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Parapharyngeal space tumors (PPS) are rare. They represent less than 1% of all head and neck tumors. They are of various histologies. Pleomorphic adenomas originating from the parotid (prestylian parapharyngeal space) are the most frequent. We report the case of a 50-year-old patient treated for a pleomorphic adenoma (PA) of PPS. His initial complaints were apnea and dysphagia. The correct diagnosis was preoperatively suspected by magnetic resonance imaging (MRI). The surgery was carried out using two approaches: transoral and cervical transparotid approaches. Definitive histology was consistent with encapsulated pleomorphic adenoma. In the present work, we reviewed clinical, radiological, and histological features of parapharyngeal space tumors. We tried to summarize the common surgical approaches. The chosen approach is, in fact, scheduled taking into account several parameters including tumor volume and purpose of the surgery.

Because nasopharyngeal carcinoma (NPC) has a close association with Epstein-Barr virus (EBV), measuring serum EBV DNA and anti-EBV serum marker concentrations could be a feasible method for NPC diagnosis, monitoring and probably screening especially in a community at risk. The aim of this study was to determine the EBV pattern in sporadic NPC and in high risk NPC Tunisian families in order to evaluate their risk factors and help for NPC screening. The rates of anti-EBV antibodies and EBV DNA were determined in the serum of 47 healthy members randomly selected from 23 NPC multiplex families with two or more affected members, 93 healthy Tunisian community controls chosen with the same age, sex and geographic origin as unaffected individuals and 66 EBV positive sporadic NPC patients whose serum was available before and after treatment. Unexpectedly, significant lower concentrations of anti-EA (Early Antigen) IgG and anti-VCA (Viral Capsid Antigen) IgG were found in unaffected members from NPC families than in healthy controls while viral loads were negative in all the tested sera. For sporadic NPC patients, anti-EA IgG and anti-VCA IgA concentrations were significantly higher than in healthy controls and these rates decreased after treatment. The level of EBV DNA load varied according to the condition of the tumour. This study suggests that in the Tunisian NPC families, screening for malignancy is based on serum concentrations but not on EBV DNA load while in the sporadic NPC group, serologic markers and EBV DNA load are complementary for diagnosis and follow-up.

Resume Objectif Etudier les aspects evolutifs et therapeutiques du plasmocytome extramedullaire de la tete et du cou. Patients et methodes Cinq cas de plasmocytome solitaire extramedullaire ont ete colliges dans le service de radiotherapie de l’institut Salah-Azaiz, entre 1997 et 2008. Il s’agissait de trois femmes et deux hommes âges de 36 a 77 ans. Le siege du plasmocytome etait la fosse nasale dans trois cas, le sinus ethmoidal dans un cas et une adenopathie sous-mandibulaire dans un autre cas. Le bilan n’a pas retrouve d’infiltration myelomateuse dans tous les cas. Le site tumoral initial a ete irradie a une dose de 40 a 45 Gy apres une exerese chirurgicale dans quatre cas et une biopsie dans un cas. Parmi les cinq patients, deux n’ont ete irradies qu’apres la recidive. Resultats Quatre patients etaient en situation de remission complete avec un recul de 12, 36, 52 et 72 mois. Une transformation myelomateuse est survenue avec un delai de huit ans. Conclusion La radiotherapie fait partie du traitement de reference des plasmocytomes extramedullaires. Le controle local semble dependre de la dose de radiotherapie, qui doit etre au minimum de 45 Gy. La possibilite d’une transformation myelomateuse a long terme justifie la realisation d’un bilan initial complet, la determination des facteurs predictifs et une surveillance reguliere.

Objective The aim of this review is to study natural history, presentation, treatment and prognosis of head and neck liposarcomas. Methods We carry a retrospective study about 15 patients diagnosed and treated in our department between 1969 and 2001. Therapeutic modalities included surgery, radiotherapy and chemotherapy. Results Tumors were mostly localized at the neck (4 cases) and the scalp (3 cases). Cervical lymphadenopathy was found in 1 case. Chest X-ray found showed metastases in 2 cases. Histological types of liposarcomas were the following: myxoid (5 cases), well differentiated (4 cases), pleomorphic (4 cases), round cell (1 case) and dedifferentiated (1 case). Twelve patients underwent surgery. Complete excision was performed in 7 cases. In the other 5 cases, exeresis was incomplete and 4 of them received postoperative radiotherapy. Three patients were not operated because of vascular involvement (1 case) or metastasis (2 cases), and they all received radiotherapy alone. After initial treatment, complete remission was achieved in 7 cases (46.7%). A recurrence occurred in 8 cases (53.3%). Two of these patients were reoperated and another one received chemotherapy. In the other 5 cases, the tumor was uncontrollable and no additional treatment has been proposed. Five-year survival rate was 87% for patients who underwent surgery alone, 75% for those who underwent surgery and postoperative radiotherapy, and 0% for those treated by radiotherapy alone. Conclusion The mainstay of treatment of head and neck liposarcomas is surgical excision and the prognosis is largely determined by the histological grade and the clinical stage.

Resume Objectifs. – Le carcinome epidermoide hypopharynge est associe a l’un des pronostics les plus defavorables des cancers de la sphere ORL. L’objectif de ce travail etait d’analyser ses modalites therapeutiques a l’institut Salah-Azaiz (Tunis) et de comparer leurs resultats. Patients et methodes. – Cette etude retrospective porte sur 271 carcinomes epidermoides de l’hypopharynx, colliges au service de chirurgie carcinologique cervicofaciale et ORL de l’institut Salah-Azaiz sur une periode de 25 ans (1977 a 2002). L’âge moyen des patients etait de 56 ans ; le sex-ratio etait en moyenne de 1,2 (homme/femme). L’indication d’un traitement a visee curative a ete initialement retenue pour 149 (55 %) patients, qui ont ete les seuls retenus pour l’analyse des resultats. Resultats. – Nous avons retenu l’indication d’un protocole incluant chirurgie et radiotherapie postoperatoire pour 26,2 % des patients. Le taux de mortalite postoperatoire etait de 5,1 % ; le taux de morbidite operatoire etait de 46,2 %. Pour 13,5 % des patients, la radiotherapie postoperatoire a ete definitivement interrompue en raison d’une alteration progressive de l’etat general en cours de traitement. Nous avons pose l’indication d’une radiotherapie exclusive pour 59,7 % des patients. L’âge moyen etait de 56 ans et le sex-ratio de 1,2. La telecobaltotherapie externe etait delivree en fractionnement et etalement classiques. La radiotherapie a du etre definitivement interrompue en cours de realisation dans 32,6 % des cas devant une aggravation de l’etat general ; le taux de morbidite liee a la radiotherapie etait de 33,3 %. Un protocole de conservation d’organe avec chimiotherapie neoadjuvante a ete propose a 21 patients (14,1 %). L’âge moyen etait de 53 ans (28 a 65 ans) et le sex-ratio (homme/femme) de 0,5. Le taux de survie globale etait de 25,5 % a un an, 18,1 % a deux ans, 11,4 % a trois ans et 7,4 % a cinq ans. Tous les patients a qui a ete proposee une chimiotherapie sont decedes en cours de traitement. Les taux de survie a deux et a cinq ans des patients traites par chirurgie et irradiation et par irradiation exclusive etaient respectivement de 21,5 et 12 %, et 18,3 et 10 %. La difference de survie entre ces deux populations therapeutiques n’etait pas significative (p = 0,08). Conclusion. – Tandis que la chimiotherapie neoadjuvante apparait dans notre serie comme grevee d’une lourde mortalite, l’association chirurgie–radiotherapie et la radiotherapie exclusive semblent d’une efficacite similaire dans le traitement du carcinome hypopharynge.

Resume Objectifs. – Le carcinome epidermoide hypopharynge est associe a un pronostic defavorable, auquel l’envahissement ganglionnaire cervical et les metastases a distance contribuent. L’objectif de cette etude etait d’evaluer la frequence, le mode et la signification pronostique de ses metastases regionales et a distance. Patients et methodes. – Les auteurs rapportent une etude retrospective portant sur 271 carcinomes epidermoides de l’hypopharynx, colliges au service de chirurgie carcinologique cervicofaciale et ORL de l’institut Salah-Azaiz de 1977 a 2002. La frequence et les caracteres histocliniques des metastases regionales et a distance ont ete evalues, ainsi que leur signification pronostique chez les 155 patients traites dans un but curatif. Resultats. – 39,1 % des cancers etaient classes N0, 24,4 % N1, 10,7 % N2 et 25,8 % N3 (UICC 2002). Les adenopathies cervicales etaient significativement plus frequentes pour les tumeurs classees T3T4 que pour les T1T2 (soit, pour les tumeurs des sinus piriformes, celles de la region retrocricoarytenoidienne, celles de la paroi posterieure et celles etendues de l’hypopharynx, respectivement : p = 0,001, p = 0,007, p = 0,047, et p = 0,0005). Trente-deux patients ont beneficie d’un evidement ganglionnaire cervical. Parmi ceux dont le cancer a ete classe N0, il a ete trouve 46,9 % d’atteintes ganglionnaire (N+) ; les frequences de la rupture capsulaire n’etaient pas significativement differentes pour les cancers classes N0N1 et pour ceux qui etaient N2N3 ( p = 0,11). Les taux de survie globale a deux et a cinq ans etaient respectivement de 32,5 et 20,5 % pour les patients dont la tumeur etait classee N0, 10,9 et 0 % si elle etait N3. Les differences de taux de survie a deux et a cinq ans entre les patients dont la tumeur etait N0N1 et ceux dont elle etait N2N3 etaient significatives ( p = 0,04). Un echec ganglionnaire cervical a ete constate chez 18,5 % des patients. Le site tumoral n’influencait pas significativement le taux d’echec ganglionnaire ( p = 0,98), non plus que le statut clinique N ( p = 0,34). La rupture capsulaire etait un facteur significatif de prediction d’un echec ganglionnaire ( p = 0,007). Le taux de dissemination metastatique a distance etait de 2,6 % au premier examen, et secondairement de 12,3 %. Les metastases a distance etaient significativement plus frequentes chez les patients dont le cancer etait initialement classe N2N3 que chez ceux dont il etait N0N1 ( p = 0,03), ainsi qu’en cas de rupture capsulaire ( p = 0,0009). Conclusion. – Le carcinome epidermoide de l’hypopharynx est caracterise par une grande lymphophilie, qui est liee a l’extension locale, et par la grande frequence des metastases ganglionnaires occultes. Le statut clinique ganglionnaire ne semble pas influencer la probabilite de survie ; cependant, la rupture capsulaire constitue un facteur de prediction d’un echec ganglionnaire cervical et de metastases a distance. Les metastases a distance sont frequentes, particulierement en cas de tumeur etendue aux plans local ou regional, et la probabilite de survie est dans ce cas nulle a un an.

Resume Objectif de l’etude. – Rapporter les aspects cliniques et les resultats therapeutiques dans une serie tunisienne de lymphomes primitifs nasosinusiens. Patients et methodes. – Notre etude retrospective a porte sur les cas de lymphomes primitifs nasosinusiens histologiquement confirmes, diagnostiques et traites a l’nstitut Salah Azaiz de 1975 a 1997. Le bilan initial a comporte : examen clinique general et ORL, des examens biologiques (numeration et formule sanguine, vitesse de sedimentation, dosage de la LDH), une echographie abdominale, une biopsie medullaire et une tomodensitometrie du massif facial depuis 1985. Nous avons procede a une relecture des lames histologiques. Le protocole etait base sur la radiotherapie avec ou sans chimiotherapie. Resultats. – Nous avons collige les dossiers de 22 hommes et trois femmes (sex-ratio = 7). L’âge moyen etait de 48 ans (extremes : 18–78 ans) et le delai moyen entre le premier symptome et la premiere consultation de quatre mois. Le symptome le plus frequent etait represente par l’obstruction nasale (65 %), plus rarement une epistaxis (40 %) ou une rhinorrhee (20 %). L’examen ORL a retrouve dans 20 % des cas une tumeur des fosses nasales, bourgeonnante et unilaterale dans 80 % des cas et associee a une adenopathie cervicale dans 25 %. Les lymphomes etaient de haut grade dans 60 % des cas et localises (stade IE) chez 60 % des patients. Tous les lymphomes de stade IE de bas grade ont ete traites par irradiation exclusive alors que l’association de radiotherapie et de chimiotherapie a ete utilisee pour les lymphomes de stade plus avance (huit patients). Le taux de survie actuarielle a cinq ans etait de 69 % pour toute la population avec un pronostic favorable pour les lymphomes de stade IE et ceux de bas grade de malignite. Conclusion. – La radiotherapie reste le traitement standard des lymphomes primitifs nasosinusiens localises de stade IE. Dans les stades plus avances ou avec les tumeurs volumineuses, l’association therapeutique represente la meilleure option therapeutique a l’instar des autres lymphomes de la sphere ORL.

We report three cases of Hodgkin's disease (HD) involving the nasopharynx. Their clinical presentations, morphological and immunohistochimical features and their therapy modalities are discussed. The patients were aged 36, 41 and 77, presenting with increasing bilateral nasal obstruction in one case and a cervical mass in the two others. Histological study showed mixed cellularity type of HD in all cases. The Reed Sternberg cells expressed both of CD15 and CD30 in one case, and only one of them in the other cases. In one case, LMP1 was detected, CD20 and CD3 were not. HD of nasopharynx should be differentiated from EBV-associated lymphoproliferations. The treatment is based on radiotherapy that can be associated to neoadjuvant chemotherapy if nodes are involved.

The concept of larynx preservation in locally advanced laryngeal or hypopharyngeal squamous cell carcinoma has evolved during the last three decades, especially with the advancement of nonsurgical strategies. These nonsurgical strategies include: (1) radiotherapy alone; (2) concomitant chemoradiotherapy (CCRT); and (3) induction chemotherapy followed by radiotherapy or CCRT and concurrent anti-epidermal growth factor receptor (EGFR). To date, the best approach for larynx preservation has yet to be defined. In this article, we review and discuss important recent randomized phase II/III trials investigating larynx preservation in order to facilitate the selection of an appropriate strategy in the clinical setting. However, the decision of larynx preservation should always be a multidisciplinary approach.

L\'hemangiome larynge de l\'adulte est extremement rare et presente des specificites histologiques et evolutives. Il peut representer un veritable defi therapeutique. Les auteurs rapportent 9 cas d\'hemangiomes larynges de l\'adulte colliges retrospectivement sur une periode de 20 ans (1984- 2004). Il s\'agissait de 6 hommes et 3 femmes, âges en moyenne de 46 ans (17 a 75 ans). Les signes d\'appel etaient la dysphonie dans tous les cas, associee a des hemorragies de sang rouge exteriorisees par voie buccale chez 3 patients. Le diagnostic reposait dans tous les cas sur la laryngoscopie directe qui retrouvait une tumefaction glottique de couleur bleutee, a surface lisse. Une biopsie avec examen anatomo-pathologique a ete systematique. Tous les patients ont beneficie d\'une exerese instrumentale de la masse par voie endoscopique, avec hemostase locale. Deux recidives ont ete notees, pour lesquels une seconde exerese instrumentale par voie endoscopique a ete realisee avec une evolution favorable. Le recul moyen etait de 9 ans. The adult laryngeal hemangioma is extremely rare and presents histological and evolutionary particularities. It can represent a real therapeutic challenge. The authors report 9 cases of adult laryngeal hemangiomas over a period of 20 years (1984-2004). It was about 6 man and 3 women, old on average of 46 years ( 17 - 75 years). The signs of appeal were the dysphonia in every case, associated to bleedings of red blood exteriorized with oral way in 3 cases. Diagnosis based in every case on the direct laryngoscopy which found a glottic tumefaction of bluish colour, in smooth surface. A biopsy with anatomo-pathological exam was realized in every case. All the patients benefited from an instrumental excision of the mass by endoscopic way, with local haemostasis. Two recurrences were noted, which benefited from a second endoscopic instrumental excision, with a favorable evolution. Average follow up was of 9 years. Journal Tunisien d\'ORL et de chirurgie cervico-faciale Vol. 16 2006: pp. 5-7

The aim of this retrospective study is to detennine prognostic factors for response to CT and survival, in patients (pts) treated with neoadjuvant CT and radiotherapy (RT) for locoregionally advanced non-metastatic NPC. From February 1988 to December 1992, 100 untreated pts received 3 courses of CT regimen with Cisplatin 100 mg/m2 Day l and Adriamycin 30 to 50 mg/m2 Day l for group I (36 pts), 50 to 75 mg/m2 for group II (39 pts) and 75 to 90 mg/m2 for group III (25 pts), repeated every 3 weeks, followed by locoregional RT. Response was assessed after 1 cycle of CT, before and after RT, by the measurement of node areas. Pts characteristics: 70% were men, median age 36.6 years (11 – 66 yrs); T1T2 = 26 pts, T3 = 25, T4 = 49, N2 = 39, N3 = 61; histology; UCNT = 64, non-UCNT = 36. All characteristics were well balanced in the 3 groups. The mean response rate after I cycle was 54.9%, after 3 cycles 78.8%. After RT, we observed 83% of CR, 14% PR and 3% of failure. Twenty seven pts relapsed. With a mean follow up of 27 month, the 3-yrs overall survival (OS) was 73.5% and disease free survival (DFS) 64.2%. Age, sex, node area, histology, T, N, Adriamycin dose-intensity, clinical response to CT, duration of RT and delay between CT and RT were variables studied in univariate and multivariate analysis (Cow model). In univariate study, the factors influencing significatively as and DFS were response after 3 cycles of CT (P Conclusion High dose Adriamycin regimen seems to improve the response rate to CT, which is correlated to as and DFS. Further studies with dose escalation of Adriamycin and growth factors should be done to increase the survival of the bad prognostic pts.

Nerve tumors arising from the sympathetic chain are uncommon slow-growing tumors and represent a diagnosis challenge. Their malignant degeneration is rare. Definitive pre-operative diagnosis may be difficult as investigations are not usually helpful. We report the case of a 23-year old woman who presented with an asymptomatic solitary left cervical swelling. She was evaluated with sonography and computed tomography. Complete surgical excision of the lesion was carried out and histologic examination revealed a schwannoma. Post-operatively, the patient showed clinical findings of Horner’s syndrome. Pathologic and radiological evaluation, differential diagnosis of this neoplasm and its management are discussed.

Primary thyroid lymphoma (PTL) is defined as a lymphoma involving only the thyroid gland or both thyroid gland and neck lymph nodes, without contiguous spread or distant metastases from other areas of involvement at diagnosis. Despite its rarity, PTL should be promptly recognized because its management is quite different from the treatment of other neoplasms of the thyroid gland. In the present study, we report a case of PTL treated by surgery and adjuvant chemotherapy. Otherwise, literature review allowed us to define main characteristics of this located lymphoma.