Open partial horizontal laryngectomies: a proposal for classification by the working committee on nomenclature of the European Laryngological Society.

We present herein the proposal of the European Laryngological Society working committee on nomenclature for a systematic classification of open partial horizontal laryngectomies (OPHL). This is based on the cranio-caudal extent of laryngeal structures resected, instead of a number of different and heterogeneous variables present in existing nomenclatures, usually referring to eponyms, types of pexy, or inferior limit of resection. According to the proposed classification system, we have defined three types of OPHLs: Type I (formerly defined horizontal supraglottic laryngectomy), Type II (previously called supracricoid laryngectomy), and Type III (also named supratracheal laryngectomy). Use of suffixes "a" and "b" in Type II and III OPHLs reflects sparing or not of the suprahyoid epiglottis. Various extensions to one arytenoid, base of tongue, piriform sinus, and crico-arytenoid unit are indicated by abbreviations (ARY, BOT, PIR, and CAU, respectively). Our proposal is not intended to give a comprehensive algorithm of application of different OPHLs to specific clinical situations, but to serve as the basis for obtaining a common language among the head and neck surgical community. We therefore intend to present this classification system as a simple and intuitive teaching instrument, and a tool to be able to compare surgical series with each other and with non-surgical data.

Subtotal laryngectomy: outcomes of 469 patients and proposal of a comprehensive and simplified classification of surgical procedures.

Long-term oncological and functional results from a retrospective study on 469 patients over a 10-year period of subtotal laryngectomies (SL), 399 supracricoid partial laryngectomies (SCL) and 70 supratracheal partial laryngectomies (STL) are presented. The mean follow-up time was 97 months (range 60­165 months). Acute complications, types and rates of late sequelae, functional results, 2-year post-operative scores of laryngeal function and quality of life are reported. The observed long-term results were: SCL, 5-year overall and disease-free survival: 95.6, and 90.9%, respectively; 2-year post-operative laryngeal function preservation: 95.7%; STL, 5-year overall and disease-free survival: 80 and 72.9%, respectively; 2-year post-operative laryngeal function preservation: 80%. The performance status scale for laryngeal function preservation showed very high 2-year scores, with no significant differences depending on the type and extent of surgery. The adopted type of function-sparing surgery provided overall and disease-free survival rates that were somewhat better than those reported in studies based on organ-sparing protocols with chemoradiotherapy. The rate of total laryngectomy of completion in this series was 4.4%. A new classification of the current horizontal partial laryngectomies is also proposed, namely "Horizontal Laryngectomy System" (HOLS), based on the extent of surgical removal of laryngeal structures.

Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg-type and other: an update on morphology and treatment.

Primary cutaneous B-cell lymphoma (PCBCL) is an heterogeneous group of lymphoproliferative disorders, which account for 25-30% of all primary cutaneous lymphoma and include three main histotypes: 1) primary cutaneous marginal zone B-cell lymphoma (PCMZL); 2) primary cutaneous follicular center cell lymphoma (PCFCL); 3) primary cutaneous diffuse large B-cell lymphoma (DLBCL), leg type (PCDLBCL-LT). PCMZL and PCFCL are indolent lymphomas, with an excellent prognosis despite an high rate of cutaneous recurrences; in contrast, PCDLBCL-LT is clinically more aggressive and usually requires to be treated with multi-agent chemotherapy and anti-CD20 monoclonal antibodies. PCDLBCL-LT histologically consists of large round cells (centroblasts and immunoblasts), is characterized by strong bcl-2 expression, in the absence of t(14;18) translocation, and resembles the activated B-cell type of nodal DLBCL. Recently, the term primary cutaneous DLBCL-other (PCDLBCL-O) has been proposed to include diffuse lymphomas composed of large transformed B-cells that lack the typical features of PCDLBCL-LT and do not conform to the definition of PCFCL. Some clinical studies suggested that such cases have an indolent clinical course and may be treated in a conservative manner; however, data regarding the actual prognosis and clinical behaviour of these peculiar cases are still too limited. The spectrum of primary cutaneous DLBCL also encompasses some rare morphological variants, such as anaplastic or plasmablastic subtypes and T-cell rich B-cell lymphoma, and some recently described, exceedingly rare DLBCL subtypes, such as intravascular large B-cell lymphoma and EBV-associated large B-cell lymphoma of the elderly, which often present in the skin.

Subcutaneous 'lipoma-like' B-cell lymphoma associated with HCV infection: a new presentation of primary extranodal marginal zone B-cell lymphoma of MALT.

BACKGROUND: Hepatitis C virus (HCV) infection has been linked to lymphoproliferative disorders. Marginal zone B-cell lymphoma (MZL) represents one of the most frequent lymphoma subtypes associated with HCV infection. We describe an unusual subset of HCV-associated MZL characterized by subcutaneous presentation. MATERIALS AND METHODS: A series of 12 HCV-positive patients presenting with subcutaneous nodules that revealed lymphoma infiltration at biopsy. Molecular analysis of immunoglobulin heavy chain (IGH) gene rearrangement and FISH investigations for t(11;18)(q21;q21) and t(14;18)(q32;q21) were carried out in nine patients. RESULTS: The 12 patients (median age 69.5 years), all with positive HCV serology, presented with single or multiple subcutaneous nodules resembling lipomas. Histologically the lesions showed lymphoid infiltrates, consistent with extranodal MZL of mucosa-associated lymphoid tissue (MALT). Functional IGH gene rearrangements were identified in nine tested patients, with somatic mutations in 82%, indicating a histogenesis from germinal center-experienced B cells. The t(11;18) was found in two of nine cases. Staging did not show any other lymphoma localization. In two patients, a response was achieved with antiviral treatment. Extracutaneous spread to MALT sites occurred in a case. CONCLUSIONS: Our observations expand the spectrum of HCV-associated lymphomas to include a subset of extranodal MZL characterized by a novel primary 'lipoma-like' subcutaneous presentation and indolent clinical course.

Bone marrow histology in marginal zone B-cell lymphomas: correlation with clinical parameters and flow cytometry in 120 patients.

BACKGROUND: Among marginal zone lymphomas (MZLs), bone marrow (BM) involvement features are well established in the splenic marginal zone lymphoma (SMZL); few data are available for extranodal marginal zone lymphoma (EMZL) and nodal marginal zone lymphoma (NMZL). PATIENTS AND METHODS: Incidence and patterns of histologic BM involvement are studied in 120 MZL patients (48 SMZL, 59 EMZL, 13 NMZL) at onset and during follow-up; relationships between clinical features, BM histology and flow cytometry (FC) are analyzed. RESULTS: At diagnosis, BM involvement occurs in 90% SMZL, 22% EMZL and 54% NMZL (P<0.0001); at reevaluation, incidence raises to 96% in SMZL and 34% in EMZL. Concordance between histology and FC is found in 87% of cases; most discordant cases have positive histology but negative FC. SMZL and EMZL show a nodular BM infiltration; the interstitial pattern is frequent in NMZL (P<0.0001); sinusoidal localization is typical of SMZL, frequent in NMZL and occasional in EMZL (P=0.0001). Stage, leukemic disease, B symptoms, more than one extranodal involved site, splenomegaly, elevated beta2-microglobulin, serum monoclonal component, International Prognostic Index (IPI) and age-adjusted IPI are directly related to BM infiltration. CONCLUSIONS: The different prevalence of BM involvement in MZL subtypes reflects their heterogeneous dissemination modalities; histology seems more sensible than FC to detect BM infiltration; development of BM involvement during follow-up is typical of EMZL.

Correction to: A practical algorithmic approach to mature aggressive B cell lymphoma diagnosis in the double/triple hit era: selecting cases, matching clinical benefit.

The first and family names of the authors were interchanged and are now presented correctly. The original article has been corrected.

A practical algorithmic approach to mature aggressive B cell lymphoma diagnosis in the double/triple hit era: selecting cases, matching clinical benefit : A position paper from the Italian Group of Haematopathology (G.I.E.).

An accurate diagnosis of clinically distinct subgroups of aggressive mature B cell lymphomas is crucial for the choice of proper treatment. Presently, precise recognition of these disorders relies on the combination of morphological, immunophenotypical, and cytogenetic/molecular features. The diagnostic workup in such situations implies the application of costly and time-consuming analyses, which are not always required, since an intensified treatment option is reasonably reserved to fit patients. The Italian Group of Haematopathology proposes herein a practical algorithm for the diagnosis of aggressive mature B cell lymphomas based on a stepwise approach, aimed to select cases deserving molecular analysis, in order to optimize time and resources still assuring the optimal management for any patient.

[Three-quarter laryngectomy. Our experience: indications, surgical technique, results]. / La laringectomia tre-quarti. Nostra esperienza: indicazioni, tecnica chirurgica, risultati.

Three-quarter laryngectomy is regarded as a supraglottic laryngectomy which has been extended on one side to the glottic plane. Indications are those for supraglottic laryngectomy at the vestibular level and of cordectomy extended to the arytenoid at the glottic level (undamaged lower paraglottic space, preserved motility). Surgical technique includes total removal of the laryngeal vestibule together with the hyoid bone, pre-epiglottic space and one supracricoid hemilarynx (vocal cord, arytenoid, medial wall of the entrance of the pyriform fossa) through an internal, subperichondrial approach. Reconstruction of a new cord on the hemilaryngectomy side is through a triangular strip of the exterior thyroid perichondrium. From 1975 to 1989 44 patients underwent a three-quarter laryngectomy in the authors' department. Functional results were positive in all cases. The oncological results were as follows: 6 recurrences (3 local, 2 locoregional lymph node recurrences and 1 distant metastasis) and 4 primary secondary tumors. 9 patients expired (6 of T, 2 of secondary tumor, 1 of other causes). The actuarial 5-year survival rate for the tumor was 79%.

Plastic tracheostomal-widening procedure: the "petal" technique.

Severe tracheostomal stenosis after total laryngectomy may require the permanent use of a tracheostomy tube which undoubtedly represents a personal and social handicap (cosmetic impairment, accumulation of sputum, noisy breathing, cough stimulation, tube management). In patients with voice prostheses, this is a major obstacle to phonation and device maintenance. Stenosis is so pronounced, in some cases, as to cause dyspnoea. The main causes of stenosis are perichondritis of the upper tracheal rings or, more frequently, a defect in the tracheostoma preparation. All such cases require surgical revision of the tracheostomal diameter, for which numerous procedures have been described in the literature. The "petal" technique, adopted by the Otorhinolaryngology O.U. in Vittorio Veneto, for four years, has been used in 59 patients. The technique is described and results of retrospective study, to assess outcome, are outlined. In 40 cases, outcome was immediately satisfactory, while recurrence of stenosis was observed in 19 patients, 9 of whom preferred to accept tube dependence while 10 were reoperated, with permanent successful results in 6 cases. In our opinion, since this is an easy surgical procedure to perform and, in the majority of cases, is carried out under local anaesthesia with good patient compliance, absence of complications and good long-term results, this should be considered the method of choice for surgical widening of permanent tracheostomas.

[Treatment of laryngeal adenoid cystic carcinoma]. / Sul trattamento del carcinoma adenoido-cistico laringeo.

Laryngeal localization (nearly always hypoglottic) of adenoid cystic carcinoma is quite rare (0.6% of all malignant laryngeal tumors). In most cases the local development of the neoplasm is slow, of an infiltrating and highly invasive nature (affecting the cartilage, thyroid gland, esophagus). On the other hand in some cases, particularly in the laryngeal-tracheal localization, the tumors take on an exophytic, sessile, polypoid aspect. Although the evolution is quite slow prognosis is poor due to the latent spreading of distant metastasis (average survival is 8 years). Prognosis is further worsened by delay in diagnosis due to the poor, aspecific initial symptoms. C.A.T. and N.M.R. have made a marked contribution to improved diagnostic definition, especially in terms of tumor extension, which is of great importance in therapeutic staging. Given the poor results obtained with radiotherapy and chemotherapy the only possible treatment is surgery which, in most cases, must be quite radical. Only in those forms which are still localized (with endoluminal exophytic development) is it possible to perform conservative surgery. Two paradigmatic cases are reported here indicating two different tumor extensions: in the first case total laryngectomy was performed and extended to the thyroid gland and the upper portion of the trachea; in the second, a partial glottic-subglottic laryngectomy was performed with functional restoration of laryngeal function. Conservative surgery is only possible if the hypoglottic neoformation is limited to the larynx, if it is mainly exophytic in nature and if histological examination reveals a low degree of malignancy (a high degree of cell differentiation). Since this type of carcinoma tends to give rise to latent distant metastases prognosis is, in all cases, poor no matter what surgical technique is employed for the primary tumor. Therefore it is imperative that a conservative laryngectomy be performed in all cases where it proves oncologically possible and particular attention should be paid to the quality of the patient's post-surgery life.

El Sistema de Salud de la ciudad de Buenos Aires en el siglo xix en relación a las enfermedades de transmisión sexual / THE HEALTH SYSTEM IN THE CITY OF BUENOS AIRES DURING THE XIXth CENTURY, RELATED TO THE DISEASES OF SEXUAL TRANSMISSION

The health system of the City of Buenos Aires during the XIX century, related to the diseases of sexual transmission, is presented. The "General Hospital for Acute Diseases Dr. Juan A. Fernandez" of universitary character with a well gained prestige in his influencial zone, accumulates the highest technology in this programmatic area, and is today related with the great epidemic disease of sexual transmission from the XXth. Century: AIDS. Not always is well known the fact that, more than a century ago, this Institution was created to confront also to an epidemic of sexual transmission: syphilis. Is then purpose of this revision to introduce to the reader in the behavioral situation at the time of its foundation, the sanitary problems that conducted to its creation and the history of his former years, all of that related to the evolution of the sexually transmitted diseases at the endings of the XIX century.

Dysregulation of global microRNA expression in splenic marginal zone lymphoma and influence of chronic hepatitis C virus infection.

The precise molecular pathogenesis of splenic marginal zone lymphoma (SMZL) is still unknown. Clinical and epidemiological data suggest that chronic hepatitis C virus (HCV) infection may have an etiological role in a subset of cases.We performed a large-scale microRNA (miRNA) expression profiling analysis of 381 miRNAs by quantitative reverse transcription PCR (Q-RT-PCR) of 26 microdissected splenic tissue samples (7 HCV(+) SMZL; 8 HCV(-) SMZL and 11 non-neoplastic splenic controls). Single assay Q-RT-PCR and miRNA in situ hybridization (miRNA-ISH) were used to confirm the results in an independent cohort. Unsupervised hierarchical clustering of miRNA expression profiles demonstrated a distinct signature of SMZL compared with the normal splenic marginal zone. Supervised analysis revealed differentially expressed miRNAs, including miRNAs with previously recognized tumor suppressive or oncogenic potential. Five miRNAs were found significantly overexpressed in SMZL, including miR-21, miR-155 and miR-146a, whereas seven miRNAs showed significantly reduced expression, including miR-139, miR-345, miR-125a and miR-126. Furthermore, we identified miR-26b, a miRNA known to have tumor suppressive properties, as significantly downregulated in SMZL arising in HCV-positive patients (P=0.0016). In conclusion, there is a characteristic dysregulation of miRNA expression in SMZL with a possible implication in its molecular tumorigenesis.

Ketanserin in parenteral treatment of acute essential hypertension: a dose-response curve.

This study assesses the efficacy, safety, and dose-response curve of ketanserin, according to parenteral routes of administration, in the treatment of acute severe essential hypertension. We studied 50 patients with supine diastolic blood pressure (SDBP) phase V greater than or equal to 110 mm Hg after 2 h of continuous BP monitoring. Ketanserin was administered as follows: 5 mg i.m. (Group I: SDBP X = 115.7 mm Hg); 10 mg i.m. (Group II: SDBP X = 119.0 mm Hg); 5 mg i.v. (Group III: SDBP X = 123.9 mm Hg); and 10 mg i.v. (Group IV: SDBP X = 130.8 mm Hg), according to initial severity of symptoms and BP. All patients were monitored, and most of them were on antihypertensive treatment. [Total group mean age: 57.9 years (range: 38-78 years)]. There were 35 men and 15 women. Systolic BP fell progressively, from a mean of 177.9 mm Hg (basal) to 156.4 mm Hg at 60 min postadministration (Group I), p less than 0.01; from 188 to 164 mm Hg (Group II), p less than 0.05; from 192.1 to 157.9 mm Hg (Group III), p less than 0.01; and from 207.5 to 168.3 mm Hg (Group IV), p less than 0.01). SDBP decreased from a basal mean value of 115.7 to 92.1 mm Hg at 60 min (Group I), p less than 0.01; from 119.0 to 98 mm Hg (Group II), p less than 0.05; from 123.9 to 101.6 mm Hg (Group III), p less than 0.01; and from 130.8 to 105.8 mm Hg (Group IV), p less than 0.01. Heart rate decreased slightly in all groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Nijmegen breakage syndrome-associated T-cell-rich B-cell lymphoma: case report.

In 1981 Weemaes et al. first described the Nijmegen breakage syndrome (NBS), a rare autosomal recessive disorder characterized by stunted growth, microcephaly, immunodeficiency, spontaneous chromosome instability, and a peculiar predisposition to cancer development. Most NBS-related malignancies are lymphomas, but their pathologic features have rarely been specified. We report here the case of a northern Italian 8-year-old child who, 2 years after the diagnosis of NBS, developed a diffuse large B-cell lymphoma (T cell-rich B-cell lymphoma variant). The histological and immunobiological features of the lymphoma population are analyzed and discussed in detail.

Lymph node reticulum cell neoplasm with progression into cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma: a case study.

AIMS: To detail on sequential biopsies the morphological and immunohistochemical features of a case of primary lymph nodal fibroblastic reticulum cell (FBRC) tumour which progressed into a clinically aggressive cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma. METHODS AND RESULTS: A 70-year-old female underwent surgical excision of an enlarged submandibular lymph node. The nodal architecture was effaced by a neoplastic proliferation of medium to large cells, round to oval to spindle in shape, growing in a storiform pattern. The tumour stained for vimentin, CD68, factor XIIIa, alpha1-antitrypsin, fascin and actin. Dendritic and endothelial cell markers were negative. A diagnosis of FBRC tumour was made by combining pathological and clinical data. The patient received no therapy but 5 months later the tumour relapsed, exhibiting a deceptively pleomorphic cytology, phenotypic changes (strong cytokeratin positivity), intense p53 expression and aggressive clinical course with fatal outcome. In-situ hybridization for Epstein-Barr virus was negative. CONCLUSIONS: We speculate that the morphological changes and p53 expression of the relapsing neoplasm might reflect tumour cell dedifferentiation, in keeping with the aggressive clinical course. The intense p53 expression suggests that this oncoprotein might also play a role in reticulum cell tumorigenesis.

Análisis epidemiológico y gestión sanitaria de la epidemia de gripe A H1N1: experiencia en la ciudad de Buenos Aires (invierno de 2009) / Epidemiological analysis and sanitary management of the influenza a H1N1: experience in the city of Buenos Aires (outbreak in winter 2009)

Las pandemias de influenza son eventos impredecibles pero recurrentes, que suponen consecuencias gravosas para las sociedades en todo el mundo. Desde el siglo XV y XVI, con el famoso sudor anglicus, se han descrito pandemias de influenza con intervalos más o menos regulares, entre 10 y 50 años, cuya gravedad y repercusiones han sido variables destacándose en el siglo XX la "gripe española" de 1918/1919, probablemente una de las mayores y más mortíferas pandemias de la historia humana, la influenza asiática entre 1957/1958 y la de Hong Kong entre 1968/1969. En 2003 surgió preocupación mundial ante una posible pandemia de influenza aviar (H5N1) que era sindicada como la gran favorita para un próximo evento de este tipo; no obstante nunca llegó a sortear la imposibilidd de propagarse de humano a humano. En abril de 2009, la OMS (WHO) comenzó a recibir reportes de personas con un nuevo tipo de virus de influenza A (H1N1) en México y EE.UU. La rápida diseminación internacional ulterior llevó a la propia OMS a declarar el 11 de junio de 2009 la primera pandemia de influenza en 41 años. En Argentina y luego del llamado inicial de alerta de Ministerio de Salud de la Nación, el Gobierno de la Ciudad de Buenos Aires inició procesos destinados al reporte, detección y vigilancia epidemiológica de los casos locales. A principios del mes de mayo se difundieron las noticias de los primeros casos de Gripe A confirmados en el país y el 30 de junio se decretó la emergencia sanitaria en la ciudad profundizándose un proceso de análisis sobre las medidas a tomar. Las acciones específicas tomadas por el GCBA y su MSAL pueden agruparse en acciones sobre el sistema de salud y sus efectores y acciones de estrategia comunicacional. Se concluye de este episodio pandémico que la Gripe A desplazó a la gripe estacional siendo la circulación viral en adultos mayormente virus A H1N1...

Influenza pandemics are unpredictable but recurrent events, involving serious consequences for societies worldwide. From the fifteenth and sixteenth century, with the famous anglicus sweat, there have been described pandemics of influenza within more or less regular intervals, between 10 and 50 years, whose severity and impact has been variale, emphasizing in the twentieth century the "Spanish flu" of 1918/1919, probably one of the largest and most dedly pandemics in human history, between 1957/1958 the Asian flu and the Hong Kong flu from 1968 to 1969. In 2003, concern arose about a possible global pandemic of avian influenza (H5N1), which was syndicated as the most probable cause for an upcoming event of this king, yet it never get around the inability to spread from human to human. In April 2009, WHO began to receive reports of Mexico and the U.S. The rapid internatinal spread further les WHO to declare on June 11 th 2009 the first influenza pandemic in 41 years. In Argentina and after the initial warning call of the Ministry of Health of the Nation, the Government of the City of Buenos Aires initiated processes for the reporting, detection and epidemilogical surveillance of local cases. In early May, the news spread of the first confirmed cases of influenza A in the country and on June 30 was declared a health emergency in the city getting stronger a process of discussion of measures to take. The specific actions taken by the GCBA and The Ministry of Health can be grouped into action on the health system and its effectors, and communications strategies and activities. We conclude from this episode of pandemic Influenza that the A virus H1N1 has replace the seasonal flu virus, because influenza virus circulation among adults was mostly H1N1 virus, that in spite of the fact of being getting trough a pandemic episode, the overall death rate was lower of that of the seasonal flu...