[Sarcomatoid carcinomas of the upper airways]. / Carcinomes sarcomatoïdes des voies aéro-digestives supérieures.

INTRODUCTION: The sarcomatoid carcinoma of the superior aerodigestive tracts is a rare malignant tumour which presents diagnostic and therapeutic challenges. MATERIAL AND METHOD: We report 11 cases of spindle cell carcinomas of the upper airways. RESULTS: Sex-ratio was 10:1 and the mean age was 57.3 years (30 - 75 years). Nine patients were smokers and 4 presented with a history of radiation exposure. Tumoral locations were as follows: larynx: 6, hypopharynx: 1, oropharynx: 1, nasopharynx: 1, oral cavity: 2. Histological diagnosis in 3 cases required the use of immunohistochemical studies. Four patients were first seen at an advanced stage. This left 9 patients and among them 7 received a curative treatment: 5 by surgery alone, 1 by surgery and radiotherapy, 1 by radiotherapy, 1 by chemo-radiotherapy for the nasopharyngeal lesion and 1 by chemotherapy alone. Two patients died from their disease before treatment. With an average delay of 15.4 months, the rates of global survival and disease-free were 5/9 and 4/9 respectively. CONCLUSION: The sarcomatoid carcinoma of the upper airways is rare, but not unusual. Its diagnosis benefits from progresses in immunohistochemistry, but also from advances in the field of molecular biology. Its treatment and natural evolution remain controversial.

Evaluation et prise en charge de l'envahissement mandibulaire dans les carcinomes epidermoides de la cavite orale et de l'oropharynx

Objectif : Le but de notre etude est l'evaluation de l'atteinte mandibulaire dans les carcinomes epidermoides de la cavite orale et de l'oropharynx. Materiel et methodes : Il s'agit d'une etude retrospective a propos de 34 patients colliges sur 6 ans (1999-2004) et ayantun carcinome epidermoide de la cavite orale et/ou de l'oropharynx avec envahisse- ment mandibulaire confirme a l'anatomopathologie.L'atteinte mandibulaire a ete evaluee par l'examen clinique; l'orthopantomographie; la tomodensitometrie; et par les constata- tions peroperatoires du chirurgien. Tous les patients ont eu un evidement ganglionnaire; une exerese tumorale associee a une mandibulectomie segmentaire interruptrice ou conservatrice. Resultats : L'envahissement mandibulaire a ete suspecte cliniquement chez 70;5des patients devant l'adherence de la tumeur a l'os. Apres examen clinique et imagerie; cette atteinte a ete diagnostiquee chez 88;2des patients. Dans 11;8des cas; l'atteinte n'a ete suspectee qu'en peroperatoire. L'examen histologique a confirme l'atteinte osseuse chez tous les patients. La mandibulectomie segmentaire a ete pratiquee chez 17 patients devant l'atteinte du canal mandibulaire. Vingt-huit patients ont eu une radiotherapie postoperatoire a la dose moyenne de 64 Gy. Les taux de recidive; de metastase et de deces etaient respectivement de 11;7; 17;6et 17;6pour les patients qui ont eu une mandibulectomie segmentaire; et de 23;5; 17;6et 23;5pour ceux qui ont eu une mandibulectomie conservatrice. Par ailleurs; 76;5et 29;5 des patients ayant eu respectivement une mandibulectomie segmentaire et une mandibulectomie conservatrice avaient une gene fonctionnelle importante lors de l'alimentation.Conclusion : L'examen clinique; l'imagerie et l'examen peroperatoire sont d'un apport capital dans l'evaluation de l'atteinte mandibulaire. Une atteinte epargnant le canal mandibulaire justifie une mandibulectomie conservatrice permettant d'avoir de meilleurs resultats esthetiques et fonctionnels tout en assurant un controle carcinologiquement satisfaisant

Les cancers de l'orbite et l'etude retrospective : a propos de 31 cas

Introduction : Les cancers de l'orbite sont rares. Ils sont caracterises par leur diversite histologique et leur agressivite locoregionale rendant le traitement difficile et mutilant. Dans ce travail; nous rapportons notre experience dans la prise en charge diagnostique et therapeutique de ces tumeurs. Materiels et methodes : Notre etude retrospective a concerne 31 cas de cancers de l'orbite colliges sur 13 ans (1993- 2005). Tous les patients ont beneficie d'un examen clinique complet; d'une imagerie du massif facial (TDM et/ou IRM) et d'une biopsie de la tumeur. Le traitement a ete base sur la chirurgie; la radiotherapie et/ou la chimiotherapie. Resultats : La symptomatologie clinique etait dominee par les signes ophtalmologiques et les algies faciales. L'imagerie a montre dans tous les cas un processus expansif tissulaire a point de depart orbitaire; avec lyse osseuse orbitaire chez 16 patients (51;6); une extension au massif facial chez 7 patients (22;6); endocraniennes chez 6 patients (19;4) et des formes bilaterales atteignant les deux orbites dans 3 cas (9;7) L'anatomopathologie montrait une predominance des lymphomes malins non hodgkiniens (32;3) et des carcinomes epidermoides (32;3); suivis des rhabdomyosarcomes embryonnaires (19;4). Douze patients ont ete traites par chirurgie et radiotherapie postoperatoire; dix patients par une association radio-chimiotherapie; et neuf autres par une chimiotherapie neo-adjuvante. La survie globale etait de 67;8 a 3 ans; 48;4 a 5 ans et 22;6a 10 ans

[Topical and systemic chemotherapy with 5-fluouracil in facial carcinoma secondary to xeroderma pigmentosum]. / Chimiotherapie locale et systemique par 5-fluorouracile dans les carcinomes de la face secondaires au xeroderma pigmentosum.

OBJECTIVE: to evaluate the feasibility, tolerance/toxicity and therapeutic efficacy of 5-fluorouracil (5-FU) by topical application and systemic use, in facial carcinoma associated with XP. METHODS AND PATIENTS: This is a prospective study of 10 patients with a median age of 22.9 years and a sex ratio of 4. Tumour lesions were facial mainly in the jugal and temporal region (36%). Chemotherapy indication was discussed in multidisciplinary committee, the topical 5-fluorouracil was applied locally twice a day, whereas the systemic treatment consisted of FUFOL protocol (every 4 weeks a combinaison of a short perfusion of 340 mg/m2 5-FU and preceded by an infusion of 20mg/m2 of folic acid, day 1 to 5); or C-FU protocol, combining continuous infusion of 5-FU (1 g/m2) 5 days associated with cisplatin (100 mg/m2, day 1) every 3 weeks. RESULTS: The median topical treatment duration was of 12 months in 10 patients. We noted a full tumoral regression in 10% of cases. Concerning systemic treatment, the median number of FUFOL cycles was 4 (2 to 6) and we observed a complete response in 6 patients (60%), partial in 2 cases (20%). Treatment was well tolerated in most cases except for the cutaneous irritation on 5-FU application zone and a 4 grade cisplatin otoxicity. CONCLUSION: Systemic or topical chemotherapy represents an interesting palliative option for facial carcinoma associated with XP, avoiding reiterated surgery and its cosmetic consequences.

[Regional failure of hypopharyngeal squamous cell carcinoma]. / Echecs ganglionnaires du carcinome épidermoïde de l'hypopharynx.

PURPOSE: Lymph nodes and distant metastases contribute to the poor prognosis of hypopharyngeal squamous cell carcinoma. The purpose of this study is to estimate the frequency, mode and prognosis factors related to regional and distant metastasis. PATIENTS AND METHOD: The authors' report is based on a retrospective study concerning 271 hypopharyngeal squamous cell carcinoma, compiled in the service of Surgery of Head and Neck Cancers of the Salah-Azaïz Institute (1977-2002). Frequency and histoclinical characters of cervical and distant metastases have been assessed, as well as their prognosis factors among 155 patients treated in a curative purpose. RESULTS: 39.1% of the tumours were classified N0, 24.4% N1, 10.7% N2 and 25.8% N3 (UICC 2002). The cervical nodal invasion was significantly more frequent for the classified tumors T3T4 than for the T1T2 (for pyriform sinus, postcricoid esophagus, posterior wall, and total hypopharynx, respectively : P =0.001, P =0.007, P =0.047 and P =0.0005). A cervical lymphatic evidement was preferred in thirty two patients. Among N0, 46.9% were N+; The frequencies of the capsular effraction were not significantly different for the N0N1 from for the N2N3 (P =0.11). The two and five years survival rates were respectively 32.5 and 20.5% for N0, and 10.9% and zero for N3. The survival differences after two and five years between the N0N1 and the N2N3 were significant (P =0.04). A regional failure was noticed for 18.5% of the patients. The tumoral site did not influence significantly the rate of nodal failure (P =0.98), neither the clinical status N (P =0.34). Capsular effraction was a significant factor for the regional failure (P =0.007). Distant metastasis significantly occurred more frequently among the patients initially classified N2N3 than those classified N0N1 (P =0.03), and in case of capsular effraction (P =0.0009). CONCLUSION: Hypopharyngeal squamous cell carcinoma has a high rate of lymph nodes metastasis, correlated to the local extension, and a high rate of occult nodal metastasis. Lymph node clinical status does not seem to have influenced the patients survival; however, capsular effraction constitutes a major prognosis factor of regional failure and distant metastasis. Distant metastases are frequent, particularly in case of wide local and regional tumoral extension.

[Hypopharyngeal squamous cell carcinoma: retrospective study of 149 patients]. / Carcinome épidermoïde de l'hypopharynx: étude rétrospective de 149 traitements à visée curative.

PURPOSE: Hypopharyngeal squamous cell carcinoma is associated to one of the most unfavorable prognosis among the cancers of the head and neck. The purpose of this study is to analyze its therapeutic modalities in the Salah-Azaïz Institute (Tunis) and to compare their results. PATIENTS AND METHOD: This retrospective study concerns 271 hypopharyngeal squamous cell carcinomas, compiled in the Carcinologic Surgery Department of Head and Neck of the Salah-Azaïz Institute over a period of 25 years (from 1977 to 2002). The average age of the patients was of 56 years; sex-ratio was on average of 1.2 (man/woman). The indication of a curative treatment was initially retained for 149 (55%) patients, who were the only ones retained for the analysis of results. RESULTS: We retained the indication of a protocol including surgery and postoperating radiotherapy for 26.2% of the patients. Postoperation mortality rate was 5.1%; the operating rate of morbidity was 46.2%. For 13.5% of the patients, postoperating radiotherapy was permanently interrupted because of a gradual deterioration of the patients' health in the course of treatment. We retained the indication of exclusive radiotherapy for 59.7% of the patients. The average age was of 56 years and the sex-ratio of 1.2. The external radiotherapy was conventional. Radiotherapy had to be permanently interrupted in progress in 32.6% of cases on account of an deterioration of the patients' health; the rate of morbidity of the radiotherapy was 33.3%. We indicated a protocol of conservation of organ with induction chemotherapy for 21 patients (14.1%). The average age was of 53 years (28-65 years) and sex-ratio (man/woman) of 0.5. The global survival was 25.5% at one year, 18.1% at two years, 11.4% at three years and 7.4% at five years. All the patients selected for chemotherapy died in the course of treatment. The rates of survival in two and five years according to protocols surgery-radiotherapy and exclusive radiotherapy were respectively: 21.5 and 12%, and 18.3 and 10%. The difference between the rates of survival of this two protocols is not significant (P =0.08). CONCLUSION: Although the induction chemotherapy entails a particularly high death rate in our series, the association surgery-radiotherapy and the exclusive radiotherapy seem to be similarly efficient for the treatment of the hypopharyngeal carcinoma.

[Metachronous cancers after treatment for undifferentiated nasopharyngeal carcinoma]. / Cancers métachrones après traitement des carcinomes indifférenciés du cavum.

OBJECTIVE: To collect second cancers occurring in the head and neck area after treatment by chemotherapy and/or radiotherapy for undifferentiated nasopharyngeal carcinoma in Tunisia. PATIENTS AND METHODS: This is a retrospective study of patients developing second cancers after treatment for nasopharyngeal UCNT by radiotherapy and/or chemotherapy. To be retained in this study, second tumour had to be histologically different from the initial UCNT, localised in the irradiated area and occurring after a minimal delay of 3 years. RESULTS: We collect 11 cases of second cancers occurring among 2346 patients treated from 1984 to 2001 in Tunisia (0.46%). Patients have been treated for nasopharyngeal UCNT mainly advanced T3-T4 (72%) or N2-N3 (63%). Median age was 20 years (11 to 48) with a sex-ratio of 0.3 (3 M/8 F). Treatment protocol included primary chemotherapy in 4 cases (adriamycin-cisplatin) or adjuvant (in 4) associated to the loco-regional irradiation at a mean dose of 72 Gy (70 to 75). Median delay of second cancer occurrence was 9 years (3 to 17). Tumors were epidermoid carcinomas in 4 cases, fibrosarcomas (2), osteosarcomas (2), glioblastoma (1) and basocellular carcinomas in 2. Second tumors have been treated by surgery alone in 4 cases and chemotherapy alone in 7 patients. No patient have been reirradiated. Median survival was 17 months, 4 patients died and 7 are still alive including 4 in complete remission (24+, 36, 36 and 48+ months) and 3 with progressing disease (8, 16 and 18 months). CONCLUSION: Even very rare, second cancers after treatment for UCNT need to be detected and have a poor prognosis.

[Thyroid papillary microcarcinoma. Salah Azaiz Institute experience]. / Microcarcinomes papillaires de la thyroïde. Expérience de l'Institut Salah Azaiz.

The incidence of thyroid papillary microcarcinoma appears to be increasing and therapeutic modalities remain controversial. This retrospective study concerns 25 cases, from 1978 to 1999. The circumstances of diagnosis were: a cervicotomy for thyroid nodule (88% of cases), cervical node metastases (8%), and osseous metastases (4%). The sensitivity of ultrasounds was 22.2%, that of scintigraphy was 9.1% and that of extemporaneous histological examination was 18.2%. Multifocality was found in 16% of cases and bilaterality in 16.6%. Treatment modalities were guided by histological examination. Adjunctive radioiodine ablation was used for 20% of the patients. The treatment of the osseous metastases was completed with external irradiation. The average follow up was 4.8 years (4 months to 19 years), with a rate of survival without disease of 100%.

[Thyroid oncocytomas]. / Les oncocytomes thyroidiens.

OBJECTIVE: Thyroid oncocytomas are rare tumors. They put problems of differential diagnosis enter malignant and benign forms. The modalities of the surgical treatment are controversial, in form as benign as malignant. Their prognosis is also debated. MATERIAL AND METHOD: Our retrospective study concerns 111 thyroid oncocytomas, so 6.5% of thyroid operated in our centre between 1981 and 2001. The sex-ratio of the patients was 0.16 with an average age of 41 years (15 to 72 years). RESULTS: All the fixed nodules, those associated to a recurrential paralysis, to tangible nodes or/and to microcalcifications on the radiography of the neck was malignant. Histological extemporaneous exam was not decisive in 16% of cases, among which 33% showed themselves malignant in the definitive exam, which put in evidence 9 carcinomas (8%). We treated benign oncocytomas by a loboisthmectomy for the isolated nodules, and the subtotal or total thyroidectomy for multinodular glands. For oncocytic carcinomas, we realized a total thyroidectomy with ganglionic taking and histological extemporaneous exam, followed by evidment in case of invasion (2 patients). For the 5 patients having presented a residual fixation, we obtained a white cartography after radioactive iodine administration. For the benign oncocytoma, we did ot notice any recurrences with an average drop of 28 months (2 months to 7 years). For carcinoma, 8 patients on 9 presented an actuarial survival without disease with an average drop of 44 months (18 months to 8 years). CONCLUSION: The diagnosis of malignancy of thyroid oncocytomas can be strongly evoked on however fickle clinical and radiological criteria; it can be eliminated only after definitive anatomo-pathological exam.

[Mucous malignant melanoma of the neck and face. Experience at the Salah Azaiz Institute (Tunis)]. / Mélanomes malins muqueux cervico-faciaux. Expérience de l'Institut Salah Azaiz (Tunis).

The mucosal melanoma of the head and neck is rare and of late diagnosis. This retrospective study concerns 17 cases brought together in 30 years. Sex-ratio was of 1.1, the average age of 58 years. Tumoral seats were the following ones: nasal cavity and paranasal sinus (n = 10), nasopharyngeal cavity (n = 2), gingival seat (n = 2), palate (n = 1), laryngeal seat (n = 1), middle ear (n = 1). Tumoral extension was classified an follows: stage I: 52.9%, stage II: 17.6%, stage III: 29.4%. Melanomas were achromic in 23.5% of cases. On the therapeutic plan, 47.1% of the patients were treated in a purpose palliative because of the importance of the tumoral extension. Seven patients (41.2%) were treated surgically; three among them received an additional radiotherapy for an insufficient tumoral excision or adenopathy in break capsular. Two patients (11.8%) were treated by exclusive radiotherapy. The rates of survival were 17.6% in 2 years, and 5.9% in 5 years. The average duration of survival was of 18.1 months. The causes of failure were essentially local and metastatic.