[Removal of lateral orbital wall for management of orbital cavernous hemangioma]. / Dépose de la paroi externe de l'orbite pour le traitement des hémangiomes caverneux de l'orbite.

INTRODUCTION: A relatively rare condition, cavernous hemangioma of the orbit poses a problem due to difficulty of the surgical approach. Modifications in surgical approaches and better understanding of these tumors preoperatively allow for less disfiguring elective surgery than before. MATERIALS AND METHODS: Five cases of orbital cavernous hemangiomas reviewed in our department between 2005 and 2009 underwent surgery by external lateral approach with removal of the lateral orbital wall. RESULTS: Resection of the tumor was complete in all cases, with one case of capsular rupture. Short- and long-term morphologic and aesthetic results were satisfactory in all patients. CONCLUSION: The external lateral approach with removal of the lateral orbital wall is a safe technique that allows good exposure of the tumor, facilitating its removal.

[Aneurysmal cyst of ethmoid bone: a case report]. / Le kyste osseux anévrismal de l'ethmoïde : à propos d'un cas.

AIM: To report on the value of imaging in the diagnosis and assessment of aneurysmal cyst of the ethmoid bone. CASE: A 12-year-old girl with a stuffy nose, revealing an aneurysmal bone cyst of the ethmoid, explored by CT, MRI, who had a preoperative arteriography with embolization. CONCLUSION: Aneurysmal bone cyst is a benign bone lesion which rarely involves the facial bones. Imaging plays an important role in the diagnostic approach, the assessment of the lesions, and the treatment using the techniques of interventional radiology.

[Congenital insensitivity to pain: difficulty of management]. / L'insensibilité congénitale à la douleur : difficultés de prise en charge.

INTRODUCTION: Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare disorder, most often of genetic origin. CASE REPORT: The authors present the case of two siblings, 10 and 13 years old, both followed-up since the age of 2 for CIPA diagnosed after discovering insensitivity to pain during iterative falls, burns, and of severe oro-digital self-mutilating behavior. Sural nerve biopsy and an electromyogram confirmed the diagnosis. DISCUSSION: CIPA with anhidrosis is a very rare disease. It is characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self-mutilating behavior, and sometimes mental retardation. Complications of this insensitivity (non-treated fractures, burns, and oro-digital mutilation) may be lethal. Treatment remains preventive. The patient must observe a very strict hygiene. Prevention for maxillofacial involvement consists in breaking the cycle of oral self-mutilation.

Solitary plasmocytoma: ghost tumour?

Solitary plasmocytoma is a rare tumour that belongs to the myeloid dysplasia group. The authors present an unusual evolution of this pathology through a clinical case. A 42-year-old patient was diagnosed with solitary nasosinusal plasmocytoma following biopsy of the right nasal fossa process; several weeks after this the tumour spontaneously regressed. Extramedullar plasmocytoma is a myeloid dysplasia that seldom evolves into multiple myeloma. Spontaneous regression has been described for some malignancies, implying immunological factors, but not previously for extramedullary plasmocytoma.

[Florid cemento-osseous dysplasia of the jaws]. / Dysplasie cémento-osseuse floride des maxillaires.

INTRODUCTION: Florid cemento-osseous dysplasia is a benign and rare tumor of the jaws. It is more commonly seen in middle-aged black women. Most cases are asymptomatic and are found during routine radiographic examination. OBSERVATIONS: We report two complicated cases of florid cemento-osseous dysplasia, one with facial deformity and the other with chronic osteitis. DISCUSSION: The diagnosis of florid cemento-osseous dysplasia is based on clinical and radiological features. The lesions are commonly bilateral and symmetrical.

[Orbital exenteration]. / L'exentération orbitaire.

PURPOSE: Orbital exenteration is a disfiguring surgery. The surgery is mostly performed for advanced neoplasms of the eyelid in an attempt to achieve cure with tumor free margins. Reconstruction is a real challenge, especially in elderly patients with significant comorbidities. PATIENTS AND METHODS: We operated 15 patients presenting with palpebral and orbital tumors, between January 2000 and December 2007. We collected the clinical data concerning patients, tumor, treatment, and recurrences. RESULTS: Ten male and five female patients with a mean age of 56 years at diagnosis presented with ulcerative palpebral malignant tumor, and impaired ocular motility. Basal cell carcinoma was the most common (80%). All patients underwent exenteration, (subtotal three, total eight, and extended four patients). The cavity was filled with a temporal muscle flap in ten cases, Mustardé flap in three cases, latissimus dorsi myocutaneous free flap in one case, and a jugal V-Y flap in one case. The mean follow-up was 23 months with good healing without radiotherapy tissue alteration. Four patients had a recurrence and one patient died from metastases. DISCUSSION: The goals of reconstruction are functional and esthetic. Given the initial tumoral extension, we choose to use a regional or microsurgical flap for functional reconstruction. The flap provides a good cutaneous coverage, rapid healing, closure of orbital nasal and sinus communications, or of orbital and cranial communications. It is not damaged by radiotherapy.

[Intramasseteric hemangioma operated by intraoral approach]. / Hémangiome du masséter opéré par voie intra-orale.

INTRODUCTION: Hemangiomas are benign vascular tumors, of unknown origin. Skeletal muscle localization account for less than 1% of cases. Masseter muscle localization is most common in head and neck (36%). In this case, treatment is usually surgery via an extra-oral approach which imposes parotidectomy with dissection of facial nerve branches. We report a case of intramasseteric hemangioma operated via an intraoral approach and we describe the benefits of this approach. CASE REPORT: A 34-year-old male patient with no prior history of trauma consulted for left masseter swelling having evolved for several years. Clinical examination revealed a soft, painless, well-defined swelling, about 5cm long, with a positive Wattle sign in the left cheek. The CT exam suggested a vascular lesion, located in the deep bundle of the masseter muscle. Preoperative embolization was followed by surgical exeresis via an intraoral approach. The post-operative evolution was uneventful. No recurrence was observed at one year of follow-up. DISCUSSION: Intraoral intramasseteric hemangioma exeresis is possible and does not seem to lead to more complications than with the facial approach. It prevents the significant drawbacks due to cutaneous incision, parotidectomy, and dissection of facial nerve branches via a facial approach.

Oto-Rhino-Laryngology (ORL) tumor presentation in a case of systemic AA amyloidosis.

A 64-year-old male with no underlying disease presented with the development of multiple skin nodules, loss of sensation in the extremities, hoarseness, macroglossia, and pain in the oral cavity. Direct laryngoscopy showed nodules involving the oral cavity, oropharynx, supraglottic region, and vocal cords. Biopsy from skin nodules showed amyloid deposits staining with Congo red. Immunohistochemical staining was used for AA protein and was positive. Biopsy from the oral floor was also positive for amyloid. Oto-Rhino-Laryngology (ORL) involvement has been reported in approximately 40 percent of AL amyloidosis patients, but does not appear to be frequent in AA amyloidosis. Cutaneous manifestations in AA amyloidosis are rare, although cases with lesions presenting as purpura are reported occasionally; we are not aware of other cases of ORL nodular involvement in systemic AA.

[Giant pleomorphic adenoma of the parotid gland]. / Adénome pléomorphe géant de la parotide.

INTRODUCTION: Slow evolution pleomorphic parotid adenoma may be diagnosed late. These giant tumors have a functional, esthetic, and social impact. They raise prognostic issues because of the risk of degeneration. We present a case of a giant pleomorphic adenoma involving the parotid glands and try to explain the reasons for the diagnostic delay and describe therapeutic specificities. CASE REPORT: A 54 year-old male patient consulted for swelling of the parotid region slowly evolving over the last 10 years. This lesion had been unsuccessfully treated by conventional methods. A painless 20cm long plurinodular mass was located in the right lateral cervical region. Its consistence was heterogeneous. The CT scan revealed a heterogeneous tumor with hyperdense and hypodense areas without any associate lesions. A total conservative parotidectomy was performed; the anatomopathological examination confirmed the diagnosis of a pleomorphic adenoma, weighing 1.2kg for a diameter of 19cm, without malignant degeneration. The postoperative evolution was uneventful. DISCUSSION: The recommended treatment for giant parotid adenoma is total conservative parotidectomy. Besides its functional and esthetic impact, the giant parotid adenoma is at high risk of malignant degeneration. Documentation on the nature of the lesion, its evolutive potential, and an early surgical management could limit its incidence.

[Primary extranodal ENT tuberculosis]. / La tuberculose primitive ORL extraganglionnaire.

OBJECTIVES: The aim of this sinusonasal study was to study several locations of ENT extranodal tuberculosis. MATERIAL AND METHODS: Our study focused on eight cases of tuberculosis in the cervicofacial region gathered in the ENT department of the Rabat Specialization Hospital (Morocco) between August 2004 and August 2006. They comprise two cases of rhinopharyngeal tuberculosis, two cases of tuberculosis, two cases of endobuccal tuberculosis (mandibular and lingual locations), and two cases of glandular tuberculosis (thyroid and parotid locations). RESULTS: These locations are rarely affected by tuberculosis. The clinical signs as well as radiology and endoscopy are not specific. The final diagnosis is based on histology. Bacillus cultures are necessary when caseum tuberculosis is not found, causing problems of differential diagnosis with granulomatous and malignant lesions. PCR provides quick bacterial antigen detection when cultures are negative. The treatment provides a quick and complete recovery while recurrences are infrequent. CONCLUSION: ENT tuberculosis is rare if excluding the nodal locations. It should be mentioned as a differential diagnosis, especially in endemic areas. Most often, this is a surprise diagnosis.

[Sinonasal adenocarcinomas reviewed. Prognostic value of WHO 2005 histological classification]. / Les adénocarcinomes nasosinusiens revisités. Intérêt pronostique de la classification histologique OMS 2005.

OBJECTIVES: The WHO 2005 histological classification separates sinonasal adenocarcinoma (ADC) into three classes: intestinal-type adenocarcinoma (ITAC), low-grade sinonasal ADC and high-grade sinonasal ADC. The goal of this study was to check the relevance of this classification on the prognosis of patients treated for ADC. PATIENTS AND METHODS: All the files of patients treated consecutively in the ENT department of the Montpellier University Hospital for ADC between 1980 and 2003 were retrospectively re-examined. Each case was reviewed for anatomical and pathological data based on the immunohistochemistry results according to the WHO 2005 classification, with a study of a panel of markers: cytokeratin 7 (CK7), cytokeratin 20 (CK20), Villin, CDX2 and EGFR. The epidemiologic data, the methods of treatment and the follow-up were studied. The survival probabilities were calculated using the Kaplan-Meier method and the survival graphs were compared using a log-rank test. RESULTS: Sixty-two files were reviewed. Twelve patients were reclassified into the adenoid cystic carcinoma category and excluded from the study. In the 50 remaining cases, there were 36 ITAC cases, four low-grade ADC cases and 10 high-grade dedifferentiated carcinomas. For all of the ADC cases, the total survival at 5 years and without recurrence was 64 and 52%, respectively. The analysis of the three subgroups showed a total survival of 72.2% for ITAC, 100% for low-grade and 20% for high-grade ADC with a significant difference (p=0.044). This immunohistochemical distinction was mainly based on the expression of CK20 found in 98% of the ITAC cases and absent in low- and high-grade ADC patients. CONCLUSION: The WHO 2005 classification for sinonasal ADC provides a valuable prognosis by showing a difference in the progression profile between ITAC, low-grade ADC and high-grade ADC. Moreover, broader studies should be conducted to investigate the different subtypes of ITAC.

[Maxilla-mandibular brown tumors as a first sign of parathyroid adenoma]. / Tumeurs brunes maxillomandibulaires révélant un adénome parathyroïdien.

OBJECTIVES: Through a novel observation of parathyroid adenoma revealed by brown tumors of the jaws and a review of the literature, the authors describe this rare mode of primary hyperparathyroidism discovery. MATERIAL AND METHODS: The patient was a 23-year-old woman who consulted for a recurrent tumefaction of the maxillary; histology showed reparative giant cell granuloma. RESULTS: The clinical examination found an osseous tumefaction in continuity with the zygomatic bone and a gingival tumefaction on the mandible symphysis. The radiological findings showed two osteophytic lesions: mandibular and maxillary. The phosphocalcic metabolism was disturbed and the parathormone rate was high. The etiologic search consisted of a MRI of the neck, which showed a mass behind the thyroid gland, suggesting a parathyroid adenoma. The diagnosis was confirmed at surgical exploration. After removal of this tumor, the blood calcium rate dropped sharply and the bone tumefaction progressively regressed. CONCLUSION: Brown tumors are a rare mode of parathyroid adenoma discovery, and the jaw location is exceptional. The diagnosis is based on the parathormone rate, and radiological exams generally find the etiology. Treatment is based on surgery of the parathyroid adenoma.

[Papillary adenocarcinoma on ectopic thyroid tissue]. / Adénocarcinome papillaire sur tissu thyroïdien ectopique.

OBJECTIVES: To report a case of papillary adenocarcinoma occurring on ectopic thyroid tissue in the hyoid bone region. MATERIAL AND METHODS: A 57-year-old patient consulted for a cervical mass appearing 1 year before, with no signs of compression. The thyroid function was normal. RESULTS: The examination showed a firm tumefaction, fixed on the hyoid bone, with undetermined limits, measuring approximately 8 cm. The thyroid gland was in the normal pretracheal position. The cervical tomodensitometry objectified an osteolytic process centered on the hyoid bone with a fleshy zone of tissue density and calcifications. Cervical MRI provided a more precise assessment of the extension of the mass and confirmed the integrity of the other structures, in particular the endo-larynx. The tumor was totally resected via a cervical approach. The pathologic study suggested a papillary adenocarcinoma on ectopic thyroid tissue. The treatment was completed by a total thyroidectomy with normal histological results. CONCLUSIONS: Papillary adenocarcinoma on ectopic thyroid is a very rare situation. Its diagnosis is histological. Its treatment is primarily surgical, sometimes associated with radioiodine I-131 therapy.

Plemorphic adenoma of the infratemporal space: a new case report.

Plemorphic adenoma is a frequent benign tumor of the major salivary glands. It could also develop from accessory salivary glands. We are reporting an extremely rare case of pleomorphic adenoma developing from the infratemporal space. The final diagnosis was based on histological confirmation. The treatment was mainly a complete resection via an anterior transmaxillary approach. Diagnosis, clinical behaviour, and treatment of pleomorphic adenoma of the infra-temporal space are reviewed from the literature.

[Primary tuberculosis of the parotid gland]. / Tuberculose primaire de la glande parotide.

OBJECTIVES: To report on a case of primary tuberculosis of the parotid gland in a 13-year-old child, and to stress the corresponding diagnostic pitfalls. CASE REPORT: A 13-year-old child presented with an isolated mass of the right parotid area. Radiological findings were in favour of a cystic lesion. Skin tuberculin test was positive. Bacteriological and histopathological examinations were realized after spontaneous fistulization of the mass, and allowed the diagnosis of tuberculosis of the parotid gland. Evolution was favourable under antituberculous chemotherapy. DISCUSSION: Localization of tuberculosis in salivary glands is rare. Diagnosis is difficult because there are no specific clinical, radiological or biological signs of the disease. Only bacteriological and histopathological findings can confirm the diagnosis. Fine needle aspiration cytology is very helpful, and permits to avoid abusive surgery. Treatment is based on antituberculous drugs and allows usually a favourable evolution.

[Cranio-cervical junction malformations: a rare cause of bilateral vocal cord paralysis]. / Les malformations de la jonction craniocervicale: une cause rare de diplégie laryngée.

OBJECTIVES: To report a rare and reversible cause of bilateral vocal cord paralysis in an adult. MATERIAL AND METHODS: A 44 years old woman has consulted for a laryngeal stridor with hoarseness. This symptomatology was preceded by chronic headache and dizziness. The examination noted bilateral vocal cord paralysis in paramedian position, and a cerebellum syndrome. The patient underwent urgent tracheotomy. RESULTS: A magnetic resonance imaging was performed for the etiologic research, and revealed a Chiari I malformation with a compression of the bulb. Neurosurgical decompression permitted resolution of the symptoms, and selvage of the tracheotomy was possible 2 months after surgery. CONCLUSIONS: Bilateral vocal cord paralysis is rare on Chiari I malformation. It's secondary to a direct compression or an ischemia of the brain stem. The oto-rhino-laryngologists have to know this etiology to make the diagnosis early and permit a neurosurgical treatment.

[Pleomorphic adenoma of the tongue base]. / Adénome pléomorphe de la base de langue.

INTRODUCTION: Pleomorphic adenoma is the most common lesion of minor salivary glands but is exceptional in the tongue base. REPORT CASE: We report the case of a 70-year-old woman with pleomorphic adenoma of the tongue base. The patient consulted for difficult swallowing, which developed over 4 months. Physical examination and computed tomography revealed a cystic tumour located in the right side of tongue base. The tumor was removed by transoral resection. The histological examination demonstrated a pleomorphic adenoma. DISCUSSION: We will discuss the diagnostic and therapeutically modalities of pleomorphic adenoma of the tongue base with a review of the literature.

[Cervicofacial cellulitis revealing cutaneous lymphomas]. / Dermohypodermites cervicofaciales révélatrices de lymphomes cutanés.

INTRODUCTION: The cervicofacial localization of cutaneous lymphomas is rare. These lymphomas usually present as a long-lasting and treatment-refractory papule or nodule. Lymphomas can also be revealed by cervicofacial cellulitis. CASES: We report 2 cases of cervicofacial cellulitis revealing a cutaneous lymphoma. The diagnosis was proved by multiple biopsies, performed because there was no clinical improvement in spite of an aggressive and adequate antibiotherapy. Our 2 patients were treated by radio and chemotherapy. DISCUSSION: Cutaneous lymphomas are lymphocytic proliferations stemming from cutaneous lymphoid tissue, without nodal, medullary, or visceral localization. Their clinical presentation is quite polymorphic, and cellulitis is one of the modes of revelation, especially forehead and neck localization. They have no portal of entry and are resistant to treatment. The diagnosis relies on histology, and biopsies must be performed if there is a suspicion of lymphoma. The treatment is radio and chemotherapy, and the evolution depends on the tumoral stage.

[Solitary bony plasmocytoma of the mandible]. / Plasmocytome solitaire osseux de la mandibule.

INTRODUCTION: Solitary plasmocytoma is a rare malignant tumor, belonging to the larger family of plasma cell proliferative diseases observed in bones. The mandible localization reported here is extremely rare. CASE REPORT: A 57-year-old man consulted for a tumefaction of the right mandible. Physical examination revealed a mandibular tumor with an osseous consistency covered by a healthy mucous. Radiologically, the tissue processed from the mandible, which presented a defect and an eroded cortical. Hemimandibulectomy was performed. Pathologic examination of the operative specimen demonstrated plasmocytoma. Complementary radiotherapy was performed. Outcome at 13 months was favorable, without recurrence of multiple myeloma. DISCUSSION: Plasma cell tumors are rare, about 10% of them are solitary plasmocytomas observed in intra- or extramedullar sites. Pathology is required for confirmation. An exhaustive assessment is needed to rule out multiple myeloma. Most solitary forms are observed in the spine. Appropriate treatment combines surgery and radiotherapy. Careful surveillance is required because of the frequent progression to multiple myeloma.

[Mesenchymal chondrosarcoma of the mandible]. / Chondrosarcome mésenchymateux de la mandibule.

INTRODUCTION: Mesenchymal chondrosarcoma is a rare form of chondrosarcoma and mandibular localization is rare. OBSERVATION: We report a case of mesenchymal chondrosarcoma of the mandible in a 23-year-old patient who consulted for a voluminous tumor of the right mandible. Radiological findings suggested a malignant tumor. A hemimandibulectomy and a pelvectomy were performed. The histological assessment showed zones of neoplastic cartilage and small ovoid cells, typical of mesenchymal chondrosarcoma. DISCUSSION: Mesenchymal chondrosarcoma is a rare malignant tumor of skeletal and extra skeletal origin. The most frequent localizations are the femur, the ribs, and facial bones. Histologically, these tumors have a typical biphasic pattern consisting of both small cells and islands of atypical cartilage. Because of its rich vascular component, this lesion has often been confused with hemangiopericytoma. Surgery is the usual treatment. The prognosis is very poor with a high risk of relapse and metastasis.