Sabato 27 ottobre 2012 - Pacini Editore

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268 Schmidt SM, Schag K, Mu¨ ller MR, et al. Induction of Adipophilin- Specific Cytotoxic T Lymphocytes Using a Novel HLA-A2-Binding Peptide That Mediates Tumor Cell Lysis. Cancer research 2004;64:1164-70. Tennant DA, Durán RV, Boulahbel H, et al. Metabolic transformation in cancer. Carcinogenesis 2009;30:1269-80. Yamashita T, Honda M, Takatori H, et al. Activation of lipogenic pathway correlates with cell proliferation and poor prognosis in hepatocellular carcinoma. J Hepatol 2009;50:100-10. Vander Heiden MG, Cantley LC, Thompson CB. Understanding the Warburg effect: the metabolic requirements of cell proliferation. Science 2009;324:1029-33. Primary Hodgkin lymphoma of the anus: case report and brief review of the literature M.R. Ambrosio1 , B.J. Rocca1 , M.G. Mastrogiulio1 , A. Barone1 , G. De Falco1 , A. Costa2 , C. Bellan1 , S. Lazzi1 1 Department of Human Pathology and Oncology, Pathological Anatomy Section, University of Siena, Italy; 2 Surgery Unit Valdichiana Hospital, Montepulciano, Italy Background. Primary anorectal lymphomas are very rare, with a higher incidence in human immunodeficiency virus positive (HIV+) patients. Most are high-grade B-cell non-Hodgkin lymphomas (NHL) often associated with Epstein-Barr virus (EBV). Anorectal Hodgkin lymphoma (HL) is rarer, accounting for 20% of gastrointestinal HL in HIV+ individuals. In immunodeficiency virus negative (HIV-) patients it is uncommon and it has been reported particularly in the context of inflammatory bowel disease (IBD). Primary HL of the anus has never been previously described. We report the first case of anal HL in a HIV- patient without IBD, highlighting the importance of differential diagnosis with EBV-induced lymphoproliferative disease (EBV-LPDs). Materials and methods. a 83-year-old man presented to the Surgery Unit of Valdichiana Hospital complaining of tenesmus and mucous bloody diarrhea, that lasted for 1 month. Digital rectal examination revealed a friable, hemorrhagic lesion located in the anal canal. The rest of the physical examination was unremarkable. No superficial lymphadenopathy or hepatomegaly were identified. A rectoscopy showed an ulcerative lesion of the anal canal. The ulcer extended through the entire anal wall, the margins were protruding. A whole body computed tomography (CT) scan showed an ill-defined tumor, 40x15 mm, extending through the entire right wall of the anal canal. Mild (10 mm) regional lymphadenopathy was also identified. No periaortic lymphadenopathy or lesions in the liver, spleen and lungs were detected. A complete blood count was normal. The clinical differential diagnosis included: localized anal carcinoma, anal isolated ulcer, Crohn disease. Tissue samples were taken; formalin-fixed, paraffin-embedded tissue blocks were cut and stained for hematoxylin and eosin. The following antibodies were checked: CD45, CD20, CD3, CD30, CD15, OCT-2, BOB-1, LMP-1, Cytomegalovirus (CMV). In situ hybridization analysis for EBV small-encoded RNA (EBER) was also performed as well as Polymerase chain reaction (PCR) for Human Papillomavirus (HPV). Results. the surgical specimens consisted of 5 friable brownish fragments ranging between 10 and 40 mm in maximum diameter. Histologically, all the fragments showed similar morphologic features: not well-circumscribed ulcerated mucosal lesions were associated to a polymorphous infiltrate with a mixture of lymphocytes, plasma cells, histiocytes, immunoblasts and scattered eosinophils. Reed-Sternberg (RS) and Hodgkin cells (HC) were present in variable number. The adjacent squamous epithelium showed reactive nuclear atypia. The neoplastic cells were positive for CD30 and CD15 with a paranuclear and membranous pattern of staining, and for LMP-1; they were negative for CD45, CD20, CD3, Oct-2, Bob-1. Immunohistochemistry for CMV was CONGRESSO aNNualE di aNatOmia patOlOGiCa SiapEC – iap • fiRENzE, 25-<strong>27</strong> OttOBRE <strong>2012</strong> negative. In situ hybridization analysis for EBER was positive whereas PCR for HPV was negative. The histological, immunohistochemical and in situ hybridization findings supported the diagnosis of classical HL. A post-diagnosis 2-fluoro-2-deoxy-Dglucose (FDG)-PET/CT showed an area of abnormally high FDG uptake in the anal canal and in the left inguinal lymph nodes. The patient received pelvic irradiation with 40 Gy. At present (6 months after the initial diagnosis) the patient is alive without any clinical, endoscopic and radiologic signs of local relapse or active HL. Conclusions. This is the first case of an isolated anal localization of HL in a HIV- patient. In our review of the literature, we have identified only two cases of ano-rectal HL but no cases exclusively located in the anus. In both cases the patients were male of 33-year old, HIV+. The lack of complete clinical, and immunohistochemical data as well as EBV status in one of the cases calls into question the diagnosis of primary HL. Diagnosis of extranodal HL can be challenging because of the polymorphic nature of the cellular infiltrate, the paucity of the malignant cells and the fact that normal anorectal mucosa is devoid of lymphoid tissue. However, lymphoid infiltration can be induced in the context of chronic inflammation or immune deficiency. Differential diagnosis with atypical lymphoid hyperplasia, particularly associated with virus, as such as EBV, may be difficult and a combination of clinical, morphologic and immunophenotypic parameters is useful in reaching the correct diagnosis. EBV-LPDs are lymphoid proliferations that arise as a result of immunodeficiency due to a primary immunodeficiency or immunoregulatory disorder (i.e. HIV infection, posttransplant setting, iatrogenic cause such as methotrexate and TNF-ɑ antagonists and ageing). In physiologic circumstances the inherent propensity of EBV to induce B-cell proliferation is counterbalanced by complex immunologic interactions that maintain the overall number of EBV-infected B cells in the body at a very low level. Immunosuppression affects various aspects of immune homeostasis and surveillance, thus permitting the emergence of EBV-induced EBV-LPDs. Specifically, in the elderly the T-cell response seems most profoundly affected, with the accumulation of clonal CD8 positive T-cells with mature, memory cell phenotypes but diminished functionality, owing to a markedly restricted epitope specific repertoire and a 100-fold decrease of T-cell specificities. In EBV-LPDs the lesion are superficial and well-demarcated with a rim of small lymphocytes, and plasmacytoid apoptotic cells which are considered a hallmark of retained normal control mechanisms. Moreover, RS and H-like cells showed wide range in size; they are CD45 and Oct-2 positive, with variable expression of Bob-1 and CD15. On the contrary in HL, RS and HC, as in our case, are CD30 and CD15 positive and CD45 negative with lack of expression of Oct-2 and Bob-1. In summary, we report an unusual case of HL arising in the anus of 83-year-old man in which only the association between clinical, histological and radiologic findings led us to a correct diagnosis. The importance of distinguish HL from EBV-LPDs, particularly in organs not common affected by HL, is stressed and the need for caution when considering such a diagnosis in HIV- patients with no history of IBD is underlined. references Dojcinov SD, Venkataraman G, Raffeld M, et al. EBV positive mucocutaneous ulcer-a study of 26 cases associated with various sources of immunosuppression. Am J Surg Pathol 2010;34:405-17. Pagano L, Ratto C, Teofili L, et al. Isolated primary Hodgkin’s disease of rectum. Haematologica 2000;85:986-7. Valbuena JR, Gualco G, Espejo-Plascencia I, et al. Classical Hodgkin lymphoma arising in the rectum. Ann Diagn Pathol 2005;9:38-42. Vasiliu V, Suarez F, Canioni D, et al. Anorectal Epstein-Barr virus infection mimicking Hodgkin lymphoma in an immunocompetent man. Am J Surg Pathol 2010;34:1715-9.
COmuNiCaziONi ORali Solitary T-cell psuedolymphoma with monoclonal TCr rearrangement: is it a true pseudolymphoma or a T-cell lymphoma in early stage? G. Crisman1 , D. Signoretto2 , S. Bonin3 , G. Trevisan4 , P. Leocata1 1 Anatomia Patologica, Dipartimento di Scienze della Salute, Università dell’Aquila, L’Aquila, Italia; 2 U.C.O. Anatomia Patologica, Università di Trieste, Trieste, Italia; 3 Dip. Univ. Cl. di Scienze Mediche, Chirurgiche e della Salute, Università di Trieste, Trieste, Italia; 4 U.C.O. Dermatologia e Venereologia, Università di Trieste, Trieste, Italia Background. Cutaneous Pseudolymphoma refers to a group of skin disorders characterized by a benign lymphoprolipherative reaction that may simulate cutaneous malignant lymphomas clinically and/or histologically. Material and methods. We report on a case of a 43-year-old woman presented with a solitary, indolent, erythematous plaque on the right hand. Anamnestic data were unremarkable. In the clinical suspicion of a Pagetoid Reticulosis (PR), supported by the persistence of the lesion even after several localized treatment and the site of involvement, a punch biopsy has been performed. Results. Histological features revealed a band-like subepidermal infiltrate, mainly composed by lymphocytes, with minimal exocytosis of lymphocytes into the epidermis. Several plasma cells have been noticed as well. Immunohistochemistry for CD3, CD4, CD8, CD43, CD20 and CD5 showed a predominance of T-cells, but B-cells resulted consistently present. Several cells were also immunopositve for CD138, and kappa and lambda immunoglobulin, thus a consistent presence of plasma cells was noticed. An inconsistent expression of CD56, and few CD30+ cells have been also observed. Clonally rearranged T-cell receptor genes have been detected twice. Conclusions. Even though the clinical presentation, the histopathological features and the clonal rearrangement of the TCR genes were suggestive for PR, the presence of a consistent Fig. 1. Clinical presentation of the patient. Fig. 2 A and B. histological features revealed a mixed inflammatory infiltrate involving the upper dermis with few foci of epidermotropism (H&E, 10x and 20x magnification). Fig. 3 A and B. immunoistochemical stains: strong positivity for Cd3 (fig. 3a. 4x magnification) and Cd8 (fig. 3B, 10x magnification). Fig. 4. monoclonal rearragement of the tCR genes. 269 number of plasma cells in the context of a such mixed infiltrate suggested a diagnosis of solitary T-cell pseudolymphoma. This entity represents one of the most challenging diagnosis among cutaneous lymphomas. Due to its unclear differential diagnostic criteria and to its variable clinicopathological features, many overlaps with cutaneous small/medium pleomorphic T-cell lymphoma have been reported so far, thus generating a confusing literature on this topic. Several cases of solitary T-cell pseudolymphoma with a monoclonal rearrangement of the TCR genes have been reported and it is still unclear if they represent wholly benign lymphoid proliferations or variant of cutaneous T-cell lymphoma with a very favorable course. Anyhow, surgical excision is the gold standard treatment in those cases and recurrence are uncommon. The authors would like to underline the importance of the clinicopathological and biomolecular correlation in the aim to achieve the correct diagnosis and avoid overtreatment. references 1 Hodak E, Phenig E, Amichai B, et al. Unilesional mycosis fungoides: a study of seven cases. Dermatology 2000;201:300-6. 2 van der Putte SC, Toonstra J, Felten PC, et al. Solitary nonepidermotropic T cell pseudolymphoma of the skin. J Am Acad Dermatol 1986;14:444-53. 3 Beltraminelli H, Leinweber B, Kerl H, et al. Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma: a cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance? A study of 136 cases. Am J Dermatopathol 2009;31:317-22. 4 Martin SJ, Cohen PR, Cho-Vega JH, et al. CD8+ Pagetoid Reticulosis Presenting as a Solitary Foot Plaque in a Young Woman. J Clin Aesthet Dermatol 2010;3:46-9. 5 Cho-Vega JH, Tschen JA, Duvic M, et al. Early-stage mycosis fungoides variants: case-based review. Ann Diagn Pathol 2010;14:369-85. 6 Matsuzaki Y, Kimura K, Nakano H, et al. Localized pagetoid reticulosis (Woringer-Kolopp disease) in early childhood. J Am Acad Dermatol 2009;61:120-3.
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202 gli attuali approcci multidimen
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204 of Florence, University of Pisa
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212 Key words: EGC, Prognosis, Trea
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214 Tab. VI. univariate analysis: 5
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216 the surgeons perform a pancreat
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COmuNiCaziONi ORali PLAP in normal
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COmuNiCaziONi ORali or identificati
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COmuNiCaziONi ORali references 1 Ro
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COmuNiCaziONi ORali Tab. II. TCGC-S
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COmuNiCaziONi ORali plastic disease
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COmuNiCaziONi ORali antibodies prio
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COmuNiCaziONi ORali Fig. 3. fibroma
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COmuNiCaziONi ORali microarray anal
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COmuNiCaziONi ORali True 3q chromos
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COmuNiCaziONi ORali To our knowledg
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COmuNiCaziONi ORali TESTA COLLO Lym
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COmuNiCaziONi ORali references 1 Sa
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COmuNiCaziONi ORali of the paranasa
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Pathologica 2012;104:359-420 POSTEr
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PoStER references 1 Gerber DE, Minn
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PoStER In our case the endofibrotic
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PoStER Fig. 1. Kaplan-meier surviva
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PoStER believed the use of atypical
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PoStER references 1 Goepel JR. Beni
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PoStER MALT lymphoma of the rectum:
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PoStER 3 Ciulla A, Castronovo G, To
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PoStER for protein expression of PA
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PoStER Grossly, an irregular villou
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PoStER Expression of ror-1 in pancr
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PoStER (see Fig. 1) placental site
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PoStER Tissue were fixed in 10% for
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PoStER invaded focally adjacent tun
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PoStER bination chemotherapy the pr
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PoStER 21 to 55 years in age, the l
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PoStER 9 Klein KA, Stephens DH, Wel
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Pathologica 2012;104:421-423 InDICE
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