[Trichoblastic carcinoma]. / Un carcinome trichoblastique.

INTRODUCTION: Trichoblastic carcinoma is a rare malignant adnexal tumor. Its presentation is non-specific and the diagnosis is always histological. CASE REPORT: A 56-year-old patient presented with a trichoblastic carcinoma of the eyelid and eyebrow. It was located on the lower end of the scar, 7 years after the incomplete excision of a tumor. There was a large subcutaneous extension of the tumor, well beyond the visible margins of the skin lesion. The treatment was a broad surgical excision followed by radiotherapy. There was neither local recurrence nor metastasis 20 months later. DISCUSSION: This clinical presentation was atypical and had never been reported. We discuss the therapeutic management of this rare tumor.

[Mucoepidermoid carcinoma of the parotid gland in young children]. / Carcinome mucoépidermoïde de la glande parotide chez le petit enfant.

INTRODUCTION: Mucoepidermoid carcinoma of the parotid gland is a rare malignant tumor mostly affecting adults. It accounts for 1 to 3 % of head and neck malignant tumors. It is very rarely observed in children under 10 years of age. OBSERVATION: A four-and-half-year-old boy was brought to consultation in our department, in June 2006, for a left cervical swelling having progressively increased over the previous two years. Cervical US confirmed the presence of a 2.3cm long tumor of the left parotid area, hyperechogenic and heterogenic, associated to a homolateral lymphadenopathy in the jugular and carotid region (zone II), 2cm in diameter. A total parotidectomy preserving the facial nerve was performed. The biopsy extemporaneous histological examination suggested a low-grade mucoepidermoid carcinoma. Surgery was completed by functional neck dissection. DISCUSSION: Our four-and-half-year-old patient was one of the youngest cases ever reported. Malignant parotid gland tumors are very rarely observed in children: less than 19 cases have been published worldwide. Its occurrence in a young child under ten years of age is very rare. Mucoepidermoid carcinoma accounts for one third of all malignant parotid gland tumors.

[Langerhans cell histiocytosis of the thyroid]. / Histiocytose langerhansienne de la thyroïde.

Langerhans histiocytosis is a disease with a very diverse clinical spectrum and a very varied prognosis. The thyroid localization is relatively rare, and raises diagnostic and therapeutic difficulties. Diagnosis often requires recourse to clinical, radiological and pathological confrontation. Combined medical and surgical treatment is indicated and requires close multidisciplinary cooperation. On the basis of a new observation and data in the literature, we present a progress report on the clinical and therapeutic options preferred by various authors.

[Desmoid tumor of the parotid gland]. / Tumeur desmoïde de la glande parotide.

INTRODUCTION: Desmoid tumor is a benign microscopic tumor that belongs to the group of the deep fibromatosis. It usually arises from facial or musculoaponeurotic structures in the abdomen but rarely is located in the head or neck. Locally, it is characterized by a massive invasion with strong potential for recurrence. CASE REPORT: A 37-year-old woman was hospitalized for a swelling of the left parotid gland region which had progressed for eight years. Physical examination revealed a tumor in the parotid gland, which was hard and adherent deeply, measuring 4 cm/3 cm and sensitive to palpation; without satellite nodes nor facial paralysis. The treatment consisted in a superficial parotidectomy. Pathology was in favor of a desmoid tumor. After an 18-month follow-up, the patient is healthy without any sign of recurrence. DISCUSSION: Desmoid tumors are deep fibromatosis characterized by their slow growth and especially by considerable infiltration of the adjacent structures but without potential for metastasis. Although very rare, the cervical localizations are especially aggressive. This clinical case illustrates the clinical features of this rare affection and offers matter for discussion of therapeutic difficulties.

[Neuroendocrine carcinoma of the parotid gland]. / Carcinome neuroendocrine de la parotide.

INTRODUCTION: Neuroendocrine carcinomas are rarely observed in the parotid gland. CASE REPORT: A 65-year-old woman with an uneventful history was hospitalised for a tumor in the left parotid gland which had progressed for 8 months. The clinical manifestations and the radiological findings were in favour of a malignant process involving the parotid gland. Search for local and regional extension was considered to be negative. The surgical procedure was very difficult but enabled conservative resection of the tumor by total parotidectomy. Pathology results were in favour of a neuroendocrine carcinoma of the parotid gland. External radiotherapy was proposed, but interrupted by the patient after having received 40 Gy. After a 13 month follow-up, the patient was in good general condition without any sign of relapse or metastasis. CONCLUSION: Parotid neuroendocrine carcinomas are rare but need to be individualized owing to their poor prognosis with frequent occurrence of hepatic and pulmonary metastasis.

[Primary sub-mandibular gland tumours: experience based on 68 cases]. / Les tumeurs primitives de la glande sous-maxillaire: à propos de 68 cas.

UNLABELLED: Sub-mandibulary gland tumours are less common than tumours of the parotid and pose many clinical and therapeutic challengers. OBJECTIVE: We report our experience of sub-mandibular tumours and review the literature. PATIENTS AND METHODS: Retrospective studies of sub-mandibular gland tumours presenting to our department between 1986 and 2000. RESULTS: 68 cases were reviewed comprising 37 benign and 31 malignant tumours (15 females and 33 males). Average age of patient was 46 years and all presented with a sub-mandibular swelling. Clinical suspicion of malignancy was associated with symptom of pain, cervical adenopathy, nerve palsy, skin and/or bone invasion. CAT Scans were performedd to assess tumour extent / invasion. Definitive diagnosis was by complete excision and pathological examination. Pleomorphic adenoma (n= 32) were the most frequent benign tumours. For malignant lesions (n= 31) the most frequent were: Adenoid cystic carcinoma (n= 10), epidermoid carcinoma (n= 5), adenocarcinoma (n= 5), mucoepidermoid carcinoma (n= 3), malignant non Hodgkinien lymphoma (n= 5). Treatment was by total surgical excision of the submandibular gland for the begnin tumours. For the malignant lesions it was associated acording to their extension with other anatomical region or in case of adenopathy with neck dissection. Radiotherapy was performed in 24 cases and chemotherapy in 10 cases. CONCLUSION: Malignity in sub-mandibular gland tumours is more frequent than in the parotid gland. Any delay in diagnosis or inappropriate management may result in a poor prognosis for the patient.

Orbital osteoma: three case reports.

INTRODUCTION: Osteoma is the most frequent benign tumor of paranasal sinuses. It is generally asymptomatic and usually discovered by chance during radiological imaging. Three cases with orbital extension are reported and discussed. OBSERVATIONS: 1st observation: Mrs. M.N. is an 18 years-old caucasian female who presented a stony orbital deformity associated with chronical dacryocystitis. Tomography of the orbit revealed a probable ethmoidal osteoma compressing the lacrimal canals. The management consisted in a surgical excision of the whole tumor by external approach, associated with a dacryocystorhinostomy. The patient's follow-up for the last 12 months was normal. 2nd observation: Miss K.A. is a 16 years-old caucasian female who came to consultation for a swelling of the medial angle of the left eye. Computed tomography images showed a fronto-ethmoidal process displaying a bone density consistent with an osteoma. The tumor was removed through an external ethmoidectomy. The patient was free of symptoms at 6 months follow-up. 3rd observation: Mrs. F. Z. is a 45 years-old patient who presented a 5 years history of right painful exophthalmos. The computed tomography was consistent with a fronto-ethmoidal osteoma with intraorbital extension near the optic nerve. The surgical excision was limited to the intraorbital portion. Postoperative complications included ptosis and diplopia. DISCUSSION: Osteomas most commonly affect the fronto-ethmoid sinuses. They rarely show intraorbital extension or cause intracranial complications. They are generally asymptomatic. Symptoms are generally of late onset and are a consequence of tumoral growth and compression of neighbouring organs, as it is the case in our patients. Tumoral exophtalmos is the major ophthalmological sign. The computed tomography is of a great contribution, not only for diagnosis but also for the choice of the surgical approach. The classical surgical technique consists generally in a surgical excision of the osteoma. This surgery may induce ocular or neurochirurgical complications. CONCLUSION: Osteomas of the paranasal sinuses are usually asymptomatic. If they become voluminous, they may cause orbital manifestations and serious complications. The rarity of ethmoidal osteoma with orbital growth made our cases interesting to report.

[Tuberculosis of the parotid gland]. / Tuberculose de la glande parotide.

INTRODUCTION: The aim of this study is to emphasize the difficulties of diagnosing parotid gland tuberculosis because of its non specific symptoms and to incite the physician to seek it more frequently. MATERIAL AND METHOD: Six cases of parotid gland tuberculosis were diagnosed within a 4-year period and included in a retrospective study. RESULTS: There were two men and four women, mean age 45 years. Four patients underwent superficial parotidectomy with frozen section analysis which enabled us to rectify the diagnosis. Medical treatment was based on a 6-month course of bactericidal chemotherapy. Outcome was favorable in all the cases with nine months average follow. DISCUSSION: Symptoms of parotid gland are misleading, pathologic findings are of increasing importance for diagnosis, which with the new serology techniques may improve further.

[Hydatic cyst of thyroid gland]. / Kyste hydatique de la thyroïde.

INTRODUCTION: Primary hydatid cyst of thyroid gland is an exceptional localization. The thyroid gland is an uncommon site even in Morocco where echinococcal disease is endemic. The aim of this study was to report our experience of this rare disease, and to review diagnosis problems and management. PATIENTS AND METHOD: Retrospective study of six patients with primary hydatid cyst of thyroid gland during a 4-year period. RESULTS: We report six cases of primary hydatid cyst of thyroid gland. Symptoms were isolated thyroid nodules. Diagnosis was based on echography and echinococcal immunologic test. However, hydatic origin was suspected in only 50% of patients preoperatively and immunologic test had 33% false positive rate. Surgical management was a lobo-isthmectomy with a total resection of the cyst in four cases (67%) and a resection of the cyst prominent dome in two cases (33%). Diagnosis was always confirmed by macroscopic aspects and pathology. In all cases, the postoperative course was uneventful and no recurrence occurred with a 19 months follow up. CONCLUSION: Primary hydatid cyst of thyroid gland is a potential but rare situation. Positive diagnosis can be difficult during preoperative period. Cyst size and diagnosis doubt are two crucial criteria to decide the optimal surgical strategy (lobo-isthmectomy with total cyst resection vs resection of the cyst prominent dome).

[Thyroid tuberculosis]. / Tuberculose thyroïdienne.

OBJECTIVES: The aims of this study were to emphasize the difficulties of diagnosing thyroid tuberculosis because of its non specific symptoms and to encourage physicians to seek it more frequently. MATERIAL AND METHODS: Six cases of thyroid tuberculosis diagnosed within a 3-year period were included in a retrospective study. RESULTS: There were two men and four women, mean age 45 years. Four patients underwent lobo-isthmectomy with frozen section analysis which enabled us to rectify the diagnosis. Medical treatment was based on a 6-month course of bactericidal chemotherapy. Outcome was favorable in all the cases with 17 months average follow up. CONCLUSION: Symptoms of thyroid tuberculosis are misleading, pathologic findings are of increasing importance for diagnosis, which with the new serology techniques may improve further.

[Actinomycosis of the middle ear: case report]. / Actinomycose de l'oreille moyenne: à propos d'un cas et revue de la littérature.

INTRODUCTION: Actinomycosis of the middle ear are rare and often atypical and remain ignored for a long time. This delayed the diagnosis and obscured the forecast of the disease. AIM OF STUDY: Our objective is to give a progress report on this affection in the light of a new observation and data of the literature. CLINICAL CASE: 58 year old patient who consults of a left chronic otitis media with a posterior perforation. A CT scan had shown a filling of the middle ear in favour of a cholesteatoma. A tympano-mastoidectomy was realized and the histological examination of the product of excision had sowed that it acts of an actinomycosis of the middle ear. The treatment consisted of penicillin for 5 months. The continuations are good after 14 months of follow-up. CONCLUSION: The actinomycosis of the middle ear must be known to evoke the diagnosis in various clinical circumstances. Its diagnosis makes it possible to adopt an adapted and non aggressive therapeutic attitude, avoiding heavy surgical gestures, sources of complications or after-effects. The antibiotherapy containing penicillin is the essential element, either separately, or in complement of gestures of surgical drainage.

[Benign cementoblastoma. Apropos of a case and review of the literature]. / Le cémentoblastome bénin. A propos d'un cas et revue de la littérature.

The cementoblastoma is a rare benign tumor of odontogen origin that interests the roots of the teeth especially the lower molars. The evolution is often silent, the radiological exam is not specific, the diagnosis of certainty is histological. The surgical treatment assures the recovery without aftermath. The authors report a case of cementoblastoma at a patient of 37 years for which he has benefited of a resection by vestibular way and debate diagnosis and therapeutic modes of this affection.

[Chondrosarcoma of the jaw. A case localized to the mandible]. / Chondrosarcome des os maxillaires. Une localisation mandibulaire.

Chondrosarcoma is a malignant cartilaginous tumor rarely found in the jaw bone. Tumor progression is slow with strong propensity to local recurrence after surgical removal. The prognosis is poor and depends on the degree of differentiation and the quality of the resection. We report a case of mandibular chondrosarcoma that exhibited particularly massive extension to soft tissue. We discuss the epidemiology, diagnosis, treatment and follow-up of these tumors.

[Osteosarcoma of the jaws. Apropos of 12 cases]. / Les ostéosarcomes des maxillaires. A propos de 12 cas.

We report 12 cases of osteosarcoma (10 located in the mandible and 2 in the maxilla). There were 8 men and 4 women who were older than usually observed in osteosarcoma at other sites (8 of 121 patients were over 30 and the average age was 34 years). Clinical signs were variable. Bone tumefaction was the most frequent sign, with a diameter greater than 5 cm in most cases (10 out of 12). Surgery alone was used for 8 patients and radiosurgery for 3. Follow-up is available for 8 patients. All presented local recurrence with extensive tumors progression. All died within an interval of 3 to 30 months despite palliative surgery and/or radiotherapy or chemotherapy.

Parameningeal rhabdomyosarcoma. Report of 19 cases.

Parameningeal rhabdomyosarcomas are associated with a poor long term survival. This is due to the fact that they are aggressive tumours with early involvement of the skull base. The authors report a series of 19 cases between 1984 and 1995 with a relative proportion of children, male patients and tumours of embryonal type. Three patients were operated on and only one of them was found with tumour free margins. Systemic chemotherapy using the VAC-VAd protocol was administered in 18 patients and intrathecal chemotherapy was delivered in 5 patients with involvement of the skull base. Nine patients were irradiated with doses varying from 45 to 55 Gy. Twelve patients died from 20 days to 4 years after initial treatment; 6 were lost to follow up and only one patient is living without any evidence of disease after 11 years.