Castleman’s disease (CD) is a rare lymphoproliferative disorder. Clinically CD has been subdivided in two forms: unicentric and multicentric. The unicentric type is limited to a single anatomic lymph-node-bearing region. The present report describes two cases of unicentric CD: the first was an abdominal localization treated with a laparoscopic

approach; the second was a submaxillary localization treated with a classical approach. In case 1 the laparoscopic approach permitted to reach diagnosis, not clear after diagnostic imaging procedures, and enabled a total and excellent resolution of the patology because our patient,after eight months of follow up, has had no evidence of recurrence of the disease. In case 2 we want to highlight that CD should be considered in the differential diagnosis of a solitary neck mass and that the surgical treatment is diagnostic and curative at the same time

Berni, Aalberto

Blasi, Sara

Campana, Francesco Paolo

Maturo, Alessandro

Palazzini, Giorgio

Polichetti, Paolo

Sgueglia, Monica

Tartaglia, Francesco

Tromba, Luciana

Scientific Open-access Literature Archive and Repository

IntroductionCastleman’s disease (CD) is a rare lymphoprolifera-tive disorder, first described as a pathologic entity in1954 and defined by Castleman et al. in 1956 (1).Flendrig and Schillings distinguished two basic patho-logic types and one mixed variant (2), while Keller etal. designated hyaline-vascular, plasma cell and hyalinevascular plasma cell type (3).Clinically CD has been subdivided in two forms:unicentric and multicentric. The unicentric type is li-mited to a single anatomic lymph-node-bearing regionand is rarely associated with systemic symptoms. Themulticentric type is characterized by a more extensivelymph node involvement, severe systemic symptomsand a progressive clinical course with potential for ma-lignancy. The disease may occur anywhere along thelymphatic chain most commonly in the torax. It is re-latively rare in the neck, abdomen, retroperitoneumand pelvis. We present two cases of unicentric CD. The firstwas localized between the lesser curve of the stomachand the left hepatic lobe. It was treated with a laparo-scopic approach. The second was a submaxillary loca-lization treated with a classical approach.SUMMARY: Unicentric localization of Castleman’s disease treatedwith laparoscopic and traditional approach. Report of two cases.F. TARTAGLIA, S. BLASI, A. BERNI, M. SGUEGLIA, P. POLICHETTI, A. MATURO, G. PALAZZINI, L. TROMBA, F.P. CAMPANACastleman’s disease (CD) is a rare lymphoproliferative disorder.Clinically CD has been subdivided in two forms: unicentric and mul-ticentric. The unicentric type is limited to a single anatomic lymph-no-de-bearing region. The present report describes two cases of unicentricCD: the first was an abdominal localization treated with a laparosco-pic approach; the second was a submaxillary localization treated witha classical approach. In case 1 the laparoscopic approach permitted toreach diagnosis, not clear after diagnostic imaging procedures, andenabled a total and excellent resolution of the patology because our pa-tient, after eight months of follow up, has had no evidence of recurren-ce of the disease. In case 2 we want to highlight that CD should be con-sidered in the differential diagnosis of a solitary neck mass and that thesurgical treatment is diagnostic and curative at the same time. RIASSUNTO: Due casi di malattia di Castleman a localizzazioneunicentrica trattati con tecnica laparoscopica e tradizionale.F. TARTAGLIA, S. BLASI, A. BERNI, M. SGUEGLIA, P. POLICHETTI, A. MATURO, G. PALAZZINI, L. TROMBA, F.P. CAMPANALa malattia di Castleman è una patologia rara a carattere linfo-proliferativo. Clinicamente può assumere due forme: unicentrica emulticentrica. La forma unicentrica è caratterizzata dall’interessa-mento di una singola stazione linfonodale. Nel presente lavoro sono ri-portati due casi di malattia di Castleman unicentrica: il primo a lo-calizzazione addominale trattato con approccio laparoscopico; il secon-do a localizzazione sottomascellare trattato con  approccio tradiziona-le. Nel primo caso l’approccio laparoscopico ha permesso di definire ladiagnosi, non chiara dopo le procedure diagnostiche strumentali e latotale ed eccellente risoluzione della patologia. La paziente, infatti, do-po otto mesi di follow up, non presenta recidiva. Il secondo caso con-ferma che la malattia di Castleman deve essere considerata nella dia-gnosi differenziale di una formazione solitaria del collo e che il tratta-mento chirurgico può essere diagnostico e terapeutico allo stesso tempo. University of Rome “La Sapienza” Department of Surgical Sciences© Copyright 2008, CIC  Edizioni Internazionali, RomaUnicentric localization of Castleman’s disease treated with laparoscopic and traditional approach. Report of two casesF. TARTAGLIA, S. BLASI, A. BERNI, M. SGUEGLIA, P. POLICHETTI, A. MATURO, G. PALAZZINI, L. TROMBA, F.P. CAMPANA  G Chir Vol. 29 - n. 10 - pp. 399-402Ottobre 2008399articoli originaliKEY WORDS: Unicentric Castleman’s disease - Lymphoproliferative disease - Laparoscopy.Malattia di Castelman unicentrica - Malattia linfoproliferativa - Laparoscopia.400F. Tartaglia et al.Case reportsCase n. 1A 36 years old woman, with a history of increasing fatigue, vi-sited her family physician to execute a general check up. The bloodinvestigations showed an increase of alanine amino transferase(ALT) and aspartate amino transferase (AST) serum levels. The abdominal ultrasound (Fig. 1) and the CT scan (Fig. 2)revealed the presence of a roundish mass of 3 centimeters in dia-meter with well-defined margins behind the left hepatic lobe nearto the second and the third segment. The nuclear magnetic reso-nance (Fig. 3) confirmed the presence of a solid contrast enhancedmass with a poorly vascularized central area, heteregenous surface,regular margins, behind the second and third hepatic segment.Moreover the abdominal magnetic resonance showed two lesionswith the same characteristics of 15 and 4 centimeters in diameterbehind the hepatogastric ligament. One of the two lesions seemedto be next to the superior margin of the pancreatic body. The FDGPET-TC total-body scan (Fig. 4) highlighted an increased activityin correspondence of the solid tissue in the hepatogastric ligament,behind the liver. It also demonstrated a slight raised activity in cor-respondence of the gastric antrum without morphological andstructural changes. The imaging findings were sufficient to hypothesize alymphoadenopathy but were not enough to estabilish the diagno-sis definitevely. In fact the close contiguity of the main mass to theliver didn’t allow to exclude the hepatic nature of the lesion. So shewas admitted to our hospital for further investigations.Laboratory tests showed sierological evidence of past Epstein-Barr virus (EBV) and Cytomegalovirus infections. On physicalexamination no superficial lymphadenopathy was found. Gastrointestinal endoscopy revealed hiatus hernia while colo-noscopy was negative for endoluminal lesions. Pelvic ultrasound(US) didn’t show any ovarian or uterine abnormalities. A needlebiopsy of the lesion under US guidance was performed but it wasnot sufficient to estabilish an histological diagnosis, because it justrevealed breeding grounds of hepatic steatosis.Then the patient was referred for surgical evaluation by the la-paroscopic approach. The patient was placed in supine straddle position and a gene-ral anaesthesia was administered. Two trocars were introduced toFig. 1 - The US exam reveals an hypo-echogenic mass between the stomachand the left hepatic lobe.Fig. 2 - The CT scan shows a mass of 3 centimeters in diameter with well-de-fined margins behind the left hepatic lobe near to the second and the thirdsegment.Fig. 3 - The MR shows a a solid  contrast-enhanced  mass with a poorly va-scularized central area.Fig. 4 - The FDG PET-TC total-body scan highlighted an increased activity incorrespondence of the solid tissue in the hepatogastric ligament describedby the previous exams.perform a diagnostic laparoscopy. The inspection of abdominal ca-vity revealed the presence of voluminous lymph nodes that cove-red the lesser gastric curvature. The liver, taken up with a fan re-tractor, didn’t barely adhere to lymph nodes. Therefore, with theaid of other two trocars, lymph nodes were dissected and removedby means of an endo-bag. The histologic examination of lymph nodes showed irregulargerminal centres with prominent hyalinized vascular proliferation.The hypervascular mantle zones were surrounded by smalllymphocytes in a concentric onionskin pattern with focal hyalinedeposits. Even if immunohistochemical findings revealed negativeexpression for CD68R and HHV8, the morphological aspects we-re typical for hyaline-vascular type of Castleman’s disease(HVCD). The patient’s postoperative course was without complicationsand she was discharged in third postoperative day and referred tothe hematologists for the follow.up. No recurrence has occurred after eight months follow up pe-riod and she is leading pregnancy without difficulties being to se-venth month of gestation at the moment of the drawing up of thepresent report.Case n. 2A 45 years old woman came to us because of the appearanceseven months before of a mass localized in the left submandibolarregion. The lesion was asymptomatic and was discovered duringself-examination. She had no family history of malignancy or inhe-rited history. She didn’t have systemic symptoms such as fever, fa-tigue, sweats and weight loss. The physical examination revealed the presence of a painlessroundish mass of two centimeters in diameter, with a smooth sur-face and sclerotic consistence in the left submandibolar region. Blood exams were normal and didn’t show sierological eviden-ce of Epstein-Barr, HIV and cytomegalovirus infections.Radiographs of the chest showed no abnormalities.An ultrasound of the region highlighted the presence of a clu-ster of hypoechoic lymph nodes of two centimeters in diameterwith an high grade of vascularization. Therefore it was decided toproceed to a surgical removal of the mass. In the left submandibolar compartment a cluster of voluminousand sclerotic lymph nodes tightly adherent to an enlarged andscirrhous submandibolar gland was removed. The frozen sectionexam revealed signs of chronic lymphadenopathy of uncertain natu-re therefore it was decided to remove also the submandibolar gland. Definitive histological examination of the lymph nodesshowed an increased number of withering hyalinized germinal cen-tres surrounded by concentrically layered mantle zones and an ex-tensive angiogenesis in the form of radially penetrating capillariesand venules with high endothelium. Immunohistochemical exami-nation demonstrated the presence of B lymphocites (CD20+ andCD79+) and of T cells (CD3+) and the permanence of dendriticfollicular cells (CD21+) in the germinal centres which were alsonegative for the expression of Bcl-2. Immunohistological diagnosiswas consistent with hyaline-vascular Castleman’s disease. Insteadhistological examination of submandibolar gland was consistentwith chronic sialoadenitis.The patient was referred to the hematologists and no recurren-ce has occurred after an year follow up period. DiscussionCastleman’s disease is a rare lymphoproliferative di-sorder that is uncommon, atypical and of unknownetiology. It is characterized by two distinct entitieswith similar histology but different course and thera-peutic response. The pathogenesis is still unknown buthuman Herpes virus-8 (HHV-8) and EBV play a roleespecially in immunocompromised hosts. Multicentric Castleman’s disease is a systemic di-sorder with generalized peripheral lymphoadenopathy,fever, night sweats, anemia, weight loss and it can beassociated with polyneuropathy, organomegaly, endo-crinopaty, M protein and skin change (POEMS syn-drome). This type of CD is progressive and has a poorprognosis. The unicentric type of CD is rarely reported in as-sociation with systemic symptoms and it interestsyounger age as in our patients. The hyaline-vascular(HV) subtype is more common in the localized forms.Virtually it can occur in any anatomical site wherelymphoid tissue is found but it is more frequent in thethorax (mediastinum, lung hilum) in proportion withvarious series ranging from 38.5% to 86% (4). It caninvolve neck (5), retroperitoneum, pelvis, mesentery,chest wall. In the English literature we found some reports ofunicentric CD. Few cases during pregnancy have beenreported. Hernandez reports the case of a pregnant pa-tient who developed a localized axillary hyaline-typeCD (6). Hata et al. describe the case of a 52 years oldmale with asymptomatic rectal polyp, which was dia-gnosed as Castleman’s disease concomitant with ga-stric adenocarcinoma (7). Przkora et al. report the ca-se of a locoregional HVCD with infiltration of the bi-le duct wall, localized infiltration of the liver andlymphadenopathy including suprapancreatic lymphnodes causing obstructive jaundice (8). A lymphade-nopathy in porta hepatis, along the common bile ductand the lesser curvature of the stomach causing ob-structive jaundice is reported by Al-Salamah et al. (9).It was classified as a expression of HVCD.In our case n. 1 the preoperative diagnosis was pro-blematic because of the absence of considerable symp-tomatology and of undisputed diagnostic investiga-tions. In fact, the abdominal ultrasound, the MRI andthe FDG PET-TC total body scan didn’t supply enou-gh informations to define the nature and the exact per-tinence of the lesion. On the contrary it was necessaryto perform further exams such as the colonoscopy andthe gastroscopy to exclude that the lymphoadenopathycould be a metastasis of an unknown primary. The-refore in order to formulate a definitive diagnosis itwas decided to proceed to an exploratory laparoscopy,that it allowed to identify with precision the locationof the lesion, its relationships with the surrounding or-gans and to perform its removal with consequent hi-stological diagnosis. Furthermore the laparoscopic ap-proach enabled a total and excellent resolution of the401Unicentric localization of Castleman’s disease treated with laparoscopic and traditional approach. Report of two casespatology and our patient, after eight months of followup, has had no evidence of recurrence of the disease. In English literature few cases of a laparoscopictreatment of abdominal unicentric Castleman’s diseasehave been reported (10, 11). Nevertheless in our casethis approach has allowed to unfold the diagnosis andit has been curative with the advantages derived froma mini-invasive approach.Our second case does not introduce any specific ele-ment of discussion because the lymph nodal nature ofthe mass was already demonstrated in the preoperativetime. However we want to report it for completenessand to highlight that Castelman’s disease should be con-sidered in the differential diagnosis of a solitary neckmass. The choice of treatment was the complete remo-val of the mass en-bloc with the submaxillary gland ob-viously performed by a traditional surgical approachthat was diagnostic and at the same time curative.402F. Tartaglia et al.1. Castleman B, Iverson L, Menendez VP. Localized mediastinallymph-node hyperplasia resembling thymoma. Cancer 1956,9: 822-3.2. Flendring JA. Benign giant lymphoma : clinicopathologic cor-relation study . In : Clark RL, Cumle RW, eds. The year bookof cancer. Chicago. Year Book Medical Publisher, 1970: 296-299.3. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular andplasma cell types of giant lymph node hyperplasia of the me-diastinum and other locations . Cancer 1972; 29(3): 670-683.4. Chih-Hao Chen, Hung-Chang Liu, Kwang-Yi Tung, JIE-JenLee, Chien-Liang Liu, Tsang-Pai Liu. Surgical Outcome of su-perficial and deep Castleman disease. J. Surg. 2007; 77: 339-343.5. Kooper DP, Tiwari RM, Van der Valk P. Castleman’s disease asa uncommon cause of a neack mass. 1994; 251(6): 370-2.6. Hernández JL, Gómez-Román J, Ramos-Estébanez C, Nan D,Martín-Oviedo J, Riancho JA et al. Human herpesvirus 8 andEpstein-Barr virus coinfection in localized Castleman diseaseduring pregnancy. Haematologica 2005; 90(10): e98-e101.7. Hata T, Ikeda M, Ikenaga M, Yasui M, Shingai T, YamamotoH, et al. Castleman’s Disease of the rectum: report of a case.Dis Colon Rectum. 2007; 50(3): 389-94. 8. Przkora R, Bruns C, Zulke C, Heinkelein J, Rummele P,Schalhorn A, et al. Castleman’s disease causing obstructivejaundice. J Hepatol. 2003; 38(4): 548-9.9. Al-Salamah SM, Khan IA, Khalid K, Al-Shakweer WA. Castle-man disease presenting as obstructive jaundice. Saudi Med J.2005; 26(1):111-3.10. Brusciano L, Rossetti G, Maffettone V, Napolitano V, Izzo D,Pizza F, et al. Laparoscopic treatment of an uncommon abdo-minal localization of Castleman disease. Surg Laparosc Endo-sc Percutan Tech. 2005; 15(4): 241-3.11. Corcione F, Caiazzo P, Cuccurullo D, Settembre A, MirandaL, Pirozzi F, et al. Laparoscopic treatment of unicentric Castle-man’s disease with abdominal localization. J Laparoendosc AdvSurg Tech A. 2005; 15(4): 400-4.References

Unicentric localization of Castleman’s disease treated

with laparoscopic and traditional approach. Report of two cases

La malattia di Castleman è una patologia rara a carattere linfoproliferativo. Clinicamente può assumere due forme: unicentrica e multicentrica. La forma unicentrica è caratterizzata dall’interessamento di una singola stazione linfonodale. Nel presente lavoro sono riportati due casi di malattia di Castleman unicentrica: il primo a localizzazione addominale trattato con approccio laparoscopico; il secondo a localizzazione sottomascellare trattato con approccio tradizionale. Nel primo caso l’approccio laparoscopico ha permesso di definire la diagnosi, non chiara dopo le procedure diagnostiche strumentali e la totale ed eccellente risoluzione della patologia. La paziente, infatti, dopo otto mesi di follow up, non presenta recidiva. Il secondo caso conferma che la malattia di Castleman deve essere considerata nella diagnosi differenziale di una formazione solitaria del collo e che il trattamento chirurgico può essere diagnostico e terapeutico allo stesso tempo

TARTAGLIA, Francesco

BERNI, Alberto

MATURO, Alessandro

PALAZZINI, Giorgio

TROMBA, Luciana

BLASI S

SGUEGLIA M

POLICHETTI PE

CAMPANA FP

Archivio della ricerca- Università di Roma La Sapienza

Unicentric localization of Castleman's disease treated with laparoscopic and traditional approach. Report of two cases.

http://eprints.bice.rm.cnr.it/5504/1/article%28167%29.pdf

Unicentric localization of Castleman’s disease treated with laparoscopic and traditional approach. Report of two cases

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