re presented. First cse is 30 yers old mn with 10 pck/yers smoking history ws dmitted with cough nd persevering interstitil opcities. Second cse is 34 yers old mn with 15 pck/yers smoking history ws dmitted with persistnt cough. On thorx CT the first cse hd reticulonodulr opcities t the perifery of the upper nd middle zones, second cse hd multiple prenchyml cystic nodulr lesions. Open lung iopsy performed, immunohistochemicl exmintion CD1, CD68, S100 detecting ntigenpositive nd histologiclly nd rdiologiclly confirmed dignosed of PLCH. Key Words: Pulmonry Lngerhns cell histiocytosis, rdiology, histology Introduction Pulmonry Lngerhns Cell Histiocytosis (PLCH) is rre form of interstitil lung diseses tht primrily ffects young dults (1-5). The certin incidence nd prevlence re unknown. No occuptionl or geogrphic predisposition hs een reported, ut mjority of the ffected popultion hve history of ongoing or pst smoking. The Lngerhns cell is the pthologic cell type of PLCH. Lngerhns cells lso demonstrte positive immunohistochemicl stining for S100 protein nd hve strong presence of CD1 ntigen (CD1) on the cell surfce. Ptients with PLCH re mostly determined y sole or comintion of severl ltered presenttions such s; incidentlly detected findings on chest rdiogrphs including spontneous pneumothorx nd/or with respirtory or constitutionl symptoms (6,7). High resolution computed tomogrphy (HRCT) of the chest cn provide significnt dt to led the dignosis. A comintion of the fetures such s stellte nodules lung volume nd costophrenic ngle spring re highly specific for PLCH (8,9). Becuse of the rrity; we reported the vried rdiologicl fetures of two cses with histologiclly nd rdiologiclly confirmed dignosed of PLCH. Cse report Cse 1: A 30 yers old mn referred with two month history of dyspne, cough, sputum nd night swets. Before his dmission, he hd n ntiiotherpy in nother epicenter, his complints hve regressed. He ws referred to our hospitl ecuse of the persevering normlities detected on chest rdiogrphs. On his initil dmission; physicl exmintion nd routine hemtology prmeters including ESR were within norml rnges (ESR ws 7 mm t the end of first hour). Sputum cultures including for tuerculosis were negtive. Blood gses nd pulmonry function tests were norml. In nmnesis, he hd 10 pck/yer smoking history. The chest X-ry reveled reticulonodulr opcities t the perifery of the upper nd middle zones. In thorx computed tomogrphy (CT) exmintion; pulmonry prenchyml nodules with rnge of 1-5 mm in dimeter were detected, lesions were mostly with centriloulr distriution (rrows), ut few were locted t supleurl region (rrow hed). Mgnified investigtion of the pulmonry prenchym on chest CT reveled centrl hypo-dense components leding cvittion (Imge 1,). Open lung iopsy performed to the ptient, immunohistochemicl exmintion This report ws provided s cse of council in TÜSAD 2014 ntionl rething congress *Corresponding Author: Dr Hüly Güntr, Deprtment of Pulmonry nd Criticl Cre, Yuzuncu Yil University Medicl Fculty, Vn, Turkey, Phone: +90 (432) 215 04 70-6135, Moil Phone: +90 (506) 511 88 27, E-mil: hulyguntr@hotmil.com Received: 27.10.2014, Accepted: 20.03.2015
CD1, CD68, S100 detecting ntigen-positive nd lngerhns cell histiocytosis dignosed (Figure 1,,c). Fluorodeoxyglucose-PET (FDG-PET) ws performed the ptient for determining the systemic involvement, FDG PET imging ws negtive. Smoking cesstion ws recommended, he stopped smoking nd still in follow up. Imge 1. Chest CT imge from 27-yer-old smoker mle with iopsy-proven Pulmonry Lngerhns cell histiocytosis demonstrting multiple pulmonry nodules which mostly pronounced erly in the disese. ) Nodules were 1-5 mm in dimeter, mostly with centriloulr distriution (rrows), ut few were locted t supleurl region (rrow hed). ) Mgnified exmintion on chest CT reveled centrl hypo-dense components leding cvittion. c Fig 1. lrge clusters of cells tht re comptile with eosinophilic cytoplsm infiltrting Lngerhns cells (H & E x10). ) memrnous stining of CD1, Lngerhns cells (immunohistochemicl exmintion, x20). c) S100 proteins in the nucler nd cytoplsmic stining, Lngerhns cells (immunohistochemicl exmintion, x20). 46
Cse 2: A 34 yers old mn, who ws n employee s technicin of n operting room, ws referred with two month history of cough nd incresed sensitivity to odors. Physicl exmintion ws norml. He hd 15 pck/yer history of smoking. After non-specific ntiiotherpy, HRCT ws performed ecuse of the progression in cough. Chest CT imge reveled multiple prenchyml cystic nodulr lesions rnging 5-20 mm in dimeter, mostly with the wll-thickness of few millimeters. Vrile shpes with loulted contours were detected s the result of the confluence of 2 or more lesions. Coronl reformt CT imge demonstrted the mid nd upper zone predilection (Imge 2,). Blood gses nd pulmonry function tests were norml. He ws dignosed s lngerhns cell histiocytosis ecuse of typiclly HRCT findings, smoking cesstion ws recommended, he left smoking nd still in follow up s symptomtic. Discussion The histiocytic disorders re rre conditions chrcterized y norml infiltrtion of specific orgns y cells reproduced from monocyte/mcrophge or dendritic cell origin. Lngerhns cell histiocytosis (LCH) is specific type of histocytic syndromes which is chrcterized y infiltrtion of the tissues with Lngerhns cells (10). LCH my ffect n isolted orgn or my ct s multisystemic disese (formerly termed s Hnd- Shuller-Christin or Letterer-Siwe disese) (11). In our cses, pulmonry involvement ptterns were demonstrted solely. The signs nd symptoms of PLCH re nonspecific nd should prompt considertion of superimposed infection such s Aspergillus or tumor (12, 13). Nonproductive cough, dyspne nd pleuritic chest pin re the most common symptoms (1,5,14). Cough ws the most frequent symptom in our cses. The pulmonry involvement y the disese predomintes in the mid to upper zones of the lung nd spres the costophrenic ngle which is in contrst with the typicl lower zone involvement of idiopthic pulmonry firosis (8,15). Lesions frequently extend widely into the prenchym of the lung surrounding the ronchovsculr structures, producing the soclled stellte lesions tht re chrcteristic of this disorder. Interstitil firosis nd smll cyst formtion occur with dvncing disese; the mechnism for cyst formtion is unknown (7). Reticulonodulr infiltrtions re dominnt findings in chest CT in erly stge wheres cystic lesions nd emphysem re more predominnt in dvnced stge. In the first cse of our report; reticulonodulr infiltrtes were predominnt, cvitted nodules locted t the perironchil nd centriloulr region were determined. These findings supported tht the disese ws t its erly stge. However; in the second cse, findings including diffuse cyst formtions locted t the upper nd middle zones of the pulmonry prenchym indicted tht the disese ws on n dvnced stge especilly when compred with the first cse. The clinicl signs nd symptoms of PLCH re mostly my not e sufficient to distinguish itself from other non-specific pulmonry diseses. HRCT of the chest cn provide n ccurte finl dignosis if is demonstrting well-descried CT findings of the issue, s prticulrly in the second cse. Imge 2. Chest CT imge from 33-yer-old mle with iopsy-proven Pulmonry Lngerhns cell histiocytosis. ) Cysts were 5-20 mm in dimeter, mostly with the wll-thickness of few millimetres. Vrile shpes were seen s the result of the confluence of 2 or more cysts. ) Coronl reformt CT imge demonstrted the mid nd upper zone predilection. 47
Pulmonry function tests re norml out 20% of ptients. Restrictive pttern is commonly seen in erlier stges, while ostructive pttern ecomes dominnt in dvnced stges (12,16). In our presenttion, two cses hd norml pulmonry function tests. FDG-PET CT scns my show incresed uptke in PLCH, especilly t erly stge in the course of disese. Krjicek et l. (17) evluted series of 11 ptients with PLCH; five of the ptients hd norml FDG uptke in their lungs. The ptients with FDG-PET positivity were more likely to hve nodulr pttern, suggesting erlier disese; those with negtive FDG-PET scns were more likely to hve cystic pttern nd less nodules, suggesting n dvnced disese. In the first cse of our report; FDG-PET ws negtive which could e explined y the existence of the smll sized nodules in pulmonry prenchym, ecuse previous reports reveled tht FDG ctivity in PET CT cn e negtive for the nodules with sizes less thn 1 cm (18,19). Surgicl pproches including Video Assisted Thorcic Surgery or open lung iopsies my e involved to provide definite dignosis of PLCH; thus, demonstrtion of the specific histopthologicl fetures cn e otined (20,21). Lngerhns cells lso demonstrte positive immunohistochemicl stining for S100 protein nd hve strong presence of CD1 ntigen (CD1) on the cell surfce. In the first cse open lung iopsy specimens showed CD1, CD68, S100 detecting ntigen-positive nd lngerhns cell histiocytosis dignosed. There is no widely ccepted definite tretment guideline protocol for PLCH mngement in recent literture except few recommendtions tht were reported regrding to tretment of dult ptients with PLCH The most effective component of the PLCH tretment is indicted s smoking cesstion. It ws reported tht the smoking cesstion my solely prevent the progression of the disese so, it should e strongly encourged (14,21). In conclusion, PLCH is n extremely rre enign disese. The ptients could referred with different cliniclly nd rdiologiclly findings. References 1. Tzi A, Soler P, Hnce AJ. Adult pulmonry Lngerhns' cell histiocytosis. Thorx 2000; 55: 405-416. 2. Vssllo R, Ryu JH, Coly TV, Hrtmn T, Limper AH. Pulmonry Lngerhns'-cell histiocytosis. N Engl J Med 2000; 342: 1969-1978. 3. Vssllo R, Ryu JH. Pulmonry Lngerhns' cell histiocytosis. Clin Chest Med 2004; 25: 561-571. 4. Sundr KM, Gosselin MV, Chung HL, Chill BC. Pulmonry Lngerhns cell histiocytosis: emerging concepts in pthoiology, rdiology, nd clinicl evolution of disese. Chest 2003; 123: 1673-1683. 5. Tzi A. Adult pulmonry Lngerhns' cell histiocytosis. Eur Respir J 2006; 27: 1272-1285. 6. Bsset F, Corrin B, Spencer H, et l. Pulmonry histiocytosis X. Am Rev Respir Dis 1978; 118: 811-820. 7. Vssllo R, Limper AH. Pulmonry Lngerhns cell histiocytosis. Interstitil Lung Disese, 4th ed, King TE Jr, Schwrz MI (Eds), B.C. Decker, Hmilton, ON, Cnd 2003. p.838. 8. Kulwiec EL, Lynch DA, Aguyo SM, Schwrz MI, King TE Jr. Imging of pulmonry histiocytosis X. Rdiogrphics 1992; 12: 515-526. 9. Lcronique J, Roth C, Bttesti JP, Bsset F, Chretien J. Chest rdiologicl fetures of pulmonry histiocytosis X: report sed on 50 dult cses. Thorx 1982; 37: 104-109. 10. Fvr BE, Feller AC, Puli M, et l. Contemporry clssifiction of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Prolifertions. Reclssifiction Working Group of the Histiocyte Society. Med Peditr Oncol 1997; 29: 157-166. 11. Komp DM. Historicl perspectives of Lngerhns cell histiocytosis. Hemtol Oncol Clin North Am1987; 1: 9-21. 12. Crusmn RS, Jennings CA, Tuder RM, et l. Pulmonry histiocytosis X: pulmonry function nd exercise pthophysiology. Am J Respir Crit Cre Med 1996; 153: 426-435. 13. Knight RK. Hemoptysis in eosinophilic grnulom. Br J Dis Chest 1979; 73: 181-186. 14. Cminti A, Hrri S. Smoking-relted interstitil pneumonis nd pulmonry Lngerhns cell histiocytosis. Proc Am Thorc Soc 2006; 3: 299-306. 15. Kim HJ, Lee KS, Johkoh T, et l. Pulmonry Lngerhns cell histiocytosis in dults: highresolution CT-pthology comprisons nd evolutionl chnges t CT. Eur Rdiol 2011; 21: 1406-1415. 16. Vssllo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinicl outcomes of pulmonry Lngerhns -cell histiocytosis in dults. N Engl J Med 2002; 346: 484-490. 17. Krjicek BJ, Ryu JH, Hrtmn TE, Lowe VJ, Vssllo R. Anorml fluorodeoxyglucose PET in pulmonry Lngerhns cell histiocytosis. Chest 2009; 135: 1542-1549. 18. Higshi K, Ued Y, Seki H, et l. Fluorine-18-FDG PET imging is negtive in ronchiololveolr lung crcinom. J Nucl Med 1998;39: 1016-1020. 48
19. Ersmus JJ, McAdms HP, Ptz EF, et l. Evlution of primry pulmonry crcinoid tumors using FDG-PET AJR 1998; 170: 1369-1373. 20. Suri HS, Yi ES, Nowkowski GS, Vssllo R. Pulmonry ln-gerhns cell histiocytosis. Orphnet J Rre Dis 2012; 7: 16. 21. Juvet SC, Hwng D, Downey GP. Rre lung diseses III: pul-monry Lngerhns' cell histiocytosis. Cn Respir J 2010; 17: 55-62. 49